Youngest sister a match for bone marrow transplant
by Randy Parks
Lizz (right) and Shelby Duhn sporting the T-shirts that are for sale as a fundraiser. (Photo by Randy Parks)
On Dec. 2, Elizabeth “Lizz” Duhn, 16, will travel to Seattle, Wash., for the start of what she hopes will close the chapter of her life that she refers to as “The Gata-2 mutation.”
The trip to Seattle will begin with rigorous testing to make sure Lizz is completely healthy. She will then undergo chemotherapy to kill her bone marrow and have a bone marrow transplant, with Lizz’s youngest sister, Shelby, being the donor.
It was the 2008-2009 school year, and Lizz was in sixth grade in Prineville, when she began to have pain coming and going in her right side and stomach area.
The first diagnosis was that she had appendicitis, but tests ruled that out. “They told me I was having ‘abdominal migraines,’ ” Lizz said.
The pain soon subsided, and Lizz got back to the business of being a sixth-grader. On New Year’s Eve, Lizz broke out with a rash from the shoulders down and began running a fever. As a precaution, Lizz was taken to the emergency room, where a blood test showed she had a low white blood cell count.
With the possibility that Lizz had leukemia, Lizz was referred first to Bend, then to Oregon Health & Science University (OHSU) in Portland for a bone marrow biopsy.
The biopsy showed no sign of cancer, but there was little cellularity in the marrow.
“I didn’t have all the cells, and there were weird-looking cells,” Lizz said. “Blood cells are usually little round cells, but mine looked all crumpled up.”
With no immediate answers, doctors began to run a battery of tests. “They did a million and one tests, testing for everything,” Lizz said.
After all the testing, there was still no definitive answer. “I was home-schooled during middle school because I had very little energy, and would spike fevers and get rashes,” she said.
Finally, she was diagnosed with aplastic anemia, or bone marrow failure, with an unknown origin. Because she wasn’t getting any worse, the doctor told her to go out and keep being a teenager, and her condition would either get better or it would get worse.
In December 2010, the Duhns moved to Burns, not expecting what was to follow.
Except for what she called “little things,” Lizz was doing well until Thanksgiving weekend 2012. She was at a friend’s house when she noticed she had swollen lymph nodes, and she felt, “so tired.”
Tests showed her complete blood count (CBC) had dropped, and it was decided she should go to Doernbecher Children’s Hospital in Portland, a division of OHSU.
There, it was discovered that her liver was inflamed, all lines of her blood cells were failing, and she was experiencing migraines.
She was hospitalized for eight days, during which time she had three blood transfusions, another bone marrow biopsy, and a peripherally inserted central catheter, or PICC line, inserted for additional transfusions to come. (A PICC line is a small, slender tube inserted into the upper arm, and moved through a vein until it is near the heart.)
There was still no definite diagnosis for her condition, but doctors knew her bone marrow was failing, and recommended weekly blood draws and transfusions if her blood levels dropped again. Meanwhile, the doctors would continue to look for an answer.
So began weekly trips to the local hospital for blood draws and dressing changes.
In January 2013, her blood count was again low, and she went to Harney District Hospital for a transfusion of two pints of blood. Just about the time the first pint was done, Lizz began to run a fever, and things went downhill in a hurry.
The fever got to 105 degrees and was still rising. Lizz began to vomit, her digestive system was failing, and her body was beginning to shut down. Nurses packed her in ice to reduce her body temperature, and a CBC showed no increase in blood. “It was like my body was destroying the blood they were giving me,” Lizz said.
With the fever down, but still in need of blood, another transfusion was started the following day. Once again, her fever began to spike, and at 102 degrees, Lizz was again packed in ice. At the same time, OHSU was contacted to provide air ambulance transport to Portland.
“It was terrifying,” Lizz said. “They stopped giving me blood, and I was freezing, from both the fever and the ice.”
As the fixed-wing aircraft was coming in to pick Lizz up, the power was knocked out in a good portion of Harney County, including the Burns airport.
Unable to land, the plane turned back, and a helicopter was sent from Bend for the transport. With not enough room for her father, Frank, in the helicopter, he started the drive to Portland, while the helicopter headed for Bend with Lizz aboard.
Once in Bend, Lizz was transferred to a fixed-wing aircraft, pointed toward Portland. The Portland airport was fogged in, however, and the plane had to land in Hillsboro, where Lizz was again transferred to ground ambulance and taken to Doernbecher. The trip lasted about three hours, and her father arrived just one hour later.
Once in Portland, Lizz said she was “drowned with antibiotics, and her vital signs were checked every two hours.”
Doctors determined that there was a gram rod negative infection in her PICC line, and when the transfusion was started, the blood flushed the bacteria into Lizz’s system. “It was a bacteria that is normally found in your stomach, and it somehow got into the PICC line,” Lizz said. “Once it got in my body, the immune system began to fight it.”
