Article to read on Posterior Shoulder Instability

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3445168/
This article leaves me with some questions about the certainty of only posterior dislocations for kiddo. It sounds so incredibly rare to have true posterior shoulder instability with dislocations (as opposed to sublux), but this is what we have been told she has all based off her first dislocation in the 4th grade, which was as a result of being kicked in the back shoulder at school as a child tried jumping over her. I am very curious to know if she has multi directional instability. I am going to read this more closely and do some research and ask the team.

Other questions this brings up:
1) How do we know that we have ruled out labral tear worsening or additional labral tears/lesions?

2) Does she have unidirectional, bidirectional, or multidirectional instability? And is it strictly posterior? I have definitely heard different doctors use the terms posterior and multidirectional, but I cannot find it written on any paperwork from any of her orthopedic doctors.

3) Have we ruled out bankart lesions?

4) Have we ruled out glenoid retroversion/rim erosion?

5) Have we ruled out reverse Hills-Sachs lesions?

6) Past MRI reports and doctor reports mentioned thickening of SGHL, but no explanation was given as to if that is important to this ongoing problem. Also, dyskinesis was mentioned in print, but no discussion from doctor as to relevance.

Each of these would need to be known in order to know how to proceed to fix the problem and I don't recall discussing any of these with the orthopedic surgeons with the only exception being that she was said to have a torn labrum that should heal on its own without surgery in 2013. No follow up from that Dr on that issue.

This article (Click here) has good definitions of the types of EDS and says the following regarding hypermobility and surgery:  "Shoulder instability in patients with EDS is common.25,29,45 Unlike patients with traumatic instability, patients with hyperlaxity and instability are more likely to experience recurrent subluxation than dislocation.29 The evaluation of shoulder instability has been well described.45 However, because shoulder symptoms in hypermobile patients are not always the result of instability, rotator cuff impingement and other conditions need to be considered.29Nonoperative treatment should be maximized before surgery is indicated.25 When surgery is required, open inferior capsular shift is the gold standard, but the results of arthroscopic procedures may now achieve similar results.29"

I will add articles here, as I find them. If you happen to be reading this, and you have an article to share, please add it in the comments. Thank you.

Disney From a New Point of View

I remember that first trip, when our girls were only 3 and 5. They were full of excitement and energy and everything seemed to fall into place, perfectly. The magic was definitely with us. Once we caught that infectious desire to return to our new found happy place, we took any opportunity we could find to visit again. My oldest daughter was never a night owl, and she was always in bed by 8pm, but it didn't cramp our style. It wasn't until our last trip that we had to make such a change in the way we toured the World. With our oldest child in a wheelchair for the first time, we were forced to look at Walt Disney World from a new point of view, and guess what? It was just as magical.

Our oldest has a connective tissue disorder called Ehlers Danlos Syndrome (EDS). While EDS presents itself differently in different people, for our daughter, once she hit those growth spurt years, it presented as non stop inflammation of joints, ever present level of pain, and the worst symptom...her joints started dislocating daily. Her shoulders are the worst, now, with daily should dislocations. Her knees and ankles used to give her more trouble. What that meant for a day at Walt Disney World was that she couldn’t walk the parks safely, she couldn’t do all the rides, and she didn’t have the stamina that other kids her age may start with. When embarking on such a big and expensive vacation, it is a little difficult to consider that you will have to go slower, see less, and pay the same, but we found that this wasn’t the case at all.

To start with, we rented a small wheelchair in our home town and we utilized that in the airport, which worked great. Once we got to Disney, we were a little nervous that people may give her dirty looks or make rude comments since the average person would never know by looking at her that she is struggling to keep her shoulder connected, or her knee cap from sliding out. People see only a young, vibrant girl and therefor, it is easy to assume that she could be faking to take advantage of any perks of being in a wheelchair. I was ready to explain to anyone who stopped us what EDS was and how it affects her, but you know what? No one did comment. The cast members did not treat her any differently than they treated her sister on two legs. If anything, she got a bit more positive attention. While she did feel self conscious that there were eyes on her from other people, I let her know that curiosity is normal and encouraged her to smile and not worry.

We still started our day in the same early to rise fashion that we always do, and we hit our favorite rides and shows. While the wheelchair may have slowed us down a bit, we were still able to do everything we wanted to do. Cast members are well trained in dealing with someone in my daughter’s situation. She is able to transfer on her own, so she was able to stand up and get into a ride while her chair was moved to the ride exit for her use. The system is so smooth and efficient that we never felt hindered. For shows, there are designated areas for the wheelchairs, which were open for us each and every time.

Break time was longer, with leisurely meals and relaxing time by the pool. We didn’t make it to the evening shows and fireworks, as my daughter was too worn out by the time evening rolled around, but we didn’t let that worry us at all. The way I figure it is that anything we miss during the trip is a good excuse to come back again another day. Also, if there is one thing that repeat trips to Disney World has taught me, it’s that the magic is often found in unplanned moments, so we kept our eyes and hearts open and we had a beautiful trip.

Our first trip to Walt Disney World in a wheelchair was just as wonderful a trip as any from previous years. If I had to weigh it out, I may even say it was better because my daughter was able to do everything that she wanted to do before this disability took hold of her body and began to dictate her life. She works hard in physical therapy, year round, and she wears braces that have given her some additional freedoms, but she is unable to safely attend school due to such easy and frequent shoulder dislocations. Being able to use her safe wheelchair space while touring the parks is the only way such a crowded environment would work for such a fragile child.

Should you ever find yourself ready to embark on a magical adventure with an unexpected injury looking to ruin your fun, don’t give up on the plan. Disney is unparalleled when it comes to accommodating special needs of all shapes and sizes. It truly is a magical place for every visitor.

It has been a couple of years, and I am sad to say that even though my daughters legs are improving, her shoulders are worsening. There is no cure for Ehlers Danlos Syndrome, and we can only hope that age and physical therapy will save her from losing the use of her shoulder joints over time. We continue to see specialists and search for innovations that may help her. In the meantime, we are prepping for our second trip to Walt Disney World from the slightly lower view of a wheelchair, and I can tell you... the view looks great from here.

