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

Some days are diamonds, Some days are stone


Quoting John Denver today!

I was reading through the comments today and yes my emotions and my stress level are stretched probably to capacity on most days, as I am always on edge about the next second, which seems to be evidenced this week with a headache/migraine that doesn't want to go away. Of course, in addition to Lennon's care there is other real life stuff going on adding to the mix of things.

Yesterday, Lennon cut his finger on a nail on a cabinet...nothing dramatic perhaps for you and me, but for someone who has coagulation issues and clotting either does or does not happen this can be challenging at best. Lennon was a trooper while the adults in the house were a bit panic stricken but we maintained and did the bandaging, and watched very closely. Thankfully the bleeding stopped, but this morning, when we took the bandage (gauze and tape since Lennon will not keep on a band-aid)  it of course started to bleed again because as the gauze dried in with the blood it pulled the scap/clot right off. So we bandaged back up and are leaving it alone (sort of). We are keeping an eye on him and what he does with it.

He was a little tired today, took a nap and perked right up. Now he's in bed sleeping like an angel. Today I observed Lennon differently. I am used to him being emotional in order to get his way or simply because he isn't feeling good. One of your dogs got off his chain overnight and it appears that he was hit by a car. This afternoon we dealt with the aftermath of looking for our dog, finding him in a ditch and explaining to the kids what happened. I was talking to Lennon's brother (Jarod) and Lennon asked what had happened. I told him that Draco got hit by a car and died, and this might sound harsh but I just couldn't sugar coat this one.
Lennon yelled : "that's not fair, he's never coming back" and with that buried his head into his blanket.

I still have a difficult time putting into words how this affected me. I know that Lennon was close with Draco (not as close as with our other dogs, but close). He loved that dog. At any time he would be in the back yard playing with all three dogs. He literally watched him grow from puppy stage to big dog. It is a sad day in our home. We picked Draco from the first puppy litter Dakota (mom) had with Tank (dad).
I don't know how this will affect Lennon if at all in the next coming days and how I will be able to react to it. With that said, I will say good night and thanks for reading as always.

Petra

What we learned today


Lennon had his weekly appointment today and so the word is that Lennon has chronic liver disease caused by the lack of blood flow to the liver. This means that the clot in the portal vein, the collapse of the hepatic vein and the clot in one other vein do not allow enough blood flow to the liver. This means that we can only hope that these clots perhaps will eventually go away, that they will not get bigger or that more accumulate over time. In hopes that the liver can heal itself throughout time. This also means that he's at risk for developing more clots, experiencing liver failure more and more with time and that there is that chance (a good chance) he will face another transplant at some point in his life if the clots do not go away on their own.

For now he is well. His protein is restricted and it will continue to be restricted until further notice (as long as those clots exist and liver failure is present). The IV meds should hopefully be converted to oral or g-tube as it is in his case and he could potentially be back in school within a few weeks. It will be discussed, evaluated and based on Lennon's health. If he cannot handle it or if it isn't safe for him, he will go back to being homebound for education. Quite clearly, the doctor does not want him on the monkey bars, but that is easier said than done with a child who LOVES to climb. So his medical is being managed and maintained. And we are ensuring that he has the best quality of life (at home, with or without school), but he will have it. The docs will continue to watch him closely, check for jaundice, rashes in the mouth or anywhere else and so forth.

And this is all I have right now, I think. Lennon is a little moody today, but we are allowed a day where we are not on our top game.


*** Adding on to this post***

So it came to my attention that I should perhaps let everyone know that Lennon is on a medication regimen that is a blood thinner (aspirin) and should in time remove the clots. However, Lennon has developed a coagulation problem in his blood which leaves him to be at risk for bleeds. Thus when he falls I have to check him to see how it is bruising. He also tends to get minimal (not gushing..just kind of there) nose bleeds and minimal bleeding from his mouth. For this reason Lennon takes vitamin K (something for thickening the blood and reduce bleeding risk). Management of the aspirin and the vitamin K are done closely watching his blood level and adjusting his medications as needed.

It is quite apparent that his road of medical turmoil is not over and that complications are anticipated at one time or another. The complications can be a variety of ones. There is not only the potential that if the liver continues to fail due to the lack of blood flow he is facing an additional liver transplant, but there is that risk of bleeding. There are many facets of Lennon's care that cannot be predicted or speculated on.

Of course this doesn't mean I don't worry (quite the opposite) but I also don't want to be in panic mode when I don't need to be. The doctors are keeping a close eye on him, the nursing staff is taking care of him, and we at home watch him closely, paying attention to every mood swing, behavioral episode (for a lack of a better word) and the hyperactivity if it elevates.

I have never nor will I ever turn down any prayers, positive energy or anything else that is beneficial in our journey and foremost in Lennon's journey. I will continue to write posts about the good days and the bad days. There will be days and weeks perhaps of silence, but know that in the day of silence it is then when we experience normalcy.

Quiet


It's been seemingly quiet at the home front without too many changes, no concrete results of what happened last month or what is going on with the liver. I know that the EPV (Eppstein Barr Virus) numbers are incredibly elevated and we are hoping that the IV medication is taking those levels down.

This week we will be discussing taking out that pic-line and even though we've had central line before I'm glad when it's out and gone. If you remember he had two infections - one with his port (yeast) and one in the central line (ecoli) and so I'm a little nervous even though we once again do what needs to be done.

Lennon is quite spunky these days, funny, goofy, ornery, loving, full of himself. Yesterday he made me several valentines while I was laying down on the couch. Once I got up, he said "Mom, you forget to make my valentine". So we sat down together and I created his valentine. 

Follow-Up with the Doctors


Lennon is still on IV medication to bring down the EBV (Eppstein Bar Virus) levels in his system. His ammonia has been maintaining at 75, which in terms for Lennon is probably normal. All but one test has returned from the mayo clinic and they all are negative..so still waiting on one more result and well then what? I don't think anyone knows. I know there is talk about doing another biopsy in a few weeks to see how the liver is looking then now that he's been changed to IV meds. In addition, they are going to have to think/talk about taking the pic line out. I remember the last one he had and despite stitches.. well it just fell out...not exactly something I want to happen now that he is having some coagulation issues (which he didn't have the last time when it just fell out).

This is kind of the medical stuff.. don't know much of anything other than he looks good. And I mean he really does look good! We have a behaviorist schedule to come visit next week, but with the way he has been I wonder if that is even going to be relevant..and then again we do still deal with aggression and other behavioral stuff. Overall though he has been sweet and just the cutest thing to watch and listen to...sometimes I wish I had an video cam going all the time or readily available because some of his moments are so precious. Tonight he read me a story (instead of me reading to him), and he played with his cars, even if it was for a short while..he played nonetheless.

He's been very polite too when asking for a drink, saying please and 'thank you mommy'. At the moment he's watching a movie with his brother. So I leave with a note that he is doing well, even if he is not all the way out of the clear...then again he could be...one can never tell.