Living with sickle cell disease

Sickle cell disease is the UK's fastest-growing genetic disorder which affects around 18,000 people.

It predominantly affects people of sub-Saharan African and Afro-Caribbean descent, but can also be found in people of Mediterranean, Middle Eastern, and South Asian descent.

Sickle cell disease is a potentially life-threatening inherited condition that is caused by abnormal haemoglobin levels. This results in red blood cells becoming sickle shaped during episodes of dehydration, low blood oxygen levels and infection.

Sickled red cells can block or damage blood vessels which results in painful episodes commonly known as a 'sickle cell crisis' or simply a 'crisis'. This can lead to acute or chronic organ damage including to lungs, liver, kidneys, eyes and brain.

During a crisis, it is important to get treatment as soon as possible. This may mean using medication that you've been prescribed, or attending a specialist service such as our Acute Sickle Cell Unit (ASCU).

What's in your blood

Your blood is It is made up of different types of blood cells and plasma.

Plasma is a straw-coloured liquid that carries the blood cells around your body.

The different types of blood cells are:

Red cells contain a substance called haemoglobin which is the red pigment that carries oxygen to the tissues of the body and removes carbon dioxide from the tissues.

White cells are part of your immune system. They help to detect and deal with infections.

Platelets are fragments of cells which help to clot your blood when your blood vessels are damaged. They work by clumping together around the damaged area to stop blood loss.

Adults have about five litres (seven to eight pints) of blood. Normally red cells last about 120 days in your blood, but this is less for people who have sickle cell disease. They are then removed by the body, so your body always needs to replace them.

Top-up transfusions

A blood transfusion is a way to put red cells into your body. This could be as a planned procedure or be performed in an emergency.

The two main types of blood transfusions are:

Simple transfusion (top-up) – Bags of blood, called units are given to you through a small tube called a drip connected to a cannula or line placed in one of your veins, usually in your arm.

Exchange blood transfusion – a procedure that replaces sickle blood with non-sickle blood.

You might need a simple (top up) transfusion if...

A simple transfusion might be needed if your haemoglobin level falls too low.

This can happen during a sickle cell crisis, which destroys your red blood cells.

Please contact your GP or hospital doctor immediately if:

  • your urine is a dark colour
  • your eyes and skin are yellow
  • you feel light-headed
  • you feel weak
  • you easily become tired or breathless
  • on a longer term basis to treat chronic complications such as stroke, kidney or lung problems.

Other reasons for needing a simple transfusion are:

  • to get you ready for an operation. It will reduce the risk of complications from the general anaesthetic and the surgery;
  • to reduce the risk of complications to you and your baby if you are pregnant.

For more information about transfusions, please speak to your haematology doctor, sickle cell nurse specialists or day unit nurses.

Sickle eye disease

People with known sickle cell eye disease (retinopathy) or anyone on treatment with Desferrioxamine, (Desferral) or Deferasirox (Exjade) should have an annual ophthalmology review at the hospital as a minimum.

Even if you do not have any eye complications, you should make sure that you have a full eye test at your local opticians at least every two years to find any potential problems early.

Some eye problems can occur suddenly and need urgent attention

Vitreous haemorrhage (bleeding between the lens and the retina) and retinal detachment are not uncommon, particularly in patients with HbSC and HbS Thalassaemia during pregnancy.

If you experience problems with your vision, particularly loss of an area of vision, flashing lights or a ‘shower of floaters’ you should go to your nearest eye casualty clinic.

Eye casualty clinics (eye A&E) in south east London

St Thomas’ Hospital

If your condition is urgent, please call the eye casualty telephone service.

Phone: 020 7188 4336

Location: rapid eye access clinic, eye department, St Thomas' Hospital, ground floor, South Wing, block C, Westminster Bridge Road, London, SE1 7EH

Opening times: Monday to Friday, 8.30am to 4pm

The St Thomas' rapid eye access clinic is for existing patients and those who live in south east London.

