With such a unique disease we have some unique challenges to
deal with. For the last week we’ve been struggling with a new problem:
First a little background. In addition to diabetes, not
having a pancreas means Finlay can’t produce the enzymes he needs to digest his
food. We have to give him his enzymes with each meal. During the day we use
Creon, small spheres of enzyme that we mix with his food. At night, when we
feed him through his gastrostomy tube straight into his stomach we use Pancrex,
a powdered form of enzymes that we dissolve in water and pass through his tube.
This has been working pretty well and has helped us keep his
blood sugars steady(ish) and him as healthy as possible.
But now that has all changed. Pancrex, the drug we rely on
for him to digest his feeds at night, has been discontinued.
So how can we give him enzymes during the night? The only
drug available to us is the Creon that he takes orally during the day.
Option 1: Wake him up
and give him some Creon capsules.
This worked the first night. We woke him up and he sleepily
did as he was told, opened up and swallowed the enzymes mixed with some apple
puree. The poor wee man didn’t know what
was going on. By the next night he had worked out this was going to be a
regular occurrence and he wasn’t happy. Tired, angry, crying and in no mood to
start eating anything. A ten minute struggle to get him to eventually swallow
his food was enough to convince us this was not a long term solution.
Option 2: Pushing the
small spheres through his gastrostomy tube.
Our dietitian found a research paper that suggested we could
mix the spheres in thickened water and push them through the tube. The photo
shows our result. The spheres just clumped together at the end of the tube.
Assuming this was the spheres not getting through the valve at the start of his
gastrostomy button we put on an unblocked tube and flushed with water. But the
water wouldn’t budge. It turned out some spheres had got through the valve and
become blocked in the gastrostomy button, the bit of tubing that sits in his
abdomen wall linking the feeding tube to his stomach. I had no option but to
change the gastrostomy. Luckily with all the fuss, he had woken up so I took
out the button, got him to place some
cloth over the opening to stop his stomach contents spilling out onto the bed (which is quite impressive for a tired, sleepy 3yo),
and quickly got his spare gastrostomy out of the box, lubricated it and
squeezed it through the hole into his stomach and inflated the balloon with
water to hold it in place. Not a successful evening!!
Option 3: Crush the
spheres with a mortar and pestle, mix them with water and stick them down the
tube.
This sounds simple enough but here we were outdone by the
clever people who designed the spheres. The enzymes are contained in these
little spheres which are all crammed into a capsule. When the capsule is
swallowed it dissolves in the acid of the stomach releasing the
spheres. Now this is the clever bit. The spheres have an enteric coating that
protects the enzymes through the stomach acid, then when they are passed into the
duodenum (into where the pancreas would normally release the enzymes), the
alkaline environment dissolves the enteric coating allowing the enzymes to
digest the food, mimicking closely what would happen in a normal person.
But when you crush these spheres, the enteric coating
becomes separated from the enzymes. Then when you add water and try pushing
through the gastrostomy, the coating from hundreds of little spheres all
coagulates together and forms a thick blockage in the tube. Failed again!
Some of the enteric coating after I picked it out of the end of tube with a needle
Knowing that the enteric coating dissolves in alkali we
unblocked the feeding tube by passing some dissolved baking soda through the
tube but this was another frustrating evening.
Option 4: So after many emails between us, the hospital, the
pharmacist, and apparently other hospitals in both New Zealand and Australia we
think we have a solution. We are crushing the spheres in bicarbonate (baking
soda) to dissolve the coating then passing through his tube, flushing with
plenty of water. It seems to be working. So far.
Another unique week of unique challenges for a unique child.
