Keeping well with Sickle Cell for adults

Information for patients

What is sickle cell?

Sickle cell 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. The red cells contain haemoglobin which carries oxygen around the body and gives blood its red colour. People with sickle cell make crescent shaped red blood cells that can cause problems because they do not live as long as healthy blood cells and can block blood vessels. The red cells can become sticky and inflexible and the blood vessels that the cells are transported in can also become sticky causing the cells to stick to each other and the vessel walls.

This process may also cause problems in the organs where sickling is happening. It also affects the immune system. In sickle cell, there are problems that happen suddenly and are often painful – these are known as sickle cell crises. The long-term effects of crises and background sickling (that may have no symptoms) are known as chronic complications. It is a serious and lifelong health condition although treatment can help manage many of the symptoms.

People who engage with their red cell team often do better in staying healthy. You will be offered regular appointments in our red cell clinic to monitor and treat your sickle cell.

Early detection of complications may help to slow the progress and keep you well longer.

Screening tests may include:

• Blood tests.
• Urine tests.
• Echocardiogram.

Please cancel your appointment if you are unable to attend so that we can use the time to see another patient.

Sickle cell can cause a wide range of symptoms

The main symptoms are:

• Painful episodes.
• Increased risk of serious infections.
• Anaemia.

Painful episodes

Episodes of pain known as sickle cell crises are one of the most common and distressing symptoms of sickle cell. They happen when blood vessels to part of the body become blocked.

The pain can be severe and last for several days or weeks. It can affect any part of the body but is most common in the limbs or back. The frequency of painful episodes can vary a lot from person to person.

It is not always clear what triggers pain, but sometimes painful episodes can be caused by:

• Weather (such as wind, rain or cold).
• Dehydration.
• Emotional stress.
• Strenuous exercise.
• Infection.

Infections

People with sickle cell are more vulnerable to infections, particularly when they are young. Infections can range from mild, such as colds, to much more serious and potentially life threatening, such as meningitis.

Vaccinations and daily doses of preventative antibiotics can help reduce the risk of many infections.

Anaemia

Nearly all people with sickle cell have anaemia, where the haemoglobin in the blood is low. This level of anaemia can vary from person to person. Each patient with sickle cell will have an individual steady state haemoglobin level.

Haemoglobin is the substance found in red blood cells that’s used to transport oxygen around the body.

This does not usually cause many symptoms, but sometimes it can get worse if you become infected with the virus that causes slapped cheek syndrome (parvovirus) which can lead to a sudden drop in the number of red blood cells and may cause additional symptoms, such as headaches, a rapid heartbeat, dizziness and fainting. It usually treated with a blood transfusion.

Other problems

Sickle cell can also sometimes cause a wide range of other problems.

These include:

• Delayed growth during childhood and delayed puberty.
• Gallstones, which can cause tummy (abdominal) pain and yellow skin and eyes (jaundice).
• Bone and joint pain.
• A persistent and painful erection of the penis (priapism), which can sometimes last several hours.
• Painful open sores on the lower legs (leg ulcers).
• Strokes or transient ischaemic attacks, where the flow of blood to the brain is blocked or interrupted.
• A serious lung condition called acute chest syndrome, which can cause a fever, cough, chest pain and breathing difficulties.
• Swelling of the spleen, which can cause shortness of breath, a rapid heartbeat, tummy pain, a swollen tummy and anaemia.
• Eyesight problems, such as floaters, blurred or patchy vision, reduced night vision and occasionally sudden vision loss.
• High blood pressure in the blood vessels that carry blood from the heart to the lungs (pulmonary hypertension).
• Kidney or urinary problems, including blood in the urine and bedwetting.

Sickle cell usually requires lifelong treatment

Children and adults with sickle cell are supported by a team of different healthcare professionals working together at a specialist sickle cell centre.

Your healthcare team will help you learn more about sickle cell, and work with you to come up with an individual care plan that takes into account all your needs and health concerns.

Preventing painful episodes

The main thing you can do to reduce your chances of experiencing a painful episode (sickle cell crisis) is to try avoiding possible triggers.

