Sickle cell disorder: what pharmacists need to know

Sickle cell disorder is the most common inherited haemoglobinopathy in the UK, affecting more than 19,000 individuals and disproportionately impacting people of African, Caribbean, Middle Eastern and South Asian heritage — yet persistent health inequalities continue to delay pain management and limit access to specialist care.

With World Sickle Cell Day falling on 19 June this week, haemoglobinopathy specialist nurse Kimesha Kangethe and highly specialist pharmacist Meera Patel explore how community and practice pharmacists can play a vital role in improving outcomes and tackling inequalities for patients living with the condition.

Sickle cell disorder (SCD) is a genetic condition that affects the ability of red blood cells to carry oxygen around the body.

Red blood cells in individuals with SCD tend to alter in shape under stressful conditions and become sickle shaped rather than smooth, round, and flexible. This abnormal shape causes the blood cells to break down more quickly and block blood vessels, leading to painful episodes known as sickle cell crises, and long-term organ damage.¹

Related Article: Lyme disease: what pharmacists need to know

Stressful conditions can include cold weather, dehydration and strenuous physical activity, which are recognised triggers for vaso-occlusive crises (VOCs) – the most common and well-recognised form of sickle cell crisis.²

Psychological stress can also precipitate crises, likely through vasoconstriction and increased metabolic demand, contributing to sickling of red blood cells.³

Some individuals do not have SCD but carry the sickle cell trait. They usually have no clinical symptoms but can pass the mutated gene on to their children. SCD is an autosomal-recessive genetic condition, so when both parents are carriers, there is a one in four chance that their child will have SCD.⁴

Prevalence

SCD is the most common inherited haemoglobinopathy in the UK, affecting around 19,000 individuals, with 250–300 infants diagnosed annually through newborn screening.⁵

This condition disproportionately affects individuals of African, Caribbean, Middle Eastern and South Asian heritage and remains associated with health inequalities, including delays in pain management and variation in access to specialist care, often leading to negative patient experiences.

These inequalities are highlighted in the all-party parliamentary group No One’s Listening report published in November 2021.⁶

Health impacts

Complications

SCD is associated with common complications including chronic anaemia, fatigue, recurrent pain, acute chest syndrome, and increased infection risk.

The elevated infection risk associated with SCD stems from asplenia – resulting from repeated blockage of small blood vessels by sickled red blood cells, which causes tissue in the spleen to die from a lack of blood and oxygen. As a result, the spleen's ability to produce antibodies diminishes over time, increasing susceptibility to infection.

Other long-term complications can include stroke, retinopathy, avascular necrosis, pulmonary hypertension and iron overload from blood transfusions.¹

Sickle cell crises

Sickle cell crises are periods of excruciating, unpredictable pain for patients. They require immediate attention and should be treated as urgent. Patients may describe severe pain in their bones, chest, back, abdomen or limbs. They may also present with fever, breathlessness, headache, confusion, priapism – a painful erection lasting two hours or more – or new neurological symptoms.¹

A VOC is the most common and well-recognised form of sickle cell crisis, caused by obstruction of blood vessels by sickled red blood cells leading to severe pain.

Other types of sickle cell crises include:

• Acute chest syndrome
• Aplastic crisis
• Splenic sequestration
• Infective crises

Patients often know when something feels different. Any chest symptoms, low oxygen saturations, decreased neurological signs or pain that is not settling should be escalated quickly in line with local protocols.

Sickle cell crises are frequently misinterpreted as drug‑seeking behaviour, particularly in relation to opioids, reflecting persistent biases and health inequalities affecting the predominantly Black and ethnically diverse populations living with the condition.

Treatment options and pain management

Management of SCD focuses on prevention, disease modification targeting the underlying pathophysiology of the condition and treatment of acute VOCs.

Disease-modifying therapy

Hydroxycarbamide remains the first-line treatment option for SCD. It is the mainstay disease‑modifying therapy and primarily works by increasing foetal haemoglobin, which reduces sickle cell crises by preventing red blood cells from becoming sickle shaped.⁷

Preventive care

Preventive care includes penicillin prophylaxis, folic acid and vitamin D supplementation, and vaccination.

To prevent infections, penicillin prophylaxis is usually offered to young children with SCD from three months to five years old. Long-term antibiotic use is also common in high-risk individuals.

Routine vaccinations, such as the five-yearly pneumococcal vaccine and the yearly influenza vaccine, are also recommended to reduce the impact of infections on the body and better manage the condition for patients with SCD.

With regards to supplementation, folic acid is often recommended because it improves the production and quality of red blood cells, while vitamin D is often recommended to improve bone strength as bones are usually weakened by avascular necrosis.

Related Article: Charity urges public to complete blood pressure checklist

Red blood cell exchange transfusion is also a key intervention in both acute and preventive management of SCD, particularly for complications such as acute chest syndrome, stroke prevention, and recurrent severe VOCs.

