International Physiotherapy Group for Cystic Fibrosis
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INTERNATIONAL PHYSIOTHERAPY PRACTICE IN INFANTS WITH CYSTIC FIBROSIS SURVEY RESULTS 1998 β 2000 .
Introduction.
Cystic fibrosis (CF) is characterized by viscous pulmonary secretions, impaired
muco-ciliary clearance, obstruction of small airways, fibrosis and ultimately respiratory failure
[1,2]. Improved survival has been attributed to better nutritional status, intensive treatment with
antibiotics and physiotherapy [3]. Without newborn screening, a diagnosis of CF is usually made with
the onset of respiratory symptoms, failure to thrive, steatorrhea or a presentation of meconium
ileus in the neonate [4]. Physiotherapy has traditionally been introduced at the time of diagnosis
[5]. In a study of fetal lungs during the second trimester of pregnancy, accumulation of mucin in
the tracheobronchial glands in the infants with CF was observed compared to controls, but other
changes were lacking [6]. It is not clear whether inflammation, infection or excessive mucus
constitutes the initial pulmonary insult. Clinically asymptomatic infants with CF have demonstrated
early airway inflammation, infection and pathogens on broncho-alveolar lavage in the first weeks of
life [7,8]. These early changes are linked to reduced mucociliary clearance and obstruction in CF
[9]. Physiotherapy has for decades been widely accepted as an integral part of the regular treatment
of patients with CF [5]. The main aim of physiotherapy is to compensate physically for the impaired
mucociliary clearance with the objective of retarding the development of lung disease as much as
possible and to preserve lung function and physical function [10]. The widespread introduction of
newborn screening worldwide for CF in the past two decades has led to an early diagnosis of CF,
often in the first few months of life, when many infants are clinically asymptomatic.
Physiotherapists meeting at international conferences noted that despite the widespread use
of physiotherapy in infants with CF, little was known about its worldwide use. There are no
internationally accepted guidelines for physiotherapists treating infants with CF.
We
hypothesized that differences exist in a number of aspects of the physiotherapy management of
infants with CF internationally, with different outcomes in different regions, and that there is a
need for evidence based practice. The aims were to determine:
(1) the demographics of the
organization of physiotherapy and the experience of therapists treating infants in CF centres;
(2) the use of newborn screening;
(3) education of the providers of daily physiotherapy;
(4) infection control practice in inpatient and outpatient settings, and,
(5) the timing of
the introduction of daily physiotherapy after diagnosis, the different techniques prescribed, and
the use of adjunctive inhalation therapy
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