LEWISTON — On Thursday, the Flynn family got two phone calls.
The first let them know doctors had found a near-perfect bone-marrow match for 13-year-old Jordan.
The second, an hour later, told mom Doreen to rush Jordan to Portland for an emergency platelet transfusion. Her platelet count was so dangerously low that an accidental bump — from her little sisters, from the kids at school, from the family dog — could cause life-threatening internal bleeding.
But of the two, the first call — there's a match; there's a transplant date — was the scariest.
In a family in which three girls have Fanconi anemia, a rare genetic blood disorder that destroys bone marrow and makes people extremely susceptible to cancer, emergency trips to the hospital are common. That's life fighting FA.
That Jordan needs a bone-marrow transplant means the medication to hold it at bay is no longer working. FA is winning.
The Flynn family has been living with Fanconi anemia for the past decade. Of Doreen's five children, three have the disease: Jordan and her twin sisters, 7-year-old Julia and Jorja.
The twins have been doing well, though Jorja's platelet count has dipped in recent months, a sign the disease is wreaking havoc on her body earlier than anticipated.
Jordan, a smart, sarcastically funny eighth-grader at Lewiston Middle School, is struggling. Although she looks healthy, her bone marrow has deteriorated to the point of no return. Doctors recently found pre-leukemia cells.
Life with FA is a roller-coaster ride, filled with joyous news and devastation, sometimes both at once. This is no exception.
The good news: Jordan's bone-marrow donor matches hers nine points in 10, and the transplant has an 80 percent success rate. She is surrounded by supportive family and friends, many of whom are involved in an April 15 event that will double as a benefit dinner to raise money for the family's transplant expenses and a middle school dance to give Jordan the semi-formal she'll miss while she's in the hospital.
And the family recently won a lawsuit against the federal government, an early but significant step in allowing some bone-marrow donors to be paid and, the hope is, to increase the number of people willing to donate — possibly when Julia and Jorja need it.
The bad: A bone-marrow transplant isn't the end of the disease; it's only the latest treatment.
In exchange for weeks of feeling ill, months isolated in the hospital and at least six months away from home and school, Jordan could get some more time.
"It definitely won't be easy, of course, but I'm hoping that . . . " Jordan paused briefly and sighed, ". . . we'll get through it."
FA is a rare genetic disorder that affects about 1 in every 131,000 children in America, according to the Fanconi Anemia Research Fund. About 30 babies are born with FA every year in the U.S.
Both parents have to carry the same defective FA gene for a child to have the disease. Because the gene is recessive, children have a 25 percent chance of inheriting FA. The disease affects both boys and girls, as well as all ethnic groups, though it has a higher incidence among Jewish people of Eastern European descent and people native to South Africa.
Most FA patients develop bone-marrow failure. A bone-marrow transplant can fix that problem, but patients will always be at very high risk of developing various forms of cancer, including leukemia, head and neck cancer and gynecological cancers.
Although some FA patients live to middle age, the average life expectancy is just under 25, according to the research fund.
Doreen Flynn found out when Jordan was 4.
As a little girl, Jordan was too thin and too small for her age. She wasn't thriving. A blood test showed a low platelet count. It could be leukemia, doctors said. It could be cystic fibrosis. It could be FA.
If Jordan had to have one of the three, Doreen hoped for what most parents hope against: leukemia. At least that was curable.
It was not to be.
"I knew something was up when they told me Jordan could wait in the waiting room with a social worker and I got called back (to the exam room) by myself," Doreen said. "The doctor, the team of doctors that was there, basically said, 'The FA test came back positive; there's not a whole lot known about it,' and they handed me a book to read."
The book, put out by the Fanconi Anemia Research Fund, was one of the few resources available to FA families. Overwhelmed but with the urgent sense that she had to know everything immediately, Doreen plowed through the book over a weekend.
When Jordan asked why she was sick, Doreen tried to distill a book's worth of information down to the simplest, least frightening explanation she could think of.
"I told her she had bad blood," Doreen said.
It's the same thing she tells her 7-year-olds.
For years, Jordan stayed moderately healthy, but the doctors and FA experts said it was only a matter of time before she would need a bone-marrow transplant. Transplant success was highest with a sibling donor, but neither of Jordan's older brothers matched. Doreen and her then-husband decided to have another baby. With the help of in-vitro fertilization, they could all but ensure they'd have a child who was both free of the disease and a bone-marrow match.
Four embryos were tested. Two were a match and FA free. But Doreen believes there was an error with the testing, a mistake during implantation, or both. When Julia and Jorja were born, only one was a match for Jordan, though it didn't matter. Both newborns tested positive for FA.
Doreen was devastated.
"I felt like I failed them," she said as Julia snuggled in her lap and Jorja tried to get a computer game to work. "I blamed myself."
Seven years later, the enormity of the situation still hasn't sunk in.