Posted by All Ears on December 3, 2014 12:29 PM 

Ongoing illness is isolating

In the past 4 years I have learned that a chronic health issue is consuming in ways that can trump connection with friends and family. Friends that I have had for years don't pick up the phone to call to see how things are going in relation to the shoulder instability, doctor appointments, and run arounds. Loved ones don't quite know what to say.  There are times when I am so overwhelmed with fear, worry, conflict, and anxiety and I look around me and see that life goes on. No one seems to notice our little patch of struggle, and if they do, they have enough of their own struggles to draw their attention.

It seems to me that having a chronic health issue is something that is truly untranslatable to those who haven't been closely touched by such issues. Sadly, many people have been affected by a wide variety of chronic health issues, and I wouldn't wish that on any one. My point is just that it's hard to feel like you are sinking, and when you look up, see everyone walk by without offering you a hand. On the flip side, I know there is nothing they could do and that I can just step forward and see if the next step is the one that elevates us out of this pit. I can focus on the fact that we are not sunk, and take strength in the growths, no matter how small. I do know I should not feel sorry for myself, or for my child. Yet, I am realizing that years of disconnection on an issue that is so consuming for our family is a wedge between relations outside this bubble of life we know. I wonder how people manage around that. There must be ways to handle this with grace, compassion, and acceptance.

Illness, on a long ongoing basis, is isolating. We make efforts to stay connected, and some days those efforts cost more than others. I am grateful for modern technology, which offers me the ability to easily access other individuals who have pulled themselves from the same pit, or who are in it beside us. Their voices, stories, and companionship are a beacon and a compliment to the friendships and love from family that have helped to build us into the individuals we are today. I have found good connections with groups through facebook, but I imagine there are many others, as well. If you have a favorite EDS support network, please share it here and I will be happy to check into it.

Another shoulder specialist to research

Adding

• Dr Guido Marra at Northwestern/Loyola https://fsmweb.northwestern.edu/faculty/facultyprofile.cfm?xid=25884 and 
• Dr Peter Millett in CO http://drmillett.com/ and 
• Dr Mike Yergler http://www.sbortho.com/bios/Yergler_M.html in Indiana 
• Dr David Roberts IL Northshore pediatrics http://www.northshore.org/apps/findadoctor/physicians/david-william-roberts
• Dr Bassem Elhassan at Mayo Clinic http://www.mayoclinic.org/biographies/elhassan-bassem-t-m-d/bio-20055031

to my list to research for shoulder specialists.

POTS - and we are welcomed to another Ehlers Danlos Syndrome partner in crime

For me to research later : http://www.dysautonomiainternational.org/page.php?ID=30
For my own experience, I had symptoms of POTS in my growing teen years and into my early 20s and now it is completely gone (well, pretty much - so long as I eat and drink properly). So, my hope is that for my daughter, this will be something she outgrows. Also, I hope that fainting is something she can avoid by recognizing how to treat POTS as well as how to more safely and how to recognize signs.
More research to do, but we'll take any help we can get. We don't want our fragile girl dropping to the ground due to this. She had a black out session yesterday, but it was very short, and she was sitting. First steps, we'll be increasing fluids, electrolytes, and doing some additional reading and research!
Again, very hopeful she has a mild version of this to deal with, as her mom and aunts did.

Homeschooling and Bracing - is it helping?

You know what. It IS helping! Kiddo still battles with shoulder instability, but by staying in a dual enroll program where she only does 1 class at school, and the rest at home, her dislocations have cut back dramatically. Previously, she was not able to get through more than 2.5 hours of a school day and she would begin repeat, painful dislocations. The tearing feeling inside the joint was getting worse as these dislocations increased and reducing was getting more difficult.  Now she has some days where she is not dislocating at all, and others where she does dislocate, but only 1-3 times instead of many.

The brace seems to have helped reduce the number of dislocations and the pain in dislocations has gone down since the brace has been added. So, she can't attend a school day, but there is some improvement. She is still in physical therapy and now the focus is on core stability and positioning to help support the shoulders in all movements. This will require a lot of relearning basics and I don't foresee it being easy for a 14 year old to pick up on, but small steps and improvements.

Still, even with some areas of increased improvement, the limitations are long. We don't know what to do next. The genetic specialist advises surgery and he has referred us to surgeons.  We have been to see orthopedic surgeons at IBJI and at Rush and they do not feel that Kiddo is a candidate for surgery. They tell us the surgeries won't hold, won't work. So genetic specialist says that we need to keep looking and consider Cincinnati. Others report Steadman Clinic and Boston. Success rates are so low, and our daughter is still growing, so we are very torn about what to do next. At the moment, since we do see some improvement in our current plan, we will continue this path in hopes it propagates more healing and strength building in the upcoming months. Perhaps, if we can get her through these growing years before surgery, her odds of successful surgery will be higher? We will plan to talk to more specialists in the upcoming year, as well.

To research : Reconstruction in Chronic Shoulder instability

Open Reconstruction of the Anterior Glenohumeral Capsulolabral Structures with Tendon Allograft in Chronic Shoulder instability article link, here. Coming back to read this, per suggestion of another woman who found a measure of success with this process. There has to be hope! Please comment other experiences that you've had that have been helpful.

A Mom Epihany : Why so resistant to home schooling my child wth Ehlers Danlos Syndrome?

I think I just figured out why we have been resistant to homeschooling. We have/had a vision of what our daughters life would be like and by pulling her out of school, it is announcing that her disability is debilitating. That she cannot just "get better" and that we have to plot a new course. These are not bad things, in and of themselves, but I think we have been holding hope that she would just be better one morning, and her shoulders would be fine. In a way, homeschooling has been a flag that we have lost that hope. I know that is not the way to look at it, and I understand. It was just a bit of an epiphany this morning.

I feel like it's the right course and I am ready. I have to talk to my husband more, and then my child, and then the school. We may ease into it. We may wait a bit longer. We'll see how it goes, but if my husband seems resistant, I can understand that it isn't homeschooling he is shying away from, but the idea that his eldest won't be able to do the things that she wants to do. That her world will be modified. Again, I know that modified is not bad and that she can have a full life. I am not saying otherwise, just saying that I feel like I understand something about myself and my husband that I may not have realized, previously...and I feel like that's a good thing to understand.

