September 4, 2018 — Each year around the globe, the third Saturday in September is recognized as World Marrow Donor Day. The main objective of World Marrow Donor Day is to raise awareness about being a stem cell donor and the impact stem cell donation makes on a patient’s life. On Saturday, September 15th you can be sure COTA’s Staruk Family will be sharing the story of how a stem cell donor literally saved their son’s life. Just a week later on September 23rd, the Staruks will be celebrating their son’s three-year stem cell ‘transplantversary.’
Haylee and Tom Staruk of Middletown, Maryland, have travelled a long, stressful and costly transplant journey with their son, Everett, who is their third child. Everett’s older sisters Aurore and Matilda have also been impacted by this journey as well.
When Haylee and Tom brought Everett home from the hospital in September 2014 they were indeed anticipating a hectic ‘new normal’ as they settled in to raise their young daughters and their newborn son, but little did they know ‘normal’ was going to be very different than they ever imagined. When Baby Everett was just four weeks old, Haylee and Tom noticed his head would occasionally tremor. Their pediatrician was not too concerned but they decided to consult a pediatric neurologist just to be certain Everett was fine. The specialist told the Staruks Everett was neurologically immature, which was nothing serious, due to his small size. As a precaution, she sent Everett to have a brain sonogram, which turned out to be ‘normal.’
Then in October 2014 Everett’s eyes began involuntarily oscillating (nystagmus) and the pediatric neurologist ordered an MRI of his brain. Everett was 14 weeks old and after the MRI was done, Haylee and Tom received a call saying everything was ‘normal.’ In February 2015 the Staruk’s pediatrician referred the family to a local Infants & Toddlers program because Everett had poor muscle tone and was failing to reach developmental milestones. So they took him to weekly physical therapy and monthly occupational therapy sessions to help their baby catch up. During one of these sessions, the physical therapist noticed Everett did not reach or track for toys so a concern was raised about his vision.
In April 2015 when Everett was eight months old, a pediatric neuro-ophthalmologist at Johns Hopkins Hospital told them that despite nystagmus and intermittent strabismus (crossed eyes), Everett had ‘normal’ vision. After Haylee told this specialist Everett’s MRI was also ‘normal’ he referred the family to a developmental pediatrician. A month later in May, an assessment of Everett was conducted by a developmental pediatrician at the Kennedy Krieger Institute. The family’s new pediatrician consulted with a pediatric radiologist at Johns Hopkins to provide a second opinion of Everett’s first MRI conducted at 14 weeks. The second opinion of the MRI indicated Everett had a delay in myelination of the brain. Myelin is the insulation that protects nerves and promotes the efficient transmission of nerve impulses. Haylee and Tom immediately knew Everett’s condition was not ‘normal’ and instead was quite serious.
In July 2015 after a second MRI and genetic testing, Haylee and Tom were told Everett had Pelizaeus-Merzbacher Disease (PMD), which is an extremely rare condition impacting the brain and spinal cord. PMD
is caused by a genetic mutation that creates an inability to form myelin in certain areas of the central nervous system. As a result, individuals with this condition have impaired intellectual functions (such as language and memory) and delayed motor skills (such as coordination and walking).
“After learning more about the devastating diagnosis, panic and desperation set in. We began feverishly scouring the Internet for resources, research opportunities and to connect with other families facing the same crisis. We learned about research happening at Duke University Hospital that involved a stem cell transplant using donated umbilical cord blood and immediately sent an email to the coordinating nurse,” Haylee said. Their first conversation with Duke’s Pediatric Bone Marrow Transplant (PBMT) team happened on July 24th and by August 2nd they checked into the Ronald McDonald House of Durham, North Carolina (300+ miles away from their home), to begin Everett’s preliminary testing.
On August 10th after receiving information from a transplant social worker at Duke, Haylee called the Children’s Organ Transplant Association (COTA) to learn more about fundraising for transplant-related expenses. COTA uniquely understands that parents who care for a child or young adult before, during and after a life-saving transplant have enough to deal with, so COTA’s model shifts the responsibility for fundraising to a community team of trained volunteers. On August 21, 2015, the Staruks officially became part of the COTA family, and on September 9, 2015, a COTA fundraising specialist trained volunteers for the COTA campaign in honor of Everett S. COTA is a 501(c)3 charity so all contributions to COTA are tax deductible to the fullest extent of the law, and COTA funds are available for a patient’s lifetime. This group of committed church members, friends, professional colleagues and family members, i.e. COTA Miracle Makers, quickly got to work organizing fundraisers to help with mounting transplant-related expenses.
“Once the COTA fundraising began, the overwhelming outpouring from the community to COTA in honor of Everett provided our family so much hope,” Haylee said. “So many people hundreds of miles away back home were supporting us with their prayers and their gifts to COTA. It was truly difficult to not have hope once we learned how many people cared about Everett and about us.”
