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

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

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 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.



...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.

Could today be the day??????????

We hope! Lennon is doing better. His liver function is slowly becoming more normal. His kindeys look good and his lungs are way better. Currently they are weaning him of the breathing machine so that he is required to breathe on his own. So if he does well today could be the day that the breathing tube is removed, if not we will try again tomorrow. He still has a long recovery ahead but everyone (from the professional perspective) is pretty positive and confident that things are definitely and finally heading into the right direction.

Some of you who follow this blog, sorry for not calling etc. but I know everyone understands that funds are tight, time is stressful. I do appreciate everyone's positive thoughts and energy in my son's recovery.

Mom's don't like setbacks!!!! And we need breaks!

Monday afternoon they took out the breathing tube...but it appears that Lennon was not ready. He struggled most of the night, his Oxygen being in anywhere from 67-91 and the CO2 getting higher and higher. While I consider myself a strong individual on many levels, I should never watch them take the tube out, because I watched my son not breathe, and that is the worse feeling ever. Tuesday morning they put him back on the breathing tube, because he struggled too much.
One of his lungs had partially collapsed and there was some fluid that was interefering with the normal breathing process. His lung is reinflated (breathe) but ultimately Lennon was not ready.

He will do things on his terms, the way he wants to when he is ready. This is Lennon!!!

So yesterday I went home and hung out with my other kids (more or less). I think the important part was I was there for them and available if they needed me. Plus I needd to rest. I was close to if not exhaustion that could have hospitalized me, but then I wouldn't be any good to Lennon. When he wakes from his sedation, I need to be able to support him and be there for him.

The support by our community has ben overwhelming but we certainly appreciate everything and anything that comes our way. The thought to make things easier for us is so unreal because in the last year or so, we did everything and anything ourselves. We certainly realize that now is where we need the help, but we are folks who have a hard time asking. Call it a pride thing, call it what you will. Nonetheless, we are gracious and thankful.

If you want to help go to hippiescreations.etsy.com ...this is one of my stores online where I sell my artwork. Thanks!

Stay tuned....

Things are looking up

So the second surgery was on friday and it went well. There was some swelling in his abdomen so they left it open but covered and kept him sedated. This morning they closed the area and said that everything looks good thus far. His liver enzymes are coming down which is a good thing and means that his liver is slowly but surely adjusting and working in him.
There are some concerns about his kidney because his urination decreased and while the transplant surgeons are okay with that, some numbers are coming back elevated that shouldn't be. However, Lennon being Lennon he decided to do business in the OR so I am feeling pretty good about things.
They will stop sedation at 1:00 p.m. today and then take out the breathing tube and some other tubes once he is awake.

Lennon is a pretty lucky guy and more so he is meant to be here. He has survived the odds numerous times and he continues to defy them every step of the way. I am lucky and honored to not only be his mom but to know him, and I belief that he will do something great but also important with his life. I am just amazed that he has received not one but two livers in one week.

Thanks to everyone that has send us their prayers and thoughts; that has helped me and my family financially, emotionally and in every other step of the way.

Here is to hope

Lennon went into surgery at 1:00 p.m. today. He was not feeling well this morning, certainly in a lot of pain from the first surgery. He looked exhausted when I came back to his room this morning. Let's hope and pray and give positive thoughts that this surgery will go great, that everything will be functioning like it is supposed to and that he can soon be as healthy as he should be.

Here it goes...

The phone call came and another liver is on its way. The surgery is schedule for after 11. Wait for the update.

An update without an update

Lennon is doing well but we are still waiting on the new liver. So for now we wait....

Alright here is the deal...

...while I have a moment...

So he had his surgery last night, everything went well, until they took the ultrasound and found that one of the arteries is clogged. They rushed him back into surgery to fix it. The docs placed him on heparin IV in hopes that this would help the situation. This morning, they took another ultrasound, and while half of it appeared fine, the other half didn't. So they did a CT Scan to get a better picture. And it is clogged.

So his body is accepting the liver fine, but it is that artery that could cause problems later down the road...abcess, infection, other parts dying off etc. So now he is back on the transplant list but has an extreme high priorty (No. 1 so-to-speak). And so we wait...again...

This afternoon, they took away the sedation and he slowly woke up. They took out the breathing tube and some other things. First thing out of his mouth "I'm thirsty". He doesn't want to hear he can't have anything for awhile. So anway, he looks really good though. His eyes aren't dialating when he is stressing, his skin looks good...physically he almost appears like a child I didn't know before. Of course he isn't all that pretty err..handsome..eyes are kind a puffy...but heck he was sedated for more than 24 hours.

