How does spina bifida affect life expectancy. What are the different types of spina bifida. Can spina bifida be treated with fetal surgery. How is spina bifida diagnosed and managed after birth. What are the long-term challenges for individuals with spina bifida.

Understanding Spina Bifida: A Comprehensive Overview

Spina bifida is a complex birth defect that affects the spine and spinal cord. This condition occurs when the neural tube, which forms the brain and spinal cord, fails to close properly during early fetal development. The result is an opening in the spine that can lead to various neurological and physical challenges.

The severity of spina bifida can vary widely, ranging from mild cases with minimal symptoms to more severe forms that require extensive medical intervention. Understanding the different types, causes, and treatment options is crucial for those affected by this condition and their families.

Types of Spina Bifida

There are several types of spina bifida, each with its own characteristics and potential complications:

• Spina Bifida Occulta: The mildest form, often with no visible signs or symptoms.
• Meningocele: A moderate form where the protective membranes around the spinal cord protrude through the opening in the spine.
• Myelomeningocele: The most severe and common form, where both the membranes and spinal cord protrude, leading to significant neurological impairment.

Is there a genetic component to spina bifida? While the exact cause is not fully understood, research suggests that both genetic and environmental factors play a role. Some families may have a higher risk of having a child with spina bifida, and certain genetic mutations have been associated with an increased likelihood of the condition.

Fetal Surgery: A Groundbreaking Treatment Option

One of the most significant advancements in spina bifida treatment is fetal surgery. This innovative procedure allows surgeons to repair the spinal defect before birth, potentially reducing the severity of neurological damage.

The Fetal Surgery Process

For those who qualify for fetal surgery, the process typically involves:

1. Admission to the hospital around 25 weeks of pregnancy
2. Administration of betamethasone to promote fetal lung maturity
3. A surgical procedure lasting 1-2 hours
4. Close monitoring in the hospital for about four days post-surgery
5. Continued medication and modified bed rest for the remainder of the pregnancy

Are there risks associated with fetal surgery? Yes, like any surgical procedure, fetal surgery carries risks such as premature labor, membrane rupture, and potential complications for both the mother and fetus. However, for many families, the potential benefits outweigh these risks.

Post-Surgery Care and Monitoring

Following fetal surgery, careful monitoring is essential to ensure the best possible outcome for both mother and baby. This includes:

• Weekly follow-up visits to assess pain, wound healing, and signs of complications
• Regular ultrasounds to monitor amniotic fluid levels and fetal well-being
• Monthly measurements of fetal head size and growth
• Close communication with medical professionals regarding any concerning symptoms

How long does the recovery process take after fetal surgery? The initial recovery period typically lasts about two weeks, but mothers are often required to maintain modified bed rest and avoid work for the remainder of the pregnancy. The exact timeline can vary based on individual circumstances and any complications that may arise.

Alternative Management: Non-Surgical Approach During Pregnancy

For those who choose not to undergo fetal surgery or do not qualify for the procedure, careful monitoring throughout the pregnancy is crucial. This typically involves:

• Continued prenatal care with an obstetrician or transfer to a high-risk specialist
• Monthly ultrasounds to track fetal growth and monitor for signs of hydrocephalus
• Preparation for potential complications during and after delivery

Can spina bifida be managed effectively without fetal surgery? While fetal surgery has shown promising results in reducing certain complications, many individuals with spina bifida who do not undergo the procedure can still lead fulfilling lives with appropriate medical care and support.

Delivery Considerations for Babies with Spina Bifida

The method of delivery for babies with spina bifida depends on several factors, including whether fetal surgery was performed. Key considerations include:

• Mandatory cesarean section for mothers who underwent fetal surgery
• Decision between vaginal delivery or C-section for those who did not have fetal surgery, based on medical advice
• Delivery in a specialized center with access to a neonatal intensive care unit and pediatric specialists

Why is specialized care so important during delivery? The complex nature of spina bifida requires immediate access to specialized medical professionals who can address potential complications and provide optimal care for the newborn.

Immediate Post-Birth Care and Surgical Intervention

After birth, babies with spina bifida require immediate and specialized care. The process typically involves:

1. Close monitoring for neurological, respiratory, and infectious complications
2. Protection of the exposed spinal cord with a sterile bandage (if fetal surgery was not performed)
3. Comprehensive neurological assessment
4. Consultation with pediatric neurosurgeons, plastic surgeons, and other specialists
5. Surgical repair of the spinal defect within the first few days of life (if not previously repaired)
6. Potential placement of a shunt to manage hydrocephalus, if present

How quickly is surgical intervention typically performed after birth? For babies who did not undergo fetal surgery, the repair of the spinal defect is usually performed within the first few days of life to minimize the risk of infection and further neurological damage.

Long-Term Management and Quality of Life

Living with spina bifida often requires ongoing medical care and support throughout life. Key aspects of long-term management include:

• Regular medical check-ups and monitoring of neurological function
• Physical therapy and occupational therapy to maximize mobility and independence
• Management of bladder and bowel function
• Addressing potential learning disabilities and cognitive challenges
• Psychological support for individuals and families
• Adaptive equipment and assistive technologies as needed

What is the life expectancy for individuals with spina bifida? With modern medical care, many people with spina bifida can expect to live well into adulthood. Life expectancy has significantly improved over the past few decades, with some individuals living into their 50s and beyond. However, life expectancy can vary depending on the severity of the condition and the presence of associated complications.

Challenges and Achievements

While living with spina bifida presents unique challenges, many individuals with this condition lead fulfilling and productive lives. Advances in medical care, assistive technologies, and societal support have opened up new possibilities for education, employment, and personal relationships.

Do individuals with spina bifida face limitations in their daily lives? The extent of limitations varies greatly depending on the severity of the condition. Some individuals may require mobility aids or assistance with certain tasks, while others may have minimal physical limitations. With appropriate support and accommodations, many people with spina bifida pursue higher education, successful careers, and independent living.

Advances in Research and Future Prospects

Ongoing research into spina bifida continues to improve our understanding of the condition and develop new treatment approaches. Some areas of active investigation include:

• Genetic factors contributing to spina bifida risk
• Refinement of fetal surgery techniques to improve outcomes
• Development of tissue engineering approaches for spinal cord repair
• Exploration of stem cell therapies for neurological regeneration
• Improvement of assistive technologies and mobility devices
• Enhancement of educational and vocational support programs

What promising developments are on the horizon for spina bifida treatment? While it’s difficult to predict specific breakthroughs, researchers are optimistic about the potential for new therapies to further improve outcomes for individuals with spina bifida. Advances in fields such as regenerative medicine and neurotechnology hold particular promise for addressing some of the long-term challenges associated with the condition.

The Importance of Holistic Care

As our understanding of spina bifida grows, so does the recognition of the importance of holistic care. This approach considers not only the physical aspects of the condition but also the psychological, social, and emotional well-being of individuals and their families.

How can society better support individuals with spina bifida? Increasing awareness, promoting inclusive educational and workplace environments, and ensuring access to comprehensive healthcare and support services are all crucial steps in empowering individuals with spina bifida to reach their full potential.

Prevention and Public Health Strategies

While not all cases of spina bifida can be prevented, public health efforts have made significant strides in reducing the incidence of this condition. Key prevention strategies include:

• Folic acid supplementation before and during early pregnancy
• Prenatal genetic counseling for high-risk families
• Public education about the importance of prenatal care
• Fortification of foods with folic acid
• Research into environmental factors that may contribute to neural tube defects

Has the incidence of spina bifida decreased over time? Yes, the implementation of folic acid fortification programs and increased awareness of the importance of folic acid supplementation have led to a significant reduction in the incidence of spina bifida in many countries. However, continued efforts are needed to further reduce the occurrence of this condition worldwide.

The Role of Genetic Counseling

For families with a history of spina bifida or other neural tube defects, genetic counseling can play a crucial role in understanding risks and making informed decisions about family planning. Genetic counselors can provide information about:

• Inheritance patterns of spina bifida
• Available genetic testing options
• Strategies for reducing the risk of having a child with spina bifida
• Support resources for families affected by the condition

When should individuals or couples consider genetic counseling? Genetic counseling can be beneficial for anyone planning a pregnancy, but it is particularly important for those with a family history of neural tube defects, previous pregnancies affected by spina bifida, or certain medical conditions that may increase the risk of having a child with spina bifida.

Global Perspectives on Spina Bifida Care

The management and outcomes of spina bifida can vary significantly across different regions of the world. Factors influencing care include:

• Access to advanced medical technologies and specialized care
• Availability of fetal surgery and other innovative treatments
• Implementation of folic acid fortification programs
• Cultural attitudes towards disability and inclusivity
• Economic resources allocated to healthcare and support services

How do outcomes for individuals with spina bifida differ globally? In regions with access to comprehensive medical care and support services, individuals with spina bifida often have better long-term outcomes and quality of life. However, in areas with limited resources, challenges in accessing specialized care and support can lead to more significant complications and reduced life expectancy.

International Collaboration and Knowledge Sharing

Efforts to improve spina bifida care globally include:

• International research collaborations to advance treatment options
• Training programs for healthcare providers in developing countries
• Global initiatives to promote folic acid supplementation and fortification
• Partnerships between medical centers to share expertise and resources
• Advocacy efforts to increase awareness and support for individuals with spina bifida worldwide

What role can international organizations play in improving spina bifida care globally? Organizations such as the World Health Organization (WHO) and various non-governmental organizations (NGOs) can contribute by promoting best practices, supporting research, facilitating knowledge sharing, and advocating for policies that improve care and support for individuals with spina bifida in all regions of the world.

Spina Bifida Treatment Options and Information

What If I Choose To Have Fetal Surgery?

If you qualify for fetal surgery and decide to move forward, you will be admitted to the hospital at around 25 weeks for surgery. Prior to being admitted you will receive two shots of a medication called betamethasone to help your baby’s lungs mature in case the baby is born soon after fetal surgery. You will then be admitted to Women and Infants Hospital where you will have blood work drawn on the day of surgery.

Your actual surgical procedure will be performed at Rhode Island Hospital. Your team will consist of physicians and nurses from both Women and Infants Hospital (maternal fetal medicine and neonatology) and Rhode Island Hospital/Hasbro Children’s Hospital (pediatric surgery, pediatric neurosurgery and anesthesia) will perform the operation. The operation lasts one to two hours and you will recover in the post-anesthesia care unit (PACU, or recovery room) for several hours. You will then be transferred back to Women and Infants Hospital, where you will stay on the labor and delivery (L&D) unit for observation. Because this type of surgery is very invasive, you must be monitored closely for signs of labor, your water breaking, and fetal distress. Once you are stabilized on the L&D unit, you will be transferred to the Antepartum Care Unit, where you will stay until it is time to go home. You can expect to be in the hospital for about four days.

All patients must be on medication to help stop contractions until 36 weeks of pregnancy. You will need to be on modified bedrest for at least two weeks after surgery. Return to normal activity will depend on whether you develop contractions with increased activity. You will need to be out of work for the remainder of your pregnancy. After surgery, you should be cared for by a high-risk pregnancy service that will allow standardized postoperative care, ultrasound evaluation and delivery. If you are local, that care will be provided by the Maternal Fetal Medicine Department at Women and Infants Hospital and ultrasound surveillance will be provided by the Prenatal Diagnosis Center. You will need weekly follow up visits to assess any pain you are having, wound healing, signs of rupture of the membranes, and premature labor. Weekly ultrasounds to assess the amount of amniotic fluid, whether the membranes are intact, and fetal assessment will be scheduled. Monthly measurements of the baby’s head and growth will also be completed. 

You will be asked to stay in close contact with our nurse coordinator with any signs and symptoms of labor, membrane rupture, decreased fetal movement and other signs that the baby or you are having complications.  There may be times that you have to be admitted to the hospital for the duration of your pregnancy or even early delivery. 

What If I Choose Not To Have Fetal Surgery?

You should continue your routine prenatal care with your obstetrician or have your care transferred to a high-risk obstetrician for specialized care for both you and your fetus. You should discuss with your obstetrician if your care needs to be transferred to a high-risk obstetrician. Your fetus should be followed by monthly ultrasounds to monitor your baby’s growth, and for signs of worsening hydrocephalus. 

What Are My Delivery Options?

If fetal surgery has been performed, the uterus is more fragile and Cesarean section is mandatory. If you opt to have the spina bifida repaired after birth, route of delivery (either vaginal or C-section) will be determined by your obstetrician. 

A newborn with spina bifida is at risk for severe infections and other complications, so it is recommended that mothers deliver in a center with immediate access to a specialized neonatal intensive care unit and immediate availability of pediatric neurosurgeons and pediatric plastic surgeons.

What Will Happen at Birth?

After the birth, the baby with spina bifida will be monitored very closely for any neurological, respiratory or infectious problems. If the mother did not undergo a fetal surgery to repair the spina bifida, the exposed spinal cord will be protected with a sterile bandage to avoid further injury. Tests will be performed to determine which nerves are affected by the spina bifida.

A consultation with a pediatric neurosurgeon and pediatric plastic surgeon (as well as other specialists, such as urologists) will be obtained rapidly, and a procedure to repair the defect typically occurs in the first few days of life. If there is associated hydrocephalus, a shunt to drain the brain of excess CSF may be placed as well.

If the mother has had fetal surgery for the baby’s spina bifida, the baby will be admitted to the newborn intensive care unit (NICU) for observation, assessment of the incision, and evaluation for associated complications of spina bifida (hydrocephalus, Chiari-II malformation, clubfoot, etc.).

What Is the Long-Term Outcome for Spina Bifida Patients?

The long-term outcome for babies with spina bifida varies based on the location of the defect (the higher on the spine, the worse the chances of normal lower extremity function) and the presence of other complications.

Hydrocephalus is the most common associated condition; however, as long as the excess fluid in the brain is successfully drained, children can develop normally. Sometimes, the shunts malfunction or become infected, which requires further treatment and operations. A recent study of mothers who participated in the Management of Myelomeningocele study (MOMS) found enduring effects of prenatal surgery for myelomeningocele with improved motor functioning and a reduction in Chiari-II malformations and hydrocephalus, as well as better gross and fine motor functioning and quality of life, with reduced negative impacts on families[2].

Spina bifida is not usually deadly (although there is an increased chance of sudden death within the first two years). Neurological and motor function (particularly of the legs) as well as bowel and bladder function can be affected to varying degrees. Children and adults with spina bifida are at a higher risk of serious infections, including urinary and kidney infections.

References:

1. “The Management of Myelomeningocele Study,” sponsored by the National Institutes of Health.
2. Houtrow AJ, Thom EA, Fletcher JM, et al. “Prenatal Repair of Myelomeningocele and School-age Functional Outcomes.” Pediatrics. 2020;145(2):e20191544

It’s Time to Talk about Spina Bifida (and feel comfortable about it) 

When teenagers at a youth camp were asked what they would like to learn about in a survey conducted by ABC Medical, 70% responded, “being able to talk about my disability without feeling uncomfortable and answering questions.”

While Spina Bifida is the most common permanently disabling birth defect in the United States, a strikingly low number of people know about what it entails and/or comfortable talking about it with others.

So, we gathered some information and stories from those who live with spina bifida to provide talking points to anyone who’s looking for additional information and insights. If at least one kid feels less uncomfortable after reading what we have gathered here, we will feel good.

