Sunday, November 16, 2008

Up till now..

Well at this point, it is mid-November and you can continue our story, me, J, my husband, R, and little Al at my regular blog, This page is just to catch up until now with Al's first year or so. Hopefully the next year is less crazy or at least a good kind of crazy.

We continue to be very grateful to the donor and family for choosing to give life to our little one. We're thankful to everyone who has chosen to be a donor and save a life - or many lives.

After transplant

Al went home feeling okay and on a very small dose of oxygen, about 1/32 liter. After transplant, checkups are twice a week for the first 12 weeks and then switch to once a week. He was doing well for a few weeks, even had a Curious George birthday party planned for about a month after his actual birthday.

Then, he got sick again. His breathing got worse and worse. His oxygen flow went up to being on 3/4 to 1 liter all the time - even more than before the transplant. So much that the oxygen delivery company got mad at me for calling asking for more all the time. His whole body swelled up and he threw on an extra 1 kg in water weight. He would wake up with his face so puffy that one eye would be swollen shut. And he was miserable. He'd cry all day long and nothing would console him. It was a lot worse than before the transplant.

At first his doctors thought he had gotten pneumonia and he started antibiotic treatments that didn't work. The were baffled because all the outward symptoms pointed to rejection but his ECHO (heart ultrasound) and EKG's all looked fine. We don't have any pictures of that time. Life was so miserable for all that there wasn't much time for any picture taking.
Eventually we decided to treat him as though he were rejecting and start a 4 day, 8 dose IV steroid treatment. Homecare came out to the house and gave the effusions. Several days later, it looked like it had worked. He perked up and lost his extra fluid weight and within another week or so was able to go completely off oxygen.

Now at this point symptom-wise, Al is fine except the reflux and feeding. He still has a hard time taking much of anything by mouth. He did just start eating dairy again and has been tolerating it. Yogurt is his favorite new food. He still gets most of his nutrition from taking formula through his tube.

We just have our fingers crossed that Al will be able to continue to grow and hopefully learn to do more toddler things like eat and play and become mobile and talk. Kids do not deserve to have this kind of life. But he's been a champ and gotten himself through it.

Sunday, September 7, 2008

Ring, Ring, (or more like Buzz Buzz)

One Saturday night in September, it was past 11 pm, Al was asleep in bed, mom was asleep watching TV and dad was on the computer. Suddenly, dad heard a faint buzzing sound and realized it was the phone. Mom had put her phone on vibrate earlier and forgot to switch it back to ring. He saw there were 20 missed calls! Allison had tried several times and then had finally called Grandma and Grandpa to try and track us down. They called a bunch of times over the course of 20 minutes. Finally got in touch with Allison and

there was a potential donor and it was time to get to the hospital.

Mom and Dad had already made a list of everything that needed to be done to leave the house quickly and had a bag packed to go. Poor little guy got pulled out of bed and thrown in the truck not knowing what was going on. He stayed quiet and awake the entire drive while dad sped off to the hospital. Somehow they made by midnight which meant they had technically arrived within an hour of the first missed call.

Al went straight up to the CICU where he was expected but no one had heard yet about the status of the transplant. His nurse drew some blood to run some labs. He got weighed in - 6.7 kg, took his first dose of Neoral - the main immune suppressant that he'll take for the rest of his life and got all set for surgery - assuming it would go through.

His first dose of the Neoral was not fun. Because the liquid form of the med is so sticky and oily, he can't have his binky for 10 minutes afterwards. He was super unhappy about that and screamed and screamed and just about woke up all the other babies on the unit!

Finally he chilled out and got some rest and so mom and dad decided to get some rest too.

Then at about 4 am, Dr. Goldberg came in and woke up mom and dad. He said: I'm going to fly to the place where the heart is to pick it up. From what I've heard - its a good heart. Mom and dad signed consent and he was off on his way. (Because organ donation is anonymous, the recipient's family is not to be told any identifying information about the donor including their location. In this case it was in some city that is about 1 hour 45 minutes by airplane) The surgery was planned to start around 8 am.

When he woke up, Alan got some last snuggles with mom and dad.