Really the frustrating part is waiting...and bless the family of the first donor...and unfortunately the next one to come. This is something I cannot describe how this feels. Knowing someone elses baby has died, but has given life to my son. It is unreal...that huge heart that family has to make such a difficult decision. I want to thank them from the bottom of my heart.

The outreach to my family has been amazing...we have prayer chains all over the world..WOW! Thank you! Thank you for having the faith and the trust that he will be ok. Thank you for the emotional support to us s, who are taking this a lot harder than he is (probably).

Thank you!
(green is Lennon's favorite color).

I will update more as I know more....the big thing is to keep Lennon healthy and waiting....

Lennon had his surgery

Lennon had his initial surgery. Things are a bit shakery. May need more surgery. Please pray for him and hold for the next update. I will go into this some more at a later date.

Lennon had his surgery

Lennon had his initial surgery. Things are a bit shakery. May need more surgery. Please pray for him and hold for the next update. I will go into this some more at a later date.

Lennon had his surgery

Lennon had his initial transplant surgery. Things are shakey. Please hold for further update.

No school on Saturdays

So we have been home for a few days and well so far so good I suppose. He seemed a little off the other day, but then yesterday seemed to do really well. We had a good time goofing around, which is not something we do very often. My work hours aren't the best (3-11) so I miss a lot of what is going on after school. I was off yesterday and the day before :o)
L's shakes have done away for the most part, every now and again we can still see some shaking going on, but lord nothing what had been. Unfortunately his behavior is back, the behavior that we have such a hard time controlling, and trust me I'm a believer in firm discipline, but L doesn't get it half the time. It's rather quite frustration because some behaviors are so typical for his age and I am happy to see them, but he is really extreme in everything, there is no inbetween with anything. He appears to me more cranky than ususal and aggressive to the point my poor animals hide when he gets around. Well hiding is probably not the right word, but they aren't in his path. This is the frustrating part of his brain damage that a lot of folks don't understand. His outbursts are unreasonable but then he is 5 and at 5 everything tends to be unreasonable (keeping in mind his delays his behavior is quite typical for the terrible threes).
He wants what he wants, when he wants it, NOW. He wants to play video games, he wants candy, he wants mommy to hold him, he wants to color, he wants to go outside, he wants chips, he wants dinner, he wants to watch cartoons, he wants to play with cars, he wants to go to school..he wants to do all of these things in 10 minutes or less.

Today is Saturday and he wants to go to school. It is hard for him to understand that Saturday and Sunday's there isn't any school.
L: no school because I have a headache?
Me: Uhh no, you don't have school because its the weekend. There is not school this weekend.
L: You need to call the doctors because I have a headache
Me: But I think you are fine and we don't need to call the doctor
L: no my head hurts, I need an IV in my IV Port

These are typical daily conversations with Lennon. He complains about his head or his eye and I do believe that he hurts, but I can't convince him that we don't always need to go to the doctors.

All in all today seems to be ok for now. I am sure by the time I return from work, I will hear everyone's frustration about L not listening to nothing anyone says. But this is life, this is his life, this is my life, this is our life.

30 days...its like an infomercial

I talked to the transplant co-ordinator! Catching up and following up on L's health, hospital stay etc. In conversation she stated that once the thirty days are up (November 29 will be 30 days) it will ultimately happen shortly thereafter. Hmm, makes you wonder. While it could certainly happen now - it does seem more likely it won't happen until the end of Nov - beginning of Dec. Well me might be able to spend the holidays at home. Which would be nice, but I couldn't think of a greater gift then for L to be relatively healthy and live a relatively normal life. I say relative because the reality is he has suffered brain damage and he is behind. He does function well and some processes that weren't well before are certainly improving.

And in case you are wondering, L is home as of late Saturday night. We played catch-up on sleeping yesterday, I did some well needing to do homework for class and L was himself - playing in his halloween costume. And the only medication he is on is for the UCD, at this point no behavior modication or ADHD control - oh boy it will be an exciting of couple a weeks (bad english here eh?).

Here is to good health!

Don't use Abilify if your child has a UCD!!!!!!

So L has been on the abilify to help with some of his behavioral issues (due to brain damage etc.) well, it works like a charm. It works too well, because it will cover the typical symptoms we know about L to have when he is in a hyperammonia state. Thursday L, was not sleeping well but settled in nicely once I was home from work. He went to school but two hours later he was back home. We noted again that he is still shaking tremendously and so I placed a call to Neuro-Development. Well in the mean time, something ..call it mommy-instinct if you will ... I thought well maybe we oughta have his ammonia checked, just to be safe. We went into the lab (not the ER) and took blood, waiting for about two hours - and his levels were 269!!!!!! This was at 12 p.m., at 6 p.m. his level was 290 and at 6 a.m. his levels were 280.

