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

And he has the power....


to convince the docs he doesn't need much..just time :o) So the other day they were talking about the video-scope procedure to help Lennon get cleaned out from the fluid. In the mean time. the TPA'd the chest tube and voila it drains again. The x-ray between yesterday andtoday...night & day...black & white. Everything that was white, that shouldn't be white isn't white anymore. YAY lennon, because he has the will andthe power. He is off the C-PEP and on the small nose oxygen and his numbers keep looking better. He is kinda out of it because he has to take methadone due to the narcotics he had been on and to help him with withdrawls. He is resting very nicely at the moment. One can notice that the fluid is leaving as some areas of his body that were swollen are not longer swollen, or at least not by much. He has to become more active and mobile, I think to help the draining situation. That is what I have for now, this minute. So as always stay tuned.

Carbamoyl Phosphate Synthetase Deficiency or CPS for short


We received an official diagnosis today from his liver biopsy. The specific Urea Cycle Disorder is called Carbamoyl Phosphate Synthetase Deficiency (CPS).

"Carbamoyl phosphate synthetase (CPS) deficiency is a urea cycle defect that results from a deficiency in an enzyme that mediates the normal path for incorporation of ammonia" (Roth, 2007).

emedicine website Roth, K. (2007) retrieved from http://emedicine.medscape.com/article/942159-overview

Frequency
United States

CPS deficiency is rare. As with all the urea cycle defects, as well as most of the inborn errors, citing incidence figures is impossible because new cases are generally diagnosed randomly without the benefit of population screening.

Mortality/Morbidity
Mortality and morbidity rates are high. Untreated CPS deficiency is likely fatal.

The multiple primary causes of hyperammonemia, specifically those due to urea cycle enzyme deficiencies, vary in manifestation, diagnostic features, and management. For these reasons, the urea cycle defects are considered individually in this article; however, hyperammonemia is the common denominator and generally manifests clinically as a common constellation of signs and symptoms. As a consequence, the most striking clinical findings of each individual urea cycle disorder relate to this constellation of symptoms and rough temporal sequence of events.

Symptoms include the following:
Anorexia, Irritability, Heavy or rapid breathing, Lethargy, Vomiting, Disorientation, Somnolence,
Asterixis (rare), Combativeness, Obtundation, Coma, Cerebral edema, death

Other websites:

CPS http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=237300
National Urea Cycle Foundation http://www.nucdf.org/index.htm

Fluid...Fluid...and more Fluid


So, Lennon has had the breathing tube out but has been on the C-Cap (?). Today they did a CT Scan because the chest x-ray indicated that he is FULL of fluid as it was completely whited out. And so yes he has a bunch of fluid on his right lung and hence the difficulty he has recovering and well breathing well on his own. His CO2 has been quite high, high enough to consider putting him back on the breathing tube. Sigh.

Well, it seems they might be doing a procedure in which they will go in with a small video camera and suction out some of the fluid pockets, place a new chest tube or something to help the removal of the fluids.

If we can get the fluids under control, everyone is pretty confident that he will recover fairly well. His liver is functioning, blood looks pretty good and his kidney function is doing well.

I know more tomorrow about when and if they are doing the procedure to remove the fluid.

Off the breathing tube


Someone's thoughts and prayers have been heard. Lennon is off the breathing tube, but he is still struggling to breathe as he is still so full of fluid on the inside. He has a mask on his little face that is a bit big, but gives him the oxygen that he needs. It appears that his body and in parts his mind, simply are taking their time to heal and recovery from everything he has dealt with so far. Every now and again his eye will peek open and then he goes back to sleep, his hand will move in hopes to find another hand or his beloved cat that he has had since he was three months old.

The other day, I was asked if I regret the decision to have the transplant. I don't think I do. The alternative looks far worse than what he is dealing with now. Living with an unknown type of Urea Cycle disorder, without indication that the diet and medicatio appear to be enough could have a much different outcome. In reality it could have meant more brain damage, it could have meant coma, or it could have meant death.
I like the alternative of life much better and if I am to trust these doctors as much as I have so far, well then Lennon will be ok.

All of his numbers (liver, kidney and blood) look good, probably the best they have been. His ammonia has been 31, which is the lowest that it probably has ever been in his lifetime. One has to keep the faith and hang onto something to belief, to belief that he will be ok, he will walk out of here a whole new little person.
I have to believe that he is destined to be on this earth for some time and that it simply is not his time to leave. Not now and not for awhile. Without these thoughts, faith and trust I would not be able to hang on, and my demise would be his demise.

The last five weeks probably have been the hardest in my life, in his life (maybe) and in anyone's life affected by this little guy. It gives to show that every day matters (in the words by Danny Gregory, its also a book title and a good book too).