"I'm in part denial that they even have FA," Doreen said. "I'm so focused on Jordan. When I speak about it, I find myself forgetting about the twins. I don't do it on purpose."
'Tell me everything'
In the Flynn household, chaos is normal. Little sisters annoying older ones is normal. Squabbles over the Internet, mom's attention and bedtimes, normal.
Also normal: blood tests, painful bone-marrow biopsies, platelet counts and treatment protocols.
Doreen said she answers, age appropriately, any questions the girls have about their disease and treatment. Jordan disagrees.
Through Camp Sunshine in Casco, Jordan has made friends with other FA kids and their families from around the world. Some of those friends have died over the years. She knows that can happen to her.
At 13, she thinks her mother doesn't tell her quite enough.
"I answer all questions you have," Doreen insisted Thursday as the conversation turned to the upcoming bone-marrow transplant.
"How do I know what to ask when I don't know anything?" Jordan said.
"Well, how do I know what you want to know if you don't ask?" Doreen said.
"Tell me everything," Jordan challenged.
"Anything you know."
"I know a lot," Doreen said.
Jordan sat back on the couch. "Then we'll be here for a while."
What Jordan does know, among other things: her FA has progressed to the point that she needs a bone-marrow transplant. She'll have to undergo radiation and chemotherapy to prepare her body for that transplant. Her hair will likely fall out, including the section she dyed bright pink because it was the favorite color of a girl who died of FA. She'll miss the end of this school year and the beginning of the next, her freshman year in high school.
And she knows how she feels.
"Scared is definitely one. Nervous." She thought for a moment, then added with a small, playful laugh, "And I'm feeling very poopy about it."
Her sisters know Jordan bruises easily and that soon she'll have an operation.
The transplant is scheduled for the end of April at Memorial Sloan-Kettering Cancer Center in New York. Jordan will stay in the hospital for the first couple of months. If all goes well, she'll move to a nearby Ronald McDonald House, getting daily checkups at the hospital for a few months more. The transplant and after care are expected to take at least six months. The family will be separated most of that time, Doreen in New York with Jordan and Doreen's fiance, Shawn Gummoe, at home with her other four children and, sometimes, his three.
Jordan's transplant and hospital stay will be covered by insurance and Doreen, who works in debit card operations at TD Bank, will work remotely from a company laptop, allowing her to receive a paycheck. But Doreen will have expenses in New York, as will the family when they visit. To help, friends are throwing a benefit dinner Sunday, April 15, at the Franco-American Heritage Center in Lewiston.
Also normal in the Flynn household: media interviews.
In recent years, the family has been written about or filmed about a dozen times. Recently, NBC interviewed Doreen and the kids for an upcoming 10-minute segment to air on Rock Center with Brian Williams. (The air date has not yet been announced.)
Years ago, the interviews centered on the girls and bone-marrow drives designed to find matching donors. Today, they center on a lawsuit in which Doreen is lead plaintiff. The suit, spearheaded by the Institute for Justice, a nonprofit, Virginia-based public interest law firm, seeks to allow bone-marrow donors to be paid. Doreen and others hope payment will spur more people to donate. More donations will mean more matches.
Currently, federal law prohibits bone-marrow donors — like organ donors — from being compensated. The suit argues that advances in medicine make bone-marrow donation more like plasma donation, which can be compensated, than to kidney donation, which cannot.
In traditional bone-marrow harvesting, doctors stick a needle through a hip bone and remove bone marrow. It's considered surgery. It can be painful. There are risks to the donor.
The alternative method, peripheral blood stem cell donation, is nonsurgical, less painful and has fewer risks. In PBSC donation, donors receive injections to increase the production of blood-forming stem cells, their blood is removed through a needle in one arm and passed through a machine that separates out the blood-forming cells. The remaining blood is returned to the donor's body through a needle in the other arm.
The donated cells are put into the recipient's body intravenously. The cells move through the bloodstream to settle in the bone marrow, where they grow and produce new white blood cells, then platelets and red blood cells.
In 2011, 76 percent of adult donors went through PBSC donation, rather than the traditional bone marrow harvest, according to the National Marrow Donor Program.
In December, the 9th Circuit Court of Appeals sided with Doreen, agreeing that bone-marrow donors could be compensated, but only those who go through PBSC donation.
"It's a partial win, but, yes, it's a win," Doreen said.
The U.S. Department of Justice asked the court to reconsider its decision. Last week, the court declined, signaling another victory for Doreen's side.
The department has fewer than 90 days to decide whether it will appeal to the U.S. Supreme Court.
Doreen said she joined the lawsuit and does the interviews to raise awareness of the need for bone-marrow donors.
"Without them, we wouldn't be going to transplant," Doreen said.
And she has two other daughters to think about. Jordan's FA emerged when she was 11.
Jorja's began at age 7.