Managing The Little Wheezer

1. Managing The Little Wheezer! Liz MacIntosh Children�s Cystic Fibrosis Physiotherapist Barts and the London Children�s Hospital London, UK Thanks for the introduction� Firstly I�d like to highlight there are no conflicts of interest or commercial bias in this presentation.Thanks for the introduction� Firstly I�d like to highlight there are no conflicts of interest or commercial bias in this presentation.

2. Introduction Conventional chest physiotherapy Reflux (Button et al, 1997; Heine et al, 1998; Button et al, 2003) Avoid head down position (Orenstein, 2003; Prasad & Dhouieb, 2008; Borowitz et al, 2009) Alternative airway clearance techniques Positive expiratory pressure (PEP) (Constantini et al, 2003) Physical activity, inhalation therapy and airway clearance (Lannefors et al, 2004) Assisted autogenic drainage (AAD) (Van Ginderdeuren et al, 2003) Today I�m going to introduce to you a case study on an infant with CF. Firstly I wanted to outline the range of airway clearance techniques which physiotherapists can employ in the management of symptomatic infants with cystic fibrosis (CF). Traditionally, conventional chest physiotherapy consisted of twice a day percussion in head-down postural drainage positions. However, in the last decade a lot of research demonstrated an increase in gastro-oesophageal reflux in infants with CF when treated with postural drainage positions incorporating a head-down tilt. This has led clinicians in many centres both in the UK and other countries to avoid this head-down tilt and modify the postural drainage positions. Alternative airway clearance techniques for infants with CF include: The use of the PEP, or positive expiratory pressure mask: In a one year randomised controlled trial in 2003, Constantini et al found PEP was safe and equally effective as postural drainage and percussion in infants with CF. In Sweden in the 1980�s, changes were made to the physiotherapy management of infants with CF, moving to an individualised programme of physical activity, inhalation therapy and airway clearance. Alternatively, Assisted autogenic drainage (AAD) is the adaptation of autogenic drainage for infants and children unable to perform autogenic drainage themselves. Van Ginderdeuren in 2003 evaluated AAD and found there was no provocation of gastro-oesophageal reflux with this technique. Despite the research I�ve just outlined, there isn�t a strong long-term evidence base which fully supports a specific airway clearance technique in the management of infants with CF.Today I�m going to introduce to you a case study on an infant with CF. Firstly I wanted to outline the range of airway clearance techniques which physiotherapists can employ in the management of symptomatic infants with cystic fibrosis (CF). Traditionally, conventional chest physiotherapy consisted of twice a day percussion in head-down postural drainage positions. However, in the last decade a lot of research demonstrated an increase in gastro-oesophageal reflux in infants with CF when treated with postural drainage positions incorporating a head-down tilt. This has led clinicians in many centres both in the UK and other countries to avoid this head-down tilt and modify the postural drainage positions. Alternative airway clearance techniques for infants with CF include: The use of the PEP, or positive expiratory pressure mask: In a one year randomised controlled trial in 2003, Constantini et al found PEP was safe and equally effective as postural drainage and percussion in infants with CF. In Sweden in the 1980�s, changes were made to the physiotherapy management of infants with CF, moving to an individualised programme of physical activity, inhalation therapy and airway clearance. Alternatively, Assisted autogenic drainage (AAD) is the adaptation of autogenic drainage for infants and children unable to perform autogenic drainage themselves. Van Ginderdeuren in 2003 evaluated AAD and found there was no provocation of gastro-oesophageal reflux with this technique. Despite the research I�ve just outlined, there isn�t a strong long-term evidence base which fully supports a specific airway clearance technique in the management of infants with CF.

3. Baby Ruby Diagnosed with cystic fibrosis (CF) at 4 weeks Flatulent bowels and tachypnoea Blue episode � 3 day hospital admission Micro-creon Oral antibiotics Reflux medications Formal airway clearance With the knowledge of this background information on the variety of airway clearance techniques investigated in the physiotherapy management of infants with CF, I am now going to introduce baby Ruby, who was a baby diagnosed with cystic fibrosis (CF) through newborn screening at 4 weeks of age. At her newborn screening visit meeting the CF team for the first time post diagnosis, she was symptomatic with flatulent bowels and tachypnoea. That evening she had a blue episode resulting in a 3 day hospital admission for close monitoring, further education and management. She was started on micro-creon, oral antibiotics, reflux medications and formal airway clearance was taught. With the knowledge of this background information on the variety of airway clearance techniques investigated in the physiotherapy management of infants with CF, I am now going to introduce baby Ruby, who was a baby diagnosed with cystic fibrosis (CF) through newborn screening at 4 weeks of age. At her newborn screening visit meeting the CF team for the first time post diagnosis, she was symptomatic with flatulent bowels and tachypnoea. That evening she had a blue episode resulting in a 3 day hospital admission for close monitoring, further education and management. She was started on micro-creon, oral antibiotics, reflux medications and formal airway clearance was taught.

4. Initial Physiotherapy Management Percussion in modified postural drainage positions (CPT) Exercise and play