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Authors

  1. Lomas, Paula Harff MAS, RN, CCRP
  2. Fowler, Susan B. PhD, RN, CNRN

Abstract

Objective: A survey was conducted to estimate how many adult patients with cystic fibrosis who receive care at centers accredited by the Cystic Fibrosis Foundation (CFF) also have biological children with the disease.

 

Methods: A six-item questionnaire was sent to 144 CFF-accredited care centers and satellites. After one month, a reminder e-mail was sent to the nonresponding centers.

 

Results: A total of 119 responses were received. Forty (34%) of the responding centers reported having at least one and as many as five patients with cystic fibrosis who also had biological children with the disease. A total of 66 such patients were identified. Of these, 39 (59%) were newly diagnosed themselves as a consequence of prenatal testing or a child's diagnosis.

 

Conclusion: Despite some study limitations, these findings show that a number of people with cystic fibrosis are having children who also have the disease. Moreover, many parents are learning that they have cystic fibrosis only after their child is diagnosed. Further research is needed to better estimate both the proportion of adults with cystic fibrosis who are having children with the disease and the proportion late-diagnosed, as well as to explore the psychosocial needs of these patients and their children.

 

Keywords: cystic fibrosis, cystic fibrosis research, family planning, genetic testing, carrier testing, newborn screening