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Ehler's Danlos Syndrome & Hypermobility

Ehler's Danlos Syndrome (EDS for short) is a disorder that is inherited and passed through genes in families - mainly affecting connective tissues such as skin, blood vessel walls, and joints. Connective tissue is made up of a mix of proteins (collagen is one of these proteins) and other chemicals that provide the strength and the elastic nature of the skin, blood vessels, and joints. There are several types of EDS ranging from a hypermobility type to a rare life threatening form of vascular EDS that affects the heart. Visit the Ehler's Danlos National Foundation site as a great resource for indepth reading about EDS types, diagnosis, doctors, and more.

Maybe you can guess where I am going with this and why I am writing about EDS when Meghan has POTS. One of the things that EDS affects is blood vessel walls. People with POTS already have trouble with receptors receiving messages that tell the blood vessels when and how hard to constrict - so it is thought that EDS can be an underlying condition that predisposes someone to POTS. (Of course not all people with EDS have POTS and not all people with POTS have EDS). It is thought the lack of collagen in the blood vessel walls increases the difficulty for blood vessels to squeeze hard enough for pumping blood, and the vessels are more relaxed and loose which leads to blood pooling in the lower extremities.

For Meghan, she has the hypermobility type of EDS - which means her joints are looser than they should be, due to less collagen, and she can be at a higher risk for a hyper-extension injury. She was diagnosed by a rheumatologist using a beighton score. The doctor gives one point for each area of hypermobility. Meghan's elbows and knees both go slightly past where they should - same with fingers and wrists. I actually did not notice until the doctor pointed it out. Now it seems obvious. Sometimes when she stands, her feet can be pointing sideways in opposite directions. When she was younger, she used to have trouble with her ankles and I would notice she would stand with her feet pointing outwards. Mostly, though, it went unnoticed. She would often drop things, and it is thought that is because the connective tissue in her fingers wasn't as strong for gripping.

There are some overlapping symptoms between POTS and EDS - such as gastric problems, joint pain, and fatigue. People with EDS might also notice easy bruising and/or skin sensitivities. Meghan has skin that is dry (the chlorine from swimming does not help this) and a sensitivity to hand lotions and anti-bacterial gels and washes that are popular. Her hands turn bright red and feel like they are burning. She is also always in need of chapstick.

EDS can have a wide range of effects on a patient - Meghan's symptoms are mild in comparison to many. We saw a physical therapist briefly to see if that would help with joint pain. It was expensive and insurance covered only 6 visits - although with each doctor, nurse, or therapist, we have always learned something new, even with the not-so-good ones. Our therapist introduced us to some occupational therapy tricks that are helpful to those with over-active nerves. Things like a weighted blanket can be relaxing when you can't relax overall. Meghan had trouble with her shoulders and a weighted shoulder bean bag helped her to become more conscious of holding her shoulders tense and the need to relax them. Being aware is important.

She needed to know to be careful with her knees, ankles, elbows etc. She will also have to be careful of repetitive motions - like with swimming. She has had some pain in her shoulders while swimming breaststroke. Less collagen in her tendons and connective tissue can lead to break down of these areas.

Some patients have trouble with dislocations - even when doing simple, everyday tasks. Often times symptoms of EDS do not become prominent until adolescence.

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