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

It is the song that never ends....


...it goes on and on my friend...

This seems to be the reoccuring theme these days. L's levels have been pretty normal/low 55 and 78, so nothing to crazy to worry about. But his behavior doesn't collaborate his levels. L has been extremely violent, uncooperative, not listening and kinda crazy if you will for a lack of a better word.
One moment he is sweet and loving L, and the next he is the holy terror. So now they are trying to admit him to KCRC for observation period with the neuro-development people and behaviorists. Of course this means that medicaid has to approve it, which that can be a battle. So will see, but for the moment he is going home!

Which one is harder


I don't know which one is more difficult to deal with the part where he is mentally gone - incoherent - unable to "get" where he is, and who is with him - or the part where he is so violent that it actually requires either four adults or retraints to keep him safe. Again this is a function beyond his control and while sedation works wonders, with his ammonia being up it isn't the best solution because we can't use it as a baseline for behavior at this point indicating normal levels.

It breaks my heart sitting here, watching him, unable to truly comprehend what is happening. I can explain to him why until I am blue in the face - and he will not understand what I am saying. He understands the words - he doesn't comprehend the meaning! Nothing will distract him to be nice, gentle and good - I can't trust a hug because he is quick with his teeth.

I imagine how easy it is to be judged at the moment for allowing the doctors and nurses to restrain my child. Realize this however, that this is for his safety. He can pull his IV out, he can bite the line - he can jump of this bed - run into walls as well as kick, claw, bite and scream at everything and everyone. I certainly don't wish my son to be restrained - but I certainly want him safe. The ammonia is elevated and this is what it can do. It messes with his brain every single time - and every single time it is elevated he sufferes a little more (or some) damage.
Again I feel like he is slipping away from me - underneath all that care he is given - slowly but surely I am loosing him - but I have to believe that he is a fighter - he will not go down easy - The Angel of Death will just have to wait a bit longer!

The world is slipping under my feet


Today while I was at work I received the phone call that L had to be taken back to the ER. Levels came back at 187, not as terrible as it had been but still bad enough considering what abnormal levels can do to you.

As I am writing this update, I feel like the world, no not the world but L is slipping away from me underneath my hands. It seems no matter what we do at home and the best care everyone gives to him, it doesn't matter. I want to hang on to hope and find the strength that he will survive and that he will be there, graduate from Elementary School, Middle School and Highschool. But also that he is enjoying life, finding the right partner for him..all the things we wish that our children should accomplish to achieve their happiness. I am barely hanging on to that hope.

My relationship with my partner is strained as we can't find the time to breathe, let alone find time together. It is us doing it, all the time not only taking care of L but our other children, who being left behind in the process as we are attempting to save L's life. They are hurting as well, not only because (I feel) they are being neglected half the time but also because they are worried, they are scared to death just like us the adults.

Friends tell me prayer chains are all over the world (literally) and I can't ask any more of that. So thanks to all that think about L and in his time of need. Thanks to all that think about us in our times of struggle and heartbreak. Just thank you.

The scariest moments are....


...when the person you love looses all mental abilities alas is incoherent, doesn't recognize anything and is in a delirious state and all because of the ammonia going up.

Thursday into Friday..another night in the hospital...I had brought home a new kitten (naming her doodle ..short for doodlebop). I was heading to bed when I heard some comotion and sure neough there was L with holding his blanket, rambling things that didn't make sense. He had vomited and well his mental state wasa clear indication he needed to be taken in right away. I drove into town in the rain...it was not a good night for me because I was tired and exhausted, emotionally a wreck and well I don't like to drive at night, I don't like driving in the rain and I don't like driving at night in the rain.. but it didn't matter what I like, this is about L's life, not mine.

As soon as we got to the ER, they drew his blood, got us cozy in the ER room and we waited - not only for results but a room upstairs. The PICU was full and no one could be moved - so other arrangements had to be made.

Level 309 - gasp - why why why? What is it that is not working so that we are in the ER every weekend. Sometimes twice in one week. We are noticing that his brain is shutting down when his levels go up, he begins to drool indicating, that this part of his brain is partially gone. We also think that the last two times of elevated levels have done more damage to his brain.

