I’ve known I have Marfan since I was a baby because of some physical characteristics. It wasn’t a big deal – me and my mom did our annual check-ups together. She was the first person who got the diagnosis of Marfan Syndrome, after many doctor’s appointments to find out why some of our relatives died suddenly.
My aortic root increased from 3.7cm to 4.3cm in 2016, when I started to take betablockers daily. I’m not a sport fan but try to keep active. I don’t run any more but practice gym and Pilates.
The first big Marfan challenge in my life was a scoliosis operation in 2018. I didn’t have pain, but my spine was compressing one of my lungs and because of the syndrome the degrees would get worse, so this was the best preventive option. I had a pretty good recovery including months of physiotherapy.
In 2021, my mom had an aortic dissection and unfortunately did not return from the 12-hour surgery. Even reading a lot about how complex it is, it was really hard for my family to get through. 3 months later I had my cardiologist appointment and to my surprise my aneurism had increased to 5 cm in 6 months – in Brazil this is the minimum size for a Marfan person to get the operation. Now I was really scared, it was hard to hear that at 30 years old and I did not want to accept the common surgery. Then I remembered the posts about PEARS that I’d read and got in touch with ExoVasc.
Tal replied to my email some days later and since no countries in America use PEARS he forwarded me to a hospital in London. I got the quote but it would be hard to afford it without health insurance. I was thinking with my family how to make it. I also talked about PEARS with many doctors in Sao Paolo – unfortunately they don’t know the procedure and promptly said it didn’t work. Some months later Tal sent me an email. There was an ongoing negotiation for a PEARS centre in Brazil, including surgeries, but it was on hold because of the COVID 19 pandemic – they set a possible date and he would like to know if I was interested. I remember I shouted “claro!” (“of course”, in Portuguese ????) in front of my computer.
I had a problem regarding the CT scan for prothesis manufacture. My arrythmia had also increased in 2021 and it didn’t allow good images. My cardiologist, the only doctor that supported my decision about PEARS, suggested replacing the betablocker with anti-arrhythmic medication. After 15 days I repeated the CT scan and it followed the PEARS protocol.
The PEARS clinical trial was rescheduled a couple of times but I tried to keep positive – PEARS was the only option in my mind. Since the surgery would be in the south of Brazil, I started to plan the trip for me and my family from Sao Paolo to Porto Alegre city and it helped to keep me busy beyond the work hours.
We took a flight on 6 March 2022 and had the opportunity to have dinner with Tal, Dr. Austin from Guy’s and St Thomas’ Hospital who came to teach the PEARS technique, Dr. Renato Kalil from Porto Alegre who is managing the research as well as one other patient and his wife. It was an amazing evening with such great people, still hard to believe I was with all of them in front of me and the surgery was scheduled for 3 days later. We talked a lot, and they supported all our questions.
The next day I had the COVID 19 test, took a walk in the city and had dinner with my family. On Tuesday I was hospitalized at Hospital Divina Providência to undergo some medical tests. I was confident in the process and my husband spent the night with me waiting for the next day.
9 March 2022 – Finally, the surgery day, they came to take me to the surgery room at 1pm. I remember seeing Dr. Austin and Dr. Kalil in the surgery room and many nurses and doctors, time to relax and trust. ????
I know I left the surgery room around 4 hours later, saying few words to my dad, without the tube but still sleepy. I remember officially waking up around 10pm in intensive care, thirst, no pain in chest. The nurse brought me some water and I slept again – the medication is powerful. Some hours later I woke up with back pain, I could not stay on the bed and asked to be transferred to an armchair.
Post-op day 1
I had an echocardiogram and x-ray in the morning before breakfast and everything looked well! I was feeling tired and also short of breath, still no pain and was really well assisted by nutritionists and physiotherapists from hospital. I could have one 30 minute visit per day, then my sister came bringing my mobile phone and my washbag. I was still sleepy, my back was killing me so nurses managed it with medication and transfer to an armchair.
During this night I had nausea because of one of the pain medications and suspended part of them.
Post-op day 2
This was a special day, I could take a shower by myself even with the shortness of breath. I had visits from cardiologists and Dr. Kalil – the drain was good, I should be transferred to a private room the next day. Some hours later Dr. Austin and Tal visited me. It was a relief to hear from Dr. Austin the surgery had been straightforward. They would take the flight back to Europe the next day, this was the last time I saw them both.
Post-op days 3 and 4
I felt my recovery was much better in the private room, I could have someone from my family with me until my last day in hospital, without all the machines. I also could walk through the hospital, eat better food and take less medication. Dr. Kalil told me I should leave the hospital next day.
Last hospital day
Early in the morning I had an x-ray, blood tests and some hours later the echocardiogram – everything looked well and I left the hospital around 3pm. The recommendations were not to carry heavy things, be careful when getting up from bed, take some medication and have a CT scan in 1 month. I and my family had dinner that day, we were so happy to be together again!
Then I spent one more week in the hotel, taking short walks and reading and then took the flight back to Sao Paolo.
The first month was challenging, my family supported me in daily tasks, it was hard to sleep well because of the position, and I still felt a bit tired. The result from the first CT scan after surgery was the prothesis was well but there was a pleural effusion. This was the only intercurrence, and I took diuretics to get rid of it.
2 months after the surgery I returned to Pilates classes and I can take longer walks.
PEARS changed my life, now I can have other plans and dreams for my life – one of them is regulate PEARS in Brazil. We were the first 3 patients to have the PEARS surgery in the Americas and I’m patient number 593.
I would like to thank all the staff at Divina Providência Hospital, as well as Dr. Austin, Tal Golesworthy and Dr. Kalil. You’re the best!
