Oct 24, 2017
Seema Hess and her husband John (one of phaware’s co-founders) are caregivers to their son, Iain, who was diagnosed with pulmonary hypertension at age 5. Seema shares a raw account of Iain's recent double lung transplant surgery. She details the four months Iain spent in ICU. Seema also shared this experience through a Children’s Organ Transplant Association blog: cotaforiainh.com/blog.
Hello, I am Seema Hess from Boulder, Colorado. My son Iain was diagnosed at the age of five with idiopathic pulmonary hypertension.
When he was diagnosed he had super systemic pressures even though he seemed to be asymptomatic. It caught us all off guard. It even caught his pediatrician off guard because we never went to see her. Iain was never sick. We would see her once a year when we went in for our annual checkup and that's when she heard a loud second heart sound because we were planning on a big family trip to India, she wanted us to be seen by Children's Hospital, but nobody was particularly concerned. We thought it was perhaps a murmur as my husband's father had one.
We found out then that he had pulmonary hypertension but that he would need to have a heart catheterization to confirm. The prostacyclins were just becoming a little bit more prevalent in the community for severe pulmonary hypertension. We had done our research and we knew that when Dr. Ivy came in to talk to us that we were just going to have him put in the port, so that he could have the IV prostacyclin. Many people know [the medication] has a half-life of four minutes, which meant that as his body became more used to it, he would only have 30 minutes before all his blood vessels shut down if the line went down or the medication was stopped.
After a few years Iain was transitioned to another medication which had a longer half-life and gave him and us a little bit more freedom. It meant he could go on playdates which he had not really been able to do before because it was too dangerous. That worked out for a while. He was on some other medications, and honestly, he did pretty well. He had to stop playing soccer I think when he was about eight as that got too much for him. Then he transitioned
He was also playing baseball. I think he had to stop when he was about ten or eleven, and that was very disappointing for him. He loved, loved baseball. After that, he transitioned to climbing because it didn't require quite so much cardiovascular strength. And then I think when he was about fourteen, fifteen really, we could see his endurance declining pretty rapidly.
At that time, we started to look at transplant facilities, ultimately in the end we did opt to go to St. Louis for a Potts Shunt, and the idea was that hopefully would have put off the transplant. Unfortunately, it didn't work. Iain's condition was too advanced by that time. And soon after it was pretty evident that he was declining so quickly that he had to be listed rapidly. And so, we made the decision to move out to St. Louis and split up the family. And my younger son went off to California to live with my brother and we went to St. Louis.
Iain's health was going downhill even faster at this point to the point of early January, everybody was getting a little bit more concerned about where he was on the list. Iain was getting offers but the surgeon Dr. Eghtesady and Dr. Grady and the team were not thrilled with those particular lungs that came in. It was good that Iain was getting offers but we were getting very concerned that the right ones were not coming in, perhaps these had been already rejected by other centers.
We were hoping to get him pushed up on the list. But before that happened, fortunately, we got a call late one night and I was still awake and Iain was still awake, and I lay in bed and I heard the phone ringing. And the phone next door it seemed like that, in the next apartment just kept ringing and ringing and ringing and ringing. And then finally my phone rang and they said that they had the perfect lungs come in, and would we in the next two hours get Iain bathed and ready to go into the hospital for about 2 PM.
For me it was almost like waiting for the baby to come. I was excited, a little bit scared, and I think Iain was the same way. We went in, they were prepping him. He was due to leave us about 11 AM I believe. That day was a little strange now. The surgery was going to start at 12. So, he was a little ... We were all a little bit excited, a little bit apprehensive.
So that morning before he went in, it was a little bit relaxed. A dog came in to visit him, even one of the therapy dogs, so that was quite nice. And then he was wheeled away, and we were shown to a specific little room. I don't believe I actually left that room because I was too afraid to leave it. I was still pretty excited though about these lungs coming in and we knew approximately what time they would be coming in. They were coming in at 4 PM and sure enough about 4:10 PM, we heard the helicopter come over, and they called us just 10 minutes later to say that those lungs were in, and they sent us down a picture of the lovely fresh healthy lungs.
