SOLANA BEACH, California — How incredible would you feel if you knew you could save the life of a beautiful 15-month-old baby girl, with something as innocuous as a cheek swab? The surrender of a few minutes on your part might provide a chance to reach milestones for this child and her parents. Consider her first day of school, learning to ride a bike, losing her first tooth, observing her bat mitzvah, dressing for a first date, kvellling at her chuppah, welcoming her own child. By registering with Be the Match, you may be offering Zoe and her parents all of these magical moments with minimal effort on your part.
Since I was 18, I have been donating whole blood. The first opportunity was at my local synagogue in Mamaroneck, New York. My parents encouraged me to join them and I was elated to know that for less than half an hour of my time, my blood might benefit up to four people in need. This was heady stuff indeed! I always prided myself that I had an A+ in blood. Well, it was really no reflection on me, merely my lucky genes.
How far science and technology have come since the 1970s! Last week I learned of something so amazing that I believe the future is here…and we can participate in its fantastic capabilities. What I’m referring to is the ability to share our blood-forming stem cells to save those afflicted with Leukemia, Sickle Cell and other blood cancers. Often, these stem cells are donated through a non-surgical procedure that resembles a plasma donation.
In a phone call with Erica Sevilla, spokesperson for Be the Match, I was introduced to the goals and achievements of the National Marrow Donor Program. During our talk, Erica shared with me a poignant story of Zoe Smith, a 15-month-old girl, and her parents, Kirsten and Theo.
Zoe and her family were visiting her grandparents in Idaho when COVID-19 struck. During the stay-at-home orders, Zoe began exhibiting irritability which was uncharacteristic of her usual “really reasonable” self. Her mom and dad went to several doctors, and received a number of misdiagnoses. Zoe’s health continued to decline and she became less active and more withdrawn. Finally, a trip to the ER provided answers, but not something any parent wants to hear. The ER docs had asked Zoe’s parents, “What do you want us to do?” Kirsten and Theo wanted an exhaustive battery of tests done, even though they did not want to subject their daughter to any more poking and prodding.
The team came back and reported on Zoe’s very low white blood cell count. Finally the doctor, not an oncologist, said, “What we see is consistent in patients with leukemia.” Zoe’s parents couldn’t fathom this news, yet they were somewhat relieved that they had a diagnosis to explain Zoe’s uncharacteristic behavior. The nearest hospital was in Salt Lake City. It was a four and one half hour drive, but as Theo went home to pack up, the medical team at Children’s Primary Hospital in Utah had reviewed Zoe’s test results and declared her unfit for the car ride. Zoe and Kirsten were airlifted to Salt Lake City.
The pandemic impacted the small family, and while mother and daughter acclimated to the hospital, Zoe’s dad was not allowed to visit for some days. During this time, the tests revealed that Zoe was suffering from not one, but two types of blood cancers. The first one is Chronic Myeloid Leukemia or CML, a chronic cancer that is generally seen in adults and treated with oral chemotherapy. The second type is Acute Lymphocytic Leukemia (ALL). Zoe received a blood transfusion and was obviously in a lot of pain. Before the onset of her illness, she’d been starting to walk, but now could barely pull herself up. She’d lost muscle tone and weight, and Kirsten reported that she was more like a 5-month-old in her movements. All she wanted to do was breastfeed and be held by her mom.
Following her first round of chemotherapy, Zoe perked up and became more mobile. She was allowed to leave the hospital for a bit, and the family is staying at the Ronald McDonald house, near the hospital. Each Monday, Zoe returns to see the oncologist and she is visited by a Home/Health aid 4 or 5 days a week. Zoe is still severely immunocompromised and is awaiting a blood stem cell transplant from a perfectly matched donor.
Typically only 30% of matches come from family members. The other 70% of those in need look to find a donor on the Be the Match Registry. There is no perfect match in Zoe’s immediate family, nor is there anyone out of the 35 million individuals on the international registry. “Why is it such a challenge?” I asked of Sevilla.
