Phase 2 trial of montelukast for prevention of pain in sickle cell disease.

Cysteinyl leukotrienes (CysLTs) are lipid mediators of inflammation. In patients with sickle cell disease (SCD), levels of CysLTs are increased compared with controls and associated with a higher rate of hospitalization for pain. We tested the hypothesis that administration of the CysLT receptor antagonist montelukast would improve SCD-related comorbidities, including pain, in adolescents and adults with SCD. In a phase 2 randomized trial, we administered montelukast or placebo for 8 weeks. The primary outcome measure was a >30% reduction in soluble vascular cell adhesion molecule 1 (sVCAM), a marker of vascular injury. Secondary outcome measures were reduction in daily pain, improvement in pulmonary function, and improvement in microvascular blood flow, as measured by laser Doppler velocimetry. Forty-two participants with SCD were randomized to receive montelukast or placebo for 8 weeks. We found no difference between the montelukast and placebo groups with regard to the levels of sVCAM, reported pain, pulmonary function, or microvascular blood flow. Although montelukast is an effective treatment for asthma, we did not find benefit for SCD-related outcomes. This clinical trial was registered at www.clinicaltrials.gov as #NCT01960413.

Improving Medication Adherence with Two-way Short Message Service Reminders in Sickle Cell Disease and Asthma. A feasibility randomized controlled trial.

INTRODUCTION: Sickle cell disease (SCD) is a childhood and adult disease that primarily affects African Americans, characterized by life threatening sequelae mitigated by medications. One-way and two-way short message service (SMS) medication reminders have differing efficacy in chronic diseases. There is limited literature about SMS medication reminders in SCD. OBJECTIVE: The goal of this study was to test the feasibility, defined by recruitment/acceptance, retention/attrition, and technology utilization, of two-way SMS medication reminders in individuals with SCD with and without asthma. MATERIALS AND METHODS: Participants were randomly allocated to standard care or reminders. Two-way SMS reminders were automated using Research Electronic Data Capture (REDCap) for hydroxyurea, fluticasone, budesonide and montelukast. Adherence was measured using the Morisky Medication Adherence Scale-8 (MMAS-8). Asthma control was assessed using the Childhood and Adult-Asthma Control Tests (ACT). Participants were enrolled 28 to 60 days with a common termination date. RESULTS: The recruitment rate was 95% (47/49) and 82.9% completed the study. Among the 47 study participants enrolled, 51.1% were male, 61.7% were adults, median age was 20 (range: 3 to 59), and 98% were African Americans. Of the 26 participants receiving messages, 20% responded on over 95% of the days and usage varied with an average response rate of 33%, ranging from 21% to 46%. Medication adherence scores improved significantly in the intervention group (3.42 before, 5.46 after; p=0.002), but not in the control group (3.90 before, 4.75 after; p=0.080). Childhood-ACT scores improved in the intervention group (19.20 before, 24.25 after). Adult-ACT scores within the intervention arm were unchanged (21.0 before, 22.0 after. ACT scores did not improve significantly. CONCLUSION: This study demonstrated the feasibility for two-way SMS medication reminders to improve medication adherence in a high-risk population where daily medication adherence is critical to health outcomes and quality of life.

Improved Guideline Adherence With Integrated Sickle Cell Disease and Asthma Care.

INTRODUCTION: In children with sickle cell disease (SCD), concomitant asthma is associated with increased morbidity and mortality when compared with children with SCD without asthma. Despite the well-established burden of asthma in children with SCD, no paradigm of care exists for the co-management of these two diseases. METHODS: To address this gap, an integrated SCD and asthma clinic was created in a community health center that included (1) a dual respiratory therapist/asthma case manager; (2) an SCD nurse practitioner with asthma educator certification; (3) an onsite pulmonary function test laboratory; (4) a pediatric hematologist with expertise in managing SCD and asthma; and (5) application of the National Asthma Education and Prevention Program guidelines. A before (2010-2012) and after (2013-2014) study design was used to assess for improved quality of care with implementation of an integrative care model among 61 children with SCD and asthma followed from 2010 to 2014. RESULTS: Asthma action plan utilization after initial diagnosis increased with the integrative care model (n=16, 56% before, 100% after, p=0.003), as did the use of spirometry in children aged ≥5 years (n=41, 65% before, 95% after, p<0.001) and correction of lower airway obstruction (n=10, 30% before, 80% after, p=0.03). CONCLUSIONS: Although the use of an integrative care model for SCD and asthma improved evidence-based asthma care, longer follow-up and evaluation will be needed to determine the impact on SCD-related morbidity.