Katy's Story

Katy, my daughter, is now a beautiful young woman of seventeen.  I am proud of her for her intelligence, curiosity, humor, spunk, determination and sheer will to live and carry on, despite constant medical challenges.  I don't think it is untruthful to say, that when a person meets Katy, she takes hold of their hearts.  Part of it is her story, but mostly, it is Katy's inquisitive and sharp mind, her zest for life despite the hardships, and her tenacity to keep going.  I'd like to share some background information about Katy, in case you do not know her story.

Katy was born with a cleft palate.  This led us to the discovery that she had a genetic disorder called Stickler's Syndrome.  Her first surgery was at 8 months old to repair the palate.  She crawled and walked late, but we thought it was the Stickler's syndrome and had no worries about overcoming these small obstacles.  Her determination was evident even at such a young age as 18 months.  In her first 4 years, she was a delightful baby and toddler who always sat still and behaved wonderfully.  We moved to Utah from Wyoming, she started preschool, danced, made friends and got a new sister, Emily.

After Emily was born, when Katy was 4, she started fainting.  After, several tests, her pediatrician thought it could be Katy's heart.  So, Dr. O'Mara sent Katy for an evaluation in cardiology.  That day, July 1, 2003, we walked away with the diagnosis of Pulmonary Hypertension (PH), which is high blood pressure in the right side of your heart and lungs.  It is progressive, and eventually terminal.  There is no cure.  Only medicines to potentially slow down the progression.

About a year later, after trying oral medications to manage the PH, Katy (age 5) started a continuous IV medication called Flolan.  She had a central line placed in her chest, Jim and I learned how to prepare the medication every day (called mixing), learned all emergency protocols and hoped for the best.  Katy suddenly became a different kid.  She had energy and was able to do things that the other kids could do.  She wasn't normal, but she was able to keep up better than she ever was before.

Now we fast forward.  In the 9 years she was on the continuous infusion medications of Flolan, and later Remodulin, Katy continued to have many problems and procedures.  Her body did not like central lines, and in 9 years, she had 10 central lines.  One line stayed in for 4 years.  She had heart catheterizations, multiple line infections, or just respiratory infections, with hospitalizations.  I've lost count of how many PICC lines Katy has had (central lines that go in through your arm instead of chest).  She had a retinal detachment.  She also had multiple palate expanders and teeth pulled.  She was poked, prodded, and invaded on a regular basis.  Over time, Katy battled more and more for physical energy, to the point that going to school was difficult.

And then, on July 3, 2012, Katy went in for a routine heart catheterization.  Her doctor at Stanford came to talk with me, and we learned that Katy was in right heart failure.  It was time to list her for a double lung transplant. By September 2012, Katy was on the transplant list.  We waited 14 months for her new set of lungs.

On November 20, 2013, we received the transplant call from Dr. Conrad.  We were lucky enough to be in California already, due to checkups.  On November 21, 2013, Katy received her double lung transplant at Lucile Packard Children's Hospital at Stanford.  In hindsight, she recovered from the transplant well.  We lived in California for 4 months post transplant.  Before we left, Katy was diagnosed with medication induced diabetes.  On March 8, 2014, Katy and I arrived home in Utah.

We continued to see her doctors at Stanford every month.  By June, Katy's health was in decline.  Ultimately, she was diagnosed with a form of Lymphoma called Post Transplant Lymphoproliferative Disease.  She was in stage 4 and was very sick by the time she started chemotherapy.  Her intestines were riddled with tumors and the first treatment of chemo almost killed her.  The therapy worked so well, it left 10 holes in her intestines and she became septic.  She spent a month in ICU and three months total in the hospital after the cancer diagnosis.  Upon discharge, Katy and I lived in a studio apartment in Mountain View, CA while she finished her cancer treatments.  Soon before coming home, Katy had more abdominal surgery to take down her ostemy and remove her gall bladder.  The surgery went well, but several days later Katy was given too many fluids and ended up back in the ICU fighting for her life.  Her lungs were filled with fluid and she had to be intubated.  Yet another nightmare.

Amazingly, Katy rapidly recovered from this incident.  She and I finally returned home on Jim's birthday, February 9, 2015.  Katy was extremely debilitated.  She spent the rest of the school year attending physical therapy, doctor's appointments and building her strength.  By the following September, Katy finally returned to school and a pretty regular life.  Overall, she has been healthy and happy, getting good grades, making friends, and learning to drive.  Medical stuff is never out of our lives, but we were all looking toward a happy and healthy future, at least for a few more years.

Recently, the only thing plaguing Katy were dropping platelets and continued low white blood cell counts.  Since her blood counts are checked on a regular basis, we were seeing a concerning trend.  On September 20, 2016, Katy had an appointment with her oncologist/hematologist here at Primary Children's Hospital.  Dr. Afify thought perhaps Katy was making antibodies that were killing her platelets and possibly her white blood cells.  We started with some blood tests, but decided upon a bone marrow biopsy on Friday, September 23.  By Monday, September 26, we were given the devastating diagnosis of Acute Myeloid Leukemia.

Obviously, I cannot give anyone the full story of Katy's medical challenges in a few short paragraphs.    There are many gaps.  But I can tell you that Katy has been put under anesthesia for procedures or surgeries probably 40 times or more.  She has had multiple PICC lines placed while awake.  She has endured endless amounts of blood draws, poking, prodding and too many indignities to count.  All I can say is that my seventeen year old Katy is the bravest person I know.  She keeps fighting and hoping for a better future.  I am lucky to have such a young woman be my daughter.

Christina