A little while after 8, he went off to the OR to start the surgery. The surgeon for this procedure, Dr. Max explained that it would be a little more complicated than a normal transplant. Because of all of his previous procedures, Al's pulmonary arteries were very narrow and these would have to be fixed as well.
Mom and dad went off to the waiting room again to wait with Al's grandma's and grandpa's. This time Allison and Carrie were bringing the updates. The surgery would normally be about 6 hours, but in usual fashion for Al, it took a lot longer than that - pretty much all day.
Mom and dad got word that the chest was open and that the scar tissue was being cut through. Once the doctors got word that the new heart had landed in Denver, he was put on the heart/ lung bypass machine. The next step was to work on the pulmonary arteries. Finally, Dr. Goldberg came back to the hospital, with the cooler. He said: I saw the heart. Its beautiful. And he dragged it off into the OR. The surgery continued for hours. The surgeon used part of the donor's pulmonary arteries and grafted them onto Al's. This required a cold arrest process which means that the body is cooled enough that circulation stops all together. It can only be done for about 15 minutes at a time. This had to be done three times while the pulmonary artery was repaired. This is very dangerous as circulation to the brain and the vital organs is completely stopped but it was necessary to repair properly.

Because the old heart was going to research, it had to be dissected immediately in the OR. So, Allison offered to take a camera back and get a photo of the old heart. Ready...

Here is the old one:

The old heart was a mess - misshapen and swollen and covered in scar tissue. Its pretty unbelievable that the old heart had made it as long as it had. This made it very clear that the transplant was the right choice.
Eventually, the heart was in. Slowly he came off the bypass machine and was pumping on his own. But, after all that he had been through during the surgery, bleeding was bad. It took hours to try and soak up all the excess blood in the chest. Finally the surgeon decided to leave the chest open and leave gauze in the chest cavity to stop the bleeding and then wait a few days to close up.
Finally right about 12 hours after going into surgery, mom and dad got to see their baby. He was a mess after the surgery but all things considered was doing pretty well. The gloves there are ice packs to get his temperature down.

Scary Night
Mom and dad ate some dinner, and saw their Al to say goodnight. He was still very sedated. He needed a little bit of pacemaker help which isn't unusual. Mom and dad kissed him goodnight and went down one level to their sleep room at the hospital. Right as they were getting their bed made to go to sleep, they got a call from the assistant in the CICU that they should come back. Mom and dad got on the elevator and sprinted to the unit.
Everyone was surrounding the bed. Al's blood pressure suddenly had dropped dramatically and he went into a spell of cardiac arrest. A Code Blue was called and the crash cart pulled over and he received 30 seconds of CPR. As we were arriving, the patch covering his open chest was opened to go in and check out the heart. What they discovered - the heart had swollen from all the trauma and was being compressed by the gauze that had been left in. After removing it, his numbers got much better and the bleeding had mostly stopped. It was a huge relief.
For the next several hours, Al stayed teetering on the edge as to whether or not his heart new heart would be able to make it on his own. The surgeon was trying to decide whether to put him on ECMO. It is a machine basically like a heart/ lung bypass used in surgery, but it can be used for a longer period of time in the ICU. Al's heart would start doing worse and his pressure readings would look bad and then as soon as the ECMO was getting set up, he'd get better and the doctor would hold off. Al had a huge crew of people hovering in his room all night. Dr. Max, the surgeon stayed in the room all night focused on the monitor. Often Al would need a blood transfusion. Instead of delivering it by a pump like usual, his surgeon sat by the bed and pushed the blood in by hand with a syringe to try to add the blood at the exact speed necessary. Al kept doing this back and forth all night long. Finally first thing in the morning, the doctors did decide he needed the ECMO machine and got him put on.
The ECMO machine sat on this metal cart. Al had 2 metal tubes going into his chest. His blood would leave him and go into the machine. An oxygenator would add oxygen and the blood would be warmed and then pumped back in, basically working as an artificial heart and an artificial lung so his heart could have a break. He was still on the ventilator at a low rate just to give the lungs exercise while they weren't needed.
Being on this machine is very risky - especially the longer spent on it. Because the blood goes through this machine, it needs to be thinned with Heparin. This creates a significant bleeding risk, especially to the head. There was about a 40% chance of head bleeding which could cause permanent brain damage.
After exactly 2 days, doctors decided to try taking him off and to hope for the best. Fortunately he did okay and after the days of rest, his heart was ready to pick up the slack and start pumping. The right ventricle was still weak for a few days which is fairly normal but he did okay. The next day, his chest was stapled shut.
Slowly all the usual stuff came off as he got more stable. Chest tubes came out. Lines came out as he was on less IV drugs.
The breathing tube came out.
His breathing still wasn't great and he had to go on the Cpap machine for awhile. It was very uncomfortable.

