People with acute hepatic porphyria (AHP) are more likely to file for long-term sick leave and disability pension, but are not at more risk of premature death than the general population, a nationwide study from Norway found.
The study, “Sick leave, disability, and mortality in acute hepatic porphyria: a nationwide cohort study,” was published in the Orphanet Journal of Rare Diseases.
AHP comprises three metabolic disorders — acute intermittent porphyria, variegate porphyria, and hereditary coproporphyria — all caused by abnormalities in the production of heme, a molecule essential for red blood cells to transport oxygen.
AHP is characterized by acute attacks of severe abdominal pain, nausea, vomiting, and general malaise that may require hospitalization. Those experiencing symptoms typically report low health-related quality of life and frequent need for long-term sick leave and disability pension. Patients with severe and recurrent acute attacks also may experience debilitating manifestations between attacks, such as chronic pain and fatigue, as well as high levels of unemployment.
However, it is still unclear if rates of unemployment and access to long-term sick leave and disability pension reported in AHP patients are higher than those of the general population.
Researchers in Norway conducted a nationwide study to address this question, while also evaluating the risk of premature death compared with the general population.
The study was based on data from 333 patients with a confirmed diagnosis of AHP, whose records were pulled from the Norwegian Porphyria Centre (NAPOS) database and linked to several other national compulsory registries. It also included data from 5,819,937 individuals from the general population, which served as a reference for comparison.
Results revealed that compared with individuals from the general population, AHP patients were 1.5 times more likely to file for long-term sick leave and 1.9 times more likely to request disability pension.
These increased rates corresponded with disease severity, with patients who had been hospitalized at least once due to acute attacks having the highest risks (2.1 times more likely to file for sick leave; and four times more likely to request disability pension). No increased likelihood was found for AHP mutation carriers who were still asymptomatic.
Investigators also found that AHP patients requested access to a disability pension at a median age of 45, approximately 21 years earlier than individuals from the general population. This was in line with a prior study from Sweden, which found that about 20% of symptomatic AHP patients accessed long-term sick leave or disability pension at a mean age of 45.
Analyses also revealed that although AHP was linked to a higher risk of mortality due to hepatocellular carcinoma (HCC), patients were not at a higher risk of dying prematurely than individuals from the general population.
“In summary, we demonstrated in a large population-based cohort study that persons with symptomatic AHP were at increased risk of accessing long-term sick leave and disability pension due to their porphyria. This risk increased with AHP severity,” the researchers wrote.
“Although the risk of dying from HCC was found to be increased in symptomatic AHP, we found no evidence of overall increased risk of premature death in persons with symptomatic or asymptomatic AHP,” they added.
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