Harper Ryals led a normal, healthy life until she was two years old. Suddenly, the little girl had a huge growth spurt, began having difficulty breathing and developed a persistent blue tint to her skin.
Her mother, Rebecca Newman, a homemaker from Mississippi, took her to the doctor and discovered that large swaths of the blood vessels in her lungs and brain were tangled and malformed, depriving her body and organs of oxygen.
Harper, now five, was diagnosed with a rare disease called hereditary hemorrhagic telangiectasia (HHT), which currently affects about 75,000 people in the United States.
Doctors discovered Harper had an even rarer form of the disease, which meant no traditional treatment would work to restore oxygen flow to the girl’s body, short of a double lung transplant. Without one, she would die.
Harper’s genetic form of HHT was declared terminal, meaning she was in the last phase of a progressive disease or just before death. It’s the updated medical term used instead of “terminal.”
Harper, with her mother Rebecca Newman and father David Ryals, stayed at the Ronald McDonald House when they were in Arkansas for one of many out-of-state medical visits.
She developed severe and debilitating migraines due to the loss of oxygen to her brain and had little energy to continue with her day as the level of oxygen circulating through her body was very low.
“Without a transplant, his life expectancy was running out. He needed continuous supplemental oxygen,” Ms Newman said in a GoFundMe that she would need funds for medical expenses.
HHT is a disease that affects the way blood vessels are formed and can affect any part of the body.
Instead of forming separately and passing blood normally, in HHT, blood vessels form tangled groups called arteriovenous malformations that can cause bleeding and blood clots.
These also reduce the amount of oxygen circulating through the body, which is what happened in Hayden’s case, causing his skin to turn blue.
Typically, when HHT lodges in the lungs, it does so in isolated areas, in larger clusters that surgeons can treat.
But Harper has a rare genetic type of HHT that causes many smaller clusters of malformed blood vessels to be distributed throughout her lungs.
“It’s something that really happens by a crazy astronomical fluke in the conception process,” Ms. Newman said. said TODAY.
Because the blood vessel problems in Harper’s lungs were so widespread, doctors were unable to perform surgery to correct individual blood vessels.
The only possible treatment would be to transplant two new lungs. Doctors did not give her a time frame, but declared her illness to be terminal, the updated terminology for terminal illness, meaning that without treatment the disease would eventually kill her.
Lung transplants are still quite rare in children and only a few dozen are performed each year.
This is partly because it is difficult to find healthy lung donors who match the recipient’s blood type and body size. Also, the person who died to donate the lungs must not have suffered organ damage before passing them on to the recipient, making a successful donation a rare occurrence.
Dr. Tina Melicoff, medical director of the lung transplant program at Texas Children’s Hospital where Harper was treated, told TODAY.com that these factors add up and many people wait a long time to find a pair of lungs that fit their needs.
“Even with those low numbers, wait times can be very long, and longer than for adults,” Dr. Melicoff said.
About 75 percent of candidates for a lung transplant in 2022 received the transplant within a year, according to the Scientific Registry of Transplant RecipientsDuring that wait, about 13 percent died or were removed from the waiting list.
Harper was included on the list in April.
Harper spent her fifth birthday recovering from a lung transplant in the ICU at Texas Children’s Hospital.
Before she was placed on the transplant waiting list, Harper and her family traveled to New Orleans on a Make-A-Wish trip. While there, she contracted a virus and became seriously ill, remaining in the pediatric intensive care unit for four months.
Finally, the family received word that a pair of lungs were available for transplant. On June 15, she received a new pair of lungs.
The transplant was a success. Ms Newmann told TODAY she saw that her baby’s skin had returned to its blue colour and was now pink.
Still, the little girl has a long road ahead of her.
Ms. Newman shared that her daughter would have to travel to different specialists in Mississippi, Arkansas and Missouri for the rest of her life to continue monitoring the other buildups on her brain and ensure her new lungs are functioning properly.
Despite these setbacks, the little girl is set to start kindergarten soon. Her mother shared that through it all, Harper has remained a “fun, loving, energetic, smart, beautiful, talented little girl. She has the biggest heart I know and is truly a gift from heaven.”