You may be advised to:

• Drink plenty of fluids to avoid dehydration.
• Wear warm clothing to stop you getting cold.
• Avoid sudden temperature changes, such as swimming in cold water.

Self-help for treating a sickle cell crisis

If you have a sickle cell crisis, you can usually manage it at home.

The following things can help:

• Take over-the-counter painkillers, such as paracetamol or ibuprofen (do not give aspirin to children under 16 unless a doctor has prescribed it) – if the pain is more severe, your GP may prescribe stronger painkillers such as codeine.
• Have plenty to drink.
• Use a warm towel or a wrapped heated pad to gently massage the affected body part – many pharmacies sell heat pads that you can use for this purpose.
• Distractions to take your mind off the pain – such as watching a movie.

You may need treatment with very strong painkillers, such as morphine, in hospital for a few days.

Inpatient Care

You may need to be admitted to hospital for a procedure or for urgent treatment. Admission usually takes place following a review in A&E or our assessment area J95 on level 5 Bexley Wing.

Red cell patients are usually admitted to one of two haematology wards at SJUH.

You may be admitted to the young adults unit

or The assessment ward

You may be placed on another ward in Bexley Wing if there are no beds available on the haematology wards, but we will try to relocate you as soon as possible.

What happens during an inpatient stay?

You will be admitted under the care of a red cell/myeloid consultant haematologist. A consultant will review you and confirm your management plan within 24 hours of admission. Report any new symptoms and have any questions ready and do not leave the ward unnecessarily. On Mondays and Thursdays, a red cell or myeloid consultant will lead the ward round. As an inpatient, your primary point of contact is your named ward nurse or the ward doctor. They will liaise directly with senior members of staff if require. All medication is kept by the nurses to avoid accidental dosing errors. If you bring in any medication from home, please tell your ward nurse.

Please tell the nurses or doctors looking after you if you know that you have an individual pain management plan.

This will have been discussed at a red cell clinic appointment and you should have received a copy of it. If so, it will also be attached to your electronic patient record at the hospital.

Discharge from hospital is a medical decision. Because of the bed pressures in the NHS, we are not able to offer a prolonged stay for recuperation. ‘Fit for discharge’ is an assessment based on factors such as your mobility, independence and need for intravenous medication. Your planned discharge date will be discussed with you in advance. We will normally provide you with a limited amount of medication that you may need ‘to take out’ (TTO).

Treatments for sickle cell

Hydroxycarbamide

• A medicine called hydroxycarbamide (hydroxyurea) may be recommended to reduce the frequency of painful episodes. It may also help to prevent some sickle cell complications. You usually take it as a capsule once a day.
• Hydroxycarbamide can lower the amount of other blood cells, such as white blood cells and platelets (clotting cells), so you’ll usually have regular blood tests to monitor your health.

Preventing infections if you have sickle cell

• People with sickle cell are more vulnerable to infections.
• Most people need to take a daily dose of antibiotics, usually penicillin, often for the rest of their life.
• Long-term use of antibiotics will not pose any serious risks to your health.
• You should have all the routine vaccinations, and possibly also additional vaccinations like the annual flu vaccine and the hepatitis B vaccine.

Treatments for sickle cell-related anaemia

• Anaemia often does not cause symptoms and may not require specific treatment.
• But dietary supplements like folic acid, which helps stimulate the production of red blood cells, may sometimes be required to help improve anaemia.
• Anaemia caused by sickle cell disease is not the same as the more common iron deficiency anaemia.
• Do not take iron supplements to treat it without seeking medical advice, as they could be dangerous.
• If anaemia is particularly severe or persistent, treatment with blood transfusions or hydroxycarbamide may be necessary.

Red Cell Exchange

Your doctor may recommend red cell exchange treatment for you.

Why do I need automated red cell exchange?

In an emergency you may be given this treatment to help improve symptoms such as:

• A severe sickling episode affecting your lungs (acute chest syndrome).
• An acute stroke affecting your brain.

