Editon 2 Volume 2 CFW Newsletter
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From the Editor's Desk
Sam Hillyard

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A letter from Herman Weggen to the AAFC Romania

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MAILBAG

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MICROBIOLOGY
New and Emerging Respiratory Tract Infections in PWCF
Tom Coenye, PhD

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AND THE QUESTION IS?
Possible Pancreatitis?
CF Carriers and Ulcerative Colitis
Prenatal Diagnosis Overseas

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PHYSIOTHERAPY
Gastroesophageal Reflux in Infants
Dr Brenda M Button
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CLOSE-UP ON NATIONAL ASSOCIATIONS
Canada
Cathleen Morrison
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PSYCHOSOCIAL
Gender and Interpersonal Relationships
L Gee, J Abbott, S P Conway
C Etherington and A K Webb

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BOOK REVIEWS
Alive at 25
Andy Lipman

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MEDICAL TOPIC
Cystic Fibrosis: A Geriatric Problem
Warren J Warwick
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Transplant Q & A
Joanne Schum

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PROJECTS
CFW and Burke Bear
Christine Noke
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RESEARCH & DEVELOPMENT
Discovering and Developing Drugs: The CF Foundation Way
Robert J. Beall
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PHYSIOTHERAPY TOPIC:

Dr Brenda M Button

Gastroesophageal Reflux (GOR) in Infants:

A Time for a Change?

Dr Brenda M Button

 

INTRODUCTION
Since the discovery of the CF gene in 1989, newborn screening has been carried out in many parts of the world. This has meant that CF has been diagnosed at a much younger age than previously - usually within the first two months of life when most infants are well and have no chest symptoms. Before newborn screening, a diagnosis of CF was usually made because of recurring chest symptoms and it was logical to start chest physiotherapy right away. However, early diagnosis with newborn screening has resulted in discussion about when chest physiotherapy (also known as airway clearance techniques) should be started after diagnosis. Two studies using a bronchoscope to look into the lungs of infants with CF and to obtain samples of lung secretions were published in 1995. They reported signs of early inflammation, infection and presence of thick mucus in the airways of these very young infants before they displayed any symptoms of lung problems such as cough. These studies have persuaded most CF centres to start chest physiotherapy as soon as CF is diagnosed. The big question is what sort of physiotherapy should be done in these very young - usually symptoms and cough free - infants?

Most chest physiotherapy techniques used with infants with CF have been adapted from techniques used in older patients with daily sputum production. This approach has not considered the uniqueness of the very young infant. The aims of this article are:

1. To describe the differences between infants and mature individuals that need to be considered during physiotherapy treatment.

2. Present what is known about gastro-oesophageal reflux (GOR) in infants and children with and without CF.

3. To describe the different forms of chest physiotherapy being used in different parts of the world at this time.

4. Discuss what may need to change in the physiotherapy management of infants with CF.

Differences Between Infants and Mature Individuals with CF

• Infants are immature at birth and not just little adults.
• They have more cartilage in their rib cage and airways making these more elastic and flexible.
• They depend mostly on their diaphragm for breathing because the breathing muscles situated between the ribs (the intercostal muscles) are underdeveloped.
• Their ribs are more horizontally positioned than in adults causing the diaphragm to be flatter and less dome-shaped making them more likely to have gastro-oesophageal reflux which is an abnormal increased frequency and duration of regurgitated stomach contents into the oesophagus (the tube that directs food from the mouth to the stomach).
• Infants are predominantly nose breathers with a proportionally larger tongue. The suck-swallow-breathe mechanism relies on finely developed controls. The gastric and respiratory systems have much in common. They share a common nerve supply via the Vagal Nerve and share a common pathway through the back of the throat
allowing air to move into the lungs via the trachea while food passes into the stomach via the oesophagus.

