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Cystic Fibrosis



how do you know when cystic fibrosis is in its advanced stage in an adult, what are some of the things that would occur


Every patient with cystic fibrosis is different, so each patient must be considered individually. I am assuming that this question relates to CF lung disease.


The most common indicator of advancing disease in CF is the FEV1 on the pulmonary function test. Increasing frequency of admissions or need for antibiotics, inability to gain and maintain weight, decrease in exercise tolerance, worsening evidence of bronchiectasis on chest Xray or CT scan may also suggest progression of CF pulmonary disease. Patients may have worsening of complications such as CF related diabetes control, CF related arthritis, osteoporosis, etc.


With very severe disease, patients develop an oxygen requirement and CO2 on a blood gas may increase, suggesting need for help with breathing, particularly at night, using bi-level ventilation. This is a machine with a face mask that helps supplement a patient’s breathing so that they do not need to work as hard. At this point, lung transplant is a consideration.

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