So 12 hours later we finally get a room on the floor - normal routine - L wants a popsicle, he is hungry. He is eating his black olives, some french fries and drinking Pepsi.
Speaking of that, the weird thing was that this time he did not stop eating or drinking - he indicated being hungry - eating some baby food and drinking his tea or water - this episode was not normal in the sense of what we were used too. Anyhow, so we are in the room and blood is drawn - well wouldn't you know that his level is 86 - we are going home!

Lennon is still kinda out of it but they had him sedated which he didn't get to sleep off and so I suspect that is why he is a little croggy and down. He is kinda mellow - again not what I am used to - what damage was done to his brain? That is what I am wondering. And so the day goes on, we have dinner, take showers, take medications and go to bed. L slept through the night. This morning he is helping on the paper route - well because I have to go to work shortly and the papers were way LATE and so this is what works. And L didn't mind - he likes to go on the paper route.

We're Home......Again


Yes, we are home again. L's levels were 58 and he is eating good. Notification was send to the Liver transplant team and so the process begins. I don't know what is more scary - these hyperammonia episodes and the risk of him becoming severly brain damaged ...or even worse.. one the other hand the transplant brings a lot of other risks - the immune system being nothing - forever being on medication - well either way he wil be on medication.

Someone told me I should get the super-mom-of-the-year-award - between everything that is going on with L - working - school - our other kids -my art stuff - well I am just doing what I have to do - there is that hidden drive - the energy and focus that gives you the boost to to do all that you need to survive and better yourself in the process.

As heart braeking every episode is and as tough they are on us as a family and our finances - we do what we have to - don't wallow in pitty because there isn't any time for that. I cherish every moment that all of my kids are healthy and the time that we laugh and have fun together - even if they are few and far in between.

Tired


I am beyond tired and certainly know that my driving force is the love for my son. I haven't seen my house or bed since Friday as I travel between work and the hospital. Last night L decided he didn't want the IV - played with the tape - and ultimately pulled out his IV. They had to replace it of course, but come this morning the line and his blood was not working and all the heparin and TPA didn't do any good. And the IV was replaced - again.
He will be staying in the hospital again tonight - his level is around 138ish - thus still not well enough to go home - though he is eating a bit better.

That's all folks.

Out of control


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 it up, he could be looking at a liver transplant, which essentially will "cure" the UCD but give him other issues - no immune system, will be on other medications. And so forth. If it does come down to that, I hope that his body does not reject it and that this could be life-saving for him despite the risk attached to the transplant.

I have not wrapped my head around all of this. I am at work, exhausted from being up a long time, sleeping a few hours and hoping like hell that he will be ok. He was more coherent this morning that yesterday but I still didn't see a huge improvent in recognizing my son. He was cranky for sure. They had him in a crib for safety overnight, but this morning he was SO NOT HAPPY about it. Can't cage the Lennon, ya know. They are giving him a regular bed now - per conversation with my partner.

Quite a few people have asked me why I come to work and I'm like, well you are not paying my bills, someone has too. Right? Thus we shift change in order for us to keep our job and earn money. Besides I can't do anything at the hospital, exept sit there and keep him as calm as possible - keep in mind he is extremely ADHD and sitting still is not his best skill at best.

I love this kid - he is amazing and right out a miracle. He is resisting death without a doubt, he is a fighter - and I hope he continues to fight because he is the coolest guy ever - ok my other kids are cool too but they are "normal" and not sick or anything. This makes it hard too. L gets all the attention, and my other kids have to "suffer" though they are not neglected in a sense of not having food and shelter but I feel that L's disorder often keeps me from equally sharing affection among everyone else.

Level 88


So the ammonia went below 100...88 to be correct..and so L is home from this visit. They did his MRI in the a.m. and I am curious to know what they can or cannot tell me. His future is undetermined but we'll do the best we can. I think my frustrations are really to the point that we cannot control his ammonia and there is no rhyme or reason what may cause it to go up. While stress is one of them, I would love to provide a stress-free live but tht is unrealistic. I don't know if I want him on additional medication to cope with his stress problems because I want to enjoy my son even with his quirks. I think this one was triggered by stress (some of it self-induced) and a nasty cold he is trying to battle.