So yes I belief that the Abilify - no matter how well it made him behave - has covered the typical symptoms of: lack of intake, lethargy (sp), aggression, mental alertness, so I did note his drooling once again. Good indication his brain is shutting down.

These shakes he is having make me nervous. I almost hope that it comes from the ablilify and that now that he is no longer taking it, those will go away. I hope this is not something else or something new. Perhaps it is a symptom? I just keep thinking about how long has his ammonia been high and we didn't know this time.

Well, we are heading to the PICU in a bit to get the amminol IV hooked up as his levels are not coming down with just Lipids and D10.

Have a nice day!

The transplant list

We received the phone call, Lennon is on top of the transplant list. So now we have to be patient with anticipation, keeping him healthy and be grateful. I am batteling to determine which is worse, the waiting or the surgery and I came to conclusion that the waiting is worse at the moment, but the surgery is scarier than the waiting.
My gradidute goes out to the donor that is helping to save my son's life. Medical technology is amazing, but there is nothing more amazing than a person being an organ donor so that another person may live. I am an organ donor, always have been and I hope that many who read this blog, who may not be organ donors - please do consider it. I know sometimes religion, beliefs, life get in the way - but what an amazing gift to give - LIFE.

As I sit here - writing my thoughts to the world - I recognize I have given life and it is quite miracelous. Thus a donor would merely give the gift of continuing life.

As I think about what life may be after the surgery - I can't imagine it. The hope is that the UCD is cured per say and that Lennon will lead a normal and healthy life. The question is will he recover from some of the brain damage he has suffered, will he catch up in his development - will some of the behaviors go away? I know, there is no telling until everything is said and done, but one can't help but wonder about it. It's a human thing to do eh?

Satisfaction and being satisfied

L has a real hard time with being satisfied. He says he's hungry, and it takes 5 minutes to figure out what he would like to eat, then you fix it, and then he doesn't want it. This is the real frustrating part on our daily routines. You monitor his food, make suggestions of what he can have, he decides on something but then does not eat it. Then you have to make sure he does eat and drink, because when he stops doing those...well his ammonia will go up...however I must say the last three weeks have been almost unreal. We have been home without interruption. But I still don't feel like that I can breathe any easier because it can turn any minute.

I recently had a conversation with his teacher and they are now in the process of trying to figure out what to do for next year. He is no where near ready to go to kindergarden, but he cannot be held back. No medication (as of yet) has worked to keep him focused for anything more than 5 minutes, though I do see some sloooooooooowwwwwww progress. The frustrating part is that he is not maturing, but he is functioning. His speech while not perfect, has improved tremendously. He still has a low-tone in his reflexes and more often than not requires MY undivided attention because no one else will do.
He very easily becomes obsessed with one thing and has difficulty accepting that it will not happen such as playing the video game, which wants to change from game to game. It's like he wants to do everything so that he has not missed out on anything...because the reality is that one bad spill it could be over...

But that is why the transplant is in the process...Yesterday he had his MRI of the abdomen, another blood test and another visit with the doctor. L did really well accepting that we were not staying there for the night. He did ask if he was sick and if that is why we went to the hospital. So he does get some of it, and probably recognized that he wasn't feeling ill. The transplant is scary and I am attempting to only keep the positive things in mind, but can't help think about the what-ifs...and man I can't tell you what it feels like to imagine not having your loved one around..and I hope that I will not have to face that before it is my time to leave this physical place we call earth.

I must say that all the hospitalizations and me missing time from work for 2 months has messed up our finances. We are struggling to catch up on the things that fell behind and the light at the end of the tunnel doesn't seem to be getting any closer. For anyone reading and following this blog, I don't want charity but I do encourage to purchase my artwork and jewelry instead. I don't like asking for help, but perhaps I can promote my art work and create some additional funds to help us out. My family has been supergood in dealing with the bare minimums, but christmas is too close to not worry about not having anything for them...oh boy did I make any sense... Please if you are inclined to give...do it by purchasing one of my pieces at http://hippiescreations.etsy.com/ or visit my other website at http://www.hippiescreations.com for additional information.