We should also add that, the things we discuss in this post are all personal stories. Everyone has a different way of doing things. Therefore, if you have an alternative way of doing something or a feedback, please comment below so we can consider adding them next time we revise this piece.

What is Spina Bifida and what should I tell others about it?

You should definitely know the scientific way to answer this question.

It literally means “split spine.”

Spina Bifida happens when a baby is in the womb and the spinal column does not close all of the way (See pic below). Scientists believe that it occurs due to a combination of genetic and environmental factors. Folic Acid intake before and during pregnancy is critical to prevention. According to the Spina Bifida Association website, women should take a vitamin with 400 mcg (0.4 mg) of folic acid each day during the years of their lives when they are possibly able to have children.

Every day, about 8 babies born in the United States have Spina Bifida or a similar birth defect of the brain and spine.

Original Source: Wikipedia

Here are some creative ways others have answered this question:

“Spina Bifida is like a broken wire. If the wire is broken, the message cannot get from one end of the wire to the other end. Because the spina is broken / not all there / developed the messages cannot get from one end to the other. I have explained this to kids that I work with and it works!”- Ryan Krygier, Grand Rapids, MI.

“If a child asks what Spina Bifida is I usually use the analogy of a zipper that doesn’t not zip all of the way and how Kaiden’s spine formed.   I tell them that God created him different and He does not make mistakes.” – Misty, Shelbyville, IN

“I was born with a hole in my back. When I was 3 months old, doctors closed that hole but I still have some problems, because of being born with it. It means that sometimes I don’t do everything like everyone else does. I have to change how I have to do it. I live my life and am married, have children, grandchildren and a career. I am just like you- just a little bit different.” Monica Still, Pennsylvania

What are some conditions that you can experience as a result of spina bifida? 

According to the Spina Bifida Association, some people can have some of the below conditions as a result of having spina bifida: 

What is one thing you wish people knew about spina bifida?

“The one thing that I wish people knew about Spina Bifida is that even though it might be extremely painful or very hard for you to do it sometimes, if you are determined and put your whole heart into it something, you can accomplish your goals that you’ve set for yourself. I know from experience how trying hard it can be and I know all about wanting to give up but none of us are about quitting. We all are fighters because of what we’ve dealt with having to push ourselves every day to just be healthy and not sick. ..so put all of that into your goals and you can do anything! Best to you all!” – Tiffany, Durham, NC

The thing I wish for most for other parents is to understand that it is okay if their children ask questions.  Many times we have had children come up to us and ask Kaiden what happened.   He will usually ignore them and becomes slightly annoyed… my answer is that he was born that way and his legs work differently than ours.  They usually shrug their shoulders and move along.   I think he gets annoyed because he doesn’t see it as any big deal and does not understand why they would not already know!  Please do not scold your child for asking or saying something you feel is “inappropriate”.  I use this as time that we can practice handling difficult situations before he has to handle them on his own.  It is a learning process for everyone.” Misty Schaff, Shelbyville, IN

Photo Original Source: Interview with Misty Schaff

“I really wish people knew that we grow up. I am lucky to be in the first generation of kids to grow up with spina bifida. We aren’t kids anymore. Adults have different needs than children and that needs to be recognized. Prevention is important but more research is needed to see how a person ages with Spina Bifida. More money must be spent on research into better procedures for improved outcomes for tethered cord releases, alternatives to invasive procefures to manage incontinence and improved shunts. ” Monica Still, Pennsylvania

Can children and adults with spina bifida enjoy life and get involved with sports and arts?

Well, of course!!! It’s easy to get offended by a question like this. Don’t! Instead, channel your emotions towards a constructive answer. This person clearly needs some education. 

According to the Spina Bifida Association website, with some guidance, children with Spina Bifida can lead full lives. “Most do well in school, and many play in sports. Because of today’s medicine, about 90% of babies born with Spina Bifida now live to be adults, about 80% have normal intelligence and about 75% play sports and do other fun activities. “

“When I was in school I played the flute and piccolo and was in competitions and marching band. The marching band was difficult for me due to the standing and band camp every year where we had to march in place for hours at a time run around the field with our instruments and had to move in a lot of different positions for the competitions. I also participated in concerts as well as every football game. I very much enjoyed it, but it wasn’t easy, and I pushed myself every day to be the best. I was first Chair every year and I was 1st in every competition I played at. We also played at graduation every year and I remember this specific year we played stairway to heaven by Led Zeppelin and it was so amazing that I literally cried. I can’t imagine anything in my life since then besides giving birth to my 3 beautiful healthy children that I have experienced such happiness and joy knowing that I was a part if this amazing school band. We were called “The Scarlett Knights” and every year in our competition except one we were number 1. This was the only hobby I bet really had or ever wanted…I was great at something and it gave me hope, and a sense of belonging.” Tiffany, Durham, NC

“Kaiden plays with the RHI (Rehabilitation Hospital of Indiana) Mini Pacer’s wheelchair basketball team.  Kaiden does not have to look far to find other athletes to look up to.  The RHI Racers team were the national champions in their division in 2015.  Kaiden also participates in adaptive swimming clinics and has tried adaptive archery through RHI. ” Misty, Shelbyville, IN

I’m a 19 yo with spina bifida. I enjoy drawing, riding horses, and listening to music. She is in AP art class in school and dreams of becoming a tattoo artist. ” Jade, Texas

On the Importance of connecting with others:

“From my personal experience relationships with others with Spina Bifida really helped me get through life, so if you have Spina Bifida or are expecting a Spina Bifida child I encourage you to make relationships with other Spina Bifida people or parents with other parents with Spina Bifida children. If you try something different, don’t give up even when you really want to because if I gave up trying to walk, I wouldn’t be able to walk at graduation this June. If you’re a Spina Bifida patient and get stressed out about the world around you, talk to someone who is your age and gets your Spina Bifida and everything about you. That’s what I do, and it makes me feel like a normal person. Be an advocate for what you believe in. For example, if people go against handicap laws, don’t stop fighting until your voice is heard and something’s been done about it.” Jade, Texas

“Connect with others who have the same level of spina bifida as you do or that your kid does. This is very important, because every level is different with spina bifida, just like in spinal cord injury. Another important thing to keep in mind is people need to find out for sure, if they have a latex allergy or not. Nowadays, when babies are born with spina bifida, parents are automatically told that their babies have latex allergy without going through the proper testing. If people with Spina Bifida knew for sure if they did or “did not” have that allergy, for products could be used.” Ryan Krygier, Grand Rapids, Michigan

I have recently joined a few support groups, such as the “Rolling with Spina Bifida.” The conversations are helping me with learning more about Spina Bifida as well as having the support system there.” Tiffany, Durham, NC

As we said before, everyone’s experience is very personal. Your age, your level of spina bifida, your support system can all impact the kind of lifestyle you have living with spina bifida.

How do you explain your “spina bifida” to others? Feel free to share in comments section. 

Spina Bifida Fact Sheet | National Institute of Neurological Disorders and Stroke

What is spina bifida?

Spina bifida is a birth defect that mainly affects the spine. Normally in the first month of pregnancy, a special set of cells forms the “neural tube. ” The top of the tube becomes the brain and the remainder becomes the spinal cord and structures around it.  In spina bifida, the neural tube doesn’t close all the way and some of the bones of the spine don’t close in the back.

Often, abnormalities of the brain (such as hydrocephalus, described below) accompany abnormalities of the spine because the neural tube closes first in the middle and then closure proceeds both upward and downward—meaning that if something happens that prevents normal formation of the spine, it may also prevent normal formation of the part of the brain that is forming (closing) at the same time.

The term neural tube defect describes a group of conditions, including spina bifida, that occur when the neural tube does not close all the way.

Each year approximately 1,400 babies born in the United States have spina bifida, according to the U.S. Centers for Disease Control and Prevention.  The exact cause of spina bifida is unknown.  There is no cure but most people with spina bifida lead long and productive lives.  Scientists suspect genetic, nutritional, and environmental factors all play a role in spina bifida.  People with spina bifida have different abilities and medical issues. 

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What are the types of spina bifida?

There are four types of spina bifida:   occulta, closed neural tube defects, meningocele, and myelomeningocele.  The symptoms of spina bifida vary from person to person, depending on the type and level of involvement.  

• Occulta is the mildest and most common form in which one or more bones of the spinal column (called vertebrae) are malformed.  The name “occulta,” which means “hidden,” indicates that a layer of skin covers the opening in the bones of the spine.  It is often found by accident on an x-ray or similar test.  This form of spina bifida very rarely causes disabilities or symptoms. 
• Closed neural tube defects are a diverse group of defects in which the spine may have malformations of fat, bone, or the membranes (called the meninges) that cover the spinal cord.  Many of these neural tube defects require surgery in childhood.  People with this type of spina bifida may have weakness of the legs and trouble with bowel and bladder control.  These issues may change or progress as children grow.  It is important to have close follow-up with doctors to minimize these changes as much as possible.
• Meningocele occurs when a sac of spinal fluid pokes through the spine.  This fluid is normally only around the brain and spine, but a problem with the bony covering over the spine allows it to poke out in this case.  The malformation contains no nerves and may or may not be covered by a layer of skin.  Individuals with meningocele may have minor symptoms.
• Myelomeningocele is the most severe form of spina bifida.  A portion of the spinal cord or nerves is exposed in a sac through an opening in the spine that may or may not be covered by the meninges.  The opening can be closed by surgeons while the baby is in utero or shortly after the baby is born.  Most people with myelomeningocele have changes in their brain structure, leg weakness, and bladder and bowel dysfunction.  Myelomeningocele is often called a snowflake condition because no two people with the condition are the same.  Typically, the lower in the spine the opening occurs relates to less symptoms in the person.  People with myelomeningocele require close follow-up with physicians throughout their childhood and lifespan to maximize their function and prevent complications such as kidney failure. 

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How does folic acid help?

Folic acid, also called folate, is an important vitamin for the development of a healthy fetus. Although taking this vitamin cannot guarantee having a healthy baby, it can help.  Studies show that women of childbearing age who add folic acid to their diets can significantly reduce the risk of having a child with a neural tube defect.  Therefore, it is recommended that all women of childbearing age take a daily vitamin supplement with 400 micrograms of folic acid before and during early pregnancy.  

Foods high in folic acid include dark green vegetables, egg yolks, and some fruits.  Many foods—such as some breakfast cereals, enriched breads, flours, pastas, rice, and other grain products—are now fortified with folic acid.  Many multivitamins contain the recommended dosage of folic acid as well.   

Women who already have a child with spina bifida, who have spina bifida themselves, or who have already had a pregnancy affected by any neural tube defect are at greater risk of having another child with a neural tube defect.  These women should take a higher prescription dose of folic acid before and early in pregnancy.

Spina Bifida Complications

Spina bifida’s impact is determined by the type of defect and in the case of myelomeningocele and closed neural tube defects the size and location of the malformation.   

Spina bifida complications may include:

• Abnormal sensation or paralysis, which mostly occurs with closed neural tube defects and myelomeningocele.  People with these conditions typically have some degree of leg and core muscle weakness and loss of feeling in the groin and feet or legs.  The sensation can be more significant on one side of the body.  Typically, the lower in the spine where the condition occurs results in less weakness and loss of feeling.  The strength and feeling do not improve with age due to nerve damage.  People with these types of spina bifida may lose strength and sensation as they grow and mobility can become more difficult with age.  People with these conditions may walk independently or use some combination of leg braces, walkers, crutches, or wheelchairs.  As they age, they may require more of these supports. 
• Chiari II malformation, in which parts of the brain called the brain stem and the cerebellum (hindbrain) protrude downward into the spinal canal or neck area.  It is almost always seen on advanced imagining of the brain in people with myelomeningocele, but it rarely causes symptoms.   When it does, this condition can press on the spinal cord and cause a variety of symptoms including difficulty breathing, swallowing, and arm weakness.  Surgery is sometimes required to reduce pressure in this area.
• Blockage of cerebrospinal fluid, causing a condition called hydrocephalus.  Hydrocephalus is the abnormal buildup of the fluid that surrounds the brain.  Most people with myelomeningocele have this condition, which is not seen in the other types of spina bifida.  This buildup can put damaging pressure on the brain.  Hydrocephalus is commonly treated by surgically implanting a shunt—a hollow tube—in the brain which allows drainage of the excess fluid into the abdomen where it is absorbed by the body.  The tube is tunneled under the skin and not very noticeable to others.  Another treatment option is an endoscopic third ventriculostomy or ETV, a procedure that creates a new path for the fluid to flow.
• Meningitis, an infection in the meninges covering the brain.  It can sometimes be associated with shunts.  Meningitis may cause brain injury and can be life-threatening.
• Tethered cord syndrome can occur with all forms of spina bifida, although it is very rare in individuals with spina bifida occulta.  Usually the spinal cord and nerves float freely.  A tethered cord means that there is some type of tissue attached to and pulling the cord down.  This can cause damage to the nerves and decrease feeling and strength, as well as problems with bowel and bladder control.  It is surgically treated if a person has symptoms.
• Bowel and bladder incontinence affect most individuals with myelomeningocele and closed neural tube defects.  The nerves at the very bottom of the spine control bowel and bladder function and don’t usually work properly in people with these types of spina bifida.  Most people with myelomeningocele and some types of closed neural tube defects need a regimen or other assistance to drain their bladders periodically or to have regularly scheduled bowel movements.  
• Learning disabilities, including difficulty paying attention, understanding concepts, impaired motor skills, impaired memory, and difficulty with organization and problem solving are commonly seen in children with myelomeningocele.  People with strength lower down in their legs tend to have less difficulty than those with more leg weakness.  Evaluation for an individualized education plan is recommended for all children with myelomeningocele. 
• Other complications such as skin ulcers, low bone mineral density, impaired male fertility, obesity, and kidney failure can be seen in people with myelomeningocele and neural tube defects as they age.  Additionally, people with myelomeningocele are at risk for precocious puberty (when changes to that of an adult occur too soon), sleep apnea, and depression. 

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How is spina bifida diagnosed?

In most cases, spina bifida is diagnosed before birth (prenatal).  However, some mild cases may go unnoticed until after birth (postnatal).  Very mild forms of spinal bifida are found when doing tests for other conditions or may never be detected.

Prenatal Diagnosis

The most common screening methods used to look for spina bifida during pregnancy are maternal serum alpha fetoprotein (MSAFP) screening and fetal ultrasound.  A doctor can also perform an amniocentesis test. 

• Maternal serum alpha fetoprotein (MSAFP) screen.  At 16 to 18 weeks of pregnancy, a sample of the mother’s blood is taken to measure the level of a protein called alpha-fetoprotein (AFP), which is made naturally by the fetus and placenta.  During pregnancy, a small amount of AFP normally crosses the placenta and enters the mother’s bloodstream.  Abnormally high levels of AFP may indicate that the fetus has spina bifida or other neural tube defect.  This test is not specific for spina bifida and cannot definitively determine that there is a problem with the fetus.  This means that a high AFP level alone is not enough to be sure the fetus has a neural tube defect.  If a high level of AFP is detected, the doctor may request additional testing, such as an ultrasound or amniocentesis.

