International Physiotherapy Group for Cystic Fibrosis
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INTERNATIONAL PHYSIOTHERAPY PRACTICE IN INFANTS WITH CYSTIC FIBROSIS SURVEY RESULTS 1998 – 2000 .
Discussion.
Data from two-thirds of IPG/CF member countries relating to the diagnosis and
physiotherapy treatment of infants with CF was collected and analyzed. The results of this
questionnaire demonstrated widely varying practices between centres in many aspects of
physiotherapy. The demographic information relating to size of centres suggests that this survey
captured a broad picture of the physiotherapy treatment of infants with CF worldwide and can
therefore be accepted as representative of worldwide practice at the beginning of the 21st century.
The experience of physiotherapists treating infants with CF ranged from a few months to 4 decades.
This evidence supports the need for treatment guidelines for physiotherapists commencing work with
patients with CF for the first time.
There are no randomized controlled trials to provide an
evidence base for the early introduction of daily physiotherapy in clinically asymptomatic infants
diagnosed with newborn screening. Some physiotherapists argue against the early introduction of
daily physiotherapy in the absence of symptoms, conversely, many experienced physiotherapists
working in internationally recognized CF centres reason for the early introduction of physiotherapy.
Their justification comes from studies using broncho-alveolar lavage (BAL), in young infants
diagnosed with newborn screening, where early inflammation and infection, often with pathogens, are
identified in clinically asymptomatic infants [7,8]. Furthermore, these physiotherapists believe
that infants tolerate treatment better when it is introduced early in infancy and becomes part of
the daily routine of the infant and family. They speculate that this may in turn enhance
participation and adherence to treatment in the longer term [11].
The study by Sutton and
colleagues demonstrated the usefulness of inhaled saline to increase the effectiveness of sputum
clearance [12]. Nebulized saline may hydrate the thick viscous secretions sometimes found in the
lungs of clinically asymptomatic infants with CF, which in turn may improve muco-ciliary clearance.
No long-term clinical trials have been carried out to investigate the effectiveness of regular use
of nebulized saline, broncho-dilators or DNase in infants with CF and the risk of increased
infection from contaminated equipment. This is an area requiring research.
Respiratory
symptoms are usually characterized by a combination of clinical observation and assessment including
breathing pattern, respiratory rate, sound of coughing or crying, chest sounds on auscultation,
oximetry and chest radiograph. When respiratory paediatricians used these criteria to determine
whether a patient was asymptomatic or not, the infants with apparently normal respiratory status
were found to have diminished airway function using full and partial forced expiratory manoeuvres
[13]. Infants responded positively to bronchodilators and physiotherapy [14]. The absence of
respiratory symptoms on clinical examination and radiology was found to underestimate the degree of
lung disease [15]. Infant pulmonary function tests have not yet been standardised and there is no
normative data. It was concluded that it is not possible to assess a patient as being asymptomatic
based on clinical examination alone [15]. High resolution computed tomographic scans and
broncho-alveolar lavages (invasive and expensive techniques) offer more sensitive complementary
information and a more accurate description of the respiratory state in infants with CF [7,8]. Even
though the onset and intensity of lung disease is highly variable, most begin to develop a
progressive pulmonary obstruction during early childhood [7,9]. Some physiotherapists experienced in
treating infants with CF believe that the early introduction of physiotherapy at the time of
diagnosis may delay the progression of lung disease as physiotherapy is the only physical means of
compensating for decreased muco-ciliary clearance, one of the hall-marks of CF [11].
Newborn
screening has been available for more than two decades and has become standard practice in many
regions. Until the introduction of newborn screening, diagnosis was usually made because of
respiratory symptoms at varying ages from months to years. Connett and colleagues evaluated the
outcomes of the early commencement of CF treatment, including physiotherapy, following newborn
screening compared to delayed diagnosis in 73 patients with homozygous Delta F508 over ten years. At
ten years, patients with delayed diagnosis required significantly more antibiotics and inhaled
steroids to maintain their health and had more radiological changes [16].
There has been a
tendency to extrapolate physiotherapy techniques used in mature patients with chronic cough and
sputum production to developmentally immature infants, many of whom are clinically asymptomatic
[17]. The horizontal alignment of the cartilaginous ribs result in mechanically less efficient
intercostal muscles and a flatter diaphragm.and consequent wider sterno-costal angle reducing the
effectiveness of the anti-reflux barrier [18]. Infants ingest frequent liquid feeds and spend much
of their time in recumbent positions. Poor truncal tone causes infants to adopt a slumped sitting
position increasing intra-abdominal pressure. All of these factors predispose the infant to
gastro-oesophageal reflux [19].
