From analyzing 100 assessments of coping, the authors critiqued strategies and identified best practices for constructing category systems. From current systems, a list of 400 ways of coping was compiled. For constructing lower order categories, the authors concluded that confirmatory factor analysis should replace the 2 most common strategies (exploratory factor analysis and rational sorting). For higher order categories, they recommend that the 3 most common distinctions (problem- vs. emotion-focused, approach vs. avoidance, and cognitive vs. behavioral) no longer be used. Instead, the authors recommend hierarchical systems of action types (e.g., proximity seeking, accommodation). From analysis of 6 such systems, 13 potential core families of coping were identified. Future steps involve deciding how to organize these families, using their functional homogeneity and distinctiveness, and especially their links to adaptive processes.

http://psycnet.apa.org/journals/bul/129/2/216.pdf

Searching for the structure of coping: a review and critique of category systems for classifying ways of coping.

In a review of the literature from 1948 to 2001, 122 studies were found that correlated structural or functional social support with patient adherence to medical regimens. Meta-analyses establish significant average r-effect sizes between adherence and practical, emotional, and unidimensional social support; family cohesiveness and conflict; marital status; and living arrangement of adults. Substantive and methodological variables moderate these effects. Practical support bears the highest correlation with adherence. Adherence is 1.74 times higher in patients from cohesive families and 1.53 times lower in patients from families in conflict. Marital status and living with another person (for adults) increase adherence modestly. A research agenda is recommended to further examine mediators of the relationship between social support and health.

/pdf/social-support-and-patient-adherence-to-medical-treatment-a-4pbumyhsad.pdf

Social support and patient adherence to medical treatment: a meta-analysis.

This comprehensive State of the Art review summarizes the current published knowledge base regarding the pathophysiology and microbiology of pulmonary disease in cystic fibrosis (CF). The molecular basis of CF lung disease including the impact of defective cystic fibrosis transmembrane regulator (CFTR) protein function on airway physiology, mucociliary clearance, and establishment of Pseudomonas aeruginosa infection is described. An extensive review of the microbiology of CF lung disease with particular reference to infection with P. aeruginosa is provided. Other pathogens commonly associated with CF lung disease including Staphylococcal aureus, Burkholderia cepacia, Stenotrophomonas maltophilia, Achromobacter xylosoxidans and atypical mycobacteria are also described. Clinical presentation and assessment of CF lung disease including diagnostic microbiology and other measures of pulmonary health are reviewed. Current recommendations for management of CF lung disease are provided. An extensive review of antipseudomonal therapies in the settings of treatment for early P. aeruginosa infection, maintenance for patients with chronic P. aeruginosa infection, and treatment of exacerbation in pulmonary symptoms, as well as antibiotic therapies for other CF respiratory pathogens, are included. In addition, the article discusses infection control policies, therapies to optimize airway clearance and reduce inflammation, and potential future therapies.

Pathophysiology and management of pulmonary infections in cystic fibrosis.

While originally characterized as a collection of related syndromes, cystic fibrosis (CF) is now recognized as a single disease whose diverse symptoms stem from the wide tissue distribution of the gene product that is defective in CF, the ion channel and regulator, cystic fibrosis transmembrane conductance regulator (CFTR). Defective CFTR protein impacts the function of the pancreas and alters the consistency of mucosal secretions. The latter of these effects probably plays an important role in the defective resistance of CF patients to many pathogens. As the modalities of CF research have changed over the decades from empirical histological studies to include biophysical measurements of CFTR function, the clinical management of this disease has similarly evolved to effectively address the ever-changing spectrum of CF-related infectious diseases. These factors have led to the successful management of many CF-related infections with the notable exception of chronic lung infection with the gram-negative bacterium Pseudomonas aeruginosa. The virulence of P. aeruginosa stems from multiple bacterial attributes, including antibiotic resistance, the ability to utilize quorum-sensing signals to form biofilms, the destructive potential of a multitude of its microbial toxins, and the ability to acquire a mucoid phenotype, which renders this microbe resistant to both the innate and acquired immunologic defenses of the host.

