There are no guidelines on sickle cell patient referral to pulmonologists, associated outcomes, and best therapeutic interventions.
Current evidence shows that children with SCD are more likely to be diagnosed with asthma than patients without sickle cell. Even more, an asthma diagnosis has been associated with increased incidence of acute chest syndrome, vaso-occlusive episodes, pulmonary hypertension, stroke, and early mortality.
“Optimizing asthma management in SCD is essential in reducing morbidity for children with this lifelong disease, and early recognition and prevention of asthma may ultimately improve quality of life and survival in children with SCD,” the investigators wrote.
For children living with sickle cell disease, integrated care involving a variety of specialists may help optimize outcomes and quality of life for these patients. However, guidelines regarding patient referral to pulmonologists and associated outcomes are currently lacking.
A team led by Shikha Saxena, MD, of Monroe Carell Junior Children’s Hospital at Vanderbilt, conducted a single-center retrospective cohort study that aimed to evaluate associations between care by a pulmonologist and asthma and acute chest syndrome exacerbations.
The team followed a total of 192 patients with sickle cell disease between the ages of 0 – 21 years. All patients were admitted to the Children’s Hospital of Philadelphia Hematology and were simultaneously diagnosed with asthma, wheeze, or cough.
The investigators grouped the population into treatment 2 cohorts: 70 patients were evaluated by a pulmonologist and 122 remained on standard of care. Patients who were in the pulmonary cohort had at least one outpatient evaluation in the hospital’s Division of Pulmonary and Sleep Medicine during the course of the study period.
As such, the primary outcome sought by the investigators was the rate of emergency department visits and inpatient admissions due to asthma exacerbation, albuterol-responsive cough/wheeze, and ACS.
Saxena and team compared average rates of visits and admissions between the cohorts, as well as well within the pulmonology cohort prior to and following randomization.
They noted that the overall mean number of pulmonary visits for the cohort was 3.14 with mean follow-up being 2.88 years. Follow-up was comparable with the non-pulmonary cohort (2.89).
Patients who saw a pulmonologist were more likely be prescribed short acting beta agonists (albuterol or levalbuterol) (P = .004), preventative asthma medications (inhaled corticosteroid, combination inhaled corticosteroid and long acting beta agonist, or montelukast) (P<.001), and hydroxyurea (P = .008). They also had higher rates of completion for ACT, ACP, and PFTs (P<.001).
A total of 96 patients had at least 1 hospital visit, with exactly half from each cohort. A higher proportion of patients in the pulmonary cohort had visits for asthma (P = .007) and acute chest syndrome (P<.001).
Further, 48 pulmonary patients had at least one visit to the emergency department for asthma exacerbations albuterol-responsive cough/wheeze, or acute chest syndrome.
“The average rate of hospital visits per year for these patients decreased from 3.93 [95% CI, 1.57-6.29] before the initial pulmonary visit to 0.85 [95% CI, 0.48-1.23] after the initial pulmonary visit (P = .014),” the investigators noted. Even more, 10 of the patients with the highest rate of admissions prior to the study saw marked decreases following pulmonary visit.
The rate ratio between pre-pulmonary visit to post-pulmonary visit was 0.40, thus indicating that hospital visits following pulmonary visits was 0.40 times the rate prior to visit.
“In conclusion, this study supports the role of the pulmonologist in the multidisciplinary care of children with SCD and asthma,” Saxena and colleagues wrote. “Evaluation and management of children with SCD and asthma by a pulmonologist was shown to improve outcomes, as demonstrated by more standardized asthma care and decreased rates of ED visits and hospitalizations for asthma exacerbation and ACS.”
However, additional studies are needed to better understand the benefits of specific therapeutic interventions that may help establish evidence-based guidelines for optimizing pulmonary outcomes in children with SCD.
The study, “Benefit of Pulmonary Subspecialty Care for Children with Sickle Cell Disease and Asthma,” was published in Pediatric Pulmonology.