168 research outputs found
Appetite stimulants use in cystic fibrosis
No full textCystic fibrosis (CF) is an autosomal recessive disease. It affects multiple body organs. The lungs and pancreas are the most affected which results in progressive lung damage and pancreatic insufficiency. Due to the disease process, CF patients require significantly higher caloric intake than recommended for other individuals. The nutritional goal for CF patients is to achieve normal growth and development and, once genetic potential is reached, to maintain good nutritional status throughout life. Evidence has shown that lung function is closely associated with nutritional status in CF and that nutritional status is an independent predictor of survival. Most CF patients are on a high calorie diet to help achieve normal growth and development and maintain good lung function. Inadequate caloric intake in CF can lead to malnutrition. Malnutrition in CF requires careful, multidisciplinary history taking, physical exam, and overall patient/family assessment. Only by determining the actual cause of the malnutrition can appropriate and safe therapies be used to treat it. Appetite stimulants, although efficacious in treating malnutrition in CF, should only be prescribed if decreased food intake secondary to inadequate appetite is the principal cause of the malnutrition and all other contributing factors have been assessed, ruled-out or treated. In this review, we attempted to summarize the use of several appetite stimulants used in CF and other diseases to improve appetite and maximize caloric intake. Pediatr Pulmonol. 2008; 43:209–219. © 2008 Wiley-Liss, Inc.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/57930/1/20766_ftp.pd
Symptomatic increase in intracranial pressure following pancreatic enzyme replacement therapy for cystic fibrosis
No full textA newly diagnosed 5-month-old infant with cystic fibrosis (CF) developed signs and symptoms of increased intracranial pressure (ICP) within days of starting pancreatic enzyme replacement therapy. Symptoms promptly resolved on two occasions after stopping enzyme replacement. At 10 months of age, enzyme replacement was well tolerated. © 1995 Wiley-Liss, Inc.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/38601/1/1950190615_ftp.pd
Inducing sputum in patients with cystic fibrosis could be helpful, but is time-consuming and expensive
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Evaluation of a cystic fibrosis transition program from pediatric to adult care
No full textPurpose As the cystic fibrosis (CF) patient population median survival increase, the need for transitioning their care to adult care centers increase as well. We have a structured transition program since the early 1980s. The purpose of this study is to evaluate the experiences and opinions of patients in our adult CF center who went through a formal transition versus those who did not, in an attempt to evaluate the overall process and to identify means for improvement. Methods A questionnaire was given to adult CF patients at the University of Michigan during a clinic visit or inpatient hospitalization, after consent was obtained. Inclusion criteria included diagnosis of CF in childhood, previous care in a pediatric center, and current participation in our adult CF clinic. Results A total of 91 patients completed the questionnaire. 44 went through our structured transition program. On average, patients who participated in a formal transition process had higher satisfaction with both CF programs, perceived health status, and independence but no differences in their level of anxiety about transferring to the adult program. Patient opinions regarding when to transfer care were considered more often in patients who participated in a transition program (88% vs. 62% and P = 0.02). Conclusion Although structured transitions programs do not appear to decrease patient anxiety during this stressful period, it does appear to improve patient satisfaction, perceived health status, and independence. These findings suggest that establishing a transition program could be important in optimizing patient health, continuity of care and improving adherence. Pediatr Pulmonol. 2013; 48:658–665. © 2012 Wiley Periodicals, Inc.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/98797/1/22647_ftp.pd
Predicted values for lung diffusing capacity in healthy children
No full textLung diffusing capacity was measured in 92 healthy Caucasian children (52 boys and 40 girls), ranging in age from 6 to 17 years, using the single breath carbon monoxide technique (DL CO ). Multiple regression equations were developed to predict the DL CO value. Standing height, gender, weight, and age were significant factors in the prediction of DL CO . Sitting height was not a significant covariate when standing height was in the model, but it could replace standing height in the equation. Equations are presented for the calculation of predicted DL CO in children based on standing or sitting height, age, sex, and weight. Pediatr Pulmonol 1991; 10:267–272.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/38596/1/1950100408_ftp.pd
Overcoming barriers to a successful transition from pediatric to adult care
No full textAs life expectancy for people with cystic fibrosis (CF) has increased dramatically, so has the need for a guided, structured transition from pediatric to adult‐focused care. A formalized transition program allows for seamless transfer of patients between providers, helping to ensure continuity of care, and avoid potential declines associated with inconsistent medical care. New CF Center guidelines issued by the CFF strongly recommend that each center establish a transition program for age‐appropriate transition to an adult CF clinic. In this article, we explore the remaining barriers to establishing a transition program in a CF Center and offer examples of several successful models. We describe CFF‐sponsored and other initiatives that exist to support centers in establishing a transition program and discuss the need for ongoing research in this area.Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/139102/1/ppul23778_am.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/139102/2/ppul23778.pd
The Use of Home Spirometry in Pediatric Cystic Fibrosis Patients
No full textMedication adherence is poor among pediatric cystic fibrosis (CF) patients, with adolescents having one of the lowest adherence rates. We wanted to identify an adherence intervention that would be acceptable to CF adolescents and assess its feasibility. We surveyed 40 adolescents with CF and asked about barriers to and motivators for their own adherence and to generate ideas for potential adherence interventions. Since most of the respondents chose frequent spirometry at home and medication reminders for interventions, we selected 5 subjects, 10 to 14 years of age, with CF to test the feasibility of home spirometry and medication reminders in pediatric CF patients. This article summarizes the results of both the survey and the feasibility pilot study
Bleeding diathesis due to vitamin K deficiency in an infant with cystic fibrosis
No full textAbstractWith the wide implementation of newborn screening for Cystic Fibrosis, infants are being diagnosed in the presymptomatic phase of the disease. Nutritional deficiencies (hypoalbuminemia) and fat soluble vitamins A, D and E deficiencies, due to pancreatic insufficiency and malabsorption, have been reported in the past at the time of diagnosis.1,2 Rarely, infants with CF present with severe bleeding disorder, secondary to vitamin K deficiency, in the first months of life.3 To our knowledge, this is the first case report illustrating bleeding diathesis in a one month old infant with CF. He was diagnosed by newborn screening and presented with a gastrointestinal bleeding due to vitamin K deficient coagulopathy
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