Opinions from Parents,
Caregivers, and Doctors
|Disclaimer: The following testimonials and suggestions
are not intended to replace the advice of your child's physician.
The personal stories and medical suggestions are to supplement the
information you may have received from your child's physician.
Please consult your individual physician before proceeding with any
If you have any questions or wish to reuse any of the following information, please contact Jeanna H, Elizabeth's grandma, via e-mail at Red542000@aol.com.
Elizabeth starting losing her swallow after Strept throat soon after our first trip to Stanford. She got really ill and would gag when eating or refused eat at all.
Our doctor's nurse told me " There is nothing more doctor can do for Elizabeth" . Their opinions have since changed because of how well Elizabeth has been doing for the past 3 years.
I needed help figuring all this out . I was so upset watching Elizabeth lose weight and she could not sit up hardly at all or she would choke. I was emailing Laura Stants (SMA Support) almost every day and she told me about Dr. Schroth. Dr. Schroth called me with in minutes of my email to her and the next week we were on our way to UW. I also talked to another parent that had just lost a baby to SMA . She told me to get the nissen/g-tube as soon as possible because we could save Elizabeth's life.
After our first visit to Dr. Schroth and a swallow study was done we found out Elizabeth was silently aspirating on liquids. Dr. Schroth explained what we needed to do and about the nissen. She recommended this procedure and explained why. I believe that if we would not of done the surgery when we did Elizabeth may not be here today. I believe Dr. Schroth help save Elizabeth's life. If Elizabeth would of been diagnosed sooner we would of gotten the surgery sooner.
The nissen makes it so if Lizzy has been eating and has issues and needs a cough the chances of her aspirating are not likely. She also can have treatments around the clock every two hours if needed also if she is sick with respiratory illnesses.
I do vent Elizabeth while eating because she is not able to throw up. The venting helps if her stomach is upset and she can blow the food up her tube to the 60ccs syringe. I noticed venting works better with Elizabeth. Not all kids need to be vented but Lizzy does.
The nissen is a choice that some parents/doctors do not agree with with but having lived with a child with SMA for over the past few years and the many respiratory illnesses she has faced and hearing how many other children with SMA have passed away from Aspiration Pneumonia that did not have the nissen then I highly recommend the surgery at the same time as the g-tube.
I believe getting it all done at once saves having to have another surgery down the road.
This is only my opinion from my experiences with Elizabeth.
We flew out to see Dr. Bach when Charlie was 10 months old. He was still nursing as well as eating baby food. As we were walking out the door to leave, Dr. Bach said "oh yea, you really need to consider getting a g-tube soon". John and I said Thank you and left to catch our flight home. We were sitting in the cab laughing to ourselves because Charlie was so strong and eating so well. Why in the world would we even think about a g-tube. Well that was in June, and funny thing, by October after listening to so many other parents, Charlie was getting his G-tube and Nissen even though he was strong and still eating.
We talked to so many parents about the "one thing they wish they had done differently" and almost all of them said that they wished they had gotten the g-tube proactively while their child was strong and healthy. It convinced us. Why wouldn't we want Charlie to have the surgery that was most likely going to be inevitable while he was strong and healthy. Why wait until he was sick and losing weight and the surgery would be potentially harder on him. It was the best decision we made. He had the procedure done in October and he continued to eat until the following June when it became too hard. But the best part was that we were able to use the g-tube overnight to supplement him. We didn't have to worry about force feeding him enough orally because we had the g-tube to fall back on. It was the best decision we made.
At 6 weeks of age, Kyle James was diagnosed with sma type 1. By 4 months of age, he had lost 2 lbs and wouldn't eat at all. We were at my mother in laws and had suctioned Kyle when I looked at him and he was grey and blue. We rushed him to our little ER and he was satting 85. He was transported to a bigger hospital and put on oxygen, which is a no no! Kyle had aspiration pneumonia. Two weeks later, he went into surgery for his gtube and nissen fundoplication. He did well and the the next morning he was extubated without bipap. He lasted 6 hrs and the crashed big time. He just struggled the whole night in NIV and finally was intubated. Our doctor called us in the room and told us Kyle had a blood infection and might not make it. Well, Kyle did pull through, but he had numerous extubations that failed, he almost died from them and just got weaker. We finally transferred to another hospital where he spent another month just recovering from his illnesses. So, just for a much needed gtube, Kyle spent 2 months in the hospital, had a blood infection, had 2 blood transfusions and coded about 4 times. If we had just had the surgery done BEFORE he needed it, we could have avoided the traumatic experience it was. Who knows what it did to Kyle, physically and emotionally. Please, a gtube is one of the most imporant things for our children.
