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.
