Intensive Physical Therapy Rehab

Here we are a skip away from Ehlers Danlos Awareness month and it has been a month of heart ache. Bean's hips and shoulders have gotten worse in the past 4 months. Her walking is greatly reduced. She has switched to a wheelchair to get around the school, but she cannot attend the majority of the time due to so many painful and frequent dislocations. She is losing strength and motivation, which is a bad combination. We started seeing a Therapist to help talk through these difficult feelings. I say "we" a lot, when really it is all her. I am a part of her process, but I don't know the pain she struggles with. I am just here to support and learn, help and love.

I have learned that I cannot lift a teenager who is unable to get up after all limbs have dislocated at once. We are working on talking through strategy to get limbs back in place, one at a time, using core strength and so far, she is doing a good job of this. She no longer uses the stairs and stays on the first floor of the house. She will do stairs once in a while, but it is very difficult and scary for her, so she opts not to use them unless it is really necessary.

If you have read the past posts, you can see we are going in the wrong direction, but that doesn't mean we have given up on hope for the right direction to make itself known. Currently, our EDS medical team is working to find an intensive physical therapy rehab group that can work with this unique situation. They recommend she do homebound school for the remainder of the year and focus on this rehab as priority one. We are all on board with this, as what we are doing doesn't seem to be helping.

I'll be back with posts for awareness and hope throughout May, and I hope to read your stories and comments, too. I've been following Meg's journey and she continues to break my heart and inspire me all at the same time.

POTS. Salt, salt and more salt

To combat the sodium problem, the original recommendation was for my daughter to drink Gatorade every day. Gatorade has a lot of sodium, granted, but it also has artificial ingredients and sugar. A little investigation revealed that if you purchase the
G2 line of gatorade, you get a lower sugar product that does not contain the same crazy ingredients, and they even have some clear varieties that avoid coloring agents. So, that is good, but it still delivers sugars and without the movement of regular exercise, that is not great, so we add a lot of salt to food, but we still need her drinking plenty of liquids and it's been a challenge to keep the levels high enough to make the impact that is required to reduce the POTS symptoms and raise that blood pressure.

A co-worker recommended a product to us that is an

electrolyte powder packet from Pure. It contains some sodium (not as much as a gatorade, but still a nice amount) and some magnesium, which is also recommended. I like that the magnesium dose is small enough to not bother her touchy stomach, and I like that this product does not contain a long list of minerals and amino acids. We don't need all of those ingredients, so this seems pretty good. The only problem is the flavor. It is a powder that you mix with water. To me it tastes like nothing....like water, but to my daughter, it is like drinking a glass of sweat. So, that is not an option for regular use.

Douglas Labs make a similar product in a capsule format (Klean Electrolytes), which would be much more doable for my daughter. The only problem with this is that I don't know exactly how much she should receive and if the other ingredients would get too high if we went to the level she needed of the sodium/mg. I have that question out to a pharmacist at the moment and I will follow up with the answer when it comes in.

Finally, there are basic salt tablets that deliver sodium, alone. The amount of sodium is about the same in one tablet as in 2 gatorades. That is very targeted and perhaps it is where we need to go. The doctor did suggest it as an option, though I am not sure how to take such high doses of sodium and not throw the body off in terms of ATP or general hydration. I feel like we need more specific guidance rather than "increase sodium intake". This will require a bit more research. In the meantime, I will wait to hear back from the pharmacist and start my question list for follow up with the doctor. My hope is that the right balance of sodium, liquids, and compression garments will prevent the need for blood pressure raising medication at this time.

Irish EDS and HMS information

This site has some very helpful information for explaining EDS to schools, teachers, family and friends. Especially helpful are the pamphlets geared toward children, found here.

Check out their site for photos that simplify what types of movements fall into the hypermobility category, without having to perform the human tricks to show others. I am curious if Irish ancestry plays a heavy role in the genetic trait. I know we have family from Ireland, if we follow the family tree back a ways, but I don't know where this genetic condition is most common. Check out the great information found on the Irish EDS & HMS site, here, and follow them on facebook for more information to share on rare disease day and EDS awareness month.

Tilt Table Test

I will come back to delve into this further, but the tilt table test was done today. She threw up afterwards, 3 times, and the doctor told us that is not normal. He said she has clear POTS based on the testing. The doctor advised 2 medications. One to lower blood pressure and the other to act as a stimulant to the central nervous system. He also advised starting the medicine slowly, and using compression socks daily.
Again, I will come back and fill in more details, but I wanted to get this down quickly.

---Follow up a little over one week later---
We have started with compression sleeves at about 20 for pressure. These seem to be helping, or we are just lucky. We're being very careful to make sure she gets her fluids before getting out of bed, and lots of salt! We've also been trying different electrolyte drinks and powders. So far, she hates all of them, except some gatorade. So, the G2 is lower in sugar, lower in artificial junk, and that is our current choice (plus all usual liquids). This is helping...I think.

We haven't started the medicine yet, as we wanted to see how she responds to the other things, when applied consistently.

---Follow up in April ---
The tilt table testing caused some off kilter balancing in her system. She threw up immediately after the test, and multiple times that day, and then the following week. Ever since, she has had constant motion sickness in vehicles, which she never had before. She experiences the nausea daily, which is new since that test, and she is still upset about the actual testing experience. She would not recommend it or advise to another EDS-er in her situation.

POTS, fainting and wheelchairs

Well, kiddo climbed a mountain, so to speak, during her little sisters birthday party. She participated in a climbing event that left both her father and I in shock that she could do it! The smile on her face was worth the world. She was so happy, just being a kid.