So far, our child has not been able to stay in school for a full day since last Easter, when her shoulder dislocations started occurring every day. She did much better at home over the summer, increasing strength through PT and experiencing 4 days at a time without dislocations. Now that school is wrapping up week four, she is back to frequent shoulder dislocations and increasing pain/severity.
A blessing in disguise of home school would be that it would make it much easier for her to continue physical therapy. While we do not want to take her out of her social opportunities, the laws in our state allow for her to attend some classes at school, which may help alleviate that concern.

I'm pretty sure that parenting is never as easy as we all suspect it could or would be, and with special health considerations, the road can be bumpy. What we're learning as we go along, is that as long as we hold each other up and keep traveling down that road (walking, wheeling, assisted, or other), that is the key. We won't give up.

The other day, my daughter was in a lot of pain and her spirits were as low as they could get. She expressed that she didn't believe in blessings because she didn't think a higher power could possibly be looking out for her.  Wow, that stopped me in my tracks. My poor girl, in such pain, and I couldn't fix it. I could support her, say I'm sorry, offer her comfort and love and help to get stronger, but I can't fix it. That is hard for her, and it's hard for me, too. I do think that God, if you believe in that sort of thing, gives us what we need and He must see something in our family that indicates a strength we don't understand. Maybe that's naive, and maybe I'll be joining my daughters disenchantment in a few years, but for now, I feel a blessing in realizing that modifying the path and plotting a new course may just bring about some wonderful opportunities. While it's sad to step away from the idea of what life looks like and turn toward another vision, I suspect that in doing so, we will all be rewarded with happiness, success, and joy in ways that would not otherwise be possible.

So, while we may have been looking at home schooling as a letting go of hope, I am seeing it in a new light today. Today I see it as a better chance, and if that isn't hopeful, I don't know what is.

Bilateral Custom Support Vest has arrived - Ehlers Danlos Syndrome posterior shoulder

Well, it's finally here! After many weeks of waiting, back and fourth measuring, and more weeks of waiting...Hooray! We worked with the fantastic people at Scheck and Siress (Angela and Arnold) to come up with this hopeful solution for the ongoing bilateral posterior dislocations that occur multiple times each day. The vest was designed so that my daughter can put it on and snug it up herself with straps at front of each arm and on each side of rib cage as well as a zipper front. It was designed with as much breathability as possible, though it is still somewhat hot, heavy, and constrictive. It needs to be somewhat snug and firm to be supportive, so it's a good mix.  The bottom photo shows two inserts that sit inside the vest (you cannot see them from outside). They are a firm plastic that help sit right behind the scapula, in hopes to prevent some dislocations. These have been custom molded to fit her back, to make them as comfortable as possible.  They are removable, as well, and the velcro can be taken off and put on the front if they were to be placed on the back of the vest for comfort, for any reason.  Obviously, if we did that, they would not provide as much help, but it's good to have versatility.

We have been to many doctors and this is or current "last hope". No one has a better idea for her, so we can pray that this really helps. Day 1 didn't go great, but getting used to a new brace will always take time to adjust to. Our hope is that this will help her with ongoing good posture and proprioceptive (spelling?)  input to aid in her muscles holding those lose joints together. I hope and pray that this will allow her to do more. It's too soon to tell, however, so I will have to come back and update you in a few months. Please let me know if you have any questions. I know the people at Scheck and Siress said they have made similar devices for one shoulder, but this is the first bilateral that this team has made.  They seemed to have thought of everything and they really did a fantastic job.
As far as bulk, it is very streamline for a product like this, but it does look a little "football player" mode on a young teen. My daughter opted to just wear it over a cotton shirt without trying to hide it under sweaters, and more power to her. She's one brave young woman.

School Days with Ehlers Danlos Syndrome - Got a 504?

One of the first steps in getting safe procedures in place at the schools is to work with the appropriate school team to ensure a 504 Health Plan is in place. You can bring any specialized documentation from your Doctor to the school for use in creating this plan.
If you don't have it already, print a copy of "An Educator's Guide: Meeting the Needs of the Ehlers Danlos Child" by the EDNF group, and take a copy to the school.
What is a 504 plan?
Taken from WrightsLaw, at this link, a 504 is defined as "...a civil rights law that prohibits discrimination against individuals with disabilities. Section 504 ensures that the child with a disability has equal access to an education. The child may receive accommodations and modifications."

Because the rules and legal process that the schools are required to follow can be very complicated to a parent who is unfamiliar with the terminology or the protocol, I recommend familiarizing yourself with Advocacy Guidelines here, and general basics of IDEA, 504 and IEP on Wrights Law, here. 

It is essential to have the legally binding documents that are relevant to your unique needs in order for the school to have access to the resources they may require to accommodate the individual situation. While there will be instances where an EDS child requires an IEP, most children will be directed the route of a 504 plan.  If disability and resulting accommodations are the only goal, the 504 plan is designed to assist. If there is an academic issue or other situation present that will require an IEP, the 504 can be worked into the IEP. 

While some schools will be very knowledgeable and accommodating of how to utilize a 504 plan for your Ehlers Danlos child, others will not have the knowledge and some schools have even given false information to parents. I suspect that this comes from lack of understanding of EDS and lack of understanding of the federal guidelines of 504 plan. Your child's teacher is not likely to be the person you need to talk to about this, so the first step is tracking down the person who represents and reviews cases. Talking with your school principal is a good place to start.

I have found that going into a school with all the specific recommendations, in writing, from the Doctor to be extremely useful. The school was unsure what to do without that information. For example, my daughter is medically excused from PE classes, unless adaptive PE can be provided for her (which, in our school, it cannot). Special accommodations can be implemented into the school classrooms or grounds, such as a wheel chair access ramp if needed. Have the OT/PT/Doctor write out what adaptive devices are needed, make copies, and take this to your school team so that they can understand what steps they will need to take. 

Exercise patience as it will take time for the school to get through their process of meetings to facilitate the plan. And keep in regular contact with your team lead and the school nurse to discuss what is working, and what isn't working. Be flexible with items that are not necessary, and be assertive with doctor advised modifications that are required. You are allowed legal council to attend meetings and help you, if you feel help is needed. We have had great success in working with our medical team and the school team, however, our daughter still gets injured by accident in school.  It is not a perfect system, but it is one we are currently all willing to work with. Under different circumstances, homeschooling may be a better option, but we are not there just yet.