“…leads to an increase in pressure on the stomach…”

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In the infant the oesophagus is proportionally much narrower and shorter than the adult. Infants take frequent liquid feeds. Their musculature (the arrangement and condition of their muscles) is immature causing them to spend proportionally more time lying down, including after feeds. They tend to consume larger meals than their stomach can hold causing them to overflow, vomit or spit up (possette) after feeds. When held in a sitting position immature muscles supporting the spine allow a slumped sitting posture which leads to an increase in pressure on the stomach making gastro-oesophageal reflux (GOR) more common.

GOR is common in all infants under a year with 18-40% of normal infants having it. In numerous studies it has been found to be more common in infants with CF. The majority of infants outgrow GOR around eighteen months to two years as they become more mature, eat more solid food and spend more time in the upright position.

Can Gastro-Oesophageal Reflux Affect the Lungs?
Inhalation of acidic gas or sometimes stomach contents can lead to inflammation and infection in the lungs in some patients. Irritation of the oesophageal lining from regurgitated acidic stomach contents may stimulate the Vagal Nerve which supplies the lungs and stomach causing reflex broncho-spasm and sometimes wheeze.

How Does a Baby who has Gastro-Oesophageal Reflux Behave?
Burping, crying, stretching, drooling, frowning, mouthing, irritability, yawning, and croup like sounds may indicate reflux. Some infants try to stop the reflux from coming up to the mouth by pulling in the chin and turning the head to the side which is called Sandifer’s Sign.

Gastro-Oesophageal Reflux in CF
GOR was first diagnosed in CF in 1975. Further studies in the 1980s found that children with CF had more GOR than their siblings without CF. Furthermore, children with CF and GOR had significantly poorer lung function than those without GOR. One study in Belgium found that more than three-quarters of a group of twenty-six children with CF under five years of age had significant GOR.

In four different studies in infants and children with and without CF, postural drainage positions (tilting head down 30 – 45 degrees) have been shown to increase the number of reflux episodes during treatment.

Five Year Study Investigating the Effects of Standard Physiotherapy with Head down Tilt (SPT) Compared to Modified Physiotherapy without Head Down Tilt (MPT) on Gastro-Oesophageal Reflux
In Melbourne, Australia, we studied twenty newly diagnosed infants aged two months to measure the effects of standard physiotherapy (with head down tilt), referred to as SPT, compared to modified physiotherapy (without head down tilt), referred to as MPT, to find out what effects these treatments have on gastro-oesophageal reflux. Percussion and thoracic squeezes were applied to both treatment groups. We used 30 hour oesophageal pH monitoring to measure the number of reflux episodes during treatment. Oesophageal pH monitoring involves introducing a probe into the oesophagus via one of the nostrils where it remains for a period of time and measures the pH (acid or alkaline) in the oesophagus which determines whether GOR is occurring.

Standard physiotherapy (SPT) positions
Modified physiotherapy (MPT) positions
Standard physiotherapy (SPT) positions
Modified physiotherapy (MPT) positions

The SPT treatment regimen resulted in significantly more gastro-oesophageal reflux episodes than the MPT treatment regimen. Parents agreed for their infants to be randomly assigned to either SPT or MPT for their daily treatment. When they were more mature, physical activities were included in their daily routine. These included activities such as running, jumping on a trampoline, tri-cycling, blowing and laughing games and positive expiratory pressure (PEP) therapy in the form of ‘Bubble PEP’.

BUBBLE PEP SET-UP

BUBBLE PEP SET-UP

1- Milk or orange juice carton
2- 13 cm (water level)
3- Oxygen Tubing
4- PEP Resistor
5- Manometer and PEP conector

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Five years after entering into the study both groups had very good lung function. However, the MPT group had significantly better lung function and fewer lung changes on their Chest X-Rays.

“…protected them from increased reflux while still being an effective treatment technique…”

What we can conclude from the study is that infants in the MPT (no tip) group were not disadvantaged by not being tilted head down. To the contrary, their lung function was slightly but significantly (in the scientific sense) higher and they had fewer Chest X-ray changes than the SPT (with tip) group at five to six years of age. We speculate that not tipping the MPT group may have protected them from increased reflux while still being an effective treatment technique, and that may be the reason why their results were slightly better than the SPT group.
This study has been published in the March 2003 edition of Pediatric Pulmonology.

