Steph emailed me a few months ago after she stumbled upon my blog during her research while preparing for heart surgery. She was thankful to find my story which helped prepare her for her surgery. Before and after her surgery, we had a few phone conversations and lots of texts. Recently, I encouraged her to write a guest blog post if it would help her in any way. She is an excellent writer, and a super nice person who is thankful and happy with life, yet struggles with the difficulties of living with heart disease (and Marfan Syndrome). Please take a read. Thank you Steph!- Anthony
I feel my heart pounding through my chest and I wonder is this the last time? Will I experience this sensation forever? This feeling is not pleasant at all. As the beat gets stronger, my pulse begins to thump in my ears. My mind starts to wonder; will my heart ever have a steady beat?
These are the thoughts that raced through my mind since the age of 7. As I take you on this journey of living life with Marfan Syndrome; there will be some pain, anger, joy, happiness, and sorrow, yet most of all there will be the strength of a warrior.
Marfan Syndrome is a rare connective tissue disorder. An individual could be diagnosed at any age. Luckily for me, I was diagnosed from the moment I was born. The doctors looked at my long skinny fingers, listened to my pounding heart beat and knew that this life of mine would be an adventure of battles and successes. In synopsis, by the time I was diagnosed there was not a lot known about Marfan, but there was enough to keep me going at a healthy pace. You see, Marfan Syndrome does not just affect the heart, but it affects the whole skeletal structure, which can lead to scoliosis, it affects the eyes, leading to retinal detachments and lens dislocations, complications can arise with the lungs, it affects the rate of growth and the amount of weight that is possible to be gained. Marfan Syndrome does not have to be within the family heritage; it is a gene mutation within fibrillin1 gene with the mutation of chromosome 15 and I am one of the rare cases who was the spontaneous gene mutation within my family. Though I faced my struggles with accepting each aspect of having Marfan, the endless hospital visits, the countless surgeries one after the other, and the lifetime medication. I can not lie and say I never felt that it was unfair or that I never became angry, but I can say that I always held my ground and knew I was lucky. Lucky to have a family that supported me, doctors who knew what to do, and friends that believed me in. Although, life with Marfan does not only affect one physically, but it takes an emotional toll as well. Some people do not realize growing up with a chronic illness could be challenging because there are times one may feel misunderstood and alone. There are times where a person may wonder what he/she did and why this is happening. As I embraced these experiences throughout life, there was the ultimate concern and worry I always carried around with me. When will I have open heart surgery to prevent more damage to my heart; when will I feel a normal heart beat?
Diagnosis of my heart: Aortic Aneurysm, Mitral Regurgitation and Mitral Valve Prolapse. In simple terms, my aorta was enlarged (4.6 cm) and the chances of dissection and bleeding out is when the aorta reaches (5.0cm) is increased.
I spent 23 years visiting the cardiologist with the wonder, “would this be the year he says I need surgery?” Yet, little to my surprise every year was an answer of “your heart is stable, so we do not want to go in too soon.” By the time I was ready to graduate from college, I begged and pleaded to have the surgery before I moved from the East to the West Coast to attend graduate school. My cardiologist was adamant that I did not need surgery yet and there was a high chance of me actually never needing the heart surgery. So, there I was trying to understand how from the age of 7 years old I used my birthday wishes for my heart to be healed, by a teen I realized it was not my choice, and now here I am an adult hearing that I may not need the surgery-yet will be able to live with the complications? See, one may feel happy from that news; yet my complications also created rhythmic issues, such as Atrial Fibrillation and Ventricular Tachycardia. This caused my heart rate to increase drastically at any moment of the day and one of the main correlations to the problem was the mitral valve.
Yet, I continued on with my life and moved to start graduate school. Naturally I knew that I would need a new cardiologist and went to Stanford Hospital because they have an entire clinic dedicated to Marfan Syndrome, which I found to be pretty amazing. I will never forget my first visit; my nerves shaking, my mind rushing with thoughts, seeing a new cardiologist after all these years; terrified me. He was warm and confident in his words, I knew that I made the right choice, though the news I heard that day changed my life forever. After completing an echo, which measures my aorta and tracks the leakage in my mitral valve and said the words “You will need surgery within the next year or so, your aorta is 4.6cm not 4.2cm and the mitral valve leakage is severe.” I could feel my heart sink into the pit of my stomach, here I was just accepting the thought of never having the surgery and now I am being told that I have to decide when is the best time to have the surgery. Tears flowed from my eyes as I suddenly felt overwhelmed with hope and fear. All my emotions were stirring around my mind as I knew that this upcoming year would be the best time to have the surgery.
