Resources for Physicians - Management and Surveillance
Updated: June 14, 2010
Intravenous Augmentation Therapy
Intravenous augmentation therapy (regular infusion of purified human AAT to augment deficient ATT serum concentrations) has been recommended by the Canadian Thoracic Society for affected individuals whose FEV1 is 35% to 65% of predicted and have never smoked or have quit smoking yet continue to show rapid decline in FEV1 despite optimal medical therapy; however, definitive controlled trials have not been carried out.
Intravenous augmentation therapy is not useful for A1AD liver disease.
It is recommended that patients avoid:
smoking (both personal and passive)
an occupation with exposure to environmental pollutants
exposure to mineral dust, gas, and fumes
Expression of the disorder can be modified in asymptomatic individuals by lifestyle changes, including avoidance of smoking and occupations with exposure to environmental pollutants used in agriculture, mineral dust, gas, and fumes.
Regular exercise and good nutrition are expected to be beneficial in maintaining lung health, as are maintenance of fat-soluble vitamins.
Liver function should be evaluated periodically in all individuals with PI ZZ, including those who did not manifest childhood liver disease.