TREATING SICKLE CELL

Treatment overview

There’s no single way to manage sickle cell disease. The optimal care for you depends on your symptoms and needs. It may not only include medical treatments but also some alternative and complementary approaches.

Your healthcare team will discuss the different treatment options with you and recommend which are most suitable for you, considering your preferences and circumstances. Let’s discuss the medical treatments they may prescribe.

There are lots of ways to manage your sickle cell symptoms. For more information about what you can do to manage symptoms during a pain crisis, head over to "Managing pain".

Types of treatments

Various medicines and interventions are available to help manage sickle cell disease and its symptoms.¹ They generally fall into one of the following categories¹:

Treatments to help manage symptoms like pain or prevent complications
Medicines that target sickle cell disease itself
Stem cell transplantation in certain cases

Let’s discuss some of the common medical treatments for sickle cell disease. This isn’t an exhaustive list, and your healthcare team may be able to discuss others.

Clementine living with sickle cell disease and campaign graphics overlay

Treatments to manage symptoms

Antibiotics and vaccinations

Sickle cell disease makes people more prone to infections.^1-3 Risk of infection is even higher if you have had your spleen removed.³ That’s why your healthcare team will recommend certain vaccinations.^1-3 As a child, you should have had vaccinations against various types of infections, including meningitis, pneumococcal disease and hepatitis.^2,3

Some vaccines protect you for a very long time, others require regular boosters. For instance, a flu vaccine should be renewed every year.³ So, you might like to check with your healthcare team if you need any vaccine boosters.

A daily dose of antibiotic for further protection against infections is also recommended for many patients.^1-3 If you have had your spleen removed, you will need to take this for the rest of your life.^1-3 You can discuss your need for daily antibiotics with your healthcare team.

Painkillers

Periods of pain (“pain crises”) are the most common symptom of sickle cell disease.⁴

You can usually manage mild to moderate pain at home with over-the-counter painkillers.¹ Ensure that you check with your healthcare team first about which ones to get and how you should take them, as well as when you might need treatment at hospital.

If the pain is not manageable at home, you may need to go to hospital. The healthcare team there can give you the care you need.^1,3,4

Treatments to target the disease

Hydroxycarbamide (hydroxyurea)

Hydroxycarbamide is an oral drug that is used to prevent pain crises in sickle cell disease. It works by reducing the growth of cells in the bone marrow and helps prevent red blood cells from becoming sickle shaped.^5,6

To find out what the shape of your red blood cells has to do with your symptoms, you might like to check out "Understanding sickle cell disease".

People who take hydroxycarbamide have blood tests on a regular basis to monitor and evaluate the benefits and risks of the treatment.⁶

Monoclonal antibodies

People with sickle cell disease have more of a protein called P-selectin, an adhesion molecule that causes red blood cells to become sticky.⁷ Monoclonal antibodies are found in medications used to help prevent pain crises by binding to this protein to stop red blood cells sticking to the walls of the blood vessels. This medicine is usually given through an infusion (drip) into a vein – the first two infusions are given two weeks apart, after which they are every four weeks.⁷

Blood transfusions

People with sickle cell disease have red blood cells that do not live as long as healthy red blood cells and can get stuck in blood vessels.⁸ This causes the symptoms of sickle cell disease, including pain and periods of feeling weak and tired.⁸ To find out more about what your red blood cells look like if you have sickle cell disease, click here.

Your healthcare team may prescribe blood transfusions to increase your red blood cell levels and provide your body with healthy red blood cells that can carry more oxygen.^1,9

Blood transfusion means you receive someone else’s blood through a tube placed into your vein in your arm. The blood comes from blood donors and undergoes careful checks before you get it.⁹

There are different types of transfusions¹⁰:

Top-up (simple) transfusion: You receive non-sickle cell blood through a small tube that is usually placed in a vein of your arm, but none of your blood gets removed from your body
Exchange blood transfusion: Your blood is exchanged with non-sickle cell blood