Our school also provides time with an occupational therapist, though it is extremely limited.  I have not found that these sessions truly do anything to help my daughter. Instead, I look at the time with the OT as a good bridge between the school staff and the medical team. The OT / PT they assign at the school are in contact with our private OT/PT and they pass along the instructed guidance and modifications to try. They assess if those modifications are working, and they advise if changes are required.

Going to meetings for creation of these documents can be very stressful, which is why I stress the importance of educating yourself on the process, the terminology, and your legal rights as a parent. WrightsLaw is the best resource I have found for this information. If you have a better resource, please share.

We have a great school here, and I am fortunate to have so many people looking out for my daughter. Even still, the IEP/504 plan she has is absolute key to keep her safety a priority and to enable the school to modify resources as required. There are many specifics of this that I can go into, but I will save those topics for other postings.  Today, I just want to stress that the federal government program is available for your safety and use, and I encourage you to utilize it, if you fit the need for the program.

DonJoy Shoulder Cradle for night time

First, we laugh when we see this photo because it is not entirely a restful sleep when your arms stick straight up in the air.  That is a problem in and of itself, but the shoulders dislocating every day and night were a worse problem.  When the shoulder instability is at it's very worst, we add this Donjoy Shoulder Cradle to one arm at night time, and we use the Donjoy Ultra Sling (shown here) This combination has helped her out tremendously. We don't use the bolster underneath, and the other sling has straps going across the front, so it's all a bit ridiculous looking, but this combination helps her to stabilize long enough for that constant instability to tone up. It took weeks of doing this every night, and on very bad days, she will sometimes wear these during the day, but only for sitting. We find that this cradle doesn't suit her well for moving about (it does have velcro strap to help keep it on, but it's a bit heavy and adds to problems if she walks around with it. In addition to the immobilizers, when needed, we raised the head of the bed and used body pillows to create support where we can. This makes it very hard to toss and turn, which is a good thing. If you think these may be of help, talk to your medical team and see what they think. The Don Joy products have been our best brace/immobilizers to date.

Penagain Y shaped pen for writing

We tried every writing device and combination you could imagine for my daughter's hands and the hyper extension/low muscle tone. She didn't really take to anything well enough to correct the problems. Dr. Tinkle gave her a Y shaped Penagain to try out. This was a very clever option for her that would have been great, had we introduced it at a younger age.  The trouble was that she was old enough that it was very difficult to get used to. She did really well with working on switching her grip on her own around 6th grade, and that has worked fantastically. Before that, she was not ready. I think this Y shaped pen would have been a life saver during the elementary years and I would have loved to have known about it early on.
We also found that typing was easier for her, and especially on keyboard with soft, low profile keys. Her school has provided her a laptop to use for all classes, ever since her OT advised such. This option has been very good, and it also cuts down the number of items she would be required to carry with her from class to class.

Slant board - adjustable and collapsible

Early on, my daughter's occupational therapist recommended the use of a slant board to help support her wrists. We found this affordable solution that travels easily, as it is collapsible and durable. The one we have adjust with heavy duty velcro for different angles of writing. When collapsed, this is incredbily thin, though not entirely light weight. It can travel in a bag for school use, though we opted to keep binders as makeshift slant boards in each classroom. For home use, though, this is a nice option that can travel all over the house for the kid who loves to write and draw. Some schools will purchase items like this for the students to use, if the OT through the school system advises it, so it is worth looking into if your OT recommends a drafting desk or slant board to support the hands and wrists. We purchased ours online, here.
In addition to the slant board, her Genetic Doctor recommend Y shaped pens and other support options. I will add a listing soon.

Shoulder Taping with Leukotape - our experience

Almost every PT we have seen wants to try taping the shoulders. For many people with ehlers danlos syndrome, this is a daily help. For my daughter, it was not a successful option. There are two reasons this didn't work well for her:
1) She still dislocated as often with the tape as she did without. This could be an issue of needing to change the technique, or it could be that is lacked the level of stability she needed.
2) Taking the tape off caused dislocation of the shoulder, which increases the instability.
Because of that, we don't use this, but if you are interested, you can talk to your physical therapist and or med team about the options for your own needs. This type of tape is thicker and less flexible than a kinesio tape. It is meant to be used with a layer of the white roll underneath the tan tape, to protect the skin (so it is a two part taping). Again, I very strongly advise having your PT or professional team member help you with this and have them teach you how to use this support, if it is one that you would like to try. If you want to see the type of taping that was used for my daughter's posterior shoulder instability, Click here and scroll to page 6. This shows images as well as written description of the process our PT used.
Another interested power point on taping options with Kinesio tape can be found here. I am not familiar with these document creators, but I recognize some of the techniques as those that our medical team uses. In the second link, I found the thumb MCP stability method on page 48 to be interesting, though we have no experience with it, nor has our occupational therapist advised it. I am curious if others have been prescribed this approach, and if so, was it helpful?
As a follow up on our daughter's needs, the team opted to utilize immobilizers for an as need basis as well as a custom neoprene vest/wrap, which is being made at this time.

Taping knees for support Ehlers Danlos Syndrome kinesio

 I will have to start with a cautionary warning: Have your physical therapist, Ortho, or other professional medical team members show you how to properly apply taping for your needs as each person will have slightly different needs.  Also, there are tips and tricks that you won't intuitively know, so it really is key to have a professional walk you through this once before taking over at home. Finally, before using any tape product, your team will likely want to do a test patch to make sure your skin can handle the adhesive and fabric.

My daughter's knees starting giving her a lot of trouble in elementary school. When we walk for more than a couple blocks, her knees, ankles, or hips would begin to give so much pain and instability that she would need to stop, sit, or just go home. The change came along with her growth spurts and the laxity that allows her joints to move too far in the wrong directions.  As for the knee, itself, that patella wants to slide out with far too much ease. The taping is only a mild support, and it won't help with more severe problems, but when combined with my daughter's level of ability and ongoing exercises through PT and walking, we find that the Kinesio Tape helps when used as a preventive. How do we use it? We utilize the Patellar alignment model illustrated in combination with good posture, supportive orthotics and supportive shoes. By combining these, she is able to do a bit more and go a bit farther than she could, otherwise. It is a good precaution to take before she uses the elliptical, or before we attempt a longer amount of walking than average. 