As planned treatment:

• As part of a long-term programme to prevent stroke or other sickle complications.
• If you have a number of painful sickling episodes affecting your lungs (acute chest syndrome) and hydroxycarbamide (hydroxyurea) has not helped.
• If you have frequent and disruptive episodes of acute pain and hydroxycarbamide (hydroxyurea) has not worked/helped these.
• To prepare you for an operation.

The Therapeutic Apheresis Suite is located on Level 3, Bexley Wing and provides automated red blood cell exchange transfusion, stem cell collection and plasma exchange procedures. Treatment is by appointment only. Patients receiving regular exchange blood transfusions will receive their next appointment directly from the Apheresis Team.

Please ensure that you are warm and well hydrated when you attend. You should also bring a small supply of analgesia or other usual medicines that you may need on the day.

Please attend your blood transfusion appointments on time and try not cancel with less than 24 hours notice. Also, please notify the Apheresis Unit if you are late so that we can see if there is still a slot for the same day. We run a very tight schedule and one chair is used several times per day. If you arrive more than 1 hour late without prior notice, there may no longer be a place for that day, and we will have to cancel the transfusion.

Stem cell or bone marrow transplants

Stem cell or bone marrow transplants are the only cure for sickle cell disease, but they’re not done very often because of the significant risks involved.
Stem cells are special cells produced by bone marrow, a spongy tissue found in the centre of some bones. They can turn into different types of blood cells.

For a stem cell transplant, stem cells from a healthy donor are given through a drip into a vein.

These cells then start to produce healthy red blood cells to replace the sickle cells.
A stem cell transplant is an intensive treatment that carries a number of risks.
The main risk is graft versus host disease, a life-threatening problem where the transplanted cells start to attack the other cells in your body.

Stem cell transplants are generally only considered in adults with sickle cell disease who have severe symptoms that have not responded to other treatments, when the long-term benefits of a transplant are thought to outweigh the possible risks.

Treating other problems

Sickle cell disease can also cause a number of other problems that may need to be treated. For example:

• Gallstones may be treated with gallbladder removal surgery.
• Bone and joint pain can be treated with painkillers, although more severe cases may require surgery or joint replacement.
• Persistent and painful erections (priapism) may require medication to stimulate blood flow or using a needle to drain blood from the penis.
• Leg ulcers can be treated by cleaning the ulcer and dressing it with a bandage.
• People at increased risk of having a stroke, or those who have had a stroke, may need regular blood transfusions or treatment with hydroxycarbamide.
• Acute chest syndrome, a serious lung condition, usually requires emergency treatment with antibiotics, blood transfusions, oxygen and fluids given into a vein – hydroxycarbamide may be needed to prevent further episodes.
• People who need a lot of blood transfusions may also need to take medicine called chelation therapy. This reduces the amount of iron in their blood to safe levels.

Hints and tips for keeping well whilst travelling

• Plan in advance and research where you are travelling to and ensure you have had the vaccines required for that area or country (if necessary) and also know how to access healthcare and where it is located.
• Ensure you have been prescribed anti-malarials by your doctor if your destination is a risk for malaria and that you have started the course as advised by your doctor.
• Make sure that you take a supply of your regular medicines for the duration of the trip.
• Carry a supply of your usual pain medicines in case of a painful episode.
• Keep all of your medicines and health information in your hand luggage.
• Get a summary of your healthcare information such as a recent clinic letter so that you can pass it on to a hospital in case you need treatment abroad – take a photo and keep it on your phone.
• Travel when you are free from infection and/or crisis.
• Get a good night’s sleep on the night before your departure.
• Drink plenty of water and non- alcoholic drinks prior to and during your flight and continue to do this for a few days after your arrival.
• Avoid smoking.
• Get up and stretch your legs during the flight, walk up and down the aisle a few times to improve your circulation.
• Watch out for any unusual symptoms of pain or breathlessness and inform the cabin crew immediately if you feel unwell.
• Ensure you have bought travel insurance to cover you during your trip.

Please contact your red cell team if you have any questions about sickle cell. They want you to stay healthy for as long as possible.

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