When one donates stem cells or marrow, there are protein markers in the blood that are called human leukocyte antigens (HLA). These markers determine the suitability and likelihood of a successful stem cell transplant. The HLA is derived from an individual’s ethnicity and his parents and grandparents’ genes. Zoe’s grandfather is 100% Ashkenazi. This means Zoe is ¼ Ashkenazi Jewish and the likelihood is that her best match will come from someone of Eastern European extraction with similar genetic and ethnic background.
The challenge is that as our world becomes more global and diverse, it is increasingly more difficult to find perfect matches for the HLA. This is why the registry is seeking more individuals who are a mosaic or manifest blended inheritance. To this end, Be the Match seeks healthy adults of all backgrounds between the ages of 18-44. They provide a free kit for an at-home cheek swab and all expenses related to testing or procedures are covered by Be the Match.
If a member of the registry is identified as a perfect match for a patient, doctors will request one of two donation methods. Nearly 80% of donors are asked to give peripheral blood stem cells (PBSC) in a non-surgical procedure. The other 20% will have liquid marrow extracted from behind their pelvic bones while under anesthesia. Both methods require testing and preparation over a 4-6 week period that will require about 20-30 hours of the donor’s time.
For each of the five days prior to PBSC donation, a donor will receive an injection of filgrastim, a drug that stimulates white blood cells and stem cell production. The shot is not painful according to donor Jaclyn Puccini, and recovery is speedy. The actual donation takes between 2 to 6 hours, and resembles a plasma donation where blood comes out of one arm and is returned to the other. The donor recuperates his blood cells within days. For more information about joining the registry visit https://join.bethematch.org/savezoe. The Donor Services team walks donors through the entire process, and procedures are covered by insurance and some government support for the non-profit.
Prior to a transplant it is essential that Zoe undergoes intensive chemotherapy to suppress her malfunctioning immune system and eliminate the cancerous cells. Once in this highly immunosuppressed state, the patient is ready to receive the new blood cells and for the next 100 days must be quarantined and monitored, until the patient begins producing the donor’s cells on his own.
Returning again to Puccini, I was curious to know what made her sign up for the registry. Her story is a personal one. Approximately three years ago, her mother was diagnosed with Acute Lymphocytic Leukemia (ALL), the very same rare cancer Zoe suffers from. Since the best match may come from family members, Jaclyn and her siblings all joined the registry. None of them was a 100% match, yet, amazingly Be the Match found 4 unrelated matches on the registry. Based on her age and HLA type, a young healthy Polish woman proved the perfect match, and Jaclyn’s mom made a full recovery. I was elated to hear that in 2019, Jaclyn’s mom was able to go to Poland and meet the woman, whom she described as her sister. But that is not where Puccini’s story ends.
Ten months after her mother’s transplant, Jaclyn received a phone call from Be the Match. She was the match for a young girl with a blood disorder. Though she acknowledged she is no fan of needles, Jaclyn felt this was serendipitous. “I just wanted to repay this selfless stranger that took the time to do this for my mom.” Though Puccini told me she passed out when she gave blood, she eagerly submitted to the five days of injections and the few hours of the procedure. She reported “laying around, watching movies” for a day. Would she do it again? I queried. “In a heartbeat!” In fact, Puccini became so passionate about Be the Match and all that they provide, that she reached out to them, and has become a Community Manager on the organization’s social media team. She dreams of helping to recruit more donors and save more lives.
Zoe’s story deserves many more chapters, and each potential donor brings a greater chance of matching her HLA, and giving Zoe healthy blood so that she can grow to bring even greater joy to her parents, family, community and the world. A simple cheek swab may reveal that you are a perfect match for someone in need. At the end of our phone call, Kirsten Smith told me, “I’m passionate about people signing up so others will be helped, even if it is not Zoe.” Please look at the attached link and consider joining the registry for Zoe Smith because a few minutes of your time may mean a lifetime of hers. https://www.bethematch.org/zoe
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Eva Trieger is an educator and a freelance writer specializing in coverage of the arts.
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