Elliana Janae - Mira McCullough obituary photo
 
In Memory of

Elliana Janae - Mira McCullough

June 11, 2014 - May 11, 2017

Obituary


Elliana had a rare syndrome called Heterotaxy. This means her organs duplicated, forming mirror images. It affected the heart, lungs, liver, intestines, and much more. She was left isomerism, meaning she formed two left sides instead of left and right. She had two left atriums (top chambers in heart), two left lungs, midline guts and liver. Her heart was flipped and on the opposite side. Her stomach was the only thing on the correct side. With that syndrome, you can be born without a spleen (fights infection), with multiple non-functioning spleens (grapelike), one non-functioning spleen,...

Elliana had a rare syndrome called Heterotaxy. This means her organs duplicated, forming mirror images. It affected the heart, lungs, liver, intestines, and much more. She was left isomerism, meaning she formed two left sides instead of left and right. She had two left atriums (top chambers in heart), two left lungs, midline guts and liver. Her heart was flipped and on the opposite side. Her stomach was the only thing on the correct side. With that syndrome, you can be born without a spleen (fights infection), with multiple non-functioning spleens (grapelike), one non-functioning spleen, or no spleen at all. By the grace of God, she had one functioning spleen! She did have a clotting disorder and needed blood thinners for two years (injections twice a day).
At three months old, Elliana had a feeding tube placed along with a surgical procedure to correct her malrotated intestines. At any time, her intestines could twist on themselves so we decided to correct it before it happened. A little after that, she had her first open heart surgery. They were putting bands on the arteries that brought the blood to her lungs; she was over-circulating. Since birth, she would breath in the 60-70s. Sometimes I wouldn't be able to feed her because of it. At six months old, Elliana had her second open heart surgery. This would be her eleven hour corrective surgery, making her a two ventricular repair. Our amazing surgeon, Dr. James Quintessenza, had created Elliana's heart to be one of its own. Three weeks after her surgery, the doctors told me she would need a tracheostomy to help open her airway. Her pulmonary artery was leaning on her airway, needing a vent to push it open. Her heart was already enlarged so everything was smooshed inside. Her heart took up the majority of her right side.
Elliana spent five months straight in the hospital and was finally released April 2015. Elliana learned to do things she has never done before: sit, stand, walk and crawl. She learned over 60 signs in sign language and loved to read books. She loved her walks and being outside. Elliana's fine motor skills progressed so quick, we were securing her neck ties with tape! She would pick the Velcro and pull her trach out! She loved to pull Velcro apart and absolutely loved to drink water! She would sign more and point to a syringe, letting us know her exact demands! She did not want to eat any food by mouth for a really long time; it was a long-term goal. Eventually, Elliana was interested in food and would eat more than a few bites.
December 22, 2016, we brought Elliana into the hospital for a lump on her collarbone. That spiraled out of control, leading to a pulmonary hypertension crisis. Her lung vessels were constricting (squeezing), not allowing air through and causing her blood to back flow. She went into right sided heart failure.
Dr. Quintessenza was the chief of pediatric surgery and medical director for 19 years at John Hopkins All Children's in St. Petersburg, FL. Due to Dr. Q taking a wonderful opportunity to restart University of Kentucky's pediatric heart surgery program, I knew we had to follow him.
I requested for second opinions at Cincinnati Children's Hospital, where he was waiting on rights. (Got his rights in January 2017)

We were accepted to Cincinnati Children's Hospital in Ohio and Medicaid arranged the flight.
We arrived in Ohio January 16, 2017, where our lives would forever change.

Elliana had severe pulmonary hypertension at this point. Her pressures going to the lungs, through her lungs, and leaving her lungs were super high. Pulmonary hypertension is a disease that can only be controlled with medication, nitric and prayer. When your pressures reach a certain point, they can be deadly. This is what put her at such high-risk. We ended up starting an IV medication for this disease.
We were evaluated for a heart transplant at five centers: Cincinnati, Boston, CHOP, Atlanta, and Houston. All five denied her with no surgical option.

The middle of March, she took a crash.
It was go to the Cath lab and place a stent into her top atriums, or die. The surgeon didn't want to operate with high lung pressures and he really didn't see anything significant wrong to operate on. She had a lot of little issues resulting in high pressures. On March 21, 2017, the stent was placed and she sailed through. When she came back though, it was a different story. Her blood was not getting to her left atrium, she had no lung sounds, was being bagged to maintain low oxygen saturations, and was maxed out on four blood pressure medications! We had no room to go up.
The doctors told me she would not make it through the night. Well, she proved us wrong!

The week before Easter, we had a care conference to withdraw support. Elliana was needing to be so sedated, the littlest bit awake, her blood pressure would drop and her heart would go into a funky rhythm. This was an indicator her heart couldn't handle being awake; signs she was in heart failure and not doing good. She was on four blood pressure medications again and on a ton of sedation drips to keep her under. The doctors couldn't tell me what part was failing or even what her heart was doing with the rhythms. Children with Heterotaxy are so complex, nothing compares in their textbooks. Again, Elliana had her own plans and did a complete turnaround after that meeting. She was on little support with none to pull! Praise Jesus!

The Friday before Easter, April 14, 2017, Elliana had another heart catherization to measure her lung pressures. By the grace of God, the pressures through the lungs and leaving the lungs had dropped significantly! Due to new data and Dr. Q advocating for us, we were going to be re-evaluated for transplant at Cincinnati Children's!

May 10, 2017, we were accepted for a heart transplant!
May 11, 2017, Elliana had a stroke and a massive brain bleed, leaving us no choice but to let her go.


She was the smartest, sassiest, full of life child I knew. She was so limited in life, yet so happy. Every picture I have of her she has the biggest smile!
I have no regrets being her mother, I know I did everything I could for her. I am at peace knowing she is not limited now, running wild and free! Her death was as pain free as it could've been, as well as happening as it did so unexpectedly. I will forever remember her love and joy she brought to us all! She was so observant, she would know your every move.

I want everyone to remember her for the girl she was. A hero.

We want to personally thank everyone involved in Elliana's care. From the surgeons, doctors, fellows, nurses, sitters, therapists to the house cleaners, volunteers, and techs. It takes a village with her and I don't want anyone feeling left out. There are so many people, the list goes on. We could not have done it without everyone! A personal thank you to Dr. Quintessenza for believing in Elliana and giving her life. He advocated for us with all his might, and we thank you.

We want to thank every donor that has helped make her burial so special and putting us at ease!

Thank you!