The second trimester MSAFP screen may be performed alone or as part of a larger, multiple-marker screen.  Multiple-marker screens can look for neural tube defects and other birth defects, including Down syndrome and other chromosomal abnormalities.  First trimester screens for chromosomal abnormalities also exist but signs of spina bifida are not evident until the second trimester when the MSAFP screening is performed.

• Ultrasound.  A fetal ultrasound uses high-frequency sound waves to create a picture of the developing baby inside the womb.  It is highly accurate in diagnosing some birth defects during pregnancy, including spina bifida.  Fetal ultrasound can be performed during the first trimester (usually between 11-14 weeks) and the second trimester (usually at 18-22 weeks), and diagnosis is more accurate during the second trimester.
• Amniocentesis.  In this test, a doctor removes a sample of the amniotic fluid that surrounds the fetus and tests it for protein levels that may indicate a neural tube defect and genetic disorders.  

Postnatal Diagnosis

Closed neural tube defects are often recognized at birth due to an abnormal fatty mass, tuft or clump of hair, or a small dimple or birthmark on the skin at the site of the spinal malformation.  Spina bifida occulta is usually found when x-rays are done for another reason.

In rare cases, myelomeningocele and meningocele are not diagnosed during routine prenatal tests.  The baby will be diagnosed when they are born with a bubble on their back.  Babies with myelomeningocele and closed neural tube defects may have muscle weakness in their feet, hips, and legs that result in joint deformities first noticed at birth.   Mild cases of spina bifida (occulta, closed neural tube defects) not diagnosed during prenatal testing may be detected postnatally using ultrasound or X-ray imaging to look at the spine. 

Doctors may use magnetic resonance imaging (MRI) or a computed tomography (CT) scan to get a clearer view of the spinal cord and vertebrae.  To evaluate for hydrocephalus, the doctor will request a head ultrasound, CT or MRI to look for extra cerebrospinal fluid inside the brain.

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How is spina bifida treated?

Treatment depends on the type of spina bifida a person has.  Myelomeningocele and meningocele require a surgery to close the bubble shortly after birth to prevent infection such as meningitis.  Most people with myelomeningocele have hydrocephalus and most of them will need a shunt placed as an infant.  Children with a closed neural tube defect may need surgery to prevent further complications such as weakness and bowel and bladder function.  Generally, people with spina bifida occulta will not need any treatment.

Prenatal Surgery

Prenatal (before birth) surgery involves opening the mother’s abdomen and uterus (or womb) and sewing shut the abnormal opening over the developing baby’s spinal cord.  This is thought to protect the baby’s spinal cord from ongoing damage in the uterus.  The Management of Myelomeningocele Study (MOMS) showed that prenatal surgery to close the defect in the spinal cord improved outcomes compared to children who had postnatal surgery for spina bifida.  Data from the 2012 study showed that prenatal surgery reduced the need to drain fluid from the brain, improved mobility, and increased the chances that a child will be able to walk independently early on.  This study was funded by the National Institutes of Health’s Eunice Kennedy Shriver National Institute of Child Health and Human Development.

The procedure does not restore lost neurological function that happened before the surgery, but may prevent additional damage from occurring during the rest of the pregnancy.  Although prenatal surgery poses some risk to the fetus as well as to the mother, the benefits are promising and are still being studied.

Postnatal Surgery

In treating myelomeningocele and meningocele, the key priorities are to prevent infection from developing in the exposed nerves and spinal cord through the spinal defect, and to protect the exposed nerves and spinal cord from additional trauma.  Therefore, a child born with these types of spina bifida who has not undergone prenatal surgery will have surgery to close the defect and minimize the risk of infection or further trauma within the first few days of life.

Treatments for Complications

Some children with myelomeningocele and closed neural tube defects will need surgery to improve the alignment of their feet, legs, or spine.  Children with myelomeningocele usually have hydrocephalus and may require surgery to help drain fluid in the brain, such as the placement of a shunt or ETV.  Multiple surgeries may be required to replace the shunt, which may become clogged, infected, or disconnected.

Some individuals with myelomeningocele or closed neural tube defects require assistive devices for mobility such as braces, walkers, crutches, or wheelchairs.  The location of the defect on the spine often determines the type of assistive devices needed.  Children with a defect high on the spine will have little movement of the legs and will use a wheelchair for mobility.  Children with a defect lower on the spine usually have more strength in the legs.  They may be able to walk independently, or they may use crutches, leg braces, walkers, and wheelchairs depending on the activity.  Children with myelomeningocele usually have some degree of delayed mobility, so they are referred to physical therapists early on to maximize their strength and function.

Treatment for bladder and bowel dysfunction typically begins soon after birth.  Children with myelomeningocele and some closed neural tube defects have damage to the lowest spinal nerves which control typical bowel and bladder function.  Some children may be able to urinate typically, but most will need to drain their bladders with a catheter or thin tube 4-6 times a day to remain dry in between and to prevent kidney damage.  Kidneys are monitored closely so that medications or surgeries can be performed to prevent renal failure.  To prevent bowel accidents many people with myelomeningocele and closed neural type defects will use rectal medications or large volume enemas to have planned bowel movements.  Close follow-up with a spina bifida specialty clinic is recommended to develop a safe bowel and bladder program.

Treatment for progressive tethering of the spinal cord (called tethered cord syndrome) can be treated with surgery to help prevent further neurological deterioration.

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What research is being done?

The National Institute of Neurological Disorders and Stroke (NINDS), one of the National Institutes of Health (NIH), is the primary federal supporter of research on brain and nervous system disorders.  NINDS conducts research in its laboratories at the NIH in Bethesda, Maryland, and supports research through grants to major medical institutions across the country.  In addition to NINDS, other NIH Institutes support research on spina bifida and neural tube defects. 

Genetic studies.  In one study supported by NINDS, scientists are looking at the hereditary basis of neural tube defects and hope to find the genetic factors that make some children more likely to have a neural tube defect.  These researchers are also studying gene expression during the process of normal neural tube closure, which will provide information on the human nervous system during development.  Findings may lead to ways to prevent these disorders. 

In addition, NINDS-supported scientists are working to identify, characterize, and evaluate genes involved in neural tube defects.  The goal is to understand the genetics of neural tube closure and to develop information that will lead to improved clinical care, treatment, and genetic counseling.

Other scientists are studying genetic risk factors for spina bifida, especially those that reduce the effectiveness of folic acid in preventing spina bifida.  This study will shed light on how folic acid prevents spina bifida and may lead to improved forms of folate supplements. 

Developmental studies.  NINDS supports and conducts a wide range of basic research studies to understand how the brain and nervous system develop.  These studies contribute to a greater understanding of neural tube defects such as spina bifida and offer hope for new ways to treat and potentially prevent these disorders as well as other birth defects.

Surgery.  Results of the Management of Myelomeningocele Study (MOMS) (see Prenatal Surgery) showed significant benefit to the developing baby.  Because fetal surgery has shown promise, NINDS-funded researchers are also developing new methods, such as stem cell patches and tissue engineering, to add to the prenatal repair of spinal defects. 

Other NIH research efforts.  More information about research on spina bifida supported by NINDS and other NIH Institutes and Centers can be found using NIH RePORTER, a searchable database of current and past research projects supported by NIH and other federal agencies.  RePORTER also includes links to publications and resources from these projects.  Enter “spina bifida” to start your search.

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How can I help with research?

Consider joining a clinical study.  Both healthy individuals and those with a disease or condition can participate in medical research studies (sometimes called clinical trials or protocols) to help researchers better understand a disease and perhaps develop new treatments.  For information about clinical studies on disorders including spina bifida and how to participate in one, please contact the NIH’s Patient Recruitment and Public Liaison office at 800-411-1222 or visit the Clinicaltrials. gov website.

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Where can I get more information?

For more information on neurological disorders or research programs funded by the National Institute of Neurological Disorders and Stroke, contact the Institute’s Brain Resources and Information Network (BRAIN) at:

BRAIN
P.O. Box 5801
Bethesda, MD 20824
800-352-9424

Information also is available from the following organizations:

Eunice Kennedy Shriver National Institute of Child Health and Human Development
Information Resource Center
P.O. Box 3006
Rockville, MD  20847
800-370-2943 or 888-320-6942 (TTY)

Spina Bifida Association
4590 MacArthur Blvd. NW
Suite 250
Washington, DC 20007-4266
[email protected]
Tel: 202-944-3285; 800-621-3141
Fax: 202-944-3295

March of Dimes
1275 Mamaroneck Avenue
White Plains, NY 10605
[email protected]
Tel: 914-997-4488; 888-MODIMES (663-4637)
Fax: 914-428-8203

MedlinePlus
National Library of Medicine
National Institutes of Health

“Spina Bifida Fact Sheet”, NINDS, Publication date January 2021

NIH Publication No. 21-NS-309

Back to Spina Bifida Information Page

See a list of all NINDS disorders

Publicaciones en Español

Espina Bífida

Prepared by:
Office of Neuroscience Communications and Engagement
National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892

NINDS health-related material is provided for information purposes only and does not necessarily represent endorsement by or an official position of the National Institute of Neurological Disorders and Stroke or any other Federal agency. Advice on the treatment or care of an individual patient should be obtained through consultation with a physician who has examined that patient or is familiar with that patient’s medical history.

All NINDS-prepared information is in the public domain and may be freely copied. Credit to the NINDS or the NIH is appreciated.

Current State and Future Directions

Abstract

The care for adults with spina bifida is an important area to study. As increasing numbers of patients with spina bifida survive into adulthood, they expect to thrive and receive the best possible care into adulthood to maintain their health. Understanding the health needs in this emerging and changing population will help clinicians provide the best anticipatory care for adults with spina bifida and continue to improve outcomes. This will also impact pediatric care by improving the ability to determine preventive methods from early on and understand the impacts of pediatric care and decisions over the lifespan.

Keywords: adult, myelomeningocele, spina bifida, transition

Spina bifida (SB) occurs in about 2.7 to 3.8 per 10,000 live births,1 and more than 75% of people with SB live to adulthood.2 Since the advent of antibiotics to treat surgery-related infections, great strides have been possible in surgical interventions with implantable devices. The ventriculo-peritoneal (VP) shunt to treat hydrocephalus was invented in the mid 1950s.3 Since its introduction in the 1970s, clean intermittent catheterization (CIC) has improved the renal health and longevity for people with spinal cord lesions, reducing the risk of renal failure.4–6 Optimizing neonatal surgery for infants born with SB has reduced early morbidity and mortality, and now people with SB live to adult years and participate in fulfilling lives. The new knowledge frontier is to optimize the health and community participation of people with chronic health conditions such as SB.

This article serves to provide an overview of the major health and psychosocial outcomes among the adult SB population and report on areas where data are needed. We begin by discussing the medical needs of adults with SB and outline the difficulties and benefits patients face when making the transition from pediatric to adult care. We then discuss the role of a national registry and the ways in which outcome measures could help improve the medical management of adult patients with SB. Finally, we outline the predominant areas of health that affect daily life of individuals with SB and propose that improved community participation should be one of the ultimate goals that guide clinical decision-making in patients with SB.

Medical Care Needs Into Adulthood

A need for coordinated care for adults with SB was described in 1994 by Kaufman et al, who demonstrated the increased occurrence of preventable conditions in a cohort of patients with SB after their multidisciplinary clinic disbanded.7 This patient population was compared to a similar cohort in a clinic that continued to provide multidisciplinary care for adults. Data were collected from patient charts 3 years after closure of the clinic and revealed that 45% to 66% of the patients no longer received consistent care from primary care providers (PCPs) and specialists.7 This was especially significant among the older patients. When compared to a multidisciplinary clinic that stayed intact, the patients with the least consistent care had more comorbidities than the control patients.7 Even though the clinicians once seen by patients in this clinic were all still available after it disbanded, there was no coordinator of care. This resulted in a lack of follow-up care and missed treatments.7 Issues of aging with SB may be compounded by the lack of coordinated care.

Lack of regular care by a PCP may result in increased preventable secondary complications and higher rates of emergency department (ED) admissions.8–11 Previous research has reported increased rates of ED visits among adults with SB compared to the general population.8

Due to hospital or provider policy, insurance networks, or patient preference, people with SB may need to move to different providers or hospitals for their health care needs into adulthood. However, this transition may be met with resistance from patients and their parents as it can mean leaving multidisciplinary care clinics and instead receiving care from multiple adult specialists, who may not coordinate care or treatment decisions and may not have a specific knowledge or interest in treating SB. 8,12–15 One study found that the mean number of specialists seen by an adult with SB was 3.8 and that 71% of adults with SB reported difficulty in accessing medical care.16

Generally, transitioning to knowledgeable adult providers is appropriate and indicated for chronic childhood onset conditions, and there should be a planned, purposeful move to the new care provider or team.17 Transitioning to care providers can also be difficult due to reduced family support, concerns with executive skills, and lack of access to knowledgeable adult care providers.17

Transition From Adolescent to Adulthood

Although some pediatric SB clinics continue to provide care for their patients into adulthood, many have age limits and patients must seek adult care elsewhere. Transition from adolescence to adulthood involves acquiring adult life skills and experience to manage health care tasks and decisions and developing and learning a new role as a recipient of care in an adult setting. A lack of motivation, reduced adherence in adolescence, or lack of clinical guidance on how to transition from long-term pediatric providers to adult care may cause patients to forego routine primary care or subspecialty appointments. Due to the range in ability levels and support systems among adults with SB, a one-size-fits-all model does not appropriately address all aspects of transitioning care.14

Resistance to the transition from pediatric to adult care is multifaceted and may come from anyone involved in an individual’s care. Transition may be delayed because of anxiety about the change in providers or a lack of initiative due to apathetic feelings regarding such changes.14 There may also be little incentive for patients to leave the safety and familiarity of the care and trusted relationships they have had their whole lives. Pediatric providers may feel resistance to terminate care of such patients, particularly due to lack of trust in and limited training of adult physicians in treating conditions that previously only affected children. Resistance may also come from parents who may feel that a transition to adult care would begin to exclude them from the dynamics of care and decision-making processes.12

On the other hand, it is possible that transitioning to adult care may provide patients with feelings of autonomy and independence in their abilities to manage care. Young adults who have made the transition describe feeling empowered to take responsibility in adult care settings.12,14 Parents describe being surprised by their adult offspring’s ability to step into the leading role, and they recognize the benefit in allowing this transition, despite their anxiety about their own loss of control.18

Transitioning to new providers can be facilitated by a coordinated approach that involves the pediatric care team and the family.12,17 Discussions about the timing of the transfer of care should take place early among the patient, family, and pediatric provider.12,17 This may help prepare the family and reduce their feelings of anxiety about making the transition in the future.12 The Transition Readiness Assessment Questionnaire (TRAQ) may be a helpful tool for clinicians in determining the degree to which there is resistance to making the switch from pediatric to adult care.15

There is a documented difficulty with executive function skills amongst some individuals with SB when compared to the general population.19 This may affect provider, parent, and patient readiness to transfer responsibility of important health care activities and decisions. One study found that among patients with SB, higher reported executive functioning was directly correlated with increased medical autonomy, when controlling for age, IQ score, and disability level.19 Additionally, knowing the amount of support adults receive from their support network may help clinicians better understand how to counsel young adults as they and their families proceed through the transfer of responsibilities for tasks and decision making to ensure the best outcomes. 12,19

Role of Registries

The Centers for Disease Control and Prevention (CDC) registry for SB is a collaboration of 19 SB clinics across the country that collect data from all patients with SB to measure demographic information, treatments, and outcomes.20 The registry is beginning to describe data elements at all ages and compare care and outcomes across centers. This information may help promote improvements in clinical pathways. For example, from previous cohort publications, much has been learned about morbidity and intellectual issues related to shunt malfunction and infection, resulting in efforts to reduce the frequency of shunt placement by 10% to 25%.21,22

There is a need to follow individuals with SB as they age in order to better elucidate the prevalence of specific complications. While current research cites correlations between functional ability and select outcomes, greater emphasis on potentially preventable secondary complications is warranted. Registries may be useful in filling in gaps in the knowledge of secondary complications among adults with SB and areas in which the clinical care they receive may be lacking. More literature is needed on both the prevalence of such secondary complications and evaluation of methods that attempt to treat or prevent and reduce these outcomes. Data collected from the registry may help clinicians guide their management of adult patients and may reveal areas where research is lacking. Such evaluations may also further improvements in pediatric care. For example, several studies have shown the benefits of a plantigrade positioned foot in reducing pressure ulcers, which can lead to osteomyelitis and partial amputation.23–25 As these complications are areas of concern that occur among adults with SB, early intervention and orthopedic treatment of pediatric patients may improve these long-term health outcomes. Recognizing and understanding adverse health outcomes in adults with SB may thus inform treatment plans in pediatric care settings with the ultimate goal of improving long-term functioning.

