My Story

I was born in Rexburg, Idaho and cared for by an angel. There was a nurse there after I was born that noticed I was turning blue. She had Sarah001worked at Primary Children’s before, and knew that blue babies that don’t respond with oxygen have something wrong with their hearts. So this angel saved me when she called in the doctor. I was given some steroid medicine to keep a duct open and flew on the life flight jet to Primary Children’s. Once I got there and had some tests done, the doctors found out that I had several heart defects. (You can see some of these defects on the Mayo Clinic website.)

  • Pulmonary Atresia – the pulmonary artery didn’t finish growing and attach to my heart
  • Transposition – the aorta and pulmonary artery didn’t cross over and the aorta grew into the wrong side of my heart
  • ASD/VSD Atrial & Ventricular Septal Defect – there was a hole in my heart between the atria and another one between the ventricles
  • Hypoplastic Left Ventricle – my left ventricle didn’t ever develop, so I basically had a three-chamber heart

So, when I was 5 days old, I had my first surgery. They put in a shunt to connect my pulmonary artery to the aorta. (The duct that they kept open is the patent ductus which connects these while the baby is developing and not breathing yet. It’s supposed to close off after birth, but that’s the only way my blood was getting any oxygen, so when it started closing off, I turned blue.) A week after surgery, I got to go home.

The doctors were hoping that my ventricle would grow, but it never did. When I was 13 months, I was really purple and had a hard time Purple_Sarah016whenever I tried to crawl. I had to fly back to Primary Children’s and had an emergency surgery to put in another shunt and saved my life again. The first shunt was closing off with scar and ductal tissue, and I wasn’t getting any oxygen. That’s when they decided to go ahead with plans to do the Glen and Fontan surgeries, but I had to get better first.

I had my Glen 5 months later. They opened up the septal tissued between the atrium and ventricle and hooked the main arteries from my neck directly into to my pulmonary artery. I had lots of problems after this surgery, and about 5 months and 2 more surgeries later, I was finally better and got to stay at home.

Just before my 5th birthday, I had my Fontan surgery. They hooked the main arteries from the bottom half of my body directly into my pulmonary artery so it now looked like an “H”. With a Fontan, the blood flows from the body and then through the lungs (just because of gravity and pressure), into the single atrium, and then through the ventricle and pumped by the ventricle back out to the body. It’s really weird, but it’s worked pretty well for me!

When I was 10 my protein levels in my blood started going down. This is one of the symptoms of PLE (Protein Losing Enteropathy), where the proteins are not absorbed in the intestines and it makes more fluid to flush it out which causes swelling, diarrhea and vomiting. I didn’t have any of the other symptoms yet, but the doctor told us they would probably come. A lot of Fontan kids get PLE because the blood flow and pressure is all wrong for the other organs in the body.

I had a pretty good, healthy childhood considering all that was going on inside of me. When I got into my teenage years and I’d get a cold or something, it would kick my PLE into gear and I’d have a few weeks with the PLE symptoms. When I was a sophomore in high school, though, the PLE hit really hard. I got so sick I had to stay home from school more than I was there. After months of this and trying some other medicines, I went to the hospital for albumen infusions (IV protein treatments) to raise my levels and see if it would help the symptoms go away. It didn’t seem to be helping, but the doctor kept me there a few days and then started me on a new medicine (sildenafil). Eventually, it helped me get over the flare-up and kept the PLE under control for a while. My feet are often swollen and my tummy hurts a lot.

The October after I graduated from high school, I got a stomach virus that kicked the PLE into high gear again. After a week or so and no SarahGradimprovement, we were sent back to Primary Children’s where my heart doctors could work with me better. We couldn’t tell if the fever, diarrhea and vomiting were from the illness or the PLE. After a lot of tests, questions and labs, they sent me back home to get better. When the fever still didn’t go away, I was life-flighted again to Primary Children’s (this time on the helicopter) for more testing. My fever was higher and I had a lot of fluid in my belly. They put me on IV antibiotics and we did lots of tests (scans, ultrasounds, tapped the fluid, scopes, etc.) and most were dead ends. We were happy that things looked so good, but couldn’t explain why I was so sick. They finally decided I must have had bacterial peritonitis (infection in the fluid in my belly). They pumped me full of antibiotics and diuretics to lose the infection and fluid.