What About Older Children, Teenagers and Adults with CF?
We also studied twenty-four young people with CF who complained of one or more of the following symptoms - abdominal or chest pain, weight loss, heartburn, acid regurgitation and vomiting or waking up at night with an irritating dry cough. During 24hr oesophageal pH monitoring each young person had two sessions of chest physiotherapy which included breathing exercises, percussion and thoracic squeezes in the same four positions as in the SPT regimen pictured earlier – the three head down positions were angled at 30 degrees (see earlier diagram). One third of these young people were diagnosed with gastro-oesophageal reflux confirming that reflux is quite common in young people with cystic fibrosis. During the study, 20 of the 24 patients had more episodes of gastro-oesophageal reflux during chest physiotherapy compared to background time when they were not doing physiotherapy.

“They were offered a trial of PEP mask therapy in upright sitting…”

Six of our teenage patients repeatedly complained of symptoms of reflux during postural drainage in head down positions. They were having frequent hospital admissions for chest infections. Their lung function continued to drop in spite of maximum treatment. They were offered a trial of PEP mask therapy in upright sitting (see photo) which they found much more comfortable than head down physiotherapy in terms of reflux symptoms.

 

PEP mask therapy
graph of FEV1

 

 

 

 

 

 

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We graphed their lung function during the last two years of postural drainage before starting PEP in upright sitting, and then progressively recorded their lung function over the next two years. We were surprised to discover that there was a significant improvement in lung function in the first six months (see graph of FEV1 above) which then reached a plateau that was maintained for the following eighteen months. Annual days in hospital for chest infections were calculated for the final year using postural drainage and compared with the first year using upright PEP therapy. They all spent significantly fewer days in hospital in the PEP year. They all preferred PEP therapy in sitting and found it much more comfortable than head down physiotherapy in terms of reflux symptoms. This paper was published in the Australian Journal of Paediatrics in 1998. We are currently carrying out a study in adults with CF.

What Different Types of Physiotherapy Techniques are Currently Being Used Around the World in Infants with CF?
An International Survey was completed in 1999 and provides a snapshot of the different types of physiotherapy being used with infants with CF in one hundred and sixty-six centres in twenty-seven countries. The majority of centres had between 50 and 200 patients. There were a few very large centres. The findings were:

• Approximately a third of the centres surveyed carry out newborn screening. This confirms many infants worldwide start chest physiotherapy at a much younger age than prior to discovery of the CF gene and the introduction of newborn screening.
• Traditional head down tilted postural drainage is used at about half of the centres surveyed.
• Modified positions (without head down tilt) are used at just under half of centres surveyed.
• PEP mask therapy is used at about a fifth of centres surveyed - mostly in Denmark and Italy.
• Passive Autogenic Drainage (Belgium) and the techniques developed in France, Germany and Sweden were all used at just about a fifth of centres surveyed.

“…scientific evidence rather than traditional practice.”

What Changes to Chest Physiotherapy Should be Considered?

• The infant should be considered as a unique and immature being and not be treated as a little adult.
• The results of all the research studies should be considered by the team of each CF Centre and physiotherapy treatment regimens should be based on scientific evidence rather than traditional practice. This requires more research and a re-think of how infants, children and adults with CF should be positioned during chest physiotherapy in the future.
• There is a strong case for not tipping head down at any stage during life with CF, rather carrying out treatment in horizontal or upright sitting positions as is current practice at many centres worldwide.

However, it is not recommended that chest physiotherapy treatment techniques should be altered without first talking to your therapist and medical doctor.


Dr Brenda M. Button
Physiotherapist
Melbourne
Australia

b.button@physio.unimelb.edu.au

Editor’s Note: For a copy of the references please contact me: editor@cfww.org

 


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