Supriya Jambhekar

Predictors and Outcome of Low Initial Forced Expiratory Volume in 1 Second Measurement in Children with Cystic Fibrosis

OBJECTIVE To identify the characteristics of children with cystic fibrosis with low initial forced expiratory volume in 1 second (FEV1) % predicted and to investigate their outcome. STUDY DESIGN Patients were categorized into low or high initial FEV1 groups using cluster analysis. Comparisons of the demographic and clinical data were performed between the 2 groups. RESULTS From 122 children, 21 clustered into the low and 101 into the high FEV1 group. The mean FEV1 was 69% ± 12% predicted for the low and 95% ± 12% predicted for the high FEV1 group (P < .001). The low FEV1 group had lower body mass index percentiles (P = .003), were hospitalized more frequently (P = .001), and had been on dornase alfa longer (P = .006). Low FEV1 group had more patients with positive cultures for Pseudomonas aeruginosa (P = .002) and Stenotrophomonas maltophilia (P < .001) and had more total number of cultures positive for mucoid P. aeruginosa (P = .009) and methicillin resistant Staphylococcus aureus + P. aeruginosa (P = .005). The low FEV1 group continued to have low FEV1 measurements, their FEV1 declined slower, required more hospitalizations per year (P = .01), and had more cultures for mucoid (P = .003) and nonmucoid P. aeruginosa (P = .02) ± methicillin resistant S. aureus (P = .002) in comparison with the high FEV1 group. Poor adherence was associated with lower initial FEV1 values in females, and early, rapid decline of FEV1 in males. CONCLUSIONS Some children with cystic fibrosis may present with poor lung function early in life and continue to have subnormal lung function associated with reduced body mass index, more frequent hospitalization, and higher rates of infection. Such children may benefit from careful evaluation and close follow-up.

Role of a Respiratory Therapist in Improving Adherence to Positive Airway Pressure Treatment in a Pediatric Sleep Apnea Clinic

BACKGROUND: Many pediatric patients need positive airway pressure (PAP) for treatment of obstructive sleep-disordered breathing. Adherence to PAP (defined as percent of nights with PAP use of > 4 h) is often poor and not sustained long-term. With any chronic disease, education has been shown to help with patient outcomes. Education of patients and parents regarding PAP can be provided by different healthcare professionals. There is no published literature assessing the role of respiratory therapists (RTs) in improving adherence to PAP in children. We hypothesized that the addition of RT visits to a PAP clinic would improve PAP adherence. METHODS: RT services for PAP patients were introduced in a multidisciplinary pediatric sleep clinic in May 2006. We identified children who had been followed in clinic, and had adherence download information before and after introduction of RT services. We collected demographic, polysomnography, and CPAP adherence data at clinic visits. RESULTS: Forty-six subjects met criteria for inclusion. The mean ± SD age was 14.9 ± 6 y. The mean ± SD apnea-hypopnea index was 26.7 ± 30 events/h. Other than the addition of the RT intervention, all subjects continued to receive the same clinical services as before. Subjects were divided into 3 groups, based on baseline adherence: 0% use, use for 1–50% of nights, and use for > 50% of nights. There was a statistically significant improvement in PAP adherence in the subjects with baseline use of 0% and 1–50%, but no improvement in those with > 50% use at baseline. There was no significant change in PAP use at subsequent RT visits. CONCLUSIONS: Utilization at clinic visits of an RT trained in the use of PAP improved adherence in pediatric subjects with obstructive sleep-disordered breathing when their baseline PAP adherence was < 50%.

Assessment and Treatment of Obstructive Sleep-Disordered Breathing

Obstructive sleep-disordered breathing (OSDB) is a condition that affects 1% to 3% of the pediatric population. These disorders are difficult to diagnosis and left untreated may be serious, including not only medical comorbidities but also cognitive, academic, behavioral, and emotional sequelae. This article is designed to bring awareness of the severity and prevalence to family physicians and pediatricians. It reviews detailed information concerning OSDB, including the predisposing factors, assessment of presenting features, and treatment.

Diagnosis of pediatric obstructive sleep disordered breathing: beyond the gold standard.