Postural drainage with head down tilted positions is widely
used internationally (see Table 3). The evidence for use of postural drainage comes from studies in
mature patients with chronic sputum expectoration and until relatively recently has not been studied
in infants. Postural drainage with head down tilted positions of 30º has been shown to provoke
increased episodes of gastro-oesophageal reflux in very young infants of 2 months diagnosed with CF
using newborn screening and some positions have been found to be more reflux provoking than others,
in particular prone head down [20]. A study in older infants using lesser angles of head down tilt
and different positions to the previous study [20] including the avoidance of the prone head down
position did not demonstrate an increase in reflux [21]. A five-year study of twenty neonates
randomized to standard physiotherapy or modified physiotherapy has shown that the modified
physiotherapy group had significantly better lung function and fewer radiographic changes at six
years of age [22]. Orenstein, in a recent editorial recommended that modified physiotherapy
positions, avoiding head down tilt, should be used with asymptomatic infants with CF [23].
The aim of Autogenic Drainage (AD) developed by Jean Chevallier in Belgium in the 1970s is
to achieve an optimal expiratory airflow progressively through all generations of bronchi without
causing dynamic airway collapse [24]. Assisted Autogenic Drainage
(AAD) sometimes described as
passive AD is the adaptation of AD to infants and young children not yet mature enough to carry out
this technique independently. AAD carried out by the physiotherapist supporting the infant in
upright sitting on an exercise ball and utilizing bouncing was not found to provoke episodes of
gastro-oesophageal reflux [25].
A study of the effect of the French Technique of manually
increasing expiratory flow with thoracic compression combined with pharyngeal suction to stimulate
cough on gastro-oesophageal reflux was undertaken in 1991 [26]. Treatment in the supine horizontal
position was associated with increased gastro-oesophageal reflux compared to 30? head up.
Furthermore, oropharyngeal suction was associated with significantly more gastro-oesophageal reflux
than naso-pharyngeal suction. It was concluded that treatment should be carried out in the 30? head
up tilted position and that oropharyngeal suction should be avoided [26].
The Swedish
Technique was developed after a long term study from 1981-1983 which demonstrated that the effects
of physical activity/exercise combined with the forced expiration technique (FET) was equally as
effective as postural drainage, percussion and the FET and much preferred by infants, young children
and their families [27]. Since 1983 postural drainage and percussion have not been used in Sweden
with any patients regardless of age. For infants, toddlers and young children the physical handling
/ activity is interspersed with gentle therapist assisted thoracic compressions towards residual
volume during expirations [11]. Recently published data demonstrates that this treatment philosophy
has resulted in better than average long term outcomes [11].
Positive expiratory pressure
(PEP) therapy was developed in Denmark in the early 1980s and was found to be at least as effective
as postural drainage [28]. Since 1984 PEP in upright sitting has been the only technique used in
Denmark where better than average outcomes have been reported using aggressive anti-biotic therapy
and PEP physiotherapy [29]. PEP therapy has also been used in infants in Italy for a number of
years. PEP was found to be as effective as postural drainage in a 12 month study of infants with CF,
and the treatment of choice for parents and infants [30].
The majority of therapists
recommended that physiotherapy ideally be carried out before meals. If that was not possible then at
least one hour after meals / feeds. A number of therapists believed that at least 2 hours should
pass before physiotherapy is undertaken. Delayed gastric emptying is known to occur in CF [31]. In a
study comparing the effects of standard postural drainage (SPT) and modified postural drainage (MPT)
on gastro-oesophageal reflux, infants had significantly more episodes of reflux during SPT than MPT
even though treatment was carried out at least two hours after feeds [17].
Concern has been
raised that if infants engage in non-nutritive sucking during physiotherapy, particularly in head
down tilted positions, that gastro-oesophageal reflux may be further increased. In a recent study
[20] postural drainage (with head down tilt) was associated with gastro-oesophageal reflux, crying
and lower oxygen saturation during treatment. Non-nutritive sucking was associated with a
significant reduction in reflux episodes during postural drainage. Oxygen saturation during postural
drainage was significantly lower during crying than other arousal states and non-nutritive sucking
was associated with a significant increase in oxygen saturation. It was concluded that the use of
non-nutritive sucking and infants falling asleep during treatment probably does no harm [20].
It is not possible to undertake double blinded placebo controlled trials with
cardio-respiratory physiotherapy techniques. The number of patients at different centres is often
not large enough for adequate power to detect a change when comparing two techniques. This survey
has highlighted the need for well-designed research studies that may need to be extended to single
blind multi-centre trials for adequate power.
The results of this survey show that newborn
screening is becoming more widespread with the majority of centres introducing physiotherapy at the
time of diagnosis often in the first 4-8 weeks of life. As postulated, wide variations in
physiotherapy organization and practice were found, little of which is underpinned with scientific
evidence. Regional tradition and cultural influences are strong factors in the selection of
physiotherapy techniques for use with infants with CF. There is an urgent need for further research
into the treatment of infants with CF so that practice is based on scientific evidence and best
outcomes are achieved.
