A 76-YEAR-old woman diagnosed with cystic fibrosis, has become the oldest ever first presentation case in Ireland.
The majority of patients with CF are detected in infancy, however due to the increased recognition of milder phenotypes of the condition, more patients are being diagnosed in adulthood.
In a study published in the Irish Medical Journal this week, researchers from the University College Cork (UCC) Cystic Fibrosis Centre explain that the elderly woman was referred for assessment of possible bronchiectasis. She reported a history of recurrent childhood pneumonia, asthma and had a remote smoking history.
She had a daily cough and frequent hospital admissions with lower respiratory tract infections. Though initial tests and examinations on her chest were normal, a chloride sweat test resulted in a firm diagnosis of CF. People with CF have a much higher level of chloride in their sweat and the UCC paper points out that it remains the “gold standard” for diagnosis worldwide.
At 76, this patient represents the oldest ever first presentation of CF in Ireland. Diagnosis of the condition in adult patients was first reported in the medical literature in teh 1970s. US and UK registry data reveal the oldest patients with CF in these countries are 82 and 79 years respectively.
CF patients diagnosed in adulthood had similar lung function decline, and died from similar causes, compared to those diagnosed in childhood. Importantly, this highlights the morbidity of CF lung disease in this group and that it does not run a benign course.
UCC researchers said the patient in this case remains stable at 78-years-old and the diagnosis allows for her care to be coordinated by the university’s adult care centre. She now attends for three monthly outpatients follow-ups, receives earlier and more aggressive antibiotic therapy for pulmonary exacerbations and genetic counselling has been provided to her family.
“Our case report highlights that in those with appropriate clinical findings, investigations to exclude CF should be instigated at any age,” commented UCC authors.
They also said illustrates how adults with milder phenotypes of CF can expect to live a normal lifespan.
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