What is the relationship between Costochondritis and Ehlers-Danlos Syndrome. How does Costochondritis affect those with hypermobile EDS. What are effective treatments and management strategies for Costochondritis in EDS patients.

The Complex Interplay of Costochondritis and Ehlers-Danlos Syndrome

Costochondritis and Ehlers-Danlos Syndrome (EDS), particularly the hypermobile type (hEDS), often coexist, creating a challenging medical scenario for those affected. This intricate relationship stems from the underlying connective tissue abnormalities characteristic of EDS, which can predispose individuals to inflammation and instability in the costal cartilages connecting the ribs to the sternum.

Individuals with hEDS may experience more frequent and severe episodes of costochondritis due to the increased laxity of their connective tissues. This laxity can lead to excessive movement and stress on the costochondral junctions, triggering inflammation and pain.

Why is costochondritis more common in EDS patients?

The prevalence of costochondritis in EDS patients can be attributed to several factors:

• Hypermobility of the rib cage and spine
• Increased susceptibility to tissue inflammation
• Poor proprioception and body awareness, leading to inadvertent strain
• Delayed tissue healing due to collagen abnormalities

Recognizing the Symptoms of Costochondritis in EDS

Identifying costochondritis in the context of EDS can be challenging, as the symptoms may overlap with other comorbidities. However, certain hallmarks can help distinguish this condition:

• Sharp, stabbing pain in the chest wall, often mistaken for cardiac issues
• Tenderness and swelling along the costal cartilages
• Pain exacerbation with deep breathing, coughing, or physical activity
• Possible radiation of pain to the back or abdomen

It’s crucial for EDS patients experiencing these symptoms to seek medical evaluation to rule out other potential causes of chest pain.

How does costochondritis pain differ from other types of chest pain?

Costochondritis pain is typically:

• Localized to specific areas along the chest wall
• Reproducible with palpation or specific movements
• Not directly related to exertion or relieved by rest, unlike angina
• Often accompanied by visible swelling or redness in the affected area

Diagnostic Challenges and Approaches for Costochondritis in EDS

Diagnosing costochondritis in EDS patients requires a comprehensive approach, as traditional diagnostic methods may be less reliable due to the underlying connective tissue disorder. Healthcare providers should consider the following strategies:

1. Thorough physical examination, including careful palpation of the chest wall
2. Detailed patient history, focusing on the nature and triggers of pain
3. Exclusion of other potential causes through appropriate imaging and laboratory tests
4. Consideration of the patient’s EDS diagnosis and its potential influence on symptoms

Are there specific diagnostic tests for costochondritis in EDS patients?

While there is no definitive test for costochondritis, especially in the context of EDS, the following diagnostic tools may be helpful:

• Ultrasound imaging to assess inflammation in the costal cartilages
• MRI to evaluate soft tissue abnormalities and rule out other conditions
• Bone scans to identify areas of increased metabolic activity
• Specialized physical examination techniques tailored to hypermobile patients

Management Strategies for Costochondritis in EDS Patients

Effective management of costochondritis in EDS requires a multifaceted approach that addresses both the acute symptoms and the underlying connective tissue disorder. Key components of a comprehensive treatment plan may include:

• Pain management through appropriate medications and topical treatments
• Physical therapy focused on improving posture and strengthening supportive muscles
• Gentle stretching and range of motion exercises to maintain flexibility without exacerbating symptoms
• Use of supportive devices such as rib belts or kinesiology tape to provide stability
• Stress reduction techniques to minimize tension in the chest and upper body
• Patient education on proper body mechanics and activity modification

Which pain management techniques are most effective for costochondritis in EDS?

While individual responses may vary, many EDS patients find relief from costochondritis pain through:

• Topical anti-inflammatory creams or gels
• Gentle heat or cold therapy, depending on personal preference and response
• Low-dose, short-term use of oral anti-inflammatory medications, when appropriate
• Transcutaneous electrical nerve stimulation (TENS) therapy
• Acupuncture or dry needling, performed by practitioners experienced with EDS

The Role of Physical Therapy in Managing Costochondritis with EDS

Physical therapy plays a crucial role in the long-term management of costochondritis in EDS patients. A tailored physical therapy program can help:

• Improve posture and body awareness to reduce stress on the costal cartilages
• Strengthen the muscles supporting the rib cage and upper body
• Enhance proprioception to prevent overextension and injury
• Develop pain management strategies specific to the individual’s needs
• Educate patients on proper breathing techniques and ergonomics

What specific physical therapy techniques are beneficial for EDS patients with costochondritis?

