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 using them at night is then she has more opportunity to go bare footed and explore with her toes during the day, which is something she really likes to do. And since the hands are used more than any other limb then that really limits the time they can be braced and it takes much longer to be effective. My daughter really didn't give me much trouble in the night. You know and then I started the stretching test with her, if she could use her muscles to pull her foot into the dorsiflexed position then I would not brace her, and then the same with the finger, and when it was still curled I would say, "Ut oh, it is still curled." And that seemed to work with her, but she is really easy to "trick" into doing such things, which I am very lucky.
Well, my daughter hasn't shown me having any of these problems yet, is there something we need to be doing now anyway or what are the symptoms because our therapist hasn't said anything to us about any of this.
We have found that we had to realize what was going on, on our own and then present it to the therapist.
Also, what about who can you turn to when things are so different?
We always thought that there was only one way of taking care of these kids and the therapists and doctors would know what to do.
No, that is not true. You gotta look at the school that they went to, you gotta look at their opinions and their views and how they interpreted what they learned and everything. In my own city, she has had two different physical therapists, the first one, we couldn't take it, her views and her opinions were just awful, you know, they were just so definitely not appropriate for my daughter, so we had to change. And the different doctors that the families, you can talk to probably everybody in this room and their set up for their child is going to be different, but the most important thing is that you know your child, you understand your child needs, you take the information they give you and you may have to sort through it a little bit but you have got to find out what is best for your child. Because you see her, him or her, I am sorry, you see her everyday and you know what her so well, you know her body, watch her body, and watch it closely. That is what most of us probably have had to do, that is what I had to do.
I honestly, I have a therapist that have dealt with a lot of SMA kids, and I got her address through Children's Health Services and I feel that this therapist is the most valuable tool that I have and so over my pediatrician and over my orthopedist and going over everybody else, I trust her information more than any body and I don't know how anybody else feels about that. But you have to take with the grain of salt that these doctors do not deal with the physical therapy aspect.
We live in San Diego, and you get in as an emergency patient and they will pay for your visits there and you will go on regular visits once a month so it doesn't affect your child nearly as severely. You go in and you tell them what you want to do and tell them who you want to see and you get to see the pulmonary specialist if that is who you want to see. You can see a social worker, therapist, whoever, so if anybody has a muscle disease clinic in their area, I'd go check it out.
I have a question; can anyone share what they do for potty training?
My little boy follows me around the house, so I just started by taking him to the bathroom with me. Yeah, we do that too, and ours has a little chair in there they sit on when we are going.
Has anyone had trouble with constipation? Oh yeah. What kind of medications are used for that? We are on Myrolax.
Yes, that works wonderful. I keep being told to fix it with diet and fix it with the stander, and I am sorry I am doing everything I can, she is on a high fiber diet, and I don't feel that it is right that she should always be constipated.
I feel really bothered when I give her a regular medicine, you know, I am not a medicine giving Mom, and when they prescribe one, oh there must be a way that we can avoid this.
We seem to be in a little more control with the Myrolax, because you can adjust the dosage according to how well your child goes, so we give ours every Thursday, and until they go potty, and then finally I was like you know I don't think that Myrolax is doing anything. We are doing our standing and he is eating tons of fruits trying to go and then he has the most wicked episode of constipation and that was it.
We give Myrolax once a day in the morning and it helps us a lot, it seems to make them go a little smoother and it is less lumpy. It is a powder and you mix it in with their juice.
What do you recommend doing about getting a prescription?
We got ours from our pediatrician who we should have switched from because she knows nothing about SMA and has no time to learn about it but because we have two children and she has been our pediatrician before we just kind of stuck with her and she was the one that gave us this prescription when we said having trouble with constipation. And she is willing to do any thing that is possible to help us.
Did you try any herbs or anything? You know, I have heard that herbs can help with that. Oatmeal and orange juice.
Does anybody use acidophilus? It is in our body, it is actually a natural friendly type bacteria that is in our body. Acidophilus is a supplement that you can take and it helps digestion. My daughter used to be on Myrolax and it worked very well for her but since she has been on acidophilus, she doesn't take Myrolax anymore.
