At an age when most young ladies are spending their time reading YA fiction, taking selfies or fiddling with makeup and clothes, 13-year-old Abby Howard of Perry is getting a crash course in medicine while doctors try to figure out what is causing the high blood pressure in her heart and lungs.
Abby’s condition has a name: pulmonary arterial hypertension (PAH), which simply means she has high blood pressure in her heart and lungs. Her doctors at Omaha Children’s Hospital don’t know the reason for Abby’s PAH, so they are running her through a gauntlet of tests to find out, including lung biopsies, bronchoscopies, echocardiograms, EKGs, MRIs, PFTs, RHCs and several other invasive acronyms.
“This little girl is so brave,” said Amanda Howard, “but she is fighting a battle.” Amanda is Abby’s mom. She first noticed symptoms when Abby was less than a year old.
“When Abby was a baby — 8 months old, to be exact — she stopped eating, and I took her to my pediatrician at that time,” Amanda said. After some uncertainties, Abby was admitted to Methodist Jennie Edmundson Hospital in Council Bluffs, where they discovered her lungs were full of fluid. She was rushed from there to Children’s Hospital and Medical Center in Omaha.
“Later that night, Abby’s vitals start crashing, and she goes into cardiac arrest,” Amanda said. “It took them 15 minuets to get her back.”
Shaken by this close call, Amanda was then faced with the first of many hard medical choices for her daughter.
“I remember that Dr. Fletcher was called in, and he told us he believed Abby had PAH,” she said, her voice shaking with memories from 2005. “He told us he couldn’t prescribe her meds unless we knew that’s what she had, so I signed the paper for Abby to have the RHC.”
RHC stands for a right-heart catheterization. The procedure involves guiding a small, hollow tube to the right side of the heart and then into the pulmonary artery. It yields very accurate measurements of blood pressure in the heart and lungs.
Amanda consented to the RHC, and “after that it seemed like Abby started to get better with the meds.”
But Abby was not out of the woods. A few months after her first crisis, as she neared her first birthday on Sept. 14, 2005, Abby’s Omaha doctors told Amanda that her daughter would need a double-lung-and-heart transplant.
“We were set to meet that January with the transplant team in Houston, Texas,” Amanda said, “and do testing to see if she qualified. But the day before we were supposed to leave, Dr. Fletcher ordered another echo, and it looked good, so they had us wait. We never had to go.”
Things slowly improved after that and by the time she was 6 years old, Abby was doing so well that she came off all her medications and was eating without a feeding tube — “a healthy, happy, normal kid,” Amanda said.
A routine echocardiogram at the end of August again showed high pressures in Abby’s heart, and suddenly she was repeating the full battery of tests she took a dozen years ago, with a stress test, RHC and CT scan in September and a lung biopsy in October.
The Omaha doctor “said he thinks she has lung damage and he said if we don’t get a handle on this, it will drastically shorten her life,” Amanda said. New drugs were tried, and Abby was put on 24-hour-a-day oxygen because her oxygen level “would drop down when she would walk around, and she was very fatigued, short of breath and had chest pain.”
But the biopsy detected no disease in her lungs, so in November a rheumatologist was called in to do blood work — to look for an autoimmune disorder — and another bronchoscopy was ordered — to check for aspiration or foreign matter inhaled into the lungs. By early December all the test results were in.
“We found out Abby doesn’t have an autoimmune disease,” Amanda said, “nor is she aspirating, which is fantastic news. Abby has now been labeled as having an interstitial lung disease of unknown origin with secondary PAH.”
In other words, the doctors haven’t quite pinned it down. And less fantastic is the prognosis for PAH, which is a progressive, degenerative disease with no cure.
“The meds can slow it down, but it doesn’t stop it,” she said. “It’s a nasty disease that does not slow down. That’s where we’re at.”
How is Abby handling this latest series of disruptions? “She’s pretty resilient,” according to her mother, and getting off round-the-clock oxygen has raised her spirits.
“I think she really hated having to wear the oxygen 24/7,” Amanda said. “I think she felt out place. I know she hated having to wear it to school because she felt like kids were looking at her. My husband and I have both noticed that she seems to be a little bit happier now. It’s strictly at nighttime and strictly with exercise now, so that gives her a little bit of a break.”
Amanda and her husband, Chris Howard, are working people without great stores of wealth to draw upon. They live in the Bar-Jac Mobile Estates on Perry’s north side with Abby, her younger sister, Grace, 9, and her younger brother, Blake, 4.
“We are struggling to make bills and survive,” Amanda said, “but at the same time Abby is so worth it.”
As if medical anxieties weren’t enough, a further stress on the Perry family arose last summer in the wake of Sabrina Ray’s murder. Grace and Blake had attended the daycare center run by Marc and Misty Ray, and the revelation of the inner workings of that house of horrors caused Amanda to quit her regular Monday-through-Friday job and take an every-weekend job so that she and Chris could keep their youngest kids out of daycare.
“It just shook us up really bad,” she said. “Going back and forth to Omaha is killing us. We’ve been going back and forth to Omaha every one to two weeks since the end of August, and we were barely making it before because after the whole thing was Sabrina and everything, we didn’t want our kids back in daycare after that, so I took a pay cut and found a job where I work every weekend and Chris works during the week. That way our kids are never at daycare. It may sound mean, but I’m just not comfortable putting my kids back in daycare.”
“2017 was a hard year for us,” Amanda said. “I’m glad it’s over.”
The uncertainty in Abby’s diagnosis still left the Howards unsettled. The doctors at Omaha Children’s Hospital “are stumped,” Amanda said. “They have no idea what is wrong.” Responses to her post in a PAH discussion group led Amanda to decide to seek a second opinion.
“I made a post one time,” she said, “saying something about being a little frustrated that Abby’s doctors can’t figure out what’s wrong with her lungs, and people said, ‘You don’t have to be discouraged with your doctors to get a second opinion. If you in your heart feel like your child needs more answers, reach out and get a second opinion.’ So that’s pretty much how it happened. I was feeling discouraged that they can’t figure this out after all the tests she’s been through, and I want another new set of eyes to look at her to see if maybe they’ll pick up on something because they haven’t seen her all 13 years.”
With any luck, the family will travel to Milwaukee Jan. 23 for a second opinion, and Abby will have a busy day of testing, with an echo at 11 a.m., an MRI of her lungs and chest at noon, a stress test at 1 p.m. and an EKG at 2 p.m. Afterward, the family will meet with the specialist to discuss ways forward.
“If they don’t find anything, she’ll come back to Omaha and have genetic testing done,” Amanda said, “and if genetic testing doesn’t show anything, she’ll be labeled as idiopathic PAH.” “Idiopathic” is the fancy term for a disease or condition that arises spontaneously or for which the cause is unknown.
Amanda has shared a wealth of stories on a Facebook page called Team Abby, and a GoFundMe page has been set up to help defray the travel expenses to Wisconsin.
“We have no idea how we are going to make it to Milwaukee,” Amanda said, “but we will figure it out.”