At about five o'clock, one of the nurses was going off, and she came in to say that they were beginning to clean things up. They would be prepping him to be closed up. At 7 PM the new nurse came in and said that, well, there was some bleeding, but you know this was pretty common and it shouldn't be too problematic.
At 8 PM, I said to my husband, John, “we haven't had any news, and this seems to be taking a long time.” I think it was probably soon after that that they came in and said that Iain was bleeding heavily, and that it was a problem. We didn't panic at that point, but obviously it was scary. About an hour and a half later, I think that was when the surgeon came in and said that they really couldn't stop the bleeding and they were doing everything they could.
At that point, we were very upset because it looked very bleak. Things looked very bleak indeed. We were very upset obviously, but it would cycle through, he's going to die, he's not going to die, we can't possibly die, we can't lose him through our heads, and it would just cycle through.
At about eleven o'clock I believe they called in Dr. Eghtesady because the surgeon, whose name escapes me right now, but he had already been on I think over 24 hours doing other surgeries, even before the transplant, and only had an hour break before he gone into Iain's surgery. Dr. Eghtesady came in and explained what the plan was to try and find out where Iain was bleeding from because they just couldn't find it. They knew that perhaps one of the issues was as he had the Potts Shunt, there were a lot of adhesions that had formed.
When they had taken the lungs out this had caused the bleeding. This is one of the issues anyway. But they couldn't quite hone in on where the bleeding was. So, they continued trying. At this point it's about one o'clock. Dr. Eghtesady came in and said they thought they were ready to actually close him up. We were relieved but he was definitely not out of the woods. We knew that Iain can stay on the ECMO.
We knew that being on an ECMO machine is not a good thing, a good thing that they have them to keep people like Iain alive. But I think the statistic that Dr. Eghtesady told us were that 50% of kids do not leave the hospital if they've gone on ECMO. However, Dr. Eghtesady was ready to close up. He went and he came back 10 minutes, less than 10 minutes later, to say, no in fact, they couldn't because he was still bleeding too much. And again, that starts the cycle of what's going to happen. He's going to die. Dr. Eghtesady explained what he was going to do, and it was going to be a little bit more problematic than they had hoped. He said he'd have to take the lungs back out to try and find the bleed. It was upsetting, but of course they had to do what they had to do. So, you just have to accept it at this point.
I think it was probably around 2:30, 3:00, and Dr. Eghtesady came back and said, actually they didn't have to take the lungs back out, and they had padded. They'd put a lot of padding into the chest cavity, but they were going to keep it open, because they still did not know where this bleed was coming from. And that after that they would leave the chest open but they were going to try and stabilize enough to get him back up which they did.
So, I think it was about 4:00 AM. They took him up, and then we went up probably around 6:00 or 7:00. The room was packed full of people of, all sorts of people. The ECMO machine, the nurses who were managing the ECMO machine are unbelievable. Those people worked like navvys. It was just unbelievable to watch. The team work with everybody.
It was almost too scary to go in there because we didn't want to disrupt their flow. It was just amazing. Obviously, Iain was not awake. There was just so much paraphernalia around him that we couldn't get close to him. I think we might have been able to touch his toe but we really, really didn't want to disrupt the flow there.
A specialist would come around and talk to us, and I know I talked to Dr. Blatter about whether or not we should be bringing our other children out to see Iain because he was obviously in a very precarious situation. I didn't know. I didn't know what to do. We didn't know whether we should cause them that anxiety that their brother wasn't awake. Their brother was not the brother that they knew and it would have been incredibly scary to see him with his chest open and not making any eye contact, and just not awake. In the end, I opted not to bring the other children out. I think looking back it was probably the right thing to do even if something terrible had happened. I just wouldn't have wanted them to go through that agony. It was awful, more awful for Iain, but he wasn't aware of it.
