ABSTRACT
BACKGROUND: The epidemiology of painful episodes in infants and younger children with SCD has not been well studied, particularly for pain managed at home. PROCEDURE: SCD infants identified by newborn screening were enrolled in a longitudinal observational study of pain symptoms requiring parents to report the presence or absence of pain daily. When sickle cell related-pain events occurred, pain occurrence, location, associated symptoms and the treatment provided also were reported. RESULTS: 103 children were enrolled at a median age of 7.2 months; 50 had an SS genotype, 32 SC, 6 SB(0)thalassemia, and 15 SB(+)thalassemia. Parents/guardians reported for a median of 3.8 years (range 0.3-7.6 years) assessing pain for a total of 141,197 days, excluding any period of recurrent transfusions, with an additional 28,079 days of missing data (16%). Children had pain reported on 2,288 days (1.6%), representing 768 distinct episodes of pain, of which 108 required hospitalizations (14%). Pain locations and symptoms consistent with dactylitis were most prevalent (80%) in the 0-12 month age group, and became progressively less prevalent thereafter. Group-based trajectory modeling of pain episode or pain day frequency identified several trajectory groups with progressively older ages of peak pain frequency, which included 40-45% of SS/SB(0)thalassemia and 10-12% of SC/SB(+)thalassemia children. CONCLUSIONS: Pain is relatively infrequent in SCD infants and young children and commonly managed at home. Analyses of longitudinal pain trajectories suggest several different pain trajectories, differing in their frequency, age of onset, and age at peak pain frequency with clinical implications for hydroxyurea management.
Subject(s)
Anemia, Sickle Cell/complications , Pain/etiology , Age Factors , Anemia, Sickle Cell/therapy , Child, Preschool , Female , Follow-Up Studies , Health Services , Hospitalization , Humans , Hydroxyurea/therapeutic use , Infant , Longitudinal Studies , Male , Pain/diagnosis , Pain/drug therapy , PrognosisABSTRACT
OBJECTIVE: To characterize polysomnographic (PSG) findings of children with sickle cell disease (SCD) suspected of having sleep disordered breathing (SDB). METHODS: Families of 100 consecutively referred children with SCD completed the Children's Sleep Habit Questionnaire during a routine visit to identify concerns regarding sleep habits and sleep behavior. Of these, 48 children were identified as displaying behaviors suspicious of SDB. Nineteen agreed to an overnight PSG. The results from the PSGs of the SCD with obstructive sleep apnea syndrome (OSAS) group (SCD-OSAS; group 1) were compared with the results of 10 age, sex, and ethnicity-matched patients identified as OSAS with no medical comorbidities (uncomplicated OSAS; group 2). RESULTS: SDB was identified in 79% of the SCD group. As compared with the uncomplicated OSAS group, the SCD with OSAS group displayed nocturnal desaturation with lower nadir values, of longer duration, with a 4-fold increased risk for oxygen desaturation below 85%, higher percentage of total sleep time with end-tidal carbon dioxide (ET CO2) values >50 mm Hg, with a 3.7-fold increased risk for spending more than 25% of total sleep time with ET CO2 more than 50 mm Hg and higher peak ET CO2 with a 7-fold increase for peak ET CO2 above 53 mm Hg. CONCLUSIONS: Children with SCD suspicious of SDB may have not only a higher incidence of OSAS, but also more severe nocturnal desaturation and hypercapnia as compared with children with uncomplicated OSAS.
