Cystic fibrosis. End-stage care in Canada.

OBJECTIVES: To determine the circumstances in which individuals with cystic fibrosis (CF) die, the role of different caregivers, and the extent of palliative care for CF patients. DESIGN: Mailed survey of CF physicians. SETTING: CF centers in Canada. PATIENTS: All CF deaths in 1996 known to centers in Canada. RESULTS: The mean age (+/- SD) at death of the 45 individuals included in the study was 25.8 +/- 13.5 years. The major cause of death was respiratory (34 patients; 75.5%). Nutritional concerns were common. Lung transplantation was considered in 42 patients (93.2%), with 7 patients (17.1%) being entered on a list, but it was carried out in only 2 patients (4.4%). Autopsies were performed on only 10 patients (22.2%). Most patients died in hospital (37 patients; 82.2%), and 7 patients (15.6%) died in ICUs while receiving intermittent positive-pressure ventilation. Palliative care was never discussed in 10 patients (25%). In a further 16 patients (40%), it was not discussed until the last month before death. CONCLUSIONS: Respiratory disease remains the most common cause of death in CF patients. Lung transplantation is frequently considered, but most patients die without having had a transplant. Discussions on end-of-life care could be considered sooner.

Pregnancy and cystic fibrosis: a case-controlled study.

STUDY OBJECTIVES: Changes in cardiorespiratory and pulmonary function that occur with normal pregnancy along with increased maternal and fetal demands related to cystic fibrosis (CF) may augment morbidity for the woman with CF. Status prior to pregnancy is implicated in pregnancy outcome and maternal life expectancy postpartum. The purpose of this study was to investigate the effect of pregnancy on these patients' course during pregnancy and document prepregnancy status and 2-year postpregnancy survival. DESIGN: Patients with documented pregnancies were matched to nonpregnant CF patients of similar age (+/-2 years), severity of airflow obstruction (percent predicted forced expiratory volume in 1 min [+/-15%]), weight (+/-10 kg), height (+/-5 cm), and pancreatic sufficiency status at 1 year preconception. PATIENTS: Using their 1-year preconception data, seven women with CF and with documented pregnancies were matched to nonpregnant control subjects. All patients were pancreatic insufficient. INTERVENTIONS: Weight, forced expiratory volume in 1 min (% FEV1), FVC, Schwachman-Kulczycki (S-K) and Brasfield scores, sputum cultures, pregnancy outcome, and pulmonary exacerbations were followed from 1 year preconception, during pregnancy, and 2 years postpregnancy. MEASUREMENTS AND RESULTS: Mean weight gain during pregnancy was 5.2 kg. There were no differences between the groups in the rate of decline for pulmonary function or S-K scores over time. Greater rate of decline was noted in the pregnancy group, however, for body weight and Brasfield scores in the postpartum interval. One patient in the pregnancy group died 6 months postpartum. CONCLUSIONS: Pregnancy has little adverse effect on patients with stable CF, but poor outcomes can occur in individuals with more advanced disease.

Colonic carcinoma in two adult cystic fibrosis patients.

Two adult women with cystic fibrosis (CF) who developed colonic carcinoma, both at age 31, are described. In both patients the carcinoma occurred in the midtransverse colon. The diagnosis had not been suspected, partly because of the patients' relatively young age. In case 1, the symptoms also mimicked the distal intestinal obstruction syndrome. At diagnosis she was shown to have metastases to the regional lymph nodes. In case 2, despite a long history of chronic pulmonary and sinus disorders, CF was not diagnosed until the patient was 36 years old. The incidence of gastrointestinal malignancies has been shown to be significantly increased in patients with CF. As the life expectancy of the CF population increases, vigilance for gastrointestinal cancers in CF patients is important, as illustrated by these two cases.

Eradication of methicillin-resistant Staphylococcus aureus from the lower respiratory tract of patients with cystic fibrosis.

Two of 95 patients followed in an adult cystic fibrosis clinic consistently grew methicillin-resistant Staphylococcus aureus (mrsa) on sputum culture. Sputum Gram stain consistently showed +4 polymorphonuclear leukocytes and +4 Gram-positive cocci in clusters. Both patients were co-infected with Pseudomonas aeruginosa and required multiple hospitalizations for treatment of pulmonary exacerbation, resulting in significant infection control concerns. Multiple courses of antibiotics, including ciprofloxacin and clindamycin regimens, failed to eliminate the mrsa. A combination of oral rifampin and clindamycin was successful in eradicating the organism from both patients. Over a 12-month period following therapy, in both patients none of 13 sputums showed Gram-positive cocci in clusters on Gram stain and none of 13 sputum cultures grew mrsa. Successful eradication of mrsa has greatly simplified infection control measures on subsequent hospitalizations, reducing costs and enhancing patient comfort.