If her knees worsened, the medical team has a supportive brace that they would have her use, but for now, she is able to work with this very minimal intervention and continue increasing strength, bit by bit.

As you already know, movement, exercise, and strengthening are key components to success with ehlers danlos syndrome. Having a knowledgeable team is the missing link, to be sure. We have been very happy with the medical team we have established around the Chicago area. One thing we learned, early on, was that using too much support in the form of braces quickly creates more problems than we started with, so we opt to use the minimal support needed and we strive to exercise, in some form, every day. Since my daughter is still a kid, it is most effective when we turn this into a family effort to help keep her on task, and to keep all of us mindful of staying healthy.
Some of the tips our PT gave us was to rub the area where applying the tape to create friction, which allows it to stick better.  Also, bending the knee (see the bolster under left knee) and pulling slightly when applying.  The PT recommended cutting the tape in rounded edges to prevent it from rolling up. Again, when you meet with your support team, they can show you, in person, how to do this. It could be that for your needs, a different approach or tape would be required.

We also have instruction for using a thicker two layer tape for shoulder support, which I will add next. That did not work for us, but some one else may find the information useful, so I will add it to the blog.
As for the finished result, you can see the pink tape knees on the right. This is the method that works best for my child, and the tape has not damaged her skin, which is fantastic. I hope this idea is something that you may find helpful to discuss with your team!

Walt Disney World with Ehlers Danlos Syndrome

I'm curious to hear from you if you have tackled this park with the concern of joint dislocations on getting "bumped" in the crowd. Also, I am curious if a ride has been too rough at a park like this? We have been to Walt Disney World many times.  We avoid the thrill rides and we take long breaks and stop to smell the flowers, but the last time we went we required a wheel chair. I found that this allowed kiddo to participate much longer and it gave her a safe space. When just walking, she can't get very far before her hips, knees, and ankles are too fatigued and with her current and ongoing shoulder instability, the fear of getting bumped is a worry. Please comment with your experiences. I would love to hear how you navigate the "World".
:)

Here are some of our family tips and tricks that we have picked up along the way:

• Go early, take breaks, and don't do it all. The way I figure it, anything we don't cover on a trip is a good reason to go back! It isn't fun if you over do it, and we so often find magic in the unplanned moments in Walt Disney World.
• Go on "green" days. Sites like EasyWDW and Undercovertourist give you advice about which parks are less crowded on which days, and I find that is a good way to travel the parks.
• Use a wheelchair, ECV, or stroller if needed! If walking is not an option for you due to instability of joints or other Ehlers Danlos complication, then take that tool and use it. Also, the extra space it provides you will serve as some protection from getting bumped in the crowds. 
• Wear good shoes. This should be obvious, but orthotics and support are essential when doing walking on a trip like this! Don't let fashion trump smart footwear in this instance.
• Plan ahead. It is very helpful to know what route you will take, what basic rides you want to do, and to schedule your FastPass Plus options in advance. Have meals booked in advance so you have spaces to sit down and rest. We find the long, slow, Disney meals to be a wonderful break from a busy morning or afternoon at the parks.
• Bring ice packs in a small cooler, or instant ice packs. Know where the med care centers are (on the map), and bring required medications in properly labeled containers. Bring any supports you may need.
• Don't park hop. Some may disagree with me on this, and I will say that the exception will be if you go to a park in the morning, take a nice afternoon break at your hotel, then do a second park for a show that night. That is OK, but going from one park to another is adding a lot more walking and travel to your day. I generally plan a day with the minimal amount of back and fourth travel. We will spend a Magic Kingdom afternoon break at a close by monorail resort for a long, slow meal, and a rest by the water before going back to a park. A swim or nap at the hotel is also good. By running from park to park during the day, however, you can wear yourself out just in the process of getting from transportation to the first ride, so I do advise avoiding that when you can.
• Get plenty to drink, stay hydrated, wear sunblock, and follow all the general precautions that would apply to anyone traveling at Walt Disney World.
• Avoid the rougher rides if you can't handle the jostling. For us, the thrill rides are out because our kids are scared of them, but even if they weren't scared, I know my oldest couldn't ride a rough roller coaster, safely. Luckily, Disney has many sit down shows, calm rides, and simple experiences that are still magical without the jarring. 
• GAC card, do you need it? That varies from person to person.  We have not felt the need to use one. When you have a wheel chair, the staff at Disney knows what to do with you and they don't need to a GAC card. If you have a unique accommodation that needs to be made, then it may be right for you. 

So, an average morning for us is to get up early, go to a park before it opens and be among the first in line. We hit our favorite rides, in order that makes sense, and we utilize fastpass plus. We stop for breaks, snacks, shows, or a long lunch. Generally, we are ready to leave the park shortly after lunch and take a break at our resort, or at a restaurant. We can then go back to a park for an early evening show or a few rides with fast pass. Our kids can't make it up to the late shows and they haven't made it up past 9pm on any Disney night in the past few years, so we are early to bed and early to rise. If you are the opposite, you can adjust accordingly.

 As for those unplanned magic moments, there truly are many reasons to go slow. The themes at Walt Disney World are what make such a difference. By going slow and looking at the details, you can uncover magic that is often overlooked. Talking to cast members is often fun and enlightening. I remember during one of our first trips, a cast member noticed our daughters love of reading and she came out of her store and brought a story book. She sat down and read my daughter a story right there in the park, and while that may not sound like much, for my little bookworm, being told a story in that setting with a smiling member of the Disney team was a very rewarding moment. During a different trip, my youngest was so tired, she fell asleep on my lap on a bench. I sat and watched the people go by, saw the show in the distance, and just enjoyed a beautiful day. Once, we stopped to visit the dolphins at the Epcot aquarium and they came right to the glass an interacted with our kids. There are so many moments of surprises and fun that we never would have planned or enjoyed had we been focused only on the rush from point A to point B.

Want to read more? Here is a published article on going through Walt Disney World in a Wheel Chair with EDS. Click here to read . 