Effective physical therapy interventions may include:

1. Gentle manual therapy to improve rib mobility and reduce inflammation
2. Postural retraining exercises to alleviate stress on the costal cartilages
3. Core strengthening to improve overall stability and support
4. Breathing exercises to enhance lung capacity without exacerbating pain
5. Proprioceptive training to improve body awareness and prevent overextension

Lifestyle Modifications to Manage Costochondritis in EDS

Adapting daily activities and habits can significantly impact the frequency and severity of costochondritis flares in EDS patients. Key lifestyle modifications to consider include:

• Ergonomic adjustments to workspaces and sleeping environments
• Careful pacing of activities to avoid overexertion
• Use of supportive pillows and cushions to maintain proper posture
• Adoption of low-impact exercise routines that don’t strain the chest wall
• Stress management techniques such as meditation or gentle yoga
• Dietary considerations to reduce inflammation and support overall health

How can EDS patients modify their daily activities to minimize costochondritis flares?

Practical strategies for daily living include:

• Using a backpack or rolling bag instead of carrying heavy shoulder bags
• Avoiding repetitive arm movements that stress the chest wall
• Taking frequent breaks during activities that require prolonged sitting or standing
• Practicing good sleep hygiene and using supportive pillows to maintain proper alignment
• Employing assistive devices for tasks that may strain the upper body

The Importance of Mental Health Support for EDS Patients with Costochondritis

Living with chronic pain conditions like costochondritis, especially in the context of EDS, can take a significant toll on mental health. Addressing psychological well-being is crucial for overall management and quality of life. Consider the following aspects of mental health support:

• Cognitive-behavioral therapy to develop coping strategies for pain and stress
• Support groups or peer counseling to connect with others facing similar challenges
• Mindfulness practices to reduce anxiety and improve pain perception
• Psychoeducation on the relationship between chronic pain and mental health
• Exploration of creative outlets as a form of emotional expression and stress relief

How does addressing mental health impact the management of costochondritis in EDS patients?

Prioritizing mental health can lead to:

1. Improved pain management through stress reduction and relaxation techniques
2. Enhanced adherence to treatment plans and lifestyle modifications
3. Better communication with healthcare providers about symptoms and concerns
4. Increased resilience in facing the challenges of chronic pain and disability
5. Overall improvement in quality of life and daily functioning

Navigating the Healthcare System with Costochondritis and EDS

Effectively managing costochondritis in the context of EDS often requires navigating a complex healthcare system. Patients may benefit from the following strategies:

• Assembling a multidisciplinary care team familiar with EDS and its comorbidities
• Maintaining detailed records of symptoms, treatments, and their effectiveness
• Advocating for comprehensive evaluations that consider the interplay between EDS and costochondritis
• Seeking second opinions when necessary, particularly from EDS-knowledgeable specialists
• Exploring integrative medicine approaches that complement traditional treatments
• Staying informed about current research and treatment options for both conditions

How can EDS patients effectively communicate their costochondritis symptoms to healthcare providers?

To ensure clear communication with healthcare providers, patients should:

1. Keep a detailed pain diary, noting triggers, duration, and intensity of symptoms
2. Use specific language to describe pain characteristics and location
3. Provide a comprehensive medical history, including EDS diagnosis and other comorbidities
4. Prepare questions in advance of appointments to address key concerns
5. Bring a trusted friend or family member to appointments for support and advocacy

Living with costochondritis and EDS presents unique challenges, but with proper understanding, management, and support, individuals can achieve improved quality of life and symptom control. By addressing both the physical and psychological aspects of these conditions, patients can develop effective strategies for long-term well-being. Continued research and increased awareness among healthcare providers will further enhance the care available to those navigating the complex interplay of costochondritis and Ehlers-Danlos Syndrome.

Comorbidities Common with Ehlers Danlos Syndrome

As I’ve mentioned numerous times throughout this blog, people with a form of Ehlers-Danlos Syndrome or Hypermobility Spectrum Disorders seriously win the bad health lottery when it comes to comorbidities, or conditions you have along with a primary condition. (Aka “co-occurring conditions”.) In fact, the list sometimes seems a little unreal, as it hardly seems possible a body could experience so much trouble.

Edited to add (ETA) March 1, 2022: I am finding “co-occurring conditions” to be a preferred term over time, as it’s less pathologizing and, sounds less, well, “morbid”. Alas, the title of this page is probably linked in many places so I probably won’t change it any time soon. But I just wanted to note that I’m slowly changing my terminology with time. I will probably use a mix of the two below for the foreseeable future.

But again, if I didn’t have the pleasure of experiencing several of them myself, and observe upwards of 10,000 fellow patients daily in various online support groups with every variation on the theme, I would have trouble believing it too.

But it’s true. And the sooner the medical system wraps their collective brain around this painful fact, the quicker we’ll save time, money, energy and suffering for all involved. One group now has over 30,000 –now 60,000! – in it. They keep growing quickly!

