May is Ehlers-Danlos Syndrome Awareness Month

How AWARE are you? Take this quiz to find out:

1. How many types of Ehlers-Danlos Syndrome are there?

a) 1

b) 4

c) 14

d) 32

2. The Ehlers-Danlos Syndromes affect which body system?

a) skin

b) heart

c) digestive

d) neurological

E) all of the above

3. What do people with Ehlers-Danlos Syndrome look like?

a) pale, tall, and skinny

b) short with a curvy spine

c) covered in scars and bruises

d) sagging skin

e) any, all or none of the above

4. When in the lifespan does Ehlers-Danlos Syndrome onset?

a) birth

b) puberty

c) after trauma or illness

d) parenthood

5. Is Ehlers-Danlos Syndrome life threatening?

a) yes

b) no

c) no, but it can significantly impact quality of life

d) it depends

6. How do people with Ehlers-Danlos Syndrome get around?

a) wheelchair

b) walker/cane

c) braces

d) sensible shoes

e) like a high-class athlete or performer

7. How is Ehlers-Danlos Syndrome treated?

a) vitamins and minerals, nutrition, hydration

b) carefully selected exercise, lifestyle modification, counseling

c) adaptive equipment and supports

d) prescription medications

e) none, it is incurable

8. Why are people with Ehlers-Danlos Syndrome called Zebras?

a) zebra stripes represent the abnormal appearance of the skin in EDS

b) doctors are taught to look for the most common diagnoses instead of being distracted by the possibility of rare disorders like EDS by being told ‘When you hear hoofbeats, look for horses, not zebras!’

c) living with EDS is like being a jungle animal

d) the debate of ‘white with black stripes’ vs ‘black with white stripes’ represents the aspects of the disorder that remain poorly understood

9. What is the easiest way to spot a zebra?

a) look for stripy skin

b) look for a history of doing the splits or entertaining contortions

c) look for joints that seem to bend the wrong way

d) test their Beighton score

10. What do people with EDS need in 2020?

a) to be seen, heard and believed when they relate challenges

b) more public awareness and acceptance of the disorder

c) more healthcare providers who are educated, aware, or simply willing to read about EDS

d) more research into diagnosis, management of EDS

ANSWER KEY (yes, there were a lot of ‘trick questions’)

1. C: there are 14 types of EDS, each named for which body system is most impacted. All the types involve some degree of joint hypermobility. Only the most common types (hEDS and HSD do not yet have identifiable genetic markers that can be tested to confirm diagnosis)

2. E: all types of EDS impact connective tissue, which is part of the composition of every system of the body! Some types impact connective tissue of one system more than others, while the most common types (hEDS, HSD) impact the joints of the body the most and are also associated with GI, neurological, cardiovascular and immune disorders

3. E: Ehlers-Danlos Syndrome is called an ‘invisible illness’ for a reason. Most people with EDS look ‘fine’ or just like anyone else. Some rare types do cause spinal deformities or obviously different appearing skin. In some people, difficulty with chewing/swallowing/digestion/muscle building can cause a gaunt appearance; other people have activity limitations, metabolic changes, or changes in tissue deposition that lead to increased adiposity.

4. A, and B-E too: EDS is genetic, so it is present at birth. In some of the very rare types, it may be diagnosed shortly after birth related to joint dislocations, unexpected bleeding, or failure to develop movement as expected. But most types remain undiagnosed until symptoms begin to significantly interfere with life AND the symptoms are presented to a health care provider knowledgeable about EDS. Puberty, trauma, pregnancy/parenthood can all be a trigger that leads to greater awareness of symptoms. However, many people with EDS wait 5-50 years from the awareness of symptoms to diagnosis due to a lack of knowledgeable providers.

5. D: vEDS, or vascular EDS, and some other rare types of EDS can be life-threatening disorders, especially when undiagnosed; with timely diagnosis precautions and management can allow longer life. Most types of EDS, however, impact quality of life much more than longevity.

6. A-E: mobility varies dramatically among people with EDS. In fact, many individuals can use all these modes of getting around in the same lifetime, and sometimes even the same month. While many disorders are progressive, the progression of EDS is very unpredictable. Many people with the most common types, hEDS/HSD, have phases of very high function, very low function and everything in between despite conscientious management of their disorder. Fun fact: most people diagnosed with hEDS/HSD relate a history of exceptional talent in athletics, music, or the arts. Often, difficulty utilizing these talents are what drives individuals to seek a diagnosis.

7. A-E (again): There is no cure for any types of EDS. All of the listed options can be beneficial in optimizing function and quality of life in EDS. Most treatments are focused on minimizing associated conditions in the various body systems. At various phases of the disorder, medications and adaptive equipment are especially helpful. The need for lifestyle management, right-size exercise and movement, nutrition, hydration, and care for mental health are never ending for those with EDS. Even at the very best phases, the individual is always working to manage life with unique connective tissue.

8. B: This is good advice for young doctors most of the time, but it does a disservice to those who are zebras. Many ‘zebras’ are accused of lying, faking, seeking some sort of gain, or simply dismissed when they don’t respond to treatment as expected or when diagnostic testing is inconclusive. While it’s smart to look for the most common cause of a complaint first, it would be better for all of us if the next step was to look for more rare causes of a complaint like EDS before moving to accusations or dismissiveness.

9. Not A! Probably B & C. Definitely D: The Beighton Scoring System is the most widely accepted way to assess joint hypermobility and is quite easy to check on your own and then show your healthcare provider. This link explains it all: https://www.ehlers-danlos.com/assessing-joint-hypermobility/ CAUTION: moving your body parts to extreme/awkward positions routinely can damage supportive structures, so avoid the temptation to ‘show off’ or do ‘party tricks’ just because you can; taking photos and showing them from your phone is a great way to achieve validation without abusing your body!

10. A, B, C, D: Yes, yes, yes and more yes!