Our doctors would say to go on Antibiotics and then you are killing off good antibodies and all the good germs that you have. They do have a powdered form of acidophilus that you can buy. It does many things for your body. My daughter had troubles with secretions that would build up on her throat and it actually helped get rid of these secretions, it helps with her digestion, and it helps with a lot of things. And there is a lot of information about it and I am not educated enough to explain, I just know that as far as my daughter goes, it has helped with her bowels and it has helped with her secretions. I mean, the big thing is her secretions, because she would, it was not a virus, it was not an infection, it was not a cold, she wouldn't ever run fevers with it, but she would always just have this accumulation of secretions that would sit on her voice box and it would just stay there. And I don't know if any of you know the anatomy of the throat where the trachea and the esophagus is but the voice box is actually inside the trachea and that interferes with breathing, that interferes with the air and all that getting through, and if it was to get overloaded and the child is laying down a lot then this could go into the lungs and you know, there is your pneumonia. My daughter is upright a lot, we don't have to worry about that but by the same token it was always right there on her throat and she coughed, she would cough non-stop. She had this shallow little chuckle cough and she would lose her breath because she couldn't stop coughing. She would wake up in the morning for an hour straight coughing. And my doctor, her neurologist had told me from the get go that SMA children and I am not sure if this is common with all SMA children, but with my daughter it held true that couldn't get rid of her secretions. And he told me that he didn't know what to do about it. I fought with it and I fought with it. It seemed like the cold would trigger the secretions, okay, once she got the secretions, she couldn't cough them up because she didn't have that umph to cough the mucus to get it up off the voice box and get it to go over to the esophagus. There is actually like a ledge that the voice box creates and to get it up over the wall of the trachea it would have to go at least a couple of inches which was too much for her. She just didn't have the strength for that. And she would just always have this cough forever, it would hurt her in eating, she didn't eat very much, cause when it got overloaded it would go over into the esophagus and give her the sensation of being full, plus that cough would interfere with her swallowing issues. But anyway, this acidophilus would naturally say somehow, I can't say it is like an antibiotic, I don't know exactly how it works, but it keeps these secretions under control where the build up is not there and now she never coughs. If she coughs she is sick and I know, I always fought with that too because she is always coughing and I don't want to go rushing with the antibiotics if she not really sick. So now I know, if she coughs we are ready with her nebulizer, and we are ready with her CPT (chest percussion treatment), and we are ready if a fever comes up. Well, now I give her the acidophilus in her Juice+, but that is a whole other issue, but we started out on with the dairy free acidophilus which is kept in the refrigerator. I give her a half a teaspoon. The dosage on it said for ages 4 to adult and I thought she is almost 3 that is pretty close and I will go ahead and give her for an adult. That is the only dosage it gave, but my doctor threw a fit. He threw a fit, but I only gave it to her once and she got the runs from it and he told me to try half of what is recommended on the bottle and that seemed to be a better level for her.
I asked the doctor about the thing about milk, and he seems to think that as long as your child is gaining weight and he does not recommend you keeping the milk away from them.
I took my daughter off milk. My daughter used to drink, I mean she loved milk, you give her a glass and it would be gone and that is where she was getting a lot of her weight gain from cause she loved milk, she loved cheese, she loved meat, she didn't like fruit, she didn't like vegetables, she didn't like any of that stuff, and I heard of this diet that they should be meat free and dairy free and all of that and I am very naive and as far as the diet goes, I just dived right in blinded and everything into it which was bad I regret doing it this way but what I did was I took my daughter off all meat and I took her off all dairy, I am talking everything, even cookies that had dairy in it. And I saw changes in my child, with my own eyes, that day I saw my daughter be able to hold her head up better, I saw my daughter be able to sit up without falling so much. I saw her with my own two eyes be able to raise her hand up and say, "MOM!" I saw my daughter, her endurance, I saw her over a two week period before I got her the amino acids into her, I saw her, just taking her off those complex complete proteins, I saw her advance in all different ways. I saw her mood jump up sky high. I saw so many things and there is no way nobody is ever going to take that away from me because I saw it with my daughter. As far as the professionals go I have heard a lot of them too say well this child does not need that diet and that very well may be true. My daughter is on the weaker type 2, she is very weak and it helped her. It really helped her. I am sorry.
It helps because a lot of these children have trouble absorbing proteins. So without it, some SMA children can show signs of malnutrition. And they can have these symptoms just like that and so you take them off the dairy and we saw the same thing with our child. And she got to where when you pull her arm and she will pull it back and she has got a real good pull now, and she can pull her feet and when she is sitting in the car and reading a book, there is no better reason to change.
My only regret was I took her off meat and milk at the same time and now here a year later I have the question: Is it meat or is it milk? What is the problem? And in the last couple of months I have introduced meat back into her diet and one of the ones who taught me the diet to begin with, kind of yelled at me, they said no you can't do this. They said that their daughter is 12 and they too tried to introduce meat into their diet and on first sight they didn't see any side affects but over the long haul they ended up seeing a lot of the weakness come back and a lot of the secretions come back. I don't know about that but as far as my daughter goes I give her meat now, I do. I give her simple meats like hot dogs, where the protein is not as strong and complex as say when it comes to pork chops or something like that. But yeah, you do one little piece of this diet at a time, that way you can see with your own eyes what is doing what. Everything else other than when I first took her off the meat and dairy, everything else I did one step at a time so I could see with my own eyes that this is helping and believe the changes that it has made and I tell you what, to get that first hug from my daughter that I actually felt those little arms squeezing me. And when she hugs you and you feel that warmth and you feel that energy coming from your child and we always have our child's arms lying around us but it is not the same as feeling that tug.
We were wondering about our daughter because the doctor is no help. She may be teething and I had a cold last week, but she has been coughing for 10 days. You know and since I had a cold last week, I thought I had given it to her and with her teething.
Is she running a fever?
No. Not at all.
Cause that is what my doctor says, because I fought with my doctor and I said you know I keep bringing her to you and you keep telling me that there is nothing wrong with her and I am like, I am at home, how am I supposed to know, when do I need to come to you and when do I not need to come to you. And he said to watch for the fever. If it is a cold, if it is a virus, an infection, or anything that you need to worry about there is going to be a fever involved.
