Cystic fibrosis programs

I was hospitalized, diagnosed with cancer, have been the grateful recipient of eight blood transfusions and just completed round one of chemotherapy. I could not have afforded my medication without you. Apply Donate. Status Open. About Cystic Fibrosis Cystic fibrosis CF is an inherited disease that causes progressive damage to the respiratory system and chronic digestive system problems but can involve multiple organ systems of the body. We also offer a multidisciplinary care team including:.

The Cystic Fibrosis Therapeutics Development Network , is the largest cystic fibrosis clinical trials network in the world. Because of our affiliation with this network, your child will benefit from the latest drug trials, clinical studies and a wealth of research in cystic fibrosis treatment. Our physicians also serve as faculty at UT Southwestern, leading the next generation of doctors and advancing the standard of care for cystic fibrosis.

Your child will be in good hands with our team. Cystic Fibrosis Program. The diagnosis and treatment of cystic fibrosis requires multidisciplinary care.

Following national and state guidelines regarding health measures, treatment and testing is important. Learn more about the comprehensive national cystic fibrosis guidelines we following by visiting the Cystic Fibrosis Foundation. If you would like to make an appointment, please call our office at or our Nurse Manager Mary Hendry at You can also make an appointment online below. View Dr. Stephen's profile ». The categorization of the patients was done on the basis of sputum or throat swab examinations from the year before the stay.

At least three previous 3 monthly results had to be available and, in addition, a recent swab from within the last 2 months before the stay. Furthermore, all patients were educated in personal hygiene measures along the guidelines for infection control in outpatient cystic fibrosis patients as published previously [ 14 ] including disinfection of hands, no close contact to other participants, and strict usage of personal equipment for inhalation.

At all sites the appropriate hygiene surface and cleaning procedures were in place. In all programs the patients usually had private rooms; in case of small children these were shared with a family member accompanying them.

Patients usually brought their own personal inhalation devices, sterilizing equipment for these, and their medication with them. Inclusion criteria were the diagnosis of cystic fibrosis by clinical symptoms and a positive sweat test or conclusive molecular genetics and a clinically stable condition. In a subset of patients these data were obtained after one week, in addition daily peak flow measurements and a 6 min walking test on a treadmill was performed at the beginning and the end of these stays.

In a first step, differences between the initial measurements taken at the site and the last measurements taken at the site were performed. Therefore only complete before and after pairs are considered and the number N of which is indicated.

For an optimal comparison, the subjects were carefully matched for age, Pseudomonas state, sex, and baseline values of weight and lung function.

For the 68 rehabilitation stays in the German sites, 5 patients could not be matched due to non-documented matching variables and in three patients no fitting matching partner could be identified.

Summary data are given as medians with range or interquartile range. The retrospective chart review of the results from the rehabilitation centers was performed anonymised. The use of all data obtained was approved by the local institutional review board for human studies of the University of Munich.

From all different subjects and the total number of different rehabilitation stays noted in our files to have been sent to rehabilitation programs, records were retrieved. Values for peak flow and a 6 min walk test were solely reported in a small subgroup of patients for certain seasons. First we analyzed all cystic fibrosis patients staying at the Israel site and those at the German and Swiss sites separately. Taken together, rehabilitation programs at both sites led to improved lung function and weight.

During these stays additional measurements at the end of the first week on average on day 9. Whereas lung function clearly improved during the first week, weight improved continuously over the whole rehabilitation period Fig.

This observation is in agreement with the observation that the gain of weight, but not the improvement of FEV 1 was proportional with the duration of the rehabilitation programs Fig. Additional measurements at the end of the first week on average on day 9. The Israel groups were not included as their stay was always fixed to 3 weeks.

A direct overall comparison of the Israel and German rehabilitation programs did not show significant differences of the basal data and of the changes associated with the rehabilitation with respect of age, sex, percentage of Pseudomonas positive subjects, weight, and lung function. To circumvent some of these problems an additional comparison was made after very careful matching of subjects for age, Pseudomonas state, sex, and baseline values of weight and lung function.

Again, no differences for the improvement of lung function Fig. The figure illustrates the wide range of individual changes observed. Dotted lines connect matched subjects. Note that these are different subjects matched by chance and not the same individuals staying at both sites.

Differences were assessed by Wilcoxon-Test and p values are indicated. The overall completeness of the reports on quantitative key outcomes was clearly low in all rehabilitation programs investigated. While lung function improved early on and then remained stable, the gain of body weight was sustained and proportional to the length of the stay. Inter-individual responses were much more pronounced than variability within subjects. This is to our knowledge the largest systematic analysis of rehabilitation programs in patients with cystic fibrosis published so far.

One of the strengths of this study is that it included all patients of a predefined group of subjects from our centre that participated in the mentioned rehabilitation activities. Since complete and unbiased up-to-date evaluation by the rehabilitation program providers are not published in an accessible way, we decided to do this analysis from the perspective of a site submitting patients, accepting potential disadvantages of this approach. First, the submitting centre did not always receive the appropriate medicinal and psychosocial information necessary to judge on the success or failure for a particular patient, including missing data for key variables before and after the program, reports on exacerbations during a stay, data on other untoward events or on psychosocial problems.

Second, the precise nature and frequency of interventions performed by the patients often remained unclear. We can only infer from the procedures stated in the program description provided by the rehabilitation centres what might be offered to the patient. Third, the results we present here, only concern cystic fibrosis patients from our centre and results of patients from other submitting sites are unknown.