Hemophilia in the Elderly in India: Obstacles to Overcome and Methods for Providing Comprehensive Care

April 17, 2023 is World Hemophilia Day. The theme of the event this year is “Access for All: Prevention of bleeds as the global standard of care”.?Building on last year’s theme, the call to action for the community in 2023 is to come together and advocate with local policy makers and governments for improved access to treatment and care with an emphasis on better control and prevention of bleeds for all people with bleeding disorders (PWBDs). This means the implementation of home-based treatment as well as prophylactic treatment to help those individuals have a better quality of life.?

Abstract

Hemophilia is an extremely uncommon inherited bleeding illness that affects males more frequently than females. The number of elderly people in India who have Hemophilia is growing in tandem with the country's rising life expectancy rates. This creates difficulties that are unlike any others in the administration and care of this group. The purpose of this article is to provide an overview of the prevalence of hemophilia in senior citizens in India, as well as the problems and techniques for controlling the condition. It highlights the significance of comprehensive care, individualised preventive regimens, pain management, support for mental health, as well as education and awareness, in order to improve the quality of life for elderly people in India who have Hemophilia. The development of comprehensive care models, the identification of barriers to care, and the evaluation of the efficacy of interventions to help vulnerable populations should be the focus of more research in the future.

Introduction

Hemophilia is a rare inherited bleeding illness that is connected to the X chromosome. It affects roughly 1 in 10,000 males around the world. The disorder is characterised by a shortage of coagulation factors VIII (hemophilia A) or IX (hemophilia B), which can result in spontaneous bleeding episodes as well as persistent bleeding following injury or surgery. The life expectancy of persons who have hemophilia has greatly increased as a result of advancements in treatment and prevention, which has led to an increase in the number of elderly people who have the disorder.

In India, the prevalence of hemophilia is believed to be 1 in 5,000 males, with over 18,000 recognised cases and many more undiagnosed cases and many more undiagnosed. According to research published in the Indian Journal of Hematology and Blood Transfusion, the percentage of senior citizens in India who are affected with Hemophilia is projected to increase from 5% in 2016 to 9% in 2026. The Indian population is getting older, and as a result, healthcare practitioners and governments have a responsibility to acknowledge and treat the specific needs and experiences of elderly people who have hemophilia. This article presents a comprehensive analysis of the difficulties experienced by elderly people in India who have hemophilia, as well as recommendations for effectively managing these difficulties.

Prevalence and Obstacles to Overcome

It is anticipated that the number of elderly people in India living with hemophilia will rise as a result of developments in therapy as well as the country's overall ageing population. Those in their senior years who have Hemophilia have a number of obstacles, some of which are connected to age-associated comorbidities, limited access to specialised care, and psychosocial concerns.

In elderly patients with hemophilia, age-related concomitant illnesses such as cardiovascular disorders, renal failure, and osteoporosis are frequent. Comorbidities like these can make bleeding episodes worse and raise the risk of consequences.

In India, elderly people living with Hemophilia face a substantial obstacle in the form of restricted access to specialised medical care. Because there are not enough specialised Hemophilia treatment clinics, particularly in rural regions, patients often receive inadequate prophylaxis, suffer from delayed diagnosis, and have difficulty properly managing consequences.

The mental health and quality of life of elderly people who have hemophilia can be significantly impacted by a number of psychosocial issues, including feelings of social isolation, despair, and anxiety. A lack of understanding of the ailment, as well as cultural factors, might make these problems even worse.?

Treatment

The primary treatment for hemophilia is replacement therapy, which involves the infusion of the missing clotting factor. The type and frequency of treatment depend on the severity of the disorder and the frequency and severity of bleeding episodes. This involves administering the deficient clotting factor, such as Factor VIII (FVIII) for hemophilia A or Factor IX (FIX) for hemophilia B, to prevent and treat bleeding episodes [2].

There are two main types of replacement therapy for hemophilia: clotting factor concentrates and fresh frozen plasma (FFP). Clotting factor concentrates are the preferred treatment for hemophilia because they are more effective, safer, and easier to administer. Clotting factor concentrates are manufactured from human plasma or recombinant DNA technology and are available as a powder that is mixed with sterile water before infusion.

