What triggers a sickle cell crisis. How to prevent sickle cell crises. What are the main types of sickle cell crises. How to recognize symptoms of a sickle cell crisis. When is a sickle cell crisis considered a medical emergency. What are effective management strategies for sickle cell disease.

Understanding Sickle Cell Disease and Crises

Sickle cell disease (SCD) is a genetic condition affecting red blood cells (RBCs). In SCD, genetic mutations cause RBCs to become rigid and sickle-shaped, impacting their ability to transport oxygen efficiently. This abnormal shape can lead to blockages in blood vessels, resulting in severe pain episodes known as sickle cell crises.

These crises can occur suddenly and persist for days, weeks, or even months. While the exact causes aren’t fully understood, certain triggers can precipitate a crisis. Understanding these triggers and implementing preventive measures is crucial for managing SCD effectively.

Common Triggers of Sickle Cell Crises

Several factors can trigger a sickle cell crisis, often by causing blood vessel constriction:

• Physical or psychological stress
• Cold weather exposure
• Pain
• Alcohol consumption
• Tobacco use
• Dehydration
• Infections
• Low blood oxygen (hypoxemia)

Hypoxemia can result from strenuous exercise, high altitude, or certain medical conditions. It’s important to note that a crisis may be caused by a combination of these factors, making it challenging to identify a single trigger.

Types of Sickle Cell Crises

Sickle cell crises manifest in various forms, each with distinct characteristics:

Vaso-occlusive Crisis (VOC)

VOC is the most common type of sickle cell crisis. It occurs due to complex interactions among sickle cells, endothelial cells lining blood vessels, and other blood components. VOCs can cause severe, debilitating pain and often precede other complications.

Splenic Sequestration Crisis

This crisis type primarily affects young children and results in pain and swelling of the spleen due to blood entrapment within the organ.

Aplastic Crisis

An aplastic crisis occurs when the body fails to produce enough new RBCs to replace those already in circulation. Viral infections, such as parvovirus B19, can trigger this crisis, leading to sudden paleness and weakness.

Acute Chest Syndrome

Acute chest syndrome is the leading cause of hospitalization and death in individuals with SCD. Symptoms include chest pain, cough, fever, low blood oxygen, and lung infiltrates. This syndrome can develop following a VOC.

Hemolytic Crisis

During a hemolytic crisis, a large number of RBCs are destroyed rapidly, resulting in a sudden drop in hemoglobin levels and anemia.

It’s worth noting that individuals with SCD may experience multiple types of crises simultaneously.

Recognizing Symptoms of Sickle Cell Crises

Identifying the symptoms of a sickle cell crisis is crucial for timely intervention. Common symptoms include:

• Severe bone pain
• Chest pain and shortness of breath
• Severe infections (especially in children)
• Severe anemia
• Weakness and low blood pressure
• Loss of consciousness
• Jaundice (yellowing of skin, eyes, or mouth)
• Severe organ damage, including stroke

These symptoms typically develop over hours or days. In the case of a VOC, pain may be localized in specific body parts such as the back, legs, knees, arms, chest, or abdomen.

When Sickle Cell Crisis Becomes a Medical Emergency

Certain symptoms indicate a severe sickle cell crisis requiring immediate medical attention. The Centers for Disease Control and Prevention (CDC) recommends seeking emergency care if you have SCD and experience any of the following:

• Fever of 101°F (38°C) or higher
• Severe shortness of breath
• Intense chest pain
• Abdominal swelling
• Seizures
• Severe headaches
• Sudden weakness
• Decreased consciousness
• Painful erection lasting more than 4 hours
• Jaundice

Additionally, consult a doctor if you experience persistent pain unresponsive to home treatment or sudden vision issues.

Preventive Strategies for Sickle Cell Crises

While it’s not possible to prevent all sickle cell crises, implementing certain strategies can help minimize their occurrence:

1. Stay hydrated by drinking at least eight glasses of water daily.
2. Dress warmly in cold weather and always carry an extra layer.
3. Avoid high-altitude activities like mountain climbing or unpressurized air travel.
4. Engage in light to moderate exercise, avoiding strenuous activities.
5. Manage stress through relaxation techniques and adequate rest.
6. Maintain a balanced diet rich in fruits, vegetables, and whole grains.
7. Avoid alcohol and tobacco products.
8. Get regular check-ups and follow your doctor’s recommendations for preventive care.

