Feb 9
kellyHLHS , Posted on SUPPORT NETWORK Blog

Living Life Fully With Half of a Heart (HLHS)

On November 23rd 1988, the day before Thanksgiving, my parents welcomed me into the world. Unlike most newly diagnosed Hypoplastic Left Heart Syndrome (HLHS) babies, my parents had no idea that they were welcoming a little girl with a complex heart condition. A moment that should have been filled with joy and excitement quickly turned hectic and extremely alarming when the doctors noticed I was extremely cyanotic. I was rushed immediately to the NICU for an array of tests, eventually yielding a scary diagnosis. I have a complex single ventricle physiology consisting of mitral atresia, d-TGA with pulmonary stenosis and a hypoplastic left ventricle. But despite all that, I was stable enough for the time being. I was discharged that Monday, and sent home with my overwhelmed and petrified parents.
As my Mom and Dad navigated the waters of being first time parents, to a sick child none-the-less, it was a daily struggle to get me to eat, gain weight, and remain energized. At three months old I suffered a seizure and my oxygen saturation levels plummeted. I was rushed to the Children’s Hospital of Milwaukee for my first heart surgery, the bidirectional Glenn Shunt. This procedure increased an opening between the aorta and pulmonary artery, causing my health to improve. At the age of 2 I underwent another heart surgery, the first stage of the Glen Procedure. At four years old I underwent yet another surgery, The Fontan Procedure.  My dad always reflects back on this, saying “that’s when we almost lost her. When they took her off of the heart-lung machine and her heart didn’t start back up; they came out and said ‘Houston, we have a problem here’”. Miraculously, after tearful praying in the waiting room and the determination of my incredible surgeons and medical team in the OR, a shot of epinephrine got my heart started again.
Recovery from the Fontan wasn’t pleasant, although luckily I was too young to remember much. I spent 3 weeks in the hospital, while my family alternated between the hospital and the Ronald McDonald house. I was suffering complications from pleural effusions, had 7 chest tubes in, and was either NPO or on an extremely low fat diet. Needless to say, I was NOT a happy little girl. There are numerous pictures of me looking exceptionally grumpy – particularly on Halloween. Though I must admit, I think the favorite picture is me scowling next to Ronald McDonald, who had come in for a surprise visit. But hey – could you blame me?
In addition to my parents, my Grandmother was with me every step of the way. In fact, she held me even before my mother did, and as a result, we have always had the most special relationship. I would whimper to her “no more owies grandmommy, no more owies” when my veins collapsed and my IV’s blew, yet I didn’t hesitate to loudly yell when she would accidentally step on my chestubes. I also didn’t hesitate to express my displeasure when they did not give me enough local anesthetic for the removal of my chest tubes and I sassed the doctor, yelling “it’s my tummy and it hurts!” Even at the age of 4, I was learning the importance of being assertive about my body. Having gone through three open-heart surgeries by the age of 4 is no joke. I was a tough little girl, and have turned into an extremely strong woman. And honestly, I really wouldn’t change it. But words of wisdom: Listen to your body. You know best. Listen to your child when they tell you how they are feeling. And always, always advocate.
In the words of my mom, “from age six we just sailed, and everything was great. We never treated her as a cardiac cripple. She was always just a little girl that happened to have a heart condition but no special favors; she didn’t require any special assistance. She was just a normal little girl” I cannot thank my parents enough for raising me that way – it’s truly made all the difference in my overall outlook and the way in which I live my life.
Fast-forward 17 years to my junior year in college. Where yes, I went away to school. I lived in the dorms. I battled the daily germs, the late nights, and the stress of studying. I had to pace myself, but really, I was just like any other college student. I even spent three months abroad on the island of South Caicos, doing marine biology research and conservation. And then, my senior year, I just started feeling cruddy. I couldn’t pinpoint anything specific, but I always felt so bad, so tired, and to the point where it seemed I was constantly short of breath. My saturations seemed to be dropping into the 70’s, but we couldn’t figure out why. Per usual, I kept plugging along.
In the fall of 2011 I was working as a tech at the hospital and also part time waiting tables at Ledo Pizza. Thankfully, my (now) husband was working that evening too. I wasn’t feeling well, and ended up passing out. Poor Mike was terrified. “She collapsed right in my arms, literally. I was holding her elbows and we got through the door she collapsed. It was so lucky I was there, should could have hit her head”. Needless to say I went to the hospital by ambulance, and Ledo was down two servers for the night. At the hospital the doctors ruled out arrhythmia, lung problems, heart failure and an array of other issues, but still didn’t know what was wrong. It’s hard being in the pioneer generation – prior to the late 80’s a lot of HLHS patients weren’t living into adulthood. As a result, there are still so many unanswered questions about my health and prognosis long term. I ended up scheduling a cardiac catherization three months out and endured the day to day with low saturation levels. My catherization ended up being nine hours (longer than any of my prior open heart surgeries!) where my doctor discovered that my body had been growing collateral vessels (probably for decades) in an attempt to compensate for my lower oxygen levels. I was only in the hospital for a night, but the recovery was tough. It was at this point that I started experiencing complications for the first time in 18 or so years.