No, we won’t have everything listed below in the same patient. But… very often one person will experience a fair number, easily ten or more of the following. And family members (if known) may experience even more as well as overlapping ones with the patient. Do keep an open mind!

Mast Cell Activation Disorders

I think it helps when you remember that collagen runs through 80% of the body, so affects virtually every system. To borrow a phrase from MCAD savvy Dr. Afrin, a la Occam’s Razor, “do you really think there are 57 different things wrong with your patient, or maybe just a couple underlying systemic conditions that might explain it all?”

That said, one of the most common, with some of the most disabling effects yet least often diagnosed is any form of MCAD, or  Mast Cell Activation Disorders , which includes the more rare forms of Mastocytosis and the newly recognized much more common, but rarely diagnosed MCAS: Mast Cell Activation Syndrome. The Mast Cell Diseases Society (formerly The Mastocytosis Society until 2020) just established an ICD-10 code for this in 2016 after lots of hard work. (As well as HaTS since 2016.)

Think of MCAS as being like “extra” or “hidden” non-food and chemical “allergies” that traditional allergy testing cannot find. (The mast cells are being activated, but NOT via the traditional IgE-mediated pathway, but this can result in anaphylactoid reactions – just like allergies – just the same, though not all episodes will be fully throat-closing or require epi, so may not be recognized.)

Dr. Josh Milner at the NIH actually studied this comorbidity of atopy (allergic diseases including MCAD) and connective tissue disorders (most commonly hypermobile Ehlers-Danlos) as he shared here in the summer of 2015 at the 3rd Annual Dysautonomia International Conference. (The forms of HSD weren’t invented yet.)  The study ultimately found a subset of patients who have a form of Hereditary Alpha Tryptasemia Syndrome (or Familial Tryptasemia), that is, a baseline elevated tryptase exhibiting a familial (genetic) inheritance pattern. (Described here and here in autumn, 2016.)

I’ve been given to understand that all with this condition are hypermobile, leading many to suspect it may play a role in hypermobility or hEDS. That said, this only explains a very small subset of the hEDS patient population still and this hasn’t been confirmed with any studies yet. (Correlation does not equal causation.)  In other words, not all with HaTS have hEDS, and vice versa: not all with hEDS necessarily have HaTS.

Many more like myself are still very hypermobile with lots of MCAS reactions in the absence of elevated tryptase, begging the question: what else is causing our hypermobility? And others suffer oodles of soft tissue troubles and tears, and all of the pain and comorbidities of hEDS, but are not flexible at all, and never have been. And it is unclear if all have elevated tryptase or not. (I doubt it.) And I was actually in the study.

MCAD manifests in lots of GI trouble (nausea, vomiting, constipation and sudden diarrhea), frequent urination, skins signs including flushing and various rashes including hives and urticaria pigmentosa (persistent hive-like patches that wax and wane), and all signs of the various levels of anaphylaxis, which most don’t realize comes in grades according to the number of body systems involved. (Not all is throat closing!)

I’ll share more on this elsewhere, but just wanted to be sure people were aware how common we are finding it in the EDS /HMS /Fibromyalgia and autistic community. It is often mis-diagnosed as Multiple Chemical Sensitivity or porphyria. Here’s a bit on when to suspect it.

Lastly, I’ll note that there are often BOTH bio-mechanical (physical, e.g. impinged nerves, muscle tension, herniations, loose ligaments), and bio-chemical (e.g. allergy, MCAD) causes to many of our issues (e.g. headaches, frequent urination) lending to the diagnostic and treatment challenge! Don’t forget to consider both when trying to diagnose and treat. Yes, tricky! Last updated April 15, 2023:

Semi-common Comorbidities (Unofficial)

With that, here is an unofficial list of common and semi-common comorbidities (besides hypermobility)  I’ve observed in the EDS community online:

• Fibromyalgia
• Chronic fatigue, often diagnosed as CFS/ME
• Chronic Pain of ALL kinds, including RSD/CRPS, arachnoiditis, neuropathies, radiculopathy, neuralgia, fibromyalgia, etc. often invisible on scans
• MCAD (Mastocytosis or the newly recognized Mast Cell Activation Syndrome aka MCAS – ICD-10 code to come soon hopefully in 2014)
• Fibromyscular dysplasia (deformity of the arteries, especially leading to the kidneys)
• Autism  (all “levels”) and related conditions (OCD, ADHD, SPD, anxiety, depression)
• Auditory Processing Disorders
• Semi-circular Canal Dehiscence SSCD (a rare bony defect in the petrosal temporal bone, creating a new opening between the inner ear and middle cranial fossa) causing auditory issues (hearing bodily sounds much louder than external sounds, more), can be on one side, or bilateral (both sides)
• Apraxia (trouble actually moving the mouth properly to speak) and/ or situational (so-called “selective” which is a misnomer) mutism in some settings or speech burnout even in hyperverbal people who have “hit a wall” or are too stressed sometimes. Also, stuttering.
• Dystonia – or movement disorders from multiple causes too (including dopa responsive dystonia)
• Mood disorders, especially anxiety and depression (likely organic in origin and/or secondary from things like histadelia or high histamine from occult MCAD)
• Involuntary Emotional Expression Disorder (IEED) (co-occurs with POTS) – crying easily, unexpectedly
• Thyroid issues (high and low, often auto-immune despite normal TSH “levels”)
• MS and other auto-immune disorders (RA, Sjogren’s, Lupus, Ankylosing Spondylitis, OA, more)
• Arthritis of all kinds, especially early onset Oestoarthritis in the spine, neck and hands, but RA common also (really AI also)
• Irritable Bowel Syndrome (IBS) & proclivity toward constipation, but with quick flips to diarrhea (likely food allergies/MCAD)
• “No burp syndrome“, aka C-RPD in which people cannot burp at all or ever, not just sometimes
• Intussusception (folding over/overlapping) of the GI tract at any age
• Compression syndromes, including: Median Arcuate Ligament Syndrome (MALS), Nutcracker Syndrome (NCS), Superior Mesenteric Artery Syndrome (SMAS), and May Thurner (MT)
• Incontinence at any age (often from occult tethered cord and/or MCAD or allergy induced)
• Uterine or rectal prolapse, pelvic floor dysfunction
• Frequent (seemingly idiopathic) nausea and vomiting (may be from impinged vagus nerve/MCAD/hiatal hernia/gastroparesis/Chiari) including cyclical vomiting syndrome
• Dysautonomia of all kinds, most notably poor temperature and BP regulation (high or low, see POTS below)
• Raynaud’s phenomon (blood vessel constriction from cold, stress) pronounced “ray-noe’s”) – a form of dysautonomia
• POTS (Postural Orthostatic Tachycardia Syndrome) – a subset of dysautonomia involving BP drops and syncope (fainting)
• Hyperadrenergic POTS (aka HyperPOTS) – a subtype of POTS involving more variable BP and adrenaline responses
• NMH (Neurally Mediated Hypotension) – another form of dysautonomia affecting BP
• Livedo Reticularis (purplish/white “mottling” on skin surface from likely small capillary spasming)
• Frequent joint dislocations and subluxations (partial dislocations) or being so-called “double-jointed“
• Club foot at birth
• Partial syndactyly (connected fingers or toes seen at birth)
• Dupuytren’s Contracture of the hand
• Snapping Hip Syndrome
• Slipping rib or ribs
• Mitochondrial disorders and diseases
• Kidney trouble including diabetes insipidus
• Diabetes miellitus and Metabolic X syndrome
• Sensory Processing Disorders
• Tinnitus (ringing in the ears)
• Empty Sella Syndrome (common wtih Chiari Malformation apparently too fwiw)
• Insomnia (trouble falling and staying asleep, multiple causes in the EDS patient including pain and hyperadrenergia)
• Sleep apnea, both obstructive airway issues and Central Nervous System (CNS) Apnea (neurologic in origin requiring a sort of breathing “pace maker”)
• Cranio-cervical settling (which may cause the CNS Apnea) and attendant neuropathic issues and glaucoma
• Hypotonia (unusually weak muscles despite “training”) sometimes presenting as “floppy babies”
• Syncope and pre-syncope (fainting and near fainting) and unusually low BP
• Dizzyness (with or without syncope, or fully passing out)
• Common Variable Immune Deficiency (CVID) of all kinds leaving us prone to frequent & worsening recurrent infections of all kinds, especially respiratory & UTI’s
• Deviated septum
• Chronic and recurrent sinus infections and sinusitis (treating MCAD helps some), swollen turbinates
• Interstitial cystitis
• Lymphedema (usually secondary and bilateral) and angioedema (the latter comes with MCAD triggering usually)
• Lipedema (also spelled “lipoedema” in the UK, not the same as lymphedema, which often comes with it but is secondary ) an adipose (fat) tissue disorder causing unavoidable weight gain
• Dercum’s disease (see Lipedmea above, looks a lot like that plus MCAD) causes fatty lipomas
• Endometriosis
• PCOS and menorraghea (very heavy periods)
• NCAH (Non-classical Congenital Adrenal Hyperplasia) has also been associated with some EDS
• Tendonitis and bursitis of all kinds (aka “soft tissue rheumatism”, alt. tendinitis)
• Keratoconus or thinning /”pointy” corneas that lend to astigmatism
• Uveitis (inflammation of hte uvea of the eye, common with ankylosing spondylitis)
• Varicose and spider veins, often early onset, easy bruising and bleeding from same
• Phlebitis