Current Health Data Among Adults With SB

An extensive review of care for adults with SB by Dicianno et al highlighted areas where research is currently lacking.26 As more individuals with SB are living later into adulthood, it is necessary to understand the frequency and type of care they receive in a given year to identify any gaps in their health management. Much research to date has focused on the association of lesion level with difficulties in multiple areas including cognition, ambulation, and bowel and bladder continence. Although this is useful as a baseline to compare or predict expected outcomes, these factors are not amenable to change at this time. Our recommendation is to shift the focus from such correlations and to instead identify outcomes that are amenable to change in order to improve health, functional quality of life, and community participation among patients. We will discuss current trends and cost of medical care among adults with SB and then highlight major areas that may be useful for clinicians in identifying secondary complications among this population.

Based on previous data regarding hospitalization rates among patients with SB, there is a need for increased awareness and monitoring of secondary complications. Individuals with SB have significantly more hospitalizations per year than the general population.8,9,26 This finding was greatest among the patients between 18 and 39 years of age.27 Data from a 2-year period (2004–2005) of adults 18 years of age and older with a secondary diagnosis of SB or associated conditions revealed that among 7,670 hospitalizations, 34% of the cases were due to potentially preventable secondary complications.8 Of these cases of hospitalization due to preventable complications, 35.7% resulted in death. 8 Adults with SB were also reported to have greater than 2.5 times the mean number of 30-day long readmissions when compared to the general population.27 One study reported 4 diagnoses associated with a higher likelihood of readmission for patients with SB regardless of age or gender: septicemia, chronic ulcer of skin, skin/subcutaneous tissue infection, and infective arthritis.27

One study demonstrated that patients with SB also have higher rates of inpatient admissions when compared to patients with cerebral palsy and traumatic brain injury.10 This study sampled patients from a Canadian health care system where there is full public access to primary care, but there were no significant associations found between having a PCP and the number of inpatient care episodes. This may indicate a need for increased contact and communication with a knowledgeable PCP in order to prevent secondary complications.

Decreasing the rate of secondary complications could also significantly decrease medical costs. A previous study found the cost ratio of care for patients with SB compared to patients without SB to be 13.0 in children and adolescents ranging from 2 to 17 years of age. The cost ratio for adults aged 18 to 44 years old decreased to 6.8, however there are increased ongoing costs of medical care among this population.28 PCPs can work with patients to identify areas of risk for complication, thereby preventing the need for hospitalization, decreasing costs, and improving overall health. For example, a study examining the prevalence and cost of inpatient admissions for urinary tract infections (UTIs) demonstrated 22.8 inpatient admissions per 1,000 patients with SB annually compared to 0.44 admissions per 1,000 patients without SB.9 It is estimated that reducing the number of UTIs in patients with SB by 50% could decrease health care costs by approximately $4.4 million per 1,000 patients.9

There is a need for a comprehensive health care team for adults with SB, as these patients are at higher risk for health issues when compared to the general population. The primary aspects of general health concern in SB that we will be discussing include neurosurgical, urological, orthopedic/mobility, skin, metabolic, and psychosocial well-being ().

Table 1.

Health factors affecting adults with spina bifida

Shunt malfunction and neurosurgical care

Shunt malfunction is an area of key concern in adults with SB in relation to outcomes and mortality. Hydrocephalus is reported in 62% to 80% of adults with SB.16,29,30 A national study of insured individuals with SB found complications from devices, grafts, or implants to be the second highest hospitalizing condition after UTIs.8 Shunt revisions have been associated with poorer achievement in functional areas such as employment status, driving a car, and independent living.31 Lack of achievement in these areas has been shown to be higher when shunt revisions occur beyond 2 years of age.31

The diagnosis of shunt malfunction can be difficult due to the overlap in symptoms between shunt malfunction and sleep apnea or depression. Understanding best diagnostic and treatment methods and outcomes is important to improve care of hydrocephalus. Symptoms of shunt malfunction may include headache, nausea, vomiting, and dizziness.26,32 Drowsiness or lethargy appear to be common symptoms of shunt malfunction in long-term shunt-dependent patients. However, shunt symptoms in patients with chronic hydrocephalus can be subclinical and without significant or clear findings on imaging.32 Unrecognized shunt malfunction may be a major underlying cause of death in adults with SB.2 The registry may be helpful for determining the usual rates of shunt revision and the incidence of concomitant conditions such as sleep apnea or depression.

Diagnostic methods to identify sensitive symptom sets or findings are needed to identify which patients require surgical intervention. This is a key factor for families, who may have anxiety about neurosurgical transition. Radiological assessment should be performed to diagnose shunt malfunction; such imaging may still show small ventricles due to intermittent draining, ventricles with low compliance, and slit ventricle syndrome.26 Therefore, it is helpful to have baseline imaging to serve as a comparison to future imaging. In some patients, imaging is not diagnostic and, if symptoms continue, revision may be clinically indicated to improve symptoms. It can be difficult to detect neurologic changes in someone with a disability at baseline, especially with a new provider.33 The lack of specificity of these findings and the overlap with sleep apnea and depression highlight the importance of having a team or provider who knows the patient and his or her medical history.

As neurosurgeons may not routinely follow nonsymptomatic adult patients with SB at some centers, other key providers who know the patients and/or family well should monitor patients for shunt-related symptoms. A well-documented neurologic exam is key, as subtle signs may include brainstem findings of eye changes (optic nerve changes, changes in nystagmus or strabismus), swallowing issues, or new sleep apnea and snoring.

A Myelomeningocele Shunt Emergency Card has been developed and is free to print as a communication tool for a patient with a shunt (Ann and Robert H. Lurie Children’s Hospital of Chicago website: https://www.luriechildrens.org/en-us/care-services/specialties-services/spina-bifida-center/resources/Documents/spina-bifida-emergency-card.pdf.)

Tethered cord syndrome

Tethered cord syndrome (TCS) results from tension on the spinal cord. It has been reported in 20% to 55% of patients with SB; increasing prevalence is positively correlated with the amount of time since repair.26 A review study reported that adults with TCS typically present with back pain (57%–78%), motor weakness (44%–79%), and/or sphincter dysfunction (34%–71%).34 The mean age of TCS onset reported among these patients ranged from 34 to 43 years of age.34

A longitudinal study also cited scoliosis, gait changes, and change in spasticity as the common preoperative symptoms in adult patients with TCS.2 This study demonstrated that tethered cord release surgery improved or stabilized symptoms in 97% of a sample of 23 patients, whereas 3% of those patients reported worse symptoms post surgery.2 More research is needed on the natural progression of TCS and the timing of intervention within the population of adults with SB. Frequency of this condition, risk factors, and optimal diagnosis and management could be better understood via a registry.

UTI

UTI may be the most common preventable secondary complication, and it has been reported in approximately half of adults with SB on an annual basis.26,29 Methods to decrease this incidence rate should be tested, with reduction in antibiotic use as a secondary goal. Renal failure has been reported in 6% of one study population and each of these cases was preceded by multiple UTIs within a year, highlighting the importance of counseling patients on proper bladder management.29 It is important to determine whether bacteriuria or cystitis (inflammation) is occurring, as some episodes may not need to be treated with antibiotics. Specific guidelines developed from registry information could help families and clinicians determine when antibiotics are necessary. Frequency of antibiotic-resistant bacterial growth is an important additional question that could be addressed via a registry.

A majority of adults (71%–85%) with SB are on CIC, and the majority of this subgroup is responsible for performing their own catheterizations.2,35 The proportion of individuals who report continence is greater among adults on CIC versus adults not on CIC.2

A study on urological outcomes on adults with SB reported that 17% of a sample had surgery for stone disease. Of the surgeries, 57.4% were for renal stones, 31.1% for bladder stones, and 11.5% for ureteral stones.35 Data on surgery for stone disease (bladder, ureteral, and renal) demonstrated that the majority of surgeries were performed on patients older than 18 years of age compared to pediatric patients younger than 18 years of age (80.4% vs 19.6%; p < .001).35

Monitoring of the long-term function of the bowel and bladder is warranted with anterograde cecostomy enema use, Mitrofanoff bladder stoma use, or other bladder and bowel regimens including ileocystoplasty, which shows high incidence of bladder calculi and renal scarring.36 Registry comparisons between outcomes and complication rates using various bowel and bladder management methods may aid in understanding optimal management recommendations from childhood.

Cancer

Cancers may be underinvestigated in people with disabilities. One study comparing individuals with disability to the general population found that approximately 18% of individuals with disability had personal experience with cancer compared to only 7% of the general population.37 Additionally, women with disabilities were 17% more likely to report noncompliance with gynecologic cervical exams and 13% more likely to report noncompliance with mammogram screening guidelines than women without disabilities.37 Some pertinent barriers of adherence to cancer screening guidelines among individuals with disabilities include lack of health insurance and physical barriers at health care institutes in terms of mobility and getting into the correct position for screening procedures.38,39

Bladder cancer is rare in this population, however it can occur at an earlier age and present with different symptoms when compared to the general population.40 Symptoms reported in one case series on 8 patients included gross hematuria, urosepsis, renal failure, difficulty catheterizing, increased frequency of UTI, and sterile pyuria.40 Chronic inflammation poses a risk for the development of cancer, therefore patients with SB may be particularly at risk.41 Median age of diagnosis of bladder cancer in this population is reported to be 37 years old, and 6 months is the median survival period.40 Even though this diagnosis may be infrequent among individuals with SB, a high index of suspicion should be maintained with long-term urological care and immediate evaluation is necessary for any urological changes.41 The rates of testicular cancer in this population are not described.

Hypertension

Reported rates of hypertension in adults with SB are between 5% and 25%.16,29,35 There is a significant increase in the risk for prehypertension and hypertension with age in patients with SB compared to the general population 42 One study reported that fewer than half of the adults in a sample of patients with SB were considered normotensive. The reported rate of hypertension was 10.5% in adults with SB between the ages of 18 and 44 years old.42 Rates of hypertension among this sample were directly correlated with age: 40.6% of adults 45 to 64 years old and 70.3% of adults 65 years or older.42

Rates of hypertension in adults with SB have been shown to correlate with obesity. Data from one study of this population showed that 77% of obese adults had hypertension while only 26% of non-obese adults with SB were hypertensive.16 Optimal treatment and outcomes of treatment of hypertension may be better understood via a registry.

Metabolic syndrome

Criteria for metabolic syndrome include waist circumference (>40 inches in men; >35 inches in women), blood pressure above 130/85 mm Hg, fasting triglyceride >150 mg/dL, fasting high-density lipoprotein cholesterol <40 mg/dL in men or <50 mg/dL in women, and fasting blood sugar over 100 mg/dL.43 Rates of metabolic syndrome among individuals with SB (defined as having 3 or more risk factors) have been reported in a previous study to be 32% compared to 23% of the general population.44,45 In this study, only 6% of patients with SB had zero risk factors of metabolic syndrome.45

Weight management and physical activity may be protective factors against developing metabolic syndrome. One study found that 76% of adolescents and young adults with SB were either inactive or extremely inactive.46 Within this sample, physical activity was reported to be 50% lower than the general population and aerobic activity was 42% lower.46 Adults with SB may have more barriers to routine physical activity such as medical complications, lack of social support, and limited accessibility to equipment and facilities suited for individuals with disabilities.47 Due to the high prevalence of one or more risk factors for metabolic syndromes among patients with SB, it is necessary to provide counseling to these patients on lifestyle choices to prevent such risks and to determine most effective treatment/facilitation of lifestyle modulation and healthy lifestyle adoption.

Obesity rates among adults with SB have been reported between 30% and 37% in the literature.16,46,48 Higher obesity rates have been reported among adults with public insurance compared to adults with private insurance.48 Additionally, a higher proportion of adult women were considered extremely obese when compared to men.48 Waist circumference and percentage of trunk fat may provide more accurate measurements of adiposity in this population than solely using body mass index (BMI) as a measure of obesity.45,49

The potential for diabetes and its complications and atherosclerotic disease associated with metabolic syndrome are concerning and should be monitored for via a registry.

Sleep apnea

Sleep apnea has been reported in approximately 10% to 20% of children with SB.50–52 This may consist of obstructive, central, or mixed sleep apnea. In the general pediatric population, the rate of sleep apnea is about 4%.50 Further research is needed to determine the prevalence of sleep apnea among adults with SB. Risk factors for patients with SB are different than risk factors in the general population. Chiari II malformation, lesion level, and use of a wheelchair for ambulation are some of the SB-specific factors that may increase risk for sleep disordered breathing.50,51,53 Additionally, decreased strength in respiratory muscles or orthopedic conditions such as kyphosis and scoliosis may be associated with higher risk for restrictive lung disease.51 Previous research has hypothesized that sleep apnea is often undiagnosed in patients with SB as it is not often assessed during clinical care.51,53 Additionally, sleep apnea symptoms, such as morning headache, lethargy, and somnolence, overlap with depression and shunt malfunction, making the determination of optimal treatment difficult.54

Skin breakdown

Chronic skin ulcers are the primary diagnosis in approximately 6% of adults with SB, and skin and subcutaneous skin infections account for approximately 5% of hospitalizations among this group.8 Acute hospitalizations for skin diseases and diseases of subcutaneous tissue were reported to be higher in adults compared to youth with SB (14.6% vs 7.4%).11 One cohort study found that wounds were more prevalent in a teenage population than in adults 20 years old and older.55 The foot and ankle were the most common location of wounds in adults, followed by the buttocks and the knee and thigh.55

Foot and ankle wounds are more prevalent in patients with a lumbar or sacral lesion level, whereas patients with thoracic level lesions are more prone to wounds on the buttocks.55 Pressure ulcers are more prevalent in males, wheelchair users, and patients with higher lesion levels, shunts, or bladder incontinence.56 A registry could be helpful in identifying risk factors related to pressure ulcers on which to focus preventive efforts.