Those first few days were precarious. Iain was on ECMO, and he although stayed somewhat stable the bleeding is still happening. But it was easing off a bit. And on this third day I think they decided that they could close him up, which was a relief and that he could come off ECMO. They did a test beforehand I believe to make sure that he could come off ECMO. So, it was determined that Iain had suffered pretty significant damage to the lungs because of all the blood products he had received. During the surgery, the team had instituted the massive transfusion protocol twice on Iain, which meant that they didn't even have to send down orders for blood. It just kept coming up for him. He went through so much blood product. That blood obviously kept him alive but the sheer quantity that went through him damaged the lungs.
There were a lot of differing opinions between all the experts how to best treat this, and the other thing that people were aware of is that they really wanted to do a biopsy. But he was just not in a good spot to go down for a biopsy. So that was put off. But one of his amazing critical care doctors suggested rotating him, that this had been done on adult patients, and I don't think they had done it very often on pediatric patients, but given Iain size they decided to try it. And this is pretty problematic given all of the chest tubes that were still in Iain, and these were massive chest tubes because he was still bleeding.
So, the first time they decided that they were going to be rotating him. They were rotating him so that his face would be downwards, and they wanted him about five to six hours facing down and then back on his back. This was a big production and I believe the first day they did, that there were 12 people to turn him. They continued doing that. And in the end that treatment was successful. However, Iain had, in all of this time, had lots of medications to keep him both paralyzed and out of it because when they would try to lift the sedation, Iain would be clawing at the tube going down into his mouth because he was clearly gagging on it. But he needed it. So, they kept him heavily sedated and chemically paralyzed.
As the graph dysfunction got better they started to try and wean him off some of the heavy sedation. After they started to bring him out of the various drugs because he needed really to be moving, but it became clear that Iain was going into a delirium.
We'd have brief flashes where we would see Iain, but on the whole, he was in this, what they call, an ICU delirium, which is fairly common if you've been under a lot of sedation for a long time which he clearly had. How to get him out of this ICU delirium, again various people had different ideas, but he was definitely supposed to sleep at night but he didn't. All of this caused problems. They decided that they were going to give him a certain type of drug.
As this happened, Iain started becoming worse mentally and started clutching at his body and his limbs would become almost rigid. And then there would be times where he seemed to be interacting. But it really was not Iain. So, we were pretty worried about his mental state at this point along with his physical state.
One evening my friend was there and she and John were trying to drag me out because something just bothering me about him, and I didn't want to leave him but they dragged me out. Fortunately, I had asked one of the other nurses, not the one caring for him that particular day, but one of the nurses that we knew too, just looking on him and she did. She felt his forehead and he had a fever. The next day his fever was rising to the point where I think it was about 106, and one of the critical care doctors said, “Oh, Seema, don't worry, your brain only melts at 108,” and I went, Oh, OK.” What I didn't realize was there's a sheet in the room which actually states what the temperature is in Celsius and Fahrenheit, and that there's a point where it stops.
I wasn't freaking out at this point, but I was certainly worried. And at one point I came in and I just said, they had been waiting for a cooling blanket but it wasn't coming up, so I just asked if they could just put some bags of ice around him. At this point, somebody told me what the temperature was, and I didn't convert it, but I converted it later on and it was 107 point something, which was awful.
He was really, really, really sick that day. Terrible. It was awful. You could see the decline, you could see him declining. You could see he wasn't himself, in a terrible state, and maybe in a way that was the worst day for me because at that point I was questioning whether we'd done the right thing. That day in particular, because I didn't want my son to suffer, and that day I could see he was suffering badly. It was horrible. It was really terrible.
However, again they had every specialist run through. Everybody was there. One of the neurologist came in and I heard a conversation and he questioned one of the medications. And then we got the diagnosis, the possible diagnosis which was the neuroleptic malignancy syndrome, which seemed to answer the problem. And this was a very rare reaction an allergy almost to one of the drugs that helps treat the delirium. To try and combat that, he was put on to dialysis again, and he'd been on the continuous dialysis previously, but they were threading a line to put him back on dialysis.
