The Science Journal of the American Association for Respiratory Care

2009 OPEN FORUM Abstracts

ALPHA-1 ANTITRYPSIN DEFICIENCY TESTING AND EDUCATION AS COMPONENTS OF A PULMONARY REHABILITATION PROGRAM

Valerie F. Naegele1, James J. Barnett2; 1Tri-City Medical Center, Oceanside, CA; 2Mission Hospital, St Joseph Health System, Mission Viego, CA

Background: The hereditary disorder alpha-1 antitrypsin (AAT) deficiency, which may lead to severe respiratory disease, is underrecognized. The ATS has recommended that all symptomatic individuals with COPD are tested for AAT deficiency. Without diagnosis, individuals with AAT deficiency are unable to receive appropriate management and medication. In the course of their everyday provision of respiratory care, RTs are ideally placed to increase the numbers identified with AAT deficiency and provide education to those affected. Here we describe our pulmonary rehabilitation program, which includes AAT deficiency testing as an established component. Method: We run six pulmonary rehabilitation programs every year. Each program runs twice a week for 7 to 8 weeks. The foremost components of the program are exercise and education. The exercise element consists of low-impact, cardiovascular, and strength training, which progresses at each session. Educational topics include the causes and treatments of COPD and other restrictive lung disorders; managing chronic dyspnea on a daily basis — incorporating breathing, airway clearance, and relaxation techniques, stress and panic control, boosting the immune system, nutrition, sexuality, travel, and activities of daily living; appropriate use of bronchodilators, anti-inflammatories, inhalers/dry powder, nebulizers, oxygen, and CPAP; when to call the doctor; and end-of-life decisions. AAT deficiency and its implications are discussed, and testing is now offered to all individuals who attend our pulmonary rehabilitation program and Better Breathers’ Clubs. Each patient tested is given a brochure about AAT deficiency. Results: From January to June 2009 we have tested 45 individuals from our pulmonary rehabilitation programs and Better Breathers’ Clubs at Oceanside and Mission Viejo, CA. Two individuals positive for AAT deficiency (both with genotype PiSnull) and one heterozygote (carrier) with genotype PiMS were detected. Conclusions: The testing of individuals who attend pulmonary rehabilitation programs and Better Breathers’ Clubs has resulted in the detection of individuals positive for AAT deficiency. The identification of these patients during pulmonary rehabilitation programs or Better Breathers’ Clubs facilitates the early detection of AAT deficiency and thereby enables more effective management of patients with the disorder. Sponsored Research - None

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