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

Ch-Ch-Changes are constant

So today we got a phone call ..some changes on his IV meds in order to bring down the number of EBV cells (eppstein bar virus) and then get that under control. Further, an additional mentioning of another biopsy will perhaps be necessary to "look" at the liver. And if that isn't enough, the dr. mentioned that Lennon will more than likely require another transplant in about a year..

Honestly, I don't know how or what I am feeling, nor what I am supposed to be feeling. How am I, as his mother am supposed to react and handle all of this again and again...seriously? Another liver transplant?

So here we are with maintaining medication via IV at home, change in his diet and feeds and the amount he needs to drink. Watching carefully for lethargy and behavior changes. Keep an eye on any lymph-node developments and watching him slowly master mile stones typical for younger children. His speech still delayed and his emotional stage still much that of a 3 or 4 year old. But in the past weeks he's put his own pants on..and that is success.

Normalcy ...I do I love thee

So we have been home since Monday and I'm super busy catching up on things and getting organized (again). Lennon is doing really well. He had a dr. appointment yesterday and his labs came back ok except for the EBV (Eppstein Bar Virus) that went from 1300 to 2300. There is still concern that Lennon may still have PTLD (as mentioned in a previous post), but they don't know for sure yet. His other numbers look better though indicated still some amount of "injury" to the liver but again we are also still waiting on test results from the mayo clinic. \
Lennon's ammonia is a steady 75, which is better than 100 but still above normal...but then again this could potentially be normal for Lennon.

So for now we are home experiencing normal until we are told otherwise. I am definitely ready for those results to come in ..no matter the answer ..because it's nerve wrecking to think about what the future may entail..I have a lot of worries and concerns in my head and my heart and once again we are taught the lesson that life is too precious. Lennon requires a lot of care but that of course doesn't mean I love my other kids any less, they just tend to fall to the side though a) because they are not sick and b) they are older. But I do cherish every conversation, hug, kiss and I love you's because well one never knows.. I remember a time where I turned my head while still holding a conversation, those days are gone (unless I am in the middle of something and being bombarded). I know try very hard to make sure that Lennon's brothers are acknowledged, the oldest is turning 18 this year and is graduating from high school. He will move on to do his own things.

I have learned important life lessons in this journey that has not finished yet ...

thanks for listening

Today Lennon is being goofy

I am currently watching Lennon be silly and goofy, singing and dancing. It is hard to fathom that there could be something seriously wrong when he looks good and behaves normal.. yes singing and dancing and mooning (yes mooning) is normal for Lennon. Oi!

This morning we learned that the test results they are waiting on probably won't be in for another week. They are checking soft muscle and some other dna testing to perhaps figure out, narrow down what is going on with him. In the mean time they are now trying to get together his IV meds so that we can go home for at least a few days, which could happen today or tomorrow. Lennon will have to come back weekly for lab work and checking in to see how he's doing.

Lennon's belly looks really good for a change. Some of the ascites has gone and his veins and vessels don't show as much anymore. You can still see a lot of them, which often reminds me of a map with a bunch of blue lines. Lennon is quite the ball of energy today, which is good but also nerve wrecking ..so far today he's done belly flops on the bed, attempted to fall of the bed and ran around in this small room.. yes I would say he's feeling good!

Of course that doesn't change the fact something is going on and something is wrong, we just simply don't know what it is ...yet. I hope that they find an answer. Now that they are talking about releasing us, it also means when the results come in ..depending on what they are...may mean being re-admitted for new and/or additional treatment.

This is all I have for now. Thanks for staying tuned to "as Lennon turns the world".

Morning folks, ready to be frustrated along with me?

Well here's the thing..now they aren't sure that Lennon has PTLD at all and send off some labs to the Mayo clinic and some local tests they can do to check for autoimmune hepatitis issues.. So to recap first it was either rejection or failure.. biopsy indicated that Lennon's liver has some dead cells in the center of his liver...why? Well because there is another clot in one of the vein/artery type plumbing...ok they ballooned that while Lennon was in biopsy..good..blood flow is going...there is hope that those dead cells regenerate ..the liver a marvelous and so important organ.
The two other sections show inflammation and/or infection, but Lennon has not or is not running a fever.. so there is lots of confusion going on there. Additional stains of the sections they took from his liver.. indicate that he may not have PTLD but some autoimmune issue...ok I am still confused on this and until results get back (sometime next week?) we don't know a much of nothing.

His ammonia is still up so they are wondering if Lennon has some oozing in the stomach and that blood that may be present can raise the ammonia..but I didn't notice anything so they are gonna add something to coat his stomach more ..in addition to the prevacid.