Pain

Pain has been reported in 25% to 32% of adults with SB.57,58 In one study, approximately 10% of adult patients with SB had neuropathic pain, and the proportion of adults with neuropathic pain significantly increased with age (3% of adults 18–29 years of age, 13% of adults 30–40 years of age, and 29% over the age of 40 years).57 Neuropathic pain was significantly higher among patients without hydrocephalus than with hydrocephalus.57 Pain is a risk factor for depression and therefore should be assessed and monitored throughout adult care.59 Neuropathic pain has been shown to have a significant impact on quality of life and happiness in other adult populations as well as in children with SB.60,61 Registries could help clarify the effect of pain on mobility and health and identify optimal methods to reduce it.

Orthopedic care and mobility

Care issues in orthopedics include spine, back pain, scoliosis, walking, amputation, osteomyelitis, and osteoarthritis. Frequency of musculoskeletal complaints (shoulder, wrist, knee/hip, ankle/foot pain, and low back pain) and their impact on mobility are yet to be described for wheelchair users and individuals with SB using other assistive devices for mobility. Some related information exists in spinal cord injury populations.

A longitudinal study showed that patients who ambulated a majority (75%–100%) of the time during their teenage years continued to ambulate a majority of the time as young adults.2 Lesion level may predict ambulation status into young adulthood, as previous studies have found a correlation between lower lesion level and greater proportion of time ambulating. 2 Understanding the factors that lead to loss of walking or conversely promote retention of mobility skills should be a goal for a registry.

Bone health

It has been reported that bone mineral density is reduced in patients with SB compared to typically developing peers.62,63 One study found that children and young adults with SB have decreased levels of vitamin D, and hypophosphatemia as a result, when compared to the general population.63 Risk factors among adults with SB include renal failure, use of epilepsy medications, and decreased physical activity.26 Similar to the general population, female adults with SB are at an increased risk compared to males.26 Decreased bone mineral density is predictive of an increased risk for fracture within this population.63 One study suggests increased supplementation, particularly for vitamin D, is necessary in this population. However, guidelines have not been published in terms of effective dose and the age at which to begin supplementation.26,63

Mental health

Individuals with SB have higher rates of anxiety and depression compared to the general population. It is important to assess the mental health status of patients making a transition from a multidisciplinary pediatric model of care into adult care. Rates of depression among adults with SB have been reported to be between 25% and 33%, while rates of anxiety have been reported as 13%.59,64 Thirty-six percent of adults with SB in one study were on antidepressants to treat depression and 63% of the individuals with depressive symptoms were on antidepressants.64 No gender differences are reported in terms of anxiety and depression.59 A positive attitude toward SB and satisfaction with family functioning have been reported as modifying factors to depression, while pain has been reported as a risk factor for both depression and anxiety.59 Depressive symptoms may go unrecognized among adults with SB, and they should be screened for these symptoms during clinic visits to ensure proper treatment. More research is needed to determine frequency of mental health symptoms and effective interventions to treat depression among adults with SB.

Sexuality and health education

In addition to medical complications, there are other health outcomes that should be addressed in the clinical setting such as sexual function and healthy pregnancy. One study found that although adults with SB obtain information on sexuality from a variety of sources, information on sexual function as it relates to SB primarily comes from health care providers.65 Among this study sample, 82% knew that sexually active patients with SB need to use a form of birth control and 96% knew sexual activity poses a risk for sexually transmitted diseases (STDs).65 However, only 70% knew that females with SB could get pregnant, 72% knew that condoms should be latex free, and 62% knew of the need for folic acid supplementation prior to conception.65 Additionally, data have shown that 24% to 35% of patients with SB reported that they have had sexual intercourse.65–67 Therefore, it is necessary to provide sexual health education during adolescence and adulthood to improve patient knowledge and adherence to health-conscious practices.

Erectile function and fertility for men with SB also need to be understood to ensure counseling and treatment for healthy sexual function. One study found that erectile dysfunction could not be predicted solely by the level of neurologic lesion.68 Twenty-three percent of patients in this study were found to have erectile dysfunction, but none of them were being treated for it.68 More research is necessary to further evaluate sexual function in adult males with SB.

Birth control counseling should be offered. Women with SB may have higher risk for adverse effects of oral contraceptives and intrauterine devices (IUDs). Oral contraceptives that are mostly progesterone may put women at a higher risk for osteoporosis, while contraceptives that are estrogen based may increase risk of hypercoagulability, particularly in persons with decreased mobility.69 Additionally, IUDs should not be used in patients with chronic UTIs, diminished pelvic sensation, or prolapsed uterus as these conditions may increase the risk of infection.69 Information about outcomes and complications of use of various forms of contraception is needed.

Risks of adults with SB having a child with SB are increased compared to the general population (4% vs 0.1–0.3%).70 Females of childbearing age in the general population require folic acid supplementation to reduce the risk of neural tube defects in pregnancy71; adults with SB may require higher doses of folic acid to achieve the same preventative outcomes.69 The CDC recommends a dosage of 4 mg/day of folic acid 1 month prior to trying to get pregnant, while a different study recommended a dosage of 5 mg/day 2 months prior to conception for women with neural tube defects or with a family history of neural tube defects.72,73 Women at increased risk for having a child with neural tube defects should adhere to these recommendations of increased folic acid compared to the general population and should continue these dosing recommendations through the first trimester.72,73 Counseling should include advice about folic acid supplementation for the patient or their partner. Additionally, female first-degree relatives or partners of male first-degree relatives should be made aware of the importance of high-dose folic acid supplementation prior to conception or during fertile years.74

Spina Bifida / NRS Healthcare

At NRS Healthcare, we are experts in daily living aids, which are products designed to help make everyday tasks easier if you have a healthcare condition or disability. Here, we provide a list of products that may be useful if you or your child has spina bifida symptoms and are finding some aspects of daily life difficult, such as:

Mobility (if you are an adult):

• Walking stick

• Rollator

• Wheelchair

• Grab rail

Mobility (for a child):

• Nimbo Posterior Posture Walker

• Wheeled walker

Incontinence:

• Incontinence pads and pants for day and night

• Skin care washes, wipes and creams

• Bed and chair protection

• Children’s continence

Using the toilet:

• Seahorse Plus

• Wheeled commode

Having a bath or shower:

• Bath lift

• Shower seat

• Children’s bath lifts and chairs

Moving, handling and transferring:

• Handling belt

• Transfer aid

• Transfer board

If you are unsure what equipment may help you, you may be eligible for an Occupational Therapy assessment from your local social service department. Alternatively, at NRS Healthcare, we have a team of Occupational Therapist product advisors, who can advise you on which daily living aids might help you. Contact them on 0345 121 8111 or email: [email protected]

PLEASE NOTE: our Product Advice Team can only give advice about equipment and products which may help you to live more independently. They cannot give any advice on medications or treatments for symptoms of this condition.

Exercise for spina bifida

It is important for a child or adult with spina bifida to exercise regularly. The NHS provides lots of tips on how to get active with a disability if you are an adult that needs to increase their exercise levels, and the Change 4 Life website provides lots of ideas for accessible activities for children with disabilities and special needs.

If you think you or your child needs to exercise more, speak to your GP or specialist team for advice. You may be referred to a physiotherapist.

Spina bifida diet

Eating a healthy diet is essential for everyone to remain healthy. Some people may experience spina bifida symptoms such as constipation and urinary tract infections (UTIs) so it is important to eat high fibre, vitamin rich foods and to drink plenty of water. The Change 4 Life website provides lots of advice about how to eat more healthily as a family.

Spina bifida and employment

Many people with spina bifida are able to work and young people with spina bifida are able to take up employment. The government’s Access to Work scheme aims to help people with a disability or health condition to find work by providing financial and practical support. Shine also provides information on Employment and Disability regarding rights, how to find suitable work, and voluntary work options.

An Uncomfortable Truth: Why Adults Should Ask to Have An Autopsy

No one likes to talk about death, especially their own death. But that is exactly what I am going to do today. I am going to talk about the possibility of my own demise and what I would like my family to do when that happens.

The concept of dying was one I grasped at an early age. I was born in 1965 when people with Spina Bifida were not expected to survive to adulthood. It was not directly said to me as a child but it was always implied that I would not have long on this earth. Even when I reached the ripe old age of 22, there was a nursing school instructor who was lecturing about Spina Bifida and telling the class how the oldest living person with Spina Bifida that she knew of was 28. It did make an impression on me. I lived my life as if there was no tomorrow. I was and am grateful for every birthday.

The internet changed my perspective of my own mortality. I started to meet people with Spina Bifida who were my age and older. I read articles and saw that people with Spina Bifida were expected to live an average lifespan. I met a woman with Spina Bifida who had climbed Machu Picchu for her 75th birthday and had a trip planned for Antarctica on her 80th. At 51 years old I had thought we had beaten the odds given to me all those decades ago.

I was wrong. Slowly but surely, I started hearing about people in their 40’s and 50’s with Spina Bifida going to bed at night and never waking the next morning. And as my contact with the Spina Bifida community increased, I heard of more and more people who died suddenly and without warning. The frightening part is that we do not know why they died.

The nurse in me started to ask questions. And the most basic question is “What does the death certificate say?”. It was then that I learned the sad truth of how medicine and societal perceptions of Spina Bifida have not kept up with the changing demographics of our community. Most of the death certificates list Spina Bifida as the cause of death. In most cases an autopsy was not performed. The medical community and even our own families still expect us to die young and do not look for other answers when we do expire.

Spina Bifida is a birth defect with a myriad of medical issues associated with it but it is not what causes someone born with it to die. It’s possible, and likely, that one of any of the known medical issues associated with it can and does cause an unexpected death. Or it very well could be something that we haven’t even heard of yet causing these deaths. But we will never know the answer unless we do research to find out. That research starts with an accurate death certificate.

In response to this newly acquired knowledge and my questions, I did something fairly radical and very uncomfortable to myself and my family. I talked to them about my death. I didn’t discuss wills or final arrangements or any of those things that you talk about when death appears imminent. Instead we talked about what I would like them to do if I died suddenly, unexpectedly, and not explainable. I asked them, and myself to imagine the unimaginable. It wasn’t easy telling my parents, my significant other, and my adult kids that I wanted an autopsy to determine the cause of my death if one day I went to sleep and never woke again. It was a necessary conversation that had to take place when I think of all the bright lights in our community that have been lost forever without explanation. I cannot think of a better way for my death to be meaningful than if it helps find the answers to prevent the same thing happening to the generations behind me.

Putting aside your own fears, causing yourself and your family discomfort in order to benefit others is a lot to ask of you. But I am asking. So much has been done to improve the lives of people with Spina Bifida, this is a way that you can ensure that progress continues. Please consider talking to your family and friends about having an autopsy if the unthinkable should occur.

Thanks for your time,

Monica Albert Still, RN, BSN

Long term problems associated with Spina Bifida

Urine and kidney problems

Almost all patients with Spina Bifida will require lifelong antibiotics to prevent infection. Approximately 70% of all spina bifida patients will have to have some form of drug therapy later in life to try to control their bladder over-activity, and approximately 70% will have to practice clean intermittent catheterisation on a life long basis.

About 30% of Spina Bifida patients will not respond well to this treatment, continuing to wet or developing pressure effects on their kidneys. These children need major surgery to be carried at some stage during later childhood. This is usually between 7 and 10 years of age.

This surgery involves operations on the bladder. Often, the bladder is made larger so that it can hold more urine. To make passing a catheter into the bladder easier and to allow the children to catheterise themselves, the child’s appendix may be connected to the bladder and the other end brought out onto the skin. A catheter can then be passed through this several times each day to keep the child dry.

This type of surgery requires a commitment from the family and the child and considerable counselling and preparation. Constant and lifelong follow up is required following this sort of surgery. However, the results of this management are good. In practical terms, urinary continence is achieved in approximately 90% of patients and it is also possible to protect their kidneys from long-term damage and the consequences of that.

Bowel problems

Most Spina Bifida children have some problem controlling their bowel movements. Constipation is the most common problem and can often be controlled by medicines, or failing this, suppositories. Sometimes enemas are required and take the form of a sachet of fluid given into the bottom.

If these treatments do not work it is possible to perform washouts of the bowel. If this is done once a day it may be possible for the child to be clean and avoid constipation. To be successful this technique requires co-operation from the child and so is usually not attempted until the child is old enough to understand the benefits and is motivated to co-operate. Parents can be taught the washout technique along with their child.

The washouts may be made more acceptable and easier by the ACE operation (Antegrade Continence Enema). In this operation the appendix is brought to the surface as a small opening on the skin. This does not leak bowel content but allows the parent of child to pass a catheter into the bowel once a day to do the washout.

In some children none of the above techniques work well. In this situation a colostomy can be formed so that all the bowel content drains into a bag on the tummy.

Hydrocephalus surgery

If a shunt has been inserted it can become blocked. This occurs in about 50% of shunts by 2 years. Sometimes it is associated with infection inside, but mostly it occurs on its own. More frequently the ventricular catheter is blocked rather than the peritoneal catheter. Sometimes the valve functions badly, though this is less common.

Blocked shunts usually require a revision operation. Although revision operations carry a slightly higher risk, both of bleeding and of infection, mostly they are uneventful. Shunts do not need routine lengthening, as sufficient tubing to allow for growth is implanted when they are first inserted. Very rarely, they need to be implanted in a cavity other than the abdomen. Once a child has a shunt it is usually needed for life.

Muscles and movement

Careful orthopaedic follow-up of Spina Bifida patients is required. Although the amount of movement and strength that the child has in the legs does not tend to change, increasing weight of the child may cause progressive problems with walking. Also the action of tendons may not be balanced and result in joint deformities. This is due to some muscles working better than others and tending to pull the joint out of shape. Operations may be needed on the foot, ankle, knee or hip muscles or on the joints themselves.

Children who have movement of the knee or foot should be able to walk. In mild cases walking may be possible unaided. In other children orthoses (splints) or calipers (support rods connected to the shoe on either side of the leg) may be needed. More severely affected children may require a wheelchair. Even those children who can walk with calipers may prefer to use a wheel chair when they are older.

Sensation

Just as the nerves to the leg muscles, bladder and so on, may be affected in Spina Bifida so are the nerves to the skin. This means that there may be areas of numbness in the legs. The extent of the numbness depends on the level of Spina Bifida. In some children only the feet are affected but in others this may extend to the level of the knees or hip. The skin of affected areas tends to be cold and is prone to damage from pressure sores or undetected injury. Care and protection of the skin of these areas may be needed.

Intellect and education

Around 75% of children with Spina Bifida have some learning difficulties. The most common problems are concentration, short-term memory, organisational skills, hand/eye coordination, and motivation. Most children can go to a normal school. Some need a special school but this is usually because of the facilities required for their physical difficulties. In adult life many people with Spina Bifida find employment, drive a car and are self-sufficient.

Sexual function

Early puberty is common although the reasons for this are not understood. Fertility is normal and many women with Spina Bifida have families. Although sensation can be affected, both sexes can enjoy a healthy sex life.

