POTS connection

My 17 year old son who has been diagnosed with POTS about 4 years ago, was just told he also has Joint Hpermobility ( Ehlers-Sanos #3). He was told the Ehlers is whats causing all his body pain. I am just trying to figure things out. Is there a genetic connection between the two? I really dont know how to help him as medicines and  physical therapy have done nothing! Any suggestions would be so helpful . Thanks.

[Heiferly]

While JHS/EDS can be genetic (although there are spontaneous forms of it, so some people have it with no family history of related disorders whatsoever), the reason that there is an association between JHS/EDS and POTS is actually ... well, mechanical.  As I'm guessing you've come to understand, JHS/EDS causes excess "stretchiness" not just on a gross scale—in the joints, etc.—but on a fine scale even down to tissues within the body such as blood vessel walls in some individuals.  If the blood vessels are too stretchy, blood is easily able to pool in the dependent limbs (the legs and feet when standing upright) as well as the abdominal bed (which you will sometimes seen called the "splanchnic bed" in technical medical literature), which is a dense web of blood vessels in the abdomen.  The more the blood pools, the less blood returns to the heart and is able to be cycled to the vital organs, especially the brain, lungs, and the heart's muscle itself.

While the *reason* for POTS symptoms may be slightly different in other patients with POTS than in patients with JHS/EDS (which is to say, not all patients with POTS have overly stretchy vessels—this is just one of many things that can cause this syndrome), the outcome is the same as it is for other POTS patients.  Thus this "functional" thoracic hypovolemia when upright causes the heart to try to compensate for it by beating faster to pull the blood back up to itself (causing the well known tachycardia of POTS).  If the tachycardia is insufficient compensation, there won't be enough oxygenation to the brain and symptoms such as dizziness, nausea, lightheadedness, tunnel vision, and headaches may result.  Ultimately, if the compensation is not enough, the person might faint.  


So that's the connection between JHS/EDS and POTS basically.  There are a LOT of different medications that can possibly work to treat POTS.  Which ones have you already tried which haven't worked?  (And in which combinations with each other?)  What other "helps" have been tried (such as abdominal binder, compression stockings, etc.)?  What type of physical therapy was tried (aquatic tx, cardiac rehab, weight training, pilates, aerobics, etc)?  

Here's our health page on treatments.  This includes treatments for various types of dysautonomia, so not all would be appropriate for POTS necessarily.  But it may give you an idea of what's out there as a starting point:

http://www.medhelp.org/health_pages/Neurological-Disorders/Dysautonomia-Treatments/show/171?cid=196

Best,
Heiferly.

[CHRISTYDRAKE]

My daughters physical therapist has told us she has hypermobility.  While my son has the POTS diagnosis, she has symptoms as well, just not as bad as him.  I am reading a book on hypermobility and so many things ring true for my daughter.  It runs in the family, now that we know what it is (my mom, my sister and 2 of her kids).

My question is, what type of doctor do you go to to get checked for EDS?  and is there anything they can do for it besides the PT she is already doing?

Christy

[Heiferly]

You may want to ask your family doctor for a referral.  I think most likely they would refer you to the rheumatology or genetics department.  The available treatments depend upon which subtype of EDS it is, and likewise, what symptoms are being suffered.  Depending on that, there are treatments that range from cardiology to occupational therapy to fitting with special orthotics; it really depends on the individual case.

It is important for you to mention that you have identified multiple family members because having this information can sometimes aid in better genetic testing and identification of which form of EDS is affecting your family.

Best,
Heiferly.