Back to the diet, like I said I just dove right in and for two weeks she didn't have any source of protein. But then I hired a dietician and she helped me organize all the importance of all the different vitamins and minerals and the protein and such. We found a product locally called SRB that had all the different amino acids in it; we went ahead and started giving her that. We gave her multivitamin, and the dietician helped balance out the diet where we started giving her fruits and vegetables and I even resorted to giving her baby food because that was what she would take, and then we worked through the doctors informing them and teaching the doctors about what this is doing for my daughter and we got the prescription for Tolerex. And now she is on Tolerex and that is how she gets all of her protein or amino acids.
One product that we found that we use from Novartis called Resource. It has got all the minerals. I think it is 200% of what they need and she just downs that stuff. She will drink it at night and I have read, you know how if your kid is not getting enough vitamins and nutrients, that their muscles are getting more damaged and I think that as strong as she is, cause she wakes up in the middle of the night and downs one and it is like her body is not getting worn down. Also, she is like a vegetarian not by our choice but her choice and I think her body is telling her, "I don't want to eat this." But we are not saying you can't eat it but she just doesn't want to anyway. It is called Resource by Novartis and we order 5 cases at a time. Where do you get that from? We buy direct. I would just try it.
My daughter is the same way. She made herself a vegetarian. Well, my daughter will only eat chicken McNuggets at MacDonalds, french fries. That is a big reason to use Resource; she eats McNuggets, french fries and sprite. And you know we buy the stuff for her, okay whatever, you know if you're going to eat it. Resource comes in an orange flavor and she will drink 2 or 3 of those a day. That works for us.
My daughter can drink whatever she wants, I am just happy if she is get any amount of food and that makes me thrilled, it doesn't make me thrilled that is still turns out to be junk food that she is eating but she is eating something and I thank god. I would like to know that she likes this and she is getting her nutritional needs because these kids have so many other issues that you don't want stress out with out.
That is the worst part of the diet, the amino acids supplements, Tolerex, Vivonex, all that stuff is nasty. It is very, very hard to get children that are not tube fed, I mean those who have a G-tube have got it made. But those that are not tube fed, it is really hard to get the child to take it and you feel bad because they have got all these other medical issues and then you feel like you need to give this to them and they just really fight you with it.
I had read about the amino acid diet and noticed that it seemed to be a major trend with the SMA children and thought it would be worth it for me to try it. I have noticed a difference with my daughter. I have been noticing stuff.
Have any of you found that your children love cheese? And then you get to the constipation issue…
My daughter is 2 and ½ and loves meat. I mean she eats meat and more meat.
How do you give your daughter the Resource?
We just put it into a sippy cup, lay it by her bed and then she drinks it when she wants to. This is not something we initiated. This is something that she did on her own and I am thinking that her body is telling her this was what it needs.
I believe that it was Dr. Richard Kelley who started talking about the cornstarch. You give your child cornstarch before bed and that is supposed to help slow down the metabolizing through the night so that way you don't have to wake them up in the middle of the night and then you don't have to worry about them going into that state of ketosis or fasting, because they will. Their body runs out of that protein in their muscles and they start breaking down their own muscles and using that for energy. And I use the cornstarch. I give my daughter a tablespoon every night before she goes to bed. She has lab work drawn, and so far all of her lab work has come out fine. All of her levels and everything are staying stable. I just mix it in with her formula at night. You can mix it with anything you want. You can put it in food. I put it in baby food for a while, bananas seemed to hide the taste pretty well. It doesn't really taste that bad it is just kinda tart, kind of chalky like. And what I don't have on top of my head but I have in my files is that you give the cornstarch to them according to their body weight.
Has anybody tried any of the tofu products and what do you think about?
I don't know if it is tofu or not, but it is sold in the same section at the grocery store like the veggie hot dogs and the veggie burgers. Is that the same thing? I did that and that was fine. I didn't have no problems with it other than the taste. Some of it my daughter rejected because of the taste. Like the veggie burger meat that I could actually fry up and make spaghetti with and it kind of smelt pretty bad.
I was wondering if anybody else ever noticed their child's breathe because our child's breathe always seems to be so strong.
One doctor I talked to said that the fruit smell of your urine and your breathe, and I am guessing that that is a fruity smell, I would think, and it just means that they are fasting, low on vitamins and minerals especially amino acids.
The cornstarch would be something that would be good for that.
I would like to just make a comment on the night sweats and hear from all of you as to what it is all about and if any of you have found anything to help with that.
My daughter doesn't sweat at all anymore.
Then there is the pooping machine, the stander. Every time you put them to stand they poop.
My daughter had her tonsils removed and her adenoids and we are in the other room and now they feel much tighter and they sleep so much better.
We have an in/Exsufflator and we haven't had to use it once. We use it because our kids with pneumonia it helps.
Do you use it when he is healthy too?
Yeah, just anytime he seems a little bit rattly.
We switched from a neurologist to a pulmonologist.
You don't use a neurologist at all anymore?