He got off the drugs and started to become more awake. But he was very uncomfortable. Spent about 48 hours with non-stop insomnia and got the shakes. He was having withdrawal from all the morphine he had been on. He started methadone and ativan which helped him to relax and feel much better. He switched from the Cpap to a high flow nasal cannula.

And eventually got stable enough to move back upstairs from the ICU. One of the first new things to try - applesauce.

Happy Birthday

While on the CPCU on the 9th floor, Al turned one! His CA Kalie got him an awesome present. A helicopter. He loved it and played with it for hours. Its a fun toy that makes loud noises.

And now that he was a big boy and finally able to have a normal fat diet (though still no dairy), Grandma Ja made him a special treat. Chocolate birthday pudding! (made to nondairy specifications)

He stayed for a little while longer, getting more stable and weaned to a lower dose of oxygen and to lower doses of meds.

Finally left the hospital after about 2 1/2 weeks which isn't long for his standards. Still wasn't 100% but enough personality shinning through to know that he was the same Al.

He went home on oxygen, ng tube for feeding, and lots and lots of meds.

Thursday, June 12, 2008


Hurry up.. and wait

Al quickly got readjusted to being back home. He got reacquainted with all his toys. He was sent home on oxygen, milernone continuously, feeding tube for the yucky formula, and of course lots of oral meds as usual.

The milernone meant being attached to a very stylish fanny pack 24/7. The line went into his thigh and ran into an IV pump that was kept in the fanny pack. The medicine came in small bags that had to be switched every 48 hours. A home care nurse came over every week to change the dressing on the line and keep it clean. Having a line right in his thigh was kinda annoying for such an active wiggly little boy.

Although not quite as cool as all the nurse friends, Al got used to being with just mom and dad instead. Dad quit his job in order to spend all day with Al while mommy worked - usually at home.

Worked hard on eating again. With getting most of his nutrition from the yucky formula by tube, Al just worked on his spoon feeding. He learned how to eat all by himself. He ate lots of yummy stuff to keep up his skills and remember that eating is fun!!!

Al would need to stay on the yucky formula until his transplant.

One of the favorite foods.. blueberries. Mom made a concoction in the blender from fresh blueberries and bananas. He gobbled them all up and had lots of fun getting to make a mess.

While he was gone, mom and dad had gotten Al a new saucer toy. At first his legs were a little too short but he grew into it and loved all the toys on it. Al got to meet friend Sarah and hang out at the pool. Unfortunately he didn't get to go swimming then because of his line but he had fun sitting by the pool snuggling with everyone.

Bath time was fun. It had to be a sponge bath because of the broviac line. Tried out the G diapers for awhile while mom was contemplating whether to make the jump to try switching to cloth.

The Move

So just as he was getting settled.. mom and dad moved. But it was a nice change. Al finally got a room of his very own and a bathroom. Not that he used either. He continued to sleep in mom and dad's room anyhow.

Al got used to the new comfty house. Everyone was on edge all the time for the call to come in for the transplant.

Any time, a call could come in that there was a donor and he'd have to be at the hospital within an hour to be ready. The estimated wait time was about 3 months. There were lots of kids at the hospital at that time waiting.

Al had fun spending quality time with daddy!

Diapers, diapers

Mom finally decided on cloth diapers. After much research and trying out some sample packs, she decided on the Bum Genius 3.0. They are awesome pocket diapers that hold up well and are easy to wash and affordable and come in lots of cute colors.

