One moment and one day at a time

I don’t know how many times this title will repeat or will be the contrast of things…but today is a good day…Lennon went to school and he was gentle and kind this morning. He was still tired and sore where his IV Port is located but certainly, he is up an running just like always. It is sometimes hard to fathom he is five years old because of his delays he is lacking some of the functionings of a child, as well as the ability to learn. It take about a year for him to learn that he is one year older and the age, and by that time is next birthday will have arrived. So if you ask him today, “how old are you”,…

We all acknowledge that our children have milestones at certain ages. When they start to crawl, walk, talk, switch from the bottle to the sippy cup. Lennon has reached most of these..well he still drinks out of a sippy cup for travel reasons though he’s capable of drinking out of a regular cup. Lennon is not fully potty trained and it has been a battle I have chosen not to fight until Lennon was ready. With all the medical issues there was no sense or need to push it. We also didn’t know if he could tell when he had to go or not. Sometimes he could and would while most times he wouldn’t. It’s one of those milestones I didn’t think I would ever…

Yes, Lennon did not like some foods – usually those high in protein. I was always left wondering why he wouldn’t eat chicken nuggets, fish sticks, mac & cheese. In addition, there are lots of veggies he stayed away from corn, peas, carrots, and other ones. He has always been big into starches (potato and rice) and no he does not eat spaghetti either. It seems as though he always knew about the food that would make him ill – run by instinct – perhaps even a certain smell? I don’t know but it is pretty amazing that he ultimately knew what he could or could not eat. I think a big part of him not eating right now is that his body is going…

We received an official diagnosis today from his liver biopsy. The specific Urea Cycle Disorder is called Carbamoyl Phosphate Synthetase Deficiency (CPS). “Carbamoyl phosphate synthetase (CPS) deficiency is a urea cycle defect that results from a deficiency in an enzyme that mediates the normal path for incorporation of ammonia” (Roth, 2007). emedicine website Roth, K. (2007) retrieved from http://emedicine.medscape.com/article/942159-overview FrequencyUnited States CPS deficiency is rare. As with all the urea cycle defects, as well as most of the inborn errors, citing incidence figures is impossible because new cases are generally diagnosed randomly without the benefit of population screening. Mortality/MorbidityMortality and morbidity rates are high. Untreated CPS deficiency is likely fatal. The multiple primary causes of hyperammonemia, specifically those due to urea cycle enzyme deficiencies, vary…

So Lennon was admitted AGAIN into the hospital. Ammonia was 327, we spend 15 hours in the ER, waiting for the PICU to open up a room. He was not mentally coherent, didn’t know where he was, probably who he was or anything else for that matter. They sedated him because he became too agitated (sp) and they were afraid he was going to pull his line out. This morning his levels were 115, which is not great but better. They are keeping him until he begins to eat proberly, so I’m thinking he might (i mean might) come home tomorrow, or Monday. They don’t want to see us again (haha) anymore I want to see them. On another note, if his ammonia elevations keep…