Childhood obstructive sleep disordered breathing (OSDB), a sleep-related upper airway obstruction that degrades sleep quality, ventilation and/or oxygenation, in turn leads to a variety of daytime symptoms and morbidity. In the past, childhood OSDB was classified into primary snoring versus obstructive sleep apnea (OSA) syndrome and it was widely assumed that childhood OSA required treatment, while primary snoring did not. Pediatric polysomnography (PSG) was originally developed to distinguish primary snoring from childhood OSA. However, the late 1990s saw an explosion of new research on the daytime manifestations and long-term morbidity of childhood OSDB, which resulted in expanded, more comprehensive definitions of childhood OSDB syndromes. It is now clear that even primary snoring, in the absence of classically defined OSA, can be associated with neurobehavioral abnormalities in children. Thus, in retrospect, we realize that ‘classical’ childhood OSA was only a subset of a larger affected population and that children previously classified as having primary snoring may have serious neurobehavioral dysfunction. Now, in the era of the expanded definition of childhood OSDB, the role of PSG in the routine evaluation of the snoring child is controversial. Given that overnight PSG is widely regarded as the ‘gold standard’ for the diagnosis of OSDB in children, we review the current usage of PSG in children for the diagnosis of OSDB, its value, limitations and possibilities for future improvements.

Characteristics and surgical and clinical outcomes of severely obese children with obstructive sleep apnea.

STUDY OBJECTIVES To describe characteristics and surgical and clinical outcomes of obese children with obstructive sleep apnea (OSA). METHODS At our institution from 2000 to 2010, 143 obese children with an overnight polysomnography (OPSG) diagnosis of OSA, excluding children with comorbidities, were identified. Relationships between demographics, clinical findings, and the severity of OSA were assessed. Presurgery and postsurgery OPSG indices were compared. We defined cure as an apneahypopnea index (AHI) < 1.5/h on the postsurgery OPSG, and we compared the cure rates of different surgeries. RESULTS A total of 143 children, median age 12.4 y (interquartile range [IQR] 9.6-14.9) and BMI z-scores 2.8 (IQR 2.6-2.9), were included. Seventy-eight (55%) (Median age 12 y [IQR 9-15]) underwent surgery: 1 had tonsillectomy; 1 tonsillectomy + uvulopharyngopalatoplasty (UPPP); 23 adenotonsillectomy (AT); 27 AT + UPPP; 11 adenoidectomy + UPPP; 8 UPPP; and 7 AT + turbinate trim ± tongue base suspension. Overall, surgery cured 19 children (26%), but AHI improved in the majority of children (p = 0.001). Similarly, the arousal index, PETCO2, and SpO2 nadir improved significantly (p < 0.002, p = 0.019, p < 0.001, respectively). AHI improved significantly in children with mild-to-moderate OSA in comparison to severe OSA (p < 0.001). Children with enlarged tonsils and no history of prior surgery benefitted more often from surgery (p < 0.004 and p = 0.002, respectively). AT was the only surgery reducing the AHI significantly (p = 0.008). Children did not lose weight despite intervention. Adherence with PAP was poor. CONCLUSIONS Surgery improved OPSG indices in the majority of obese children with OSA.

Behaviorally based adherence program for pediatric patients treated with positive airway pressure

Objectives: Positive Airway Pressure (PAP) devices are being increasingly used in pediatric populations for problems including Obstructive Sleep Apnea. There is limited literature regarding pediatric adherence with PAP treatment or successful interventions that improve adherence. This paper describes a clinical program for pediatric PAP users which was designed to improve adherence of patients prescribed PAP therapy and involves intensive assessment and behavioral intervention. Method: The design and implementation of the program is described, including the purpose, goals, programmatic structure, clinical content, type and training of personnel, strengths and limitations and financial concerns as well as the challenges with the development and application of the program’s protocol. Results: Preliminary outcome data show short-term improvements in consistent usage for many of the patients. Conclusions: The program shows some promise and provides a framework for future program development and research.

