Risk to Family Members
We receive half of our genes from our mother and the other half from our father.
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Parents of a patient with the diagnosis of FPF
Most individuals diagnosed with FPF have a affected parent.
Although most individuals diagnosed with FPF have an affected parent, the disorder may appear to have skipped a generation (i.e., the individual diagnosed has an affected grandparent) because of reduced penetrance.
Siblings of a patient
The risk to the siblings of the proband depends upon the genetic status of the patient’s parents.
If a parent of the patient is affected or known to have a disease-causing mutation on the basis of family history, the risk to the sibs of inheriting the mutation is 50%. Because the penetrance is likely reduced, the risk to sibs of being affected is less than 50%.
Children of a patient
Other family members of a patient
Screening of Relatives at Risk
We recommend that every five years, asymptomatic first-degree relatives (of individuals with FPF) older than age 50 years should undergo screening for pulmonary fibrosis.
Screening can include:
Pulmonary function tests
High-resolution CT (HRCT) images of the chest to detect early abnormalities
Complete a standardized questionnaire to assess the presence of respiratory symptoms.
Up to 50% of unaffected at-risk family members have a positive screen and require further evaluation.