Evaluation of Individualized Pain Plans for Children With Sickle Cell Disease Admitted for Vaso-occlusive Crisis at Riley Hospital for Children.

OBJECTIVE: Vaso-occlusive crisis (VOC) is the most common problem reported by patients with sickle cell disease (SCD). The objective of this study was to evaluate the impact of individualized pain plans in pediatric patients with SCD admitted for VOC. METHODS: This was a pre- and post-study of patients with SCD admitted to Riley Hospital for Children for VOC from July 1, 2019, through July 1, 2020. The primary outcome was length of inpatient stay for VOC. Secondary outcomes included final pain score, days on scheduled opioids, days on breakthrough opioids, and average morphine milligram equivalents (MME) used per day. RESULTS: Nine patients were included. The mean age was 16 years (range, 10-20 years). Key clinical findings were decreases in median [IQR] for final pain scores (7 [4.5-9] vs 6 [2.5-8], p = 0.396) and number of days of breakthrough opioid use (5 [3-8] vs 4 [2.5-5.5], p = 0.233). Following implementation of an individualized pain plan, there was an increase in median average MME per day (65.94 [53.1-97.7] vs 82.85 [41-114.3], p = 0.844). Median length of stay and days on scheduled opioids remained the same. CONCLUSIONS: This study demonstrated that use of individualized pain plans in a small population of patients with SCD might result in decreased pain scores and decreased days on breakthrough opioids.

Maintenance of an Immunogenic Response to Pneumococcal Vaccination in Children With Sickle Cell Disease.

Patients with sickle cell disease (SCD) are at increased risk for invasive pneumococcal disease because of splenic dysfunction. To mitigate this risk, patients are protected with prophylactic penicillin until completion of pneumococcal vaccination series. The objective of this study was to assess the maintenance of a protective immune response to pneumococcal vaccination in children with SCD. A retrospective review was conducted between June 2019 and June 2020 of all patients with SCD patients for whom it had been 5±1 year since completion of PPSV23 vaccination series. A total of 41 patients were analyzed. The majority of children (68%) were able to maintain an adequate immune response. There was no identifiable disease characteristic associated with maintenance of an appropriate immunogenic response. This study finds that patients with SCD are able to maintain an adequate immune response at the 5±1 year time point from completion of PPSV23 vaccination series. Similarly, patients were not found to have an increased rate of invasive pneumococcal disease even if not meeting criteria for adequate pneumococcal serum titer levels. Maintenance of pneumococcal titers suggests that there may not be a need for revaccination at the 5-year time point in this patient population.

Safety of Extended Interval Tobramycin in Cystic Fibrosis Patients Less an 6 Years Old.

OBJECTIVES: The primary objective of this study was to assess the nephrotoxicity and ototoxicity risks of extended interval tobramycin in cystic fibrosis (CF) patients who are <6 years old. A secondary objective included analyzing pharmacokinetic parameters in this age group. METHODS: A retrospective chart review was conducted of patients with CF who were <6 years old, admitted for an acute pulmonary exacerbation from January 1, 2003, to January 1, 2014, and treated with intravenous tobramycin. RESULTS: The median baseline serum creatinine (SCr) was 0.26 mg/dL among the 31 patients included in the study. Of the 20 patients who experienced increases in SCr, the absolute median increase was 0.065 mg/dL (0.033-0.1 mg/dL). Abnormal audiograms seen in 4 patients are attributable to middle ear effusion present on exam. The median dose of tobramycin was 11.7 mg/kg (11.3-12 mg/kg), the elimination constant was 0.4 hr-1(0.32-0.47 hr-1), half-life was 1.7 hr (1.5-2.1 hr), volume of distribution was 0.37 L/kg (0.31-0.47 L/kg), and median peaks and troughs fell within ranges of 20 to 30 mg/L (20.9-32.7 mg/L) and <0.01 mg/L, respectively. CONCLUSIONS: Extended interval dosing tobramycin is safe in CF patients who are <6 years old. There was no drug-related ototoxicity, and some nephrotoxicity was observed. When dosed at 12 mg/kg, similar pharmacokinetics were seen among all age groups, and concentrations were within the desired range.