Transplant Process ...the beginning stages

So L had his initial meeting with the transplant team. I am scared, worried but like the outcome even though it bares quite a few risks. L has been doing ok, but has not been sleeping well. He has been drinking and eating, so I don't think that the ammonia is elevated in the "danger zone". In addition, his attitude and behavior indicate that it is not over 100. We certainly need to watch him and do the things we know to do : gatorade, MCT oil, sugary things etc.

OK back to the transplant... L will receive a whole Liver as opposed to a part of a living donor. The reason being is that since the Urea Cycle Disorder is Genetic, there is a pending risk of it not going away due to the same genetic make-up even though no one else in the family has it (that we know of). It could be that I am a carrier, or anyone else in the family is a carrier and so there is too much risk. So he already has given his blood, now we are waiting for an MRI/A (MRI of his abdoman). The docs and transplant team will meet next week, recommend L to be placed on the waiting list, and if there is not enough points to do so, there will make additional recommendations or something like that, in either case, in about 3-4 weeks L should be on the liver transplant waiting list. Of course this then means that we could receive a phone call at any time, on any day.

In the mean time we will hope that L does not have many spills or relapses that could damage his brain (or worse). I think if he doesn't get some good sleep soon, we will visit the ER once again sometime within the next few days.

One moment and one day at a time

We have been home one week without going to the Emergency room or being hospitalized. This is good, but I still feel like I cannot breathe and relax. L looks really good, his personality is back - the sassy, funny little guy who keeps you on your toes. And yet, I am filled with anxiety that I will wake up, or get a call at work telling me that L has another hyperammonia episode.
But I must admit, it has been nice to be home on my days off, and come home from work and know that he is doing ok. A good indication for L's ammonia levels is his pupils and this morning they were normal - if is ammonia levels are elevated his pupils will get big - at times they will cover the entire Iris and pupil is all you see. His behavior has leveled out some - but we stil go through the motions of really funny to whiney and cranky - to tempertantrum from hell - but they have gotten better. Now I just need to learn to breathe during the times he is well.

Wonders and Worries

So we have been home for a few days but I am still holding my breath. L looks good physically and no indication that his ammonia levels are elevated. The only thing that shows is his aggression. Now we also deal with the typical 3-5 year old behavior and it makes it difficult to identify which is what at times. I had to pick him up from school yesterday because he was hitting other kids and pulling hair. Now I don't mind that but today I asked him to remove him from the room if possible, or put him in time out. Certainly if he gets out of hand, please do call me.

On the other hand I can't wonder and worry when he will relapse to high ammonia levels. Because it has been so unpredictable, I feel I don't have time to relax for one minute. The pressure of keeping him healthy, studying for my master's program, making sure the other kids are ok and taken care off and keeping my partner happy often places too much on me. I don't have time to breath for myself. I know it seems like I am complaining. But truly, that isn't it. I think I just get so overwhelmed that a lot of things are left on the sidelines. Which means that my relationship is suffering, and I don't know how to let go of the anxiety.

There is the constant worry...

It is the song that never ends....

...it goes on and on my friend...

This seems to be the reoccuring theme these days. L's levels have been pretty normal/low 55 and 78, so nothing to crazy to worry about. But his behavior doesn't collaborate his levels. L has been extremely violent, uncooperative, not listening and kinda crazy if you will for a lack of a better word.
One moment he is sweet and loving L, and the next he is the holy terror. So now they are trying to admit him to KCRC for observation period with the neuro-development people and behaviorists. Of course this means that medicaid has to approve it, which that can be a battle. So will see, but for the moment he is going home!

Which one is harder

I don't know which one is more difficult to deal with the part where he is mentally gone - incoherent - unable to "get" where he is, and who is with him - or the part where he is so violent that it actually requires either four adults or retraints to keep him safe. Again this is a function beyond his control and while sedation works wonders, with his ammonia being up it isn't the best solution because we can't use it as a baseline for behavior at this point indicating normal levels.

It breaks my heart sitting here, watching him, unable to truly comprehend what is happening. I can explain to him why until I am blue in the face - and he will not understand what I am saying. He understands the words - he doesn't comprehend the meaning! Nothing will distract him to be nice, gentle and good - I can't trust a hug because he is quick with his teeth.

I imagine how easy it is to be judged at the moment for allowing the doctors and nurses to restrain my child. Realize this however, that this is for his safety. He can pull his IV out, he can bite the line - he can jump of this bed - run into walls as well as kick, claw, bite and scream at everything and everyone. I certainly don't wish my son to be restrained - but I certainly want him safe. The ammonia is elevated and this is what it can do. It messes with his brain every single time - and every single time it is elevated he sufferes a little more (or some) damage.
Again I feel like he is slipping away from me - underneath all that care he is given - slowly but surely I am loosing him - but I have to believe that he is a fighter - he will not go down easy - The Angel of Death will just have to wait a bit longer!

