Huntington’s Disease-Related Mortality Patterns: A Two-Decade Analysis of Mortality Trends in the United States, from 1999–2019

Article type: Research Article

Authors: Bin Kashif, Muhammad Arham^{a; 1} | Mahmood, Samar^{a; *} | Saqib, Tahrim^b | Waheed, Syeda Tahira^c | Kumar, Piresh^d | Javaid, Aima^e | Riaz, Muhammad Asjad^f | Fatima, Urooj^g | Nadeem, Zain Ali^f | Nasir, Shahbaz Ali^h | Hassan, Afrahⁱ

Affiliations: [a] Department of Internal Medicine, Dow University of Health Sciences, Karachi, Pakistan | [b] Department of Internal Medicine, Foundation University Medical College, Islamabad, Pakistan | [c] Department of Internal Medicine, Liaquat National Medical College and Hospital, Karachi, Pakistan | [d] Department of Internal Medicine, Bahria University Health Sciences, Karachi, Pakistan | [e] Department of Internal Medicine, Fatima Jinnah Medical University, Lahore, Pakistan | [f] Department of Internal Medicine, Allama Iqbal Medical College, Lahore, Pakistan | [g] Department of Internal Medicine, Karachi Medical and Dental College, Karachi, Pakistan | [h] Department of Internal Medicine, Indus Hospital Kahna Nau, Lahore, Pakistan | [i] Department of Internal Medicine, Jinnah Sindh Medical University, Karachi, Pakistan

Correspondence: [*] Correspondence to: Dr. Samar Mahmood, MD, Department of Internal Medicine, Dow University of Health Sciences, Mission Rd, New Labour Colony, Nanakwara, Karachi, Pakistan, 74200. Tel.: +923322541290; E-mail: [email protected]; ORCID: https://orcid.org/0000-0001-8876-5427.

Note: [1] These authors contributed equally to this work.

Abstract: Background:Huntington’s disease (HD) is an autosomal dominant, progressive neurodegenerative disorder debilitating mainly in adults. Objective:This study aimed to assess the trends in HD-related mortality regarding various demographic factors. Methods:Death certificates from the CDC WONDER were studied from 1999 to 2019, for HD-related mortality in adults aged 25 + years. Age-adjusted Mortality Rate (AAMR) per 100,000 persons and Annual Percentage Change (APC) were calculated and stratified by year, age groups, gender, race/ethnicity, state, census region, urbanization, and place of death. Results:Between 1999 to 2019, 22,595 deaths occurred in adults due to HD. The AAMR increased from 0.43 to 0.54 during this period (APC = 0.50; 95% CI: 0.18 to 0.84). Old adults (65–85 + years) had the highest overall AAMR, followed by middle-aged adults (45–64 years) and young adults (25–44 years) (AAMR old: 1.01 vs. AAMR middle-age: 0.68 vs. AAMR young: 0.16). Men had slightly greater overall AAMRs than women (AAMR men: 0.54 vs. AAMR women: 0.48). When stratified by race, non-Hispanic (NH) Whites had significantly higher mortality rates than NH African Americans (AAMR NH White: 0.61 vs. NH African American: 0.35), while the AAMR were lowest in Hispanic/Latino (0.28). The AAMRs also showed variation by region (overall AAMR: Midwest: 0.63, Northeast: 0.47, West: 0.48, South: 0.46), and non-metropolitan areas had higher HD-related AAMR (0.66) than metropolitan areas (0.47). Conclusions:HD-related mortality in US adults has increased since 1999. Reflecting on the variations in trends observed, new strategies are required to optimize the quality of care in long-term care facilities.

Keywords: Mortality trends, Huntington’s disease, United States, CDC WONDER database, neurodegenerative, autosomal dominant

DOI: 10.3233/JHD-240037

Journal: Journal of Huntington's Disease, vol. 13, no. 3, pp. 329-338, 2024