Does EDS affect your digestive system? Add travel into the mix and no doubt you have concerns. We have posted many of our meals and photos of the foods we've eaten in Walt Disney World as well as Disneyland online, here. Everything we eat, due to allergies, is gluten free and dairy free. Disney does a great job catering to allergies and diet restrictions due to health issues. Click here to read more.

What is my rib DOING?! Slipping or dislocated ribs and ehlers danlos syndrome

Update: 4/30/15
So, I went to a PT a few months back and he pointed out some general poor posture and hyperextension of the back and stomach, plus using the knees instead of core and so on. SO...he taught me how to squat (much harder than I knew, since I always did it wrong) and he taught me how to use good core posture. He also made me aware of all the wonky one leg balance and stretch motions I made throughout the day that I never noticed.  So, I started concentrating on posture and core. Good good.  He also suggested a tennis ball against the wall and just lean into it when that back rib head starts to shift. So, I have done that ... and it is working!!!
I am so happy that it has been months since my rib has flared up. I just had to share. If you are dealing with this terribly painful and frustrating issue, I advise you to check with a PT or OT to see if you need any pointers on how you hold yourself and how you do your work, in relation to using the "right" muscles. And then, get a tennis ball or a foam roller and I hope it helps you.
I was also given an exercise routine that is hard for me to do, as it flares up headaches and neck aches, but I dabble in it and I will continue to work on it, going forward.

---


Ok, so I don't fret about myself and EDS (Ehlers Danlos Syndrome) because my level of hypermobility actually got better as I got older, and I feel pretty good BUT...my ribs on the back right side have been playing a fun game of shifting at the rib head off and on for a little over a year (subluxing).  It is incredibly frustrating and I don't understand what causes it.  The symptoms include feeling like I cannot catch a full breath (constant attempted yawning), and pain in an isolated area of the back (right over the rib head).  It really is such an annoyance.

I learned some stretches to help encourage the rib to go back into position, as it is not truly dislocated, just shifted slightly (sublux). This seemed to help a little in the beginning, but it got worse and over time, it would mix with neck/shoulder pain and eventually starting causing nerve problems.  I do not have time for this!  I need to be strong to help my family, so I can't be out for rib related pain issues.

I found a chiropractor who agreed to help me without doing all the snap crackle pop stuff that tends to give me migraines. So far, he has been a great help in getting those ribs aligned, but it never lasts. I have increased my stretches and I am looking forward to introducing an exercise routine to build arm, shoulder, and core strength. I also have a bit of scapula winging, and reduced muscle strength due to the pain in the shoulder area, so getting started on this strengthening program is sure to be helpful.
BUT, as many of you can relate, it is very challenging to do exercises that are new because of such easy injury. Whenever I add an exercise, I seem to be out of the game for several days with severe pain. It is ... again...annoying! I have found that I can do the elliptical machine without injury, so that is where I will start, with the stretches, and then I hope to add in weights under the instruction of the PT (to make sure I am not using wrong posture and making it worse).

So, I hope this helps, but if you have the answer to this plague, please PLEASE share. I have consulted several doctors and chiropractors, and it doesn't seem like there is an obvious fix?
Also, if anyone has tips for upper and core strengthening while using elliptical machines, I would love to hear that as well!

Ring Splints

The first time my daughter was fitted for ring splints, she was very young and her ability to hold a pencil in school was problematic. She was hyper extending so much she created her own very awkward fist type grasp to write with. Her professionals within the school district told us there was no way to change her grip and nothing to be done.  This information was incorrect, but I didn't know that at the time. As a creative child, she learned to do anything she wanted with that fist type grip, however, her occupational therapists told us that it was causing her some big problems, including nerve damage. The OT was concerned that she would lose hand use over time by continuing to use it in the manner (forcing early arthritis) By the time my kiddo was in 5th grade, she was sent to work hard on learning a new grip. When given the choice to use a new grip or to use her ring splints, she focused her attention on the new grip and she actually mastered it. I was incredibly skeptical, I didn't think she could do it.  She showed me otherwise, to our delight.
So, while she still hyper extends, her new grip is MUCH better and she is not wearing the ring splints at this time. I do want to share what we used, in the even that this information is helpful.

To begin with, the OT department at our local University custom made her these plastic ring splints. They were hard, and uncomfortable and they appeared to cut her blood flow as her fingers were always quite red/purple. She didn't like to wear them, and I can't say that I blamed her for that. Also, she was probably too young to work well with a product like this as she fought to bend against it instead of working with it. I would call that initial set a failure for our needs. The OT who fitted her for these plastic ring splints then went on to measure her for the silver ring splints, which were much more comfortable! She received a set that fit her well, though they still caused her pain when worn for a school day.

Eventually, she stopped wearing the ring splints and we tried different things.  In 6th grade she tried the silver ring splints again, but this is when she had transformed her grip, so she was doing much better without the splints than she had, previously. The people at Silver Ring Splints Company were very helpful with the OT who helped us.  This OT had never ordered them before, so he walked through a video conference to size her.  Unfortunately, he did size her a bit too small and they are not a good fit, nor are they returnable. The type that was identified as the most helpful for her were the

swan neck for finger tips and the MCP splint with bracelet for the thumbs. Because theses really were not a fabulous fit and because she was old enough to focus on her grip and make progress, we don't require her to use these at this time. Our insurance did help cover part of the costs of these splints.  Also, she had worse issues to attend to with her knees and shoulders throughout these years, so the splints ranked lower in importance when we weighed out all areas needed attention.
My opinion is that the Silver Ring Splint (click here)  products are really wonderful and they would be a great help to an adult, or teenager who is willing to work with them (or even a child who is ready to work with them).  It is essential to have them fitted properly, so make sure that your OT knows what he/she is doing and double check by following the advice on the company site.  If you want to try for insurance, you will likely have to pay up front and then submit yourself. I found it helpful to get codes and insurance papers before I met with OT so he could fill in what info he/she was required, and then I sent along a script from the Doctor, just in case.
These are expensive and there are other companies that make like products, though I cannot attest to the quality.  If you want to compare options, you can also check out Zomile (click here) , and Etsy even has some limited options (Etsty click here).
For my own child, I think the  Silver Rings Splints are likely a product she will want to come back to when she is older. The company makes a functional product that is also quite pretty and it looks more like jewelry than support.
I hope you find this information helpful! Please share if you have other vendors you like.