Lung Infections Associated with Cystic Fibrosis

Background The results of case-control studies and anecdotal reports suggest that pancreatitis may be a risk factor for pancreatic cancer, but there have been no studies of sufficient size and power to assess the magnitude of the relation between these two diseases. Methods and Results We undertook a multicenter historical cohort study of 2015 subjects with chronic pancreatitis who were recruited from clinical centers in six countries. A total of 56 cancers were identified among these patients during a mean (±SD) follow-up of 7.4 ±6.2 years. The expected number of cases of cancer calculated from country-specific incidence data and adjusted for age and sex was 2.13, yielding a standardized incidence ratio (the ratio of observed to expected cases) of 26.3 (95 percent confidence interval, 19.9 to 34.2). For subjects with a minimum of two or five years of follow-up, the respective standardized incidence ratios were 16.5 (95 percent confidence interval, 11.1 to 23.7) and 14.4 (95 percent confidence interval, 8...

https://www.nejm.org/doi/pdf/10.1056/NEJM199305203282001

Pancreatitis and the risk of pancreatic cancer

CHIP—Coping Health Inventory for Parents: An assessment of parental coping patterns in the care of the chronically ill child.

In patients with cystic fibrosis, CF-related diabetes mellitus (CFRD) has been associated with increased morbidity and mortality. Whether glucose intolerance is also associated with poor outcomes is unclear. To better define these relationships we prospectively followed a group of 152 patients with CF without diabetes for 4 yr. Patients were classified as having normal glucose tolerance (NGT), impaired glucose tolerance (IGT), or CFRD without fasting hyperglycemia (CFRD-No FH). FEV(1), FVC, and body mass index (BMI) were measured at baseline and quarterly. At baseline 45% of the patients had NGT, 38.8% had IGT, and 15.8% had CFRD-No FH. FEV(1), FVC, and BMI at baseline were comparable among these groups (all p > 0.1). After 4 yr an overall decline in FEV(1) and FVC occurred, with no change in BMI. The rates of decline for FEV(1) and FVC correlated with the glucose tolerance groups, with the highest rates of decline occurring among the CFRD-No FH group. In addition, patients in the lowest quartile for insulin production at baseline experienced the highest rates of pulmonary function decline over time, suggesting a relationship between insulin deficiency and clinical deterioration. We conclude that the degree of glucose intolerance is a strong determinant of future lung function decline in patients with CF.

https://www.atsjournals.org/doi/pdf/10.1164/ajrccm.162.3.9904075

Trends in pulmonary function in patients with cystic fibrosis correlate with the degree of glucose intolerance at baseline.

Diabetes mellitus associated with cystic fibrosis.

We evaluated patients with cystic fibrosis (CF) and moderate obstructive lung disease in pulmonary exacerbation in a double-blind placebo-controlled trial to determine the contribution of antibiotic-mediated reduction in sputum bacterial density to clinical improvement. For the first 4 days of study, all patients received bronchodilating aerosols and chest physiotherapy but no antibiotics. During this time, the patients showed significant improvement in mean FVC, FEV1, and maximal midexpiratory flow rate (FEF25-75). In 12 of 13 trials, the patients showed no significant increases in the density of Pseudomonas aeruginosa during these first 4 days. In these 12 trials, the patients were stratified by their initial FVC and randomized to receive either parenteral tobramycin and ticarcillin (n = 7) or placebo (n = 5), in addition to continued aerosol and chest physiotherapy. In the remaining trial, the patient had a significant rise in the density of P. aeruginosa and was assigned to the antibiotic group. During the next 14 days of therapy, the antibiotic group showed significantly (p less than 0.01) greater reductions in log10 colony-forming units (cfu) of P. aeruginosa per gram of sputum and greater increases in FVC, FEV1, and FEF25-75 than did the placebo group. The degree of decrease in log10 cfu P. aeruginosa/g sputum correlated significantly (p less than 0.001) with the degree of improvement in FVC, FEV1, and FEF25-75.(ABSTRACT TRUNCATED AT 250 WORDS)

Reduction of sputum Pseudomonas aeruginosa density by antibiotics improves lung function in cystic fibrosis more than do bronchodilators and chest physiotherapy alone.

Warren J. Warwick

Papers

CHIP—Coping Health Inventory for Parents: An assessment of parental coping patterns in the care of the chronically ill child.

Trends in pulmonary function in patients with cystic fibrosis correlate with the degree of glucose intolerance at baseline.

Diabetes mellitus associated with cystic fibrosis.

Reduction of sputum Pseudomonas aeruginosa density by antibiotics improves lung function in cystic fibrosis more than do bronchodilators and chest physiotherapy alone.

Risk of death in cystic fibrosis patients with severely compromised lung function.