This may get long, but here goes.
Tin Man was born with Transposition of the Great Arteries. Meaning that the 2 main arteries were reversed. Because of the anatomy of his heart he had 2 blood flow loops in his heart. One sending blood from heart to the lungs and back into the heart. The other sending blood from the heart to the body and back to the heart.
He was born at a time, and a place where they still weren't sure how to really "fix" this. Not long after he was born he had a balloon cath to poke a hole through the wall between 2 chambers. This would cause the blood to pool together to get some oxygenated blood to the rest of his body. He was still very blue, but this bought the doctors some time until they could figure out what to do.
He had the Mustard Procedure done at 18 months old to put in a baffle that mixes the blood together so oxygenated blood is sent all over the body. He was only the 2nd baby at that hospital to have this surgery. After this surgery Tin Man's blood flows was reversed. A normal heart the blood flows from the heart to the lungs to the body. In Tin Man's case, the blood flows from the heart to the body to the lungs and back to the heart. The blood flow from the lungs to the heart backs up into the lungs causing high pressures. These pressures have caused some damage to the lungs already.
The doctors never expected Patrick to be very tall. Little did they know his body planned to prove them wrong and he grew to be 6'4. They figure he outgrew his baffle around the age of 9. Nothing was ever said to his parents about this, so nothing was done to ever repair it, or make it larger. So the baffle he has is narrowed. They put a stent in it to help open it last June, but its still very narrowed. The doctor explained it as a sink with clogged pipes. Blood comes rushing toward the narrowed baffle and can't get through as fast as it flows, so it backs up into the lungs, which causes the high pressures.
In normal anatomy of a heart, a person with pressures as high as his wouldn't get a new heart because the new heart wouldn't be able to pump against these pressures. But with Tin Man's heart, once the new, healthy, heart is in, the blood flow will be normal, so the blood won't be backing up into the lungs, causing the pressures to go to normal.
The anatomy of his heart also makes things much more complicated because they can't go by the same numbers like they would with a normal heart.
They also don't measure is EF (or Ejection fraction is a test that determines how well your heart pumps with each beat). We did ask the doctor one time what his EF was and were told about 20, but that this wasn't an accurate test for him because its not a normal heart.
Every heart transplant case is different, but Tin Man's is very different because he doesn't just have a sick, weakened, enlarged heart, he also has a deformed heart.
The long journey
Tin Man was born with a heart defect called Transposition of the Great Arteries. Now remember this was 36, almost 37 years ago.
When he was 18 months old he had open heart surgery called the Mustard Procedure. This put a baffle in to help the oxygenated blood pool with the unoxygenated blood.
After that surgery everything was fine until he was 11 or 12. He started having issues with his heart rate dropping at night so he had a pacemaker put in. That was the beginning of a lot of pacemaker surgeries between infections and broken wires.
Tin Man and I met in 1997 when he was 21 (I was 17) and he was fine then. No problems. At that time he was still seeing his pediatric cardiologist (which at the time I thought was odd. I soon learned its because he couldn't find an adult cardiologist that knew anything).
Fast fast forward to 2004. His pediatric cardiologist was retiring. His replacement refused to take on an adult patient so we were without a doctor for him. We spent several months calling cardiologists all over Southern California. In Feb 2005 he got sick, and we both assumed it was a cold. Then he started getting sicker and I was worried he had pneumonia. Between his work and him being stubborn he wouldn't go to the doctor. Finally I threatened to take the kids and leave if he didn't go to the ER. I told them I wasn't having them wake up and find him dead in the bed. So he finally agreed to go. We were barely in the door when they threw him in a wheel chair and ran with him to a room. We weren't even there an hour and they were in telling us that he was in congestive heart failure and they were admitting him. He spent a week in the hospital getting meds adjusted and new meds added.
He was assigned a cardiologist then. This was one doctor that we had called and was told he didn't know how to treat him so didn't want to take him on as a patient. Ha! Now he was stuck with him.
He didn't do much, just monitored medicines, blood tests, EKGs, and echos.