Fast forward 2+ weeks and she has been dislocating and subluxing daily without the ability to walk more than 15 feet. Her body is screaming at her with every movement as if to say "What were you thinking, treating me like that?". Still, 2 weeks out, she says it was worth it.

This past weekend we saw a bit of improvement, however, today she fainted in school while sitting in her wheelchair. That baffles me. I thought the fainting was only when standing up, sympathetic nervous system, blood pressure etc? This is new, and it isn't good. The one explanation I can muster is to think she overheated, as she did have her winter coat and scarf on, indoors. Still, it's not a comforting feeling over here. Next week is the tilt table test, and new orthotics, so we'll see how that goes.

In the meantime, wheelchairs, help on the stairs, and limited movements while trying to add in some isometric exercises from PT.  Plus she has started taking more medicine. Of course we hoped to avoid that, but Aleve was about as effective as water, so she is trying Meloxicam in the morning, and 1/2 tramodol to help get through a half day of school. Voltran gel applied, topically, to the neck. Even a little aromatherapy from a www.BeanTreeSoap.com  essential oil rollerball. We'll try anything that may help.

This is another day in the life with EDS.

Hip Brace & Hope (Read to bottom for a GOOD shoulder update)

  Here is a new hip brace for my daughter to try. I'll fill you in. First, I see a lack of options for people with conditions like this. For starters, if both hips are subluxing, there is not a bilateral support, of this type, available. The reason is...not enough demand. So, instead, we use braces designed for post surgery, which are not exactly the right tool for the job, but as close as we can get. Also, we are not sure if this will hold up to the kind of wear that it may require. These are very expensive items, and without knowing more about how it will work or hold up, it is a big gamble. After my daughter has used it through a long enough span of time to get a feel, I will come back and update.

So, why do we have this and what is it for? My daughter stood up one morning, as she does every morning, but this time was different because she passed out upon standing. She fell to her bedroom floor, and her hip and both shoulders were "out". I heard her thunk, and went upstairs to see what was wrong, and found her in this situation. She woke up, quickly, but she needed help getting herself seated so that she could start putting joints back into place. It was a painful and frightening experience, but luckily the hip made a loud "thunk" and settled back into place, followed soon after by each shoulder. The trouble was that the hip began popping "out" easily and often over the next 7 days. 

The orthopedic surgeon examined our daughter and determined that the joint is not likely to be dislocating, but instead to be subluxing. We understand that the hip is a huge joint, and if it was fully dislocated, I can agree with the doctor that it would not likely be something we could manage on our own at home, in terms of reducing. The brace is suggested to add support in the form of feedback. If my daughter pays attention to the cues that the brace will give her, she can find herself in better positions throughout the day. For example, she has better stability with her legs at shoulder width apart. The brace begins to pinch, lightly, when the feet come close together. It is not enough of a pinch to make force a change, so it is a definite team effort for the brace and the wearer. The comfort is good. My daughter reports that it feels safe and secure, and it provides her with a sense of security.

Looking at this brace, on, I don't see how it could possibly prevent a sublux, but I do see how it can provide constant feedback. My daughter will wear her brace only during sessions of instability with the hip, or when she feels like it is sensitive to the point that it may sublux. That is our plan.

We have a wonderful team of people at the bone and joint institute bracing store who help to find solutions for the instability problems. This may not be the only or best option for her hip instability, but it is good to have some form of help. When the hip is unstable, there is almost no activity that is safe.

Regarding the fainting session that started all of this, we will be following up with a cardiologist in the new year, and I will post if we learn anything helpful to share at that session. She is already diagnosed with Autonomic Dysfunction/POTS and she does tend to pass out about once every 1-3 months. She has no warning for this, so she just falls and wakes up almost immediately. These falls are terrible for any person, but especially for someone who dislocates so easily.

Some good news...the shoulder instability that plagued her for years has come into her control. I don't think anything has changed in her anatomy, but it is possible that some minor tears have finally healed after so much break time. I don't know about that, but I do suspect that she has learned how to "brace herself" all the time. As long as she is not startled or taken by surprise, she seems able to do all normal activity without shoulder instability. She is able to stay in school longer and when her shoulders dislocate, she has been able to reduce them quicker. We had all but given up hope that her shoulders could improve. It was years of multiple dislocations every day, and so many doctor and surgeon visits telling us there was no hope. To come out of those past 3 years with this year...it is HOPE! And I want to pass that HOPE on to whoever is reading this. Don't give up. 

Kiddo is back to physical therapy again in a week, to start a new session. This session is geared to support her hip issue, though I am sure they will focus on core and full body. We have a great PT team here, as well.

So, that is the update for the moment. Let us know if you have found success with supporting your hip instability, and if so, through what measures? Also, if you have any questions about this post, please feel free to ask in the comments. Thank you!

--- Follow up on hip brace ---
After giving it a good try, the hip brace has proven a poor tool for this situation. Because she needs bi-lateral support, the brace only aids one side, primarily by giving her feedback for awareness. She subluxates so frequently, however, that it doesn't help or work because having the brace on makes it hard to reduce subluxations when they occur. We will consider it for a one sided hip flare up, but not working when both hips are flaring and shoulders are flaring, due to the restriction affecting reduction.

What is EDS hypermobility type? This article breaks it down.

http://www.dynakids.org/Documents/hypermobility.pdf This is the best article I have read for explaining the basics of joint hypermobility (EDS) and the common related issues that so many people experience. I can see this being useful for schools, family members, and some medical team members, too. This is not an in depth technical article, but more of an easy to understand for anyone type of article. Joint Hypermobility and Joint Hypermobility Syndrome Written by Dr. Pocinki.