Management Strategies for Sickle Cell Disease

Effective management of sickle cell disease involves a multifaceted approach:

Pain Management

Pain is a primary concern in SCD. How can pain be effectively managed during a crisis? A combination of over-the-counter pain relievers, prescription medications, and non-pharmacological approaches like heat therapy can be employed. It’s crucial to work with your healthcare provider to develop a personalized pain management plan.

Hydration Therapy

Maintaining proper hydration is essential in SCD management. Why is hydration so important? Adequate fluid intake helps prevent blood cells from becoming sickle-shaped and reduces the risk of vaso-occlusion. Intravenous fluids may be necessary during severe crises or hospitalizations.

Oxygen Therapy

During a crisis, oxygen therapy may be administered to increase blood oxygen levels and alleviate symptoms. This can be particularly beneficial in cases of acute chest syndrome or severe anemia.

Blood Transfusions

In some cases, blood transfusions may be necessary to treat severe anemia or prevent complications. What are the benefits of blood transfusions in SCD? They can increase the number of healthy red blood cells, improve oxygen delivery to tissues, and reduce the percentage of sickle cells in circulation.

Medications

Several medications can help manage SCD and prevent crises:

• Hydroxyurea: This drug can increase the production of fetal hemoglobin, reducing the frequency of painful crises.
• L-glutamine oral powder: This supplement can reduce the frequency of pain crises in some individuals with SCD.
• Voxelotor: This medication works by increasing hemoglobin’s affinity for oxygen, potentially improving anemia and reducing sickling of red blood cells.
• Crizanlizumab: This monoclonal antibody can help prevent vaso-occlusive crises by blocking the adhesion of blood cells to vessel walls.

Bone Marrow Transplant

For some individuals with severe SCD, a bone marrow transplant may be considered. This procedure can potentially cure SCD by replacing the patient’s bone marrow with healthy stem cells from a compatible donor.

Living with Sickle Cell Disease: Lifestyle Considerations

Managing SCD extends beyond medical interventions. Adopting a healthy lifestyle can significantly impact quality of life and reduce the frequency of crises.

Nutrition

A balanced diet is crucial for individuals with SCD. What nutrients are particularly important? Foods rich in folic acid, iron, and vitamins B12 and C can support red blood cell production and overall health. However, iron supplementation should only be taken under medical supervision, as excess iron can be harmful in SCD.

Exercise

Regular, moderate exercise can be beneficial for individuals with SCD. How does exercise help? It can improve cardiovascular health, boost mood, and increase overall well-being. However, it’s essential to avoid overexertion and stay hydrated during physical activities.

Stress Management

Stress can trigger sickle cell crises, making stress management techniques crucial. Effective strategies may include meditation, deep breathing exercises, yoga, or counseling.

Sleep Hygiene

Adequate sleep is vital for overall health and can help manage SCD symptoms. Establishing a consistent sleep schedule and creating a comfortable sleep environment can contribute to better rest and reduced stress levels.

Vaccination

Individuals with SCD are at higher risk for certain infections. Staying up-to-date with recommended vaccinations, including annual flu shots, can help prevent complications.

Support Systems and Resources for Sickle Cell Disease

Living with SCD can be challenging, but various support systems and resources are available to help individuals and their families cope with the condition:

Support Groups

Connecting with others who have SCD can provide emotional support and practical advice. Many hospitals and community organizations offer support groups for individuals with SCD and their families.

Educational Resources

Staying informed about SCD is crucial for effective management. Reputable organizations such as the Sickle Cell Disease Association of America and the National Heart, Lung, and Blood Institute offer comprehensive educational materials.

Financial Assistance Programs

The cost of SCD treatment can be substantial. Various programs and organizations offer financial assistance for medical expenses, medications, and other related costs.

Genetic Counseling

For individuals with SCD or carriers of the sickle cell trait, genetic counseling can provide valuable information about the condition and help with family planning decisions.

Mental Health Services

Living with a chronic condition like SCD can take a toll on mental health. Access to mental health professionals who understand the unique challenges of SCD can be beneficial.

By understanding the nature of sickle cell disease, recognizing crisis triggers, implementing preventive strategies, and utilizing available resources, individuals with SCD can better manage their condition and improve their quality of life. Regular communication with healthcare providers and adherence to personalized treatment plans are key components of successful SCD management.