Dr. Bach's team use to recommend having it done very early - while the child is healthy...lately he's seems to have gotten away from that a bit.
Personally for Connor - he lost his swallow at 10 weeks - he already has his g-tube scheduled for around 14 weeks - so we had to bump it up early. He had an n-gtube for a week or so and I can tell you, that thing went up and down in his nose because of the belly breathing.
Working with families over the years (coming up on 6) I can tell you the kids that have the g-tube and nissen done early while healthy seem to do much better that first year without infections and such. I have seen kids end up near death just due to one reflux that quickly ends up as pneumonia. As we know as the child is losing their swallow, it can go quickly or over the course of weeks. In that course of weeks situation, silent aspirations could be happening.
I also think the swallow study is a waste of time. 99% of all type 1's lose the swallow - or have a swallow that is diminished - so why bother with the study? It's just a snap shot in time and could be done at a time when reflux or swallow issue isn't happening at that exact second.
Jimmy was born 3-18-02
I assume the reason surgery is
recommended as soon as the diagnosis of SMA is made is that such
surgery will almost always be needed at some point, and by the
time that G-tube feedings become essential for nutritional
purposes, the children are necessarily weakened to the point
that the surgery carries much greater risk. I am aware that
some specialists do not feel a Nissen is required for every
G-tube. However, because I do not take care of children with
SMA, and because making decisions about the need for G-tubes and
Nissens is far from my area of expertise, it would be better to
let others guide your thinking on the issue.
Veronica was diagnosed at 5 months. We were concerned because she was considered hypotonic and not meeting developmental milestones so after a series of events she received the diagnosis of SMA. By a stroke of luck we were put in contact with Dr. Swoboda and her staff at Primary Children's Center in Utah. Going to Utah was the best and most important decision we made early in her diagnosis.
Dr. Swoboda kept stressing to us the importance of being proactive with everything, from nutrition to respiratory issues. She suggested the need for a g-tube right away and explained the reasoning was due to many times the rapid onset of the loss of swallow. If you were prepared with the g-tube then your child would not lose anything when their swallow goes. It is one of those inevitable things about a Type 1 diagnosis, your child will no doubt lose his/her swallow, when that happens is different for each child. If you are proactive with the decision your child won't lose weight waiting to have surgery, especially if the swallow is lost overnight as it seems happened with us. Doing it while they are strong makes sense as well. If a child is nutritionally weak due to poor eating prior to surgery their chances of making it through the surgery are greatly diminished.
I was also very concerned about aspiration as it became clear that Veronica was losing her swallow. It wasn't worth it to me to keep trying to feed her orally when it turned into a quick trip to the cough assist machine to get the stuff out of her lungs. It was then that the decision to be proactive became even more logical and sound.
Veronica is a little over 3 years old now. She lost her swallow when she was 8 months old and had her g-tube for 2 months prior to that happening. She has gained and thrived and been very healthy ever since.
Jacob was born 6-14-06. He entered the trial at Stanford in October
2006 and was first weighed by Dr. Wang. When we returned to
Stanford in November and he was weighed again we noticed he had only
gained 1/4 of a pound, which Dr. Wang felt was too little at his
growth stage, so we proactively got him a g-tube placed at
Thanksgiving 2006. He had not lost his swallow, but soon after
placement we noticed he gagged quite a bit while eating and so he
went to strictly g-tube feedings in December 2006. Jacob has never
had an issue with gaining weight. We have had a couple of months
where we didn't gain weight, but he has never lost weight, even
during illnesses. I attribute his nice, steady weight gain to the
g-tube and the breastmilk I have in his diet. When Jacob was 13
months old we switched from a breastmilk/baby food mix, to an AA
diet which includes breastmilk. We have tried both Vivonex and
Tolerex and find that Tolerex seems to work best for Jacob.
We weren't given much info. at all on the subject of G-tubes from Dr. Darras at Children's Hospital back in June of 2004, when she was diagnosed. When we asked about options for her comfort and nutrition, he simply replied "Take her home and love her. Enjoy the time you have with her because it won't be too long". Then he said that we could get her a NJ tube or the NG tube for the time being.....hah.... Didn't she fool him!!! So unfortunately, it wasn't until she was hospitalized at five months that we got the NJ tube put in because she was then aspirating. She was still breast feeding and I felt she was strong enough with her swallowing that she didn't need it until then.