As awful as that month was, a lot of miracles happened that we hadn’t expected. That was when I got my new cardiologist (Dr. Yetman), who is a heart doctor that works with adults that had heart defects as babies. She’s amazing, and as she helped the teams figure out what to do, she tried some things that adults need that kids don’t. It seemed to work. That trip is also when we started talking about a heart transplant. Fontan kids with PLE just get sicker and sicker as organs get damaged and shut down. A heart transplant is really the only way around that. Another miracle is that because of the scans, we discovered that I have cirrhosis (scarring) in my liver. We never would have known that without that infection and all the ultrasounds and scans to figure it all out. I’m lucky that they found it before I started having symptoms of liver failure, too. There’s lots of other good things that happened then, but the biggest is that is started the wheels turning for my transplants.

So, here we are now. I’m usually swollen and go to the hospital sometimes for a “tune-up”, but like my mom says, I’m pretty healthy for such a sick kid. I’m waiting for the doctors to decide how to help me now that the Fontan has caused so many problems. But, I wouldn’t be here if I hadn’t had one, so the angels must be still at work all around me.

10 thoughts on “My Story

  1. Sarah, you are truly a miracle. I am blessed to know you and be your uncle. Our prayers are with you and your family!
    Uncle Eric

  2. Oh wow. I saw your comment on my blog today, and obviously just spent like the last hour reading about you. You go girl!!! You are an inspiration, and I wish you the best of luck with everything!! Feel free to add my blog to your blog list – I am honored that you even asked. If you ever just need to chat, feel free to shoot me an email. I love talking with people who have similar heart issues and being able to connect with that. Again, good luck with everything!! My prayers are with you. After all of this, I am confident you are going to pull through 🙂

  3. I loved reading about you. You have alot in common with my daughter Alexis who is now 11 years old! Alexis was born with Tricuspid Atresia, ASD, VSD, Transposition of the Great Arteries, Pulmonary Atresia, Hypoplastic Left Ventricle, she’s dextrocardiac (heart in there backwards) and also siteous inversus totalis (all of her organs are reversed like a mirror image). She was also diagnosed with PLE about 3 years ago. It was so good reading about your life so far, and seeing the road we will be heading down. I would love to subscribe or whatever to your blog to follow your journey but do not know how, please let me know if this is ok with you!
    Thank you!

    1. Jeanie, Thank you for commenting! I sure wish Alexis, you, and your family luck. I know how hard the road to the unknown is. Just keep walking forward!! 🙂 I don’t mind at all! When you first get on my blog, on the top right (under the Facebook, pinterest, and email icons) there is a “subscribe to posts” window. All you have to do is type in your e-mail address and click the button underneath. Once you are subscribed, every time Mom, Dad or I post something you will get an email letting you know that my blog has been updated! 🙂

  4. It honestly brings me to tears to read this. I stumbled across your site looking for information on PLE, me son(3 years old) was born with almost all of the same defects you have and has been diagnosed with PLE following his Fontan last Sept. I am so glad to hear stories of people living full and happy lives with PLE and heart defects. You are an amazing person and I am glad to have been able to see your story. Thank you.

  5. Sarah,

    You have given me hope! My son is six months old with HLHS and had his Glenn two months ago. I hope his heart can hang on as long as yours has! I’m so grateful for strong souls like you that have gone down this road before us. There is so much unknown. I admire your courage to keep walking forward!!! Thank you for sharing your journey!!!

    Bethany

  6. Sarah…I have decided after all of these years that your name is too short. From here on out, to ME you shall be known as “SWEET Sarah” I can’t seem to think about you without putting the “sweet” right in front of it. We love you are so blessed to just be around you. Both David and I get a
    “spiritual boost” when we see you and feel of YOUR SWEET spirit. So, SWEET Sarah…have a good night! Loves to you!!

    Cindy

  7. Hey hey! I know that we have never met, but your grandpa Ed is my great uncle… so basically we are cousins. :)) I love your strength and hearing how well you are doing!! Uncle Ed will email about how you are doing every once in a while and it makes me so happy to hear how well you are doing!! Keep it up!! If you have a facebook, You can add me and we’ll start getting to know each other better!!!

    Kirsi Griner

  8. Sarah,
    I just read your story and was amazed at the similarities of my heart defect and yours. I had the transposition, the (2) holes in the chest wall and my heart worked only on 3 chambers for 18 years. I do know partly what you went through, but mine started at birth also, but in 1955.
    Even though we are far apart in age, I am confident that everything will go well with you because you a strong woman. You can e-mail me at anytime if you would like. I enjoy finding people like me somewhere in this world.
    God Bless You,
    Donna (Ditter) Krueger

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