Kings College Hospital

Go directly to the main Emergency Department, no referral required.

Phone: 020 3299 9000

Location: Denmark Hill, London, SE5 9RS

Priapism (painful erections)

Priapism is a condition which causes painful erections in boys and men with sickle cell disease.

You may have questions about priapism but are embarrassed to talk about it. It is frequently painful and distressing. This is not associated with normal sexual function or desire.

The majority of males with sickle cell anaemia (HbSS) are likely to experience a priapism in their life time.

Priapism is less common in males with sickle cell disease (HbSC) and sickle beta thalassaemia (HbSB).

There are two common types:

  • Stuttering — the most common type this typically occur at night. It is called stuttering because it is usually resolves itself, but but keeps reoccurring. Stuttering episodes can go on to become acute (or fulminant).
  • Acute (or fulminant) — a sustained painful erection lasting more than a few hours which may not resolve on its own.

Why it can happen

A priapism attack is a sickle cell crisis that is localised to the penis.

Sickled red blood cells block the supply and flow of blood in the penis causing blood to get trapped and keeping the penis in its erect state.

The trapped blood in the penis becomes starved of oxygen and this causes pain and tissue damage the longer it goes on. 

Normal night-time and early morning erections are a common trigger for priapism in patients with sickle cell disease.

Coping strategies

Helpful strategies for dealing with priapism include:

  • Staying hydrated by drinking more water,
  • taking painkillers,
  • heat packs or warm water to the area
  • Exercise such as squats, jogging, cycling or running up and down stairs can also help resolve a priapism attack.
  • Emptying the bladder during the night to avoid early morning erections has also been reported as a useful preventative measure in some cases.

Get help

If you are dealing with painful episodes of priapism, please speak with your haematology doctors, sickle cell nurses or other members of your care team.

Read more about priapism associated with sickle cell disease

Your mental health and wellbeing

Living with a health condition can affect people’s lives in many different ways.

Meeting with a psychologist or connecting with peer groups can help by giving you the chance to talk through your health difficulties and the impact they have on your life and emotional wellbeing.

Our specilist psychology service for adults and our service for children and young people can support you or someone you know to cope with the challenges of living with sickle cell and thalassaemia.

Read more about our psychology service for adults

Read more about our psychology service for children and young people

Travelling abroad

Some people with sickle cell disease may experience difficulties with air travel. If you or someone you know has had issues with flying, it is important to plan ahead.

Medical documents

Before you travel, ask your consultant or GP for a letter explaining your medical history.

If anything happens whilst you are away, having this record will be helpful for the doctors who treat you.

Vaccinations

Choose a travel agent or airline that is registered with ABTA or ATOL. You will need to inform the airline, travel agent and your travel insurance company that you have sickle cell disease. If you fail to disclose this, it may invalidate your travel insurance.

Make sure your routine vaccinations are up to date. Your doctor or a travel clinic can advise on any extra vaccines needed, including those recommended for people with sickle cell disease.

Malaria

People with sickle cell disease can still develop severe malaria. If travelling to a malaria risk area, take preventive antimalarial medication, wear suitable clothing, and use a mosquito net.

If you have G6PD deficiency, tell your GP or pharmacist before starting treatment.

Travelling with medications

Pack your medication in your hand luggage. If you are asked to carry extra supplies in your suitcase, make sure you have enough medication in your hand luggage to last a few days after arrival.

Flying

Aircraft cabins are pressurised and this results in a lower oxygen content in air within the plane.

If you’ve had a painful or respiratory episode in the two weeks before travelling, speak to your GP or haematologist first, as you may be advised not to fly. People with previous acute chest syndrome or low oxygen levels may need oxygen arranged for the flight.

Dehydration

Air conditioning used during flights can be cold and dry. It is important to keep warm and drink plenty of water. It is also advisable for you to move around at regular intervals.

If you develop diarrhoea whilst on holiday, remember to drink more water than usual and take a supply of oral rehydration sachets such as Dioralyte which you can buy from a pharmacy.