• Migraines and headaches of ALL kinds and durations, (often driven by hydrocephalus from MCAD)
• Food and drug allergies and sensitivities with a lot of paradoxic and unexpected super sensitive reactions
• Bleeding disorders including Von Willebrand’s
• Strokes
• Mitral valve prolapse
• Aneurysms of all kinds, anywhere
• Easy bruising often from no apparent cause or injury
• GERD (weak hiatal sphincters and MCAD can contribute here – the stomach produces acid in response to histamine from food reactions)
• Gastroparesis (slow or no stomach emptying) and dysmotility (poor digestive movement) beyond just constipation and IBS, possibly from impinged nerves & vessels
• Idiopathic postprandial syndrome and/ or “Adrenergic hormonal postprandial Syndrome” (sort of “pseudo-hypglycemia” in absence of low blood sugar measures)
• Chiari malformation, including occult (hidden) Chiari aka “Chiari Zero” formation (“saggy” hind brain, often protruding through the back skull, but not always)
• Tethered cord
• Syringomyelia or “syrinx formation” in the spinal cord (may be caused by long term hydrocephalus)
• Ganglion cysts, Baker’s cysts and Tarlov cysts
• Spondylolysthesis, spondylolisis (misalignment of the spinal vertebrae in various directions – front -to-back, side-to-side)
• Cranial cervical instability, esp C1-C2 “owl turns” and “bobble-head” issues (trouble keeping head on neck, literally, with resulting neurologic issues)
• Thoracic outlet syndrome, brachial outlet syndrome
• Seizure disorders and epilepsy
• Fallen arches (pes planus)
• Sciatica
• Bilateral hip dysplasia (both at birth, and later ability to “pop” – aka sublux – hips out and back in easily, which should be avoided!)
• Pelvic Obliquity (offset hip bones, torsion in lower spine)
• Cerebral Spinal Fluid (CSF) leaks including CSF (watery) rhinorrhea, CSF otorrhea (CSF leaks out nose and ears) or anywhere along the dura (lining of spinal column & brain)
• Celiac disease and all forms of gluten sensitivity
• Malabsorption and malnutrition and nutritional deficiencies despite diet and even supplementation sometimes. (Poor absorption). Especially low magnesium which lends to Hypokalemia.
• Electrolyte imbalances (often low potassium)
• Osteopoenia (low bone density) and osteoporosis (brittle bones), often early onset
• Scoliosis (deformity of spinal curve) of all kinds including kyphosis (aka “roundback”, forward bent spine)
• Hiatal hernia (stomach to esophagus sphincter) and all other forms of hernias just about anywhere (inguinal, duodenal, abdominal, etc.)
• Costochondritis (pain at front rib attachment point to sternum)
• Chondromalacia (cartilage loss) of all kinds, especially patellae (loss of cartilage in the knees, but can occur elsewhere, e.g. hips)
• Frozen shoulder
• Petechiae (reddish purple spots) or Purpura (essentially flat blood blisters under the skin especially in MCAD patients)
• Metal and other environmental allergies, especially nickel sensitivity (ELISA testing often helpful, pre-test all implant materials)
• Diastasis recti, (splitting of abdominal wall along the midline) even in males and unpregnant females
• Striae aka “stretch marks” even in males and young (prepubescent) females (i. e. not always associated with pregnancy!)
• Myopia (often severe), macular degeneration, astigmatism, keratoconus
• Strabismus (crossed eyes) or wandering eyes from likely weak eye ligaments
• Bruxism (jaw clenching, tooth grinding)
• TMJ pain and issues, subluxations and dislocations (Temporo-mandibular joint syndrome, jaw alignment trouble)
• Restless Leg Syndrome (RLS) and leg cramps (often eased by increased magnesium)
• Neuromas in the feet
• Plantar Fasciitis
• Chronically low Vitamin D and B12 levels (caution urged for those with MTHFR mutations with the latter, may need a different form of Vit B)
• Hyperparathyroidism (lending to high serum calcium levels and calcification in tissues)
• Weak or crowded teeth, many need early or partial dentures
• Receding gums
• Urticaria (hives) and Urticaria Pigmentosa (persistant hive-like patches, part of MCAD above)
• Aquagenic urticaria (in particular, from water exposure) and/or pompholyx (itchy water “blisters” on the hands and feet).
• Hair loss early, and even in women, especially those with signs of MCAD or iron imbalances
• Deviated septum (misaligned nasal cartilage)
• Trouble swallowing & choking issues, often neuropathic in origin from CCI, sometimes due to floppy laryngial tissues
• Esophageal spasms (can extend to anywhere along GI tract also)
• Skin tears or rips, trouble suturing, would dehiscence (trouble healing post surgery, especially soft inner tissues)
• Liver problems, including fatty liver and lesions, enlarged liver
• Enlarged gallbladder & spleen, appendicitis (may be MCAD driven)
• Vocal cord dysfunction (often mistaken for refractory asthma)
• Hearing loss from a variety of causes, some bio-mechanical, others neurologic
• Diverticulitis and diverticulosis
• Breast Ptosis (sagging, droopy breasts)
• Crohn’s and colitis
• Leaky gut syndrome
• Retinal detachment and tears (rips)
•  Dry eyes and blepharitis (bacterial eyelid infections), as well as retinal tears and more
• Fistulas
• Hereditary Neuropathy with Liability to Pressure Palsies (HNPP) – hat tip Ky on Twitter for this one in November 2022. (A friend with clEDs was also just diagnosed with it a couple months ago.)
• A perpetual case of dishes – seriously. From having to cook only whole, unprocessed organic foods you tolerate right? (Mine are all consuming anyway…) Last Updated 12/12/22

