SMA type 2 (under 5): Talk it out

This my first conference and I have two daughters with SMA, a 4-year-old who is type 2 and a 5 ½ that is type 3 and I just wanted to help out and get everything started. I think that this is one of the best forms for finding out what is working for your family and just help each other and say what works for you and what doesn't. Is there any topic you guys want to start off with?

We have a sleeping bag to help our child roll over you know with less friction and then we actually have a trundle bed that folds out underneath my other daughter's bed and my wife sleeps there so she is not having to wake up all the way. For a while she would wake up every, you know whenever, and go in there, and she wasn't getting enough sleep, and it was like she was getting stressed out and now we just pull the trundle bed out and she stays there. I know that is not the best solution. Sometimes I tell her that she needs to learn to do it herself.

I am going to sound really ignorant, but my son is only 2 ½ and he, uh, I don't know, is it not good for them to stay sleeping in the same position. Is it a health problem? He doesn't roll by himself and he stays in the same position all night.

I wanted to share with you all to see if maybe any of your children do this, but my daughter likes to sleep with her ankles up to her head and I am concerned that with her alignment being so far off that she is going to hurt herself that way. Bedtime is the only time she sleeps that way.

I don't know about alignment but if she sleeps like that for a long period of time just pulling her legs back down she will be stiff. You know how you feel when you get into a position for a long time. My daughter gets herself in quite a few really weird positions, she doesn't stay in it sleeping but when she is sitting and playing and stuff like that she will pull her legs up to her head, I mean with her arms, she uses her arms to lift them up. My daughter really likes playing with her legs, that is what I see her doing, she gets the joy out of moving them around, you know, it is a part of her body and she experiments with her body a lot.

My daughter, she wants to do so many things, you know, I want to do this, well you can't, and I always feel like I am going to discourage her from doing the things she can and I don't know if anybody has that problem and how to deal with that.

Well if you are keeping her active and busy with other things then she is not going be as irritable.

I am very strict with my daughter and that was kind of the way I was raised and with her condition being as fragile as it is, my heart goes out to her everyday, but I don't always let her know it in the sense of activities that she wants to participate in. I have a lot of faith in her that one day she is going to have full independence and being able to accomplish things and my goal is to help her get there and with her being so limited, there is so much that she wants to do and she cries a lot, you know, she will tell me "I want this" and "I want that" and you think, first thought is she can't do that but what I try and do is with everything that I do with her is if she says she wants to do something if a normal child can do it I find a way that she can do it. I am lucky, I don't work, I am home with my daughter all day and I have got other children but with all of us together you know, we work it through for her so yeah, okay she can't walk, so I help her. You know, I get down on the floor and get on my knees sometimes and I will hold her hips and right now she is at the point where she can do some of the work and I do some of the work. But I don't care if she gets to where she can't do so much as she grows, I am going to learn a different way to deal with it, you know I am going to adapt our home, I am going to adapt what I do for her to do these things for her. But as far as when it comes to the normal twos when they are throwing a fit they want this and they want that. Like they want this and you give it to them and then they want something else, that it just terrible twos and I know it is really hard cause they are so special and they are so sweet, my daughter, I mean, her eyes are these big blue eyes and she has got this sweet soft little voice and it is so hard, but you have got to put your foot down. I feel you have to put your foot down, and you have got to kind of find a happy medium between what is for your fragile child and what does for her socially in the real world because that is what she lives in. I don't know that is just my opinion.

Has anybody tried the Botox shots? You know in their legs for range of motion and basically loosen the muscles up enough to better their range of motion.

Have you been reading the stuff on the website?

No.

A friend of mine has a child who had the Botox injection and had very serious complications and ended up in the hospital and according to Dr. Haas, the way he feels is that it is not appropriate for children with SMA. It is more for spasticity and these children don't have that.

I asked my daughter's therapist about it after I had read it on the website to see what they thought about it and if they had heard of it and an accumulation of all her therapists they had 3 children that had the shot and had severe affects from it and they weren't affected by SMA but they were neuromuscular disorders.

I mean, you know, you take your doctors word, and from what I understand they see the majority of SMA kids in the greater Boston area and you know he was just like oh, well you need to do Botox because that will help and it turns out that fortunately my husband asks more questions than I do and he said no don't do that I am on my way.

I guess I don't necessarily agree with Botox shots for anybody let alone giving it to a child who is already dry. Well, our doctor said he had done it before so I don't know about that family but yes they had SMA too.

What my father said about it was, yes it could treat the issue by relaxing the muscle but you are putting a toxin into a system that really can't deal with it well, because what that does is paralyze muscle or any type of muscle tissue.

What do you think about nighttime bracing? Does anybody do tension bracing at night?

I do. We use the night bracing for my daughter who is type 3. She walked on her tiptoes and that really helped out. It made a really big difference, sometimes she doesn't want to wear them but she wears them because she knows that they really help her and then with my younger daughter, we just found some cheap, 10 dollar, things that wrap around her knees at night because she wants to curl her knees up and that has made a big difference too.

My daughter has this finger on her left hand that stays curled all the time and the therapists said that it is from being tight fisted even through out the day when she is not really using her hands she keeps them so tight fisted so she felt that it was not able to stay in its relaxed position like when you relax your palm and fingers will be open. And she has got two braces for her wrists where it basically keeps her fingers relaxed and after using them the finger straightened out fine. The brace has 3 wraps around it, it has got one that wraps around the bottom of the fingers, one that wraps around mid hand, and the other one wraps around her wrist but again the fingers are in that curved relaxed position and now when she is awake and playing, that finger stays straight with all the rest of them. Night bracing is awesome. She also does the night bracing on the dorsiflexion and the plantar flexion, the pointing of the toe, we used to have to do exercises with her all throughout the day on pulling her foot back for her and it started out we got a pair splints from MDA, they were supposed to be walking braces but they didn't work out for that. But her therapist said well hey, you can use them for the dorsiflexion because they didn't allow her to point her toe and then I put them on her every night and overtime, about a month or month and a half, something like that when I saw her without her braces on I saw her playing with her foot, and she would pull her foot back on her own. I mean not even using her hands; she would do it on her own. So night bracing, yeah, especially when you put the muscles in the relaxed position because they stay so tense, they get so wore out and I could go on and on about it but it works. It has done wonderful with my daughter.

I have a question about wearing them at night because I just feel like adding just one more thing to their comfortableness at night…

Yes, I know, she has got the 2 braces on her hands and then she has got the two braces on her legs and you know you look at her… When we first started doing it she wasn't rolling as well, because that is one of her therapies is getting on the floor and rolling with her and play with her and her rolling has improved so now it is a little bit of a different issue but in the beginning she was like you lay her down and she wakes up she is in the same position you put her down in and at that time we put the braces on through the day. When she is not playing and using her hands and stuff like that and she is taking a break or when she is watching TV or something and she doesn't necessarily need her fingers then I could put the braces on then and same thing with her legs if she is not standing, if she not in her stander or assistive walking or something then we put the braces on her through the day and it is really the same principle. The only thing with