Ralph C. Frates

Outcome of home mechanical ventilation in children

A retrospective study of the outcome of home mechanical ventilation in 54 children with chronic respiratory insufficiency was conducted. The childrens ages ranged from 4 months to 18 years. Tracheostomies and positive pressure ventilation were used in 33 patients, and negative pressure ventilation in 21 patients. Nine patients were eventually weaned from the ventilator, including three quadriplegic patients in whom electrophrenic stimulation subsequently was used. Over 20 years, there were 17 deaths, including three from ventilator disconnection. Life table analysis showed 1-year survival to be 84%, and 5-year survival 65%. Home mechanical ventilation, with patients cared for by family and friends, cost less (average less than

Home negative pressure ventilation: Report of 20 years of experience in patients with neuromuscular disease

1000/mo) than registered nurse or hospital care (average

536 EFFECT OF CHRONIC CIMETIDINE (C) ADMINISTRATION ON GROWTH IN CYSTIC FIBROSIS (CF) PATIENTS

16,000/mo). There was no significant difference in the number of deaths between patients cared for by registered nurses 24 hours a day and those cared for by family and friends. Home mechanical ventilation may be a reasonably safe and cost-effective alternative to prolonged hospitalization for the ventilator-dependent child.

1622 BROVIAC CATHETERS FOR PROLONGED ANTIBIOTIC ADMINISTRATION IN CYSTIC FIBROSIS PATIENTS

Twenty years of experience using negative pressure devices (NPD) at home to ventilate 40 patients with neuromuscular disease is presented. The purpose of the study was to determine the costs, complications, and clinical outcome of this form of respiratory support, and to ascertain the reasons for failure to institute effective negative pressure ventilation (NPV) in nine patients. Emerson tank respirators, used mainly to rest respiratory muscles at night, and intermittent positive pressure breathing machines were used by 98% of patients at an average equipment cost of +2,700 annually. Patients in whom NPV was initiated on an elective rather than emergent basis saved an average of +12,000 during their initial hospitalization. Life table analysis shows a five-year survival of 76%, and a 10-year survival of 61%. Complications were minor and occurred at an average rate of less than one per year per patient at home on NPV. Failure to achieve satisfactory NPV in nine patients was associated with age (six patients were younger than 3 years of age), or severe thoracocervical scoliosis, which prevented proper fitting of the NPD. For reasons of safety, economy, and quality of life, NPV at home is the preferred treatment for patients having neuromuscular disease who need respiratory assistance.

Home Positive-Pressure Ventilation: Twenty Years’ Experience

Twenty-five CF patients (7.5-17.9 years) participated in a one-year double-blind cross-over study to assess the influence of chronic cimetidine administration on growth parameters. Assessment of the group prior to entering the studies revealed coefficient of fat absorption while receiving pancreatic enzyme supplements from 35-95% (mean 64%), pulmonary function (FVC) from 32-100% predicted (mean 71%), and growth retardation with weight < 5% in 19, height < 5% in 14, and delayed bone age in 16 patients. Growth was assessed by height, weight, K40 lean body mass (LBM), muscle mass estimated by 24 hour urine creatinine, fat content by skin fold thickness, and bone age. RESULTS: 1) Average increases for any parameter were no greater on cimetidine than placebo. 2) Growth parameters did not show parallel changes. 3) No toxic effects were observed. 4) Two patients had no height increase and one patient had weight loss but 8 had decreases in LBM during the year. 5) Cimetidine reduced basal and pentagastrin stimulated gastric acid secretion at least by 50% in 19 cases. CONCLUSION: 1) Chronic cimetidine administration should not be a routine part of CF care. 2) Height and weight may be misleading parameters to follow in assessment of nutrition status.

Analysis of predictive factors in the assessment of warm-water near-drowning in children

An efficient and safe program for continuous home administration of parenteral antibiotics was developed for chronically ill cystic fibrosis children. Six patients, ages 9 to 20 yrs, who required frequent hospitalizations for pseudomonas bronchitis had Broviac or Hickman catheters placed under general anesthesia. Catheters were utilized over intervals of one month to two years. A combination of tobramycin (5-7.5 mg/kg/day) and carbenicillin or ticarcillin (300 mg/kg/day) was given every six to eight hours by patients or their parents. Peak tobramycin levels between 5 - 10 μg/ml were achieved. Compared to a control period prior to beginning the program, a decreased frequency of hospitalizations for pulmonary symptoms was noted in 4 of 6 patients. All patients benefited by their ability to maintain normal activities at home and at school. No objective improvement in pulmonary function tests utilizing peak flow measurements was observed in this patient group. No catheter-related complications were observed. Two catheters required repair because of leakage of the external segment. Prolonged administration of antibiotics utilizing the Broviac catheter may benefit some patients with cystic fibrosis. However, the long term effects of this therapy on the progression of their pulmonary disease are unknown.