On a side-note, I signed him up for research studies though I am not sure where this is going to lead as we are having difficult times when he is in the hospital and thus traveling would be even more complicated for our family.

Well, lets home we get to spend more time at home than in the hospital. I would like a few weeks without a spell but I could be asking for too much.

Beautiful Boy
by
John Lennon

Close your eyes,
Have no fear,
The monsters gone,
He's on the run and your daddy's here,


Beautiful,
Beautiful,
beautiful,
Beautiful Boy,

Before you go to sleep,
Say a little prayer,
Every day in every way,
It's getting better and better,


Beautiful,
Beautiful,
beautiful,
Beautiful Boy,

Out on the ocean sailing away,I
can hardly wait,
To see you to come of age,
But I guess we'll both,
Just have to be patient,
Yes it's a long way to go,
But in the meantime,

Before you cross the street,
Take my hand,
Life is just what happens to you,
While your busy making other plans,

Beautiful,
Beautiful,
beautiful,
Beautiful Boy
Darling,
Darling,Darling Sean.

I dedicated this son when L was born before I knew about his disorder or anything else. Though this applies to all of my boys!!

Holding my breath


L was admitted to the hospital on Saturday with the ammonia level of 237. We did the usual treatment of lipids and D-5 and at first they were going down to 147. So we figured good deal we'll be home Sunday. Well no such luck. They checked his ammonia this morning and his level was 353. So now L is in the PICU receiving his meds through his IV Port in addition to the lipids and D-5. The PICU is only required for monitoring him more closely because the nurses on the regular floor could do the same work but they cannot closely monitor him.

It worries me that his level went up like it did because this brings more reality to the fact that I can be loosing him at any point. If it was me (or even you) he would be in a coma or perhaps even dead. This reality is heartbreaking and brings me to tears and I often wonder how much longer I have with him. He is so precious. Despite his behavioral issues due to the brain damage and his delayed development, he is such a gentle soul. He is loving and he is fun and keeps my life interesting and laughing.

Tonight I am at home while my boyfriend and ex-husband keep watch over him in shifts. It is very hard not being there constantly because I am sorry but as a mother, I have to and should be there. I recognize however that even I need a break to get some sleep, take a shower and just breathe for a minute, oh and well do my homework. I must remind myself to take care of me in these times but I am not very good at putting myself first.

I long for the days of normal in which we can simply go on with our lifes, struggle to pay our bills and but food on the table, but still normal in the sense that we are working, enjoying the children, and be a family as opposed to our second home being the 7th floor of the hospital.

Our ups and downs


Yesterday went well but today is a different story. L woke up not wanting to eat breakfast, and I said alright, well at least you can drink some of your formula… HA! Yea right! He or his body decided that it wanted to reject it, and puked it all up (twice). Normally this is an indicator that he is not well, but it doesn’t appear that his ammonia levels are elevated. He seemed to be in a decent mood, more mellow than I am used to, but certainly in a good mood. But it gives us a good cause to worry about it nonetheless. Ultimately I think if he continues on this path, we will not have a choice but to go ahead with the feeding tube. However, in the mean time we are trying to avoid this as best as we can with added medication to increase his appetite.
The worries with the G-Tube are that he will pull out the plug or button, whatever you want to call it. He doesn’t get the consequences of what happens when he pulls it out, nor the possible damage he can do and the infections he can suffer. While there are contraptions to place around his belly i.e. a corset or an ace bandage, it is my fear and worry that he will still find away to get to it and pull it out. I would like to think that he’d surprise me and not mess with the button, but I have little faith in that.
It is frustrating because finance-wise it is creating a vicious circle in which we are trapped in. Ultimately we both have to work to provide food, shelter and transportation to not only L, but also the other children. However, if he continues to go into the hospital once a week, twice a week and so forth, one of us might have to give up our job and then what? This is almost impossible to think of.
And then the services for intellectual and developmental disabilities in my community, well they are lacking the essentials to support families with special needs children and family members. With the economy the way it is, some of us folks can’t get into the big town, which takes about 45 minutes to get there, and well gas money. So in addition to helping my son survive, I will venture into the research and advocacy of individuals like him.