Emotional and family support

Spina Bifida will obviously have considerable emotional effects on both the affected person and their family. Counselling and support may be needed. Financial help is also available through Social Services for children with Spina Bifida and their families.

90,000 Cleft lip and palate: the defect can be corrected

Cleft lip and palate is one of the most common congenital malformations of the upper jaw: it occurs in one third of patients with congenital malformations. In Moscow, 1 in 800 babies are born with this malformation.

An anomaly is detected, usually on ultrasound in the 1-2 trimester of pregnancy. Such news is a huge stress for future parents. But in most cases, thanks to timely and qualified treatment, cosmetic and functional defects in the baby can be eliminated.

At what age surgical treatment is most effective and how a complex anomaly is treated, said the doctor-maxillofacial surgeon, plastic surgeon of the Morozov Children’s Hospital Elvira Sergeevna Mkrtumyan.

What influences the formation of a defect?

The defect is formed in the first two months of intrauterine development of the child, when the laying of the facial skeleton occurs. The cause of the development of pathology is an abnormality of chromosomes.

The development of the defect is influenced by both hereditary factors (7%) and external (40%).External include infectious and viral diseases, smoking, drugs, alcoholism, the use of certain medicinal substances, lack of vitamin folic acid, poor ecology, and so on.

What types of crevices are there?

Clefts can be both lips and palate. They, in turn, are isolated (only the lips or only the palate) and through, when the defect extends to the upper lip, alviolar ridge and palate. Each of the species has varying degrees of severity.In addition, the defect is both one-sided (usually on the left) and two-sided.

With an isolated cleft lip, as a rule, the deformation can be insignificant, or it can be accompanied by deformation of the nasal septum, alveolar cartilage and cleft of the alveolar ridge, which further entails problems with the teeth.

With clefts of the palate (from a slight cleavage of only the soft palate to severe bilateral lesions of the soft and hard palate), children may experience problems with eating, breathing, sucking, and later with sound pronunciation.Thanks to timely surgical intervention, it is possible to obtain an ideal treatment result.

The most severe form is bilateral penetrating cleft lip and palate. But even in this case, timely treatment and rehabilitation give the baby a chance to recover.

If during the intrauterine diagnosis a cleft is found in the baby, what should the parents do?

The most important thing I would like to say to parents facing this problem is that there is no need to worry.It is important to immediately consult with maxillofacial surgeons involved in the treatment of pathology.

We advise parents-to-be even before the baby is born. We will tell you how to feed your baby correctly – preferably with a spoon or a bottle with special nipples in order to maintain swallowing reflexes, because with prolonged tube feeding, they can fade away.

Often this defect can be accompanied by pathology of the bone and cardiovascular systems, kidneys. Therefore, it is important to undergo research immediately after birth and exclude these diseases.If the baby does not have other serious concomitant diseases, we prescribe planned surgical treatment to eliminate the defect.

At the request of the parents, after the maternity hospital, the child can be transferred to the neonatology department of our hospital, where the mother will be taught how to feed the child, examined and given recommendations on how to manage it before the planned operation.

At what age is surgical treatment most effective?

The optimal age for surgery to correct a cleft lip and eliminate deformity of the nose, if there is no gross pathology among other systems, is 3 months.Earlier plastic surgeries do not provide the best aesthetic result.

If the child has a severe cleft, the correction can be done in two stages. At six months, if it concerns the palate, the soft palate, from one to two years, the hard palate. If the surgical treatment is carried out in one stage, then it is performed at the age of 9 months to 1.5 years. This has a number of advantages: the child undergoes one, not two anesthesia, the tissues are more mature, which means that the result of treatment will be more effective.We advise to undergo a plastic surgery of the palate for children under two years of age, until the child has formed an incorrect speech in the brain – the center of speech.

If the child has a severe cleft, the correction can be done in two stages. At six months, if it concerns the palate, the soft palate, from one to two years, the hard palate. If the surgical treatment is carried out in one stage, then it is performed at the age of 9 months to 1.5 years. This has a number of advantages: the child undergoes one, not two anesthesia, the tissues are more mature, which means that the result of treatment will be more effective.We advise to undergo a plastic surgery of the palate for children under two years of age, until the child has formed an incorrect speech in the brain – the center of speech.

What operations are performed for cleft lip and palate?

Correction of a congenital anomaly requires an integrated approach. It is aimed not only at eliminating a cosmetic defect, but also at the reconstruction of functional disorders, elimination of problems with swallowing, breathing and sound production. The amount and type of operational aid depends on the characteristics of each child and is selected individually.

If the cleft looks like an isolated lip or palate, the surgical treatment will be performed in one step. In the most difficult cases, the correction is carried out in several stages.

Cheiloplasty is performed in case of an isolated cleft lip without pronounced deformity of the nose. The vestibule of the oral cavity is created (the space located under the upper lip), the integrity of the circular muscle of the upper lip is restored, and a red border is formed. If the defect of the upper lip is more severe, then simultaneous cheilorinoplasty (lip and nose plasty) is performed.We perform lip plasty and place the wing cartilage of the nose in a symmetrical position. On the 7th day, the postoperative stitches are removed and the children are discharged from the hospital with recommendations for further management of the child.

An isolated cleft palate appears as an anatomical injury to the soft palate (movable palatine curtain) or to the soft and hard palate. Pathology is accompanied by impaired swallowing, breathing and sound pronunciation. Palate plasty includes restoration of the hard palate and soft tissues of the palatine curtain, closure of the alveolar ridge.After the operation, when children reach 3 years of age, classes with a speech therapist are recommended to prevent speech disorders, manifested by nasal and illegible speech.

If speech disorders are not eliminated by the efforts of speech therapists alone, then there is a surgical correction – the elimination of palatine-pharyngeal insufficiency. The operation consists in transplanting flaps on the feeding pedicle in the area of ​​the pharyngeal ring.

From the age of 5, a child is referred for treatment to an orthodontist to eliminate defects in the dental-jaw system.At the end of orthodontic treatment, the next stage of surgical treatment is performed – bone grafting of the cleft of the alveolar process to eliminate the bone defect of the alveolar process.

Morozov Children’s Hospital has accumulated extensive experience in the effective treatment of cleft lip and palate. Every year, doctors perform up to 200 operations to correct these congenital malformations.

90,000 Wolf’s mouth, cleft lip – everything is fixable

The layout of congenital clefts of the face: A – Transverse cleft of the face; B – Median cleft of the upper lip; B – Oblique cleft of the face; D – Lateral cleft of the upper lip; E – Cleft lower lip

We continue the series of publications devoted to neurosurgical diseases.The project was prepared jointly with the ICOO for helping children with neurosurgical diseases “He needs you”. We want these “terrible” diagnoses not to put pressure on parents and take away their hope. In our publications, the best specialists and experienced parents will tell you about the ways of healing and overcoming, give recommendations and show that life can continue with any, even such complex, diseases.

“Once a doctor at a children’s clinic wrote us a diagnosis of“ cleft lip, cleft palate ”, I told her:“ You seem to be a doctor, why do you write so illiterately? I don’t diagnose you with chicken brains.After that, she tore out the page and went to another doctor. Defend your children, you must do it ”(from the correspondence on the forum).

Unfortunately, in our country, facial clefts are diagnosed in 1 out of 500-1000 newborns and almost always cause parents to feel guilty and fearful. In fact, facial clefts have been successfully operated for a long time.

In colloquial speech, the cleft palate is often called the cleft palate, and the cleft lip is often called the cleft lip. However, this is not accepted in the professional world – confirms the head of the 1st surgical department of St. Vladimir’s Children’s City Clinical Hospital, Doctor of Medical Sciences, Professor Dmitry Yuryevich Komelyagin .

– We never call our patients and their diseases that way. People are very offended to hear this. These features are called “congenital unilateral cleft of the upper lip, alveolar bone, and palate” and “congenital bilateral cleft of the upper lip, alveolar bone, and palate.”

Facial clefts are divided into median and lateral. The most common clefts are the median cleft lip and cleft palate. Incomplete clefts are called blind – they can capture only the uvula, or the uvula and soft palate or partially hard palate and end behind the incisal opening.

Clefts, in which the gap from the hard palate extends to the alveolar process and the upper lip, are called full, or through.

“Why did this happen to me ?!”

Dmitry Yuryevich Komelyagin

– Dmitry Yuryevich, there is a myth that cleft faces are problems of children from disadvantaged families.

– No! We have many families with prosperous, beautiful parents, whose children have faced such a problem. Rather, the risk factor will be the fact that the parent himself was a carrier of this pathology in childhood.We have such families. For example, once we operated on a girl with a cleft upper lip, she got married and gave birth to a child with exactly the same pathology, they came to us again. We had families in which both the second and even the third child was born with a similar pathology. And we have successfully operated on all of them.

– How many years have you been operating?

– The branch was founded 26 years ago. We have been operating for 26 years now. Cleft face is a multifactorial problem; it is still difficult to identify any specific causes.It is known that the cause may be intrauterine fetal hypoxia. What caused this hypoxia? Anything. Stress? Maybe. A mother’s infection? Taking medication? Yes, it is possible. But what exactly – no one will tell you yet.

Today, the most reasonable theory is that the cause of facial clefts is a combination of hereditary factors and adverse environmental influences.

Mutation of several genes leads to increased sensitivity to bacteria in the environment.In this case, factors such as medications, X-rays, infections, and even stress and noise can cause the cleft. As a result of their exposure, the fetus experiences an oxygen deficiency during the few hours that are required for the formation of the face, and then the fused tissues suffer.

Something went wrong …

Approximately 4-6 weeks of fetal formation, the paired median nasal protuberances merge. They grow together and form the incisor bone, the nasal groove of the upper lip, and the tip of the nose.The lateral elements of the upper lip originate from the paired maxillary processes. If the tissues have not grown together for some reason, a cleft lip appears. It usually originates at the junction between the central and lateral parts of the upper lip on both sides.

The secondary palate begins to develop around the eighth week of gestation, after the completion of the primary palate. The paired palatine folds are initially separated by the developing tongue. As the lower jaw grows, the tongue moves forward and the palatine folds move down and take a more horizontal position.If something went wrong, the paired palatine folds cannot move downward and medially – a cleft appears in the palate.

The secondary palate usually develops before the 12th week. Therefore, it is optimal to diagnose congenital clefts of the face using ultrasound (ultrasound) at the 11-12th week of pregnancy (this is when the formation of the baby’s face ends).

A cleft lip can be diagnosed by a specialist if the fetal face is in the correct position. Diagnosis of the palate with ultrasound is more difficult.However, an experienced doctor can detect abnormal lateral movement of the fetal tongue. In some cases, specialists cannot detect this defect until the very birth, if the child covers his face with a pen or a leg.

D.Yu. Komelyagin : “A consultation center for pregnant women will soon be opened at the St. Vladimir’s Children’s Clinical Hospital on the basis of the department for newborns, in which, among other things, maxillofacial surgeons will also be admitted. And if, say, during an ultrasound examination, the baby was diagnosed with a malformation of the face, the mother will be able to come immediately to us for a consultation.We will calm her down, tell her how it will be possible to cure this or that defect, explain what awaits her, and most importantly, we will prove that this is not a reason to abandon the child, with the right treatment tactics, you can live a full life with facial defects ”.

Tutankhamun had a cleft in the sky. This conclusion was reached two years ago by a group of scientists who studied his mummy. The research was headed by the Secretary General of the Supreme Council for Antiquities of the Arab Republic of Egypt Zahi Hawass. Tutankhamun is not the only person who has not been prevented from achieving success and fame by clefts in his face.

The face of the famous artist Joaquin Phoenix, it seems, also bears the marks of a facial cleft. However, the artist’s biographers claim that the scar on the lip is not a trace from the operation, but a congenital “mark”. However, whether it is a hidden cleft or a cleft at all is not so important. More importantly, the scar from her did not prevent Joaquin from making a brilliant film career.

The same scar on the lip (also visually similar to an operated cleft) did not prevent Chich Marina from becoming a popular American film actor, Denis Dorokhov – a famous KVN-scholar in Russia, and Denis Lebedev – a world boxing champion.

When to operate on clefts

Forms of clefts of the palate: A – Cleft of the uvula of the soft palate; B – Cleft of the soft palate; B – Cleft of the soft and partially hard palate; D – Complete lateral cleft of the soft and hard palate; D – Complete bilateral cleft of the hard and soft palate

D.Yu. Komelyagin : Different clinics adhere to different rules and different terms of the operation. We operate on clefts of the upper lip at the age of four months (more precisely, from four to six months), depending on the situation.If the child is hypotrophic, small in weight, with low hemoglobin, then it is better to postpone the operation. Should a cleft be operated before four months? Such an operation is a painstaking work, it is important that the tissues of the baby, with which the surgeon will work, are already mature. In addition, the lungs and heart must mature so that the baby can safely undergo anesthesia and the postoperative period. There is no point in rushing. If, of course, nothing threatens the child’s life.

Often, clefts of the face are signs of a syndrome with concomitant complications (Stickler syndrome, Laws-Dietz syndrome, Hardikar syndrome, Treacher-Collins syndrome, etc.)), including Pierre Robin’s syndrome, characterized by underdevelopment of the lower jaw and retraction of the tongue. A child with this syndrome simply cannot breathe normally or breathes only in a certain position (on the side, on the stomach), because the tissues are displaced and block the airways.

Of course, in such cases, early operations are justified. This is what is called “operations for health reasons” (D.Yu. Komelyagin and the team of the 1st surgical department have a patent for the invention of a compression-distraction device that can successfully treat children with Pierre Robin’s syndrome – Approx.ed. ).

– What should a parent of a child born with a cleft look for?

– For everything. Conduct a complete examination. And after that, build a treatment plan together with specialists. For example, if he has a heart defect, of course, first of all, he needs to deal with the defect, and then – the treatment of the cleft.

But if there are no other, more important, factors, we recommend surgery for the cleft of the upper lip at the age of four months, the palate at the age of one and a half years.It is more difficult to treat a cleft alveolar bone. Previously, we offered to operate her at the age of twelve, now – we start at eight. For treatment, bone grafting is performed, that is, the patient’s own bone is used (say, from the lower jaw, tibia) or an artificial bone (bone graft).

An important point in the treatment of alveolar ridge cleft is orthodontic preparation. That is, first the orthodontist achieves the correct position of the fragments of the upper jaw relative to each other and relative to the lower jaw, and then the surgeons begin the correction.

In case of a successful operation, patients then come for follow-up observation every six months. The surgeon and orthodontist monitor how scars are formed, the upper jaw, and whether there is a deformation of the nose. Sometimes you have to do the surgical correction again. But these decisions are important to make in conjunction with the orthodontist. If the growth of the upper jaw is incomplete, surgery can be harmful.

In addition to the maxillofacial surgeon, you may need help: a neurologist, otorhinolaryngologist, orthodontist, dentist, speech therapist, audiologist, phonopedist, psychologist

“The children did not laugh, they took care of her“ teeth on the nose ”!

“For the first time I saw my sixth daughter a month after her birth,” recalls Marianna Simonova, mother of seven adopted daughters from Novosibirsk.“In the photo in the Federal database. She was there …. scary. They photographed her somehow sideways, unsuccessfully. But something told me: “Mine. If I don’t take it, the system will ruin her. ”

A few months later I was already flying from my Novosibirsk to her in Vladivostok to get acquainted. The husband said at home: “The eyes are beautiful, we will fix the rest.” So I already had my husband’s support. Arrived – picked up – sniffed. Everything. That was enough for me to make a decision. I’m taking it.