Overall, Lennon is good...just bored out of his mind I guess...stuck in this room (because we are still under isolation due to the VRE) ...so video games can occupy him some of the time... the teacher comes he's real happy and he doesn't want her to leave. So we play toot and otto and we read "the very hungry caterpillar" and allow him to play video games. He's feisty and he's "done" with it all I think. He hates them messing with him and will fight them and that can make it tough.

Anyway, until they know anymore about what might be going on, we will live here at UVA for awhile and manage life as best as we can.

Still kinda inconlusive

So the docs had their meeting today and it still seems somewhat inconclusive and they are waiting until all tests are complete. There are a few that won't be done and complete until tomorrow.. so the game plan for now is to change one of his meds from po (oral) to iv ..which is the valcyte...it may be that his body is not absorbing it properly through the stomach. They are also reducing his immune suppression medication a little bit, which is sort of a tight rope dance due to the fact that without it he could potentially reject the liver. So until they figure out the details and technicalities, we will be here in the hospital allowing the docs create their magic. Hopefully this method will work and in a few weeks we will be home, but for now we just hang out and try not to go crazy.

And hopefully we will have a more conclusive report tomorrow or Friday!

A more comprehensive report on PTLD

So I did more research on PTLD and found a more understandable and comprehensive report...

You can check it out here: http://transplantbuddies.org/library/ptld.html

Posttransplant Lymphoproliferative Disease

Here is what we know:

Out of the three sections they took from the liver in the biopsy, one of them has dead cells and the other indicate some sort of infection. It appears it is not at all what the docs expected and have a meeting planned for tomorrow. The thing that appeared in the preliminary results is Posttransplant Lymphoproliferative Disease(PTLD), however they are NOT a 100% sure that this is what it is. They also said to feel free to look it up but to not worry or scare ourselves too much because it seems that this (if this is what it is) than we caught it early enough, though I am not sure what that means either. In reading some information the treatment is additional medication, removing some medication or even chemotherapy. The doc will come by in the a.m. to talk to me to give me more information and then everyone will gather at 1 p.m. to figure out what exactly is going on and what are they going to do about it. Here is the link that I found that seems the most comprehensive about this disease http://emedicine.medscape.com/article/431364-overview.

The thing that throws me the most is that the article states its uncommon .. and it relates to the Eppstein Barr virus (EBV). Which I know those numbers have been screwy since September. One of the meds they have already removed because of this is the cellcept. I quote"
The more intense the immunosuppression used, the higher the incidence of PTLD and the earlier it occurs."

So if you want to know more, read about it but I won't know anything more of for sure until tomorrow sometimes.

Ugh ..seriously it would be Lennon who develops another uncommon thing. Well I will end this for now ..reading more about it..they did say not to think the worst when reading about the PTLD because Lennon is (if it is this) at the beginning stages. Again I don't know what this will entail for treatment nor can anyone tell whether it is from the donor liver..or it just developed etc etc.

Biopsy, Pick-Line and MRI

Lennon did well with the biopsy and they got three good samples. They also placed a pick line as he is running out of places to draw blood. In addition, they managed to get an MRI done which is good as that means we don't have to come back for another sedation. By tomorrow we should know some preliminary information from the biopsy and also some info about what the MRI showed.

Lennon is a little wet on the lung which comes from all the blood products he as received and they are gonna try and get rid of it with lasix. He sounds a little raspy and needs to cough a bit but he has a mind of his own. He doesn't want the pulsox (sp) on or the oxygen on his face. He is in little bit of pain (in the neck) as that is how they went in to get the samples of the liver but also his arm where the pick line went in.

Overall he appears to be doing well.

Uneventful Day

Today was uneventful...let me just say that Lennon without his tenex during the day is more than a handful (which he did yesterday) and with tenex he is a handful. What is tenex? Well, it is a blood pressure medicine like clonodine, but in children it works for behavioral issues with a less sedating factor like the clonodine. I think the tenex wore off around 3:30 to 4 p.m. which is sorta new but I think the elevated ammonia is contributing a lot and could affect some of the medication he's taking. He was able to play video games today which kept him occupied and mostly calm until I made him take some breaks in between. He was not a big fan but he dealt with it.

Lennon did acquire a cold which doesn't surprise me but hopefully it won't affect him too much other than just a runny nose. So in all we have just been hanging out trying to stay entertained.

The biopsy is scheduled for 10 a.m. on Monday which means tomorrow they will do a full lap work up and see what he may need as far as plasma and platelets. And then it will just be another day that we will hang out.

Overall he is in good spirits and coping being cooped in the room. He's still under isolation because the VRE is still lingering and has not moved on to better things.