Fast forward; here I am, 24 years old and just finished my first year of graduate school in California. My family flew down from New Jersey and we were going into this battle one way or another. The surgery was scheduled for June 27th at 8am and the week before I could feel my life changing. I wondered if the surgery was going to work, I thought about death, I thought about life, and most of all I thought about my loved ones. Prior to scheduling the surgery, my surgeon reassured me that my leaflets (the part of my mitral valve) could be repaired and that I would not need a mechanical valve. Honestly though, I know my body and it never plays by the rules; so I asked what would be the backup plan if it was unrepairable using my own tissue. He told me that I had the choice of using a mechanical valve or a prosthetic valve, yet proceeded to tell me that it would not be needed because everything looked perfectly healthy and could be repaired.
So, there I was two days before the surgery with my family. We went sight seeing and attempted to push our fears deep below the surface until I received the phone call from my surgeon; he explained the co-surgeon on my case reviewed my echo and saw more damage to my mitral valve than expected. I held my breath as he spoke and the words that I was most terrified of hearing were finally said; “I need you to make a decision of having a mechanical or prosthetic valve because it does not appear that he could save your own tissue.” I could feel my whole body shaking and the anger boiling in my veins, I thought to myself can I not just just have a smooth surgery; but I calmly asked for some time to think about my decision and ended the phone call. During this time, I was in the car with my brother and sister in law, sitting there in pure silence and my brother tried to reassure me, yet again tears flowed down my eyes as I could feel my fear continue to rise. By the time I came home, I frantically researched pros and cons of each valve and based on the surgeon’s opinion a prosthetic valve would not last more than 3 years in my heart and I knew there was no chance I could face this surgery twice. After consulting and great advice from Anthony, I made up my mind on having the mechanical valve.
The day finally arrived as my family and I walked into Stanford Hospital, silence crept around us as the fear vibrated off of our skin. We all had teary eyes, yet did the best to smile and believe everything was going to work out for the best. The time seemed to be going by quickly as I registered and the next minute I was taken back by a nurse to get prepared for surgery. There were only two people allowed to see me at a time, so my parents took turns with my brother and sister in law. As I waited I saw the co-surgeon for the first time to confirm my decision and I told him to go mechanical and that meant for both valves. If one could not be saved I wanted to have them both be mechanical to be done with this journey once and for all. As my parents looked at me, I suddenly felt all of my emotions freeze over. I could not cry anymore, I did not feel the fear, nor did I panic. Possibly because I asked for medication to help me relax before I went into the operating room. As we said I loved you and they rolled me off to the operating room, I did my best to fight against the medicine due to my panic of falling asleep. I told the anesthesiologist that I have been waiting for this surgery since the age of 7 and asked her to make sure they fix my heart. Proceeding this request, I started to ask about how she got into the field and what she liked about the job, I then looked to my left and saw the other anesthesiologist working on an IV line in my left wrist and asked if I should stop talking. This whole process was terrifying to me and the only way I was coping was by trying to distract myself from my own thoughts, I almost wished that time would just stop for me. Soon enough I had the oxygen mask put on me and I took three deep breaths and fell asleep.
For 11 hours the surgeons operated and poured their energy into healing my heart. Two hours after being transferred to the Intensive Care Unit, I slowly began to wake up from the anesthesia and could feel the breathing tube in my throat. My thoughts moving at a sluggish pace, knowing I could not speak; I steadily raised my arm and struggled to wave my hand. I heard the nurses saying my name, telling me to keep my eyes open and stay awake or they can not take out the tube. Probably a few seconds passed by as I closed my eyes again and slowly reopened them. Every fiber within my body was fighting to stay awake and keep my mind calm. Every thought that I had before surgery seemed to slip my mind as I laid there with the nurses reassuring me that I am fine and my heart is healed. The night seemed to drag on endlessly as I woke up in pain every half hour, but tears did not fall from my eyes this time. Instead I asked consistently if my heart was fixed and with the great patience the nurses had they reassured me that my aorta was replaced with a graft material, which is called the Tirone David-V Stanford modification procedure and that my mitral valve does not have any more leaks due to a repair with a 30 mm CarboMedic ring and needle cords. In other terms, my heart beat is finally “normal” as I heard these words, I could not help but repeat myself and ask are you sure? At that moment I did not know if I was terrified of the chest tubes and central lines or if my heart would not react well to the surgery and the corrections would slip away from me. After 5 days of being in the ICU, I was moved to a step down unit to start my physical therapy.
