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When there is a decision to make on whether the G-tube (gastrostomy tube) is an option for a child with MSUD, the views are quite polarized. Here is my pole.

Part of my life was given back to me when I discovered the G-tube, and it did not take me long to discover it. My son was diagnosed with a variant type of MSUD when he was one month short of four years of age. He had unmistakable symptoms before diagnosis, but regretfully, his physician did not interpret them correctly. Because of this, Oliver has some brain damage. Other than the shock of having a permanently brain damaged child, the rest of the new circumstances were not a huge problem for me since I have a science background.

The diet did not cause huge problems - Oliver liked French fries anyway. Getting him to take the formula was a problem. We spent 3 weeks with him in the hospital while his leucine tolerance was being tested. During this time he sported an NG-tube. Having had the taste of regular food for four years, there was no way - absolutely no way - with or without a flavoring agent, to get Oliver to drink the formula. I was sent home with an NG-tube and instructions on how to change it. Every 4 to 6 hours Oliver had to have some of his formula gravity fed through the tube. Well, that certainly took care of the uninterrupted nights I had hoped for.

Changing the tube by myself was a complete impossibility. We had to go to the hospital where they put Oliver under heavy sedation. I remember one time when they tried using just a muscle relaxant. I can still hear his muffled screams. It took 2 nurses to hold him down and he was bleeding profusely out of the nostrils. In a very short time, three things became abundantly clear: 1) Oliver would never drink the solution 2) I was wearing out rapidly 3) Neither one of us would tolerate any more NG-tube changes. I simply could not do this to a small child.

The G-tube was my salvation. Two months after the initial diagnosis, Oliver was scheduled for surgery. The procedure was very short, 15 minutes, with essentially no discomfort afterwards. The pump was not very strong, so often when Oliver rolled over in his bed, he would squeeze the hose, and the pump would sound an alarm. However, Oliver usually slept quietly, and I could sleep until the "dose done" alarm forced me out of bed.

A miracle of technology, my latest pump shuts itself off and has a stronger mechanism, so I rarely get "no-flow" alarms. It is trouble free. I take Oliver to the bathroom before I plug him in and essentially forget about him for the rest of the night.

We are now working or our third kind of "button," the Entristar. [A gastrostomy feeding button is the device inserted in the stoma, an opening to the stomach.] It is relatively new, and about three times a year the Children's hospital phones to find out if we have any problems. There are none. It functions perfectly.

Oliver can do any type of sports he would do otherwise (other than when we tried to do rolls on the bars; but he did not like that anyway). When he gets involved in mud castles at the beach, I usually put an old shirt on him to protect the tube, but then I would prefer having a shirt on him anyway when he is out in the sun. When the kids ask him what "that thing" is, he tells them that he gets some food into his tummy this way, and the kids usually say "cool" or something like that.

Oliver is a very stable, healthy child and is seldom sick. He has not missed a day of school in 18 months. However, when he does get sick, the G-tube is really handy. When he needs those calories most, I usually get them in. I pop him in front of the TV and either drip in MSUD formula or my sick day formula of electrolytes and polycose. I think this has been a significant factor in helping him get over minor illnesses fast and has prevented that spiral into something more problematic.

We go on vacation with Oliver and his pump. For air travel, we borrow a lighter travel pump from the hospital. We mounted a bracket in the camper and run the pump off the batteries if there is no electrical hook-up. If all else fails (for example, the pump broke when we were in Mexico) I simply syringe it in.

I would prefer that Oliver drank the formula. No doubt about that, but I had to pick my battles. I have huge battles with him every day: to get him to eat while taking Ritalin, to actually have both gloves and his backpack on him before he gets into the car, to do ninety minutes of homework after school, to sit straight and to close his mouth while eating, etc. There is no room in the day to fight another battle. Ultimately, the important thing is that I get the formula into him, all of it, with minimal fuss. I am pretty sure that one day he will get rid of the G-tube. (Picture Oliver on his wedding night, "Honeybunch, would you please plug me in?") The other MSUD patient in our province just got rid of her G-tube now. She is 16 and she is dating. When we decide that Oliver does not need it any more, the stoma can be closed off with two stitches.

For me, the G-tube is the perfect solution. I have not heard any unfavorable comments from parents whose children have one. It is worth looking into if your formula battles are taking up too much of your time. The beauty is that it is not permanent.


Hailey Wiggin

It is with a deep sense of sadness that we announce the death of Hailey Wiggin, 3 ½ year old daughter of Jason and Aimee Wiggin of Taunton, Massachusetts. Hailey had undergone a partial liver transplant surgery at Massachusetts General Hospital in Boston in an attempt to cure her MSUD. Her father was the donor for the liver. The surgery was performed on December 17, 2002, but severe complications set in, and Hailey died in the early hours of January 2, 2003 at the hospital. Our thoughts are with the family at this difficult time. They have our prayers and deepest sympathy.

CHANGE THE LIVES OF MSUD ADULTS AND CHILDREN

The MSUD Family Support Group is currently funding several research projects and we are proactively looking for researchers interested in developing new treatments or finding a cure for MSUD. Significant funding is necessary if we are to accomplish this goal.
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