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October 28, 2021
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A rare disease diagnosis comes with significant health care costs, study shows

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People with a rare disease paid three to five times more in health care costs than people without a rare disease, according to findings published in the Orphanet Journal of Rare Diseases.

“The name ‘rare diseases’ is actually a little misleading because there are between 7,000 to 10,000 different rare diseases and, collectively, they affect about 25 to 30 million people in the U.S., probably more. That’s about one in 10 people,” Anne Pariser, MD, director of the Office of Rare Diseases Research within the National Center for Advancing Translational Sciences (NCATS), told Healio Primary Care.

Graphical depiction of data included in article.
Tisdale A, et al. Orphanet J Rare Dis. 2021;doi:10.1186/s13023-021-02061-3.

As part of the Impact of Rare Diseases on Patients and Healthcare Systems, a retrospective pilot study, Pariser and colleagues quantified direct medical costs for people with rare diseases using medical and insurance records within NCATS and the Eversana Health Care System. They looked specifically at costs associated with 14 rare diseases using International Classification of Diseases codes. The researchers also included a control population without diagnoses of a rare disease.

Overall, Pariser and colleagues found that costs associated with a rare disease diagnosis were 1.5 to 23.9 times higher than costs for the control population.

Anne Pariser

Using the Eversana database, which included commercial and public insurance coverage from 2006 to 2020, the researchers found that per patient per year (PPPY) costs ranged from $8,812 to $140,044 for patients with a rare disease compared with $5,862 for the control population. The diseases with the highest PPPY were urea cycle disorder ($140,044), Lennox-Gastaut syndrome ($55,494) and Batten disease ($38,569), while the diseases with the lowest PPPY were eosinophilic esophagitis ($8,812), hereditary hemorrhagic telangiectasis ($14,265) and sickle cell disease ($15,700), according to Pariser and colleagues.

Using the NCATS database, which included almost exclusively Medicaid data from 2007 to 2012, PPPY costs for patients with a rare disease ranged from $4,859 to $18,994 compared with $2,211 for the control population. The diseases with the highest PPPY were for mitochondrial neurogastrointestinal encephalopathy ($18,722), urea cycle disorder ($11,937) and muscular dystrophy ($13,337), while the diseases with the lowest PPPY were eosinophilic esophagitis ($4,859), hereditary hemorrhagic telangiectasia ($5,590) and pheochromocytoma ($6,265).

Although the PPPY estimates differed in the two databases, costs for patients with a rare disease were consistently higher than those of the control group, the researchers noted.

The weighted average cost using the Eversana database for a patient with a rare disease was estimated to be $16,644 compared with $5,862 for the control group. Using the NCATS data, the weighted average annual costs were $10,695 compared with $2,211 for the control group. With these estimates, total direct medical costs for an estimated 25 million patients with rare diseases in the U.S. approach $400 billion per year, “making the cost burden similar to other high-cost diseases, such as cancer and heart failure, and exceeding those of Alzheimer’s disease,” according to Pariser and colleagues.

The findings “suggest that rare diseases represent a major impact to public health, have high unmet medical needs, and that there is an urgent and considerable need for earlier and accurate rare disease diagnosis and intervention to address medical management for rare disease patients,” Pariser and colleagues wrote.

For primary care physicians specifically, “our message is to just consider the possibility of a rare disease or a genetic disease” and recommend patients for further evaluation sooner, Pariser said.

References:

NIH study suggests people with rare diseases face significantly higher health care costs. https://www.nih.gov/news-events/news-releases/nih-study-suggests-people-rare-diseases-face-significantly-higher-health-care-costs. Published Oct. 22, 2021. Accessed Oct. 22, 2021.

Tisdale A, et al. Orphanet J Rare Dis. 2021;doi:10.1186/s13023-021-02061-3.