Before they had names, before they had cute little grins, before they had even left their mother’s womb, the Espayos twins were already in a fight for their lives.
Zoey and Zayne Espayos were born on August 30, 2013, but their story actually begins eight blood transfusions earlier. You might think the most shocking part of Reina Espayos’ pregnancy was learning that she was carrying identical twin girls. You would be wrong.
Early in her pregnancy, Reina and her husband Mark learned that both babies had a deadly genetic blood disorder called alpha thalassemia (hydrops fetalis). The disorder would leave the babies unable to make hemoglobin within their red blood cells. Hemoglobin is important because it carries oxygen throughout the body. In medical literature, the phrase “incompatible with life” often follows the description of the condition.
For the couple from Hamiota, Manitoba and their 11-year old daughter, it was a heart-wrenching moment. “We were absolutely devastated,” says mom Reina. You don’t hear much about alpha thalassemia in North America, but the less severe forms of the genetic condition (silent carrier and alpha thalassemia trait) are actually quite common in people of African, Southern Chinese, Southeast Asian, Middle Eastern and Mediterranean descent.
Mark and Reina Espayos met and married in the Philippines. Unknowingly, both were carriers of alpha thalassemia trait when Reina became pregnant with the twin girls. Any children the couple produced would have a 25% chance of having the severe form of the disorder. Their first-born daughter Zachi was perfectly healthy, but the genetic dice would roll differently for the twins. Affected by the severe form of alpha thalassemia, both girls would be in heart failure at 6 months into the pregnancy.
A plan takes shape
In addition to treating cancer, CancerCare Manitoba is also deeply committed to finding better ways to treat blood disorders. Dr. Geoff Cuvelier, a Pediatric Hematologist-Oncologist and specialist in blood and marrow transplantation at CancerCare Manitoba, began to discuss the twins’ case with the perinatology team at the Women’s Hospital to find a solution.
Working together, the two teams of specialists devised a bold plan. If it worked, it would be one for the books.
“If the perinatology team could administer in utero blood transfusions to the babies while they were still in the womb, it might reverse their heart failure and keep them alive until they were old enough to be delivered closer to term. Once the twins were born, the team at CancerCare Manitoba was prepared to keep them alive after birth with regular blood transfusions,” said Dr. Cuvelier. Adding, “But, in the long-term, we needed to think outside the box. Lifelong blood transfusions are not good for the body, so we would ultimately need to try to cure their disease through the use of bone marrow transplantation.”
Worldwide, only a few cases of bone marrow transplantation for severe alpha thalassemia patients have been reported in medical literature. There are, however, no known cases where identical twins with the disorder have survived as the result of in-utero blood transfusions followed by bone marrow transplantation. It was a doubly daunting challenge that the CancerCare team was ready to take on – provided they got the chance.
Perinatologists Drs. Gregory Reid and Jennifer Hunt began performing in utero blood transfusions in Reina’s 28th week of pregnancy. This involved passing a needle, with ultrasound guidance, into the umbilical vein of the fetus and injecting red blood cells. “Transfusing identical twins with a shared placenta was particularly challenging, as a complication with one baby could affect the other,” says Dr. Reid.
The girls – who had only previously been referred to as Twin 1 and Twin 2 – were born in late summer 2013. They were named Zoey and Zayne, weighed over 3 pounds each, and had delicate tufts of black hair. They were greeted with overwhelming love and relief. It was a huge moment, but even bigger ones were still to come – bone marrow transplants for both girls!
A big sister makes a big decision When it came time to find a bone marrow donor, the twins’ big sister Zachi stepped up. “I really wanted sisters, and they needed my help,” she explains matter-of-factly.
As luck would have it, testing proved that Zachi was a good, but not perfect match to be a bone marrow donor for her two younger twin sisters. Dr. Cuvelier decided that the chance for success outweighed the risk of rejection.
The process by which bone marrow would be taken from Zachi was demonstrated to her using dolls. For a girl afraid of needles, she showed remarkable bravery. The extraction of a litre of bone marrow (enough for both twins) would require two hundred needles being inserted and taken out from the back of her pelvic bone. Two hundred!