Al's favorite toy to chew on - the oxygen cannula. If you put it in your mouth just right, you can make some fun sounds and blow some sweet bubbles. He was constantly chewing on it and mom and dad kept having to readjust it.

And, one of the favorite books - Water Water Everywhere. Its a baby Einstein book about all the different forms of water.

Throughout the summer, Al saw his OT Ann and his PT Sarita to help him with regaining his strength. One day, Ann brought a cup and some blocks. He had lots of fun with the blocks and shaking them in the cup and putting them in and taking them out.

And he got a little stronger - learning how to sit up almost by himself. He made mom and dad and everyone else very proud.
Al got big enough to start sitting in his bumbo chair to play!
And he decided to start pursuing an art career.
Things started looking up. Al had a pretty fun summer, for having to be cooped up a lot and tied down with a bunch of cords. As September came around, it got a little more tense waiting for the day to come when the transplant would finally happen.

Thursday, March 27, 2008

We're back


Two weeks after being out, Al was back in the hospital to try to control his effusions. He got another chest tube put in to drain the fluid. And a new course of action was decided. His feeding was stopped entirely and he was put on an initial 10 days of TPN only. (Nutrition that is delivered by IV) This would stop the fat leakage because there would be absolutely no fat in the diet. Al was able to maintain some of his normal activity. He brought his awesome big purple ball to the hospital to play with.

And he kept up his personality. Nurses fell in love with Al and his sweet smile. Especially as his stay got longer and longer.

So this continued for awhile. After the 10 days without feeding, his tube drainage had stopped and so he restarted feeding his special formula.. and the fluid re accumulated again. Al went to the cath lab again to check everything out. He had a few issues. His pulmonary arteries were narrowing and he had developed some col laterals to be coiled off. A collateral is a new passage for blood flow that the body creates itself. These are bad because they are inefficient an pull circulation away from where it is supposed to go. These issues were fixed but still did not make a big difference clinically.

More Surgery
So, the next step, Al went in for a Pleurodesis surgery. Its an extremely painful procedure but not nearly as complicated as the open heart operations. A side chest incision is made. Then the outer part of the lung is basically sanded down with a Brillo pad to make it rough and scarred. The goal is that this scarred tissue will adhere to the inside of the chest wall and stick together, eliminating the pleural space where fluid accumulates.

Here he is getting ready with the surgery with mom and dad. He was sick but in pretty good spirits.

And, this is Al after the surgery. He went back to the CICU post-op to recover for a day or two. But he was a mess. He was in lots and lots of pain and kept needing more and more drugs to control it. His blood pressure dropped low enough to need dopamine - a drug to increase the blood pressure. His breathing got worse and worse.
One morning his breathing was terrible. He was working and working to get enough air in and was super unhappy. His nurse came in for her shift after mom had spent the night and she said "He looks like crap. He needs a breathing tube."
But the doctors didn't agree. They thought he should keep on trying on his own until it was absolutely necessary. Poor little man worked himself hard all day. And his awesome nurse M kept on top of everything and fought hard for little Al until he finally got intubated. It was a relief that he finally got to take a break from working so hard to breath.

He got swabbed and turned out to have an infection - probably caused by a line contamination in his PICC line. (It is like an IV that is central - which means it goes in all the way to the major arteries. He got the line in to receive his TPN nutrition). He also continued to accumulate fluid. Instead of one big mass of fluid on his right side, now that he had the pleurodesis to make his chest stick, he had little pockets of fluid in the areas that had not stuck. And his left side that had always been clear before developed an effusion as well, so he had to get new chest drains put into both sides.
The few days that Al was supposed to spend in the CICU recovering from minor surgery turned into weeks. Eventually he started to improve a little after having his chest drained and got off the breathing tube and switched to a C pap.

During this time in the CICU, Al had a very cool next door neighbor, we'll call him C. C had been in the hospital his whole life, born with HLHS, a condition very similar to Al's and hadn't been able to recover from his first surgery. At that time, C was about 3 or 4 months - a very cute baby. He has the best mommy, H who takes such good care of him. Always dresses him up in nice outfits.

