Not being able to eat safely is a big problem for children with Niemann Pick Disease. As the disease takes it's toll, the muscles weaken which makes swallowing difficult. Eventually, food and liquids start entering the lungs and causing pneumonia.
There are various stages of swallowing difficulty. Some people can do well with small pieces, others need pureed foods (like baby food) and others can do thickened liquids but not thin (like milkshakes but not water).
And then there are those who can't do any of those.
I saw this article in the New York Times: Food and the Dying Patient and it immediately brought back the feelings and emotions we had with Dan.
We knew that Dan's calorie intake had gone from 1000 calories a day to 400-500. He constantly had a cough and had trouble swallowing. But we didn't have any sort of diagnosis.
We had one swallow study that said to watch him because when he stuffs food is when he has problems with aspiration (getting food or liquid in his lungs).
But when we went to Boston, his situation had changed. He seemed to be aspirating more. So they inserted an NG tube to rehydrate him and stabilize his weight. Due to the low calorie intake his heart rate was quite low.
Taken in the Garden at Boston Children's Hospital
We were okay with that, as we knew that was a possibility. The doctors then started talking about putting in a G-tube, which we kept pushing off until we had a diagnosis.
Once we received the NPC diagnosis, it was pretty much decided that we weren't allowed to leave the hospital unless we put a G-tube in. We weren't comfortable with that decision, but wanted to get home, so we had them do the surgery.
For us, it was the worse decision we ever made.
They delayed the surgery on his scheduled day until late in the afternoon (from early morning). I understand it's a hospital and things come up, but he couldn't have ANYTHING, including water, since midnight. After finally begging to give him some food, we got a reconfirmed surgery time so the food could digest and leave his system.
Then on the day we were supposed to go home, he had a reaction to the antibiotic they were giving him. The doctors insisted it was a surgery complication and brought him back into the Operating Room. Nothing was found in surgery and once the antibiotic was stopped, his heart rate stabilized and his fever dropped.
When we got home, we had trouble getting formula and supplies because it was the 4th of July holiday weekend. After several threats of making the supply company pay for the hospital visit because we needed to feed him, we got some food.
Then he had a reaction to the first "food" (canned formula) they gave him. Had trouble getting a new prescription even though he was breaking out in hives and spitting it back at us. Then we couldn't get it delivered again.
When we finally got all that straightened out, they kept increasing his calories back to about 1000-1200 a day. Yes, I understand that he was very underweight, but to go from 400 to 1200 calories in a few weeks wasn't healthy. And the dieticians wouldn't listen to us as parents.
At home in October 2011
The picture above has a PEG tube, which is a temporary G-tube. A few days after this picture we had that tube replaced with a Mic-key G-tube.
These pictures were taken 4 months apart. In the June photo, Dan was hovering around the 15% percentile in weight, which is about where he always hovered. In the October photo, he was about at the 50% percentile in weight.
The addition of the G-tube significantly increased his calories, but at the same time deprived him of the social aspect of eating and actually tasting food. Between the calorie increase, and the lack of eating, we think Dan got depressed. The significant increase in calories, which his body couldn't handle, and the quick increase in NPC symptoms due to this "shock" to his system, I'm sure hastened his decline.
Of course, everyone is different, and every decision you need to make is different. For some, a G-tube is a wonderful addition. For us it wasn't.