And as they did that, they punctured lung. So, it was very eerie watching the entire CICU come to a stop and everybody just stopped and watched what was going on in Iain's room. It was almost like time stopped. As everybody watched. Nobody moved, and you could see almost the disbelief. And these are people who are dealing with children who are critically ill all the time, to see their faces was petrifying. But his doctor in there and the nurses were incredible.
They did not get flustered. Nobody yelled, nobody screamed. It was very, very methodical. They were truly unbelievable. He got through that crisis. He was still intubated, of course, and he needed to go, have a tracheotomy we're almost two weeks out of the surgery.
We let Iain make that decision. He was going to have to deal with it obviously with our help, but we had a brief few days where Iain was able to actually make these decisions and he was coming out of the sedation because he couldn't have a lot now. We felt he was getting better. His lungs were still damaged and he still needed to be intubated, and it looked like he was going to need to be intubated for probably two, three weeks at least. That's what we thought.
The decision was made by Iain to have the tracheostomy, because he hated having that tube down his throat. It was awful having to deal with it. Unfortunately, that requires sedation. And he went in back into this cycle of delirium which ended up most likely causing seizures.
The neurologist came in, and again we were worried about more brain damage. One day, one of the pulmonologists had come along, and I can't remember at what point she come along. She said, “Seema it's really good news. He's not in rejection but he does have pneumonia.” I am like, “Great.” This is really great news because rejection is much more difficult to deal with than an infection. He was treated for the pneumonia.
During all of this time, he still had to a greater or lesser extent a leak in one of the lungs but he stabilized somewhat. He was able to move a little bit down away from Room 5, which is the room in the cardiac ICU. During the time that Iain was there, another girl came in post-lung transplant. We were expecting her because we talked to her mother. John and I were astounded because she was wheeled in with two nurses, and I thought oh, that's what it's supposed to be like.
Iain was moved down to another room where it was a little less intense, but still ICU. I think it was at that point where he started to develop a little bit more of the pneumothorax and he had it, I guess they’re called pneumothoraces, because there were two of them that were on both sides. So, he still had chest tubes and there were attempts to try and move the chest tubes to help alleviate the air that was developing.
So, Iain's chest looked like he'd been shot. He had massive, massive holes in him, not to mention the great big scar down his front. But from that point on, he was out of the deep dark woods. if you like, not out of the woods, but we were certainly more optimistic. I don't think we stopped being optimistic. But there's levels of it. There were other issues that came up. Seemed he suffered something new every Thursday, but he got over each of those new problems slowly but surely. But he ended up being in hospital for four and a half months for various reasons, most of it towards the end because of the pneumothoraces that just would not heal. Eventually they did.
In between, I think he had suffered three infections which we now know caused permanent damage in the lower left lobe. He did suffer a lot from an inability to sleep which caused a fair amount of anxiety in him and us because it causes the seizures that he was having. He had a lot of follow-up from the neurologists because he had a little problem with his big toe. But in the grand scheme of things it wasn't a huge issue. But we knew that he had suffered some sort of brain damage. I remember at about, oh it was probably two weeks before he was due to leave, so four months, the neurologist walked past and did a double take, and said, “Oh, Iain, you look great. I never thought you would come out of everything you went through with your brain intact.” And he was, his brain was intact.
When Iain was diagnosed with pulmonary hypertension, I made a choice at that point to eliminate certain negativity in my life also to not make plans for the future because we never knew what was going to happen with Iain. I didn't want to make long term plans, even making vacation plans. We just didn't know if he was going to be sick or if he wasn't. We made some plans but everything was made with the assumption that something could change.