Orthotic shoes for women

From early on, good footwear has been a necessity in our household, and with the rolling in you see with the laxity, low muscle tone, and flat foot combination, a custom orthotic is key, as well. We get custom orthotic inserts made from a local prosthetic group, and they do a fantastic job. Normally, we add these to stable, high quality gym shoes with good arch support and stability. Some shoes we have used include Saucony shoes that offer support for over pronation, like the Stable shoe pictured and linked here. 

We have also used Asic line of shoes geared at over pronation, linked here.  Both of these companies offer search criteria by pronation issue on their main sites, which makes it easy to find out which shoe model you may want, then you can go shopping. We have been able to fit custom inserts into both these brands. New Balance also offers good options for stability.

While shopping for  gym shoes is not too difficult, shopping for sandals and dress shoes is much more trying! We have found that Clarke's, Jambu, Ecco brands all offer a few shoes here and there that can be utilized with inserts, but many do not. For such situations, a company called Foot Levelers can be useful.

My daughter was fitted for a pair of these Chaos sandals with custom orthotic insert. Because she is a younger person, the Keen and Chaos shoes run wide for her, but this sandal is somewhat adjustable (the top can be snugged tighter), which works for minimal use when going to the pool or beach. The company also offers up dress shoe options as well as slippers and a hiking boot. We were fitted for these by a local chiropractor, and I was skeptical about the orthotic being as good as the ones we buy from the prosthetic group. While the orthotic was smaller and less stable, it was formed nicely for her and it is a very good solution for someone with her specific situation. She can utilize these for the rare times of being at the pool, the beach, or she can try out a dress shoe for more formal occasions. The cost with the shoe was comparable to the custom orthotic alone at the local prosthetic group, and my insurance did pay a portion. I have been told that not all insurance companies will cover the cost of custom orthotics, however. Click here to see the different options available at Foot Levelers. You will need a professional to fit you for these custom orthotic shoe combinations.

Recently, I read a review on the Vionic Orthoheal sandals. Click here to view. These are made with a higher arch, providing more stability than the average sandal. I don't see how you could utilize an orthotic in their shoes, but their process is supposed to help take that step out of the equation for people who need more support from over pronation. It is not likely we will go this route, we may try out their dress flat at some point in time to see if it is a viable option for dress up occasions.

Finally, I have found that a local high end department store carries the widest selection of women's dress shoes that will accept an orthotic.  The store is called VonMaur (Click here for that link) , and they do have an online web presence with free shipping and very good customer service. If you have the option of going to a retail VonMaur to try out their shoes with your custom orthotic, you may find some wonderful (though not cheap) options for specialty situations such as formal occasions, uniform needs, or other. I have found the staff to be hit and miss in knowing which shoes work with orthotics, so I just look through them and find them on my own, most times. However, a staff member did point out a pair of sandals that had a removable sole that I would never have guessed was removable, so it is good to ask them, initially.

Shopping for shoes can be an exhausting and frustrating process when you require special support for Ehlers Danlos Syndrome or other custom orthotic needs. Luckily, manufacturers are improving our options and with a bit of luck, we can all find what we need without breaking the bank.

Next, I will post some additional photos of the types of shoes we have found useful with custom orthotics.

 First is an Asics brand shoe designed with extra support for over pronation and flat feet. We put the custom orthotic in (as pictured) in place of the liner that comes with the shoe.  This is the best for my daughter. She does need to buy these in a slightly larger size and width to accomodate her insert, but they are fantastic for comfort and support.

 To the right is a Mary Jane style that has an easily removable insert.  We were able to place the orthotic in this shoe, but it was a tight fit from top to bottom.  This works for those with a narrow foot, but it would limit the number of people who could fit a custom orthotic in the shoe. One thing I notice about this shoe is that it has more inside arch support than any other shoe I have tried, but the support for over pronation is not there, and requires awareness for those who struggle.

 To the left is a view of the second Ecco brand shoe insert. It is easily removed, though my daughter opted to wear this insert instead of her orthotic for a choir event. These look nice, and they are comfortable. Like the Ecco shoe above, these have nice built in arch support, but they lack a wide front base for over pronation support.  Granted, they are far better than the marjority of "dress" shoes for that need, but still, a custom orthotic may be required, depending on the individual.

Finally, the red shoe below is a Jambu sandal. These are built low and wide, which helps with over pronation. They lack support, but they are a nice option as a sandal replacement in summer. We were able to purchase and then remove the two liners in the shoe.  The second liner was glued down, so we had to commit to this in order to try it.  You can see the cream colored top liner and a thin black liner below it. Those both came out, making room for the custom orthotic. In order to fit the orthotic, my daughter went a size larger, but this worked nicely for her during summer. Still, it was not a quality shoe for long term walking, but a great option for occasions where a gym shoe didn't quite work.

As we try different shoes with inserts, over the years, I will add more photos.

S3 Brace

Image picture from Alignmed site, here.
One of the surgeons we consulted with prescribed the S3 vest brace for scapula support by Alignmed. We ordered this in the smallest size at the time. It turned out to be too big to function properly for my kiddo, so we don't know if it would be of help or not. While the waist area fits fine, and height is probably right, her width is too narrow for the support to hit where she needs it.  We have it on standby for when she is older. In the meantime, we are looking at a customized option from a local prosthetic maker, and I will post more when we have it finalized.

Joint Hypermobility Handbook - a must have primer

Dr. Tinkle is an expert on Ehlers Danlos Syndrome and a blessing to the many patients who found answers with him when they couldn't find answers elsewhere.  His book, Joint Hypermobility Handbook, is a must have for anyone with EDS Hypermobility. It is a wonderful and eye opening overview.
Now, if he would write a more expansive and detailed sequel, I would be the first in line for a copy!
Image of the book from Dr. Tinkle's publisher site, here.

Posterior Shoulder Immobilizer Donjoy Ultra Sling III

Pictured from the Donjoy website, here.


So, starting a few years ago, my kiddo began experiencing shoulder dislocation followed by shoulder instability. This was very scary for us in the beginning, and while we panic less as the years go by, we still struggle not to be enveloped by fear at times.