Report of two patients with associated conditions in addition to cystic fibrosis

PURPOSE To report two patients with associated conditions in addition to cystic fibrosis. METHODS We reviewed our database and report two patients with cystic fibrosis who had associated conditions. These patients also had novel disease causing CFTR mutations on full gene sequence analysis. RESULTS We identified 2 patients with novel disease causing cystic fibrosis transmembrane conductance regulator mutations that we report here. A 12-year-old female with cystic fibrosis, diagnosed at 18months, had normal pulmonary function tests and chest X-ray. Her main cystic fibrosis-related health issue was poor growth. Results of cystic fibrosis transmembrane conductance regulator DNA analysis showed deltaF508; L467P; and 7T/9T. She was later diagnosed with Crohns disease. An 11-year-old male with Rubinstein-Taybi syndrome, diagnosed with cystic fibrosis at 2years of age, had minimal findings on chest X-ray and pancreatic insufficiency. Results of his cystic fibrosis transmembrane conductance regulator DNA analysis showed deltaF508; 4329delCT; and 7T/9T. CONCLUSION We report 2 patients with CF who had associated conditions and also had novel disease causing CFTR mutations. Associated conditions may worsen the clinical manifestations of CF and complicate medical management.

Narcolepsy With Cataplexy Diagnostic Challenge in Children

Narcolepsy is a disabling sleep disorder characterized by excessive daytime sleepiness, cataplexy, sleep paralysis, hypnagogic and hypnopompic hallucinations, and abnormal sleep–wake patterns. Cataplexy is described as sudden and transient loss of muscle tone, often triggered by intense emotion or excitement. Hallucinations and sleep paralysis occur during sleep–wakefulness transitions and are associated with rapid eye movement (REM) sleep. Although presence of all characteristics of narcolepsy is common in adult patients, the initial presentation of narcolepsy in children maybe subtle, and concomitant cataplexy, sleep paralysis, and hallucinations may not always present. In addition, symptoms may be difficult to recognize leading to delay in the diagnosis. We present the case history of a child with narcolepsy and cataplexy who was challenging to diagnose in the primary care setting. Our patient’s presentation points out the difficulty in arriving at the diagnosis of narcolepsy with cataplexy due to the clinical picture that can resemble other conditions. In this article, we review the clinical presentation of narcolepsy with or without cataplexy in childhood and discuss the disease pathogenesis, diagnostic tools, and treatment.

An In-patient Model for Positive Airway Pressure Desensitization: A Report of 2 Pediatric Cases

Application of positive airway pressure is frequently indicated in pediatric patients with a diagnosis of obstructive sleep apnea. Adherence to equipment use is often less than optimal and can be more challenging when working with children with special needs. An in-patient protocol was designed utilizing various techniques and strategies from the medical adherence literature and applied to 2 cases. This protocol utilizes specialists from various disciplines, including respiratory therapists, psychologists, physicians, nurses, and child life therapists, as well as parental involvement. This paper outlines this protocol using 2 case studies. Both patients successfully used their equipment for greater than 4 hours at night by the end of their hospital stay of 4 days and maintained or advanced these gains at follow-up. These 2 cases suggest that more research should be conducted to further evaluate the effectiveness of similar programs.

Impact of Degree of Obesity on Sleep, Quality of Life, and Depression in Youth

OBJECTIVE Obese youth are more likely to report difficulties with sleep, depression, and quality of life (QOL). This study aims to characterize sleep problems, QOL, and symptoms of depression by degree of obesity. METHOD The cross-sectional study was conducted in a specialized obesity clinic. Obese youth and their caregivers (N = 150) were evaluated with the Child Sleep Habits Questionnaire and Pediatric Quality of Life. Youth completed the Childrens Depression Inventory. Regression models and correlations were calculated. RESULTS Degree of obesity was predictive of increased sleep difficulties and decreased QOL scores. Childrens Depression Inventory scores showed that children with more symptoms of depression had more sleep problems, and these were not associated with the degree of obesity. Adolescents with more difficulties sleeping also reported more symptoms of depression and lower QOL. CONCLUSIONS Degree of obesity negatively affected QOL and sleep variables. Patients with greater sleeping difficulties reported more symptoms of depression.