Oh TWIST!

I could actually go on, but I think that’s plenty! Hopefully this is helping to broaden people’s perspective on just how incredibly wide-ranging the effects of faulty and insufficient collagen can be.

Other things that are “signs” are not listed, as they are not technically comorbidities. (E.g. stretchy skin is a sign, not a complaint, usually, as is also blue sclerae). And, I don’t want to overwhelm anyone!

I will also add that many of the above “comorbidities” are syndromes in and of themselves, and can often explain some of the other listed issues. MCAD alone can lead to many of the issues shown, as can thyroid imbalance.

Given a choice, outside of one of the very few well-trained geneticists and specialists in the world currently I’d try to work with a functional medicine doctor and/or a naturopath (functional or not) or DO (Doctor of Osteopathy) who might be willing to drill down with you to help address some of the more underlying causes of so many disorders holistically rather than trying to solve each individual issue (unless they’re show stopping or critical of course).

And, as overwhelming as this can all be, realize several things:

a) unless you’re one of the rare few with Vascular EDS or a more rare connective tissue disorder, you probably aren’t going to die any quicker than you were before you read this list (even if it feels like it, and it can feel like it sometimes alright).

And b) you need to triage and pick your battles, and address the most disabling and life-altering with your doctor first. (I would pick the top three issues for any given visit, they won’t have time for more, and maybe not even all three at that, alas. Try asking for a longer appointment whenever possible.)

And lastly c) be patient with your doctor – they can be just as overwhelmed as you are with so many issues to treat. They cannot possibly treat them all at once, nor should they. Point them here for some good resources to help them get up to speed and find answers to all their questions too.

I haven’t even treated half of mine – it’s not worth it. I’m focusing on my nutritional health and sleep, both of which have reduced my pain levels and joint instability greatly and improved several of the other issues. Work with your doctor to determine your best plan of attack, and lastly: do not worry! THAT will not help anyone.

Many issues can at least be mitigated (reduced/diminished) if not outright solved with a variety of therapies I’ll be getting to elsewhere. I keep telling my friends, my life isn’t over since my onset storm of 2012, it’s just different. (Oh but how, ow!)

Respiratory manifestations in the Ehlers-Danlos syndromes

Review

. 2021 Dec;187(4):533-548.

doi: 10.1002/ajmg.c.31953.

Epub 2021 Nov 22.

Rebecca Bascom 
¹
, Radha Dhingra 
²
, Clair A Francomano 
³

Affiliations

Affiliations

• ¹ Division of Pulmonary, Allergy, and Critical Care, Department of Medicine, Penn State College of Medicine, Milton S. Hershey Medical Center, Hershey, Pennsylvania, USA.
• ² Division of Epidemiology, Department of Public Health Sciences, Penn State College of Medicine, Hershey, Pennsylvania, USA.
• ³ Department of Medical and Molecular Genetics, Indiana University School of Medicine, Indianapolis, Indiana, USA.

• PMID:

  34811894

• DOI:

  10.1002/ajmg.c.31953

Review

Rebecca Bascom et al.

Am J Med Genet C Semin Med Genet.

2021 Dec.

. 2021 Dec;187(4):533-548.

doi: 10.1002/ajmg. c.31953.

Epub 2021 Nov 22.

Authors

Rebecca Bascom 
¹
, Radha Dhingra 
²
, Clair A Francomano 
³

Affiliations

• ¹ Division of Pulmonary, Allergy, and Critical Care, Department of Medicine, Penn State College of Medicine, Milton S. Hershey Medical Center, Hershey, Pennsylvania, USA.
• ² Division of Epidemiology, Department of Public Health Sciences, Penn State College of Medicine, Hershey, Pennsylvania, USA.
• ³ Department of Medical and Molecular Genetics, Indiana University School of Medicine, Indianapolis, Indiana, USA.