As time went on, I switched careers and began working for a financial services company. No more 12 hour shifts on my feet all day, wearing me down. But one afternoon, about a year later, I experienced a horrific pain in the back of my right leg. I got out of bed and could barely seem to walk, but I ended up going to work for the day (much to the later outrage of my co-workers, friends, and family) I finally deemed on my way home that a trip to the ER was warranted, and a few hours later it was confirmed: I had 3 blood clots in my right leg. It was then that I was introduced to the wonderful world of heparin, coumadin, and routinely scheduled INR checks. To add insult to injury, I ended up either catching a bad virus or having adverse reactions to all the different medications and was sick as a dog, extending my hospital stay by 5 or 6 days. Knock on wood, aside from a quick bout with low magnesium that resulted in terrible chest pain and the flu and pneumonia in winter of 2014-2015, things have been good. In December of 2016 I had my first experience with Atrial Fibrillation and started a new medicine to keep it in control.
What I find the most interesting in my day-to-day life with CHD is the profound impact it has had on shaping my identity, cliché as that may seem. At 28, I feel that I am finally just beginning to "put the pieces" of my life together—particularly in regards to my identity as an adult CHD, as well as an adult with a chronic illness. I am a patient, a graduate, a financial professional, a biology student, a daughter, a wife, a sister, a friend, a granddaughter, an aunt, a survivor…the list goes on and on. What I want more than anything is to put all this together, to further piece together my identity and to be an active advocate.
And I have been. October 12th 2013 was far the best day of my life, the day I said “I do” to my best friend, my husband, Michael. Our vows definitely resonated more with us than the average younger couple – we had “through sickness and in health” down pat already. Mike is my biggest advocate, my strongest supporter, and takes the absolute best care of me. I cannot even fathom navigating adulthood with CHD without him. There will never be adequate words to describe how he cares for me, and for how much I love him. Similarly, thank you will never be enough. But he just knows.
The most important takeaway from all of this is that I am blessed in the here and now, and I am happy. I have traveled. I graduated from college and found a wonderful career path. I married the love of my life and together we bought our first home. I have made incredible friends, have a wonderful family, and have made fantastic memories. We all only get one life, lets live it to the fullest.
  • renato
    Simply wonderful, God bless you Renato
  • dadwithcad
    Kelly, Amazing and inspiring story!! As my favorite saying goes, "Dream as if you'll live forever, live as if you only have today!" I think that's a wuote that any of us with heart disease understand. Best Wishes! Mark ​www.dadwithcad.com
  • AHA/ASA Sarah Ismail
    AHA/ASA Sarah Ismail,
    Kelly, thank you for sharing such an inspiring and heartfelt story with us all. I know your story will give many hope and joy! Wishing you all the best. Best, Sarah
  • Laura196550
    Dear Kelly, I sure needed to hear your story!!!! First of all I am dying yes literally dying and it is hard I have surpassed my original time of death a year ago to be exact. I am 51 and I am young trust me my body is old but I am young, I have had full custody of my grand daughter since the second she was born. You made a very good statement and people really need to listen to your advice "LISTEN TO YOUR BODY" I knew and I know now when things are getting rough on me, and so far my body has been right. I am going through my own issues I can not literally stand my Heart Doctor I realize many of the Cardiologist have crappy bed side manners. But I quit seeing a heart doctor I decided to let this play out its own way. I am going through the I am scared to know where i am at and the I will live till God says otherwise. I just do not know what I should do my case worker is begging me to go see a new doctor I have come to the point of taking my beta blockers my pain patch my oxys my valiumn and let life do what it is it needs to do. You have exactly what I have what do you think am I wrong should I continue with a Heart Doctor or let this show play out on its own. Thanks for sharing your wonderful story I read it from start to finish
  • cdameron
    Dear Laura196550, I too loved Kelly's story... it was inspiring! Her life seems to be going well. I couldn't help but notice the desperation in your comment. No one can know what you are going through but you. I would be able to comment on one thing though, your frustration with your doctor. Being doctor phobic myself I can identify however, this I know... There are many doctors in the field of cardiology, you have choices. Not seeing one at all might not be the best choice. I also have a doctor I dislike but I work around it by utilizing his PA who has more sensitivity to my questions. I am going to say that when I change doctors which I will do down the road I will seek one who specializes in women's issues in cardiac care. It is imperative that we all find someone we are comfortable with. You don't want to get in to a emergency situation with a doctor you dislike. Do it because you have a choice. There is always time to give up at the end of our lives! Persist...
  • kellyHLHS
    Thank you, everyone, for your incredibly kind words! Laura196550-where do you live? I certainly echo cdameron's words above. This may be a silly question, but are you seeing a doctor who specializes in ADULTS with Congenital Defects? I've seen just "regular" cardiologists before and had issues... but there is now the growing specialty of board certified ACHD doctors. You can find some more information here: https://www.achaheart.org/your-heart/clinic-directory/clinic-listings/ Fight on! Sending heart hugs!
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