Yes, she had a bilateral cleft lip and palate.At that time I had already read, asked around, consulted with the surgeons. My husband and I decided – we will fix it. The only thing I was afraid of was the reactions of my other daughters. How will they receive it? Will they not laugh? She was the youngest at that time.

I then left her in the institution, supposedly they were expecting a quota for an operation for her. But I soon realized that time was passing – no one was doing it there. And she took it home.

At first, we just had to feed her. The weight was too small, the hemoglobin was too low, and the operation could not be performed in this state.How to feed her? The nipple is sucked, pressing it with the tongue against the palate. And she has a gap in the sky – there is nothing to squeeze. Then she somehow contrived to wrap her tongue around the nipple and pull out of it. She had to feed her every 2-3 hours, day and night.

Meat puree (the hemolobin must be raised) was given from a spoon. You put a spoon – and everything falls out of it, there is nothing to squeeze. You clean your nose – and you see everything right through, almost to the brain. You give the liquid and you understand – you need to be extremely careful, in no case should you choke on it.My acquaintances in the same situation, food poured out through their ears. We didn’t have that, really.

But in the end, by the age of one and a half, she was ready for surgery. Several hours of surgery, several days in intensive care without a mother – all this is over. And the brilliant result is obvious. No complications. Before the operation, I was advised by a wonderful maxillofacial surgeon, our Novosibirsk, Mikhail Vasilyevich Kolybelkin. He even helped my daughter choose the name. According to the passport, the daughter’s name is Victoria, Vika. “She can’t pronounce it yet, give her a short name.”So we got Oia, Iyushka (from the Greek “violet”). We have it – a beautiful violet, a delicate flower! So this name suits her!

And the older children, by the way, never laughed at her, on the contrary, they protected her as best they could, especially before the operation, did not let her fall, they were afraid for her “teeth on her nose” (as they called her defect).

Not scary? No, it was only scary for her, how she would endure everything. And it was even worse to leave her in the system. Children cannot be left without a family. This is especially so.Without surgery, attention, love, they very quickly become difficult, with severe developmental delays. But this is just a surgical problem. If the child is in the family, everything is fixable! ”

In preparing the article, the following materials were used:

of the 1st surgical department of St. Vladimir’s Children’s City Clinical Hospital: http://cmfsurgery.ru

Institute of Congenital Diseases of the Maxillofacial Region of Professor G.V. Gonchakov: https://www.vasha-nadezhda.ru/care

sites https: // www.operabelno.ru/metody-xirurgicheskoj-korrekcii-volchej-pasti-u-detej/

sites: https://medbe.ru/materials/plasticheskaya-khirurgiya-pri-vrozhdennykh-defektakh/embrionalnoe-razvitie-guby-i-nyeba/

Medical and clinical genetics for dentists: textbook for universities / Ed. O.O. Yanushevich., – 2009 .– 400 p.

Leaflets prepared by the fund “He needs you”. You can download the memo here.

If you need medical help or consultation, an application for remote consultation and hospitalization in specialized hospitals can be submitted here.

90,000 Weimar Pointing Dog Puppies.

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Symptoms: lethargy, weight loss, lack of appetite. Breast sarcoma is a palpable formation in the skin and subcutaneous layers. Symptoms: lethargy, loss of appetite.

For the first time a breed called Weimaraner was mentioned in the years.The document says that the breed was formed in East Germany in the vicinity of the city of Weimar. These dogs were very popular at the court of the Grand Duke of Weimar.

The disease can metastasize to other parts of the body, ulcers can appear. Hypothyroidism is a dysfunction of the thyroid gland.

Symptoms: hair loss, rough scaly skin, weight gain.Addison’s disease primary hypocorticism. Symptoms: lack of appetite, vomiting, lethargy, weight loss, tremors, diarrhea. Sometimes weakness manifests itself, fainting occurs. Granulocytopathy deficiency of protein adhesion of leukocytes. It is fraught with increased susceptibility to bacterial infections, persistent leukocytosis.

Factor XI deficiency. Symptoms: moderate bleeding, worse after surgery or injury. It can be fatal. Lack of glucose phosphate dehydrogenase.

Clinical symptoms do not appear.

Weimaraner training

Hemophilia A – prolonged severe bleeding due to impaired clot formation. The dog may die.

Details by phone. My name is Igor Kobylyansky and, as you can guess from the name of the kennel – In the beginning there was a hunt!

Tricuspid dysplasia.Symptoms: poor appetite, weight loss, lethargy, heart murmurs.

Demodecosis – local infection with a tick. Itching may be severe, or it may not be present at all. The muzzle is usually affected. As a rule, the disease goes away on its own 6 to 8 weeks later. IgG deficiency – repeated bacterial infections due to neutrophil dysfunction and lack of IgG. The dog becomes susceptible to infections. T-cell defect – an increased susceptibility to infections, especially viral ones. These dogs are characterized by dwarfism.

Symptoms: follicular papules of various sizes on the face. Ulcers may appear. Juvenile cellulite. Symptoms: The muzzle seems to be swollen due to the enlargement of the cervical and submandibular lymph nodes. Pemphigus vulgaris. Symptoms: the appearance of erosions and ulcers under the armpits and at the mucocutaneous boundaries.

Seborrheic adenitis. Symptoms: faded, brittle, dull coat; oily skin. Umbilical hernia. On the abdomen, there is a protrusion of the skin, which may contain internal organs.

However, over time, the swift and graceful hunter became an excellent home companion and tireless travel companion.Characteristics of the breed Affection for the family. The name of this German town gave the name to a beautiful and intelligent dog. Initially, professional huntsmen were engaged in breeding these cops.

May heal without treatment. Arachnoid cyst. The development of a cyst can lead to compression of the spinal cord. Hypoplasia of the cerebellar vermis – the cerebellar ridge does not develop. Manifestation: staggering, uncoordinated gait.Hypomyelination – damage to the central nervous system. Symptoms can vary, the most common are tremors of the limbs, trunk, head, and impaired coordination. Spinal dysraphia – manifests itself in a violation of gait, scoliosis, widely spaced legs.

Such a dog can lead a normal life. Limited calcification is the deposition of calcium in the skin and subcutaneous tissue. Craniomandibular osteopathy is an abnormal growth of the bones of the lower jaw, head, or limbs.

Dwarfism is a skeletal disorder leading to a decrease in size compared to the norm.Can be proportionate or disproportionate. Hip dysplasia can lead to lameness or complete immobility of the dog.

Spina bifida spina bifida can manifest itself in weakness of the hind legs, fecal incontinence. The absence of skin, muscles and spinous processes of the arches of the vertebrae in the lumbosacral region is possible. Spondylolisthesis of the cervical spine is an anomaly of the cervical vertebrae, which causes dysfunction of the hind limbs up to paralysis.

Weimaraner

Primary glomerulopathies.The onset may be asymptomatic. Possibility of lethargy, bad breath, mouth ulcers, weight loss, increased thirst, decreased appetite, vomiting. You can knit a Weimaraner bitch when she reaches 2 – 2.5 years.

If the dog is older than 4 – 5 years, pregnancy and childbirth may be associated with complications. It is better if palpation is carried out by a veterinarian, you can independently damage the fetus, especially in small breed dogs. If the bitch begins to show signs of false pregnancy, it is necessary to remove the toys.Self-licking of the nipples increases the symptoms of false pregnancy. This can be prevented by wearing a collar and blanket on the dog.

Limit protein intake. If the bitch is aggressive, extremely agitated, or has physical problems such as mastitis, medication will be needed. See your veterinarian.

Dog breed Weimar Pointing Dog (Weimaraner): description, characteristics, photos, reviews

False pregnancy can be a symptom of hypothyroidism or liver dysfunction.The dog must be closely monitored so that it does not climb into a hard-to-reach place.

Do not allow the bitch to eat the afterbirth if it is greenish-black in color with a putrid odor. Keep track of the number of successions, there should have been as many as there were puppies. Sometimes the afterbirth can remain inside and come out only at the end of labor.

If at least one afterbirth remains inside, it is fraught with metritis inflammation for the bitch. If you are not sure that all the placentas have come out, be sure to take the dog for an ultrasound scan.Remember that the dog is in a state of shock, talk to him gently, calm him down. As a rule, sooner or later, the Weimaraner bitch still accepts puppies. Cases where hatred is persistent are extremely rare. Caution: No matter what happens, even if the bitch eats all the babies, don’t blame her.

The birth of puppies was your idea and it was you who made the bitch give birth. She does not understand what she is doing, hormonal disruptions and shock force her to behave in a completely unusual way. Best of all, the Weimar Pointer feels in a large house with a decent territory, where you can run a lot and do research.But the short coat does not allow them to live outside in the cold season, so even if you live outside the city, the dog should be able to stay in a warm room.

If you live in an apartment, you will have to devote a lot of time to training and active long walks. Otherwise, you and your neighbors will go deaf from the continuous barking, and the interior will undergo changes that you will probably not like. The coat of the Weimar Pointer does not require special care.

It is short, close-fitting and tough, with no undercoat, so it can be cleaned with a rubber brush or a special glove.But if you have a long-haired variety of Weimaraner, you will have to comb your pet daily and bathe it every 2 months.

These people wanted to breed dogs only for hunting, not for sale. Therefore, the rule was adopted that no one can buy a dog of this breed without first becoming a member of the club. This led to the fact that it became difficult to buy a Weimaraner even in Germany, and outside its borders it is practically impossible. Currently, the Weimaraner is a fairly popular and well-known dog breed, which is equally well manifested both in the field and when participating in show exhibitions, breed lovers have managed to preserve the effective appearance of the dog and its working qualities.

Despite the fact that Weimaraners are still quite good working dogs, the problem of exaggerating the exterior for the sake of show exhibitions has already touched them.

In the photo – forbrust, the protruding part of the sternum. Exactly the same forbrust can be seen in modern Dobermans, and many breed breeders believe that this disease has not yet affected such Weimaraners, but I personally do not see the functional meaning in such exterior “delights”. Field and show champion.Please note that the tail of an American dog is docked – the ban on docking did not apply to the United States, while dogs in Europe and Russia are left with tails to participate in European exhibitions.

The first records of longhaired Weimaraner puppies appeared in the pedigree book of the German Shorthaired Weimaraner Club. Most of the long-haired puppies were born from short-haired dogs in the thirties of the twentieth century. In general, the appearance of long-haired puppies in short-haired parents is a fairly well-known mutation in pedigree dog breeding and is not necessarily the result of cross-breeding with any other breed.

This decision was made at a meeting of the General Committee of the International Cynological Organization FCI in October of the year and concerns, among other things, the crossing of long-haired and short-haired Weimaraner varieties with each other. However, in Germany, puppies obtained from mixed mating can subsequently only be mated with a long-haired dog.

In addition, in Germany, they are extremely attentive to the working qualities of a dog and it is practically impossible to acquire a pet of this breed there if you are not a hunter.

This is approximately how the coat of a dog from mixed mating looks, it does not have long decorating feathers on the ears, paws and tail like a long-haired dog, but much longer and denser than that of the short-haired variety. Officially, the Weimaraner blue color is not recognized by the International Cynological Organization, but it has a lot of fans all over the world who register their pets with alternative cynological organizations.

Was born in Austria and subsequently imported into the USA, where it was very successful and was used in breeding, but then the question was raised about its purebredity, and the blue dogs were rejected, considering their progenitor to be a mestizo.Now we cannot establish whether he was actually not a purebred dog, or if it was just a genetic mutation. Ultimately, in the United States, blue dogs began to be registered and allowed to participate in exhibitions. Separately, I will add that the blue color does not carry any genetic diseases in this breed.

History of the origin of the breed

Weimaraner does not have any breed diseases inherent only to him. Some breeders do tests for hip and elbow dysplasia.

Great for a family with a child, very person-centered and requires constant attention. We train well, training is a must to control the dog in the city. Like most hunting dogs, it is not suitable for the role of watchman. Frequent and active walks are necessary; with a large minus, the dog may suffer from cold. Kept two Weimaraners. Both were workers, trained and trained. The dogs were both from the Czech Republic. He lived near the forest, so there were no problems with walking.

They obey perfectly, there was a case when a dog defended from an attack. An excellent choice for people with a private house or for those who live near forest plantations, you need to walk for an hour and a half twice a day. He is clearly content with life: do you hunt with him? If not, please tell us first-hand what it is like to keep a cop at home as a pet?

I love them immensely, but all the time it seems that I cannot cope with the loads. The orientation towards a person and their social group is frantic.Doesn’t let you leave the house.

The nature of the Weimaraner

He does not pay attention to other people at all. At home he listens perfectly, on the street he begins to tear down the tower, especially if there are many smells around. Gets in the ass, starts listening better. Alas, the dog is not working. Now in general, not many hunters take Weimars, rather, those who took them take up hunting. We also wanted so, but so far not.

When someone is at home with him, there is no destruction. In his youth, he gnawed at a wooden table, which was not a pity, although later they were weaned from this.But when he is alone, he can create game.

Moreover, in protest that he was left alone. At home he likes to lie and sleep, tug-of-war, look for hidden toys. Constantly runs after someone, like a tail.

Walked once a day. Moreover, twice be sure to run well. Every weekend we go somewhere: forest, field, in the heat of the sea. Loves to swim.

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Opens the swimming season very early. He can run through any terrain, he specifically does not care what is under his feet. Therefore, he constantly cuts them.Already they took it to sew up once. My girlfriend had a wild hysterics, the surgeon did not even remove from the procedural room, but I was praised for my help. In general, I can say that half an hour of non-stop running after the puller in the morning will calm down the Weimaraner at least until lunchtime. Such a house lives, the girl somehow took it from the previous owners, who wanted to put him to sleep.

Just a working character, as she said. An inexhaustible source of energy, second only to the husky. And he tries to hunt everyone from crows and pigeons to wagtails.Weimar Pointing Dog is only bred by club members. It was his representatives who helped restore the purebred Weimaraner population after World War II. With enough walks, the claws are grinded off naturally. The breed is clean enough. Cosmetics for washing can only be used special for dogs of short-haired breeds.

About six months after the change of teeth, another vaccination is given. Long-haired ones are offered slightly more expensive than short-haired counterparts. Movement should be free, behavior friendly.This gentle wet nose immediately won my heart.

90,000 WHEN TO BEGIN PREGNANT VITAMINS: WHY BETTER EARLIER – GOOD HEALTH

There are many restrictions on the types of medications and supplements you can take during pregnancy, but prenatal vitamins are not only legal, but highly recommended. Good p

Content:

There are many restrictions on the types of medications and supplements you can take during pregnancy, but prenatal vitamins are not only legal but highly recommended.

Good antenatal care can help keep you and your growing baby healthy by ensuring that both of you are getting all the nutrients you need to get through those 9 interdependent months of pregnancy.

If you and your baby need prenatal vitamins, why are so many health professionals advising women to start taking them? before pregnancy? It’s safe? Also, have you recently checked the vitamin series? There are plenty of options here.

Don’t worry – we’ll take care of you.