Case-control and vector studies of nosocomial acquisition of Pseudomonas cepacia in adult patients with cystic fibrosis.

OBJECTIVE: To examine factors associated with nosocomial acquisition of Pseudomonas cepacia in adult patients with cystic fibrosis. DESIGN: A retrospective case-control study of 5 patients with nosocomial acquisition of P cepacia versus 20 matched controls who failed to develop P cepacia infection. Selective handwashing, air sampling, and respiratory equipment sampling also were performed. SETTING: A university hospital providing tertiary care to 95 adult cystic fibrosis patients. PATIENTS: All patients are adults with known cystic fibrosis. Case definition required multiple negative sputum cultures for P cepacia prior to and during admission, with a positive sputum culture prior to discharge. Controls had negative sputum cultures for P cepacia prior to and throughout hospitalization. Controls were matched for age, gender, disease severity, and frequency of hospitalizations. RESULTS: Factors associated with increased risk of nosocomial acquisition of P cepacia included receiving humidifier or nebulized treatments (60% versus 5%, p = .016, odds ratio = 28.5, 95% confidence interval = 1.93 to 420.58). Factors without significance included ward, room, teaching versus nonteaching status, use of steroids, sharing a hospital room with another cystic fibrosis patient, antibiotic use, presence of portocath in situ, or socializing with another individual with cystic fibrosis known to be P cepacia-positive. Air sampling studies failed to demonstrate aerosolization of P cepacia by coughing cystic fibrosis patients over a 1-hour sampling time. Handwashing studies failed to demonstrate P cepacia on hands of cystic fibrosis patients, nurses, or physiotherapists (before or after physiotherapy). Reservoirs from nebulizers consistently grew P cepacia following therapy. CONCLUSIONS: Respiratory equipment may be an important source of nosocomial acquisition of P cepacia in adult cystic fibrosis patients.

Bone and joint tuberculosis: a continuing problem.

Although tuberculous disease of bones and joints is becoming uncommon, it still occurs and may cause devastating sequelae. It is frequently not diagnosed prior to the onset of permanent damage to the joints or spine; the most important reason for this delay may be the fact that it is not considered in the differential diagnosis of monoarthritis or back pain. Most persons with the disease have other evidence of tuberculosis. Not infrequently an aggressive approach (including synovial biopsy or surgical exploration of the back) is needed to confirm the diagnosis when there are no other clues.

Yellow-nail syndrome: report of three cases.

The yellow nail syndrome, a combination of yellow discolouration of and dystrophic changes in the nails, pleural effusions and lymphedema, is thought to be relatively rare; to date 44 cases have been reported. Of a further three patients with this syndrome, one had all three features, one had the yellow nails alone and the other had pleural effusions and lymphedema without classic nail changes. Each had recurrent lower respiratory tract infections; and of all 47, chronic pulmonary infections occurred in approximately one quarter and were frequently associated with chronic sinus infections. The underlying abnormality is presumed to be a congenital defect of the lymphatics, but so far this has not been demonstrated to be the cause of the nail changes, the pathogenesis of which remains obscure.

Lifelong follow-up of inactive tuberculosis: its value and limitations.

There are 14,552 inactive cases of tuberculosis in British Columbia, representing 0.6 per cent of the population. The prevalence of inactive tuberculosis in 7 times higher among Indians than in the remainder of the population. Among all inactive cases, 60.5 per cent of patients had "good" chemotherapy, whereas 13.2 per cent had poor chemotherapy and 26.3 per cent had no chemotherapy. The risk of reactivation in those who received "good" chemotherapy was 2.2 cases per 1,000 persons per annum, whereas for those with poor and no chemotherapy, the risks were 7.8 and 6.7 cases per 1,000 persons per annum, respectively. One third of a sample of all inactive cases were found not to comply with the existing policy of lifelong annual examinations. Of the remaining two-thirds, one-half attended regularly and one-half, irregularly. All reactivations occurring during the 3-year period between 1971 and 1973 were reviewed. The pattern of attendance of these patients during a 3-year period immediately before the year of reactivation was similar to that of the sample of all inactive cases. Sixty per cent of reactivations occurring among "attenders" were diagnosed at such annual examinations. The bacteriologic status of reactivated disease was reviewed; 78 per cent were culture positive. Of patients who had received previous chemotherapy, 45 per cent had organisms that were resistant to one or more of the 3 primary antituberculous drugs, whereas previously untreated groups showed a much lower figure, 7.8 per cent. On the basis of the findings of this study, recommendations are made in relation to future policy of management of inactive cases of tuberculosis.