Pre-hospital management of cystic fibrosis patients presenting with haemoptysis

Cystic fibrosis (CF) is the most life-threatening, inherited recessive genetic disorder affecting Caucasians. The disease occurs equally in both males and females, with each parent of a CF child being a carrier of the CF gene. In Australia one child in 2500 is born with CF (Cystic Fibrosis Victoria (CFV), 2008), which is caused by a mutated gene resulting in abnormal regulation of chloride and sodium transport.The exocrine glands produce thick and viscous secretions with manifestations most apparent within the lungs and digestive system. The mucous in the lungs cause blockages in the airways which are difficult to shift. Gas trapping from mucous plugs lead to frequent infection and irreversible lung damage. Bronchiectasis occurs which involves dilatation of the airways with pooling of infected secretions. Blood vessels may rupture into the airway causing haemoptysis and death.

In the space of 70 years CF has moved from a little known genetic condition, usually fatal in infancy and early childhood, to a multi-system disorder now affecting as many adults as children (Hodson and Bush, 2007). The Australasian Cystic Fibrosis Data Registry in their 10^th annual report recently reported at 31 December 2007 held records for 2639 people with CF, with an average age of 18.2 years. The median age of survival for persons with CF in 2007 was 30.7 years with no deaths reported for persons aged less than 15 years (Cystic Fibrosis in Australia (CFA), 2009). These are promising figures as medical and health services work to improve therapies and treatment plans in order to assist individuals with this chronic disease to have longer survival and greater quality of life. Life expectancy has doubled in the last 25 years, however between 1999 and 2006 life expectancy increased by eight years; from age 29 to 37 (Cystic Fibrosis Foundation (CFF), 2007). Dodge et al report that successive cohorts have realistically indicated that those born in the year 2000 will have a survival over 50 years of age (Dodge et al. 2007), thus emphasising the need for clearer guidelines for clinicians working in the pre-hospital setting. There is no cure for CF, however with better diagnostic tools and increased therapeutic treatment survival is now available. Increased survival brings with it an associated increase in pulmonary complications and disease processes in other organs.