Sickle Cell Crisis: Triggers, Prevention, and Management

Sickle cell disease (SCD) is a genetic condition that affects the shape and function of your red blood cells (RBCs).

RBCs transport oxygen to your body’s organs and tissues using a protein called hemoglobin to capture oxygen. Healthy RBCs are donut-shaped and very flexible, which allows them to move through the tiniest of your blood vessels, called the capillaries.

However, if you have SCD, certain genetic mutations make your RBCs rigid and shaped like the letter “C” or a sickle. This makes it hard for RBCs to capture and carry enough oxygen.

Sickle-shaped RBCs can also get stuck in your blood vessels, unable to reach many parts of your body. This can cause severe pain known as a sickle cell crisis. Sickle cell crises can start suddenly and last several days, sometimes even weeks or months.

Read this article to learn more about sickle cell crises, what can trigger them, and how you can manage or even avoid them.

Scientists don’t completely understand the exact causes of a sickle cell crisis. Most of the time, it happens because of a physical trigger, but it can also occur without a known cause.

Most commonly, any triggers that cause your blood vessels to constrict can result in a sickle cell crisis. These triggers include:

• physical or psychological stress
• cold weather
• pain
• alcohol
• tobacco products
• loss of fluids (dehydration)
• infection
• low blood oxygen (hypoxemia), which can result from very strenuous exercise, high altitude, or certain medical conditions

It can be hard to identify which trigger is responsible for each particular crisis. Often, it can be due to a combination of causes.

There are several types of sickle cell crises:

• Vaso-occlusive crisis (VOC): A VOC is the most common presentation of sickle cell crisis and can cause severe, often debilitating pain. It happens due to a complex interaction among sickle cells, endothelial cells (the lining of your blood vessels), and other cells in your blood.
• Splenic sequestration crisis: This causes pain and swelling of the spleen. It happens when a lot of blood becomes entrapped in the spleen. Splenic sequestration crises most commonly occur in young children.
• Aplastic crisis: This happens when your body doesn’t make enough new RBCs to replace the ones already in your blood. Some viral infections, such as parvovirus B19, can cause an aplastic crisis. These crises cause sudden paleness and weakness.
• Acute chest syndrome: This is the leading cause of hospitalization and death in people with SCD. It causes chest pain, cough, fever, low blood oxygen, and abnormal substances accumulating in the lungs (lung infiltrates). Acute chest syndrome can follow VOCs.
• Hemolytic crisis: This happens when large numbers of RBCs are destroyed over a short time. It causes a sudden drop in hemoglobin levels (anemia).

There are other, less frequent types of sickle cell crises, most commonly occurring as complications of VOCs.

Keep in mind that you can have more than one type of sickle cell crisis at a time.

The most common symptoms of sickle cell crises are:

• excruciating bone pain
• chest pain and shortness of breath
• severe infections (usually in children)
• severe anemia
• weakness and low blood pressure
• loss of consciousness
• yellowing of the skin, eyes, or inside of mouth (jaundice)
• severe organ damage, including stroke

These symptoms usually develop over hours or days.

A VOC, which usually precedes other complications, can cause pain in the following body parts:

• back
• legs
• knees
• arms
• chest
• abdomen

Is sickle cell crisis a medical emergency?

You should get treatment for a severe sickle cell crisis right away to avoid life threatening complications.

The Centers for Disease Control and Prevention (CDC) recommends having a plan for getting help immediately, at any hour, in case of a problem.

Keep a printed copy of your medical records, pain management plan, and a list of all your medications to bring with you to the medical facility.

You should call 911, use local emergency services, or go to an urgent care facility right away if you have SCD and any of the following symptoms:

• fever of at least 101°F (38°C)
• shortness of breath
• chest pain
• abdominal swelling
• seizure
• severe headache
• sudden weakness
• decreased consciousness
• a painful erection that lasts more than 4 hours
• jaundice

Call a doctor if you experience one of the following:

• pain anywhere in the body that won’t go away with home treatment
• any sudden vision issues

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You can’t prevent all sickle cell crises, but you can avoid triggers and therefore minimize them by:

• drinking plenty of water (at least eight glasses a day) to avoid dehydration
• dressing warmly in cold weather and carrying an extra layer just in case
• avoiding mountain climbing or air flights in an unpressurized cabin
• sticking to light to moderate exercise
• paying attention to your stress levels
• washing your hands often to avoid infections
• being sure to get all recommended vaccinations, including flu and coronavirus shots and boosters
• taking folic acid supplements to prevent anemia
• avoiding alcohol and tobacco products

In addition, be sure to follow a doctor’s recommendations, including taking all recommended medications on time.

