| Abbreviation | Full Term | Details |
| BMT | Bone Marrow Transplant | Bone marrow transplant, also known as ‘stem cell transplant’ or ‘haematopoietic stem cell transplant’, can offer a potential cure for Sickle Cell Disorder (SCD). It involves replacing a patient’s bone marrow cells with healthy stem cells from a donor. This transplant can only be considered for those with severe sickle cell complications if the benefits outweigh the risks, as it is an intensive treatment option. |
| CNS | Clinical Nurse Specialist | A Clinical Nurse Specialist (CNS) in the NHS is a senior registered nurse with specialised expertise in a specific area of nursing. They are responsible for providing expert advice, managing patient care, and coordinating services, often acting as a key point of contact between patients and the multidisciplinary team. |
| HAPLO/ HLA | Haploidentical Bone Marrow Transplant | A haploidentical bone marrow transplant uses healthy stem cells from a donor who is half a tissue type match to the patient, typically a family member (like a parent or sibling), to replace a patient’s diseased or damaged blood-forming cells. It’s a common option when a fully matched donor isn’t available, making it easier and faster to find a suitable transplant donor. |
| HCC | Haemoglobinopathy Coordinating Centre | HCCs are organisations which support and oversee the specialist hospital teams within a certain geographical region. Their aims are to ensure equitable access to specialist care, new treatments and clinical trials for everyone living in that area. |
| ICPs PCPs | Individualised Care Plans Personalised Care Plans | A care plan is a written document and sets out what support and help you need. The plan should help to ensure you get the right care wherever you are. Haematology centres agree a care plan with you which may include preventative day to day management, guidance for ward staff and guidance for ambulance and emergency care services during a crisis. |
| LHT | Local Haemoglobinopathy Team | Local Haemoglobinopathy Teams (LHTs) are hospital centres which look after variable amounts of a patient’s care and treatment, but are not classed as ‘Specialist Haemoglobinopathy Teams’. Every patient should have access to a Specialist Haemoglobinopathy Team, but for those living closer to a LHT, it might be better and more convenient for them to receive some aspects of care at their local hospital. |
| NHP | The National Haemoglobinopathy Panel | NHP supports HCCs to provide expert advice on options for individuals with complex needs living with SCD, thalassaemia or rare inherited anaemias. The NHP also supports decision making on novel treatments, improving access to interventions and clinical trials. |
| PPV | Patient and public voice (PPV) partners policy | The role of patient representatives is to ensure that care is patient-centred with the views and ideas of patients being heard. The North East and Yorkshire Haemoglobinopathy Coordinating Centre (N E & Y HCC) for Sickle Cell aims to provide high-standard and equal care across the region. As an SCD patient, parten/carer you are an expert by experience. We value the role of patient representatives who can bring a unique perspective and we believe your experiences can be utilised to engage in meaningful discussions to shape the services and systems which provide your healthcare. https://www.england.nhs.uk/get-involved/resources/patient-public-voice-partners/ |
| RIA | Rare inherited anaemia | Rare Inherited Anaemias (RIA) are rare genetic blood disorders causing anaemia. There are a large number of different RIAs, and people living with RIAs are usually looked after by the same teams that look after people living with sickle cell disorder and thalassaemia. |
| SCD | Sickle Cell Disorder | Sickle cell disorder (disorder is preffered amongst the SCD patient cohort, rather than disorder) is the name for a group of inherited health conditions that affect the red blood cells. The most serious type is called sickle cell anaemia. Sickle cell disorder is particularly common in people with an African or Caribbean family background. People with sickle cell disorder produce unusually shaped red blood cells that can cause problems because they do not live as long as healthy blood cells and can block blood vessels. Sickle cell disorder is a serious and lifelong health condition, although treatment can help manage many of the symptoms. SCD is a disorder of the body’s red blood cells that is genetically inherited and lifelong. It is caused by an abnormality in haemoglobin, the part of red blood cells that carries oxygen round the body. This makes the red cells become inflexible, sticky and crescent (sickle) shaped. This is called sickling. Other blood cells and the vessels they travel in also become sticky, which interrupts the blood flow and triggers an inflammatory cascade. An inflammatory cascade is when one inflammatory reaction causes another. This may cause pain, known as vasoocclusive crisis. It can also cause problems in the organs where the sickling is happening. |
| SCT | Stem Cell Transplant | Please see above under ‘bone marrow transplant’ |
| SHT | Specialist Haemoglobinopathy Team | Specialist Haemoglobinopathy Teams (SHTs) provide clinical services, including specialist interventions, to people living with sickle cell disorder within their area. They often work with Local Haemoglobinopathy Teams to enable equitable access for their population to high standards of care. |
| Thal | Thalassaemia | Thalassaemia is an inherited (genetic) condition affecting the blood. There are different types of thalassaemia. Depending on which type you have, thalassaemia may cause no illness at all, or may be a serious lifelong condition requiring treatment. |
| Analgesia | An analgesic drug, also called simply an analgesic, antalgic, pain reliever, or painkiller, is any type of medication used for pain management. Pain caused by sickle-cell can be acute (short-lived), chronic (more longstanding) or a mixture of the two. | |
| Patient Pathways | A patient pathway is a term we use to describe the different steps on your healthcare journey. At different points in your life, you may follow different pathways. This may include: an outpatient appointment at a clinic, at your local hospital (Local Haemoglobinopathy Teams (LHTs)) or Specialist Haemoglobinopathy Teams (SHTs). Your nearest hospital is not necessarily the hospital you should attend, the provision provided by hospitals across the N E & Y HCC differs across the 3 SHTs and eighteen LHTs. | |
| Sickle cell trait | People with sickle cell disorder have two abnormal genes: one is always a gene for haemoglobin S, and the other can either be a second haemoglobin S gene or a different type of of abnormal gene (such as a gene for beta thalassaemia, haemoglobin C, or many other different variations of haemoglobin). In contrast, people with sickle cell trait (also called being a ‘carrier’ for sickle cell) have one gene for hemoglobin S and another gene for normal hemoglobin A. Though most people who have sickle cell trait don’t have any symptoms, some may experience problems in rare situations. People who are carriers for sickle cell are at risk of having a child with sickle cell disorder if their partner is also a carrier for sickle cell, for beta thalassaemia, or for a range of other different haemoglobins. You can find out if you’re a carrier of sickle cell by having a simple blood test at your GPs: just ask to a ‘haemoglobinopathy screening test’. |
