Patients with CF-related liver disease have a worse disease burden than other CF patients – including more hospitalisations and being at higher risk of other complications such as bone disease and diabetes.
A study from the Australian CF Data Registry compared 166 patients who developed severe liver disease with 166 CF patients who did not have liver disease. The mean age at diagnosis of liver disease was seven years.
It found patients with CF-related liver disease had worse FEV1 at all ages than matched CF patients without liver disease.
Hospitalisations, for both respiratory and non-respiratory indications, were also more frequent in the patients with liver disease.
Patients with liver disease were more likely to require nasogastric nutritional supplementation than other patients (12.6% v 5.4%; p=0.03).
There was no significant difference in the frequency of lung transplants or the mean age at transplantation in the two patient groups.
However the study found significantly higher rates of endocrine-related comorbidities – osteoporosis (16.2% v 8.4%), osteopenia (26.5% v 16.8%) and CF-related diabetes (38.5% v 19.2%) – in patients with liver disease.
“Our study findings, to our knowledge, is the first to link CFLD to poor bone health in CF and may suggest that the presence of CFLD has an additive detrimental effect on bone health,” the study said.
“The increased prevalence of endocrine complications in those with severe CFLD suggest consideration for earlier endocrine screening, including earlier assessment of bone disease and CFRD [cystic fibrosis-related diabetes] in those affected by severe CFLD.”
Senior investigator Associate Professor Keith (Chee) Ooi told the limbic the findings were especially important given the increasing life expectancy of patients with CF.
The latest data from the Registry shows the median survival of patients with CF is now 47 years.
“Most of our patients we expect now to transition into adult care. So we now have diseases associated with ageing such as bone health and endocrine issues,” he said.
“Everyone recognises diabetes is now a major problem in CF. Bone health is still an emerging topic. I think hopefully with this paper we can say you need to start screening now.”
“Most CF centres have a screening process but it’s not always followed.”
He said the data showed there could be some risk stratification – with patients affected by liver disease possibly requiring closer monitoring.
“The exciting thing about CF is the range of new medications coming through that try to correct the actual defect. We don’t know whether the nature of CF will change when these drugs are introduced.”
He said it was currently difficult to predict or prevent which patients would develop CF-related liver disease.