Maria Simionato - phaware® interview 375

Jul 12, 2022

Italian pulmonary hypertension patient, Maria Simionato discusses her diagnosis and how a complications following a menstrual period that lasted for over a month led her to be listed for a double-lung transplant surgery.

Maria Simionato:
My name is Maria Simionato. I'm a 24-year-old student and full-time worker from Venice, Italy. My connection with pulmonary arterial hypertension goes back to March 2005. I was seven and a half years old and I was diagnosed after an acute episode of fever and shortness of breath.

I was fortunate enough to get diagnosed right away. I got sent to get an echocardiogram right away and a doctor that goes by the name of Lucia Tessarotto, who I'll always be thankful for, basically, told my parents right away what the disease was. After a couple of weeks, I got sent to Padua which is one of the most renowned hospitals in Italy. I got a right heart catheterization and my diagnosis was confirmed. Luckily enough, I did respond well to nifedipine right away.

I was fine and asymptomatic for most of my life. There is so much uncertainty and fear that comes with the diagnosis, obviously, because there isn't much that is known about this illness. My parents were terrified and they didn't know what to do, because in Padua, the doctors knew so very little.

Back then, there was no internet at that time. There was no information line. My dad, he cannot even speak English to this day. He ordered some books from the internet that were entirely in English. He would try to translate the information. But eventually we did find some pulmonary hypertension specialists in Bologna, which is fortunately enough, like an hour and a half drive from where we live. They've been taking care of me ever since.

By that point I was eight years old. I was luckily asymptomatic for most of my life. Sometimes, I would get chest pains. I would get some shortness of breath. I was generally very lazy. All those kind of things that you would normally associate with pulmonary hypertension. I knew that I would not be able to have children. I could not do any sort of physical activity. But I was content with the life that I was living.

I've always been very active as well. I've always worked. I kept up with my studies after high school. My situation however got worse. It was September 2015. I was just a month shy of my 18th birthday. So what a lovely birthday present. Basically, I fainted. I was at school so I got to the ER after a couple of hours, then I went back to Bologna to get another right heart catheterization. My pulmonary pressure got worse again. Opsumit got added to my therapy. I have to say that Opsumit helped tremendously. I had very little side effects. It was just like a miraculous drug. I know it helped a lot of other patients, as well.

At that point I was 18, I went back to live in my normal life. I finished high school. I started university and started working. I now work as for an insurance company. I was fine for most of my life until I wasn't. That's basically what happened. In January of this year, I did have this menstrual period that lasted for a month. I lost tons of blood. Basically, I did go to my gynecologist and everything was fine. He just told me that it was some hormonal imbalance. It wasn't that big of a deal. Nothing was found that may have been concerning.

Then the last week of January, I suddenly could not get out of my bed. I could barely walk. My oxygen levels, my saturation was at all-time low. My parents and I called an ambulance and I got sent to the yard. Obviously, what we thought would happen was for me to get some iron infusion. Given the fact that I have lost lots of blood, my hemoglobin levels were again at an all-time low. Everyone thought that it was just that sort of situation where we get an iron infusion and maybe a blood transfusion. No one was concerned. Not even my doctors in Bologna.

I did go to the ER. I did get a blood transfusion. The doctors that were there at the ER were like, "Okay, given the fact that you have such a severe illness and you're taking these drugs, we're going to hospitalize you for tonight and see how it goes, but it should be fine overall." I had an oxygen saturation level that was like 84 out of 100. So I did have a small mask for oxygen. I want to make it very clear that I had never had oxygen on in my life before. I never had needed it. I got an x-ray done, obviously, because again, severe illness. They wanted to see if everything else was fine.

My lungs were white. I remember a pulmonologist came in saying they thought I had pneumonia. At first, they thought it was COVID, but they got some rapid tests done and I was negative. I did not have any other symptoms. I got sent right away to the intensive care unit. I spent a couple of days there wearing non-invasive oxygen mask, also CPAP and all sorts of masks.

Apparently, what happened during those five days is that pneumonia was fading. I had all kind of drugs. I don't really know. I was hallucinating for most of the time, I think. After five or six days, it seemed that the situation was going slightly better. But at the same time, I no longer had this pneumonia, but my blood oxygen level, my gas exchanges, my saturation was awful. I was wearing oxygen and I would probably saturate around roughly 75 out of 100. I remember that each time I would even try to stand, I would just grasp for breath. I was obviously terrified. But apparently, this pneumonia was going away. Everyone, even the doctors were like, "Maybe it's fine." It wasn't.

I was sent to a sub intensive care unit which is like off way through an intensive care unit and like a normal hospital bed here in Italy. I spent a week there. February the 12th came around, which I like to recall as the worst day of my life for me and for everyone that cares about me. At midnight, my saturation levels were awful. I was not breathing at all. I got sent back into an intensive care unit. That was the first time I actually fear for my life because I did not understand what was going on. They kept telling me that it was this pneumonia, but this pneumonia apparently was going away. I was just like, "Okay, this is it. This is pulmonary hypertension finally killing me."

On the morning of February the 12th, my doctors came in and they told me that I was going to be sent to Padua. Padua is a much more renowned hospital again than the one in the city that I live in. They told me I was going to be sedated, intubated. Then eventually, they would try all sorts of drugs on me. Anything that would work against pulmonary hypertension. That's the first time I actually worried about the word double lung transplant. Of course, I've heard of it, but I was never fully aware of what that entailed as a whole. I was terrified.