I think that also helped me as the transplant happened. And what I tend to do is create discreet little packets from each period, so I can just get through that little period and get through that problem, and I don't look at the long term. I don't look at what's going to happen if he dies, what if he died. I just don't go there. I just get through the problem that we're having at the time, focus on that.
One of the other things that John and I tend to be, we go together, but we don't have a lot of people around us. During all of Iain's hospitalizations, or Iain's treatment, I go on my own, or John and I are together. Certainly, for the big surgeries or any surgeries, John and I are together, but we don't have a big entourage. I think for us in particular that helps keeps us less worried.
I've noticed that if there are other people who perhaps, I don't want to say less worried, but less panic stricken. I've noticed that if there's somebody I'm close to, and they react in a different way then, that can make me more upset or make me start to panic. So, it's just generally the two of us. And generally, the two of us can keep each other somewhat sane going through this. We'll just talk it through.
When we knew Iain was going to have to have a transplant, the hospital suggested that we talk to COTA, Children's Organ Transplant Association www.cota.org. They do a lot of fundraising and help people fundraise. But what I found really interesting, was the blog system that they had. And my friend was going to help me communicate with our friends through the blog. We actually just got it started the day Iain went in for the transplant. It was really comforting and helpful for me to be able to communicate through my friends, and she was able to communicate with all these other people what was going on. And it meant I also didn't have to relive it for the whole community who were interested. But I could read their posts back and that was lovely.
Since I am not a social network person, I didn't realize actually how much strength I would get from that. I really, really, really found it helpful, to know that all these people cared. But for me I just have to divide every bad thing that happened to him into that little pocket and store it away. And a lot happened. There was so much stuff that happened that I didn't even put on the blog that Iain was going through. I did not put down everything he suffered.
I do have to say that when the day came for Iain to be discharged, the whole ward had a huge party for him. Everybody came to see him off. We left, but we were still in the St. Louis area because you have to stay for the full six months. And that was a little hard for Iain because he's in this sort of almost twilight area where he's getting better but he's not home, but he doesn't have all his friends and family around him. And it's just me who's boring, and certainly not as cute as the nurses or as interesting as they are, including the men who were also very ... There were some great men nurses there too. I have to say the nurses had really, really helped him to get through this emotionally. They were excellent, especially being closer to his age than me.
But the day came when we got him to come to Colorado, and he literally, we crossed over the Kansas Colorado border, and I could see him get better. It was so strange. He sat up taller in his seat. He got out of the car when we reached Boulder, and I let him. I took a photograph of him, and it was all snowy, and we'd left, I don't know, St. Louis it was probably 80 degrees and Boulder was snowy.
He blossomed, and I've barely seen him since. He's been out the door. He goes to parties, he's engaged, he's just living life to the fullest. It's amazing watching him. It's the energy that he has. It's fantastic. I saw the difference between him pre and post-transplant even when he was suffering from the pneumothoraces, the infection, probably two months after his transplant. I saw how he could walk further. It's just amazing. People look at him and are just, a lot of people just want to see him to believe that he's really here, that he's really alive, and he's really full on Iain. It's amazing. It's fantastic.
Iain, when he left the hospital has stayed in touch with a lot of the nursing staff, and all of them. And a lot of them are nurses who were in the CICU with him, and they have seen their fair share of problematic surgeries, but they all say this was the most difficult lung transplant they've seen. He had so many complications that they just had not seen before. They're amazed. They're all heavily invested in his success.
When I describe how John and I got through all of this is because we weren't going through it. I always keep in mind that Iain is the one who was suffering. We were observers. Obviously, his parents, but he was the one who was suffering through it all, and that's what I always keep in mind.
The other thing I always keep in mind, is that things could be much worse. Always know that it could be much worse. He might not have had the opportunity to have the transplant in the first place, might not have had that excellent care that he had. I think even if things had turned out badly, I could take comfort in the fact that he got the opportunity to have the transplant and he survived, he survived. I know some people don't. So, things could always be worse.
My name is Seema Hess and I'm Aware That I'm Rare.