A product that has helped her, tremendously, during the most unstable time period is the Donjoy Ultra Sling III ER (we have an older model, but it looks the same). This helps her stay reduced long enough that she can get back to physical therapy and back to life. She had tried many slings and braces, but none worked at all until we put this on her.  Her dislocations are typically posterior (out the back) shoulder, and this sets her at the proper angle to keep it in.

Benefits:It's soft and adjustable, so it grows with her. It works great for posterior dislocations of the shoulder (better than anything else we have tried)
It can be worn to bed
Easy to pack and bring along (just in case)
All adjustable, so can even be switched to other arm if needed (though I still advise letting the professionals help you with that)

Disadvantage: It's confusing! you do need a professional to size this properly for you, then take a photo so you don't forget! If you have to readjust it, you will want to reference the photos.
You can't safely wear two at once while sleeping, due to straps (if bilateral support is needed)
Can pull on the shoulder, so wrapping with fabric or wearing a collared shirt can be helpful
Only came in adult sizes when we ordered, so not an option for smaller children
If bilateral shoulder instability is present, this can be too rough on the opposite supporting shoulder

3 years ago, just 4 weeks in this brace would have her stable and ready for physical therapy like a champ, but as she ages and her shoulders get worse, this isn't working as well as it used to. We are still using it at night during the ongoing daily instability, and around the clock during the worst of times, as advised by the medical team.

Massage musings

Her hands moved along the length of my shoulder, popping and cracking their way through.
“What is making the grinding noise when you do that?” I asked her.
“The knots in your muscles. Yours are very hostile”. She responds.
Hostile. My muscles? My connective tissue itself? My genes? This Body? Hostile.
“Your worries and stress are all being held right in here”. She moves her hand lightly along the area where I can’t even remember relaxation. The tightness that holds my posture and strength and possibly even my resolve. That is where her hand circles.
My worries. I avert my eyes and think “Please don’t cry!” I’m always wound tight and crying is easy to come by on any given day these past 13 years, but I don’t want to cry here and feel the fool. My worries and stress…my mind jumps to an image of my daughter, in her immobilizer. Her shoulder unable to stay in it’s socket. Her body rejecting it’s basic design. Hostile. My genes passed on to her. My worries. My resolve not to break down and cry.
“How’s your daughter?” She asks. This young woman sees right through me. I’m sure my eyes tell my fear and sorrow in the moment before I can steady them with a cover.
“She’s in the immobilizer again. Back to the doctor tomorrow. She can’t seem to keep that shoulder in it’s socket. Hopefully they have something new to try”
“Oh. Poor girl.”
Yes, that. As the masseuse moves her hands across my shoulders, down my arms, back over the shoulders and down the spine, I feel new awakenings of pain with each area she touches. So many muscles holding tight to worries of their own. I didn’t even know they were hurting like that. Somehow I managed to overlook the individual pains and accept the overall pain in my neck and head that is almost always present. I assume this connective tissue disorder is to blame, but so what. My daughter has it so much worse.
I imagine how her body would respond to this massage. Her shoulders would certainly pop out, then suck back in, leaving that hollow space and hollow feeling for a moment, followed by a jolt of sharp pain. Her fingers, her knees, her jaw…No. No massages for her.
If I have overlooked pains and worries camping around my muscles throughout my body, they are insignificant to the non stop inflammation and pain my first born lives with. It’s unlikely she has ever lived without pain, so she probably doesn’t realize how bad it is, but she knows it is there. Fatigue grabs onto her during regular activities and it steals her away bit by bit. First with a turn of the foot, then a limp. A knee that spins inward, a hip that jostles and slags. Her joy fades as she trudges on and soon, hopefully, she can stop. Rest. Ice. Medicate. It hurts. It always hurts. And why? The genes. The connective tissue disorder that I passed on to her without realizing what I had done. Certainly some of those worries and stress are born of guilt and of sorrow.
“Finished” says the masseuse. She smiles and glides from the room, ready to see the next client. I get up, feel each newly awakened ache and swim in a heated pool of grief for my girl. I put on my shoes and go home.

May is Ehlers Danlos and Arthritis Awareness Month - food?

May is Ehlers Danlos and Arthritis Awareness Month - food? 

Shared from my food blog

It's Awareness Time! Truth be told, we are always aware of Ehlers Danlos Syndrome as it plagues the life of our child with a long list of symptoms. The worst symptom she is dealing with is recurring shoulder dislocations. We have a wonderful medical team of specialists to help us through these trying times, but there is no cure for Ehlers Danlos Syndrome. My hope is that there will be more permanent solutions and earlier identification to help people with this condition.

Now, this food related blog may seem an odd place to post about Ehlers Danlos Syndrome (EDS) and Arthritis, but there is an important connection. As the EDNF shares with us, there are many gastrointestinal tract complications that can accompany EDS such as:

• hernia
• diverticula
• delayed gastric emptying
• GERD
• adominal pain
• IBS and more

In our family, we discovered that our own bodies had strong negative response to certain foods and we find a combination of other foods can also cause upset.  We are a touchy tummy group. My daughter is diagnosed with Celiac, whereas I discovered my offending foods through trial and error as a teenager with an ulcer and chronic relfux. I can't help but to wonder, now, if EDS was the chicken, or the egg, in our quest to understand our tummies.

As for arthritis, it has been reported that a diet based on low inflammation protocol has been helpful for those suffering with this condition. Dr. Terry Wahls, known well for her work in defeating Multiple Sclerosis through dietary intervention, has a  wonderful low inflammation protocol. Is it a coincidence that low inflammation diets typically avoid gluten and dairy? Not at all. These difficult to digest proteins can be swapped out for a wider variety of alternatives. Just like with fruits and veggies, it is important to get variety in our grains and protein sources in order to attain a balance of minerals and nutrients.

So, while we are educating ourselves on new topics, and health in general, it's worth spending a bit of time boosting up those colorful fruits, veggies, and easy to digest grain alternatives such as buckwheat. We can look to reduce sugars and refined grains and look for lean pasture raised meats and omega 3 rich fish. This approach can be very helpful for a variety of health concerns, not unlike the ones mentioned above.