Come April 2007. My grandma was in the hospital so my sister and I drove from California to Illinois to see her with 4 kids. We were in Illinois for 2 weeks. When I got home, Tin Man was sick. Told me it was just little cold, no big deal. That night (I hadn't even been home 8 hours) he came and woke me up and said he needed to go to the ER. Again we get there and they grab him and take him back right away. This is when I found out that he's been sick almost the entire time I was gone. His mother told him it sounded like her cold and that it was no big deal. I was pissed!
Again he's in congestive heart failure. This time he was only in the hospital 3 days. When he was released we went to see his cardiologist a week later. He decided that he wasn't comfortable just monitoring Tin Man anymore and he needed to go see the congenital doctors at UCLA. We were happy. You know, UCLA, great hospital, great doctors. We knew he would be in great hands.
Boy were we wrong.
The doctors we saw were rude to us. Treated us like we didn't care about his health so they shouldn't either. Then told us that one of the medicines he was on screwed up his thyroid so they couldn't do anything until he got it checked out. They told us he needed to see an endocrinologist and once he got into see one, to schedule another appointment.
They refused to give us a referral, told us to get one from his primary doctor or his normal cardiologist. Both of those doctors told us that UCLA needed to give us the referral since they were the ones requesting it. 3 months of fighting between these 3 doctors and we finally got one from his normal cardiologist.
The endocrinologist told us that he didn't know why we were there, there was nothing wrong with his thyroid. So back to UCLA we go. Again they treated us like we were horrible people because it took so long to get in to see the endo. They then told us that Tin Man wasn't sick enough to be there, and pretty much told us to get out.
In May 2008 we decided we were moving. Tin Man's job was really bad. We never got to see him because they required him to work 60+ hours a week. If he tried to take a weekend off they threatened to fire him. He was complaining to a friend of his and they decided to offer him a job as office manager of their tow company.
This meant moving 400 miles from home. We went back and forth, but we finally decided to do it. Tin Man moved up here in July to start work and find a place to live. The kids and I stayed home because they were still in school (year round school) and so we could pack. Once school was out the kids and I drove up. We got there on Friday, school started on Monday. So my poor kids didn't get a summer that year, but neither of them seemed to care.
At that time we thought our moving up here was because Tin Man needed a less stressful job and I wanted the kids in better schools. Also moving away from the mother in law was a plus. It wasn't until August 2009 that we found out the real reason was Stanford.
In the beginning of June 2009 Tin Man was fine. We were again having a problem finding a cardiologist up here. Hell we were even having a problem finding him a primary doctor. No one was taking on new patients. This was the downside to moving to a much smaller area.
We just finished school and were starting summer vacation. A few weeks later he got sick. We both figured it was a cold. When we was still sick a few weeks later I knew that it wasn't just a cold. He was determined to find a doctor and not make a trip to the ER. July 12 he was getting out of the shower and noticed his lips were blue and decided to give up on finding a doctor and go to the ER.
We got to the ER and he couldn't even walk inside. He walked 20 feet from the car to a bench and couldn't go any farther. I went in to get a wheel chair and instead of giving me one, they played 20 questions as to why I needed it. I told them my husband was sitting outside and couldn't breathe. Once I mentioned heart problems, they jumped up and decided they should go get him.
When they got him into a room and hooked up to the heart monitor they saw that he was in A Fib, but ignored it. They assumed it was normal for him, so they weren't going to do anything about it. This is also the reason why this round of congestive heart failure wasn't the same as the last 2 rounds.
This damn hospital admitted him for less than 24 hours. Gave him a few shots of Lasix and sent him home. Thankfully they were smart enough to send him home with a referral to Stanford.
Unfortunately we couldn't get in to Stanford until August 26th.
Between the hospital trip and the Stanford appointment, Tin Man was sick. He couldn't breathe and would get dizzy if he walked more than 20 feet. He was in bad shape.
When we get to Stanford, we had a little way to walk to even get into the building let alone from the doors to the check in desk. Tin Man being the stubborn man he is, refused to use a wheel chair.
They had to many people scheduled for echos so they were sending him to the other side of the hospital where the in patient echos are done. From the car to the in patient echo area was about a half mile. During the echo he was having a lot of trouble breathing, which the tech noted a few times. Then after the echo he had to walk back to the doctors office.
