Haemophilia and Haemoglobinopathy Service

The HBD and the HBO Services is part of the Non-Malignant Haematology Service within the hospital. The HBD comprises of all patients with Congenital Bleeding Disorders, all rare Factor Deficiencies and all Platelet disorder including Bernard Soulier, Glandsmman Syndrome and Thrombocytopenia (ITP). The most recent data show a total of 671+, and 75 (ITP) We cover the area of Bradford, Airedale, and Halifax.

The HBO Services is made up of patients with Thalassaemia and Sickle cell disorders. There is currently in adult service, 38 Thalassaemia and 54 Sickle cell and other rare conditions: 1 Black Diamond Fan, 1 Pyropolkilocytosis, 1 Congenital haemolytic anaemia and 1 Haemoglobin Saint which is covered in the Bradford area.

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.

Help in the transition from children to adult services: Haemoglobinopathy patient journey

There are 3 full time (WTE) CNS these are 2 band 6 and 1 band 7. The service is also supported by a team of administrative Clerks and 2 Specialist Consultants who work separately with the two services.

The team operate the review of patients in clinic 3 days a week, and 4 days alternate weekly. This includes – the 2 CNS morning clinics for HBD, 1 CNS morning clinic for HBO, 1 HBD Consultant clinic + 2 weekly virtual clinic and alternate week consultant clinic for the HBO service.

Part of our job has to do with dealing with the acute phone calls from patients who call in for advice or treatment daily (Mon-Friday 09 – 05pm). We also organise and liase with the consultants for referrals to other services.

We work with pharmaceutical department within and outside companies for home delivery of patients’ chelation and factor concentrates/therapies for the management of their condition.

We liase and work with other departments outside the Haem/Onc specialist in the hospital for effective management of these patients during their crisis moment, surgery, or any procedure such as A&E, Endoscopy, ENT, Gynaecology, Orthopaedic/general surgeries, Max-fax department, and Specialist Dental care in the local community.

We undertake the education of these departments within the hospital to create awareness of these rare conditions and to have the basic knowledge of what to do when in contact with these patients.

There is a notable increase in number of patients in both services from 2021 to date.

Unfortunately, the 3 WTE CNS in these services does not match the workload as spelt out in Standards from NHS England. For instance – the CNS have not been able to undertake home visits of the patients and has not also been able to undertake the education in the wider community as required.

The recent audit on waiting times before treatment for Sickle cell patients who attended A&E in crisis shows they are not given pain relief in 30mins as stipulated in the National Standards. This is a project we are currently working on with A&E to improve.

The HBD still suffering from lack of local Physiotherapy/Podiatry service as such patient are referred to Leeds to be on the waiting list for their appointment. This is often missed or not taken up by patients due to the distance.

There is no genetic/family clinic operated within the trust due to staff shortage. As such, family screening for bleeding disorder is overlooked and adult are being diagnosed and treated as and when referred by the GP or other services when suspected to have a suspected bleeding disorder.

In the last 2 years, we have been able to recruit Psychologist to support the HBO and this has been a huge success in the management of patients’ mental health and well-being.

We have been, during this year able to organise an awareness/education day for the services within the hospital for both HBD and the HBO services and in the Bradford Shopping centre for the HBO service as well. This has improved the pathway by timely referral and contacts within the hospital to the services.

The pathway for the out of hours/emergency service for the HBD has been approved and awareness has been made to the haematology ward on the management of these patients. However, the out of hours pathway for HBO still needs to be improved to try and keep patients out of A&E.

There has also been a very good outcome of the Triage calls during working hours for both services and all the patients know the contact numbers during working hours.