Search

Background: Pain is the most common complication of sickle cell disease (SCD), and pain diaries are an effective way to capture pain experienced by both children and adults for many conditions, including SCD. The most recent data on pain experienced by children with SCD outside the clinical trial setting, particularly those with Hemoglobin (Hb) SS disease, were collected prior to the publication of the Baby-HUG trial in 2011, which found hydroxyurea to be safe for use in early childhood. One study found that school-aged children with SCD had at least one pain episode per month; another longitudinal study reported that SCD-related pain was experienced by children on 8% of pain diary days. In the era of hydroxyurea use in children, the occurrence of SCD-related pain episodes in children has not been well studied, especially among those with Hb SS disease. Methods: Parent/child dyads were recruited for a 6-month pilot study of the use of home pain plans and pain diaries from a pediatric hematology clinic based in a safety net institution. The study ran from February to November 2014. Dyads were eligible if the child with SCD was aged 5-15 years and had at least one episode of pain in the year prior to enrollment, by parent report. Parents and children were asked to fill out the diary each day. They received weekly phone calls for the first month, then monthly phone calls until the close of the study, to remind them to complete the daily pain diary and mail it back monthly; $20 gift cards were given for each diary returned. We excluded data from dyads that recorded less than 4 weeks of pain diary data. Results: Thirty-six parent/child dyads were approached and 30 (83%) consented to enroll in the study. Twenty-six (87%) dyads recorded at least four weeks of data. The average age of the child with SCD was 10.3 ± 3.0 years and 20 (77%) were insured through Medicaid. Twenty-two (85%) children had Hb SS disease, of whom 21 were prescribed hydroxyurea. Data were recorded per dyad on average 135 ± 46 diary days. One child with Hb SS disease reported no pain during the study period. The remaining 25 children had any pain and SCD-related pain an average of 8% and 4% diary days, respectively. Nearly all children reporting non-SCD-related pain had headache or abdominal pain. Ten (38%) children missed at least one day of school during the study period. Conclusions: In thissample of children with SCD, composed predominantly of those with Hb SS disease prescribed hydroxyurea, pain was an infrequent occurrence and was attributed to SCD only half the time. This study suggests that SCD-related pain experienced by children may be lower than previously reported. Further study is warranted, especially among those prescribed hydroxyurea. Disclosures No relevant conflicts of interest to declare.

Background: Vaso-occlusive episodes (VOE) are the leading cause of hospitalizations & emergency department (ED) visits in sickle cell disease (SCD), & are associated with increased mortality. Intranasal fentanyl (INF) provides rapid & powerful parenteral analgesia, with an onset of action of 5-10 minutes, & peak effect in 30 minutes. INF is a safe & effective method of pain management for children in the ED & other settings, yet it is underutilized in SCD. Objective: To study the impact of the addition of INF to standard ED management for SCD VOE on time to parenteral opioid, pain scores, ED length of stay (LOS) & admission rate. Methods: This quality improvement initiative was conducted at a tertiary pediatric ED at Children's Healthcare of Atlanta from November 2013-May 2014. Patients with all genotypes of SCD ages ≥2 years old who presented with VOE & pain score >6/10 were offered INF within 15 minutes of triage. 2 INF doses (1.5mcg/kg/dose; max 100mcg/dose) were given 5 minutes apart. Patients then continued on institutional standard protocol treatment for VOE (±PO hydrocodone, ±IV ketorolac, & ± IV morphine or IV hydromorphone). Pain scores were documented at frequent intervals by nursing staff. Patient/parent satisfaction data were obtained using a satisfaction questionnaire. Outcomes included: Time from arrival to 1st parenteral opioid, pain scores, ED LOS (time from arrival to disposition), admission rates & patient/parent satisfaction. We compared patients in this study to those who did not receive INF during the study period (n=48) & historical controls from Jan-Dec 2012 (n=231) for the 1st three outcomes. Results: 248 visits for VOE met inclusion criteria, of whom 228 received parenteral opioids (92%) & 180/228 (79 %) received INF. Mean age was 11.7±4.5 years. The majority were female (56%), 65% HbSS, 14% HbSC & 21% had other genotype. 48 patients did not receive INF; 36 were not offered INF without explanation for protocol deviation &12 patients refused. They had similar gender & Hb genotype, but were older than INF+ patients (13.5±4.0 years, p=0.01). The mean time from arrival to 1st parenteral opioid decreased significantly in the INF+ group compared to historical controls, and was remarkably shorter than those not treated with INF. There was minimal difference in LOS between INF+ group & historical controls, but LOS was shorter in the group that did not receive INF. Admission rates were significantly higher in those who did not receive INF when compared to those treated with INF & historical controls. (See Table 1) Table 1 INF+(n =180) INF-(n =48) Historical Data (n=231) P-value Time to 1st Parenteral Opioid (minutes) 29 ±15 77 ±44 35±18 p < 0.0001 LOS (minutes) 215± 86 197± 67 231±95 p = 0.028 Admission Rate (%) 48% 71% 45% p = 0.004 Mean baseline pain score was 8.5±1.5, 1st reassessment pain score after INF was 7.8±2.4 & last ED pain score prior to disposition was 5.5±3.4. Pain scores were similar in all groups. 98 families completed the patient satisfaction questionnaire, 79 (81%) of whom received INF. 65% of patients who received INF were satisfied & would like to receive the treatment again in the ED. Conclusions: The addition of INF to the management of SCD VOE significantly improved time to receipt of 1st parenteral analgesia & was well tolerated. Admission rates were significantly higher in patients who did not receive INF during our study period. The associated delay in time to receipt of 1st parenteral analgesia may have contributed to the increased admission rate. However, causality of INF on admission rates cannot be determined without further study. INF did not impact ED LOS compared to historical controls, however rapid admission turn-around time likely decreased LOS in the INF- group. INF is a safe & effective strategy to provide rapid pain relief in children with SCD & VOE. Disclosures Off Label Use: Intranasal Fentanyl (INF). Widely accepted as off label use in pediatric ED's and a variety of other clinical settings for the management of pain. It is a synthetic narcotic analgesic with a rapid onset and short duration of action. Current evidence suggests that INF is a safe and effective method of pain management for children in a variety of clinical settings..