We were called back to a room right away. We were in the room less than 5 minutes before the doctors came in. 2 doctors. One was a cardiologist and one was a pediatric cardiologist. The cardiologist was telling Tin Man since he was a new patient they were going to admit him and run some tests. He promised it would only be 24 hours, 2 days at the most. The pediatric cardiologist was shaking his head, but didn't say much. During this appointment they also found out that Tin Man's pacemaker wasn't working right.
That was on Wed. On Thursday they spent most of the day trying to figure out what to do with the broken pacemaker and if they were going to remove it or just leave it and put another one in in his belly and snake the wires up under his ribs.
On Friday they took him back to do ct scan and internal echo. I was sitting in the waiting room when the pediatric cardiologist, Dr. Talluto, walked past. He saw me sitting there by myself and came over and sat down. Told me no one should have to sit in a waiting room by themselves at a time like this. I explained to him about the problems we were having trouble finding Tin Man a cardiologist and he told me he wasn't surprised. That up until about 15 years ago not many kids born with heart defects made it to adulthood. So cardiologists have no idea how to treat them. He told me that he was a pediatric cardiologist but also worked as an adult congenital cardiologist. Meaning he saw adults with congenital heart defects.
He explained to me what they were doing, what they were hoping the results would tell them. Then told me what the next steps would be based on what the results were. Even after they were done with Tin Man, the doctor told me he wasn't leaving until he was sure I didn't have any more questions. That was the moment I realized that was the real reason we moved up here. These doctors were the total opposite of UCLA. They did and still do actually care about their patients, AND their families.
On Sunday I took the kids up to see their dad. When we walked in I could tell by looking at him that he wasn't feeling good. So I told the kids we were only going to stay for a little while so he could rest. Both kids climbed up on the bed with him and were watching Spongebob with him.
I was slowly watching his heart rate rise from 140 to 150 to 160. When he got to 170 he called the nurse and asked if he could have another shot of Lasix. Apparently this happened earlier in the day and the extra lasix helped.
Not this time. About 5 minutes after he got the shot he looked really bad. He asked the kids if they could get up real quick that he needed to get comfortable. He sat up and I swear it was like the exorcist. Projectile vomiting everywhere. I grabbed the kids and took them into the hall and then ran down to the nurses station. Then I took the kids and we went and sat out in the waiting room.
After a few minutes I went back in to see what was going on. At that point he seemed to be doing better and asked if I would bring the kids back in so he could apologize or scaring them. I brought them back in and they barely walked into the room. They were scared to get to close. The nurse told them they could hug him that it was ok, and both ran up to him. Which I think helped him. Then we left.
On the way home there was a small 5 mile stretch where I didn't have cell service. When I got home I had a voice message. When I listened to it it was nothing but moaning and heaving breathing. So I freaked out. I called Tin Man right away. First on his cell phone and no answer. Then on his room phone and no answer. Then I called the nurses station and was told that his nurse would call me back.
So I'm freaking out. 10 minutes later his nurse, not the same nurse from an hour before, calls. After the kids and I left, things got really bad. His heart rate went up to 250 and higher. She explained that they put a line in his groin to push meds, they had to shock him to get his heart to slow down. She told me that he was stable but he was in the cardiac ICU now. She gave me the new number to his room. I called him and first things out of his mouth was I'm in a room by myself and they told me you could stay all night. Will you come up here. I knew then that it was bad and he was terrified.
I dropped the kids off at a friend's house and rushed up there. I've never seen him look that bad before.
The next day, on Monday, the doctor explained to us that the top part of his heart and the bottom part weren't communicating they way they were supposed to. The bottom part of his heart sped up and instead of the top telling it to slow down it sped up too.
They said this put a damper on their plans because they weren't sure what else might be going on with it.
On Tuesday, Sept 1st they decided to go ahead and put in the new pacemaker and shock him to get it out of A Fib. The surgery went well and if everything went well he was going to be able to go home in a couple of days.
Yeah, didn't happen. He ended up with a collapsed lung from the surgery. Then they couldn't get his INR (blood thickness) where they wanted it and didn't want to send him home until then. They were worried about his heart flipping itself back into A Fib and throwing off blood clots.
