A boy named Lennon

welcome

Lennon was born with a rare metabolic genetic disorder called Urea cycle disorder - CPS Deficiency. He was diagnosed at the age of 4. In November 2008 he underwent not one but two liver transplants in order to save his life and could potentially face another transplant. This is his story.

Yeah we all shine on, like the moon, and the stars, and the sun. John Lennon

Satisfaction and being satisfied


L has a real hard time with being satisfied. He says he's hungry, and it takes 5 minutes to figure out what he would like to eat, then you fix it, and then he doesn't want it. This is the real frustrating part on our daily routines. You monitor his food, make suggestions of what he can have, he decides on something but then does not eat it. Then you have to make sure he does eat and drink, because when he stops doing those...well his ammonia will go up...however I must say the last three weeks have been almost unreal. We have been home without interruption. But I still don't feel like that I can breathe any easier because it can turn any minute.

I recently had a conversation with his teacher and they are now in the process of trying to figure out what to do for next year. He is no where near ready to go to kindergarden, but he cannot be held back. No medication (as of yet) has worked to keep him focused for anything more than 5 minutes, though I do see some sloooooooooowwwwwww progress. The frustrating part is that he is not maturing, but he is functioning. His speech while not perfect, has improved tremendously. He still has a low-tone in his reflexes and more often than not requires MY undivided attention because no one else will do.
He very easily becomes obsessed with one thing and has difficulty accepting that it will not happen such as playing the video game, which wants to change from game to game. It's like he wants to do everything so that he has not missed out on anything...because the reality is that one bad spill it could be over...

But that is why the transplant is in the process...Yesterday he had his MRI of the abdomen, another blood test and another visit with the doctor. L did really well accepting that we were not staying there for the night. He did ask if he was sick and if that is why we went to the hospital. So he does get some of it, and probably recognized that he wasn't feeling ill. The transplant is scary and I am attempting to only keep the positive things in mind, but can't help think about the what-ifs...and man I can't tell you what it feels like to imagine not having your loved one around..and I hope that I will not have to face that before it is my time to leave this physical place we call earth.


I must say that all the hospitalizations and me missing time from work for 2 months has messed up our finances. We are struggling to catch up on the things that fell behind and the light at the end of the tunnel doesn't seem to be getting any closer. For anyone reading and following this blog, I don't want charity but I do encourage to purchase my artwork and jewelry instead. I don't like asking for help, but perhaps I can promote my art work and create some additional funds to help us out. My family has been supergood in dealing with the bare minimums, but christmas is too close to not worry about not having anything for them...oh boy did I make any sense... Please if you are inclined to give...do it by purchasing one of my pieces at http://hippiescreations.etsy.com/ or visit my other website at http://www.hippiescreations.com for additional information.

Transplant Process ...the beginning stages


So L had his initial meeting with the transplant team. I am scared, worried but like the outcome even though it bares quite a few risks. L has been doing ok, but has not been sleeping well. He has been drinking and eating, so I don't think that the ammonia is elevated in the "danger zone". In addition, his attitude and behavior indicate that it is not over 100. We certainly need to watch him and do the things we know to do : gatorade, MCT oil, sugary things etc.

OK back to the transplant... L will receive a whole Liver as opposed to a part of a living donor. The reason being is that since the Urea Cycle Disorder is Genetic, there is a pending risk of it not going away due to the same genetic make-up even though no one else in the family has it (that we know of). It could be that I am a carrier, or anyone else in the family is a carrier and so there is too much risk. So he already has given his blood, now we are waiting for an MRI/A (MRI of his abdoman). The docs and transplant team will meet next week, recommend L to be placed on the waiting list, and if there is not enough points to do so, there will make additional recommendations or something like that, in either case, in about 3-4 weeks L should be on the liver transplant waiting list. Of course this then means that we could receive a phone call at any time, on any day.

In the mean time we will hope that L does not have many spills or relapses that could damage his brain (or worse). I think if he doesn't get some good sleep soon, we will visit the ER once again sometime within the next few days.

One moment and one day at a time


We have been home one week without going to the Emergency room or being hospitalized. This is good, but I still feel like I cannot breathe and relax. L looks really good, his personality is back - the sassy, funny little guy who keeps you on your toes. And yet, I am filled with anxiety that I will wake up, or get a call at work telling me that L has another hyperammonia episode.
But I must admit, it has been nice to be home on my days off, and come home from work and know that he is doing ok. A good indication for L's ammonia levels is his pupils and this morning they were normal - if is ammonia levels are elevated his pupils will get big - at times they will cover the entire Iris and pupil is all you see. His behavior has leveled out some - but we stil go through the motions of really funny to whiney and cranky - to tempertantrum from hell - but they have gotten better. Now I just need to learn to breathe during the times he is well.

Wonders and Worries


So we have been home for a few days but I am still holding my breath. L looks good physically and no indication that his ammonia levels are elevated. The only thing that shows is his aggression. Now we also deal with the typical 3-5 year old behavior and it makes it difficult to identify which is what at times. I had to pick him up from school yesterday because he was hitting other kids and pulling hair. Now I don't mind that but today I asked him to remove him from the room if possible, or put him in time out. Certainly if he gets out of hand, please do call me.

On the other hand I can't wonder and worry when he will relapse to high ammonia levels. Because it has been so unpredictable, I feel I don't have time to relax for one minute. The pressure of keeping him healthy, studying for my master's program, making sure the other kids are ok and taken care off and keeping my partner happy often places too much on me. I don't have time to breath for myself. I know it seems like I am complaining. But truly, that isn't it. I think I just get so overwhelmed that a lot of things are left on the sidelines. Which means that my relationship is suffering, and I don't know how to let go of the anxiety.

There is the constant worry...