Not all sickle cell crises warrant a trip to the emergency room. You can start with home management, but it’s important to get immediate medical attention if home treatment doesn’t have the desired effect.

Home management includes:

• over-the-counter (OTC) pain relievers
• heating pads
• drinking plenty of water
• warm baths
• massage of the affected area
• rest

If home management doesn’t work, a doctor will likely prescribe you stronger medications for pain. In addition, they’ll check for an underlying infection or dehydration that might be triggering the crisis. They may also give you intravenous (IV) fluids. In very severe cases, you may need a blood transfusion.

Certain medications are approved by the Food and Drug Administration (FDA) to reduce the frequency and duration of VOCs:

• hydroxyurea
• L-glutamine
• crizanlizumab (Adakveo)
• voxelotor (Oxbryta)

Read more about the potential cure for SCD.

A sickle cell crisis is a very painful complication of SCD. It has many triggers, most of which cause constriction of your blood vessels, resulting in the clumping of blood cells in the capillaries.

You can treat mild pain at home, but more severe cases require immediate medical attention. You can prevent sickle cell crises by avoiding their triggers.

Sickle Cell Disease | Johns Hopkins Medicine

What is sickle cell disease?

Sickle cell disease is an inherited blood disorder. It is marked by flawed hemoglobin. That’s the protein in red blood cells that carries oxygen to the tissues of the body. So, sickle cell disease interferes with the delivery of oxygen to the tissues.

Red blood cells with normal hemoglobin are smooth, disk-shaped, and flexible, like doughnuts without holes. They can move through the blood vessels easily. Cells with sickle cell hemoglobin are stiff and sticky. When they lose their oxygen, they form into the shape of a sickle or crescent, like the letter C. These cells stick together and can’t easily move through the blood vessels. This can block small blood vessels and the movement of healthy, normal oxygen-carrying blood. The blockage can cause pain.

Normal red blood cells can live up to 120 days. But, sickle cells only live for about 10 to 20 days. Also, sickle cells may be destroyed by the spleen because of their shape and stiffness. The spleen helps filter the blood of infections. Sickled cells get stuck in this filter and die. With less healthy red blood cells circulating in the body, you can become chronically anemic. The sickled cells also damage the spleen. This puts you are at greater at risk for infections.

What causes sickle cell disease?

Sickle cell is an inherited disease caused by a defect in a gene.

• A person will be born with sickle cell disease only if two genes are inherited—one from the mother and one from the father.
• A person who inherits just one gene is healthy and said to be a “carrier” of the disease. A carrier has an increased chance of having a child with sickle cell disease if he or she has a child with another carrier. 

For parents who are each carriers of a sickle cell gene, there is a 1 in 4, or a 25 % chance of having a child with sickle cell disease.

What are the risk factors for sickle cell disease?

Having a family history of sickle cell disease increases your risk for the disease. In the United States, it mainly affects African Americans.

What are the symptoms of sickle cell disease?

The following is a list of symptoms and complications associated with sickle cell disease. However, each person may experience symptoms differently. Symptoms and complications may include:

• Anemia. Because sickled cells are short-lived or destroyed, there are less red blood cells available in the body. This results in anemia. Severe anemia can make you feel dizzy, short of breath, and tired.
• Pain crisis, or sickle crisis.  This occurs when the flow of blood is blocked to an area because the sickled cells have become stuck in the blood vessel. The pain can occur anywhere, but most often occurs in the chest, arms, and legs. Infants and young children may have painful swelling of the fingers and toes. Interruption in blood flow may also cause tissue death.
• Acute chest syndrome. This occurs when sickling occurs in the chest. This can be life-threatening. It often occurs suddenly, when the body is under stress from infection, fever, or dehydration. The sickled cells stick together and block the flow of oxygen in the tiny vessels in the lungs. It resembles pneumonia and can include fever, pain, and a violent cough.
• Splenic sequestration (pooling). Crises are a result of sickle cells pooling in the spleen. This can cause a sudden drop in hemoglobin and can be life-threatening if not treated promptly. The spleen can also become enlarged and painful from the increase in blood volume. After repeated episodes,  the spleen becomes scarred, and permanently damaged. Most children, by age 8, do not have a working spleen either from surgical removal, or from repeated episodes of splenic sequestration. The risk of infection is a major concern of children without a working spleen. Infection is the major cause of death in children younger than age 5 in this population.
• Stroke. This is another sudden and severe complication of people with sickle cell disease. The misshapen cells can block the major blood vessels that supply the brain with oxygen. Any interruption in the flow of blood and oxygen to the brain can result in severe brain damage. If you have one stroke from sickle cell anemia, you are more likely to have a second and third stroke.
• Jaundice, or yellowing of the skin, eyes, and mouth. Jaundice is a common sign and symptom of sickle disease. Sickle cells do not live as long as normal red blood cells and, therefore, they are dying faster than the liver can filter them out. Bilirubin (which causes the yellow color) from these broken down cells builds up in the system causing jaundice.
• Priapism.  This is a painful obstruction of the blood vessels in the penis by sickle cells. If not promptly treated, it can result in impotence.