My hospital stay consisted of eight days; it was the most traumatizing and insightful eight days that I have ever experienced in my life. Everyday my parents were by my side and I could feel the love radiate from them and at that time, it is exactly what kept me going. There were moments where the pain would take over and I thought to myself “how am I ever going to survive this?” Yet, I kept moving forward, every physical therapy that was given to me; I did not deny it; I did not fight it; I pushed myself and participated because I knew that was the only way I was going to get out of the hospital. On the day of being discharged, I remember feeling this deep gut wrenching fear of being so far away from the medical staff, yet at the same time I wanted to run out of the hospital and feel the sun on my skin. I was home for three weeks when all seemed to be going well. Of course there were the days where I felt completely defeated as my energy was low, but the one thought that kept me motivated was that “after all these years, here I am with my heart beating a steady beat.” Then the unexpected happened and I broke out in a fever of 101.5 with sharp shooting pain on the left side of my chest. I could not lay down, I could not lay on my side, but most of all I could not breathe.
So, here I was three weeks out of surgery and I felt as if my heart was going to explode and the panic took over my mind. My mother calmly called the on-call surgeon and he said to come to Stanford right away. As we drove to the hospital all I could think was “why is this happening to me, why can I not get a break, why for once can I just not have a smooth recovery?” These thoughts circled my mind as we arrived and waited in the emergency room. I could not help myself, but cry. There was no holding back my tears; every emotion I felt was displayed to the waiting room and quite frankly I did not care. I was going to cope with the way I knew best and that was to experience every emotion regardless how terrified I was; I was letting myself feel and that was all I could ask of myself. As the nurses prepped me for blood work, IVs, EKGs, echocardiograms, all I could do was cry and there was no calming me down because I wanted my surgeon. I wanted security in someone who knew my case, I wanted to feel safe. Unfortunately, he was away for the weekend, but his team was there and I knew they were doing their best. The chest X-ray showed that my left lung was filled with fluid and that I had inflammation around my heart. I was kept overnight for observation of an “infection” and regardless of what painkillers they gave me, the pain stayed and was throbbing to the point where I tossed and turned all night.
In time the morning came and I saw the team, they gave two hypotheses for what could be happening; the first was a “type B dissection that could happen in Marfan patients and the second was pneumonia” I could feel my heart sink into my stomach and asked what he meant by a dissection. The whole purpose of this surgery was to prevent a dissection and I was told repeatedly that it was fixed. Yet, the tricky part is with Marfan no one truly knows what the body will do. There are complications from connective tissue disorders that could arise at any moment and there I lay hoping it was not the case. The tests were endless to rule out what was happening to me, CT scans, X-rays, blood work, but the most painful part of everything was knowing that the doctors did not completely understand what was happening with my heart.
There I was in the hospital again fighting for my life to just feel “settled”, I did not need everything to be perfect; I just wanted my heart to be settled and calm. The next 72 hours were a waiting game. Numerous doctors came in and gave their explanation for what may be happening. Some thought that I would need to go back in for surgery and have fluid drained. Imagine hearing that? Obviously it did not go over well with me. These were assumptions, guesses, nothing was certain and it was breaking me down in fear. In time my mom finally called my cardiologist and told him I was back in the hospital. Minutes later I saw him walking in the room and I had tears of joy spill from my eyes. I knew I would get answers at last. The timing was perfect, as he walked in on a tech doing an echo and right away my cardiologist saw the problem. I had pericarditis, an inflammation around the heart and slight fluid as well. I looked at him not understanding a word of what that meant for me. All I could say was, “does this mean I have to go back under the knife?” I never even expected myself to say a phrase like that, but the words seem to fit at the time. He smiled at me and to the grace of God, he told me “no it does not mean you need surgery, but you do have an infection around your heart; which explains the pain, so you will need antibiotics and motrin.” Part of me was so relieved that I did not know how to respond besides repeatedly saying thank you and then I thought about how life is going to remain unpredictable whether my heart is healed or not. This was a fact that I was going to have to accept and learn to live with. Of course, I was not looking for my life to be planned out perfectly after my surgery, but I hoped for some peace of mind.
After three days of waiting and wondering what was happening, the answers finally led to controlling my pain and I was allowed to go home. Leaving the hospital this time brought more fear than the first time because I wondered what would happen next. Though I knew I could not live my life in fear, I was not going to try and hide every emotion I was feeling. In all honesty this experience has been one of the most blessing times I could have ever asked for. I finally received my desire of having my heart fixed and being able to live my life. Yet I know it does not stop there, the road to recovery is a long process and there may be bumps in the road that I do not expect, but my journey has just begun. For all of those who have experienced having open heart surgery or any other surgery, you know how challenging the process could be. Although, if it is one lesson I will carry with me forever since this time, is to love a lot, forgive those who hurt you, take the paths in life you want, and most of all go through every emotion you feel because this world was not meant to be easy, but it was meant to build warriors.
Please feel free to learn more about Marfan Syndrome via Marfan.org and e-mail me with any questions you may have at Steph.Randazzo@yahoo.com
Spreading awareness to Marfan Syndrome may save a life and help inform those who are unaware of this rare connective tissue disorder.