Is to ensure that people with these lifelong conditions receive the best care and live as normal life as possible. We do this by supporting them to manage their health and make informed decisions around their care, and live healthy and productive lives.
Our role as Clinical Nurse Specialist (CNS) has to do with: providing and coordinating comprehensive care for people with these rare conditions with other members of the multidisciplinary team across different care settings, including patients’ own homes and different specialities. We also coordinate services within the network example – Leeds Comprehensive Care Centre for the HDB, Manchester, Sheffield, and Wittington – London hospital. This includes promoting evidence-based care, advocacy, and self-management skills for patients, promoting physical health, psychosocial wellbeing, and quality of life for people with HBD to support them to achieve their life goal and to reduce morbidity and mortality. We are the first point of contact for these patients and their families.
We do these by educating patients and their families about their condition and effective management in a personalised context; recognising their social status, likes and dislikes, cultural and spiritual differences. We coordinate access to a multidisciplinary team, whose core members include a medical director, nurse coordinator, musculoskeletal dental, and psychosocial experts, laboratory specialists, Endocrine, Specialist Radiology and Ophthalmic team.
We educate patients and their families on:
- how to prevent bleeding and joint damage
- how to recognise and manage spontaneous bleeds promptly
- how to manage their pain effectively and promote concordance with care plans
- the importance of Chelation therapy and healthy lifestyle.
We manage any musculoskeletal complications by making a prompt referral.
We prevent and manage inhibitors and other comorbidities.
We make referral for routine screening and counselling at diagnosis.
We provide training for home therapy and other aspects of care.
We act as advocates for these patients and assist them in accessing resources and achieving good health outcomes.
Where care is not coordinated properly, Congenital HBD and HBO can significantly reduce patients’ health and quality of life. Our role as Specialist nurses, therefore, is important at key points during the patient’s or family’s journey, such as immediately after diagnosis, when patients start self-infusing the treatment, when starting education or work, or while managing life events, such as preparing for the arrival of a baby that potentially has HBD or HBO.
