Digesting the Problem

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.