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Hemophilia Life Expectancy

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Hemophilia Life Expectancy

Over the past two decades, novel therapeutics have enhanced the life expectancy of hemophilia patients tremendously. Although hemophilia is a rare, inherited blood disorder with no particular cure, alternative treatments have improved the quality of life for hemophilia patients. 

 

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Hemophilic individuals typically bleed longer than healthy individuals due to a deficiency of blood clotting proteins. The amount of blood clotting proteins is linked to the severity of this bleeding disorder. Excessive bleeding can reduce life expectancy and impair the quality of life. 

The life expectancy of hemophilia patients may vary according to the type of hemophilia (A, B, or C) a patient has and the disease stage they are currently in. Hemophilia can be mild, moderate, or severe. Generally, about 70% of individuals with hemophilia suffer from a severe form of the disease.

 

Hemophilia Life Expectancy Without Treatment

The survival rate in hemophilic patients rapidly declines if not given adequate treatment. Without treatment, they are more likely to succumb to the disease before adulthood due to bleeding–related incidents such as internal bleeding in vital organs or intracranial bleeding (bleeding in the skull).

Back in the 1960s, before the revolutionary development of clotting factors concentrates (CFC), the average life expectancy of male patients suffering from severe hemophilia was merely 11 years old, and they usually passed away before they hit puberty.

When it comes to the overall mortality rate, the death rate of patients with mild hemophilia is twice that of healthy male populations. If a person has severe hemophilia, the death rate is likely to be four to six times higher than the general population.

 

Hemophilia Life Expectancy With Treatment

Smiling hemophilia patient with improved life expectancyThe advancement in hemophilia treatment techniques, such as replacement therapy, clotting factor concentrates infusions, home care treatment, and immunosuppressant therapy, has significantly lengthened life expectancy and improved the quality of life for hemophilia patients. 

With proper treatment and comprehensive care at the initial stage after diagnosis, the patient can expect to live a relatively ordinary life. The life expectancy of hemophilia patients who are properly treated is only 10 years shorter than that of the general male population. 

Statistics of Hemophilia Life Expectancy After Proper Treatment

The statistics of hemophilia survival rates have considerably improved to the point where some studies reported a near-normal life expectancy for patients with mild hemophilia.  

 

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Researchers recorded the median life expectancy of hemophilia patients in the British male population. According to their survey analysis, male patients suffering from the severe stage of hemophilia had a life expectancy of 63 years. Those with mild to moderate hemophilia had a life expectancy of 75 years, compared to the overall British male population, which had a life expectancy of 78 years at that time.

Likewise, based on the observational cohort study conducted on 1031 Dutch male population with hemophilia from 2001-2018, the median life expectancy was 77 years compared to the general healthy Dutch male population with a life expectancy of 83 years

On the other hand, life expectancy can be reduced for a person undergoing hemophilia treatment if infected with HIV or hepatitis C. The estimated life expectancy of mild and moderate hemophilic patients without HIV or hepatitis C virus (HCV) has been reported at 75 years in the previously mentioned Dutch study. The mortality rate for patients with non-severe hemophilia co-infected with HIV was 19% higher than the general population, mainly due to excessive bleeding and HIV-related diseases. 

Undoubtedly, on-time diagnosis and effective replacement therapy not only reduce the recovery time and hospital stay but also improve the patient’s life span. In developed countries, the life expectancy of hemophilia patients with no other health condition is almost the same as the overall general population due to continuous advancements in treatment resources and healthcare access. 

 

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REFERENCES:

  1. Kloosterman, F., Zwagemaker, A. F., Abdi, A., Gouw, S., Castaman, G., & Fijnvandraat, K. (2020). Hemophilia management: Huge impact of a tiny difference. Research and Practice in Thrombosis and Haemostasis4(3), 377-385.
  2. Mejia‐Carvajal, C., Czapek, E. E., & Valentino, L. A. (2006). Life expectancy in hemophilia outcome. Journal of Thrombosis and Haemostasis4(3), 507-509.
  3. Darby, S. C., Kan, S. W., Spooner, R. J., Giangrande, P. L., Hill, F. G., Hay, C. R., … & Williams, M. (2007). Mortality rates, life expectancy, and causes of death in people with hemophilia A or B in the United Kingdom who were not infected with HIV. Blood, The Journal of the American Society of Hematology110(3), 815-825.
  4. Hassan, S., Monahan, R. C., Mauser‐Bunschoten, E. P., van Vulpen, L. F., Eikenboom, J., Beckers, E. A., … & Gouw, S. C. (2021). Mortality, life expectancy, and causes of death of persons with hemophilia in the Netherlands 2001–2018. Journal of Thrombosis and Haemostasis19(3), 645-653.
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