Finally on Sept 8th, 13 days in the hospital, he was discharged.
March 2010 things got bad again. Both of his doctors at Stanford, the peds cardiologist and the heart failure doctor, along with his dentist decided that since a transplant was in the future, removing his teeth would be the best route to go.
March 1st he had the surgery to remove all his teeth. The surgery itself went well. Waking him up from the surgery proved to be a big problem. His oxygen level dropped into the low 70s and they had a hard time getting them back up. So this landed him in the hospital for a week.
That was when they realized things were worse they they thought. That his heart is so weak and tired that any little thing can push it over the edge. They decided to get him on the transplant list as soon as possible.
This is where it gets complicated. Because of the anatomy of his heart, his blood flows backwards. A normal heart the blood flows from the heart to the lungs to the body. In Tin Man's case, the blood flows from the heart to the body to the lungs and back to the heart. The blood flow from the lungs to the heart backs up into the lungs causing high pressures. These pressures have caused some damage to the lungs already.
Starting in April 2010 Tin Man has had many many many heart caths. They wanted to know the pressures in his before and after they give him nitrous oxide. The before numbers show them what the pressure is like right now. The numbers after the nitrous oxide show them what the numbers will look like after he had a transplant.
Because he has a congenital heart defect, his heart caths have to be done by a pediatric doctor. The pressures in a child and the pressures in adults are different, but the peds doctors were reading them the same for both. In that case it looked like Tin Man was going to need a heart and lung transplant.
The first time his case was presented to the transplant committee they decided they wanted more info. So another heart cath. This one showed the same pressures. This was also when they realized that the doctors were reading the pressures as they would for a child. So his heart failure doctor went in and looked over the numbers and found out that they are a normal, a high normal, but still a normal range for an adult.
So again with this information they presented his case again to the transplant committee on Dec 3, 2010. We got a phone call at 11 AM that morning telling us that he was accepted for transplant.
This was our Christmas present last year in 2010.
They Found A Heart!!!
Tin Man waited 15 months and 3 days for his transplant. He got the call they had a possible heart for him on March 5, 2012 at 12:05 PM.
That 1 phone call turned our lives upside down, and little did we know, it would have a devastating out come.
We got to the hospital at 2:30 pm, and had to wait. They wanted to do a kidney ultrasound because we'd found out only 2 weeks before that there was a good chance he would need a kidney transplant too. Kidneys looked good, and everything was a go.
They took him back to the OR at 3:15 AM on March 6th. At 5 AM I was updated that they got the go ahead and had made the incision. 10 AM another update telling me they were sewing in the new heart. It really happened!
He was out of surgery and in ICU around 3 PM.
Almost immediately he started having complications with his blood pressure bottoming out. 2 days after transplant they had to take him back in for emergency surgery because they thought he had a blood clot around his heart. There wasn't a blood clot, but his heart was swollen to twice its size, so they had to leave his chest open for 2 days to allow it to have room to pump while they get the swelling down.
After this complication was over, then started having problems with his kidneys. Kidneys weren't wanting to work, and because they weren't working the way they should, his blood gases were all out of whack and he wasn't breathing on his own because of it. Instead of sticking him on dialysis, they forced his kidneys to work until they shut down. On March 16 they (finally) put him on dialysis, and instantly his blood gases were better and they were able to remove the breathing tube in just a few hours.
I am so thankful that the kids were able to come up and see Tin Man that day.
Tin Man had some complications with the medicines and his fingers and toes turned black. Really black. But for a week and a half, those were the only complications. He was doing great. Joking with the nurses, begging to eat food, and even asking when he could go home. At one point he was going to unhook himself and check him out because the nurses were pissing him off.
Tin Man had his first heart biopsy on Monday the 26th. 4 samples taken, and all 4 showed 0 rejection! His body had accepted the heart with no problems. The doctors told us this wasn't common in adults and that there probably wouldn't have been another heart that was more perfect for him.
We were both so excited. This mean he was finally on the road to recovery and we could start making plans for everything he would finally be able to do.
