Sickle Cell Disease (SCD) is an inherited genetic disease in which a person’s red blood cells contort into a sickle shape, causing cells to die early, leaving a shortage of healthy red blood cells. This can lead to infection, fatigue (sickle cell anemia), and blockage of blood flow causing pain (sickle cell crisis), stroke and organ dysfunction. Mortality rate is typically and unfortunately, mid adulthood. Treatments are aimed at minimizing infection and crisis and include daily medications and blood transfusions, but the only known cure is a stem cell transplant.
Rose, the oldest of three children, was born with Sickle Cell Disease to loving parents Joy and Gary in March of 2014. Learning that she had SCD on newborn screening, Joy and Gary sprang into action to do everything in their power to mitigate the risks of Sickle Cell and ensure that Rose lived a long, healthy and productive life. They were conscious of hygiene to combat germs and sickness, avoided sick contacts, and stayed home in the winter months when viruses run rampant and the cold temperatures could cause crisis. These strict measures however came at a cost. Rose didn’t get to do things that healthy children could, such as enjoy preschool, take gymnastics classes, go to waterparks or play in the snow. She was missing out and so were her parents, but the isolation was worth it if it meant keeping her healthy. And she was. Rose was fortunate that in her first few years of life, although she had occasional fevers which were always concerning, she avoided pain crisis and serious infection.
In July 2017 Joy and Gary unexpectedly welcomed their third child George, who by the grace of God was found to be a perfect HLA Match and didn’t inherit Sickle Cell Disease and could serve as Rose’s stem cell donor. Although they were thrilled they could finally offer Rose a chance for cure, the fear of the transplant weighed heavy on them. Several centers in her area were doing transplant for SCD so Joy and Gary researched and interviewed them to find the best fit for them.
The Sickle Cell program, run by Dr. Cairo at Maria Fareri Children’s Hospital stood out far above others. Joy said that once she decided to be treated by Dr. Cairo, she felt at peace. Rose was just 3yrs old when was she was enrolled on a clinical trial using reduced toxicity chemotherapy (to decrease the risk of both short and long-term complications) and matched sibling bone marrow. Everyone they encountered at MFCH really seemed to care about Rose, from the anesthesiologist who was concerned Rose might feel cold in the pre-op area and made sure warm blankets were available, to the custodial staff who were conscientious to be quiet when entering Rose’s room overnight, to the hospitality staff who would include Rose in making her bed daily which helped her muscle strength and morale. Joy said that the nursing staff became her family. They cared for the family, not just Rose and demonstrated both expertise in their care and compassion for the difficult situation the entire family was in.
The transplant was difficult. Rose received 8 days of chemotherapy that made her nauseas, lose her hair, get fevers and feel tired, but she persevered. Her brother, George who was only six months old at the time, received 4 days of injections to stimulate his bone marrow so it could be harvested under anesthesia. Joy states that it was surreal to have both her babies in the hospital at the same time undergoing such serious procedures and at the same time she felt confident that the team at MFCH would see them through successfully.
And they did, the transplant was a success, 3 ½ years later we find Rose a happy and healthy child who now plays with other children, attends gymnastic classes and is cured from Sickle Cell Disease.
Joy and Gary credit the healing power of Jesus Christ for their miracle children and are thankful for the dedication and diligence of the director and staff of the Sickle Cell Program at Maria Fareri Children’s Hospital for making sickle cell disease patients a priority.