About the Authors
Dr. Avinash Tiwari is a Specialist palliative medicine physician at AIIMS, Raipur.
Dr. Prasad Dange is the In-charge, Clinical Heametology at AIIMS, Raipur.
Inherited bleeding disorders represent a rare but a challenging area which requires a multi-disciplinary team approach to provide comprehensive patient care. Hemophilia is one such disorder in which Hemophilia A is one of the commonest bleeding disorders and is about 5 times more prevelant than Hemophilia B. Spontaneous bleeding is seen in severe forms of this disorder with the joints (especially the knee and hip), skeletal muscles and subcutaneous tissues being the most common sites for spontaneous bleeding in severe haemophilia. Life threatening bleeds like massive gastro-intestinal bleed or intra-cranial bleeding are however uncommon. Recurrent bleeds lead to inflammatory changes in the joint that ultimately causes permanent deformity. Timely factor infusion therefore forms the backbone of Haemophilia care and deformity prevention. A Multi-disciplinary team involving a haematologists, physicians, palliative medicine physicians, physiotherapists and orthopaedic surgeons are crucial to provide comprehensive care, as per the recommendations from the World Federation of Hemophilia (WFH).
Though under-reported, it is estimated that India has about 70,000 patients with Hemophilia A and B. In addition to the financial burden that haemophilia imposes, it also impacts the family in terms of emotional and social distress. India, currently lacks a well structured national policy to address patients with Hemophilia; with some states however initiating service provision through the National Health Mission (NHM) pool. Though factor VIII concentrates are included in the Essential Drug Lists, it’s availability remains poor due to it’s high cost.
Role of Palliative Care in hemophilia
To be able to provide optimal treatment and comprehensive care for people with hemophilia, especially those suffering from a severe form of the disorder, a multidisciplinary team of specialists including a Palliative Care Specialist is needed for the effective management of the condition, it’s complications and associated suffering.
The World Federation of Hemophilia (WFH) describes that the principles of care in hemophilia to include:
- National coordination and delivery of hemophilia care
- Provisioning comprehensive care for physical health, psychosocial well-being, and improving the quality of life for people with hemophilia, to reduce morbidity and mortality
- Design family-centred care, particularly for the diagnosis and management of carriers
The Quality-of-life outcomes for people with hemophilia encompass:
- Prevention of bleeding and joint damage
- Prompt management of bleeding episodes including follow-up physical therapy and rehabilitation
- Appropriate emergency care
- Appropriate pain management
- Management of musculoskeletal complications
- Management of co morbidities
- Regular psychosocial assessment and support as needed
- Ongoing education on treatment and self-care for people and families living with hemophilia
- Shared decision-making to obtain their concurrence with short and long-term treatment and management plans
Pain management – As acute and chronic pain is common in people with hemophilia, a proper assessment of the cause of pain becomes essential. The WFH recommends the below guidelines for effective pain management.
Pain Management Strategies for people with Hemophilia
Acute and emergency care for bleeds – People with hemophilia need immediate access to emergency medicines and treatment as well as to specialist care at hospital emergency departments. The limited experience and knowledge of hemophilia management among the medical professionals, particularly in the emergency departments, may lead to serious treatment-related complications. Treatment centres should therefore develop protocols for emergency care for people with hemophilia, including those with inhibitors that include management of serious acute complications such as massive intracranial hemorrhage (ICH) and other types of major internal hemorrhage that further warrants integration of palliative care.
Provide psychosocial support – The psychosocial impact of these compounding factors may result in:
- Loss of time from school or work
- Limited participations in sports activities
- Decreased socialization and/or increased isolation
- Negative self-perceptions related to body image, masculinity, and/or self-esteem
- Lack of a sense of normalcy
- Limited physical flexibility with sexual positioning
- Challenges in personal relationships
- A role loss and/or role changes
- Increased fatigue
- Negative coping behaviours
For those patients with hemophilia and for those with chronic musculoskeletal pain or functional limitations, the WFH recommends specific individualized psychosocial assessments and intervention strategies (psychosocial counseling, educational and employment counseling, and financial planning) which helps patients achieve a better quality of life.
Ongoing psychosocial assessment and counseling should be integrated within case management and comprehensive care for carriers as well. Carriers may experience a wide range of emotional and psychosocial impacts, including feelings of guilt, sorrow, and self-blame related to reproductive choices or consequences such as passing on their genetic variant. Such feelings run across generations of a family and may also be experienced by grandmothers who were carriers and fathers with hemophilia. These carriers may therefore require a referral to psychosocial professionals (e.g., psychologists) for further support to address psychological or emotional issues that may arise during the genetic counseling process or at different life stages. The collaboration between psychosocial professionals and genetic counselors can enhance the overall patient care.
Patient/caregiver education – Ongoing patient and family caregiver education and psychosocial support are essential components of the management of hemophilia patients given the complexity and challenges of this serious complication. It is vital for clinicians, patients, caregivers, and the hemophilia treatment teams to maintain good communication through a well-coordinated plan of care.
The Path ahead:
We must keep in mind the principles and core components of integrated care and primary health care, which includes:
- Meeting people’s lifetime health needs through comprehensive preventive, curative, and rehabilitative services as well as palliative care
- Addressing the broader determinants of health through multi-sectoral policy and action that engages relevant stakeholders for integration of palliative care in inherited bleeding disorder
- Enables local communities to strengthen primary health care
- Empower individuals, families, and communities to take charge of their own health
For further reading: The World Federation of Hemophilia (WFH) 2020 Guidelines ,Third edition
For further correspondence: Dr Avinash Tiwari, Room no 7, Unit of Palliative Medicine and Supportive Care, Department of Medical Oncology, AIIMS Raipur, firstname.lastname@example.org