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

Information comes with repetition


So we once again spend the weekend in the hospital, yes I know he just came home Tuesday but we seemed to have learned something in the process.

Due to the low protein diet that L is on, he does not get enough calories and glucose in his diet. Even though he is allowed added junk food as opposed to my other children. However, it isn't quite enough. So we now have an RX for a special oil to add to his diet and more formula to add more calories.

It is my thinking that he needs to come of the Adderal and be placed on a different type of medication to increase his appetite because he doesn't want to eat half the time. Oh and he is also taken medication to increase his appetite so that he will eat more but specifically more of normal food that he can have.

His behavior was so out of control even at lower levels of the ammonia and it is frustrating. It is frustrating because you can't help him, you can't change it instantly and his brain doesn't allow him to change it. It can be very nerv wrecking and wearing on ones patience when your child changes his or her mind every minute (and this be the truth).

Well, here is to hoping we won't have to go back so soon.

A good day


I don't know how many times this title will repeat or will be the contrast of things...but today is a good day...L 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", he will say 4 but in reality he is 5. Odd enough though is that he remembers his name and where he lives without hesitations and he remembers visits to the zoo in which he saw Elephants do their busineness, and when you ask him what Elephants do, he will tell you "they poop".

There is no way for the doctors to tell me if he will ever catch up and if he will ever be able to learn or if it will always be the way it is. Academically he is behind and we kept him from Kindergarten one more year because he is simply not ready. But I can't keep him from moving forward either. Sometimes I am afraid that I hold him back way too much and I have to analyze the skills he has accomplished, the positive things he does. What throws it off is his behavior because it tends to be more aggressive, no throught process involved - pure action based upon instinct.
While I hate the labels of ADD and ADHD there is no questions that L is the extreme of ADHD. He often does not consider the consquences of his actions, nor does he "get" them. He has a hard time to stay on task, and jumps from one thing to the next within a matter of minutes.

But today is a good day. He seems happy and he looked forward to go to school.

Frustrations


So yesterday, we took L to the ER because he was acting funny..kind of lethargic, whiney..some abnormal behavior and so forth..and sure enough his ammonia levels were 276..gees...well so they admitted him, placed him on lipids and glucose and voila this morning his levels were 125 and this afternoon well enough to go home..this makes 8 visits so far this year...and very frustrating because it seems like no matter what we do at home..controlling his diet, making sure he gets his medications, and giving him the things he needs more of such as fat (calories) and sugar, it doesn't do us any good.
I know there are times that he does well for a while..in the last year it lasted 6 months...but here recently its like he is in the hospital every 3-4 weeks.. and while we have worked out a system because of work and other children it doesn't get any easier.

I would love to just find out what exactly it is that he needs when his levels go up. I mean I know they give him lipids, but what exactly other than fat is that. And yes we have asked can we get some for home that he can take orally because I'd rather not use the IV port to administer anything, if I don't have too. Quite frankly, the best thing is to cook everything with oil i.e. olive oil which we have increased in his diet but again it didn't seem to make much of a difference in the end.

I would love to get his diet under control more because he is on a strict protein diet, he is lacking some of the necessary things that we get from protein. However to supplement he is taking an amino acid formula but it isn't enough, so it seems to me. We allow more of the "junk" I wouldn't give to my other children at least not in the rations he is able to get them. He doesn't like gatorade but it is high in calorie contend that is good for him and has all that other stuff.

Well that is my rant for the moment as he is walking into the door momentarily.

August 2007


It was a good thing that in Jan. 07 we have moved to a new area. L would be ill a few more times...with the vomiting and being extremely lethargic..

In August it came to the point that L was almost not responding, and we took him to the ER. They did the usual, hook im to an IV and took down some notes of what we described..they took blood and gave him a physical. The peditrician noted some differences in the amino acids and was concerned enough to have him admitted for an overnight stay. They brought him food which he didn't keep down..and so the journey began in the discovery of what is wrong with L.
The next day due to his normal state he was released. Things were so-so...L started school but it was but two hours later they called me to pick him up...something was wrong. I called the ped. and took him in..during the trip into town, L vomitted some more. We waited for awhile at the ped office..who referred us to UVA. In the Er..again the IV...the blood tests...and the description of what we saw and admitted to the hospital.

Diagnosis:

L has Urea Cycle Disorder

We are told that normal ammonia levels should be around 30-60 but that L's were in the 450's. Essentially this could lead to coma and/or death. It causes brain damage, delayed development and more if left untreated and undiagnosed.

L was placed on IV meds, transferred to the PICU. There we spend about 5 days until his levels were below a 100 (his safe zone). He is placed on a low protein diet (15-20 g per day) and has to take medication as well as drink an ammino acid formula.

As his levels stablized we were released to go home. It wasn't even 24 hours when L woke up disoriented (so it seemed) in reality he had just lost his vision. And it was back to the hospital. The MRI indicated that L has several edema's on his brain...one right behind the opitc nerv and it is the assumption that his body attempting to get used to the normalcy in his chemical make-up with the medication..it actually went a bit crazy as the ammonia edema's are trying to flush themselves out.

L was blind for 6 months - in which it seems that another hopsitalization with high ammonia levels did flush out enough so that the edema is less than before and voila he can see.

For more information on UCD go to http://www.ureacycle.com or go to http://www.nucdf.org

So it begins


L was born in 2003. The pregnancy was good, no trauma no trouble..until the end where he had to be induced. Perfect angel, little baby boy. All was well, or so we thought.
We went home and started the new routine. Having had childrne already, it was no big deal for me. But somehow (in my gut) things were different. L couldn't be consoled, nothing would satisfy him and he constantly had a runny nose and he would just scream for weeks on end. I'd call the doc and nothing was "wrong".
L grew and went through the stages of being ill...flu symptons but not having the flu.. he'd vomit, he'd be lethargic, hyper, not sleeping (or just a little). I was in tears most of the time, ready for a break down. At age 2, L was barely speaking, not interested in the toilet and couldn't find his independence. I had him evaluated with the school system, L was developmentally delayed by 1.5-2 years. L was also aggressive in his behavior to the point I often had to restrain him from hurting himself or others. This describes the first four years of his life.
It was a constant back and forth...nice L...mean L...lethargic L...vomiting L...hospital L..and so forth. No doc recognized anything out of the ordinary...no one reported me to social workers because I was in the ER so many times...no pediatrician thought..hey maybe something is wrong...no one would listen to me..or hear my frustrations...how many years did we acknowledge that mom's know when there is something wrong about their children?

It has been frustrating, and upsetting to know that something is wrong with your child, and no one can or will tell you what it might be.

I am lucky to still have L in my life (though this could change at anytime)