Sickle cell disorder in adults : University College London Hospitals NHS Foundation Trust

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. Due to this abnormality, the red cells become inflexible, sticky and crescent (sickle) shaped. This process is known as ‘sickling’. Other blood cells and the vessels they travel in also become sticky, which:

disrupts blood flow, and
triggers an inflammatory cascade, where one inflammatory reaction causes another.

This may result in pain as well as problems in the organs where sickling occurs. It also affects the immune system.

People with SCD can experience sudden and often painful episodes known as sickle cell crises. The long-term effects of crises, and background sickling that may have no symptoms, are known as chronic complications. Our aim is to limit and prevent both of these from happening as much as possible.

Sickle cell disorder can cause a wide range of issues, including:

Infections
Gallstones
Joint damage or destruction
Loss of spleen function (making you prone to infections)
Damage to kidneys
Leg ulcers
Visual impairment
Neurological damage which may lead to stroke
Erectile dysfunction (priapism).

The signs and symptoms of sickle cell crisis may include:

Fatigue (feeling tired or weak)
Pain
Jaundice (yellowing of the white of the eyes)
Pale skin, or inside the mouth or eyes
Shortness of breath
Dizziness
Headaches
Symptoms of infection, such as fever.

Please seek medical attention straight away if you develop any of the following symptoms: high fever, chest pain or pain that does not feel like the usual sickle pain, shortness of breath, weakness (especially one sided), change in vision or extreme tiredness.

SCD is an inherited condition so it can’t be prevented.

Each carrier (trait) parent may pass on an affected gene to their child. If both parents are carriers, the chance that their child will have SCD is one in four. If one person has the disorder and the other is a carrier, the risk is greater – one in two. For this reason, it’s important that your partner is tested for SCD if you are planning a pregnancy.

If you would like to know more, please speak to the red cell team or go to your local sickle cell counselling service.

If you or your partner become pregnant unexpectedly, it’s very important that testing is done as early as possible. Contact us without delay and we will organise further counselling and assessment.

You may be able to manage a simple painful sickle cell crisis at home by taking painkillers, resting, drinking fluids and keeping warm. But if the pain becomes worse you may need stronger painkillers in hospital. We can also then treat the underlying cause (trigger) of the crisis and any complications of your SCD.

Sometimes you may feel unwell from the start of the crisis even if it’s not very painful. If you are unwell even if you have no pain, you should always contact us as you may need to come to hospital.

We may also suggest other ways of managing your condition with preventative treatments. We usually recommend this if your sickle cell crises become more frequent or if an episode was very severe. Treatment options include starting hydroxyurea or a long-term blood transfusion programme.

Some people have also had a bone marrow or stem cell transplant that has cured their sickle cell disorder.

For non-urgent medical problems or medical problems unrelated to your sickle cell disorder, for example a rash or a twisted ankle:

Contact your GP or go to your local Emergency Department (A&E). If necessary, they can contact us to discuss your problem.

For non-urgent red cell/iron problems:

Ask for your outpatient appointment to be brought forward. You may also be able to have a review in Haematology Day Care.

If you feel unwell, you should call the haematology advice line (office hours) on 020 3447 7359. It is manned by administrative staff who will contact your clinical team. Out of hours, call the adult haematology advice line on 07852 220 900 or paediatric helpline on 07950 869 745. They are manned by a senior haematology ward nurse who will contact the on-call team.

Even if you are going to an Emergency Department, it is always best to call ahead. We can then try to streamline your assessment as much as possible.

If you go to an Emergency Department in another hospital, it may take a few days to transfer you to UCLH. We are always happy to support other teams but we would not be able to coordinate your care at another hospital. To avoid any potential delays with transfer, you should try to come to the Emergency Department at UCLH if possible.

Note: If you have an infectious illness, such as cough, cold, diarrhoea, vomiting or chicken pox, do not come to clinic or Haematology Day Care. You should go to the Emergency Department and we will see you there. These seemingly minor illnesses can be very serious for some people with blood conditions and very poor immune systems. For example, those recovering from chemotherapy or stem cell transplants.

The following symptoms require immediate medical attention:

Fever: 38°C or above
Feeling very unwell
Pain that you cannot control with painkillers
New weakness, especially if you feel it more on one side than the other
If you think you are much more anaemic than usual.

Common triggers of a sickle cell crisis include:

infection
stress
dehydration
cold or hot weather
sudden changes in temperature.

Sometimes there is no trigger and a crisis happens out of the blue.

Drink a lot of fluids.
Eat healthily.
Exercise.
Make sure infections are treated quickly.
Maintain a good balance of nutrition and activity.
Make sure your vaccinations are up to date.
Avoid smoking.
Take penicillin twice a day for life.
Attend your clinic appointments so that we can review your health and monitor you for any complications.

Attending your clinic appointments is a requirement stated in the National Standards produced by the Sickle Cell Society and NHS England. These appointments are every six to 12 months in healthy people. They may be more frequent if you have health problems.

If you don’t have an appointment, please ask your GP to refer you.

If your appointment is inconvenient, please change it. We will do our best to schedule an appointment that is convenient for you.

For more information on how to stay well, visit our web page ‘Medications, vaccinations and travel in sickle cell disorder’.

The NHR is a database of people with red cell disorders (mainly sickle cell and thalassaemia) living in the UK. The overall aim of the registry is to improve patient care. It helps the NHS England to understand the specific needs of these patients and improve access to treatment.

The registry is currently being updated. It will have a patient portal where you can access your information. It will also include a research component. Your team will discuss this with you at a clinic appointment.

Mortimer Market Centre offers a wide range of sexual health services. This includes testing for sexually-transmitted infections (STIs) and HIV.

You need to book an appointment to attend the clinic, except for walk-in HIV testing clinics. You can phone the clinic for advice if you have an urgent problem or to find out the opening times.

Mortimer Market Centre

Address: Mortimer Market Centre, off Tottenham Court Road, London, WC1E 6JB

Telephone: 020 3317 5252

You can talk to your GP, local family planning clinic or community sickle cell nurse about contraception. You should tell them about your SCD and any other medical problems you may have because of SCD.

If there are any changes to your medication, please let the red cell team know.

If you would like to be referred to the UCLH stop smoking service, please speak to your nurse specialist. You can also find helpful information on the NHS Better Health website.

Sickle Cell Society

Tel: 020 8961 7795

Email: info@sicklecellsociety.org

Website: sicklecellsociety.org

University College London Hospitals NHS Foundation Trust cannot accept responsibility for information provided by external organisations.

Haematology admin team:

uclh.redcelladminteam@nhs.net

Haematology clinical nurse specialists (CNSs):

uclh.redcell.cnsteam@nhs.net

Haematology advice line (office hours, adults and children):

020 3447 7359

Adult haematology advice line (out of hours):