“We are very cognizant that parents cannot strongly influence a bone marrow donation on one child in order to save another sibling. It has to be the child’s decision to donate,” says Dr. Cuvelier. After the CancerCare team met with Zachi they were convinced she had made her own decision.
“The day of the procedure Zachi showed up with a smile on her face. You could tell she really wanted to do this for her sisters,” says Dr. Cuvelier.
There is always a risk of a bone marrow recipient rejecting the transplant and becoming very sick. For that reason, CancerCare Manitoba’s Blood and Marrow Transplant team opted to perform the bone marrow transplants one twin at a time.
Zoey’s bone marrow transplant took place on September 30th 2014 – and the waiting began. While doctors waited to see if Zoey would accept or reject the transplant, Zayne continued with blood transfusions and waited her turn.
To everyone’s great relief, the transfusions were successful, and marked a medical first for the pediatrics team in Manitoba, being the first ever in utero transfusions performed for Alpha Thalassemia in the province.
Within four weeks, blood tests would reveal disappointing results. Zoey had rejected her sister’s bone marrow. “It was a very hard day, lots of tears, “recalls Reina. Big sister Zachi also took the news hard, but “we emphasized to her that even though it didn’t take, it was not her fault” says Dr. Cuvelier.
If at first you don’t succeed
As a backup, the Blood and Marrow Transplant team had already identified a perfectly matched unrelated donor from the general public who would be willing to donate bone marrow to the twins. Within four weeks following the rejection of her sister’s bone marrow, Zoey would receive her second bone marrow transplant, this time from this unrelated donor. Enough bone marrow was harvested from the donor for both girls – half was given to Zoey, while the other half was frozen in CancerCare Manitoba’s Cell Therapy Lab for Zayne’s anticipated future transplant. Once again, the family braced themselves for success or failure.
At a time when other parents of infants are marking milestones like ‘first solid food’, Zoey was undergoing her second bone marrow transplant. The date was November 26, 2014. This time, it worked.
One month after the second transplant, test results showed that the Zoey had accepted the transplant. The donor’s bone marrow would start making new healthy red blood cells in Zoey’s body, meaning Zoey would no longer need blood transfusions to stay alive. Zoey has been entirely cured of her Alpha Thalassemia.
Anything she can do
In theory, the pressure should have been eased going into Zayne’s transplant. After all Zayne had the same disorder, the same DNA, the same medical team and the same donor. The reality was – nobody was taking identical results for granted. There were simply too many variables at play.
Success was not guaranteed… but thankfully, is exactly what transpired. Zayne received her bone marrow transplant on April 17, 2015 and is now happily following in the tiny footsteps of her twin sister.
Both Zoey and Zayne are alive, healthy, and doing great. Both girls have accepted their bone marrow transplants, with stem cells from the donor making new and healthy blood for both girls. Zoey has not required a blood transfusion in over 6-months, and Zayne, who was discharged from the Winnipeg Children’s Hospital on May 22, 2015 following her bone marrow transplant, has not needed a blood transfusion since.
“Although both girls will need ongoing follow-up through CancerCare Manitoba, I expect the twins will be healthy into the future. They have both done extremely well,” says Dr. Cuvelier.
It was yet another medical first! Identical twins cured of Alpha Thalassemia through bone marrow transplant.
Reina says the family’s journey through trying times and difficult decisions has brought her and Mark closer. They are both extremely grateful for the excellent care and support they received from CancerCare Manitoba.
As for the future, Mom reports that “Zoey is the dancer, Zayne is the singer, and they take turns being bossy”.
The Manitoba Blood and Marrow
Transplant Program was established in 1991at CancerCare Manitoba, and is one of only 6 centres in Canada that perform pediatric transplants (15-20 yearly). It’s an important component of the care that CancerCare can provide to children with cancer as well as non-cancerous diseases. Without the program, patients and family members would have to travel to Calgary or Toronto for prolonged periods of time.