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

Teeny Weeny Update


So, Lennon was awake all day!! He feel asleep at 7:30 but was back up by 9 something. So I feel like the changes we made with the formula have made a difference! Of course the nutrionist and some of the other folks still feel he's not getting what he is supposed to have ..again according to what 5 year olds should have. I can't stress enough that he is not the typical five year old. My partner, calculated it up that prior to transplant, he never took in more than 900 ml of fluid, and that is diet, well you know he snacked all day long, there was merely no set schedule for him in food intake because we trust Lennon that he knows what he needs...it is a known secret that children are well aware of what their body requires, and that they will eat accordingly. Alas, while I still cook with the food pyramid in mind, that does not mean that younger children will eat all what is on their plate. It's a key element to trust your children's instinct, ok well at least for me it is.

He's mood has improved just in the last two days..again since we made the changes in formula and not harping on him to eat like they wanted us too. Of couse he is not eating what THEY want him to, and may want to make changes in a couple of days. He is low on vitamin D, calcium, phosphate etc etc etc ..well there are supplements for that eh? This of course could by why he is complaining about his leg hurting a bit..my guess is that all these therapies while working on getting him stronger are hurting him a bit...well if I was to go workout today, tomorrow I would be sore... see makes sense to me...

I am glad to see him be funny and play. Last night after he woke up we played with his bouncy ball, and he actually had a real laugh. I think that some of his muscles still kind of hurt so it was not quite the laugh I know, but boy this laugh was just as good. He showered me in "I love you's" and while before that would have been tooooooo much on me, I now cherish them.
I do recognize that he has regressed in some ways, and that some things appear a bit difficult. His healing and recovery will take years to come in many ways, but we take it.

A follow up on my previous rant


So, after talking to the psychologist and voicing my concerns about Lennon's mental well-being (hey I study mental health) the conclusion is as follows:

We need to worry less about his oral intake! We need to focus on getting him mentally well, alas so that he will slowly recognize that he is healthy. His body is no longer ravaged by sickness as it once was. He will learn slowly in age appropriate manner why he has a scar on his stomach and why his hair fell out. He will learn that his stay at Rehab is temporary and not permanent. No longer will we push the issue about food, it there is a medical issue with nutrition the NG tube will take care of that!

With that being said, I wanted to say this as this was brought up in a comment! Yes they had standardized Lennon to what a 5 year old should be taking in. Error # 1! Lennon has never been the norm. He always controlled his food intake, when he said he was full...guess what, he was full. If he didn't like something, he didn't like it, bottom line. So the focus will now be to get him physically stronger with the assistance of the NG tube, but again not to make eating and food the issue. It will require a long time and therapy before he will eat like a normal child. And if this means that in the mean time he as a G-tube placed, then ok I can go with it. But because of his immune suppressants and current state of health, it may not be the best option to put him through another surgery.

So, the matter is now getting everyone on the team to recognize that Lennon's mental health has to take priority. He has been sick for five years, and has had 25 hospitalizations or so in the last 1.5 years. All he knows is being sick. He doesn't know what it means to be well and healthy. So I think the goal will be after March 15th, he may come home with the NG tube as is recovery is not only physically but mentally, and this takes nothing but time.

What is the right thing to do?


So I want to start this today with the positive points that have occured since we transferred to Kluge. His strength and his mood are slowly improving.

Now to what is happening that has me in tears and leaves me with frustration:

When Lennon was transferred, he was on KidsEssentials and began to eat, slowly but eating nonetheless. It may have not been about nutrition, but really allowing him to establish a relationship with food. Then he was getting too much Calories through that and his formula was changed to Jevity 1cal, every four hours and he started to eat less and less each day.
Now my thought is that if we want to encourage him to eat then we need to schedule the feed differently. I know he was only getting about 45ml, but for a child who has never had a good relationship with food, nor has ever eaten a what is considered to be a healthy diet, has been conditioned to not eat certain foods (alas high protein) this was a lot. Prior to transplant, we struggled to get him to eat, keep up with his calorie intake, minimize the protein intake which did not give him many options, well options he did not want. He was a french fry- potato chip kind if kid, and we managed to find soups that he liked and be creative with them.

Now they expect him to increase his food intake. Which with a person that has a negative relationship with food, and was trained not to eat most of what is considered to be healthy, this is not going to happen overnight. At least this is my point of view.