The world is slipping under my feet

Today while I was at work I received the phone call that L had to be taken back to the ER. Levels came back at 187, not as terrible as it had been but still bad enough considering what abnormal levels can do to you.

As I am writing this update, I feel like the world, no not the world but L is slipping away from me underneath my hands. It seems no matter what we do at home and the best care everyone gives to him, it doesn't matter. I want to hang on to hope and find the strength that he will survive and that he will be there, graduate from Elementary School, Middle School and Highschool. But also that he is enjoying life, finding the right partner for him..all the things we wish that our children should accomplish to achieve their happiness. I am barely hanging on to that hope.

My relationship with my partner is strained as we can't find the time to breathe, let alone find time together. It is us doing it, all the time not only taking care of L but our other children, who being left behind in the process as we are attempting to save L's life. They are hurting as well, not only because (I feel) they are being neglected half the time but also because they are worried, they are scared to death just like us the adults.

Friends tell me prayer chains are all over the world (literally) and I can't ask any more of that. So thanks to all that think about L and in his time of need. Thanks to all that think about us in our times of struggle and heartbreak. Just thank you.

The scariest moments are....

...when the person you love looses all mental abilities alas is incoherent, doesn't recognize anything and is in a delirious state and all because of the ammonia going up.

Thursday into Friday..another night in the hospital...I had brought home a new kitten (naming her doodle ..short for doodlebop). I was heading to bed when I heard some comotion and sure neough there was L with holding his blanket, rambling things that didn't make sense. He had vomited and well his mental state wasa clear indication he needed to be taken in right away. I drove into town in the rain...it was not a good night for me because I was tired and exhausted, emotionally a wreck and well I don't like to drive at night, I don't like driving in the rain and I don't like driving at night in the rain.. but it didn't matter what I like, this is about L's life, not mine.

As soon as we got to the ER, they drew his blood, got us cozy in the ER room and we waited - not only for results but a room upstairs. The PICU was full and no one could be moved - so other arrangements had to be made.

Level 309 - gasp - why why why? What is it that is not working so that we are in the ER every weekend. Sometimes twice in one week. We are noticing that his brain is shutting down when his levels go up, he begins to drool indicating, that this part of his brain is partially gone. We also think that the last two times of elevated levels have done more damage to his brain.

So 12 hours later we finally get a room on the floor - normal routine - L wants a popsicle, he is hungry. He is eating his black olives, some french fries and drinking Pepsi.
Speaking of that, the weird thing was that this time he did not stop eating or drinking - he indicated being hungry - eating some baby food and drinking his tea or water - this episode was not normal in the sense of what we were used too. Anyhow, so we are in the room and blood is drawn - well wouldn't you know that his level is 86 - we are going home!

Lennon is still kinda out of it but they had him sedated which he didn't get to sleep off and so I suspect that is why he is a little croggy and down. He is kinda mellow - again not what I am used to - what damage was done to his brain? That is what I am wondering. And so the day goes on, we have dinner, take showers, take medications and go to bed. L slept through the night. This morning he is helping on the paper route - well because I have to go to work shortly and the papers were way LATE and so this is what works. And L didn't mind - he likes to go on the paper route.

We're Home......Again

Yes, we are home again. L's levels were 58 and he is eating good. Notification was send to the Liver transplant team and so the process begins. I don't know what is more scary - these hyperammonia episodes and the risk of him becoming severly brain damaged ...or even worse.. one the other hand the transplant brings a lot of other risks - the immune system being nothing - forever being on medication - well either way he wil be on medication.

Someone told me I should get the super-mom-of-the-year-award - between everything that is going on with L - working - school - our other kids -my art stuff - well I am just doing what I have to do - there is that hidden drive - the energy and focus that gives you the boost to to do all that you need to survive and better yourself in the process.

As heart braeking every episode is and as tough they are on us as a family and our finances - we do what we have to - don't wallow in pitty because there isn't any time for that. I cherish every moment that all of my kids are healthy and the time that we laugh and have fun together - even if they are few and far in between.


I am beyond tired and certainly know that my driving force is the love for my son. I haven't seen my house or bed since Friday as I travel between work and the hospital. Last night L decided he didn't want the IV - played with the tape - and ultimately pulled out his IV. They had to replace it of course, but come this morning the line and his blood was not working and all the heparin and TPA didn't do any good. And the IV was replaced - again.
He will be staying in the hospital again tonight - his level is around 138ish - thus still not well enough to go home - though he is eating a bit better.

