Waiting for a bone marrow transplant: Will the family’s prayers be answered?
NEW ENGLAND, N.D. — Six-month-old Henley Johnson likes to play with her sisters, crawl on the floor with her toys and cuddle with her mom and dad. She's tiny, weighing 11 1/2 pounds, but she's strong—she has to be.
Henley, daughter of Jessica and Kyle Johnson of rural New England, was diagnosed with Shwachman-Diamond Syndrome (SDS)—a rare congenital disorder characterized by pancreatic insufficiency, bone marrow dysfunction, skeletal abnormalities and short stature.
"She doesn't know she's any different, she wants to keep up with her sisters, she coos, and she's super-attached to me—she's spent just a couple hours of her entire life away from me," said Jessica.
The life expectancy of patients with SDS is 35 years, as stated on a SDS web page. However, children with haematological abnormalities, like Henley, will have a greatly reduced life expectancy unless they can get a bone marrow transplant.
"The bone marrow transplant replaces everything—the red blood cells, the platelets and white blood cells (the neutrophils)—it's completely new building blocks to her body," she said. "If you would remodel a house, you would go in with all new framing—this is all new framing to her blood system."
Since birth, Henley has been evaluated on a month-to-month basis and doctors expressed the urgency to find a transplant donor. The problem, however, has been no bone marrow match was found within Henley's circle of family and friends—not her parents, not her sisters, no one in the family.
Would a bone marrow donor be found in time?
Returning from a recent medical trip to Minneapolis, the Johnsons learned a promising match had indeed been found ... but with conditions.
Medical history to this point
Henley was born May 26, 2017, with rapid respiration at about 90 breaths per minute. Initially, doctors thought it was transient tachypnea of the newborn—something she'd outgrow. However, after going home, Henley's parents noticed she was super pale, sometimes greyish in color.
"She was extremely fussy in comparison to our twins (Everly and Coven). The only way she'd sleep was resting on my chest in the recliner. She was hungry—that's part of the syndrome—she can't digest her food," said Jessica.
Henley was taken to Bismarck where a bone marrow biopsy was performed. It revealed neutropenia—an abnormally low count of a type of white blood cell (neutrophils)—cells that protect the body from infections. Without enough neutrophils, the body can't fight off bacteria.
"It was shocking—Henley didn't have any cells," Jennifer said. "A common cold would be a big deal for her—it's harder on her system."
Henley was flown from Bismarck to the University of Minnesota Masonic Children's Hospital for further testing. Four weeks later, the diagnosis came back as Shwachman-Diamond Syndrome.
"At that point, she and I had been away from home 44 days," said Jessica. "Henley got another blood transfusion about a month ago, but right now she's stable."
A fundraiser was recently sponsored by the New England community to help the family with medical expenses of the bone marrow transplant.
"It would make for less dependent supportive care, she'll have fewer restrictions in her life and less susceptible to infections," she said.
The family is affiliated with the national bone marrow donor program —bethematch.org. It's a simple Q-tip swab inside the cheek.
"We believe the more we share Henley's story, the more we can encourage other people to join the registry," Jessica said.
Jessica and Kyle learned they are both carriers for the gene mutation. If they were to have another child, there is a 25 percent chance the child would also be born with SDS.
"We did not know that until this happened. It's so rare there's only a couple hundred cases in the United States," she said.
To this point Jessica has been breastfeeding Henley.
"Everytime, I give her an oral medication that replaces the job of her pancreas and helps her digest her food," she said.
Then Tuesdays and Fridays, Henley is injected with a G-CFS factor to stimulate her bone marrow to produce neutrophils.
"It's her normal—she's tough. She's very social and smiley. She's very determined and just so strong for being so small. I call her my tiny mighty," Jessica said.
In addition to a bone marrow transplant, Henley will still need enzyme supplements and growth stimulants.
"She still needs oral meds every time she eats and she'll still need surgeries for her bones and joints," she said. "The meds are extremely expensive, it's ridiculous. We have good health insurance, but we're trying to enroll in a research study, where one of the medications is free—we're waiting to hear if we're accepted or not."
"When you go for a transplant, basically you're gone six months," she continued. "We plan to all go—at this point I want to be able to see my twins. I can't go through this by myself—we need to be together as a family."
In preparation for the trip, the family has sold their horses and cows.
"We're trying to downsize to make it as simple as much as possible," she said.
The family drove to Minneapolis last week to consult with doctors—to see if Henley needed another blood transfusion, to adjust her medications, and to get an update on the bone marrow search.
"What a whirlwind trip again!," Jessica wrote from Minneapolis.
Doctors are hoping Henley will make another three weeks before needing a fourth transfusion. However, the neutrophils had dropped to dangerously low levels again; hence the need to increase medications, she said.
To optimize her nutrition, Henery is being fed small amounts of pureed meat with olive oil or avocado oil, in addition to breastfeeds.
Most importantly, Jessica said, "A perfectly matched donor has been identified for Henley, but, and that word is very important, but we don't know for certain that he would be available or would go through with donation."
The Johnsons learned the match is a 22-year-old male—a perfect match down to the blood type.
"Here's where it gets complicated," Jessica wrote. "We don't have a transplant date and are trying to delay so Henley can be as big and strong as possible when she enters transplant. We can't hold this particular donor. We can't even ask him if he would be available because we don't know (the transplant date.)
Doctors at the University of Minnesota will perform another bone marrow biopsy in December to monitor any changes. "They can actually predict when the body is starting to develop leukemia changes in her cells—she hasn't had a biopsy since July," she said.
Any changes will move up the transplant date, but doctors prefer to delay the procedure until she is stronger. The transplant will take place in Minnesota.
"The goal is to transplant around her first birthday—either in April or May," she said. "We are in constant communication with the doctors as we develop the date."
As of Thursday, Henley was not responding to the increase of medication to produce more neutrophils.
"We're living on a wave—she's changing constantly. It's been literally week by week. If we don't have a response to the medication, doctors may say now we need to do the transplant. It's a lot of ups and downs. If you ask me what the hardest part is, it's just not knowing—things could change tomorrow."
Consolidated, in Dickinson, is sponsoring a swabbing event in support of Henley Johnson who needs a bone marrow transplant. Names will be added to the National Bone marrow Donor Registry.
The swabbing event is from 2:30-4:30 p.m. on Tuesday, Dec. 5, at Consolidated, 507 Main South, Dickinson.
"We want to ask you to continue to help us," said Jessica. "Our mission to grow the registry has not changed with the news about a promising match for Henley. Not even after transplant because there will always be a special someone, like Henley, that is out there waiting for their hero."
She referenced Roman, a 3-year-old child with SDS living in Texas. He and his family have spent his whole life looking for a match.
A benefit account for Henley has been set up at the American Bank Center. And you can follow Henley's journey on Facebook at healing henley.