Last Updated on May 5, 2025 by Analgesia team
Sickle Cell Disease
Overview
Sickle cell disease is an inherited haemoglobin disorder in the red blood cells. It is passed down from both parents.
Sickle cell disease (SCD) is a group of inherited blood disorders that affect the red blood cells. It is caused by a mutation in the gene that helps make haemoglobin—a protein in red blood cells that carries oxygen throughout the body.
In people with sickle cell disease, red blood cells become abnormally shaped like a crescent or sickle, making them stiff and sticky. These misshapen cells struggle to move through small blood vessels, causing blockages that reduce or cut off oxygen delivery to tissues. This leads to complications such as anaemia, pain episodes, and damage to vital organs
Acute painful sickle cell events, also known as painful crises or vaso-occlusive episodes (VOEs), are painful episodes that occur when sickled red blood cells block small blood vessels.
Their red blood cells become deformed, turning into sickle-shaped. Sickle cells die sooner than healthy cells. Normal red blood cells live about 90 to 120 days, but sickled cells only last 10 to 20 days. The bone marrow tries to make more red blood cells to makeup with the loss but can’t keep up, causing anemia. A shortage of red blood cells (RBCs) is called anemia, and people with anemia often feel breathless. Their new shapes keep them from moving properly through the body. and the shapes themselves can jam up and stick to the walls of the blood vessels. These clogs cut off the oxygen supply to these tissues, delaying a child’s normal growth and causing fatigue and extreme pain.
Sickle cell disease is a health condition caused by a gene mutation that affects the hemoglobin molecule in red blood cells, causing it to form into stiff rods.
A simple blood test will tell whether you have sickle cell trait or the disorder. Sickle Cell Society estimates that 17,500 people in the UK have sickle cell disorder.
What Causes Sickle Cell Disease?
Sickle cell disease is genetic, meaning it is inherited from both parents. A child must receive two copies of the sickle cell gene (one from each parent) to have the disease. If they inherit only one copy, they have sickle cell trait, which usually doesn’t cause symptoms but can be passed on to future generations.
The condition primarily affects people of African, Caribbean, Middle Eastern, South Asian, and Mediterranean descent. According to the Sickle Cell Society, over 17,500 people in the UK live with sickle cell disorder.
How Sickle Cell Affects the Body
Healthy red blood cells are round and flexible, allowing them to move freely through blood vessels. In contrast, sickle cells are rigid and shaped like crescents. These cells:
- Tend to clump together and block blood flow.
- Die much faster than normal cells (10–20 days vs. 120 days), leading to chronic anemia.
- Cause oxygen deprivation in tissues, resulting in pain, fatigue, and delayed growth in children.
Common Symptoms of Sickle Cell Disease
SCD symptoms vary in severity but often include:
- Chronic anemia (leading to fatigue, weakness, and shortness of breath)
- Pain episodes (vaso-occlusive crises), which can occur suddenly and last hours or days
- Swelling in hands and feet
- Frequent infections
- Vision problems
- Delayed growth and puberty
- Jaundice (yellowing of the skin or eyes)
Pain is one of the most common and disabling symptoms, and it’s often the reason patients seek urgent care.
Sickle Cell Pain Crisis (Vaso-Occlusive Episode)
One of the hallmarks of sickle cell disease is the sickle cell pain crisis, also called a vaso-occlusive crisis (VOC). These occur when sickled red blood cells block small blood vessels, stopping oxygen from reaching tissues.
Characteristics of a Pain Crisis:
- Sudden, intense pain often in the back, chest, arms, legs, or abdomen
- Can last for hours or even days
- May require hospital admission and strong painkillers
- Often triggered by cold weather, dehydration, stress, or infection
Some people experience pain crises frequently, while others may have them only occasionally. Chronic pain can also develop over time, particularly in older individuals with long-standing disease.
Serious Complications of Sickle Cell Disease
In addition to pain, SCD can lead to life-threatening or disabling complications:
- Stroke – Common in children aged 2–16. Regular screening with transcranial Doppler ultrasound is recommended.
- Acute chest syndrome – A severe lung-related complication that can resemble pneumonia.
- Kidney damage – This can lead to chronic kidney disease or failure.
- Eye damage (retinopathy) – Can cause vision loss.
- Leg ulcers – Difficult-to-heal wounds due to poor circulation.
- Gallstones – From the breakdown of excess red blood cells.
- Infections – Especially from bacteria like Streptococcus pneumoniae, which can be deadly in young children with SCD.
Diagnosing Sickle Cell Disease
SCD is typically diagnosed through a simple blood test called haemoglobin electrophoresis, which can detect abnormal types of haemoglobin. In the UK, newborn screening identifies most cases at birth.
Adults can also be tested to see if they carry the sickle cell trait, particularly if they are planning a family or come from high-risk backgrounds.
Managing and Treating Sickle Cell Disease
There is no universal cure, but treatments can help reduce symptoms, manage pain, and prevent complications. Treatment plans are typically tailored to each patient and may include:
Medications:
- Painkillers – From paracetamol to opioids for managing pain crises.
- Hydroxyurea – A medication that helps reduce the frequency of pain episodes and the need for blood transfusions.
- Antibiotics – Often prescribed to young children to prevent infections.
- Folic acid supplements – To help produce new red blood cells.
Blood Transfusions:
Regular transfusions can reduce the risk of stroke and treat severe anemia, but they carry risks like iron overload.
Bone Marrow or Stem Cell Transplant:
The only potential cure, but it’s only suitable for a small percentage of patients due to donor match requirements and the risk of complications.
Supportive Care:
- Staying hydrated
- Avoiding cold weather
- Getting vaccinated to reduce infection risks
- Managing stress to prevent triggering a pain crisis
Living with Sickle Cell Disease
Sickle cell disease is lifelong, but with the right care, many individuals lead active and fulfilling lives. Living with SCD requires:
- Regular check-ups with a specialist team
- Pain management plans
- Psychological support for dealing with chronic illness
- Education and advocacy, especially for children in school environments
- Planning ahead to manage travel, emergencies, or work demands
Thanks to advances in medicine and awareness, people with sickle cell disease today are living longer and better than ever before.
Final Thoughts
Sickle cell disease is a complex, inherited condition that affects blood and causes a range of serious health issues, especially chronic and acute pain. Early diagnosis, regular care, and a comprehensive management plan can help reduce complications and support a better quality of life.