On Tuesday March 27th he was so excited when I got there. He had passed the swallow study and they were going to let him eat the next morning. He had a little problems with his blood pressure being low, but they didn't think much of it. That evening his oxygen and blood pressure dropped, which concerned them. He also coughed up what we thought was a little bloody mucus. I stayed at the hospital until his blood pressure and O2 were back up to normal and then went to the apartment across the street from the hospital to sleep.
Just before 1 AM I got a call telling me that he was having a little trouble breathing and they were going to put him on Bipap.
Just before 8 AM on Wednesday the 28th I got a call that they were putting the breathing tube back in and that Tin Man wanted to talk to me before they did it. I got to tell him I loved him, and he told me the same. It took less than 45 minutes to get dressed and get to the hospital, and in that time he got bad, really bad.
We were told that if his brother wanted to see him alive, he needed to get there and fast.
They weren't sure what was going on but they thought he had a blood clot in his lungs. I was praying he didn't, but now wish I had been praying that's all it was.
They put him on an ECMO machine (lung bypass) so they could buy him some time to figure out what was going on. Once they got him stable (8 units of blood later) they did a CT scan. This showed that he did not have a blood clot, but that he had something in his lungs.
This was when my world was turned upside down. They did a scope into his lungs so they could see what it was and try to remove it. The things in his lungs were blood clots. The insides of his lungs were bleeding, and not sure how long they had been before it caused problems.
Early in the morning on the 29th the phone calls started that they were having problems with the ventilator because he was bleeding in his lungs, a lot of bleeding. Finally at 4 AM I got the call to get to the hospital as fast at possible.
When we got there we were told that he had been without oxygen for a while because he was bleeding so much that his lungs had filled with blood and there was no room for oxygen. So on top of everything going on with his lungs, they were afraid of brain damage too.
They took him into the cath lab that morning to insert clots in the area that was bleeding in hopes to stop the bleeding. After the bleeding stopped, they took him into the OR to do another scope and to clean out his lungs. Later that afternoon his lungs started bleeding in another area.
That evening we were called in to talk to the doctor and heart transplant nurse practitioner. We were told they've never seen anything like this before and weren't sure what to do. They had 2 options and the teams were trying to figure out which was the least dangerous and least likely to kill him.
They finally decided to stop all blood thinners (which were required for being on the ECMO) but keep him on ECMO to bypass his lungs in hopes that they will stop bleeding. This meant having a backup ECMO machine in his room, so if he did clot in the machine they could switch it right away. Thankfully he kept clotting in the dialysis machine, because that was a lot easier, and safer to switch out.
They finally took him off ECMO on Monday April 2nd. He did good. Pressures in his lungs were a little high, but the heart was doing good dealing with them. A day or so later they lightened up on the sedation a little so they could get some idea on where his brain was at.
I went in to see him and when I said his name he opened his eyes. I was so excited by that. Then when I asked if he knew who I was he nodded his head. Then I asked if I was random people and he shook his head no. The nurse asked him if I was his wife, and he said yes. I was so happy to know that he was OK!
He was doing good until Saturday the 7th. When I walked in I could tell that he had had some issues during the night because he was on a new medicine, and his numbers were kind of low. I felt bad, but we had already made plans to hang out with the kids at a gokart track near the hospital. So against my better judgement, we left. I had been away from them for a month and they needed time with me as much as I needed time with them.
About an hour after we left I got a call I knew in my heart was coming. Tin Man coded, twice, and had to have chest compressions. The pressure in his lungs were high and his heart was starting to have issues pumping against them. They decided to put him back on the ECMO to buy him some more time so they could figure out what was going on. His lungs weren't bleeding again, they were sure of that, but they didn't know what was causing the pressures to get so high.
We rushed back to the hospital so I could sign the consent form and then wait. It took about 2 hours to get him on the ECMO and almost immediately he was doing 10 times better. Again they were worried about brain damage and all, but the main focus was on figuring out what was going on.
On Sunday he was good. Stable, O2 great, pressures in lungs were a little lower. Everyone was sure he was slowly headed in the right direction. They were going to give his body a chance to rest and then assess the possibility of brain damage in a few days.