Viruses are terrible


So the fluid pocket that Lennon had behind his liver definitely has a virus. It is called VRE (vancomycin-resistant enterococci) and is resistent to most treatment and is usually contracted by people who have had surgery, have no immune system etc.
So they are giving him antibiotics but it can take quite some time weeks to months before this virus is out of his system.

Other things I know about VRE (which you can read on the links below): is that is passed on from health professionals (ARGH) and/or when a specific antibiotic like vancomycin (hence the name) is used...which Lennon has been on quite a bit over the past few weeks.

He is on contact precautions i.e. nurses and doctors have to wear additional yellow gown and gloves before entering the room.

Washing hands is critical for sure so that it is not passed on to others.

More info can be found here : http://health.utah.gov/epi/fact_sheets/vre.html or here : http://www.webmd.com/a-to-z-guides/vancomycin-resistant-enterococci-vre-overview

He is still on the breathing tube but that may come out today or tomorrow. If I had a guess tomorrow seems more likely.

Peace!

Infections...


...can creep up in many ways and bring to light many other things...

so Lennon had an MRI on Tuesday for his brain...its fine!!

Then he had an MRI on Wednesday on his belly, because he had been vomiting, labored breathing and appeared in pain..this on not so fine... he has a 6cm fluid pocket/absess behind the liver...

They placed a breathing tube in on Wednesday during the MRI, which he is still on. The main reason it was kept on, was because they did another procedure yesterday - place another draining tube in to get the fluid behind the liver. They saying it might...but only might come out today because he is so puffy due to excess fluid in his system (approx. 2 liters) that he might not do so well if the take him of the machine. He looks a lot better today then he did yesterday - concidering his puffyness.

So, the fluid behind his live is infected - this is because the liver was a piece of cut liver and when it oozed in the healing process some particles stayed behind. Now the fluid and those particles have created a nice infection.

Also, Lennon has CDef - don't know the accurate spelling - but it is related to his still - the bad bacteria in his intestine has taken over the good bacteria.. and so he is on contact precautions as that appears to be contagious. This happened due to an antibiotic he was on (bactrum) and to what is going on outside - flu season etc etc

What else... oh the draining fluid from the original surgery also shows bacteria - thus he is getting antibiotics...today he will be getting more specialized antibiotics as opposed to one that covers the whole spectrum.

So let's hope the breathing tube comes out today and that he starts to feel better. His electrolytes have mellowed out as they have been pretty whack ...his ammonia which was 140 yesterday is 81 this morning...his blood levels and his liver numbers look better...

So here is to Lennon feeling better and being himself more and more each day!

The rollercoaster that never ends


So, Lennon spend but two hours on the floor before he was transferred back to the PICU. His potassium was way high and they were worried about seizures. So, they watched him and determined they need to work on his electrolytes. On Wednesday night, Lennon took a dive of sorts. He became mentally incoherent and a CT Scan was ordered to make sure there wasn't any bleeding on his brain due to the added blood thinner. Well, the brain is fine, so the thought was that his CO2 was just out of whack. Lennon quit eating, drinking, everything was no, he didn't want to walk, he didn't want to do anything. His mental state did not improve either, thus he has been saying mommy nonstop for 3-4 days now. Yesterday, to help him they though they would take the draining tube out but his oxygen in his blood dropped and so a chest X-RAY was ordered. Fluid on the lungs!!!!!!! So they decision was made not to take out the tube but instead place a new chest draining tube in to help him release the fluids. Within an hour after the procedure he wanted a Popsicle. Well he ate about half of it. Thus given the indication that the fluid made him not hungry, made him nauseous and might be the reason for his mental state. If he does not start eating today they will feed him with a nose tube because he has to have food in order to heal, this is the wonderful thing about protein, though the concept of protein and Lennon still makes me nervous. And yet his ammonia has been in the 50's-90's. It is unreal.

My frustration point came yesterday because no one would tell us anything. No one would come talk to us about what the plan is, what is going on with him, why he is acting the way he is and what they are doing to change. The nursing staff has been awesome with the exception of a few here and there, which that happens in any profession. But what frustrates me is that the PICU team and the Transplant team are not always eye-to-eye and often may not agree with what needs to be done. I thought that if Lennon had stayed on the floor, which he truly enjoyed he may have improved on his own. Because there is more freedom to be about and well be somewhat normal. In the PICU you are stuck in bed, except that Lennon does have permission to get up. But one has to keep in mind that he has many hospital visits, both on the floor and the PICU, he kinda knows the routine and I felt like he was depressed because he was again stuck in bed and could not be as mobile as he wanted to be. At the same time, I am ok with him being in the PICU and that it somewhat appears that they are doing everything to get him better. But I also wonder, if they should have done an x-ray before yesterday but his oxygen didn't give away any reason to. I wonder if there was something more they could have done to catch what is happening now. So ultimately I wish there is more communication with us about what is happening, even if it is just a smidgen of an idea about his mental state or him not eating. I wish that both teams would be more collaborative and come to a mutual agreement. One doctor told me that the transplant team rules Lennon's care, but they are not the ones constantly with him like the PICU team. Indicating that they PICU team might have some input that should be considered. I don't mean this to be a negative venting session, but this is my place to vent my frustrations and upkeep of Lennon's care.

