physiotherapy in CF

Question

I study physiotherapy and I would like to get more information concerning the newest rehabilitation methods for a 15-year-old CF child. I am mostly interested in general outline of the disease in this particular age group and the best rehabilitation methods, which are available not only in Poland. Please, send the answer to my e-mail address.
Thank you for your help. K.S.

Answer

Dear Questioner,
Age is not the only criterion in selection of the physiotherapeutic technique. One should also consider the stage of the disease, the presence of bronchial hyperresponisveness, the level of patient's motivation. However, a 15-year-old child should be encouraged to perform an active rehabilitation.
Diversity of rehabilitation devices is huge: the most recommended and used are the devices providing increased variable expiratory pressure, like Flutter, Acapella, Cornet. When used correctly, these devices are one of the most effective way in drainage of thick, sticky mucous from the airways.
Positive opinions have also PEP mask, thera PEP, pari PEP, which provide positive expiratory pressure. Their additional advantage is the possibility to combine the inhalation wih exercises.
In less efficient patients good results were observed when Bird and Bennet Respirators was used - a device, which uses positive variable expiratory pressure. It differs from the above mentioned devices, because it promotes inspiration.
Very important component of everyday rehabilitation in CF patients is physical activity, adjusted individually to their possibilities and efficiency. Autogenic drainage is an effective, but rarely used in Poland as technique. Importantly, to use this technique no supplementary equipment is needed. This technique uses a specific way of breathing in 3 phases: low, medium and high. It can be combined with other techniques, e.g. Acapella.
Recently an compression vest has aroused the interest. Certainly it has a lot of advantages, but this is a passive technique of physiotherapy, therefore it should be recommended as a supplement to active physiotherapy.
These are only few of possible physiotherapeutic techniques, which can be used in a teenage patient.
With kind regards,
mgr Monika Domagalska

24.05.2011

23.05.11
Extra info on airway clearance:
Many different airway clearance techniques are available, but, in general, their goal is to reduce disease progression by augmenting the normal mucociliary clearance mechanism of the lungs and facilitating expectoration. They do this by altering airflow and mucous viscosity. The recommendations for using these devices are summarised in relevant guidlines (Flume, CF Trust). If further information is required Bradley et al provided a detailed summary of five Cochrane reviews which have looked at the evidence for airway clearance. The five Cochrane reviews included (Airway clearance compared with no airway clearance in CF; Conventional chest physiotherapy compared with other forms of airway clearance in CF; PEP compared with other forms of airway clearance in CF; Airway clearance with non-invasive ventilation compared with airway clearance with no non-invasive ventilation in CF) This paper concluded that evidence from the Cochrane systematic reviews indicate that no one airway clearance regimen is better than another. It is likely that specific airway clearance regimens may be more effective in some circumstances (e.g. one of the Cochrane systematic reviews supports the use of NIV compared with other airway clearance regimens in patients with more severe disease who have difficulty clearing sputum). Unfortunately, the reviews could not provide evidence-based guidance on what therapies are most effective in which circumstances. One of the reviews provided evidence that individuals may prefer airway clearance regimens that are self-administered and facilitate independence. The specific regimen chosen can be alternated within an airway clearance session and during different stages of disease (e.g. patients may prefer an independent airway regimen during a stable phase of disease and a more passive regimen during an acute exacerbation).

Extra info on phsyical activity/exercise:
There are many reasons why exercise/physical activity should be core component of treatment for patients with CF. Exercise contributes to the alleviation of dyspnoea and reduced exercise tolerance in CF. It may also be an important part of the management of diabetes in CF, as exercise improves appetite and contributes to a more positive body image. Physical training maintains pulmonary function by improving sputum clearance and reducing residual volume and may delay the onset of osteoporosis by preventing a reduction in bone mineral density. Other postulated benefits of any physical training may be decreased anxiety and depression, enhanced feelings of well-being and enhanced performance at work, recreational and sport activities. Finally, adherence to physical training is important because exercise capacity may be an independent prognostic risk factor in CF. Non-adherence to physical training may contribute to worsening signs and symptoms of respiratory disease and a reduced ability to perform activities of daily living and thus ultimately have a detrimental effect on the individual’s prognosis.

A Cochrane review has looked at the evidence for exercise training. This review has concluded that studies on the efficacy of physical training in CF are limited by the small size, duration and incomplete reporting of most of the studies included in this review. However, there is limited evidence that physical training is beneficial. The benefits obtained from including physical training in a package of care may be influenced by the type of training programme and the inclusion of aerobic and anaerobic training. Physical training is already part of the care package offered to most people with CF and there is no evidence to actively discourage this.
Judy Bradley

List of References:

- Bradley JM, Moran F, Ellborn JS. 2006. Evidence for physical therapies (airway clearance and physical training) in cystic fibrosis: An overview of five Cochrane systematic reviews. Respiratory Medicine. 100, 191–201

- Bradley JM, Moran F. Physical training for cystic fibrosis. Cochrane Database of Systematic Reviews 2008, Issue 1. Art. No.: CD002768. DOI: 10.1002/14651858.CD002768.pub2.

- Cystic Fibrosis Pulmonary Guidelines: Airway Clearance Therapies, Flume P A, et al. Respiratory Care. 2009; 54(4):522-537

- Airway-clearance therapy guidelines and implementation, Lester MK and Flume PA., Respiratory Care. 2009;54(6):733-750; discussion 751-753

- Physiotherapy in infants and young children with cystic fibrosis: current practice and future developments. Lannefors L, et al. Journal of the Royal Society of Medicine. 2004;97 Suppl 44:8-25.

- Clinical Guidelines for the physiotherapy management of cystic fibrosis, CF Trust Association of Charterred Physiotherapists in cystic fibrosis, 2002.