Development of the role-play inventory of situations and coping strategies for parents of children with cystic fibrosis.

Critiqued previous conceptual and methodological approaches to the measurement of stress and coping. Applied Goldfried and D'Zurilla's behavior-analytic model to create a context-specific measure of problematic situations and coping strategies for parents of school-age children with cystic fibrosis (CF). The sample was stratified by child's gender and illness severity. Forty-seven families (46 mothers, 32 fathers) and 8 health care professionals completed structured interviews or daily diaries to obtain the widest range of problematic situations; 1,725 situations were elicited across all participants and then content-analyzed into 97 nonredundant categories in 11 domains (e.g., Discipline, Medical Care). Few differences were found in problem frequency or difficulty as a function of either gender or illness severity. Using empirical criteria, the most frequent and difficult problem situations were selected and developed into role-play vignettes that include relevant contextual, developmental, and interactional details. The Role-Play Inventory of Situations and Coping Strategies (RISCS) consists of 31 audiotaped vignettes designed to elicit and evaluate the coping strategies used by parents of children with CF.

Effectiveness and safety of small vs. large doses of enteric coated pancreatic enzymes in reducing steatorrhea in children with cystic fibrosis: a prospective randomized study.

Among cystic fibrosis (CF) centers, usual doses of enteric coated (EC) pancreatic enzymes vary from one to six capsules per meal based upon arbitrary criteria for stool and growth patterns. Large doses of non-EC enzymes are associated with increased serum urate (SU) and urinary uric acid (UUA) but data are unavailable for EC enzymes. This study compared the effectiveness and safety of a relatively large dose (patient's usual dose) versus a small dose (1/4 usual dose) of EC enzymes in nine nourished children with CF, regarding decreasing fecal fat and stool nitrogen losses and maintaining normal SU and UUA concentrations. A crossover study design randomly assigned large or small doses to two consecutive 7 day treatment periods within each child. Large doses of EC enzymes reduced steatorrhea and increased SU and UUA. SU was normal with both treatments and UUA was normal, i.e., 17 of 18 values were between the 10th and 95th percentiles for healthy children eating a normal diet. When fat excretion was greater than 10% with small doses of EC enzymes, large doses resulted in reduced fat excretion and normal UUA. These data suggest that large doses of EC enzymes reduce steatorrhea and are safe in patients who have malabsorbtion with small doses.

Intra-abdominal complications of cystic fibrosis.

In recent years patients with cystic fibrosis (CF) have experienced longterm survival and have demonstrated a number of intra-abdominal complications. This report evaluates the intra-abdominal complications seen in 69 of 189 children with cystic fibrosis from 1972 to 1983. Forty-one patients were boys and twenty-eight girls. Complications occurred in 36 neonates, with meconium ileus (MI) noted in 33 and giant cystic meconium peritonitis (GCMP) in 3. Meconium ileus equivalent occurred in seven older children presenting with bowel obstruction. In addition, rectal prolapse occurred in 12, inguinal hernia in 10, intussusception in 3, cholelithiasis in 3, GE reflux in 4, stress ulcer in 1 and appendicitis in 1. Three infants with GCMP survived resection and enterostomy. Infants with MI were divided into simple (15) or complicated (18) cases. Nonoperative therapy using gastrografin enema was successful in three of eight with simple MI. Operative enterotomy and irrigation was successful in three cases while resection and enterostomy was done in nine. MI was complicated by atresia, volvulus and/or perforation in 18 cases requiring resection and anastomosis or enterostomy. Survival for MI was 86% compared to 36% in 25 MI patients treated in the previous two decades. Meconium ileus equivalent was successfully managed using gastrografin enema in five of seven children. Only 3 of 12 children with rectal prolapse required repair. Two cases of intussusception were reduced while one required resection. Three of 10 children had hernia recurrence due to chronic pulmonary problems.(ABSTRACT TRUNCATED AT 250 WORDS)

Changing feeding trends as a cause of electrolyte depletion in infants with cystic fibrosis.

Between June 1979 and June 1980, 16 infants with cystic fibrosis (CF) were cared for at the James Whitcomb Riley Hospital for Children. Five of these children (average age 5.8 months) had a total of eight episodes of electrolyte depletion, with six episodes unassociated with high environmental temperature, fever, or significant gastrointestinal symptoms. Their primary symptoms were poor weight gain and anorexia. According to their dietary records, these five infants, at the time of their initial presentation, had an average electrolyte intake of 8 mEq of sodium, 12 mEq of potassium, and 10 mEq of chloride per day. All infants had been fed either standard infant formula or breast milk. Infant feeding surveys indicate that the estimated average sodium intake of 6-month-old infants has decreased from 45 mEq/day in 1965 to 15 mEq/day since 1977 when manufacturers stopped adding salt to baby foods. In addition, since 1971 the percentage of infants 6 months age receiving breast milk or standard infant formula rather than cow's milk, which is higher in sodium content, has increased from 33% to 72%. This decreased salt intake places the infant with CF at greater risk for electrolyte depletion than in the past. It is expected that a larger percentage of infants with CF will have electrolyte depletion as their initial symptom especially during periods of increased sweating or when electrolyte losses are experienced during gastrointestinal illnesses. CF should be suspected in any infant with electrolyte depletion, and infants known to have CF need daily salt supplementation. Serum electrolytes should be measured if the infant is experiencing weight loss or anorexia, particularly during periods of excessive salt losses.