WRNMMC, Bethesda, MD –
The John P. Murtha Cancer Center at Walter Reed Bethesda hosted the 2021 Childhood Blood and Cancer Summit Sept. 23 at the military medical center.
This year’s summit focused on sickle cell advances, transitions of sickle cell pediatric patients to adult care, and disparities in sickle cell care regionally and globally.
September is Sickle Cell Awareness Month, as well as Childhood Cancer Awareness Month. In previous years, the summit addressed advances in the diagnosis, treatment and care of childhood cancer, which affects approximately 1 in 285 children in the United States before their 20th birthday, according to the National Cancer Institute, a part of the National Institutes of Health. Each year in the United States, an estimated 15,600 children 19 and younger are diagnosed with cancer, and globally, more than 300,000 children under 19 are diagnosed with cancer annually, the NCI reports.
Sickle cell disease (SCD) also greatly impacts people, affecting an estimated 100,000 Americans. It occurs in approximately 1 out of every 365 black or African-American births, according to the Centers for Disease Control and Prevention (CDC). Additionally, SCD occurs in about 1 out of every 16,300 Hispanic-American births, the CDC also reports.
An estimated 1 in 13 black or African-American babies are born with sickle cell trait (SCT), the CDC adds. Many people don’t know they have SCT because the trait usually does not cause illness, but those with it can pass the trait on to their children. If both parents have SCT, there is a 25 percent (or 1 in 4) chance that any child of theirs will have SCD, characterized by periods of severe pain caused by sickle-shaped red blood cells, rather than healthy round red blood cells.
In someone who has SCD the red blood cells become hard, sticky, and look like the C-shaped farm tool called a sickle. The sickle cells die early, which causes a constant shortage of red blood cells. This makes blood transfusions vital to those with SCD. Also, when sickle cells travel through small blood vessels, they get stuck and clog the blood flow, which can cause pain, infection, acute chest syndrome and stroke.
During the summit at Walter Reed Bethesda, Dr. Andrew Campbell discussed the inequities in sickle care regionally and globally, and strategies to close the gap. An associate professor for the Department of Pediatrics at George Washington School of Medicine, Campbell explained therapy for sickle cell relies on early screening to identify whether the child is born with sickle cell trait or disease, and although sickle cell is the most common inherited blood disorder worldwide, access to screening is a critical disparity in global sickle cell treatment, especially in sub-Saharan Africa.
Campbell has stated although SCD greatly impacts those in the United States, its burden is most felt in countries outside of the states, with most cases occurring in sub-Saharan Africa. There, most medical centers lack the resources needed to care for the disease. He said there is a need for international teamwork to address the global burden of pediatric disease.
In addition to Campbell, other presenters during the summit, including those with the SCD, explained the disease affects a disproportionate number of people from minority ethnic groups in the United States, some who struggle with poverty, lack of insurance and access to care. Also, they explained SCD patients many times rely on emergency departments for care during a pain crisis, and because opioid medications are often needed to treat pain, adolescent and adult patients are frequently stigmatized as drug seekers, even when they disclose their sickle cell condition.
Campbell stated more can be done in the United States to improve the experiences of sickle cell patients and decrease the inequities of health care in the United States.
“Sickle cell is a disease of health-care disparities, in terms of being under-researched, poorly understood, and the lack of appropriate therapies,” Campbell stated. He encouraged those pursuing medicine to “make a difference, go into public health to be part of the stakeholders who play a role in improving access to care for SCD patients.”
Dr. Caterina Minniti also served as a keynote speaker during the summit. She discussed the “growing pains” of those with SCD, explaining some of the challenges pediatric SCD patients face when transitioning to adult care. Those challenges can include loss of medical home care, decrease in access to ambulatory services and hematology specialists with an interest or expertise in SCD, loss of insurance, loss of an adult caregiver, worsening of the disease, and worsening of stigma.
“Health deterioration is multifactorial during care transition for young adults with SCD,” Minniti explained. She added this results in increased morbidity and mortality. “It’s not the patient, but the system that needs to change,” she added.
Stacee Springer, a clinical social worker at Walter Reed Bethesda who works closely with pediatric beneficiaries and their families, explained one of the most important aspect of a pediatric patient successfully transitioning to adult care, is identifying an appropriate provider. She said this should involve discussion with the young adult about their expectations of a new provider, health insurance needs, and identifying a provider who is accessible and willing to follow the young adult.
Minniti added health care during and after transition from pediatric to adult care should be “flexible, responsive, developmentally appropriate, comprehensive, coordinated, and [possessing] continuity.”
Springer, and Army Col. (Dr.) Dina Parekh, a pediatric hematology/oncology physician at Walter Reed National Military Medical Center, discussed the sickle cell comprehensive care program at WRNMMC. The program includes a multi-disciplinary clinic with annual visits for beneficiaries 2 to 26 years of age with all phenotypes. Those 2 and under, may be seen more frequently by the clinic. The annual visits includes a number of screenings including retinopathy, transcranial Doppler, proteinuria, pulmonary, echocardiograms, neurocognitive, high-risk immunizations, behavioral health and age-specific transition education and counseling.
Parekh said the program is tailored to the needs of beneficiaries, focused on the patient and their family, and includes input from the physicians, nurses, the clinical social worker, and administrator. Those also contributing to the care may include an art therapist, physical therapy, the patient’s school, and palliative care professionals. Springer added communication among team members is vital to the success of the program.
Paige Cephas, who has SCD and transitioned out of the Military Health System to adult care, said, “I knew when I came here [as a SCD pediatric patient], everything would be taken care of. I got everything I needed without going to various parts of the hospital.” She explained now one of her biggest challenges as an adult with SCD, is trying to keep up with the various medical appointments she has to maintain to stay on top of her care.
Regarding how the COVID pandemic has impacted sickle cell care, Campbell said many SCD patients “hunkered down at home,” afraid to come to the hospital for fear of COVID exposure. This was also the case for possible blood donors, as well as those needed for transfusion therapy. He said it also appeared sickle cell patients were less inclined to seek emergency care for pain episodes for fear of possible exposure in emergency departments.
Concerning the COVID vaccination, Campbell said during the spring, there appeared to be a slump, but right before schools started, there was a significant uptick those getting vaccinated, including the parents of SCD pediatric patients.
“The complications from the vaccine are really low,” Minniti said. “We don’t see any more complications among the SCD population that is different than we see in the general population,” she said.
For more information regarding sickle cell disease, visit the CDC site at https://www.cdc.gov/ncbddd/sicklecell/index.html.