There are several methods to treat hemophilia, including:

• Desmopressin: This medication is effective in treating mild hemophilia A and helps to avoid the use of FVIII concentrates. Desmopressin reduces blood loss and promotes clotting by increasing the levels of clotting factors in the blood [1]. Desmopressin (DDAVP)is a synthetic hormone that stimulates the release of von Willebrand factor and clotting factor VIII, which can temporarily increase clotting factor levels in people with mild to moderate hemophilia A.
• Prophylaxis: This preventive treatment involves regular infusions of the deficient clotting factor to minimize bleeding episodes and reduce the risk of long-term joint damage [3].
• Management of bleeding episodes: Prompt treatment of bleeding episodes is crucial for managing hemophilia. This may involve administering the appropriate clotting factor concentrate, applying pressure to the bleeding site, or using other supportive measures [2].
• Treatment of FVIII inhibitors: In some cases, the immune system may develop antibodies (inhibitors) against the infused clotting factor, rendering the treatment less effective. In such cases, specialized therapies, such as immune tolerance induction or bypassing agents, may be used to manage the condition [3].
• Antifibrinolytic medications: medications that prevent the breakdown of clots, which can be used to prevent or treat bleeding episodes.
• Gene therapy: a promising treatment that involves the introduction of a functional copy of the missing clotting factor gene into the patient's cells, potentially providing a long-term cure for hemophilia.
• Physical therapy: exercises and therapies that can help prevent joint damage and maintain joint mobility in people with hemophilia.
• Surgery: may be necessary in severe cases of joint damage or to remove or repair damaged tissue following a severe bleeding episode.

The treatment approach for hemophilia is highly individualized and depends on several factors, including the type and severity of the disorder, the age of the patient, and the presence of any comorbidities. The goal of treatment is to prevent bleeding episodes, minimize complications, and improve the quality of life of people with hemophilia.

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Hemophilia Treatment for Elderly Patients

Comprehensive care is absolutely necessary in order to fulfil the specific requirements of elderly people living with Hemophilia in India. This comprises individualised preventative treatment regimens, pain management, support for mental health, and educational and awareness efforts.

• Individualized Prophylactic Regimens: Seniors who have Hemophilia require individualised prophylactic regimens in order to account for age-related comorbidities and potential drug interactions. When deciding what prophylaxis to give a patient, medical professionals should take into account the patient's physical state, as well as any co-existing conditions and previous experiences with bleeding.
• Pain Management: Due to the recurrent bleeding episodes and joint damage that are associated with hemophilia, chronic pain is a prevalent issue that affects older people with the condition. It is absolutely necessary to take a multidisciplinary strategy to the management of pain, one that incorporates both pharmaceutical and non-pharmacological interventions, in order to achieve an improvement in quality of life.
• Support for Mental Health: Seniors with Hemophilia may struggle with feelings of depression and anxiety, in addition to social isolation. Providers of healthcare and professionals working in the field of mental health should work together to provide comprehensive mental health assistance and to encourage social participation.
• Education and Awareness: It is essential to enhance timely diagnosis, proper management, and social support by increasing awareness of Hemophilia among senior citizens, their families, and healthcare providers. To guarantee that people from all backgrounds have equal access to knowledge and resources, educational activities should focus on both urban and rural locations.

Conclusion

The increasing number of elderly people in India who suffer from Hemophilia poses new issues, the proper management of which requires the development of specific management strategies. In order to improve the quality of life for elderly people in India who are living with hemophilia, it is vital to provide them with comprehensive treatment, individualised preventive regimens, pain management, support for mental health, as well as education and awareness. The development of comprehensive care models, the identification of barriers to care, and the evaluation of the efficacy of interventions to help vulnerable populations should be the focus of more research in the future.?

References:

[1] Consensus Review of the Treatment of Cardiovascular ...

[2] Guidelines for the management of hemophilia - Srivastava

[3] Hemophilia A (Factor VIII Deficiency) Treatment & ...