Don't fear the tube
My son has an ultra-rare genetic disorder called Barth syndrome that, among many other things, affects his desire to eat. When he was new born, he nursed well and gained weight. But as he got older, we noticed that he would eat less and simply lose interest. We thought at first that his heart function being low was preventing him from get a good nurse and switched him to bottle feeds. But it soon became clear that he was just not interested in eating. As his doctors continued to increase the calorie count of his bottles, he would eat less and less. He was still gaining weight but the rate of gain was decreasing. Then the feeding tube was brought up. My husband and I were mortified. We had already been through so much just getting to his diagnosis... the thought of surgery was just not acceptable. We were assured that the tube was a kind of "last resort" option and he was not there yet. At the time, we were relieved, but looking back I can see how making the tube a "last resort" made the decision all that much more stressful when that time finally came. We had basically been told there was a reason to fear the tube.
We were asked to start him on solid foods even though he was still only about four months old. He needs more calories, they kept telling us. He refused rice and oatmeal... he would gag and throw up when offered purées... Feeding times were becoming extremely stressful as we tried harder and harder to make him eat or drink... knowing that if we could not, hospitalization and the dreaded feeding tube were lurking right around the corner.
Then it happened. The day I had been fearing for three months. What was worse was that this was one of the very few appointments where I was alone. My little boy had lost six ounces in only two weeks. He was being admitted for failure to thrive. He had been throwing up his morning feed the past few days and only that morning I also noticed his breath smelled bad. It turned out that the bad breath was due to him being severely under-caloried.
We spent the first few days in hospital struggling with over-zealous interns. My son's nutritionist was well aware of the different dietary needs of Barth syndrome boys but the interns just saw a new puzzle that needed solving. They were convinced he had been throwing up because he had a milk allergy. I felt that to be a little strange as he had been eating yogurt for two months but only throwing up for a week. Then they suggested a breast milk allergy and, after the scene I created, were more than a little terrified of me for a day or two. The nutritionist backed me up, finally putting the interns in touch with a Barth syndrome specialist... one ultra-rare disorder was enough for this poor kid, let's not add a second one with a breast milk allergy. But it still left the mystery as to why he was losing so much weight. We had to have the feeding tube discussion again.
Again, we were terrified by the prospect of the surgery and his needing to live with this plastic protrusion. But we were given that little out when they said we needed to wait until he was fully rehydrated and eating a little on his own again... maybe he had just been on this dehydration downward spiral... maybe he would not need the tube after all!
Only one specialist along the way had tried to reassure us that a tube was no big deal... that some parents were actually happier after the tube had been placed. But it only happened once and so was completely lost on our anxiety-riden minds.
After ten days with IV and nasal-gastric (NG) feedings, it was evident that my son was not going to eat enough on his own to support his growth. It was either stay in the hospital or get a feeding tube. My husband and I were beside ourselves. Barth syndrome being a metabolic disease, there were additional dangers to anesthesia. He also suffers from neutropenia, meaning his body doesn't produce enough of the neutrophils necessary to fight off infections. But it was the tube that we were fearing the most. Would he ever eat on his own if he could get all his calories through a tube? Would he be able to go back to day care? What would happen if he pulled it out? Could it get infected on the inside? Could he go in water? We had been told by someone along the way that immersion was not allowed for at least three months! How would he get a bath?
The GI specialist came in to talk to us. She was a bubbly, caring woman with a perpetual smile. She explained how he would be intubated and she would use an endoscope to find the best placement. The endoscope would shine a light through the wall of his stomach so that she could be sure that the placement would not impact any other organs. She would make the incision such that it would be just like a pierced ear... a small, short tract. The "peg" tube would be placed from the inside. It is the best for initial placement because it has a flat disk on the inside to prevent the child from pulling it out. Just like a new earring, it should be turned every so often to prevent scabbing. He should be bathed about 24-36 hours after placement and then once a day for the first week or two. Full immersion baths were perfectly fine. After three months, he could get a mic-key: a flat button-style tube that an extension is hooked to.
Already feeling a little better, she then sealed the deal by saying, "And the best part is, feeding times don't have to be stressful! He can eat what he wants and if it's not enough, you put the balance down the tube! Same with medications - you won't have to force him to take it if he doesn't want to but you won't be concerned that he's not getting what he needs."
Seeing our seven month-old off to surgery was not easy. But the hospital actually found an anesetheician who had heard of Barth syndrome and was aware of which medications could cause adverse reactions. The hematologist stepped in to make sure his neutrophil count was at an acceptable level prior to surgery and also recommended a prophylactic antibiotic, just to be sure.
The result was almost immediate. He was so happy to not have a tube down his nose and an IV in his hand (which, of course, also meant a foam board and fabric cast to keep him from pulling it out). The stress was gone. Over the weeks and months following the surgery he started to eat more. Feeding time was no longer a struggle. He still requires tube-feeds but is taking more and more of his daily calories by mouth. Part of Barth syndrome is a lack of fat reserves so he cannot go for extended periods without eating. He is always on the cusp of not having enough food and fluid. The tube has allowed us to feed him over night while letting him sleep - thus allowing his natural developmental progression while preventing an overnight fast. He's becoming a toddler and like any other kid, does not enjoy taking medication. He requires 12 oral doses of medications every day. We always offer them by mouth first but if he refuses or starts to spit one out, we can just put it through the tube. If he gets sick, we can keep him hydrated and so the threat of hospitalization has been greatly diminished.
Finally, the memory of what that one person said, somewhere along the line, came back to us... "Some parents are actually happier once the tube is placed."
Yes. We are. This is our success story. I wish we could have heard one before we went through the process. It would have been so helpful to know that we did not need to fear the tube.
1. "Clean, but not sterile." is the mantra to live by. You do not need to use sterile water to flush the tube, nor to fill the balloon of the button. If you can drink it, you can use it.
2. The Peg tube was quite long and we discovered that it was easiest to leave just a little slack, tuck the tube under one tab of his diaper and then tuck the very end under the other diaper tab. For feeds, we could just untuck the very end and feed it down his pant leg. This kept the tube secure so that it would not get caught on clothes nor did he pull at it.
3. For night feeds with the button-style tube, attach the extension and leave it on over night. We leave it hanging out between the bottom snaps of his pajamas. For months, I avoided all zip-up pajamas until I realized I could just snip a small hole above one of his hips and the tube sticks out that!
4. Only put fluids down the tube and always flush it with fresh water after medicine or formula.
5. At some point, a button-style tube will fall or be pulled out. That sounds scary but it is really not a big deal. However, it does need to be put back in as soon as possible and should never be left out for more than 15 minutes. Keep a water-based lubricant on hand to put it back in. It was very weird to buy for my baby, but the basic, no-frills, KY Jelly is the right stuff and is inexpensive and easy to get.
6. To help prevent that unexpected tube replacement, check the water level in the balloon every two to three weeks. Your GI specialist will let you know how much should be there - if it he level is low, add more water (and remember, clean, not sterile!).