I know some folks are concerned how I am holding up, as well as everyone else. I'm ok! I am just taking it minute by minute trying to get through the day. I don't want to speculate on the outcome of the biopsy too much because that wouldn't do me any good right now. I am taking things as they come. I've been trying to keep busy while Lennon keeps everyone else occupied.

Tentatively we may have some preliminary results on Monday but a full report towards the end of the week. They will keep Lennon for an additional day or so for observation to ensure he doesn't start bleeding and is handling everything okay.

Lennon steps here we are and go!

Plan of action

The ammonia is back down in the 90's and Lennon is acting relatively normal. He is under isolation due to the still existing VRE virus. After a communication break down that is now fixed they have added medication that should get rid of the ammonia in his intestines. They do not think that the UCD is re-emerging but rather that he is having some sort of liver failure. The word is to watch Lennon closely because he can tip over at any point with quite apparent symptoms of chronic liver failure.

The game plan is to keep him in the in the hospital to watch him.
Today (Friday) they are going to get labs for screening to see what it is today.
On Sunday they are going to check his factor levels (5 and 7 I think are the ones low) and try to stabelize with platelets and plasma. But also check other levels such as cbc and ammonia.
They are trying to schedule the biopsy on Monday which means coordinating with radiology and anesthesia, and again check the levels on Monday to see where he's at. He may require some more platelets or plasma but will need the biopsy within hours of receiving that.

My heart aches because he does look good but the elevated ammonia indicates there is something going on with the liver. He is active and busy and hates being stuck in the room. However, he is being treated as acute and critical because we know he can tip to the worst at any point. I will not speculate on the results of the biopsy and hope for the best. It is minute by minute right now.

The develoments of the day

So after the last ammonia being 99, a doctor appointment was scheduled with his gastro dr. as well as hematology. Initially I was supposed to be at my practicum site but felt I needed to be in the office today with the docs as there were some questions I wanted/needed answered.

Ultimately, levels ammonia is of concern (many of you know this from post past), but also given the fact that his factor levels are low which of course influences his ptt and inr numbers (the risk of bleeding/not clotting). The conversation is barely in my head but this is what it boils down to:

Lennon was admitted to the hospital to monitor him, his ammonia and his factors. In addition, he is looking at a liver biopsy because the concern is that a) he is having low grade rejection or b) because of all the clotting issues and thrombosis of some of his veins that the liver is slowly dying. Of course I don't want to jump the gun or conclusions and am just taking it minute by minute. However, just an hour ago I found out that his ammonia is now 164. This is pretty devastating news.

Without jumping ahead too much, Lennon may only receive one more transplant to save his life, but this decision will NOT come until after biopsy results. The chance that if the third one would not work and he would get a fourth one are not great. This is pretty harsh ~ but I get it.
I also wanted to mention that the increased ammonia does not necessarily related to the Urea Cycle Disorder but directly to that he may not be able to absorb the required medication for the CPS Deficiency or the other already mentioned issues.

At this point - with reality in my face - I am taking it one second at a time because to do anything else would not benefit me or Lennon. I do ask for prayers, positive energy and good grief I don't know what else. It is a one second at a time that I can function while maintaining strength for Lennon as well as his siblings. I am currently quite numb and in shock and think that it won't hit me until after the biopsy.

Please know that while all of this medical stuff is going on, Lennon is funny, he is playing, he is awake, he is loving and he is ultimately being 6 years old.

We always knew that

Lennon's ammonia was 99 yesterday, but I didn't get the phone call until this morning. We knew something wasn't right because he was tired, aggressive and emotional (sign something is wrong). So we went to UVA and had his blood levels checked and like I said I didn't get the results until this morning. I suspected as such but I guess part of me wanted to be a fluke or something else. I am a little distraught over it, because he was doing well, the transplant appeared to have done the trick but at the same time we knew it was not a 100% cure. Of course a level of 99 is better than the numbers his body has seen in the past, but with that being said, if he has a healthy liver than any number above norm would now just be as effective or defective (your interpretation) than on anyone else.

This morning I had to keep him home from school and scramble to get his private duty nurse in here because I had a meeting to attend. The issue is had to school agreed & approved that the nurse could go to school, than it wouldn't have been an issue..she could have just met him at school. I kept him home because the dr. wanted him on a 24-hour pedialyte maintenance. I don't trust him going to school being hooked up. So I am a little frustrated because this would have been an opportune time that he could have used a nurse in school. On the other side the company that offers private duty nursing says that if I want a nurse in school ...well that can happen...so they are going to figure all of that out and we will move on from there..

Lennon has another appointment tomorrow with Gastro as well as hematology as he still has ongoing blood issue and balancing the blood thinner with the blood thickener is a tough one...so the lesson ...I don't know.. it just appears that ones again when we begin to relax because things are going well...something goes awry.. I need to remember one day at a time..one second a day