Rachel's Story
Expect the Unexpected
Expect the unexpected, my strength and determination with Marfan syndrome through emergency open-heart surgery.
I knew I had Marfan syndrome since I was about seven or eight years old. I always needed heart checks and needed other countless operations from a young age. My heart hadn't really changed much over the years, I just had a leaky but stable mitral valve.
My Dissection -
That all changed when in the March of 2022 I suddenly had a sharp shooting pain one morning whilst I was in the shower, going through my chest and radiating through to my back. With how severe and intense the pain felt, I knew something was severely wrong which sent sheer panic straight through me. It got so severe I couldn't move or speak and was struggling to catch my breath. I felt even more fearful because at the time, I was home alone.
I managed to ring my parents to tell them what was happening, they came home and I got rushed to hospital where I was told I was having a TYPE B dissection. Up until that point, I hadn't heard of a dissection. I had never been told I was at more of an increased risk of having one. I was in hospital for just over two weeks being monitored and now, it’s medically managed. But has left many lasting effects. I have experienced a lot of severe pain in my life, but that pain was off the scale. As I thought I was dying.
My Emergency Open-Heart Surgery -
I recovered as well as to be expected from the dissection and nine months later, I had returned to normal every day activities.
Then, in January of 2023 I was relaxing one day. When all of a sudden, I started with a dull ache in my chest, breathlessness and sharp pains. I wasn’t feeling myself and the pain wasn’t going away, so I went to A&E again fearing the worst case scenario but hoping for the best.
I got transferred from my local hospital again to Liverpool Heart and Chest Hospital. Straight away, they were talking about emergency heart surgery being undertaken within two days.
I went into shutdown mode, thinking:
"Why me? This can't be happening."
The doctor was talking to me but I just looked straight through him daydreaming. All I could hear was that I needed emergency heart surgery and that my surgeon would come to see me the day after.
When my surgeon came to see me the following day and asked me how my symptoms were, I said, "They have gotten worse since I was admitted."
She then told me that she sees no other option than to do valve-sparing aortic root replacement surgery with the frozen elephant trunk procedure. She explained I may need a new mitral valve fitted or that she may be able to repair my own. She also explained why I needed the frozen elephant trunk procedure and that even though it made it a more complex operation and a longer recovery, it would future proof me and make things much easier for them to operate for the future heart surgeries I’m likely to need. All the risks were explained, such as a stroke, a major bleed, cardiac arrest and death. I went into a blur at this point. My brain felt like it was shutting down.
The reality of having emergency open-heart surgery -
I couldn't believe my symptoms that didn’t seem nearly as bad as the dissection symptoms the previous year were now leading to this. I had always known that it was very likely that I would need open-heart surgery at some point in my life, but the enormity of how big this felt was on a different scale.
My mind went into survival mode but in reality, I was desperate to go home. My surgeon said if I went home and things got worse, I may not make it back into the hospital in time. So I felt like I had no other choice then to go through with it, but it felt like I was signing my life away. The sinking feeling deep in my stomach was awful.
I didn't really get much time to think that day, as I needed numerous other tests done before my surgery the following day. Such as bloods, a lung function test, echo, ECG, chest x-ray and an MRI. It goes without saying I didn't sleep that night, I had about an hours sleep. With every thought imaginable whizzing around my head.
Day of the surgery -
I was awake from 5.00 am. I was calm on the outside, but on the inside I was thinking of all possible scenarios. I didn't think I was going to wake up again when I said my goodbyes to my loved ones. It was an awful, awful feeling like no other. I was so nervous, I just wanted it over and done with.
Recovery on the ward -
Waking up on ICU I couldn't feel much, I just felt numb. I had about 20 wires hooked up to me for drains and medications.
Later on that day the pain kicked in, but I managed through the best I could due to the regular pain relief I was provided with.
The day after the surgery, I got up out of bed for the first time and into a chair which felt like I had climbed Mount Everest. I did it, which made me feel so proud. I got moved to a normal ward early the next day. The hospital staff couldn't believe my strength and determination and how fast I could be moved onto a regular ward. They didn't expect me to be moved that fast.
I was so proud of myself, I had been in hospital now for nearly two weeks altogether and was desperate to go home.
I was on a regular ward for a further six days and was discharged 8 days after major and complex surgery, which is remarkable considering the enormity of the surgery I had just endured and I was so proud of myself.
My recovery at home -
It took me a long time to feel even close to myself again after having 2 major heart traumas in the space of a year. I was battered and bruised like I could never have ever imagined. As you may expect, it completely took it out of me physically, emotionally and mentally. My nerves were all over the place and I didn't want to be on my own incase it happened again. It was and is still so scary to think about, but I am doing reasonably well at the moment and I am getting there slowly but surely. But my life will never be the same again after what I had to go through.
Evaluation -
I always knew I was strong and resilient, as I feel you have to be with this syndrome. But that 12-18 months showed me how much of a fighter and true warrior I am, I can get through anything that life and this syndrome throws at me.
I am proud to have always had such a strong, determined and resilient attitude to life that I can rely on to get me through anything that may come my way.
My life has thrown a lot at me. I have learnt to know that with this syndrome, you need to prepare yourself to expect the unexpected and to always be ready for the battles you might need to face.
Advice -
To know your own body and to be able to trust your own instincts. If I hadn't had done that on both of these occasions and had gone to the hospital knowing that something wasn’t quite right, I dread to think of the outcome. It is so important and absolutely vital to advocate for yourself and to know what you need.
I am proud of myself and everything I have faced and overcome in my life this far. As I believe, god gives his toughest battles to his strongest soldiers after all!
Photos:
Just for guidance the first two pictures are when I had my dissection in 2022 and of me spending my 31st birthday in hospital. The nurses went to get me a birthday cake and they all sang to me on the bay.
The picture of the fabric graft is of my surgeon showing me a replica of my frozen elephant trunk from my surgery in 2023.
The last picture is one of me in happier times last year, celebrating my 33rd birthday not in a hospital!
