"Suffering the Slings and Arrows of Outrageous Fortune"

In part one, I talked about our genetics appointment and Dr. Neilson’s recommendations for Em. While I am sure that is riveting reading, part two actually affects everyone. It should be of interest to anyone who has EDS and certainly to anyone who might end up at Cincinnati Children’s to be treated.

On Friday, there was a med student who sat in on our visit. We are almost always up for educating then next generation of doctors, so we were happy to be guinea pigs, as long as they are respectful. Because he was teaching the student about EDS, our appointment went further than just chatting about what issues needed addressed.

He asked Em if she minded if he evaluated her hypermobility so the student could learn about the Beighton Scale. (He asked permission, before just jerking her around which is better than her old pain management doctor did! Needless to say, this endeared him to us!) So, he went through the test, talking to the student the whole time – and we got to listen in to his reasoning as he talked to her. Incidentally, Em’s score is 7/9 – her left elbow and pinky didn’t quite meet the requirement to get a point this time. (It bears mentioning that her score has changed over the last 3 years, but maybe that should wait for a separate post…)


But then he made the point that the Beighton scale is imperfect because it is heavily weighted to the hands – so he adds 2 more tests to help him more completely evaluate hypermobility: the reverse prayer sign and seeing how many fingers one can fit in their open mouth. At least 3 fingers up to the first knuckle indicates hypermobility of the jaw. Of course, Em was able to do both pretty effortlessly.

It seemed to me that Dr. N was taking a page out of Dr. Bravo’s play book – he also talked about how he could often tell someone is hypermobile by simply shaking hands. Often, hypermobile hands will just collapse when squeezed even a little bit. Em’s hands aren’t severely hypermobile and don’t really collapse in an extreme way so I guess she doesn’t get a point for that!

I was pleased that he knows enough not to rely only on the beighton scale and is willing to look for other signs of hypermobility. He didn’t really address the concept of multi-directional instability but it is a start.

Furthermore, he also mentioned that, as far as he is concerned, EDS and hypermobility are the same thing. I assume that doesn’t include the sort of harmless (if there really is such a thing long term) run-of-the-mill hypermobility, but rather is referring to the concept of joint hypermobility syndrome being identical to EDS. I mean, there is a little bit of a gray area when you talk about hypermobility, but, clearly, he is in the camp that doesn’t differentiate between EDS and JHS. Bless him!

Also, when the med student mentioned that she knew a bit about EDS because she has a friend with it, he asked Em what her opinion about the rarity of EDS is. When she said that it is more common than most people think, he said he agreed with her and that is what he tells people when discussing it!

When Dr. Tinkle left Cincinnati last year, there was a fair amount of angst – that he had replaced himself with a couple of doctors who he had personally trained was of little comfort, for we patients could not be sure how they would perform until we could evaluate them on our own. The first time we saw Dr. Neilson I wrote that, while he may not be a world-renowned EDS expert like Dr. T, I thought he was well on his way to being more than competent.

Now, maybe it is a given that he should have a firm grasp on the benefits and imperfections of the Beighton scale but we all know there are self styled experts who DON’T have such a grasp. Maybe it is a no-brainer that he recognize that there is no difference between EDS and JHS, but we all know that there are people still being diagnosed with Joint Hypermobility Syndrome, although they clearly have EDS. Yes, we all suspect that EDS is far more common than the statistics indicate, but no one can say for sure and most doctors prefer to side with the evidence rather than going out on a limb with nothing to support a mere gut feeling.

So, while it might be obvious to all of us, Dr. N does not have to share or talk about these opinions. That he does, makes me feel that he really is on our side and has a firm grasp on the facts – as most of us see them! This can only be a good thing for the EDS community. With new doctors such as him on the scene, we are edging ever closer to the type of treatment this long-suffering population deserves,better, earlier diagnoses for the kids, and, hopefully, to the answers that will really help us understand the many facets of this condition.

[On an amusing note that really doesn’t affect anyone else but I will share anyway: When he found out that I am the parent who doesn’t have EDS, Em got to sit in the chair and watch as I hopped up on the exam table to allow the student to see someone who is not hypermobile. She definitely had a subject who is not hypermobile in the least, which was clear when she tried to hyperextend my knees. (I figure any non-hypermobile genes/tendencies that Em got from me might be what is holding her together!) At any rate, Em immensely enjoyed observing me get tested while she sat in a chair – the tables were turned and she loved it.]






Comments on: "Genetics Follow-up Part 2" (2)

  1. That sounds awesome! He listens, asks if it’s ok to use you guys as a teaching tool, understands the JHS/EDS issue, and used more diagnostic criteria than merely the Beighton scale? That’s amazing.

    On a related note, I didn’t realize that the fingers-in-the-mouth could be used as diagnostic criteria. My entire fist will fit in my mouth and has ever since I can remember. I knew about the reverse prayer sign, because I saw it mentioned on a different EDS blog and promptly looked it up and did it, not understanding that it’s meant to be painful and difficult. But I didn’t think about my jaw.

    • It was pretty awesome – I mean, he should have this knowledge, since he replaced one of the premier experts, but we all know that is no guarantee! And whenever we have an opportunity to create awareness in the next generation of doctors, that is a really good thing. I always feel like we have done a good deed afterwards, although it was no effort at all (as long as student and doctor are both respectful!).

      It is funny how things that should be a problem are just totally normal to an EDSer. I think this is part of the problem of getting diagnosed – why bother checking into something that is just normal? When I was self diagnosing Em, I went around comparing her skin to mine, her dads and her brothers. They all tried to tell me I was the weird one because my skin didn’t stretch and theirs did!

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