That's all folks.

Out of control

So Lennon was admitted AGAIN into the hospital. Ammonia was 327, we spend 15 hours in the ER, waiting for the PICU to open up a room. He was not mentally coherent, didn't know where he was, probably who he was or anything else for that matter. They sedated him because he became too agitated (sp) and they were afraid he was going to pull his line out. This morning his levels were 115, which is not great but better. They are keeping him until he begins to eat proberly, so I'm thinking he might (i mean might) come home tomorrow, or Monday. They don't want to see us again (haha) anymore I want to see them.

On another note, if his ammonia elevations keep it up, he could be looking at a liver transplant, which essentially will "cure" the UCD but give him other issues - no immune system, will be on other medications. And so forth. If it does come down to that, I hope that his body does not reject it and that this could be life-saving for him despite the risk attached to the transplant.

I have not wrapped my head around all of this. I am at work, exhausted from being up a long time, sleeping a few hours and hoping like hell that he will be ok. He was more coherent this morning that yesterday but I still didn't see a huge improvent in recognizing my son. He was cranky for sure. They had him in a crib for safety overnight, but this morning he was SO NOT HAPPY about it. Can't cage the Lennon, ya know. They are giving him a regular bed now - per conversation with my partner.

Quite a few people have asked me why I come to work and I'm like, well you are not paying my bills, someone has too. Right? Thus we shift change in order for us to keep our job and earn money. Besides I can't do anything at the hospital, exept sit there and keep him as calm as possible - keep in mind he is extremely ADHD and sitting still is not his best skill at best.

I love this kid - he is amazing and right out a miracle. He is resisting death without a doubt, he is a fighter - and I hope he continues to fight because he is the coolest guy ever - ok my other kids are cool too but they are "normal" and not sick or anything. This makes it hard too. L gets all the attention, and my other kids have to "suffer" though they are not neglected in a sense of not having food and shelter but I feel that L's disorder often keeps me from equally sharing affection among everyone else.

Level 88

So the ammonia went below 100...88 to be correct..and so L is home from this visit. They did his MRI in the a.m. and I am curious to know what they can or cannot tell me. His future is undetermined but we'll do the best we can. I think my frustrations are really to the point that we cannot control his ammonia and there is no rhyme or reason what may cause it to go up. While stress is one of them, I would love to provide a stress-free live but tht is unrealistic. I don't know if I want him on additional medication to cope with his stress problems because I want to enjoy my son even with his quirks. I think this one was triggered by stress (some of it self-induced) and a nasty cold he is trying to battle.

On a side-note, I signed him up for research studies though I am not sure where this is going to lead as we are having difficult times when he is in the hospital and thus traveling would be even more complicated for our family.

Well, lets home we get to spend more time at home than in the hospital. I would like a few weeks without a spell but I could be asking for too much.

Beautiful Boy
John Lennon

Close your eyes,
Have no fear,
The monsters gone,
He's on the run and your daddy's here,

Beautiful Boy,

Before you go to sleep,
Say a little prayer,
Every day in every way,
It's getting better and better,

Beautiful Boy,

Out on the ocean sailing away,I
can hardly wait,
To see you to come of age,
But I guess we'll both,
Just have to be patient,
Yes it's a long way to go,
But in the meantime,

Before you cross the street,
Take my hand,
Life is just what happens to you,
While your busy making other plans,

Beautiful Boy
Darling,Darling Sean.

I dedicated this son when L was born before I knew about his disorder or anything else. Though this applies to all of my boys!!

Holding my breath

L was admitted to the hospital on Saturday with the ammonia level of 237. We did the usual treatment of lipids and D-5 and at first they were going down to 147. So we figured good deal we'll be home Sunday. Well no such luck. They checked his ammonia this morning and his level was 353. So now L is in the PICU receiving his meds through his IV Port in addition to the lipids and D-5. The PICU is only required for monitoring him more closely because the nurses on the regular floor could do the same work but they cannot closely monitor him.

It worries me that his level went up like it did because this brings more reality to the fact that I can be loosing him at any point. If it was me (or even you) he would be in a coma or perhaps even dead. This reality is heartbreaking and brings me to tears and I often wonder how much longer I have with him. He is so precious. Despite his behavioral issues due to the brain damage and his delayed development, he is such a gentle soul. He is loving and he is fun and keeps my life interesting and laughing.