For the last few days, I felt that we have not been heard. Certainly I don't want them to stop the feeds because he needs the calories and the protein, I get that. But if we want to encourage him to eat then we need to schedule them after breakfast, after lunch and after dinner, so that he is given the chance and opportunity to be hungry, and then to eat. So we were able to narrow down the schedule - 3 times a day. What they want however is to give 240 calories (he needs about a 1000 a day), with protein, but alsos give him 900 ml of fluids (water). This means he is getting 300 ml of food/fluid three times a day. My thought is that this is not going to encourage him to eat, if anything he will never be hungry. Our objective is that we do not want to end up with a G-tube in his stomach. For one, he can learn how to eat, but it won't happen overnight; 2) infections happen very easily with a G-tube since there is a hole in his stomach, and dirt is going to get in, and messing with it by Lennon himself, and cleaning it, yadiyadi. Since he is on immune suppressents he may not be able to fight of these infections that are likely to occur.

So today, we are doing 100ml of KidsEssentials 3 times a day, and no more water into the NG tube. We established that he can drink water and gatorade and thus get his essential maintenance fluids, which 900 ml is 2/3 of what he is supposed to have. We did this because the last two nights in the evening he vomitted - a good indicator that what they were doing is too much.

I have been frustrated because again I felt like I was not heard. What part of he never had a good relationship with food didn't or doesn't get through to them? But the speech pathologist that deals with feeding/eating issues will not work with him until he is stronger. There has been talk about sending him home with the NG tube, and if we would be ok with and willing to place an NG tube through his nose. WHAT? First off, I would not want to do that, this appears almost cruel to me, if I did it or anyone else who loves him and cares for him, I'd rather have a trained RN come to my house and do this. Secondly, while at UVA and Kluge, everytime they placed the NG tube, they took X-Rays, umm I don't have an X-Ray machine at my house. Certainly I know you can listen to the stomach and ensure that it is in place, but still....see my problem?

Yesterday and today I have been on this emotional roller coaster because we were not being heard, and then when we were heard or so we thought, they still "changed" it up doing their own thing. This is family centered care! Also, I felt they were not giving things enough time to work them out. Like the feed schedule that he is on now, he will be on for 4 days, and then we revisited and reevaluate to see what has changed if anything has changed. We have also been asked to spoon feeding him as he probably gets fatigued really quickly, when he has to do the spoon feeding himself, and then chewing etc. We do not have an opposition to that. But then other people are worried that we would force feed him. What? We had to feed him for the last 2 years to get him to eat, well on an off since he was born but more since his diagnosis of the UCD. When Lennon would say he is full, we would stop.
Which brings me to the point that Lennon does manipulate some of his environment. All kids do this, some more than others. But when he says, "I'm hungry, you need to turn the machine on", well that indicates that he recognizes his hunger, but perhaps does not want to do the work?!

I really think he needs to be worked with, I know that his eating and his relationship with food has to occur gradually, but I wonder if they sometimes get it. Again I reinforce the point that he has never had a good relationship with food, and he has never eaten well. I know that they are going by the developmental norm set by the standards in to how much a 5 year old should weigh and what they intake should be, and again I have to say....Hi, this is Lennon, he does not follow the norm, he is not an average kid in which the standards and developmental norms apply to.
As a matter of fact, his brothers do not follow the norm - his oldest brother is 5'9 and does not weigh what he should, he never has, he always has been tall'n'skinny.
Lennon in the past has been considered to be overweight, umm part of the Urea Cycle Disorder is that development is not going to be what it should - alas he was short for his age but up on the weight. Go figure.

I sometimes think that they forget he has had a few major surgeries, has not taken much orally since November and that his stomach is not the size that it used to be and this too will take time and is a gradual process. Next week we will have another family-care meeting and set up some specific goals, which this may mean he will come home with an NG tube and we do feeds at home. I do not have an issue with that, I'll just have to find the resource to help place the NG tube when it needs to be done, and no one should think that I won't take him to the hospital and say "hey this needs to be placed".

I am encouraged however that his strength and stamina are slowly coming back. In the fist week, he was wore out but 12 p.m. and ready for his nap. This week he stays up until 2 p.m. This is progress.

I will end this post with this:

Hi, my name is Petra. I just had a baby, his name is Lennon and he is 5 years old.

An appreciation to the readers


I just finished posting the latest update, and reviewed the blog to find another comment on Lennon's progress.