Exchange blood transfusions can happen manually or through an automated approach. In the manual approach, your healthcare team will repeatedly remove some of your blood and then give you a transfusion. In the automated approach, you are connected to a machine, that coordinates the process.¹⁰

Transfusions can also help manage and prevent complications such as stroke.¹¹ So, your healthcare team may recommend that you come in for blood transfusions on a regular basis.¹¹

Transplantation

Stem cell transplant is currently the only treatment that can provide long-term relief from the symptoms of sickle cell disease.¹ This approach involves a person with sickle cell disease receiving bone marrow from a suitable donor.¹

However, the procedure is not available for everyone¹:

It requires finding a donor who is a good enough genetic match to ensure that your body does not reject the transplant
It may be risky and can lead to serious complications. That’s why usually only people with severe symptoms of sickle cell disease are considered for a transplant

Staying on track with treatments

Remembering to take any medication you are prescribed and keeping up with appointments to see your healthcare team are part of managing your sickle cell disease. It’s not always easy to fit these into a busy life but there are some things you can do to help:

Your phone is your friend! If you find it hard to remember your medication you could set an alarm to help remind you
Set a goal. Use our goal-setting resources to plan how you can better manage your sickle cell – for example, you might aim to take your medication regularly if you currently struggle to do so
Let your healthcare team know if you’re finding it hard to get to your appointments – whether it’s transport, time, or some other reason, together you can find a solution. You might find it helpful to look at our guide to having conversations with your healthcare team first
Keep your healthcare team up to date with your contact details so you can keep lines of communication open

Whether it’s taking your medication or getting to appointments, keep talking to your healthcare team about any difficulties you might be having. Together, you can discuss the support you need to manage your life with sickle cell disease.

The treatments mentioned in this article are some of the ones your healthcare team may mention. The list is not exhaustive and not all treatments may be available to you. If you have any questions about managing your sickle cell, keep talking with your healthcare team.

Resources:

Top tips for self-management
Your wellness journal
Setting goals that work for you
Getting the most out of your healthcare appointments

VIEW ALL RESOURCES

References

NHS. Sickle cell disease: Treatment. 2019. www.nhs.uk/conditions/sickle-cell-disease/treatment/ Accessed May 5, 2023.
Public Health England. The Green Book, chapter 7: Immunisation of individuals with
underlying medical conditions. 2020. Available from: www.gov.uk/government/publications/immunisation-of-individuals-with-underlying-medical-conditions-the-green-book-chapter-7 Accessed May 5, 2023.
Sickle Cell Society. Standards for the clinical care of adults with sickle cell disease in the UK. 2018. www.sicklecellsociety.org/sicklecellstandards/ Accessed May 5, 2023.
Edwards CL, Scales MT, Loughlin C, et al. A brief review of the pathophysiology, associated pain, and psychosocial issues in sickle cell disease. Int J Behav Med. 2005;12(3):171–179.
Siklos (hydroxyurea). SMPC. www.medicines.org.uk/emc/product/10350/smpc/ Accessed May 5, 2023.
Xromi (hydroxyurea). SMPC. www.medicines.org.uk/emc/product/10549/smpc/ Accessed May 5, 2023.
Karki NR, Kutlar A. P-selectin blockade in the treatment of painful vaso-occlusive crises in sickle cell disease: a spotlight on crizanlizumab. J Pain Res. 2021;14:849–856.
NHS. Sickle cell disease: Causes. 2019. www.nhs.uk/conditions/sickle-cell-disease/causes/ Accessed May 5, 2023.
NHS. Blood transfusion. 2021. www.nhs.uk/conditions/blood-transfusion/
Accessed May 5, 2023
Howard J. Sickle cell disease: when and how to transfuse. Hematology Am Soc Hematol Educ Program. 2016;2016(1):625-631. doi:10.1182/asheducation-2016.1.625
Inusa BPD, Hsu LL, Kohli N, et al. Sickle Cell Disease-Genetics, Pathophysiology, Clinical Presentation and Treatment. Int J Neonatal Screen. Jun 2019;5(2):20. doi:10.3390/ijns5020020