Because of his continued difficulty, Al was sent back to another Heart Cath. This time things did not look so good. He had developed even more col laterals. In order to fix this, about 48 small metal coils had to be used to close this vessel off! His chest will now always be coated in platinum that can be seen from an x-ray. But the most alarming fact, his pulmonary artery pressure, a number that should be around 7 for a healthy person, can be allowed to be in the low teens for someone with his condition, was 22 which is very high. The doctor said that if he continued that way, he would likely not be a candidate for the Fontan, which is the third surgery Al should have had around the age of 3 or 4 to complete his circulation.

Continuing to play musical room chairs, Al stabilized enough to move again upstairs to the CPCU. His friends there on the 9th floor were so excited to see him again!

Feeling a little better, Al got to start eating again. Because of his diet restrictions, he was only allowed to eat nonfat foods. His favorites were peas, bananas, and pears.

What Next??

With no clear path toward improvement, Al's doctors decided they needed to figure out what to do with him. They brought his case to the weekly cardiology meeting where all the cardiologists in the hospital got together to review all of his history. They decided that his only viable option for the long-term would be transplant.

The next day, there was a care conference for everyone involved to discuss Al's future. Mom and Dad were there, along with Gma Ja and Gpa G, Dr. P, Dr. J, and M, the social worker. Basically what it came down to was that no surgeon would be willing to do the 3rd stage of surgery for Al - it was too much of a risk where even if he someone magically recovered, he would likely have the same problems next time around.
The only real chance to have a normal life would be to try a heart transplant. Over the next couple weeks, everything came into place to list him, which meant he had to be ready any moment to go into surgery. Al met A, his awesome coordinator who has done a great job taking care of him. After finally deciding on the transplant, the goal was to work at getting home.

He got to have some fun while at the hospital. OT and PT came all the time to play. Got tons of toys from home and from way too many presents. One of the favorite things - mouth swabs. They are pink sponges on a stick used to provide oral care. They taste good and he loved to suck and chew on them for hours.
Mom and Dad were amazed one day to see the pulse ox machine show a saturation of 100%!!!!

To try and get more stable, Al went on even higher doses of the diuretics. He also started taking a drug called sildenafil (viagra) to dilate his pulmonary arteries and hopefully get more blood flow to the lungs.

One Saturday afternoon.. there was a carnival right in the lobby of the Children's hospital. Al went to check it out. While there weren't many games for a kid his age.. he did "win" this blue ball with Tigger on it. A little easier to play with than the giant purple ball.

As he started to feel better, Al got to go on walks in his wagon. He would go around the hospital looking at fish, watching the giant "ball" machine and all over the place outside getting some rays.

Alan also started a new drug, Milernone. It is an IV drug given continuously for heart support. He would be continuing this drip if/ when he went home.

Eventually Al's PICC line went bad and so he went in for a very small surgery and got a Broviac line put into his thigh. Its a type of line (like an IV) thats a little more stable than a PICC line.

It was a chaotic time. Dad was working at a boring job. Mom was working but could do most of it around the hospital on a laptop. Whenever mom and dad were gone the nurses and CA's and unit secretaries loved getting a turn to go play and snuggle with Al.
Grandma and Grandpa also visited all the time and sometimes stayed for sleepovers.

One of Al's friend's mother from the UK sent a present one day. It was a book, Going On a Bear Hunt. When this friend was a baby, he would always read the book when he was in the hospital having his heart worked on. He's a big boy now, but his mommy thought of us and wanted Al to get to read it with his parents. It quickly became one of the favorite books.

Al started to get better and better to where there was finally hope that he might actually leave. He had spent so much time at the hospital that mom and dad wondered if he would even remember being home.
Al's friend C finally got a little better and got to move to the NICU where he could spend more time being a baby and be a little more comfortable.

Well after 12 long weeks.. one day, Al decided he'd had enough of this place. He got dressed, packed up his bags... and gave hugs to all his friends.

And he said.. "I'm outta here"
He went home and lounged on his sweet couch. While he was gone, mom and dad had gotten some new couches. They had gotten a special little couch just his size.
The verdict "Perfect! I think I can take up this whole thing."
Now it was time to wait wait wait.