We are part of a network with North Middlesex Hospital in London to provide services to our patients who are affected by haemoglobinopathies, which enables us to offer all of our patients an annual review with a consultant specialising in haemoglobinopathy treatment in London, and to be part of the regional network meetings to share advancing knowledge and new research into your condition.
Services we offer
People can have different forms of SCD (sickle cell disease) or thalassaemia (even within the same family), with varying needs and possible complications over their lifetime. Our service offers treatment, support and advice for all forms of these conditions, including medical treatment in our outpatient clinics, or on the hospital wards if necessary. These include:
- Regular monitoring in our local outpatient clinics (held weekly)
- Annual review with a London Haemoglobinopathy Specialist Consultant, Dr Arne de Kreuk
- Emergency department
- Admission to a ward if specialised treatment is required
- Close links to other specialties for screening and investigation (for example, eye, heart, kidney and urology tests and treatment)
- A specialist Haematology Day Unit for red cell exchange treatments and urgent reviews
- Access to genetic screening and pre-conception advice
- Close links with the Paediatric (Children’s) haematology team, and a managed move to the Adult team after age 16
- A specialist haematology laboratory and blood service
Some of the other specialists you may see
- Orthopaedic surgery
- Renal Medicine
- Genetic counselling
Annual review appointments
We offer all of our patients an annual review with a London haemoglobinopathy specialist, Dr Arne de Kreuk, which takes place at the hospital in either April or November. At these clinics, we consider any changes in your overall health, your lifestyle and review all of your investigation results and medication to make a plan with you. On some occasions Dr de Kreuk may suggest an appointment at the North Middlesex Hospital if you are willing to travel to London. He will summarise the appointment in a letter to your GP, and you should receive a copy of this too. Please bring a copy of your current medication and immunisation record to this appointment if possible.
Other review appointments
We will see some patients between their annual reviews with our London Haemoglobinopathy Consultant in our regular clinics at NNUH. Patients who are on hydroxycarbamide for sickle cell disease will need regular blood test monitoring, and some patients require regular blood transfusions and/or treatment to monitor iron levels. It may be possible to offer some of these appointments as telephone or video appointments.
If you are experiencing a painful crisis that you cannot manage at home, if you are male and have priapism, or if you feel very unwell or with symptoms of an infection, please come into the Emergency Department and make them aware of your haemoglobinopathy diagnosis. You are welcome to call us for advice first on our emergency helpline: 01603 646753.
Patients who attend the Emergency Department will usually be discussed with the Haematology team rapidly. If your problem may relate to your haemoglobinopathy diagnosis, you will be seen by a Haematologist as soon as possible after you are admitted (usually the following morning if you are admitted overnight) and each day during a hospital admission, and we will contact any other teams who may need to be involved in your care. NNUH has an Acute Pain Team who help with patients presenting in painful crisis.
Some medication for people with SCD and thalassaemia need to be monitored in hospital, and will usually be prescribed from clinic. These medications may include:
- hydroxycarbamide (also known as hydroxyurea)
- Iron chelation agents, such as
- desferrioxamine (Desferal)
- deferiprone (Ferriprox), and
- deferasirox (Exjade)
If you take strong painkillers, such as opiates, they should to be prescribed from one place only, as part of national guidance. Other medications should be prescribed by your GP.
If you have sickle cell disease, or have had your spleen removed, you should take a preventative dose of Penicillin V (penicillin prophylaxis, or equivalent such as erythromycin if you are allergic to penicillin) twice a day for the rest of your life to reduce the risk of illness and death. Some patients choose not to do this, and we can discuss this with you.
We advise our patients should take folic acid tablets (5mg once a day) to support their bone marrow in producing increased numbers of red blood cells.
We recommend that people affected by SCD and thalassaemia have the following immunisations:
- Normal childhood immunisation schedule
- Hepatitis B immunisation
- Annual influenza immunisation
If you have SCD or your spleen has been removed, you also need:
- Pneumovax (every five years)
- Haemophilus influenzae type B (Hib) vaccine – once
- Meningococcal ACWY vaccine – once
- Meningococcal B vaccine (newly available) – once
- Prevenar pneumococcal immunization – once
Please keep us up-to-date at your clinic appointments with your immunisation dates.
Travel with haemoglobinopathies
Travel can lead to an increased risk of crises in SCD, and also blood clots, infection, fatigue, dehydration from hot weather. Please try to stay well hydrated, walk around regularly during long distance travel, and make sure you have had all recommended immunisations well before you travel. If you are on a transfusion programme, consider timing a transfusion just before you travel. Make sure you have travel insurance that covers your haemoglobinopathy. We are very happy to provide any letters you may need about your condition – just let us know. The current guidelines for travel from the UK Civil Aviation Authority with SCD suggest flying with oxygen, which most airlines no longer provide. Oxygen can be hired from a range of providers to travel with if your airline cannot provide it; please contact them in advance of the flight to find out. A link to the current NHS list of airline oxygen suppliers can be found here.
People with SCD and thalassaemia are at risk of malaria, even if they previously lived in a malarial area or return there regularly. It is important to wear protective clothing and to use insect repellent, mosquito nets, and to take malaria prevention tablets. You should be aware of your G6PD (glucose-6-phosphate dehydrogenase, an enzyme) status, as some medications may cause problems in those with G6PD deficiency; this condition is also relatively common in patients who have SCD and thalassaemia.
Planning a pregnancy
If you are planning a pregnancy, please talk to your Haematology team. Patients with thalassaemia may be affected by difficulty conceiving and require more specialist help. We can discuss the genetic inheritance of your condition and help you to assess whether your baby could be affected – this depends on what genes your partner carries too. If you both have genes that might cause a baby to have a severe form of haemoglobinopathy, you could consider referral for pre-implantation genetic diagnosis, a form of fertility treatment to select an embryo that is not affected. This is a sensitive and difficult subject, and we can talk this through with you. We will review your medication with you and stop any that could cause damage to a developing baby when you are trying to conceive (this applies to both men and women). We will also review your general health, iron status, and any transfusion programme that you are on. Please ensure your immunisations are up to date and you are taking folic acid tablets if you are planning a pregnancy; if you are taking Penicillin V then please continue this.
We will review female patients closely during a pregnancy, in conjunction with our colleagues in Obstetrics. Most women have few problems and a healthy pregnancy, but the risk of painful crises can be increased during pregnancy for women with SCD (particularly due to dehydration with morning sickness), there is an increased risk of blood clots, and anaemia can worsen during pregnancy. Patients with thalassaemia major may require review by other teams – Endocrinology and Cardiology – during a pregnancy. If your baby is delivered here, the Haematology team will review you on the ward during your admission.