Teen Pregnancy in Sickle Cell Disease Leads to Early Transition
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A 17-year-old female patient with sickle cell disease (SCD; HbSS) and a medical history significant for acute chest syndrome and multiple recent hospital admissions for vaso-occlusive pain episodes presented to the emergency department (ED) with leg pain. Her mother was at work, so the patient was accompanied to the ED by her older sister. During the visit, the patient mentioned to the ED staff that she was a week late for her menstrual period. Her Toradol was stopped and her blood was drawn for testing. Her serum β-human chorionic gonadotropin (HCG) levels were elevated, and the ED team notified the patient that she was pregnant. She was admitted to the pediatric hematology/oncology unit for pain management; however, the team worked diligently to transfer the patient to the adult hospital since she was no longer considered a “pediatric patient.” The patient was then transferred to the adult hospital, and her mother later arrived to meet the new care team.
The Transition From Pediatric to Adult Care
What happens to pediatric patients with SCD who transition early to adult care due to pregnancy? The literature suggests that poor transition from pediatric to adult care negatively affects outcomes for patients with SCD, including increased ED visits and hospitalizations.1,2 Many factors have been thought to contribute to the poor transition, including sociodemographic factors (e.g., poverty, parental education, low access to adult providers) and developmental maturity (e.g., navigating lapses in insurance and articulating pain symptoms during a crisis).1 During the years of transition, patients with SCD are considered one of the most medically vulnerable populations as discussed by Dr. Jeffrey Lebensburger and colleagues; their work demonstrated that many of the deaths occurred within two years of transition to adult care, suggesting an increase in morbidity and mortality during this period.3 These patients were 18 to 23 years old, but what are the effects on patients whose care is transferred earlier and/or suddenly due to pregnancy?
This is an important issue that the hematology community should be considering more often. Studies have shown that much like their healthy peers, adolescent patients with chronic disease do engage in sexual activity. One study noted that about 40 percent of adolescent patients with conditions such as diabetes mellitus, cerebral palsy, and juvenile arthritis report sexual activity.6,7 Most of the adolescents reported first intercourse around the ages of 13 to 14 years.6,7 A multicenter study conducted by Dr. Maria T. Britto and colleagues evaluated sexual behaviors of patients with SCD and cystic fibrosis who identified as female, with a median age of 16 and a half years.2,4 Fifty-one percent of the patients with SCD reported having sexual intercourse, yet reproductive health education for adolescent girls with SCD is neither universal nor standardized in pediatric SCD programs.2,4,5
Most would argue that having the patients with SCD transition to adult care would be the most appropriate choice to ensure the patient's best chance at a healthy pregnancy and successful and safe delivery. Pregnant women with SCD have an increased risk of pain crises, mortality related to sepsis and thromboembolic events, pulmonary complications, pre-eclampsia, pre-term delivery, and still birth.7,8 It is therefore important for the patient to get care from a multidisciplinary team consisting of an obstetrician and gynecologist (OB/GYN), maternal-fetal medicine specialist, intensivist, and transfusion medicine team, often under the coordination of an adult SCD provider. A study conducted by the National Committee on Maternal Mortality showed that most maternal deaths in pregnant women with SCD in France were due to nonobstetric causes, and 40 percent of deaths were deemed preventable if multidisciplinary care had been provided.8,9 In the United States, women with SCD are 16 times more likely to die within the first year of pregnancy.9,10 Moreover, there is a paucity of adult SCD providers; therefore, most care for our patients with SCD occurs in the community. Studies show that only one-third of general internists express comfort with managing patients with SCD, and most general internists do not believe that they should be the primary provider for these patients.2 Complicating the situation further, infant and maternal mortality rates among women of color are higher nationally in comparison to all other pregnant women. This is thought to be the result of several factors, including racial discrimination and unconscious bias.7,8,10,11 Where do our adolescent patients fit into this problem and how can the hematology community support them?
The research evaluating SCD and pregnancy is limited and requires further investigation.7,8 There is a paucity of literature describing best practices for early transition of patients with SCD due to pregnancy as well as other rare chronic diseases (e.g., cystic fibrosis). However, the literature indicates that adult providers have expressed discomfort treating adolescents with chronic childhood illness, and this may be exacerbated by an abrupt transition of patients with a high level of medical complexity, including pregnancy.12 Devising hospital policies surrounding a bridged transition period during which the patient can be co-managed with an OB/GYN, maternal-fetal medicine, a pediatric SCD provider, and an adult SCD provider might prove useful by improving the chance of a safe delivery for both mother and baby while preventing an abrupt transfer of care that may ultimately be detrimental to the patient with SCD. Data regarding safe transition in SCD recommends that transition plans be patient-centered and flexible.13 Health care providers on both ends of the transition spectrum must also be educated on the importance of successful transition and work together to plan for unforeseen events (e.g., unplanned pregnancies).12 Our goal should be to ensure all individuals with SCD have a safe transition to adult care, even if that transition is earlier than expected.
Patient-Focused Questions to Answer:
- What message do we send to the pediatric patient (pregnant with SCD) when we immediately transfer them to adult care after learning about their pregnancy?
- When patients in this situation do not have a support system, whose responsibility is it to advocate for them?
- Stollon NB, Paine CW, Lucas MS, et al. Transitioning adolescents and young adults with sickle cell disease from pediatric to adult health care: Provider perspectives. J Pediatr Hematol Oncol. 2015;37:577-583.
- Brousseau DC, Owens PL, Mosso AL, et al. Acute care utilization and rehospitalizations for sickle cell disease. JAMA. 2010;303:1288-1294.
- Lebensburger JD, Bemrich-Stolz CJ, Howard TH. Barriers in transition from pediatrics to adult medicine in sickle cell anemia. J Blood Med. 2012;3:105-112.
- Britto MT, Garrett JM, Dugliss MA, et al. Risky behavior in teens with cystic fibrosis or sickle cell disease: a multicenter study. Pediatrics. 1998;101:250-256.
- Leroy-Melamed M, Jacob S, Shew ML, et al. Provider attitudes, preferences, and practices regarding sexual and reproductive health for adolescents and young adults with sickle cell disease. J Adolesc Health. 2021;69:970-975.
- De Sanctis V, Soliman A, Mohamed Y. Reproductive health in young male adults with chronic diseases in childhood. 2013;10:284-296.
- Surís JC, Resnick MD, Cassuto N, et al. Sexual behavior of adolescents with chronic disease and disability. J Adolesc Health. 1996;19:124-131.
- Smith-Whitley K. Complications in pregnant women with sickle cell disease. Hematology Am Soc Hematol Educ Program. 2019;2019:359-366.
- National Expert Committee on Maternal Mortality. Maternal deaths in France: Improving knowledge for improved prevention [French]. Report of the National Expert Committee on Maternal Mortality. 2007-2009.
- Alayed N, Kezouh A, Oddy L, et al. Sickle cell disease and pregnancy outcome: population-based study on 8.8 million births. J Perinat Med. 2014;42:487-492.
- Pregnancy-related deaths: Data from 14 U.S. Maternal Mortality Review Committees, 2008-2017. Centers for Disease Control and Prevention.
- Vaks Y, Bensen R, Steidtmann D, et al. Better health, less spending: Redesigning the transition from pediatric to adult healthcare for youth with chronic illness. Healthc (Amst). 2016;4:57-68.
- Jordan L, Swerdlow P, Coates TD. Systematic review of transition from adolescent to adult care in patients with sickle cell disease. J Pediatr Hematol Oncol. 2013;35:165-169.