Unlike simple transfusion, exchange transfusion reduces the proportion of sickled haemoglobin while minimising the risk of iron overload, making it suitable for long-term use in selected patients.

Access to regular exchange programmes, however, can be limited. Many patients with SCD require extended phenotype-matched units to reduce alloimmunisation risk – risk of the body mounting an immune response to the donated blood.⁸ Unfortunately,  there is a recognised shortage of donors from ethnically diverse backgrounds in the UK.

This remains a significant challenge and highlights the importance of national blood donation campaigns alongside careful stewardship of transfusion resources.

Curative treatments for SCD include bone marrow transplants and novel gene therapy. However, availability and commissioning may vary regionally. Awareness of these treatment options is important to support sickle cell care.

Acute VOCs

Acute VOCs require rapid, individualised analgesia – often opioids within 30 to 60 minutes.

Individuals with SCD in London should have a personalised universal care plan created for them by their hospital team, which outlines the analgesia and other elements of care they need in an emergency sickle cell crisis.

For those outside of London, the ACT NOW policy is an NHS-supported clinical framework for managing sickle cell crises.⁹

The ACT NOW policy mandates continual assessment and rapid treatment, characterised by the following steps:

• A - Analgesia: Rapid and continuous assessment and administration of pain relief.
• C - Compassion: Active listening, acknowledging patient distress, and treating without bias or delay.
• T - Test / Triggers: Running necessary clinical tests (like bloods, observations) and identifying what caused the crisis.
• N - Notify: Promptly notifying the local haematology team or specialists about the admission.
• O - Oxygen: Monitoring oxygen levels and administering oxygen if clinically indicated.
• W - Watch: Continual monitoring, evaluating the efficacy of treatments, and regular reassessment of pain.

Role of community and practice pharmacists 

Although sickle cell care remains predominantly with acute specialist teams, community and primary care clinicians can support sickle cell patients within their roles.

Community pharmacists can promote adherence, improve prescription access, advise on pain management and identify early signs of infection or complications.

General practice pharmacists are well positioned to undertake structured medication reviews, support long-term condition management and liaise with specialist teams.

They can also support monitoring of renal function, blood pressure and analgesic use, while addressing polypharmacy and nephrotoxic risk. Structured medication reviews and shared care monitoring are essential.

Related Article: Syphilis cases rising worldwide: what pharmacists need to know

Pharmacists should also be aware of red flag symptoms in sickle cell patients. These include fever, respiratory distress, neurological symptoms, uncontrolled pain, severe anaemia, priapism and vision changes. If these symptoms are present, patients must be directed to the local accident and emergency department for urgent investigation.¹⁰

Across all settings, pharmacists play an important role in helping people with sickle cell disease manage complications and improve their quality of life. Beyond clinical care, they can support safe prescribing, reduce stigma, and ensure effective discharge planning – as well as advocating against the discrimination that continues to drive inequalities in sickle cell care.

Key resources, guidelines and signposting

In summary, listening to patients, recognising red flags and acting promptly can reduce avoidable harm and improve quality of life for people living with SCD.

NICE guidance supports medicines optimisation and pain management, while British Society for Haematology (BSH) guidelines provide detailed recommendations on hydroxycarbamide use, acute crisis management and transfusion.

Acute Trust haemoglobinopathy policies should also be used to support patient care.

Patients should be signposted to resources such as the Sickle Cell Society, Red Cell Network and NHS haemoglobinopathy services.

The Sickle Cell Society provides trusted information and useful tools, including standards for paediatric care, education materials and peer support. These resources help patients understand their condition, manage day-to-day challenges and recognise when to seek urgent help. They provide practical resources that can be implemented by professionals in daily practice.

Key learning points

• SCD requires rapid access to effective analgesia during crises.
• Hydroxycarbamide is the cornerstone of disease-modifying therapy.
• Community pharmacists play a central role in monitoring and holistic support across patient lifespan.
• General practice pharmacists are key to managing long-term complications.
• Addressing inequalities and stigma is critical to improving outcomes.

Kimesha Kangethe is a haemoglobinopathy specialist nurse at the South East London enhanced sickle cell community care team at Guy’s and St Thomas’ NHS Foundation Trust. Meera Patel is a highly specialist pharmacist at the South East London enhanced sickle cell community care team at Guy’s and St Thomas’ NHS Foundation Trust.

Resources

1. Sickle Cell Society. Resources. https://www.sicklecellsociety.org/resources/
2. The Red Cell Network. Haemoglobinopathy Coordinating Centre. https://www.uclh.nhs.uk/theredcellnetwork
3. NHS England. Specialised haemoglobinopathy services. https://www.england.nhs.uk/commissioning/spec-services/npc-crg/blood-and-infection-group-f/haemoglobinopathies/specialised-haemoglobinopathy-services/