• PMID:

  34811894

• DOI:

  10.1002/ajmg.c.31953

Abstract

Persons with the Ehlers-Danlos syndromes (EDS) report a wide range of respiratory symptoms, most commonly shortness of breath, exercise limitation, and cough. Also reported are noisy breathing attributed to asthma, difficulty with deep inhalation, and inspiratory thoracic pain. The literature consists of case reports and small cross-sectional and cohort studies. One case-control study estimated twofold to threefold greater respiratory disease burden among persons with EDS as compared to controls. The differential diagnosis for symptoms is broad. Structural alterations include pectus deformities, scoliosis, recurrent rib subluxations, and tracheobronchomalacia, associated with varying degrees of physiologic impairment. Those with vascular EDS have an increased risk of pneumothorax, intrapulmonary bleeding, cysts, and nonmalignant fibrous nodules. Functional aerodigestive manifestations such as inducible laryngeal obstruction may be misdiagnosed as asthma, with gastro-esophageal dysmotility and reflux as common contributing factors. Inflammatory manifestations include costochondritis, bronchiectasis, and localized respiratory allergic and nonallergic mast cell activation. Cranio-cervical instability can dysregulate respiratory control pathways. There is a need for careful phenotyping using standardized clinical tools and patient-reported outcomes and continuing collaboration with aerodigestive specialists including otolaryngologists and gastroenterologists. Also needed is further evaluation of respiratory symptoms in persons with hypermobility spectrum disorders. Personalized monitoring strategies are invaluable for interpretation and long-term management of respiratory symptoms.

Keywords:

Ehlers-Danlos syndromes; hypermobility spectrum disorders; pulmonary function; respiratory.

© 2021 Wiley Periodicals LLC.

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2. 1. American Thoracic Society/European Respiratory Society. (2002). ATS/ERS statement on respiratory muscle testing. American Journal of Respiratory and Critical Care Medicine, 166(4), 518-624. https://doi.org/10.1164/rccm.166.4.518

3. 1. André, R., Duhamel, G., Vergoz, D., & Lavallée, R. (1965). Incidences hémorragiques et viscérales de la maladie d’Ehlers-Danlos [Visceral and hemorrhagic consequences of Ehlers-Danlos disease]. Bulletins et memoires de la Societe medicale des hopitaux de Paris, 116(10), 971-976.

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5. 1. Ayres, J., Rees, J., & Cochrane, G. M. (1981). Haemoptysis and non-organic upper airways obstruction in a patient with previously undiagnosed Ehlers-Danlos syndrome. British Journal of Diseases of the Chest, 75(3), 309-310. https://doi.org/10.1016/0007-0971(81)90011-5

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Heart attack or heartburn? When are high blood pressure and chest pain dangerous | Health

High blood pressure and chest pain is not always a reason to call an ambulance. About when to call a doctor, and when to sit out, what condition is really life-threatening, he told0296 c.m.s. cardiologist Alexander Manoilov.

Numbers are no reason

Marina Karelina, AiF-Chelyabinsk: Alexander Egorovich, you have worked as an ambulance doctor for more than ten years. You think people exaggerate the dangers of high blood pressure. Are reinsured?

Alexander Manoilov: Yes. An ambulance is often called for minor reasons, because they misinterpret and exaggerate the danger of the tonometer readings. We have 10 times more ambulance calls for “high blood pressure” than in developed countries. In the US, calls for pressure are 0.6% of all calls to an ambulance, in Europe – 1.5%. And in Russia – 5-6%.

– Maybe we have more hypertensive patients?

– No. It’s just that our patients have not learned to live with their chronic disease. You see, the numbers are not the primary reason. High numbers require adverse symptoms – shortness of breath, severe headache, vomiting, nausea, chest pain. When these symptoms join, you need to call an ambulance! But most of the challenges are when the patient is simply concerned about high performance. He has 140/100 – he sincerely believes that this poses a momentary threat. Two-thirds of the calls – the car goes to the pressure of 160/100. Unfortunately, this tactic has long been supported by the leaders of Russian cardiology, who compare the increase in pressure with an attack of angina pectoris.

– But an attack of angina really poses a threat, it can be the beginning of a myocardial infarction and end in death…

– Yes. Patients have been taught to think that the pressure has risen, which means that a catastrophe will happen in the brain and heart now …

– If you don’t need to call an ambulance, then what should you do?

– We have compiled a leaflet for such patients, you need to read it carefully. The trouble is that doctors often do not teach the patient to live with his chronic disease. They did not explain what pressure fluctuations are acceptable. No warning about antihypertensive drug withdrawal syndrome. The patient drinks the medicine, sees that the pressure has become “ideal” (someone says “my working pressure”) and quits. Makes a pass. After two or three days, the pressure does not just increase, it rises “with a jump”, up to 200. Another reason is that some doctors allow patients to “play with doses”: “If this is the pressure, then take half a tablet, if more, take a whole one.” This also leads to pressure surges! The selection of a constant daily dose is necessary.