However, my advice to new families would be to go ahead and do it as soon as they see signs of any weakness, because Cassandra probably was aspirating silently before that hospitalization. It was the cause of that first hospitalization, so I feel she would have avoided that plugging and aspiration episode, if we had known about getting her the G-tube and Nissan surgeries.
Cassie had the NJ tube until November, when it got clogged up and had been giving her much discomfort from all the moving in and out of her nose. When we went to have it changed out, they put the NJ tube up to high into her stomach and she had severe reflux, which came up and almost killed her. After she again was hospilized from this episode of severe reflux and aspiration, we opted for the G-tube and Nissan surgery. Thanks to Karen R, Kim M and Amy B who basically walked me through which surgeries to have because I once again didn't have the info. and almost got the GJ tube without the Nissan surgery, which would have been a big mistake for a child, who almost died from a sudden severe reflux episode.
I believe that the Nissan is absolutely necessary because reflux can happen at any time and also it will help with vomiting during illness. The G-tube has been a God sent because Cassie is 3.5 years old and weighs 31 pounds, while being 46 inches long!! It has kept her growing and very healthy!
My experience with children with SMA type I is that they will lose their ability to safely swallow generally sometime during the first year of life. Which means that they will need an alternative way to be fed. I recommend a gastrostomy tube with a laparoscopic Nissen fundoplication. Doing a gastrostomy tube without a fundoplication, places the child at risk for aspiration if they vomit. So, I have learned that the Nissen fundoplication is an important component of protecting the lungs and optimizing feedings and should be done with the gastrostomy tube placement.
The issue of when to do the gastrostomy and Nissen fundoplication is more difficult. Right after diagnosis, families are grieving. Some families withdraw from the reality of their child having SMA and need time. Other families research as much as they can to learn everything there is to learn about SMA so that they can make the best informed decision possible about the care of their child. Both are fine approaches. Some families also choose to have surgery before we get into the winter months. Some families need to see that their child is having some swallowing problems before they can consider a surgery. So, my point is that there is not one answer or one perfect time. However, if the child is having difficulty swallowing, has had an aspiration pneumonia or is not gaining weight well, the gastrostomy tube with the fundoplication should be performed very soon and the child should not be fed orally.
Dr. Mary Schroth
Chase has SMA, type 1. He has a G-Tube/Mic-Key Button & Nissen. He was diagnosed at 7 mos. old and within a week of that, we found out that he was silently aspirating some (no all) of his food. Chase did have some reflux at times, but there was no indication of aspiration, that we could tell, prior to the swallow test. an NJ-Tube was immediately put in for feeding (the tubing that goes down the nose) until we could get the surgery done (approx. 3 weeks later). I know it was necessary, but the NJ-Tube was a big neusance. The tape on his face caused irritation. Positioning and moving him had to be done carefully so the tubing did not get jerked out. It looked uncomfortable for him, and it got clogged at one point, which led to a disastrous night at the ER, with several nurses trying to give him an IV because the folks that insert NJ-Tubes are not in at that time of the night, so we had to stay. Inserting the IV took several hours, since he's a hard stick and we were both crying with exaustion... it was not fun! At this point in time, I was wishing that we could have gotten the surgery sooner!
Once the G-Tube/Mic-Key Button and Nissen surgery was done, things went a lot smoother. Chase no longer has reflux. Feeding is a lot easier. We do bolus during the day and continuous at night. He tolerates it fine with no problems. Our balloon of water (that holds the button in his tummy) did bust once (8 mos. after inserted). I didn't even realize it was busted, until it got caught on my sweater when I was holding him. It came part of the way out, but I quickly pushed it back in (knowing that the hole can close up quickly). I then called the doctor. A GI nurse at Children's inserted a new one and instructed us how to do it. The ordeal wasn't bad at all. Then we replaced it again at 6 mos. I was concerned about pain, but Chase was laughing the whole time, during the placement, so that made me feel good to know it was not hurting. The manufacturer says to replace every 6 mos., but our doctors say to leave it alone unless you are having problems, but they do suggest checking the balloon every few months to make sure the proper amount of water stays in the balloon.
In summary, we are extremely happy with Chase's G-Tube/Mic-Key Button & Nissen. And we definitely prefer it over the NJ-Tube!