TABLE 1 – Countries included in the
survey and number of completed regional surveys
Country |
Number of completed
surveys | | Argentina | 5 | | Australia | 8 | | Austria | 7 | | Belgium | 5 | | Brazil | 3 | | Canada | 9 | | Costa Rica | 1 | | Czech Republic | 1 |
| Denmark | 2 |
| Estonia | 1 |
| Finland | 5 |
| France | 14 |
| Germany | 6 |
| Ireland | 4 |
| Country | Number of completed surveys |
| Italy | 11 |
| Macedonia | 1 |
| Netherlands | 4 |
| New Zealand | 1 |
| Norway | 4 |
| Poland | 4 |
| Portugal | 1 |
| Romania | 1 |
| Slovakia | 4 |
| Sweden | 3 |
| Switzerland | 3 |
| United Kingdom | 11 |
| United States | 47 | |
TABLE 2: Inhalation therapy
use as an adjunct to physiotherapy in asymptomatic versus symptomatic infants
| Asymptomatic | Symptomatic |
Inhaled agent |
F/A | Occ. | N/R | F/A | Occ. | N/R |
Bronchodilator | 27[41] | 23[34] |
50 [76] | 73 [115] | 23[36] |
4 [5] |
Isotonic saline | 27[40] | 14[21] | 59 [91] |
38 [56] | 14[20] | 48 [69] |
Hypertonic
saline | 2[2] | 4[6] | 94
[137] | 5 [8] | 9[12] | 86[121] | DNAse | 9[13] | 14[21] |
77 [115] | 30 [45] | 30 [45] |
40 [60] |
Anti-inflammatory | 7[10] | 19[29] | 74 [110] |
37[55] | 38[58] | 25 [38] |
Antibiotics | 16[23] | 14[22] |
70 [103 | 50 [74] | 29[44] |
21 [32] |
Propylene glycol | 0 [0] | 1[1] | 99 [138] |
2 [2] | 4[5] | 94[118] |
Abbreviations: F/A= Frequently / Always; Occ.=
Occasionally; N/R = Never / Rarely.
The whole numbers refer to the % of centres. The numbers
between square brackets [ ] refer to the number of centres that answered the question.
TABLE 3: Airway clearance techniques and cough stimulating techniques
used with infants with cystic fibrosis internationally
Technique | Frequently/
Always | Occasionally | Never / rarely |
Postural drainage | 55%
[90] | 14% [23] | 31% [51] | Modified postural drainage | 47%
[75] | 26% [42] | 27% [44] | Clapping / percussion | 35% [55] |
12% [19] | 53%
[83] | Manual
vibration | 52% [84] | 18% [30] | 30% [50] |
Mechanical vibration |
9% [15] | 12% [19] |
79% [126] |
Positioning to  Ventilation | 58%
[89] | 18% [28] | 24% [36] | Autogenic drainage (passive) | 19%
[30] | 12% [19] | 69% [111] | French Technique | 17% [2] |
9% [14] | 74% [114] |
German Technique |
15% [2] | 7% [10]
| 78% [115] |
Swedish Technique | 19%
[26] | 8% [11] | 73% [98] | PEP therapy | 14% [2] |
5% [7] | 81% [136] |
Cough Stimulation |
31% [44] | 28% [40] |
41% [60] |
- tracheal pressure | 13%
[20] | 20% [32] | 67% [110] | - oropharyngeal stimulation | 4%
[7] | 15% [23] | 81% [126] | - oropharyngeal suction | 7% [11]
| 20% [32] | 73% [115] | - nasopharyngeal suction | 9% [15]
| 25% [39] | 66% [105] |
Postural
drainage included head down tilted positions while modified postural drainage avoided head down
tilt. Autogenic drainage (passive) consisted of gradually moving secretions towards the upper
airways with therapist assisted expirations towards residual volume generating the highest
expiratory airflow without causing dynamic airway collapse.
The French technique consisted of
acceleration of expiratory flow using thoracic
compression combined with cough stimulation using
tracheal pressure.
The German technique consisted of contact breathing stimulated by the
sensation of the therapist’s hands.
The Swedish technique consisted of physical activities in
different positions to
alter the breathing pattern combined with gentle assisted expirations
towards
residual volume to enhance airway clearance.
TABLE 4: Therapists opinions and recommendations relating to infant behaviours (non-nutritive sucking on a pacifier, falling asleep and infant distress) during physiotherapy treatment.
Therapists’ opinions
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Frequently/
Always |
Occasionally |
Never/
rarely |
| The use of pacifiers |
| Helps settle |
| Try not to use |
| No opinion |
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| 36% [54] |
| 33% [46] |
| 14% [15] |
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| 32% [47] |
| 55% [79] |
| 78% [81] |
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| Infants falling asleep / upset |
| Allow infants to fall asleep |
| Try to keep awake |
| Keep treating: mildly upset |
| Cease session: distressed |
| No opinion |
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| 41% [59] |
| 37% [51] |
| 73% [113] |
| 72% [110] |
| 9% [9] |
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| 25% [37] |
| 9% [13] |
| 15% [23] |
| 16% [25] |
| 6% [6] |
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| 34% [49] |
| 54% [75] |
| 12% [17] |
| 12% [18] |
| 85% [88] |
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