Woman, 76, oldest patient in Ireland diagnosed with cystic fibrosis

A 76-year-old woman has become the oldest patient in Ireland diagnosed with cystic fibrosis, a genetically inherited disease that primarily affects the lungs and digestive system.

The family of the woman, who was diagnosed at Cork University Hospital, has since undergone genetic counselling.

A report on the case, published in the September edition of the Irish Medical Journal, said the woman was referred to CUH’s department of respiratory medicine “for assessment of possible bronchiectasis,” an uncommon lung disease.

The woman reported “a history of recurrent childhood pneumonia, asthma and had a remote smoking history.” She also had a daily cough, producing yellow phlegm and frequent hospital admissions with lower respiratory tract infection.

The hospital carried out a battery of tests and “based on the clinical findings and investigations, a diagnosis of Cystic Fibrosis was firmly established,” the authors said.

“At 76 years of age, this patient represents the oldest ever first presentation of CF in Ireland.”

The woman has since been attending the adult CF centre at CUH for three-monthly outpatient follow-ups and receives “earlier and more aggressive antibiotic therapy” for worsening lung function due to infection or inflammation.

The doctors said the case highlights that in those with “appropriate clinical findings” investigations to exclude CF should be carried out “at any age.”

Some CF suffers “with milder phenotypes” “can expect to live a normal lifespan,” they point out.

CF is the most common fatally inherited genetic condition in Ireland, with a carrier frequency of 1:18 and incidence of 1:1353, the highest worldwide. The report’s authors said the majority of patients with CF are detected in infancy (particularly following the introduction of newborn screening in Ireland in 2011), however due to improved diagnosis, more patients are being diagnosed in adulthood.

US and British registry data reveal the oldest patients with CF in these countries to be 82 and 79 years respectively; the oldest patient previously recorded here was 61.

It also says CF patients diagnosed in adulthood “had similar lung function decline, and died from similar causes, compared to those diagnosed in childhood”

The woman in this case “remains stable” at age 78.