Presentation of an H-type tracheoesophageal fistula in an adolescent male with cystic fibrosis: A case report and review of literature.

Congenital TEFs without esophageal atresia are rare but may occur more frequently than previously documented in literature. Careful history is required to suspect the diagnoses, as most patients will present with coughing associated with solid or liquids, recurrent unexplained pulmonary infections and complaints with eating. Some patients may show signs of chronic airway changes from recurrent aspiration pneumonia at the time of presentation. Diagnosis is challenging, with multiple imaging modalities including x ray, CT scan and esophogram able to identify a fistula. Surgery is required to improve quality of life and prevent chronic airway changes, and most cases repaired have no complications.

Daily spirometry in an acute exacerbation of adult cystic fibrosis patients.

To help answer the question of length of intravenous antibiotics during an acute exacerbation of cystic fibrosis (CF), we had subjects to follow daily home spirometry while on intravenous antibiotics. CF patients, 18 and older, with an acute exacerbation requiring intravenous antibiotics had a daily FEV1. The average time to a 10% increase over their initial sick FEV1 was calculated, as well as the time to a new baseline. A total of 25 subjects completed the study. Ten of the 25 subjects did not have a sustainable 10% increase in FEV1. Of the 15 subjects with a sustainable 10% increase in FEV1, it took 5.2 days (±4.5) after day 1, while a new baseline was achieved on average at 6.6 days (±4.8) after day 1. Given the wide range of time to a 10% improvement and new baseline, it is recommended there should be flexibility in length of intravenous antibiotics in CF, not by a preset number.

Vitamin D deficiency is associated with pulmonary dysfunction in cystic fibrosis.

BACKGROUND: Vitamin D deficiency is common in CF. Whether vitamin D affects pulmonary function in CF is unknown. METHODS: Data were abstracted from clinically stable CF patients who had pulmonary function studies and serum 25-hydroxyvitamin D [25(OH)D, ng/ml] levels drawn within 2 months of each other. Findings were adjusted for multiple variables known to affect pulmonary function in CF. RESULTS: Enrollees totaled 597. Overall mean 25(OH)D level was 29.6±12.8 ng/ml (SD). Serum 25(OH)D levels showed a significant correlation with forced expiratory volume in 1s (FEV1) % predicted (r=0.20, p<0.0001) and forced vital capacity % predicted (r=0.13, p=0.0019). Multivariate analysis revealed that serum 25(OH)D remained an independent predictor of FEV1 % predicted even after controlling for multiple other factors known to affect CF lung function. CONCLUSIONS: Serum 25(OH)D levels are significantly associated with pulmonary function in CF. Further study is required to determine whether this association is causal.

Clinical pharmacist impact on care, length of stay, and cost in pediatric cystic fibrosis (CF) patients.

BACKGROUND: Cystic fibrosis (CF) patients are often treated with aminoglycoside (AG) antibiotics during infective pulmonary exacerbations. Achieving pharmacokinetic and pharmacodynamic (PK/PD) targets to improve outcomes and counteract resistance is paramount. PURPOSE: The primary objective was to compare the number of pediatric CF patients achieving AG PK/PD targets when a clinical pharmacist (CP) managed therapeutic drug monitoring (TDM) compared with usual care (UC). METHODS: A retrospective cohort study was conducted on the records of 40 CF patients that received AGs and ≥2 serum samples between 1/2007 and 5/2009. Chi-square and Student's t-test were used to analyze nominal and continuous variables, respectively. RESULTS: Twenty-nine patients with 52 courses of AGs were included the CP group, and 22 patients with 42 courses were included the UC group. Ninety-eight percent of patients in the CP group reached AG PK/PD targets compared with 71% in the UC group, P < 0.001. Patients in the CP group reached the AG PK/PD target in a mean of 1.9 ± 0.8 days compared with 4.8 ± 3.4 days in the UC group, P < 0.0001. The average LOS in the CP group was 9 ± 5 days compared with 12 ± 7.5 days in the UC group, P = 0.033. The mean number of levels per patient was 2.7 in the CP group compared with 5.2 (range of 2-20) in the UC group, P < 0.001. Resource utilization associated with drug levels, dosing adjustments and LOS were $26,549, $14,069, and $1,680,000 in the CP group as compared with $40,683, $27,812, and $1,940,000, respectively, in the UC group. CONCLUSION: CP managed TDM resulted in a significantly higher percentage of pediatric CF patients achieving AG PK/PD targets 3 days sooner with an average LOS that was 3 days shorter. CP managed TDM resulted in significantly fewer dosage adjustments, drug levels, and cost associated with serum sampling, drug wastage, and LOS.