The funny part about it is that I don't remember a single thing about that day, because I was so out of it and probably all the anesthesia that I was given later on. Pretty much everything has been deleted from my brain. They told me that as I said, I was going to Padua where lung transplant surgeries are performed. Again, I don't remember much, but I remember that I signed the paper where I was given them the permission to perform such surgery. Then it is just nothing. It was just nothing for two weeks. Basically what happened, I said goodbye to my parents which was awful.

What happened in the meantime, I was brought to Padua in an ambulance. I was put under ECMO. Obviously, ECMO is highly dangerous and highly invasive. You're at risk for infection. If I had gotten an infection at that point, I would not have been eligible for the lung transplant and I would've died. I'm just going to straight up say it.

Basically, I got there on Saturday, February the 12th at night. The following day, they tried all sorts of drugs in me, but my pulmonary pressure was reaching its peak of 150. I'm not joking. Nothing was working at all. So on Wednesday, February the 16th, I got put in the list for emergency double lung transplant. Thankfully, my body was fine. I could get the transplant. I had nothing else going on. So, I was fortunate enough to get one. I don't think I'll ever stop saying this in my life, "That was a miracle." Then February the 19th came and at 6:00 AM in the morning, the doctors called my parents and told me that a nice pair of lungs had arrived and I was getting prepped for the surgery.

I got into the surgery room around 7:00 PM and I got out of it eight hours later and the surgery went smoothly. I would like to thank also the surgeon who is brilliant. One of the best in the world that did the transplant, which is Andrea dell'Amore. I woke up a week later. This is a fun part. I don't remember anything at all. I had remifentanil which is a hundred times stronger than morphine. So, I did have trouble understanding what had happened. It took me four days to understand that I had a double lung transplant. I remember that I woke up. I could not speak. I had lost 30 pounds or something. I was a petite woman before, so I was like a skeleton. From then on, things got slowly better. After a week, I got sent to a normal hospital room and I started breathing on my own.

I started walking again. It took me a while to get back on my feet. Given the fact that I was also sedated for two weeks, I had paralysis on one foot, which was interesting. It has now gone back to normal. But of course, I had all sorts of physical consequences as my body has been through an insane amount of physical trauma. I stopped taking Opsumit and nifedipine after 17 years of my life.

This is the part of the story where everything gets good and it's actually harder to talk about. Here's the thing, living as a transplant patient is not an easy job, obviously, because you're always fearing rejection in the back of your head. The drugs that you're taking, the immunosuppressants, are not easy on your body. All of the physical and psychological trauma that you have gone through, because obviously, if you reach a point where you need an organ transplant in your life, you've obviously been through a lot. But I feel so much incredibly better now.

I had an enlarged right heart because of pulmonary arterial hypertension. Now, it has gotten back to normal. My heartbeat as slowed down. I have a perfectly fine heart now. My lungs are doing fine. So, I got sent home on April the 1st after exactly two months in the hospital. I have since gone back to get my checkup. I still have a little bit of rejection as in it's taking it's sweet time to get the therapy right and so on. But I'm feeling better than ever, even psychologically, because I never once thought that I would heal from this thing. I thought I was going to die from it. I thought all sorts of things and I didn't. I went back to visit my doctors in Bologna because I got the surgery done in Padua, but my original doctors are still in Bologna.

When they told me that I didn't need to go back to them because they consider me as someone that has healed from this thing. I don't know. I feel like I've died and was born again. It's just been insane. It's been four months and a half, and of course, it hasn't been easy. I've cried a lot. It's been intense for everyone, my parents, especially. My parents are still out of it. I feel so incredibly fortunate and blessed. I never thought that I would live a life without this illness. Now that it's not here anymore, I mean, it's still part of me and my identity, of course. But I'm still trying to find out what to do next and what to expect because I'm so relieved. But obviously, it's so hard as well.

I've gone back to doing physical activity. I gone back to running, all sorts of things. It's just this massive weight has been lifted off my shoulders. I'm just trying to be happy. I'm not asking for the moon, obviously, but I just want to live a life where I feel healthy and content with what I am and with the gift that I've received, because this has been a miracle. I do remember that when I got diagnosed, not many doctors would've perhaps believed that I was going to survive long term. Now, it's been 17 years and I'm here and I'm doing great.

Of course, I do sometimes wish things had been different. I wish that life have been kinder, but overall, I'm just very happy with the person that I am despite everything that I've been through. I don't know what's coming next. I feel like after having a near death experience at 24, you're thinking about the future, but you're also not because obviously, you just start appreciating all the small things and everything. I'm just glad that I'm still here.

I had such a hard time accepting this disease, obviously. But now, having had the experience that I went through, I think helping others understand and get through these things would be the main goal. I'm a psychology student. I'm about to graduate as well and I would love to do my final thesis also on psychological consequences of this awful disease and so on. I may be cured again, but this is still part of me. I'm on the other side of it. I would just love to give a little bit of relief to other people that might be waiting for lungs or maybe struggling with the disease because I feel that's what I do. This is what's next, I think.

My name is Maria Simionato. I'm aware that I'm rare.

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