Overall I am pleased with the hospital, and I am pleased with the care he is receiving. Now I just wish that he would get better. This has taken longer than anyone anticipated I think, from the surgeons team to the PICU to us parents.

Some answers to some questions...


Yes Lennon did not like some foods - usually those high in protein. I was always left wondering why he wouldn't eat chicken nuggets, fish sticks, mac & cheese. In addition there are lot of veggies he stayed away from corn, peas, carrots and other ones. He has always been big into starches (potato and rice) and no he does not eat spaghetti either. It seems as though he always knew about the food that would make him ill - run by instinct - perhaps even a certain smell? I don't know but it is pretty amazing that he ultimately knew what he could or could not eat. I think a big part of him not eating right now is that his body is going through this major adjustment. It's a new him, and perhaps new feeling about self (physically and mentally?). If we think about what the ammonia has done before i.e. drive him crazy, make him irritable etc. the calmness might be kind of weird to him?

About the warm/cold feeling...I have ready somewhere that individuals with UCD will be cooler than normal as the liver is not able to function fully as there are enzymes missing. So my guess is now that he has a full-functioning liver and the enzymes have been replaced (?) he now has a normal body temp. For me that was just the neatest thing!!!

As far as for the water - the big issue was that he had been so full of fluid previously and they didn't want him to "swell" again. In addition, too much water will lower your sodium (?), well this is the reason I was given. He now is drinking Breeze - similar to Kool-Aid just with nutritional elements.

Well, and so I am ending this with a positive note. Two of the draining tubes have been removed today and he was also been moved out of the PICU. He will require two shots a day for clodding issues, as well as his regular meds for the transplant (steroids, anti-rejection etc). So who knows by next week this time we truly could be home. Well, honestly all they have to do now is get his dosage correct and remove one more draining tube. Home for Christmas?!?!?!?!

Thanks for reading my blog!

The bumpy road continues....


...yesterday another ultrasound was done to determine what the fluid pocket did as far as reducing it since the tubes were placed. Well, nothing ever goes as smooth as you hope, because they found that the portal veine (??) was clodded. They can't do surgery on it, and said that this can happen from time to time. If it causes problems later on down the road, it means he may need another liver transplant but we should not concern ourselves with it too much. Generally, patients are placed on heprin (which he is on right now) for about 6 months, but the transplant team is going to contact the blood specialists to see if this is alright for a five year old.

Over all everyone is very pleased with the progress and make a big deal about how good he looks. My biggest thing is, that he is WARM!!!! Something unreal to me as he always was a bit colder to the touch than the norm.

He is also not eating and drinking as well, which btw could be part of the clodding issue. And when he does drink, it is water, which they don't want him to have too much of. But that is what he likes, plain ol' water.

And our journey continues...

On our way to normal...


We are moving towards recovery. They want Lennon to eat, drink and be jolly? Well, this is not happening fast, I can only imagine how bad his throat hurts. He is not talking and also not eating well. So now we become creative - oatmeal (one of his faves) might do the trick, hmm someone mentioned milkshakes. Wow, a milk shake...something he couldn't have before - does he even care for them is the question.

On the way positive side, his liver is functioning!!!

Do you ever wonder...


...how much a body can take?
Lennon required additional draining tubes as he accumulated too much fluid around the abdomen and pulminary section. Kind of a scary thing but his white blood cell count went up, indicating he might have an infection. He has been taken antibiotics for several days. There is still a lot of fluid around his little body and so he continues to take meds that help reduce it. Overall I think however he is doing well. His ammonia...ahh its in the 60's today...his body really doesn't know that number (much). Other levels are also coming down indicating that the liver is sloooowly starting to do its job. YAY!
He is awake, and though can't talk quite yet handles everything pretty darn well. What a trooper dealing with the restraints, the oxygen going up his nose, draining tubes on the side of his body and several IV's. He is certainly a hero to many people and I am amazing how strong his will to live is. I am partially relieved now and even though its still kind of a touchy situation feel alot better about everything. There is no game plan on how soon he will go home, but there is hope that it will be before christmas.
I decided that my tree was not going up until he goes home.

The tuff part is that I am going back to work tomorrow. However, I love the place I work for and the people are great and my supervisors are pretty awesome in which they will work with me. Some people that I work with have donated their PTO time and what a relief that is to me, I still get a paycheck! So thanks to anyone that has donated some of their hours to me so that I may care for Lennon in his recovery. You people are the best!

Be thankful for all you have in your life, take nothing for granted. Know that people care, and it doesn't matter how close or far you are from them physically or any other way.