– So pressures of 140/100 and 160/100 are not life threatening?

– Dangerous. High blood pressure of 140/90 in twenty years is likely to lead to disaster. But within an hour – no, it won’t. The concept of immediate danger and remote danger is confused in the minds of patients.

– What is a justified reason for calling?

– Increased pressure, which is accompanied by symptoms: shortness of breath, chest pain, severe headache with nausea, vomiting, epistaxis. Pressure under 200 and shortness of breath can be a sign of myocardial infarction, pulmonary edema. Pressure under 200 and chest pain that is not relieved by nitroglycerin are dangerous! 200 and an incredible headache with nausea, dizziness. 200 and nosebleeds that won’t stop.

Hypertension and addiction

– Is it true that hypertensive patients who take one drug for a long time become addictive over time and one day the drug stops working, the pressure rises?

– This is a delusion. Yes, if a person’s hypertension was poorly treated, diagnosed late, then the vessels become poorly sensitive to the relaxing effect of antihypertensive drugs. In addition, the body is used to high pressure. And then it turns on the mechanisms of fluid and sodium retention. There is a decrease in the effectiveness of the drug, due to the compensatory retention of sodium, fluid. And not “addictive”, as patients say. And do not ask for new drugs. There is no such hypertension that could not be normalized. I have been taking the same group of drugs for pressure for 20 years.

– There must have been a lot of calls for panic attacks?

– Enough. I believe that if the patient has already been diagnosed with panic attacks, then we, the doctors, can stop the attack by simply talking to him on the phone. A trained doctor can do this. You need to ask the caller to “turn on your head.” We ask him: “Tell me, do the observed symptoms threaten your life and your health? Have you already had it? And it didn’t hurt, did it?” You need to call for distracting actions: “Take a shower, wash your hands, go out into the yard, take a walk, call your loved ones.” The attack lasts no more than half an hour. Watch your breath: here is the inhale, and here is the exhale. The statistics are as follows: 20% of the population suffers a panic attack at least once in their life.

– Let’s talk about chest pain. It is not necessary to call an ambulance for any pain…

– Angina pectoris is diffuse, voluminous discomfort behind the sternum that occurs when walking, during emotional stress. Passes at rest and after taking nitroglycerin in minutes. A heart attack is similar to angina pectoris, but begins without exertion. Usually a person wakes up in the morning – and feels a diffuse sensation of pain behind the sternum. Nitroglycerin accepted – does not work. If the pain is such and it is not removed, as usual, with nitroglycerin, an ambulance is needed! But in most cases, chest pain is not related to heart disease. Very often we diagnose a musculoskeletal pathology – costochondritis (inflammation of cartilage, joints with which the ribs are attached to the sternum. – Ed.). But they call us for “chest pain.” And also “pain behind the sternum” is a common anxiety syndrome, which during a panic attack causes a feeling of discomfort in the heart. Sometimes we come for “chest pain” and it turns out to be… heartburn. Once the patient was diagnosed with reflux disease of the esophagus, he took omeprazole for a long time. After dinner, he lay down, and he developed a burning sensation behind his sternum. So, most likely, this is a reflux of bile, and not a heart attack, as you think. Sit down, drink water and wait half an hour. You can definitely do without an ambulance. Frequent users of ambulance services are marked in the card index by dispatchers. There are people who call her 20-30 times a month. And often for little reason.

Note

In hypertension, the body develops a constant deficiency of substances that lower blood pressure. Therapy for hypertension is lifelong. Treatment cannot be a course or only when the pressure rises above the norm, it is continuous. It is important not only to reduce, but also to maintain pressure with medicines at the numbers recommended by the doctor. Do not suddenly cancel any drug, as this leads to an abrupt increase in pressure and can be dangerous to health. With continuous intake of selected doses, you will not need short-acting antihypertensive drugs such as captopril, nifedipine, moxonidine.

A break in treatment for more than two or three days causes a sharp increase in pressure. If after a break in taking the drugs you find an increase in pressure, resume the urgently prescribed treatment in the same volume. But remember: the return to the initial level of pressure occurs within a few days.

If the drugs you are taking gradually begin to lose their effectiveness and the pressure rises steadily again, only a doctor can help you solve this problem.

Taking painkillers, cold medicines, hormones, supplements, some herbs, high amounts of salt and alcohol for several days can lead to high blood pressure.

Self-measurement of pressure at home is useful if you do not have daily fear of waiting for the next rise or fall of pressure. It should be remembered that normally during the day at rest, systolic pressure changes by 10-20, diastolic by 5-10 units.