Tonight I am at home while my boyfriend and ex-husband keep watch over him in shifts. It is very hard not being there constantly because I am sorry but as a mother, I have to and should be there. I recognize however that even I need a break to get some sleep, take a shower and just breathe for a minute, oh and well do my homework. I must remind myself to take care of me in these times but I am not very good at putting myself first.

I long for the days of normal in which we can simply go on with our lifes, struggle to pay our bills and but food on the table, but still normal in the sense that we are working, enjoying the children, and be a family as opposed to our second home being the 7th floor of the hospital.

Our ups and downs

Yesterday went well but today is a different story. L woke up not wanting to eat breakfast, and I said alright, well at least you can drink some of your formula… HA! Yea right! He or his body decided that it wanted to reject it, and puked it all up (twice). Normally this is an indicator that he is not well, but it doesn’t appear that his ammonia levels are elevated. He seemed to be in a decent mood, more mellow than I am used to, but certainly in a good mood. But it gives us a good cause to worry about it nonetheless. Ultimately I think if he continues on this path, we will not have a choice but to go ahead with the feeding tube. However, in the mean time we are trying to avoid this as best as we can with added medication to increase his appetite.
The worries with the G-Tube are that he will pull out the plug or button, whatever you want to call it. He doesn’t get the consequences of what happens when he pulls it out, nor the possible damage he can do and the infections he can suffer. While there are contraptions to place around his belly i.e. a corset or an ace bandage, it is my fear and worry that he will still find away to get to it and pull it out. I would like to think that he’d surprise me and not mess with the button, but I have little faith in that.
It is frustrating because finance-wise it is creating a vicious circle in which we are trapped in. Ultimately we both have to work to provide food, shelter and transportation to not only L, but also the other children. However, if he continues to go into the hospital once a week, twice a week and so forth, one of us might have to give up our job and then what? This is almost impossible to think of.
And then the services for intellectual and developmental disabilities in my community, well they are lacking the essentials to support families with special needs children and family members. With the economy the way it is, some of us folks can’t get into the big town, which takes about 45 minutes to get there, and well gas money. So in addition to helping my son survive, I will venture into the research and advocacy of individuals like him.

Information comes with repetition

So we once again spend the weekend in the hospital, yes I know he just came home Tuesday but we seemed to have learned something in the process.

Due to the low protein diet that L is on, he does not get enough calories and glucose in his diet. Even though he is allowed added junk food as opposed to my other children. However, it isn't quite enough. So we now have an RX for a special oil to add to his diet and more formula to add more calories.

It is my thinking that he needs to come of the Adderal and be placed on a different type of medication to increase his appetite because he doesn't want to eat half the time. Oh and he is also taken medication to increase his appetite so that he will eat more but specifically more of normal food that he can have.

His behavior was so out of control even at lower levels of the ammonia and it is frustrating. It is frustrating because you can't help him, you can't change it instantly and his brain doesn't allow him to change it. It can be very nerv wrecking and wearing on ones patience when your child changes his or her mind every minute (and this be the truth).

Well, here is to hoping we won't have to go back so soon.

A good day

I don't know how many times this title will repeat or will be the contrast of things...but today is a good day...L went to school and he was gentle and kind this morning. He was still tired and sore where his IV Port is located but certainly he is up an running just like always.
It is sometimes hard to fathom he is five years old because of his delays he is lacking some of the functionings of a child, as well as the ability to learn.

It take about a year for him to learn that he is one year older and the age, and by that time is next birthday will have arrived. So if you ask him today, "how old are you", he will say 4 but in reality he is 5. Odd enough though is that he remembers his name and where he lives without hesitations and he remembers visits to the zoo in which he saw Elephants do their busineness, and when you ask him what Elephants do, he will tell you "they poop".

There is no way for the doctors to tell me if he will ever catch up and if he will ever be able to learn or if it will always be the way it is. Academically he is behind and we kept him from Kindergarten one more year because he is simply not ready. But I can't keep him from moving forward either. Sometimes I am afraid that I hold him back way too much and I have to analyze the skills he has accomplished, the positive things he does. What throws it off is his behavior because it tends to be more aggressive, no throught process involved - pure action based upon instinct.
While I hate the labels of ADD and ADHD there is no questions that L is the extreme of ADHD. He often does not consider the consquences of his actions, nor does he "get" them. He has a hard time to stay on task, and jumps from one thing to the next within a matter of minutes.

But today is a good day. He seems happy and he looked forward to go to school.