The symptoms of sickle cell disease may look like other blood disorders or medical problems. Always consult your health care provider for a diagnosis.

How is sickle cell disease diagnosed?

Along with a complete medical history and physical exam, you may have blood and other tests.

Many states routinely screen newborns for sickle cell so that treatment can begin as soon as possible. Early diagnosis and treatment can reduce the risk of complications.

Hemoglobin electrophoresis is a blood test that can determine if a person is a carrier of sickle cell, or has any of the diseases associated with the sickle cell gene.

How is sickle cell disease treated?

Your doctor will consider your age, overall health and other factors when determining the best treatment for you.

Early diagnosis and prevention of complications is critical in sickle cell disease treatment. Treatment aims to prevent organ damage including strokes, prevent infection, and treat symptoms. Treatment may include:

• Pain medications. This is for sickle cell crises.
• Drinking plenty of water daily (8 to 10 glasses). This is to prevent and treat pain crises. In some situations, intravenous fluids may be required.  
• Blood transfusions. These may help treat anemia and prevent stroke. They are also used to dilute the sickled hemoglobin with normal hemoglobin to treat chronic pain, acute chest syndrome, splenic sequestration, and other emergencies.
• Vaccinations and antibiotics. These are used to prevent infections.
• Folic acid. Folic acid will help prevent severe anemia.
• Hydroxyurea. This medication helps reduce the frequency of pain crises and acute chest syndrome. It may also help decrease the need for blood transfusions. The long-term effects of the medication are unknown.
• Regular eye exams. These are done to screen for retinopathy.  
• Bone marrow transplant. Bone marrow transplants can cure some people with sickle cell disease. The decision to have this procedure is based on the severity of the disease and ability to find a suitable bone marrow donor. These decisions need to be discussed with your doctor and are only done at specialized medical centers.

What are the complications of sickle cell disease?

Any and all major organs are affected by sickle cell disease. The liver, heart, kidneys, gallbladder, eyes, bones, and joints can suffer damage from the abnormal function of the sickle cells and their inability to flow through the small blood vessels correctly. Problems may include the following:

• Increased infections
• Leg ulcers
• Bone damage
• Early gallstones
• Kidney damage and loss of body water in the urine
• Eye damage
• Multiple organ failure

Living with sickle cell disease

Sickle cell disease is a life-long condition. Although the complications of sickle cell disease may not be able to be prevented entirely, living a healthy life-style can reduce some of the complications.

It is important to eat a healthy diet with lots of fruits, vegetables, whole grains, and protein, and drink lots of fluids.

Do not take decongestants because they cause constriction of blood vessels and could trigger a crisis.

Other factors that may trigger a crisis include high altitudes, cold weather, swimming in cold water, and heavy physical labor.

Avoid infections by getting an annual flu shot, washing your hands frequently, avoiding those who are sick, and getting regular dental exams.

Key points

• Sickle cell disease is an inherited blood disorder marked by defective hemoglobin.
• It inhibits the ability of hemoglobin in red blood cells to carry oxygen.
• Sickle cells tend to stick together, blocking small blood vessels causing painful and damaging complications.
• Sickle cell disease is treated with pain medications as needed, drinking 8 to 10 glasses of water each day, blood transfusions, and medications.

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• If you have a follow-up appointment, write down the date, time, and purpose for that visit.
• Know how you can contact your provider if you have questions.

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