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Heads Up, Written Down – Episode 21: Hypermobile Ehlers-Danlos Syndrome

Heads Up, Written Down

Episode 21: Hypermobile Ehlers-Danlos Syndrome

Hypermobile Ehlers-Danlos Syndrome (EDS) is a disorder of collagen. It affects the connective tissues that make up the tendons and ligaments causing them to be more elastic. Hypermobility and hypermobile EDS are not one in the same. There is a spectrum of hypermobility, and it is a disorder that can occur on its own in approximately 8-10% of women and 1-5% of men. Hypermobility alone is not indicative of EDS.

Hypermobile EDS only occurs in about one in every 5,000 people. In order to make a diagnosis of hypermobile EDS, hypermobility has to occur along with a variety of other things like recurrent joint dislocations, hernias, or other findings on exam that a healthcare provider may determine are indicative of EDS. Only a healthcare provider familiar with the syndrome can make a diagnosis of EDS.

In order to meet the diagnosis of hypermobility, you have to meet something called the Beighton criteria. This is a joint mobility scoring system on a nine point scale. It assesses the knuckle of the pinky finger, the base of the thumb, the elbow, the knee, and the spine. The biggest symptoms that occur in this disorder are joint pain and joint dislocation. Individuals with this disorder will often present with neck pain, back pain, or pain all throughout the body. This is likely due to the fact that the ligaments surrounding the joint are lax.

There are other disorders that often occur along with hypermobile EDS. Some of these include postural orthostatic tachycardia syndrome, or POTS, which causes the heart to race when a person stands up. Some people also have mast cell disease which is an immune system disorder that presents with flushing, allergic reactions, anaphylaxis, low blood pressure, gastrointestinal, and respiratory symptoms.

People with hypermobile EDS are more likely to have migraine, and their migraine disease is more frequent and disabling. They are also very likely to present with migraine with aura. It is possible that the presence of dysautonomia or abnormal regulation of the autonomic nervous system (often thought of as the fight or flight response) could contribute to this predisposition to migraine. Other types of headache disorders are also found in EDS. These include new daily persistent headache or NDPH, a condition where a headache appears suddenly and is continuous after that time. This group also experiences headaches attributed to hypermobility of the neck (cervicogenic headache) and medication overuse.

Orthostatic headaches can also occur in this population due to spinal leaks. The sack around the spinal cord is made of connective tissue and is fluid filled – people with EDS can develop leaks or tears in the sack leading to orthostatic headaches which are worse when you stand up and better when you lie down. They can also get high-pressure headaches as well. These are worse when you lean forward, lay down, cough or sneeze, etc. (See Heads Up Episodes on Low and High-Pressure Headaches).

If anyone feels they should be evaluated to see if they fit the diagnostic criteria for hypermobile EDS, it is important to find a healthcare provider who is well versed in diagnosing the disorder. According to Dr. Martin, EDS is “en vogue” right now, and doctors are becoming increasingly savvy about this diagnosis. Geneticists and rheumatologists are two specialists that may diagnose EDS most often. If a person is diagnosed with EDS, it may

take “a whole village” of different healthcare providers to participate in their care because often there are a number of different disorders occurring in these patients at once.

In people who have EDS, even headache may need to be managed differently. If Botox therapy is used, certain spots in the neck may be avoided so that neck hyperextension and positioning problems are not increased. There are medications that may need to be avoided like muscle relaxants and those that might cause weight gain and increase the chance of joint problems and dislocation.

Dr. Martin wants people to know that if they do have EDS it is not as bleak as it may sound. “If you get the right treatment you can see positive results”. If you find the right therapy, most of the symptoms associated with EDS can be treated and

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