So yesterday, we took L to the ER because he was acting funny..kind of lethargic, whiney..some abnormal behavior and so forth..and sure enough his ammonia levels were 276..gees...well so they admitted him, placed him on lipids and glucose and voila this morning his levels were 125 and this afternoon well enough to go home..this makes 8 visits so far this year...and very frustrating because it seems like no matter what we do at home..controlling his diet, making sure he gets his medications, and giving him the things he needs more of such as fat (calories) and sugar, it doesn't do us any good.
I know there are times that he does well for a while..in the last year it lasted 6 months...but here recently its like he is in the hospital every 3-4 weeks.. and while we have worked out a system because of work and other children it doesn't get any easier.

I would love to just find out what exactly it is that he needs when his levels go up. I mean I know they give him lipids, but what exactly other than fat is that. And yes we have asked can we get some for home that he can take orally because I'd rather not use the IV port to administer anything, if I don't have too. Quite frankly, the best thing is to cook everything with oil i.e. olive oil which we have increased in his diet but again it didn't seem to make much of a difference in the end.

I would love to get his diet under control more because he is on a strict protein diet, he is lacking some of the necessary things that we get from protein. However to supplement he is taking an amino acid formula but it isn't enough, so it seems to me. We allow more of the "junk" I wouldn't give to my other children at least not in the rations he is able to get them. He doesn't like gatorade but it is high in calorie contend that is good for him and has all that other stuff.

Well that is my rant for the moment as he is walking into the door momentarily.

August 2007

It was a good thing that in Jan. 07 we have moved to a new area. L would be ill a few more times...with the vomiting and being extremely lethargic..

In August it came to the point that L was almost not responding, and we took him to the ER. They did the usual, hook im to an IV and took down some notes of what we described..they took blood and gave him a physical. The peditrician noted some differences in the amino acids and was concerned enough to have him admitted for an overnight stay. They brought him food which he didn't keep down..and so the journey began in the discovery of what is wrong with L.
The next day due to his normal state he was released. Things were so-so...L started school but it was but two hours later they called me to pick him up...something was wrong. I called the ped. and took him in..during the trip into town, L vomitted some more. We waited for awhile at the ped office..who referred us to UVA. In the Er..again the IV...the blood tests...and the description of what we saw and admitted to the hospital.


L has Urea Cycle Disorder

We are told that normal ammonia levels should be around 30-60 but that L's were in the 450's. Essentially this could lead to coma and/or death. It causes brain damage, delayed development and more if left untreated and undiagnosed.

L was placed on IV meds, transferred to the PICU. There we spend about 5 days until his levels were below a 100 (his safe zone). He is placed on a low protein diet (15-20 g per day) and has to take medication as well as drink an ammino acid formula.

As his levels stablized we were released to go home. It wasn't even 24 hours when L woke up disoriented (so it seemed) in reality he had just lost his vision. And it was back to the hospital. The MRI indicated that L has several edema's on his brain...one right behind the opitc nerv and it is the assumption that his body attempting to get used to the normalcy in his chemical make-up with the medication..it actually went a bit crazy as the ammonia edema's are trying to flush themselves out.

L was blind for 6 months - in which it seems that another hopsitalization with high ammonia levels did flush out enough so that the edema is less than before and voila he can see.

For more information on UCD go to http://www.ureacycle.com or go to http://www.nucdf.org

So it begins

L was born in 2003. The pregnancy was good, no trauma no trouble..until the end where he had to be induced. Perfect angel, little baby boy. All was well, or so we thought.
We went home and started the new routine. Having had childrne already, it was no big deal for me. But somehow (in my gut) things were different. L couldn't be consoled, nothing would satisfy him and he constantly had a runny nose and he would just scream for weeks on end. I'd call the doc and nothing was "wrong".
L grew and went through the stages of being ill...flu symptons but not having the flu.. he'd vomit, he'd be lethargic, hyper, not sleeping (or just a little). I was in tears most of the time, ready for a break down. At age 2, L was barely speaking, not interested in the toilet and couldn't find his independence. I had him evaluated with the school system, L was developmentally delayed by 1.5-2 years. L was also aggressive in his behavior to the point I often had to restrain him from hurting himself or others. This describes the first four years of his life.
It was a constant back and forth...nice L...mean L...lethargic L...vomiting L...hospital L..and so forth. No doc recognized anything out of the ordinary...no one reported me to social workers because I was in the ER so many times...no pediatrician thought..hey maybe something is wrong...no one would listen to me..or hear my frustrations...how many years did we acknowledge that mom's know when there is something wrong about their children?

It has been frustrating, and upsetting to know that something is wrong with your child, and no one can or will tell you what it might be.

I am lucky to still have L in my life (though this could change at anytime)