I wanted to say that I appreciate the support and the comments, letting me and him know that people care. Moreover it has given me feedback, it has provided me to throw out some questions at the medical team, it has given me insight and strength. I appreciate the insights about personal experience because sometimes you just feel alone in your situation and forget that other people have gone through similar situations.
It has been a difficult journey, which is not over - but realizing that just a month ago he was so critically ill we didn't know if he was going to make it - though I certainly was not going to think otherwise.

The comments, no matter how big or small as they are have been and are always welcome. Often times they have lifted my spirits a bit.

So, a thanks to you reader

Game Plan for Lennon & Update


Feeding/Food : needs to be worked on and worked out, start slowly with the food he likes prior to transplant to establish relationship with food - he also may be too tired to eat

Narcotics: are wheened alternating days 10% - this will take about 2-3 weeks before he is off that stuff

Speech: doing better - has some gaps - is at a level of a 3 yr old - but not a complete assessment has been accomplished yet due to lack of cooperation from Lennon

Physical: Doing better - gets fatigued real quick - but improvement is there

OT : more active today - some self-help skills need worked on

School: making some progress - vision is good - perhaps some depth perception issues - again takes time to warm up

They also want to address socialization because he appears to be a little too shy - more than he was before - Time frame to be at Kluge 2-4 weeks - depending on food intake and progress - if he's still not eating like he they want him to - may or may not go home with NG tube - avoiding G-tube as best as we can - may introduce meds for increased apptetite

Changes


There have been some positive changes for and with Lennon. For instance he walks more steady these days, but still has some balancing issues that only will go away over time as he gets stronger. He's new found love is the lil trike they have that can be controlled by Lennon and the Physical Therapist, he always has a blast riding it around the unit.
He's enjoying school, speech and occupational therapy as well as some therapeutic recreation. He has been able to go outside and enjoy some of the slightly warm day we had, walking but also again riding the trike.

Eating is still somewhat of a challenge, and there appear several factors that are related to this situation.
  1. He has been soo conditioned not to eat food such as anything with protein.
  2. He had the breathing tube several times, and then for a long period of time
  3. He is not used to the muscles in his throat also evident by his still quiet talk
  4. His taste buds could be off a bit, some of you remember the spicy foods he liked > Mustard
  5. Developed an aversion to food due to vomiting from sickness and withdrawals

There might be some more issues but this appear to be the main ones that make his eating problematic. So now he has to build a new relationship with food, one that is pleasant and allows him to enjoy it, but also be nutritional. By the time he will satisfy the docs, he will be ready to go home. So for now he gets to eat what he wants, but is fed via NG some KidsEssentials to give him the nutrition about every four hours. At some point this will change to three meals a day so that his body will get used to being hungry at the right kind of time.

In the mean time we are working out his medication schedule, currently he takes about 16, with the majority of them in the morning and then again in the evening. Some of them he takes three times a day. Some of them he will not have by the time he goes home. But its important to get on a routine that works for us at home, which has been quite challenging at times.

Ultimately his mood has been improved drastically. He appears more chipper and happier most days. He is still kind of shy around all the docs, nurses and therapists but since most of them are the same every day, he appears to warm up slowly. He has started to joke around a bit more, laugh and giggle at things he feels/thinks are funny. Its good to see him going back to the Lennon I know. It is interesting to note that his previous severe ADHD issues are currently not present. He is no longer figity and does not bounce all of the place. While I don't think it is completely gone away, I do think that A LOT of his behavioral and cognitive issues were related to the flucuating levels of ammonia in his system at any given minute in a day. It's good to know that the transplant did more than just save his life, it has given him an opportunity to live as much of a normal life as possible.

He is still facing some challenges for years to come but I am excited about his new life that appears to be right in front of him. Though as I write this, I still feel hesitant and reserved as rejection can still occur at any time if his levels of medication in his blood are off or not enough. I want to believe that the worst is over and that while he may still have some hurdles from time to time, none of them are going to be like anything that he has gone through in the past three months.

Thanks for reading

Settling in


So we are transitioning from the hospital to Rehabilitation. It is quite a change in environment, and what a change (already) in Lennon. He has been walking, with help as he deems necessary. He is loving the wheel chair rides, he is wearing clothes and he was outside!!

Tomorrow his hard work will begin with Speech, Physical Therapy, Occupational Therapy and Therapeutic Recreation.

They will also change his feedings to a more normal schedule. Currently they are feeding him at night, in hopes he will be hungry during the day, which appears is not panning out as they had hoped. Thus, they will feed him at breakfast, lunch and dinner! Makes sense to me.

So here is to more progress!

Physical Therapy


" I want to go walking" he said.