After 10 days of preliminary testing, Everett was officially accepted into the transplant study. The team at Duke was very clear with Haylee and Tom that a stem cell transplant would not be a cure for PMD, but would hopefully improve Everett’s quality of life and allow researchers to learn more about this devastating disease. According to Haylee, “Unlike other transplant families, we were given the choice to pursue a transplant. Thankfully, COTA certainly made this choice much easier!”
On September 7, 2015, the family travelled back to Durham to officially begin Everett’s transplant journey. He was officially admitted to the PBMT Unit at Duke University Hospital on September 13th. The isolated unit only allowed for one parent to stay full time; therefore, Tom returned home to Maryland to take care of Everett’s two big sisters while Haylee and Everett settled in. Nine grueling days of chemotherapy meant hair loss, vomiting, nausea and insomnia. September 23rd was Everett’s official stem cell transplant day.
As Everett recovered and his immune function adapted, Everett and Haylee lived in the isolation unit for another 42 days without visitors. Face Time and phone calls were the only ways to keep in touch with Daddy, Aurore and Matilda at home. On November 3rd Everett was finally discharged and allowed to relocate to an efficiency apartment in the Ronald McDonald House nearby. Tom returned to receive training on proper care of a central line, administering medications and sterile cap changes.
Haylee had to return home to Maryland on November 17th to resume her job in order to maintain health insurance for Everett and the family. That meant Tom began taking the lead on Everett’s daily care in Durham. November, December and January consisted of multiple follow-up visits each week to the hospital and living in isolation at the apartment during the rest of the time, but things appeared to be going well. Everett was on track for a ‘normal’ transplant recovery and the Staruks were hopeful the entire family would soon be living under one roof in Maryland.
That hope was not to be realized in the short term. In late January 2016 the PBMT team noticed Everett’s red blood cells were bursting. After several medication changes, Everett’s condition worsened and he needed regular blood transfusions to combat the breakdown of cells and other damage. By March 2016 Everett was officially diagnosed with aHUS, a very rare and serious side effect from his stem cell transplant and he was placed on Ecluzimab. (At $500,000 per year, Ecluzimab is one of the world’s most expensive medications.) The Ecluzimab immediately began working but caused its own series of side effects. Everett needed incredible doses of steroids that made his entire body swell and caused irritability and insomnia. Months passed and Everett was miserable. He vomited multiple times a day and developed an extreme aversion to any food or beverages near his mouth. Haylee started travelling to Durham on the weekends to give Tom a break from the exhaustion caused by Everett’s care.
Eight months later in August, the Staruks were told Everett and Tom could finally return home to Maryland. Everett would still need to live under extreme precautions and isolation, but he had finally recovered enough to be treated at Sinai Hospital in Baltimore.
“The transition home was wonderful and stressful at the same time,” Haylee explained. “No guests were allowed to visit. Big Sisters needed to completely shower every time they returned home from school or playing outside. Any sign of someone feeling ill meant we needed to evacuate our home immediately to keep Everett safe. But our little guy began to thrive again. Seeing his big sisters regularly was very motivating. He would attempt to play with toys and he laughed more often. Everett also began sleeping through the night again.”
Everett was only allowed to leave his home for hospital visits which meant all therapists had to come to the Staruk home. Each therapist had to bring a fresh set of clothing to change before each session so they did not contaminate Everett with germs. Slowly, various medications were able to be weaned and Everett continued to improve. In May 2017 the family returned to Duke for a follow-up visit where Haylee and Tom were told Everett’s immune function had finally returned to normal. Haylee was (and is) realistic about this ‘new normal’ for their family. She says, “Everett will always struggle with movement, cognition and communication. However we feel fortunate, in great part thanks to COTA, that we were able to pursue cutting edge research to provide our son with the best quality of life possible.”
“The Children’s Organ Transplant Association (COTA) has been essential to our lives. COTA funds allowed us to focus on Everett and put his needs first instead of panicking and stressing about money. COTA funds made it possible for us to travel between our home in Maryland and his transplant center in North Carolina to alternate taking care of him. We made that switch 17 times and each time COTA was on the road with us. Since Everett’s diagnosis we have been billed for thousands of dollars in co-pays and prescription medications. What would we have done without COTA? The overwhelming outpouring of support from our community to COTA in honor of Everett gave us so much hope … hope we desperately needed during some very difficult and dark days,” Haylee and Tom said.
Moving forward with Everett’s care will continue to be a challenge for this COTA family. When asked about Everett’s post-transplant care and transplant-related expenses, Haylee shared the following:
Today, Everett is a happy four-year-old boy who loves playing with cars, watching Sesame Street and making a mess. He attends a local special education preschool for a few hours each day and loves riding the school bus. Watching him laugh and play with his sisters brings joy to Haylee and Tom. Most importantly, Everett is alive and thriving – thanks in great part to his stem cell donor, his amazing team of doctors at Duke and a myriad of COTA Miracle Makers.