Lizz spent five days in the hospital, and when she had four bacteria tests come back negative, she was released. The first PICC line was also removed, and a second one put in. Lizz said she got to watch the insertion of the PICC line on a screen as it was happening, and added, “I love that kind of stuff.”
Lizz returned to Harney County with instructions to continue blood draws and transfusions every four to six weeks.
In the spring of this year, Lizz made a trip to Seattle to see an immunologist. During the visit, the immunologist told Lizz and her parents, the problem wasn’t her immune system.
Doctors at OHSU decided Lizz should meet with a bone marrow failure specialist (non-malignant), and an appointment was made with Dr. Akiko Shimamura at Seattle Children’s Hospital.
Another bone marrow biopsy was performed at the request of Shimamura, as well as a number of blood draws. “They took 18 vials of blood,” Lizz said. “My blood was sent to Boston, Cincinnati, all over the country. I’m definitely in the research bank.”
Through the efforts of Shimamura, on Aug. 9, it was determined that it was a Gata-2 mutation that was causing Lizz’s bone marrow failure.
Gata-2 is a human gene located on the third chromosome, and expressed in the formation of blood’s cellular components, which is derived from bone marrow.
“You have 26 chromosomes, and they copy each other. But I only have one third chromosome, the one that’s associated with bone marrow and making blood. It doesn’t have a ‘partner in crime,’ ” Lizz said. “So, all the percentages of the blood components, red blood cells, white blood cells, platelets and so on, are OK, I just don’t have enough blood in my body.”
Left unchecked, Gata-2 mutation will likely result in leukemia. “We’re glad it was caught before the leukemia grew,” her mother, Dena, said.
The Duhns were told a bone marrow transplant was needed to correct the problem, and Lizz would need a donor.
Tests were conducted on Lizz’s two sisters, Rachel and Shelby, and Shelby’s test showed enough of a match to qualify her as a donor.
“You have to have at least six chromosomes match so there’s less chance of rejection,” Dena said. “If a person doesn’t have sibling match, they can go down to three, but that increases the chance of rejection.”
When the Duhns arrive at the Cancer Center in Seattle Dec. 2, Lizz will undergo a battery of tests. “There’s tests on the heart, lungs, dental, eye, kidney, liver, everything, to make sure all organs are healthy,” Lizz said.
She’ll undergo 10 days of chemotherapy to rid her body of her bone marrow, and prepare it for the transplant.
Shelby, 11, will be put on iron supplements and a high-nutrient diet to boost her bone marrow, as well.
Lizz said doctors will use four skin punctures, and about 100 harvest samples, from the lower back and pelvic area to get the bone marrow from Shelby. The marrow will then be filtered and implanted into Lizz.
“After the transplant, if you were to check my DNA from a swab or the skin, it would be mine, but if you check the blood, it’ll be Shelby’s,” Lizz said. “I’ll be me, but I’ll be part Shelby too.”
Once the transplant is completed, Lizz will be taking 20 to 25 pills a day to combat any side effects. “One pill might make me sick, so I take another to correct that. Any time you disrupt something in the body, it’s so in sync with itself that it can knock everything out of whack,” Lizz said. She could be on the medication for up to two years.
She will also have a feeder tube put in to make sure she gets all the nutrition she needs, and the transfusions will continue.
For 30 days after the transplant, Lizz will be monitored closely for any infection, rejection or complications, and for the first 100 days she must remain within 30 minutes of the cancer center.
When asked about the emotional part of the process, Dena said, “We’ve run the gamut of emotions. We had the ‘madness,’ the ‘Why me?’ But now we know what it is, and we know we have a long road ahead of us.”
“It’s OK,” Lizz added. “I want to go to medical school, so I’m learning a lot.”
The transplant means that Lizz and Dena will be staying in Seattle for four to five months, with regular visits from Frank, Rachel and Shelby, so a number of fundraisers have been established or planned to help with medical expenses, travel, lodging, food and other costs.
Gata-2 T-shirts are available for $15 each at Ribbons and Roses, Bodywise Sports Center and Burns High School.
An account has been set up at Sterling Bank for donations (Elizabeth Duhn Medical Benefit), Jitters RevOlution will be donating a portion of the cost of every latte sold to Lizz’s benefit account, and refundable cans and bottles can be dropped off at the tan trailer at the front of Glory Days’ parking lot. There are also plans for a benefit dinner, silent auction and dance in February.
To help with fundraising efforts, call Zoe Thompson at 541-647-4540.
The next chapter
Doctors have only been aware of the Gata-2 mutation for the past two years, and Lizz will be the seventh person to undergo the bone marrow transplant for the mutation at the Seattle Cancer Center.
Lizz is a junior at Burns High School, and has been active in Key Club, Young Life and cheerleading. The transplant will mean a year off from everything, but once it’s done, look for Lizz to begin the next chapter of her life.