A very dry report of our last trip to Boston

Peter had his check-up in Boston about two weeks ago.  We crammed in his intestinal rehab clinic visit, a follow-up with Dr. Jennings (his surgeon), a sedated cardiac MRI, a lab draw, a bone age scan (an x-ray), and a DEXA scan all into two days. 

Peter is doing well enough overall to stay off TPN for now, but he is continuing to lose weight over the last several months.  His arm circumference and skin fold measurements have both gone down.  He has grown some in height, so he has been able to digest enough calories for lateral growth, but if he cannot stop losing weight, then he may need to go back on TPN to supplement his growth.  It looks like his caloric intake is about 2300 calories a day, which should be enough, but there is some degree of malabsorption.  We are currently trying to squeeze in a little more calories here and there over the next couple of months to see if we can prevent a need to return to TPN. 

Peter's vitamin A and D levels are remaining low even with high supplementation.  His platelets are under 30,000 now, which is a little lower than is has been.  To put that is reference, a normal count for most people is 150,000 to 400,000.  He still clots quite well though, which Dr. Kamin says is because his body has slowly gotten to that point and the platelets have functionally adapted.  His white count is still considerably low as well (under 2).  Looking at better lab news, the iron supplementation has proven good for resolving his anemia.  The rest of the labs seemed OK enough, with the exception of calcium in his urine seeming to go up higher.  Our plan is that he will have no calcium-containing foods for 4 hours before the next urine sample, and hopefully we will see less calcium in the urine.

Bone-wise, it looks like Peter's osteopenia is no worse.  It is still present, but his bone-age is about right for his age, and that is good news.  

Besides the let-down that Peter may have to return to TPN, the other disappointment was in the results of his cardiac MRI.  The MRI showed that he now has dilated ventricles, which he did not have in the past.  Dr. Kamin discussed possible causes with several of the doctors who are very familiar with Peter's abdominal issues and they do not feel this is related to intestine, liver, or spleen.  That is a bit of a relief, as I don't know how much more surgery Peter's abdomen could take.  There are two nutritional labs that are going to be drawn this week to see if they could be causing any of this, but that being the culprit is a long shot.  The most likely area of concern is the heart or lung and I expect that will be the focus of additional testing. 

Our priorities over the cardiac and nutritional follow up are that Peter gets to go trick-or-treating with his siblings and that he gets to celebrate his birthday at home, also with his siblings.  Dr. Kamin thought we would be able to work that out just fine.  Peter has envisioned such plans for trick-or-treating and for his birthday, and none of us want to disrupt those plans!