"ok but we need to wait for your meds first", I replied.

"Oh, good idea" he said.

He is just awesome and funny :o)

Well, today we are being transferred to KCRC for Physical Therapy. So while it still is a medical setting, we are in away being discharged from the Hospital. Wow. There were many days I thought this day would never come, many days I thought I might never see him smile, or have conversations with him. And today, he appears in a good mood, is having conversation (with me, lol).

He is still not eating, but is fed at night with an NG tube so that he gets his nutrition. We are slowly working on transitioning to food, which is a bit tricky at times since we now don't know what he likes. Before it was what he could and couldn't have, now its we don't have a clue and have to begin the process about food just like he was a baby.

Of course, I was reminded that there still could be some bumps in the road, this means another narrowing of the hepatic vein, rejection of the liver, fluid collections those kinda things. I think I was aware, but sometimes you just don't want to think about that. But reality is that we have lived in and out of the hospital since his diagnosis, and so ironically this is what we know. We don't know what its like to not have to go to the hospital.

Have a great day!

One step closer to home


The word on the block is, that the call is being made for Lennon to be transferred to Kluge for physical rehabilitation. He will require a feeding tube, so that he can be fed at night.

Cake with Icing


Today Lennon had another tapping of the right lung and the angio to balloon his hepatic vein. Everything went well today, but they couldn't put a sting (sp?) into his vein. The reason is that he is still growing and developing and so the veins are growing and developing with him. So I hope this is successful, because if not he will be looking at surgery to remove the clot in his portal vein and fixing his hepatic vein.

On the good side, he had a piece of cake with icing. For about two years, or rather since his diagnosis, Lennon has not been able to eat cake due to the protein in the cake mix. He had about five bites!!!

He does have to start eating regular food because if he doesn't he will have a NG tube (nose feeding) placed so that he can be fed at night while sleeping. This will not prevent him from going to rehab, like the TPN and Lipids would do.

While things are better, they still don't appear to stop. I do hope that one day, I can say on this blog that we are going home!!!!

The newest information


So we have been out of ICU for some days and here is what progressed...Lennon had no interest in food, so the docs wheened him of TPN and Lipids and today they stopped them completely, in hopes that he would start eating.

On Wednesday his sats dropped and an X-ray indicated that the right side is with fluid, and so a tap was done on thursday. In addition, an ultrasound indicated that his hepatic vein had narrowed again and that is probably the reason why his right side was with fluid.

This is the game plan, on Monday they are going to balloon it again, this time bigger than the last (stretch the vein) and possibly put a stint in it. This may require the breathing tube during the procedure, but my hope is that it is only for the procedure and not long term. If this works, which it should (cross yer finger and toes) and he does improve as he did the last time, we are heading to KCRC Rehabilitiation for physical therapy.

Of course I am worried tht this won't go as good as everyone hopes and we return to the PICU, my hope is that it works and we are here for a few more days and outta here.

He did start eating a little, moving from potato chips to some peaches!!!

And he walks....


Today Lennon walked (with assistance of course) 10 feet, from the bed to the sink. After 3 months in bed that appears to be quite an accomplishment. He also had a bath today. Yesterday, the PCA asked if he wanted a bath, unbeknown to her, this is his favorite thing to do. Well she only brought a tub that is meant to wash the head in, in bed. Lennon did not want to hear that, so today we filled it with water, and he sat in it. Certainly not what he as used to, but it provided him with some comfort for the moment.

Now he's sleeping, and has been asleep for some time. The walking and the bath, or any other activity he may participate in is still quite exhausting to him. Heck I get tired just watching him work so hard to do the things he used to do. While it will take some time to get to 'normal', I think Lennon will get there sooner than anticipated by most.

The one thing that is still a concern (to me at least) is that he is not interested in food. He eats about 3-4 teaspoons of applesauce at each meal time. Which I think this is a combination of things going on here. A) he hasn't had real food in three months, and b) he is still on TPN and Lipids that provide nutrition through the IV. I think that if they turn off the IV and give him a chance, perhaps than his appetite will increase a bit and he will start eating. Of course this is only my own rational logical explanation. He is drinking however, he loves his water!!!! And is not interested in much else.

It is good to see him smile more and be playful. It is good to see him demand his time with me but also acknowledge that he needs his own time. It is good to see that he wants to do things, but its difficult to explain that he can't because he is under contact precautions (such as go to the play area), and because he physically needs to be more careful still.

All in all, positive progress is happening!