When to start taking prenatal vitamins?

There are two answers here, but (spoiler alert!) None of them involve waiting for an ultrasound in the first trimester.

When did you decide to try to get pregnant

Ready to start a family? In addition to scheduled visits to your gynecologist, giving up birth control, and quitting unhealthy behaviors such as smoking, you should start taking prenatal vitamins.

You won’t be able to predict how long it will take you to get pregnant – it can be weeks or months – and you will not know that you have succeeded until a few weeks after conception. Pregnancy vitamins are an important part of pre-conception baby care.

As soon as you find out that you are pregnant

If you are not yet taking prenatal vitamins, you should start as soon as you get a positive urine test result.

Ultimately, your obstetrician-gynecologist may suggest you a specific brand or even a recipe to make your vitamin-related life easier, but you don’t have to wait – every day matters when you are in the first trimester. why sec).

Why take them before you get pregnant?

The fact is that pregnancy takes a lot of your energy. Your cute little fruit actually drains your body’s natural resources greatly, which is why you spend so much time in these 9 months feeling nauseous, exhausted, weak, cramping, morose, tearful and forgetful.

Your baby gets all the nutrients it needs directly from you, so it is easy to become deficient in important vitamins and minerals during pregnancy.Making sure your body has everything it needs to nourish both of you will be much easier if you start. in front of child in the picture.

Think of it as building a reserve: if you have more than enough vitamins and nutrients to thrive, you can afford to share those vitamins and nutrients with your child as he grows.

What are the most important nutrients during the prenatal period, especially during the first month of pregnancy?

While it is important to have a balanced balance of vitamins and nutrients during pregnancy, some are truly MVPs because they actually help your baby build vital organs and body systems, many of which begin to develop in the very early weeks of pregnancy.

According to the American College of Obstetricians and Gynecologists (ACOG), these are the most important nutrients you need:

Folic acid

The grandfather of prenatal nutrients, this B vitamin is responsible for creating your baby’s neural tube or structure that ultimately forms the brain and spine. Without a fully developed neural tube, a baby may be born with spinal cleft or anencephaly.

Fortunately, everyone agrees here: Folate supplementation significantly increases the likelihood of healthy neural tube growth.The American Academy of Pediatrics has long held that folate can reduce neural tube defects by at least 50 percent.

The only snag? The neural tube closes during the first 4 weeks after conception, which often happens before or just after a woman realizes she is pregnant.

Because folic acid is very effective – but only if you get it at the right time – it is recommended that all sexually active women of childbearing age take 400 micrograms (mcg) of folate daily (either as a prenatal vitamin or individually.addition).

So you have it when you need it – even if you don’t expect it! Once pregnancy is confirmed, you will need at least 600 mcg per day.

Iron

Iron supplies the fetus with blood and oxygen, helps to form the placenta and provides the extra blood volume needed throughout pregnancy. Since pregnant women are prone to anemia, iron supplementation will also ensure that you have the correct number of red blood cells in your blood.

Anemia during pregnancy is associated with a higher rate of preterm birth and low birth weight.

Calcium

Your baby spends a lot of time in the uterus, strengthening his bones and teeth. To achieve this titanic feat, they need a lot of calcium, which means you need a lot of calcium.

If you don’t get enough calcium, your baby will get everything it needs straight from the bones during pregnancy and breastfeeding.This can lead to temporary bone loss.

Are there any side effects of taking prenatal medications when you are not pregnant?

Generally speaking, prenatal vitamins and nutrients do not cause harmful side effects – otherwise, pregnant women are not advised to take them!

However, prenatal vitamins contain nutrient levels typical of pregnant women, which means they are not always the best choice for non-pregnant people in the long term.

For example, during pregnancy, the need for iron will increase from 18 to 27 milligrams. Although the short-term side effects of too much iron include mild gastrointestinal upsets such as constipation and nausea, excess nutrients can become more problematic over time.

Bottom line? If you are not pregnant or planning to become pregnant, you can postpone prenatal procedures until you really need them (for example, a few months before pregnancy, during pregnancy, and often while breastfeeding).

Are there any additional benefits?

Some celebrities swear that the prenatal period is the secret of their glowing skin and luscious curls, because they contain biotin, one of the most important B vitamins.

Rumors about biotin’s ability to grow hair, nails and skin have been around forever; Many people take biotin supplements for this very reason.

However, it has not been possible to prove any significant beauty benefits from taking biotin, so the evidence remains strictly anecdotal.

But besides biotin there are there are some additional benefits for pregnant women. For example, if you take one with DHA, you get a boost in omega-3 fatty acids, which can help your child’s brain and eye development.

You can also get thyroid-regulating iodine, which can help develop your child’s nervous system.

Finally, there is some evidence that taking prenatal vitamins can increase your chances of pregnancy.

To be clear, prenatal is not a magic cure for infertility problems, and getting pregnant is not as easy as taking a pill. But many of the nutrients in prenatal vitamins regulate the body’s systems responsible for pregnancy.

So taking one – when done in conjunction with exercise, good nutrition, and the elimination of risk factors such as alcohol and drugs – can facilitate faster conception.

What should you pay attention to during the daily prenatal period?

There are dozens of options, but be sure to check a few key things before buying a prenatal vitamin:

Regulatory oversight

This is an unusual way to remind you that some certified organization has verified the health and ingredient claims made by your vitamin manufacturer.

Since the FDA does not regulate any nutritional supplements, including vitamins for pregnant women, are approved by groups such as the Food Supplements FDA or the USP.

Doses

Compare the amounts of key nutrients like iron and folic acid in your vitamin with the recommended amounts of ACOG. You don’t want to take vitamins that contain too much or too little of what you need.

Over-the-counter (OTC) or Prescription

Some insurance companies will cover some or all of your prenatal vitamin costs, saving you money. (OTC vitamins aren’t cheap!) If you have, you can ask your supplier for a prescription instead of buying yourself.

If you still have questions about choosing the right vitamin, do not hesitate to ask your doctor for advice. And pssst, we also have some thoughts on better prenatal periods.

Tips for taking prenatal vitamins

Do you suspect that the prenatal period is causing stomach upset? There are ways to minimize some of the more unpleasant effects.

• Ask your doctor about changing brands. Sometimes the prenatal period is phrased in such a way that you just don’t like it.
• Try a different method. Prenatal medications are often available as capsules, drinks, gummies, and even protein shakes – and can help digestion in a variety of ways. Try switching from one large capsule to three gummies a day, or split the two doses 12 hours apart.
• Drink plenty of water before and after. If you have constipation, make sure your gastrointestinal system is cleansed. You can also add fiber supplements if you’re feeling confident (but get a recommendation from your doctor first).
• Experiment with food. If vitamins make you sick, try taking them with or without food. Some people find it annoying to take vitamins on an empty stomach; others believe that can only take them on an empty stomach.

Conclusion

If you are seriously thinking about getting pregnant in the next few months, starting your prenatal vitamins should be the first on your to-do list to think about before conceiving.

If you are already pregnant, start taking as soon as possible.It will help your child become strong and healthy (and also help you stay strong and healthy!).

If you’re not seriously considering pregnancy right now, but technically could get pregnant, stick to your daily folate intake. This will give you what you need if you get pregnant – without unnecessary excess prenatal nutrients.

90,000 WHAT DO YOU NEED TO BECOME A NEUROSURGERY? – MEDICAL

A neurosurgeon, also known as a neurological surgeon, is a highly qualified medical professional who specializes in surgery of the brain, spinal cord, peripheral nerves

Content:

A neurosurgeon, also known as a neurological surgeon, is a highly trained medical professional who specializes in surgery of the brain, spinal cord, peripheral nerves, and cerebrovascular system.Neurosurgeons are trained to treat a wide range of congenital brain diseases, injuries, tumors, vascular diseases, infections, strokes, and degenerative diseases of the spine.

Education can take 14 to 16 years to become a fully board certified neurosurgeon. Some organize additional scholarships to specialize in a specific area of ​​neurosurgery.

Neurosurgeons are closely related to neurologists in that both require specialized knowledge of the nervous system.

While neurologists and neurosurgeons diagnose and treat neurological disorders, only neurosurgeons perform operations. Orthopedic surgery also often overlaps with neurosurgery when it involves the spine.

Overview of Neurosurgery

Concentrations

The nervous system is a complex, complex system that regulates and coordinates the body’s activities. As a medical field, neurology focuses on three specific organs: the central nervous system (CNS), the peripheral nervous system (PNS), and the intracranial cerebrovascular system (the network of arteries and veins that carry blood to the brain).

The conditions that a neurosurgeon can cause for treatment can be broadly described as their underlying cause. This includes:

• Congenital anomalies such as anencephaly, aneurysm, hydrocephalus, or spina bifida
• Traumatic injuries of the spinal cord, peripheral nerves or brain (including skull fractures and cerebral haemorrhage)
• Benign tumors or cancers of the brain or spine
• Vascular disorders , including arteriovenous malformations (AVM) and capillary telangiectasia
• CNS infections such as meningitis, encephalitis, vertebral osteomyelitis and epidural abscess
• Degeneral abscess
• spinal muscle atrophy (SMA) and hernia of the spine
• Epilepsy and movement disorders such as Parkinson’s and Huntington’s disease
• Treatment-resistant mental disorders including severe obsessive-compulsive disorder Syndrome Disorder (OCD), Tourette’s Syndrome and Major Depressive Disorder (DMD)
• Severe pain associated with cancer, trauma or other causes

Procedural Expertise

Neurosurgery requires a high level of technical expertise as well as exceptional manual dexterity.The tools used in the trade are extensive, many of which use cutting edge technology, including microsurgery and brain implants.

The key to the success of neurosurgery is the suite of radiological instruments used to diagnose and treat neurological disorders. This includes:

• Computed tomography (CT) Computed radiography, which creates three-dimensional “slices” of the brain or spinal cord
• Magnetic resonance imaging (MRI) , using magnetic and radio waves to create highly detailed images, especially soft tissue
• Positron Emission Tomography (PET) , which uses a radioactive tracer to assess metabolic function in the nervous system
• Magnetoencephalography (MEG) A technique for mapping the brain by recording nerve signals with magnetic receptors

Equipped with these imaging tools, a neurosurgeon can perform both conventional open surgery and minimally invasive surgical procedures.

Routine open surgery

Routine open surgery requires a neurosurgeon to open the skull. Typically used in emergencies to treat traumatic injuries. A technique known as a craniotomy uses specialized instruments to remove a piece of bone (called a bone flap) that is replaced after brain surgery is completed.

Endoscopic Surgery

Endoscopic surgery involves drilling the skull to insert a tubular instrument called an endoscope to transmit video images from deep inside the brain.Guided by live images, a neurosurgeon can insert surgical instruments through accessory holes to treat intracranial bleeding, tumors, hydrocephalus (“water in the brain”), and cerebrospinal fluid leaks, among others.

microsurgery

Microsurgery is often used to remove plaque from the carotid artery that feeds the brain (carotid endarterectomy), as well as to treat aneurysms, replace herniated discs (microdiscectomy), or decompress the vertebrae of the spine (laminectomy).

Neurosurgeons will use either an operating microscope with images projected onto a monitor or powerful magnifying glasses to aid in the operation.

Stereostatic radiosurgery

Stereotactic radiosurgery uses precisely directed radiation beams to pinpoint the location of brain tumors and other abnormalities. Cameras and electromagnetic fields guide the surgical procedure, much in the same way that the Global Positioning System (GPS) can help you navigate on the move.

Stereostatic radiosurgery is often used in combination with radiation therapy to treat tumors or AVMs. Radiosurgical techniques include gamma knife and cyberknife systems.

Stereotactic radiosurgery is increasingly being used to accurately place brain electrodes or to administer gene therapy in people with epilepsy, Parkinson’s disease or Alzheimer’s disease.

Endovascular surgery

Endovascular surgery involves the insertion of a surgical instrument through an opening in the femoral artery of the lower leg.It is used to treat diseases of the brain from within the blood vessel, including stroke, AVM, aneurysm, and brain tumors.

The circulatory pathway can be pre-examined using CT, MRI or high-resolution angiogram. The operation itself focuses on real-time X-rays.

Spinal neurosurgery

Spinal neurosurgery covers the cervical (cervical), thoracic (middle) and lumbar (lower) spine.It can be used to treat spinal cord compression from trauma, spinal disc arthritis, or spondylosis (characterized by bone spurs and disc degeneration).

Drills and special tools can be used to correct compression problems, while spinal needles (scissors used to knock out bone) can help remove herniated discs. Fusion can be performed as open or laparoscopic (keyhole) surgery.

Disc replacement or spinal fusion?

Psychiatric Neurosurgery

Neurosurgery can be used to treat psychiatric disorders that do not respond to standard medications, psychotherapy, or electroconvulsive therapy (ECT). Also known as psychosurgery, it remains a controversial practice with conflicting results. Modern psychiatric neurosurgery does not use many of the old methods commonly used in the past, such as lobotomy.

Today, most psychiatric neurosurgery focuses on deep brain stimulation (DBS) for the treatment of OCD and major depression.This includes the implantation of an electrical device to stimulate parts of the brain associated with mood or anxiety disorders.

Other surgical procedures

Chronic pain surgery is a subdivision of neurosurgery. Some of the techniques used include DBS, spinal cord stimulation, peripheral nerve stimulation, and pain pumps (implanted devices that deliver pain relievers over time).

Surgery of the peripheral nervous system is also possible.It can be used to decompress nerves associated with carpal tunnel syndrome (CTS), or to reposition pinched nerves that cause said pain.

narrow specialties

Because the functions of the brain and nervous system are so extensive and varied, neurosurgeons often limit their activities to specific populations or areas of the nervous system.

Neurosurgical specialties include:

• Endoscopic craniocerebral surgery
• Functional neurosurgery (used to treat movement disorders)
• Neurooncology (with brain tumors and cancer)
• Pediatric surgery 9040 Neurovascular surgery 9040 nerves
• Skull base neurosurgery (used to treat benign or malignant neoplasms on the underside of the skull and upper vertebrae)
• Spinal neurosurgery
• Stereostatic neurosurgery

Training and certification

The education required to become a neurosurgeon and extensive, requiring at least four years of undergraduate studies, four years of medical school, and five to seven years of internship.

After receiving a state medical license, neurosurgeons must practice for several years before they can even receive board certification through the American Board of Neurosurgery (ABNS).

In the United States, only 0.33 percent of all medical practitioners are neurosurgeons. Despite the financial reward, an alarming deficit remains across the country, according to a 2017 report by the New England Journal of Medicine.

6 Tips for Choosing a Great Surgeon

Appointment Tips

People are usually referred to a neurosurgeon in an emergency or when nonsurgical treatment fails.

In a non-emergency situation, you can get the most of your visit by documenting your symptoms before the appointment. This includes information about the time, severity, duration, and location of the symptoms, as well as what you did during each event. The more accurately you can describe your symptoms, the faster the neurosurgeon can order the correct tests and assessments.

On the day of your visit, bring your insurance ID card and any lab or image tests you may have.You should also ask your PCP to send all applicable Electronic Medical Records (EMRs) prior to your appointment.

How to get copies of your medical records

Be prepared to ask any questions you need to fully understand your condition and what to expect in the future.