Christopher's First Hospitalization

On the evening of January 15^th Christopher appeared to have a cold…by January 17^th he wasn’t eating well and was unable to keep all of his medical formula down because his cough was triggering his gag reflex…so we headed to the emergency room. At the time of his admittance his CKs (or level of muscle breakdown) were 11,000 (normal range is 120-220). During his hospital stay his CK levels peaked at 70,000. There are several things that doctors worry about when children or adults have high CKs but the main concern is kidney failure. High “CKs” or creatine kinase in your blood means that you are having muscle breakdown. This can happen for various reasons, but for children with LCHAD elevated CKs typically occur when they have utilized all of their energy stored in the form of sugar. Their body turns to fat as an energy source but can’t break it down so it starts to breakdown the muscle instead. CKs are the by-product of muscle in the blood stream that doctors can measure. Myoglobin is another by-product whose levels coincide with the amount of CK in the bloodstream. It is the myoglobin that is hard on the kidneys and can create kidney failure and put a patient on dialysis. The large reason we have to take Christopher into the hospital when his body starts breaking down muscle is to get more glucose in his system so his body stops trying to use fat as an energy source and to keep him very hydrated, so as to protect his kidneys from renal failure.

It probably seems quite simple to say that having Christopher in the hospital was hard, but for all that we experienced it seems that this might be the best adjective after all. I must first say that we are really blessed to have such a wonderful metabolic team who worked extra hard at coordinating care with the pediatric docs in the hospital. Christopher’s clinic and head dietician stayed in touch with me throughout the whole process; updating me and answering questions along the way. Also we had amazing family and friends who called, talked, sent food and prayed. But having a child with a rare inherited disease like LCHAD means that the clinicians who worked so closely with him at the hospital had most likely not worked with a child with his condition. It meant that we as parents needed to encourage communication with his metabolic clinic and educate staff as much as possible. As with any communication among groups sometimes we did better than others. I found myself, worried, stressed, tired…and probably a little on edge with the docs at times. Then as he started mending in the hospital, there came the challenge of trying to find foods low enough in fat to feed him.

He was in the hospital for almost a week, when he was discharged. His doc was so nice to talk with him and give him a stethoscope so he could check his stuffed animal’s vitals at home. When we left the hospital his CKs were still not normal, and we were told that he was to do very low-key activities and not be around other kids for two weeks. Two weeks turned into almost a month, as his CK levels took three and half weeks after his hospitalization to get back down to normal. During that time we did a lot of drawing, painting, playing with animals, game…movies…but I must admit by week three we both were beyond stir crazy! Christopher has maintained a positive attitude through it all…and even when he had to go back for several blood draws after his hospitalization, although he didn’t great the blood draws with a smile, he was able to find one for his Dieticians and phlebotomist!

About a month and a half after his hospitalization

Christopher still mentions “Mr. Tubby” (his NG tube that he had to have put in while at the hospital) and checks Ni-Ni’s (his favorite stuffed animal) vitals and blood work quite regularly. He seems to be gradually processing the experience, but nighttime is still hard for him.