So, today we reviewed the left shoulder MRI arthogram and it looks good. That is fantastic news. Even with the many dislocations, that shoulder is holding it's own. I asked the surgeon about the allograft techniques and patches that some are doing. He mentioned Dr Millett's innovative work in this area, but his opinion was that any move toward this type of procedure was a guarantee for arthritis in that joint in upcoming 2-3 year time frame. He did give me two names from Mayo and he advised that if we wanted to go further down that route, go to Mayo. He said Chicago does not have answers for us. He said no one really had a solution for us, but he definitely indicated that Mayo or Boston would be better places to look.
He was friendly. He answered my questions. I just wish there was something better to say on the subject. He did agree that new advancements and discoveries are always being made and that we should keep eyes and ears open and keep on hoping for something better than the non options we have here.
When we moved to Chicago, I felt certain that we had the best medical care in the world right outside our door, and I still know that we have fantastic doctors and nurses around our area. I am just surprised that the best and brightest in Chicago are all sending us packing. It does start to feel like the world of shoulder experts is a small one, and they all seem to know of each other, if not know each other from school or working together.
So, what is the plan? Keep balancing this line between rest and exercise. Keep staying home more than going out (dual enrolling in school, using wheel chair in crowded places). Keep wearing the brace and possibly pursue a second custom brace in the future. Keep learning how to support the shoulders through core strength and posture. Keep hoping, researching, growing, learning, and moving forward. And, even though it doesn't look like our daughter will be able to stabilize these shoulders, if we can help her to stay stable enough to avoid living in immobilizers, we have won a small victory. She is doing much better since we started homeschooling. So, as small a plan as it is, that is the plan for now.
Cryoderm Icy - a step up from Biofreeze
Scapular Dyskinesis
This was written in a report for kiddo as something she is dealing with, though no real explanation or plan was given to us in relation to this muscle retraining situation. I don't know if she is able to do the PT required to retrain muscles at this time, but I was given this article by a young woman who has worked on this very thing. Posting it here to read and ask the PT about during our next visit.
http://www.ncbi.nlm.nih.gov/pubmed/12670140
A second article -pdf - that explains how to put this into practice.
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1323395/pdf/jathtrain00003-0099.pdf
http://www.ncbi.nlm.nih.gov/pubmed/12670140
A second article -pdf - that explains how to put this into practice.
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1323395/pdf/jathtrain00003-0099.pdf
Standing Still
The recent meeting with the shoulder surgeon resulted in the same direction as the previous shoulder specialist and the general surgeon before that. They all concur that there is not a surgical fix. They all concur that any surgery will fail within a short time period due to the connective tissue disorder. While the geneticist says that the surgeries will be a success in that they will add some stability, the surgeons say no way. Further more, we are told that getting one surgery will lead to another and another, and ultimately the surgeries and post healing will lead to increased damage beyond what the dislocations are doing.
What did we learn? We learned that she is not a strict posterior dislocation case and that she is truly multi directional, which makes sense. It was good to have it confirmed so we know just what we are dealing with. We were advised to go to Mayo if things got worse, but to otherwise plan to live this way. We were told that as she ages, her body will grow muscle mass that can help (if strengthened) to hold those joints in. This may be enough for her to learn to hold her own shoulders in, if she is willing and able. We were told there is no hope for a surgical fix when she stops growing.
The Doctor was nice enough and he is a well respected expert in his field. I asked if the ongoing hundreds of dislocations could have created a specific injury, over time, that could be fixed to help increase a level of stability. He said he followed that logic and that we can order a MRI Arthogram of the left shoulder (as we have not had an MRI on that shoulder) to rule out anything major. He stressed that it would, indeed, need to be significant to result in surgery and if it were a small tear, as she has had in the past, they would let it heal on its own.
The appointments with the geneticist give us hope, as he tells us about surgical solutions that can decrease the severity of dislocations, or at least decrease the number of them. Not a total cure, but a respite. But the surgeons (and we have talked to 5, in total, now) all say No. So, our hope is raised, and then dashed again and again.
The latest doctor told me that we could keep chasing surgeons until we found one that would do surgery, but that we would regret the 6 months of painful recovery and the short lived benefit followed by the damage that would result once regression took hold. He did advise Mayo, again, for future investigations if needed.
So, we know roughly where we stand. It's a precarious position to balance, if you ask me. It could be worse, so we must gather our blessings and move forward to the best of our abilities within the confines of movement that work the best for our daughter. In the meantime, I am awaiting a brilliant discovery or medical breakthrough that will make it all easier for her. That is my latest depository of hope.
What did we learn? We learned that she is not a strict posterior dislocation case and that she is truly multi directional, which makes sense. It was good to have it confirmed so we know just what we are dealing with. We were advised to go to Mayo if things got worse, but to otherwise plan to live this way. We were told that as she ages, her body will grow muscle mass that can help (if strengthened) to hold those joints in. This may be enough for her to learn to hold her own shoulders in, if she is willing and able. We were told there is no hope for a surgical fix when she stops growing.
The Doctor was nice enough and he is a well respected expert in his field. I asked if the ongoing hundreds of dislocations could have created a specific injury, over time, that could be fixed to help increase a level of stability. He said he followed that logic and that we can order a MRI Arthogram of the left shoulder (as we have not had an MRI on that shoulder) to rule out anything major. He stressed that it would, indeed, need to be significant to result in surgery and if it were a small tear, as she has had in the past, they would let it heal on its own.
The appointments with the geneticist give us hope, as he tells us about surgical solutions that can decrease the severity of dislocations, or at least decrease the number of them. Not a total cure, but a respite. But the surgeons (and we have talked to 5, in total, now) all say No. So, our hope is raised, and then dashed again and again.
The latest doctor told me that we could keep chasing surgeons until we found one that would do surgery, but that we would regret the 6 months of painful recovery and the short lived benefit followed by the damage that would result once regression took hold. He did advise Mayo, again, for future investigations if needed.
So, we know roughly where we stand. It's a precarious position to balance, if you ask me. It could be worse, so we must gather our blessings and move forward to the best of our abilities within the confines of movement that work the best for our daughter. In the meantime, I am awaiting a brilliant discovery or medical breakthrough that will make it all easier for her. That is my latest depository of hope.
A New Year, new specialists, and hope.
Nervous. New year, seeing a new shoulder specialist next week. Odds are he will order more tests, which are difficult for our daughter. Worried he will suggest to surgically fix her. Worried he will suggest there is still no fix. Kiddo expressed that last year was her favorite year because she had friends in school and it was fun. She says being at home for school makes her miss out on the interactions she enjoyed in her AP classes, with kindred spirits. Of course, she can't stay in school because her shoulders sublux and dislocate when she is there, but she wants to be there. And she wants to be able to go to high school next year. I have no control over any of it, and when the new year starts, and new specialists are on our immediate horizon, it gives me a panic and makes me feel tiny. So much hope goes into each of these appointments, and the track record is to leave with heartbreak and increased pain. Still, for now, we hope.
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.
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?
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.
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