Tracheobronchial stenting in an infant with an anomalous right main bronchus.

Congenital anomalies of the right main bronchi have not been fully described. Bronchial anomalies are usually asymptomatic unless they coexist with other abnormalities. We describe a rare bronchial anomaly in a 2-month-old girl in which the right upper lobe, middle lobe, and lower lobe bronchi all originated at the same point as a result of a developmental teratogenic long right main bronchus and an absent bronchus intermedius. Furthermore, the left main bronchus contained a proximal segment of stenosis. This combination of anomalies, along with right upper lobe atelectasis and left-sided hyperinflation, resulted in a severe right mediastinal shift and respiratory distress. The mediastinum was returned to midline with endoscopic placement of an in-dwelling tracheobronchial stent into the left main bronchus. To the best of our knowledge, the specific developmental anomaly in our patient has not been previously described.

Survey of professionals' expectations of developmental task achievement of cystic fibrosis self-care in children.

We surveyed 174 professionals with at least 6 months of experience working with children and adolescents with cystic fibrosis (CF), to obtain estimates of ages at which 50% of children with CF may be able to perform each of 44 self-care behaviors related to the treatment of CF. Respondents were 63 physicians, 36 nurses, 24 dietitians, 18 respiratory therapists, 17 nurse practitioners, 10 social workers, and 4 psychologists, who had a mean of 11.4 years (SD = 7.5) of experience working with pediatric patients with CF. Mean age estimates for self-care behaviors ranged from 5.5-13.9 years. For each item, responses varied among respondents, as reflected by a mean standard deviation of 2.75 years for all behaviors. Analyses of concordance suggested a high level of agreement among respondents for the order of mastery of specific skills related to diet and enzymes use only. To date, there are no empirically defined age estimates for when children with CF may be able to perform specific self-care behaviors involved in the management of CF. This survey is the first step in generating age estimates for self-care independence in CF. Future research should conduct an objective assessment of children's CF knowledge and skill in performing these behaviors, and compare these findings to the age estimates offered in this study.

Measuring self-care independence in children with cystic fibrosis: the Self-Care Independence Scale (SCIS).

We describe the development and psychometric properties of a survey tool designed to evaluate children's level of independence in their cystic fibrosis (CF) treatment. Children's self-care autonomy is important to measure and may have a direct effect on children's active involvement in their treatment and their adherence. Existing instruments that evaluate children's independence in their CF treatment fail to yield practical information that can help research and patient management. The Self-Care Independence Scale (SCIS) is a 44-item questionnaire that is completed by parents. The SCIS was completed by the parents of 76 patients with CF (ages 4-17 years). Youths completed two structured interviews, which screened patients for average or higher cognitive functioning (an inclusion criterion) and measured their CF treatment knowledge. Parents completed two other self-report questionnaires. Results indicated that the SCIS has acceptable internal consistency and good test-retest reliability. The construct validity of the SCIS was supported by positive correlations between patient age, number of years since diagnosis, and SCIS total scores. The concurrent validity of the SCIS was supported by correlations between a measure of nonillness-specific dependence and a measure of patient self-care knowledge. Children's SCIS total scores per age group are presented. This study supports the SCIS as a psychometrically sound measure of self-care independence in CF. This measure has several uses. For example, the SCIS may be a screening tool for adolescents who are preparing to transition to adult CF centers and who will need to assume more independence in their self-care. The SCIS may also be used to evaluate educational programs that promote self-care knowledge and skill in children with CF.