Responses to Patient with Ehlers–Danlos syndrome

Responses to Patient with Ehlers–Danlos syndrome

Jennifer Reese

2/27/2013

Thanks to all who have replied. Here are the responses I have received.
Original Post:


I saw a patient today with Ehlers-Danlos syndrome. She is 13 years old and was recently diagnosed. Her major concerns at the moment are her fingers keep dislocating at the MP joint and one hip keeps dislocating when walking. I have considered something like TheraTogs for the hip but not sure if that will provide enough support. Another option is fitting her with a hip abduction brace. If anyone has any advice for this, it would be very helpful.




Hi there

You might want to look at the DMO website. They don't have a hip brace as far as I know but may be interested in creating one?



<URL Redacted>




I have had good success with compression suits! It acts as an external ligament system. Custom fit, provides functional motion but blocks end range and prevents or retards functional joint laxity.






Of this patient population I have only seen two, but both with hip issues, and one with finger problems. Whether Theratogs will work will depend on the mechanism of dislocation, but I needed more support with both of mine. A standard hip abduction orthosis is a beast for anyone, even when they are effective.

For both of mine I ended up making custom devices, because even though the incidence of dislocation was reduced with an OTS HOs, they were not eliminated, primarily I believe because of inadequate contact from the OTS HOs. For one of these we ended up making a one-piece device out of Northvane silicone-modified polyethylene (Optiflex, Duraflex, etc.). Imagine a TES belt but with selectively extended trim lines and made of a single piece of the plastic with a simple front Velcro closure. This provided enough flexibility to sit and walk but enough greater contact that it prevented virtually all dislocations during walking and transfers. With the other we made an custom HO, because the contours of the OTS HOs did not match her very well, and the moving around of the HO may have actually increased her dislocations. Again, modified trim lines, particularly in the pelvic section and lateral thigh, and we used a relatively small pediatric hip joint (she was not large) to reduce the profile a bit.

The fingers were tough, because if you supported them enough to avoid dislocation, function was very difficult. However, my patient also dislocated more distally than just the MP joints, so you would not have to extend the support as far. The best thing we found (and it was not great) was a modified Juzo compression glove with small spring steel stays.

I would assume that the MP dislocation was during flexion, and if so, extending an HFO palmarly to just proximal to the PIP joints, with a volar cap, may keep things in place adequately while still permitting some use of the fingers. This could be of a thin PE for maximal comfort, and I assume that this patient isn't doing anything with her UEs that is high impact.








I think a Dynamic Movement Orthosis from Boston Brace might work well. You can fit her with gloves and a suit. I have had great results with them.






You might want to look into the boston brace DMOs (dynamic movement

orthoses) These are suites that have tension bands to encourage or discourage certain muscle groups.








If she is not too big a wet suit with synergistic strapping sewed on the outside will provide good hip stabilization.






I like the Theratogs idea as a first try on the hip- if it works you will have a much better chance of compliance and she will be more comfortable than she would in a HO. I recently fitted an adult ED patient with custom rings from the Silver Ring Splints company. They seem to work and they look good too...may have to fight with insurance a bit but we were able to get them paid for. Good luck!



hello,
what you can try is from the firm bauerfeind a sacroloc its normaly used for pelvic problems , but it might just be tight enough to keep the tendons under pressure to keep the hip in place , the same firm also has another brace used for dislocating hips thats called softec-coxa . i hope this onfo is off anny use for you.




Ehlers-Danlos. cool. But then I did have a weird disease magnet.

13 y/o female recently diagnosed. oh joy.

We'll start with the hand. Both hands? Right? Left? And which fingers specifically? A well fitting glove might provide sufficient support using compression, and as I expect she is in school, and the PIP and DIP are not affected, then fingerless gloves could do the trick and still allow her fingertips free to write or type. And IF, and this is the biggie, if her insurance will authorise it, Jobst customs may provide her enough support. Although, getting any insuror to cover those might be an up hill struggle.

As for the hip(s). how specifically is it dislocating? Abduction, Adduction, flexion/extension? And what is her weight range? Theratogs, provided she is petite, could be sufficient, but then you get back into coding and coverage issues.

And here's the big one. What is the positioning of her talo-crural joints? Remember that the foot-ankle complex is the foundation to the structure that is the human machine, and if the foundation is not stable, the structure above it will never be stable. If she has any valgus issues distally, then that will lead to abduction stresses at the hip, and as Ehlers-Danlos is a connective tissue disorder, any abnormal stresses from ground reaction will exacerbate the situation. If on the other hand she has varus issues, then you are in a world of hurt, as the body can compensate for valgus but cannot compensate for varus. Addressing that may be sufficient to address the hip issues, but you haven't yet provided quite enough info for me to help further.






I recently had a 19 y.o.f. patient with this dx. Her biggest problem was her ankle dislocating. I designed an Arizona type AFO and she seemed to do better. I used wrist gauntlets for her dislocating wrists. Fingers were not addressed.


I saw your email on the list serve.
Our SDO, Sensory Dynamic Orthosis, has been used in Ehlers-Danlos syndrome. I have attached a link to an introduction to the product. Feel free to contact me should you find it interesting enough.

<URL Redacted>


I received a call discussing the use of the SWASH orthosis


Try SI belt, take a good hard look at single leg stance if she is falling into pronation will effect kinetic chain all the way up pair of custom foot orthtoics or Sure step SMO's may be useful.




I have not tried this but consider the Knit-Rite prosthetic suspension aid:

bilateral power belt. Their X-small fits 21-28 waist and 12 -15 leg. I beleive they may be stronger than the TheraTogs. A custom, simular to the pwr belt, could be fashioned from neoprene.





Hi Jennifer,
Is one hand worse or are they both about the same? I would first check out Benik and order gloves with open fingers. The glove should not impede her function and leaves the distal fingers free and open. The hip may require more than she is willing to wear unless it is dislocating so frequently that she is unable to walk. If it's that bad then a good abduction orthosis may be needed. Orthomerica has a pediatric version. Good luck with that.



Jennifer Reese, CO
Hanger Clinic
Lawrenceville, GA
770-963-7046



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