Harper Ryals led a normal, healthy life until she turned two. All of a sudden, the toddler hit a big growth spurt, and she began having a hard time breathing, and developed a persistent blue tint to her skin.
Her mother, Mississippi stay-at-home mom Rebecca Newman, took her to the doctor and discovered that large swaths of the blood vessels in her lungs and brain were tangled together, malformed – starving her body and organs of oxygen.
Harper, now five-years-old, was diagnosed with a rare disease disease called hereditary hemorrhagic telangiectasia (HHT) – which about 75,000 people in the US currently live with.
Doctors discovered Harper had an even rarer form of the disease that meant no traditional treatment would work to restore oxygen flow to the child’s body – aside from a double lung transplant. She would die without one.
The genetic form of HHT that Harper lives with was declared end-stage, meaning that she was in the last phase in a progressive disease, or just before death. It is the updated medical term to use instead of ‘terminal’
Harper with her mother Rebecca Newman and father David Ryals, used lodging at the Ronald McDonald house when staying in Arkansas for one of many out-of-state doctors visits
She developed severe, debilitating migraines from the loss of oxygen to her brain and had little energy to go about her day, since the level of oxygen circulating around her body was so low.
‘Without transplant, time was ticking for her life expectancy. She was on continuous supplemental oxygen,’ Ms Newman said on a GoFundMe for medical expenses.
HHT is a disease that affects the way blood vessels form – and it can affect any part of the body.
Instead of forming separately and passing blood normally, in HHT, blood vessels form tangled clumps called arteriovenous malformations which can cause bleeding and blood clots.
These also reduce the amount of oxygen moving around the body, which is what happened in Hayden’s case, leading to her blue skin.
Normally, when HHT takes host in the lungs, it happens in isolated areas in bigger clumps that surgeons can treat.
But Harper has a rare genetic type of HHT that causes many smaller clumps of malformed blood vessels to be distributed throughout her lungs.
‘It is something that actually happens by a crazy astronomical chance in the conception process,’ Ms Newman told TODAY.
Because of how widespread the blood vessel problems were in Harper’s lungs, doctors couldn’t perform surgery to correct the individual blood vessels.
The only course of treatment would be to giver her two new lungs a transplant. Doctors did not give her a timeline, but declared that she had end-stage disease, the updated terminology for terminal, meaning that without treatment, the disease would kill her eventually.
Lung transplants are still quite rare in children with only a couple dozen being performed each year.
This is in part because it’s difficult to find healthy donor lungs that match a recipient’s blood type and body size. In addition, the person who died to donate the lungs must not have sustained damage to the organs before passing them onto the recipient – making a successful donation a rare occurrence.
Dr Tina Melicoff the medical director of the lung transplant program at Texas Children’s Hospital where Harper was treated, told TODAY.com that these factors contribute to add up, and many people wait a long time to find a set of lungs that fits.
‘Even with those low numbers, their wait times can be very long — and longer than for adults,’ Dr Melicoff said.
About 75 percent of candidates for lung transplant in 2022 got the transplant within a year, according to the Scientific Registry of Transplant Recipients. During that wait, about 13 percent died or were removed from the waitlist.
Harper was put on the list in April.
Harper spent her fifth birthday recovering from the lung transplant in the ICU at Texas Children’s Hospital
Before she was put on the transplant list, Harper and her family made a Make-A-Wish trip to New Orleans. While there, the little girl contracted a virus, and fell deathly ill – staying in the pediatric intensive care unit for four months.
Finally, the family got the news that a set of lungs was available for transplant. On June 15th, she received a new set of lungs.
The transplant was a success. Ms Newmann told TODAY she saw the color return to her baby’s skin – turning from blue to pink.
Still, there is a long road ahead of the little girl.
Ms Newman shared that her daughter would have to travel to different specialists in Mississppi, Arkansas and Missouri for the rest of her life to continue monitoring the other clumps in her brains and insure her new lungs working properly.
Even with these set backs, the little girl set to start kindergarten shortly. Her mother shared that through it all, Harper has remained a ‘fun, loving, energetic, smart, beautiful and talented little girl. She has the biggest heart of anyone I know, and she is truly heaven sent.’
