Genetics

We have a few appointments this week, the most important being at Sick Kids with the Lung Specialist on Friday. Last week we did not think we would make it until this Friday and thought we would be seeing him back in hospital by the time the weekend came around. On Sunday we did a major change to his feeding and this seems to be helping.

We had been working James up to bolus feeds (meaning he'd be fed "meals" instead of continiously around the clock). The most obvious advantage to this would be he wouldn't be hooked up all the time. The other reason we preferred this is because it's a more normal way for the stomach to function, as well this would mean we wouldn't be feeding him at night, which his body does not seem tolerate all that well. We were at the point where James was getting 120ml/hr, which is closer to James' old bolus rate from before his hospitalization ... but James started vomitting more often. Slowly he increase to three vomits per day, each vomit still started with gagging and throwing up phlegm, but then proceeded to throwing up formula as well. We were measuring 110-120ml each time he threw up. So here he was losing 200-300ml each day of the precious food we were putting into him. This on top of his 6-8 diarrhea diapers. And to make matters worse we discovered that our feeding pump was completely inaccurate all this time, meaning James could be losing anywhere from 100-250 ml of feed each day that way. So it's no wonder we were seeing our boy waste away.

When being fed at such a slow rate his body should be digesting the food about as fast as he gets it, but each vomit showed that he had at least 1 hours worth of feed in his stomach, probably more since he never reached the dry heaving stage. This concerned us and made us think that were were over-pumping him - what he used to tolerate he no longer can. So we closed the idea of bolus feeds and dropped him back down to slow 65 ml/hr continous 24 hour feeding. Day #1 - 3 vomits but only about 10-15 ml each; Day #2 - no vomits; Day #3 - 1 vomit of 30ml. Day #1 - wow ... new kids, the shine in his eyes the happiness, playing and chipper again; Day #2 - not quite as energetic, but still much better; Day #3 - not as bad as before, but not the greatest day (other factors also). Whatever the case we know that this is the better way to go. This unfortunately means nights are much rougher for us all. James does not tolerate nighttime feeding, it makes him "moan" (which is actually a funny way of breathing, we do not think it that he's in any major pain, altho could be some discomfort, it's very hard to tell) and causes him to deSAT continously throughout the night. He seems to be able to sleep through a fair amount of it ... unfortunately we are not so lucky, especially Rob who is mostly in charge of James at nights. Since he needs every ml we can get in him we spend the entire night turning the pump on and off, soon as he settles for a bit we try pumping again for a while until the next moaning, groaning, deSAT or coughing fit. But, our efforts may pay off, after only a few days we see James face fill out, he has cheeks again, and hopefully more weight will add on as we keep struggling along.

Although this change has eased some issues, others still remain, the biggest being fevers once again. Likely a sign of a secondary infection, I'm guessing they'll be putting him on a round of antibiotics to see if that helps ... more diarrhea, more sore bum ... poor guy. We also continue to see his oxygen levels swing all over and in general increase. These increased oxygen levels means he has a decreased activity level, not being very active. At the same time he's a bit more unhappy then he was a couple weeks ago. Everything just seems to be wearing him down.

But my title was Genetics, relating to our Genetics appointment day. Genetics deals with James actual disorder and mostly focuses on the protein end of James disorder. Since that is mostly controlled by diet they are the ones we go to for feeding issues. Much of the above issues were discussed today, but really there was no answer ... we have already tried all that can be thought of. Since we now know our pump is inaccuarte and seem to have minimized his vomitting, both working to make sure James gets the right amount of feed each day we hope to see some improvement in his blood work and weight (which wasn't all that great). They want to re-evaluate in a couple weeks and then go from there ... not that there are really a whole lot more options.