A patient has a metered-dose inhaler that contains 200 actuations ('puffs'), and it does not have a dose counter.

He is to take two puffs two times a day.

If he does not take any extra doses, identify how many days will this inhaler last at the prescribed dose.

Choice A rationale

Alpha-1 antitrypsin (AAT) deficiency is an autosomal co-dominant genetic disorder, not strictly recessive. While individuals with two deficient alleles (PiZZ) are at highest risk, heterozygous carriers (PiMZ) also have an increased risk for developing COPD, particularly with environmental exposures like smoking. Therefore, stating children will be at high risk is an oversimplification and potentially inaccurate regarding the genetic transmission pattern.

Choice B rationale

While genetic counseling is an appropriate recommendation for individuals with AAT deficiency to understand inheritance patterns and reproductive risks, it is not the most immediate and direct response regarding the client's personal health implications. The client's question focuses on what the diagnosis means for *them*, not their offspring initially.

Choice C rationale

AAT deficiency significantly increases the risk for developing emphysema, a form of COPD, especially when compounded by environmental factors such as smoking. AAT normally protects lung tissue from elastase, and its deficiency leads to unchecked protease activity and subsequent lung destruction. This response directly addresses the client's personal health risk and a major modifiable factor.

Choice D rationale

This statement is inaccurate. AAT deficiency is not a recessive gene in a simple Mendelian sense where only homozygous recessive individuals are affected. It's an autosomal co-dominant condition where both alleles contribute to AAT levels, and even heterozygous carriers (PiMZ) can have lower AAT levels and increased risk for COPD, especially with smoking. Thus, it certainly impacts health.

Choice A rationale

Black and tarry stools (melena) typically indicate upper gastrointestinal bleeding, where digested blood causes the dark color. This is not a characteristic of steatorrhea, which is caused by the presence of undigested fat. Steatorrhea results from pancreatic enzyme insufficiency, a common feature of cystic fibrosis.

Choice B rationale

Steatorrheaic stools are classically described as frothy, foul-smelling, and fatty due to the malabsorption of fats. The lack of pancreatic lipase, a characteristic of cystic fibrosis, prevents proper fat digestion. This undigested fat is then excreted, leading to stools that are bulky, pale, and often float due to their high fat content.

Choice C rationale

Clay-colored stools are indicative of a lack of bile pigment, suggesting an obstruction of the bile ducts or liver dysfunction. While liver involvement can occur in cystic fibrosis, clay-colored stools are not the primary descriptor for steatorrhea resulting from pancreatic insufficiency.

Choice D rationale

Orange or green stools are not typical descriptions for steatorrhea. Orange stools can sometimes be due to certain medications or foods, while green stools can result from rapid transit through the digestive system, where bile has not had time to be fully broken down. Neither describes the characteristic appearance of fatty stools.