A boy named Lennon


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

The waiting game

It's pretty daunting the waiting game. My phone rang today and it was the hospital and my heart took an extra beat or two. It was only to talk about some additional things they want to do such as check his blood oxygen level as that had been pretty low and of course lab work so they can update his listing on the transplant waiting list. Depending on the O2 level may require additional tests like what they call a bubble echo. They checked the blood flow into his lungs while we were at Pittsburgh and it looked good, but there is that concern that his breathing is not what it should be and is more than likely related to the failing liver.

If the liver doesn't get enough flow of blood in then it can't return the blood back into the system.

One thing that we have gotten in place is transport to the hospital. Lennon and I will be flying from Charlottesville to Pittsburgh when the time comes. While that is one "less" thing to worry about it doesn't take the worries and stressors away about the whole ordeal. Lennon has never flown before and I wonder how he will do getting into that airplane, flying for about 3 hours. Will he be calm? Will he be stressed and freaked out? I don't know. I for one am not a fan of flying but I will have to be the super calm mom! Why? Ha because Lennon can read me and if I am anxious then he will be anxious too.

I am trying to get whatever I can set into order. I have a business to run and some things I can't do from Pittsburgh so I have been teaching the rest of my family what to do and how to do it. Some parts of the business will not have production while others I can do in Pittsburgh.

Lennon is doing well however. He's enjoying school and just being normal. This past week he had homework and while he at first resisted the process, he was beaming with pride when he did it (with my help). He loves going to school. I think its a combination of being around other kids and learning... being challenged a little.

Well, this is all I have for the moment. I hope everyone has had a good day!

It's Official

Today I received the phone call that Lennon is on the transplant list for another liver. I am with mixed emotions but really is there another choice?

If we do not move forward what kind of mom would I be to not save my son's life and do what ever I can to give him a fighting chance?! I am glad they recommended the transplant rather than saying they can't do anything for him.

Certainly it doesn't come without risk as we know to well already. The chance of this happening again... well who knows. Everything we do has risks.
The risk of the portal vein and/or hepatic vein clotting or having thrombosis stands with every (liver) transplant. Lennon has both + one. We can hope and pray that this one will be successful and that Lennon has a fighting chance at life.

Allowing (probably not the right word) Lennon to die doesn't seem to be an option which is what would happen if we do nothing. The liver is already dying due to thrombosis and clotting. Allowing him to have a fighting chance is the right thing to do!

We know from the past that our local hospital has used partial liver and the game plan with the new hospital is to use a whole cadaver liver, which according to the surgeon will give Lennon a better chance at survival. I will not knock what our local hospital has done because I truly belief that they have done everything possible. Kudos to them for doing what is best for Lennon and recommending that another hospital specialized in peds transplants may be better at this than they are.

The reality is that if I don't do anything and do not move forward I will feel guilty for not trying. By moving forward with another transplant I know that we have done everything in our power.

Thanks for your continuing prayers and positive thoughts.

Early Reports of the hepatic Venogram

The initial report is there is a blockage on the vena cava vein. This means that if another liver transplant is recommended that more surgeons may be needed in the OR due to the added complication. The vena cava is kind of important piece as it allows the blood flow to the heart...

The Surgeons said that within the next week we will have a recommendation on how to proceed. This is kind of nerv' wrecking.

Its hard to know that your child is incredibly sick and that a risky surgery is the only thing that may safe his life.

I am still trying to wrap my head around it but its difficult and leaves me with a heavy heavy heart.

Sometimes we get so carried away in our daily lives that at times we forget what matters. I have had many emotions this week but in the end it is about spending quality time not just with Lennon but with everyone that I hold dear to my heart. Realizing where your true energy belongs can often be difficult if we get caught up in emotions due to others actions.

The last couple of years have taught me what sacrifices I am willing to make, and which ones I will not. I am grateful to the support (however it is given) we are receiving from near and far. Whatever the next week and the future brings it will not be an easy undertaking and I hope that I have the strength to support the people in my life that needed it!

Our trip to Pittsburgh

We drove into Pittsburgh to start our process of pre-evaluation for a liver transplant Tuesday night. We started our trip at midnight and Lennon was awake for most part of the trip till about 5 a.m. before taking a short nap.

The first appointment that day was labs... whew what a confusing mess...check in with registration...receive a pager..wait for pager to go off... after that we met with Anesthesia but that was short because its technically one of those things that happens later...closer to surgery.

Next we had a psychological evaluation...painless...lots of talking..lots of note taking and being told we should see a therapist.. umm yes...

Next and the last thing for that day was Radiology... Ultrasound, CT Scan and XRay. Lennon was doing so well.. I have never seen him cooperate as much as he did then. The CT Scan was interesting.. the cool thing is that it is definitely kid friendly with pirate and castle murals all over and a ships wheel on the scanner.

He was so tired that he crashed rather quickly that night. Wednesday was another big day for Lennon. Genetics clinic...really just meant going over his urea cycle disorder, the history before diagnosis and everything that happened afterward. After that we had "teaching" about liver transplantation.. most of it was not new but I really liked how they explained it all..anyway.. after that we had a meeting with Hepatology (liver specialist)...not really eventful.. so the next appointment was with infectious disease which covered exposures, live viruses and other things I can't remember. The last test of the day was Cardio which he had an EKG and an ECHO done. Lennon did well even though he started to get restless... he does not like the goo they put on you during those tests.

And now to today's fun activities.

Today... well what about today... First thing on schedule was hematology. We talked about Lennon's coagulation issue and the doc ordered 20+ tests checking for inborn, inherited and acquired blood diseases and/or coagulation issues. After that we met with transplant and were able to see the CT SCAN pictures. The docs seem confident about doing another transplant but here is where it gets tricky.

Despite the contrast given at the scan the vena cava vein could not be detected clearly or clear enough.

The superior vena cava is the large vein which returns blood to the heart from the head, neck and both upper limbs. The inferior vena cava returns blood to the heart from the lower part of the body.

I was actually trying to find a picture and I couldn't. Anyway, they admitted Lennon because ... a) they want to do an hepatic venogram in the morning and because some of his levels in the blood are off they want to be able to infuse platelets if they need to. Anyway, depending on the outcome of the hepatic venogram it could very well change the way they will proceed in terms of the "technical aspects" of the transplant surgery.

In addition, however this means this liver could be a lot sicker than the biliruben indicates when they do the blood tests.

As we were getting comfortable in the room, and they checked Lennon's oxygen it was at 88. They now are given him oxygen and will order another xray to make sure that all is well.. and that he's doing okay.. I don't know if this answers anything or not but this is our experience thus far.

It almost sounds like that they only way Lennon may have a chance at a good life is to go through another transplant. The doc feels thinks that Lennon would do well. They would use a whole cadaver liver as opposed a partial one as they have done in the past.

So this is where we are ... I think.. I may have missed something.. ok may be not.

Heading to Pittsburgh

We are heading to Pittsburgh tonight. 3 days full of tests!