Reduce risk

1. Smoking cessation

Smoking accelerates lung function decline in patients with alpha-1-antitrypsin (A1AT) deficiency (also known as alpha-1)1

Mean annual decline in FEV1 in 208 never-smokers, 697 ex-smokers, and 22 current smokers with severe alpha-1.1

Why is smoking cessation so important?

  • Smokers with even 1 deficient allele are at increased risk of developing COPD2
  • Smoking is also the most important risk factor for the development of emphysema in alpha-1 patients with the Pi ZZ allele3
  • Smoking is a key factor in determining the extent of lung involvement in alpha-1. When compared to ex-smokers or non-smokers, smokers with alpha-1 related emphysema have3
    • Greater lung function decline (FEV1)
    • Higher mortality rates
  • Alpha-1 patients (FEV1 <50%) who quit smoking had a better survival rate than patients who continued to smoke4

2. Reduce other exposures3,5,6

Patients with alpha-1 should avoid persistent exposure to hazardous respiratory environments that can cause respiratory irritation.

  • This includes exposure to household and occupational dusts, fumes, and gases and to indoor and outdoor air pollutants
  • Avoidance of exposure should be taken into account when choosing an occupation

3. Immunize3,5

To prevent infection, the following vaccinations are recommended for alpha-1 patients with COPD:

  • Pneumococcal vaccination: recommended for COPD patients ≥65, and younger COPD patients with certain comorbid conditions; shown to reduce the incidence of community-acquired pneumonia in COPD patients younger than 65 with a predicted FEV1 <40%
  • Influenza vaccination: shown to reduce the risk of serious illness (such as serious lower respiratory tract infections) and death in patients with COPD

Smoking cessation is the most important preventive measure in the development of lung disease. Secondhand smoke should also be avoided5,6

Like smoking cessation, avoidance of exposure to hazardous respiratory environments may improve the prognosis of patients with alpha-13,5

References

  1. The Alpha-1-Antitrypsin Deficiency Registry Study Group. Survival and FEV1 decline in individuals with severe deficiency of alpha-1 antitrypsin. Am J Respir Crit Care Med. 1998;158(1):49-59.
  2. Silverman EK, Sandhaus RA. Alpha1-antitrypsin deficiency. N Engl J Med. 2009;360:2749-2757.
  3. American Thoracic Society/European Respiratory Society statement: standards for the diagnosis and management of individuals with alpha-1 antitrypsin deficiency. Am J Respir Crit Care Med. 2003;168:818-900.
  4. Seersholm N, Kok-Jensen A. Survival in relation to lung function and smoking cessation in patients with severe hereditary Alpha1-antitrypsin deficiency. Am J Respir Crit Care Med. 1995; 151:369-373.
  5. Global Initiative for Chronic Obstructive Lung Disease. Global strategy for the diagnosis, management, and prevention of chronic obstructive pulmonary disease. 2016. http://goldcopd.org/global-strategy-diagnosis-management-prevention-copd-2016/. Accessed July 14, 2016.
  6. Bals R, Kohnlein T. Alpha-1 antitrypsin deficiency. Stuttgart: George Thieme Verlag KG; 2009.

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