That all changed by Monday morning. When we got there I was met at the door to his room by the nurse practitioner telling me that the pressures in his lungs were even higher (104/54) and they couldn't figure out what was going on.
About 2 hours later we sat down with the surgeon, another cardiologist, the NP, and the social worker to go over what was happening. I knew then that we were losing him.
The surgeon told us in his almost 40 years of doing this, he's never seen anything like what was going on with Patrick. He told us that they had 1 last ditch effort to try, but he wasn't optimistic it would work.
So I called and had friends bring the kids up to the hospital so we could prepare them for what was going on and what could happen. I had to look my kids in the eyes and tell them that their dad was dying. The kids handled it a lot better than I did.
I had explained to both of the kids that Tin Man wasn't responsive, but he could hear them so they could tell him anything they wanted him to know. Sugar Pea was first to go in and she walked up to his bed and told him that she loved him and he took a big deep breath and tried to talk to her. This scared the nurse, the girl running the ECMO, and me. He hadn't been at all responsive since Saturday morning.
I told him he couldn't talk because of the breathing tube, but that she could see his eyes. I asked him if he loved Jazzy too, and he started blinking his eyes really fast.
When Warrior went in he got the same reaction. I am so thankful that Tin Man made that last struggle to let them know that he loved them.
Just before 8:30 on Tuesday April 10th, I got a phone call telling me that he wasn't doing good and we needed to get to the hospital.
When I got to his room I knew he was gone. It didn't feel like Tin Man was there anymore. I hoped and prayed that I was wrong, but I knew in my heart that he didn't have the strength to fight anymore.
At 9 AM I told the doctors and nurses to stop everything and shut everything off. That was the hardest decision of my life.
Tin Man died peacefully at 9:07 AM on April 10, 2012.
Not only were we sobbing in the room, but his primary ICU nurse was, the NP, and even his heart failure doctor came in and was crying with us. We were told that Patrick and I went beyond being a patient and his wife, but we became part of their family. No one wanted to see this outcome and everyone was praying just as hard as we were that things would have ended differently.
The doctors requested an autopsy to see if they could get some answers as to what happened. I was going to request one myself because I need answers. I had to wait 6 weeks for the results. I got a call May 31 that they report was in and they wanted to schedule a time for me to come up. I couldn't that day. It was Warrior's 8th grade graduation. It was already hard enough and I wasn't about to ruin his day.
On June 6th, 3 months to the day since he had his transplant, I sat down with the transplant surgeon and the heart transplant nurse practitioner to find out the results of the autopsy. The autopsy showed that Tin Man had 1000s of micro blood clots all throughout his body. We were told that they stopped counting after 400 clots in just his lungs. The first area, the lower right lobe, where the bleeding in the lungs first started, was about 2 - 4 weeks old. These blood clots cut off the blood flow to that part of his lung, causing the tissue to die off. This is what started the uncontrollable bleeding. Over those last 2 weeks he started forming clots all throughout his body. The clots in his lungs caused the pressures in his lungs to get higher and higher until his heart couldn't pump against it. They did tell us that he received an amazing heart. That it put up with so much more than they ever would have imagined. Even after all it went through and how hard it had to work, it still looked pretty good.
They don't know what caused the clots. He was on such high doses of blood thinners that he shouldn't have been able to form clots. Let alone as many as he did. Unforutnately this is something we will never know the answer to. UPDATE: I found out in August of 2012 that the blood thinner Tin Man was on in the hospital was also being recalled at that time. It had visible particulates in it, and if these contaminated products were used it could cause multiple emboli and multiorgan failure. Everything I have read reads just like his autopsy report.
The surgeon did tell us that they did learn a lot from the autopsy and what they need to do more clinical testing on in the near future. Hearing this helped ease my mind because they were able to learn something from Tin Man's death that will help others. This makes me feel like Tin Man's death wasn't for nothing.
I think about Tin Man's donor and that person's family a lot. My heart is breaking because my husband is gone, but my heart breaks for them too. From the minute we got that phone call, that person became my hero and their family became part of our family.
I may never get to meet them, or write to them, to thank them, but I hope they will always know that I am and will be forever thankful that they gave my husband a fighting chance at a normal life.