Living with sickle cell crisis prevention living

Overview

Sickle cell disease is a lifelong blood condition you are born with. Normally, red blood cells are round and flexible, but in sickle cell disease they become hard and crescent-shaped, like a sickle. These abnormal cells can get stuck in small blood vessels, causing pain and other problems. A sickle cell crisis is a sudden attack of pain that happens when these cells block blood flow to part of the body.

Sickle cell disease is one of the most common inherited blood conditions worldwide. Millions of people are affected, especially in Africa, the Middle East, India, and the Mediterranean. It is less common in Northern Europe but still affects many people of African or Caribbean heritage.

Sickle cell disease mainly affects people whose ancestors came from parts of the world where malaria is common, including sub-Saharan Africa, India, Saudi Arabia, and Mediterranean countries. Both parents must carry the sickle cell trait to pass the disease to a child.

Symptoms

Common symptoms

• Sudden pain in the chest, back, arms, legs, or abdomen
• Swelling of the hands or feet, especially in children
• Feeling very tired or weak (fatigue)
• Shortness of breath or difficulty breathing
• Jaundice (yellowing of the skin or eyes)

Symptoms in children

• Swollen and painful hands or feet (dactylitis)
• Paleness or tiredness from anemia
• Delayed growth or development
• Frequent infections
• Crying or screaming in a baby due to pain

Symptoms in older adults

• Long-lasting joint and bone pain
• Leg ulcers that are slow to heal
• Vision problems due to eye damage
• Kidney or heart complications
• Frequent pain crises

Causes

Main causes

• Sickle cell disease is inherited (passed from parents through genes).
• It happens because of a mutated gene that affects hemoglobin, the protein that carries oxygen in red blood cells.
• A crisis can be triggered by dehydration, cold weather, stress, infection, high altitude, or overexertion.

Risk factors

• Having both parents carry the sickle cell gene
• Being born in or having ancestors from regions where malaria is common
• Being exposed to triggers like cold air, strong winds, or low oxygen
• Having frequent infections or high fever
• Having poor hydration or skipping scheduled treatments

When to see a doctor

See a doctor urgently if:

• If you have sudden, severe pain that does not go away with home treatment
• If you have breathing problems, chest pain, or a persistent fever
• If you have a painful erection that will not go down
• If you feel confused, weak, or have a severe headache

Book a routine appointment if:

• Regular checkups at your specialist doctor (usually every 3–12 months)
• If you notice new symptoms, such as worsening fatigue, frequent infections, or vision changes
• If you are planning to get pregnant or want to think about family planning
• If you have questions about managing pain or preventing crises

Diagnosis

Sickle cell disease is most often found at birth through newborn screening. It can also be diagnosed before birth with a prenatal test, or later in life if someone has symptoms. A simple blood test is used to confirm the disease.

Tests that may be done

• Newborn screening blood test (heel prick)
• Hemoglobin electrophoresis to examine types of hemoglobin
• Complete blood count (CBC) to check red blood cell levels
• Prenatal testing (amniocentesis or chorionic villus sampling) when there is a family history
• Genetic testing for parents to see if they carry the sickle cell trait

What to expect at your appointment

You will meet with a specialist (a haematologist) and other team members such as nurses, social workers, and counsellors. The doctor will ask about your symptoms and family history. They will do a physical exam and arrange blood tests. If you are diagnosed, they will discuss a care plan and how to manage pain, infections, and daily activities.

Treatment

Treatment for sickle cell disease focuses on reducing symptoms, preventing crises, and managing complications. Your plan will depend on how severe your condition is and your personal needs. The goal is to help you live as comfortably and actively as possible.

Self-care at home

• Drink plenty of water every day to stay well hydrated
• Keep warm and avoid sudden changes in temperature
• Get enough rest and pace yourself during activities
• Take pain medicines as advised by your doctor for breakthrough pain
• Follow a plan for any fever or infection, and get urgent help when needed
• Avoid smoking and second-hand smoke

Medical treatments

Doctors may recommend medicines to reduce pain and the number of crises, regular blood transfusions for severe anemia or complications, and antibiotics or vaccinations to prevent infections. A medicine called hydroxyurea is sometimes used — your doctor will explain if it is suitable for you. You may also have regular eye checks, ultrasound scans of the brain, and kidney monitoring. In some cases, a stem cell (bone marrow) transplant can offer a potential cure, but this is not suitable for everyone.

When is surgery considered?

Surgery is rarely needed for sickle cell disease itself. It may be considered for complications, such as removing the spleen if it causes serious problems, or for a stem cell transplant. Your healthcare team will explain all risks and benefits before any surgery.

Living with this condition

Living with sickle cell disease means learning to recognize your body's signals and planning ahead. Keep a pain journal to understand what triggers your crises. Always have a stocked bag with water, snacks, medicines, and contact details for your care team. Inform your school or workplace about your condition so you can adjust activities when needed.

Lifestyle tips

• Drink at least 8–10 glasses of water or fluids each day, more in hot weather
• Dress warmly in layers, especially in air-conditioned rooms or during winter
• Avoid extreme physical exertion, but do gentle activities like walking or stretching
• Plan ahead for travel, especially flights or high-altitude locations
• Have a regular sleep schedule and aim for 7–9 hours of sleep
• Practice stress-relief habits like deep breathing, meditation, or talking to a friend

Diet and exercise

Eat a balanced diet rich in fruits, vegetables, whole grains, and lean protein. Make sure to get enough folic acid (a nutrient that helps your body make new red blood cells) — your doctor can advise on foods like leafy greens, beans, and fortified cereals. Gentle exercise, such as swimming, cycling, or yoga, can help your joints stay flexible and improve mood. Avoid pushing yourself too hard, and always warm up and cool down.

Mental health and emotional wellbeing

Living with a chronic illness can be stressful, and stress can actually trigger a crisis. It is normal to feel anxious, sad, or frustrated at times. Talking to a counsellor, joining a support group, and staying connected with loved ones can help. You deserve emotional support, not just medical care. If you are thinking about harming yourself, please contact a crisis line or emergency services right away.

Prevention

Sickle cell disease itself cannot be prevented if you inherit the gene, but many crises and complications can be prevented. Staying hydrated, avoiding cold and stress, keeping up with vaccines, and following your treatment plan can significantly reduce the number and severity of crises.

Vaccines

Vaccinations are very important. Follow your national vaccination schedule, and make sure you are up to date on flu, pneumococcal, meningococcal, and hepatitis vaccines. Ask your doctor about travel vaccines and any others that are recommended for people living with sickle cell disease.

Screening programmes

If you are considering having children, genetic counselling and screening can help you understand your options. Newborn screening ensures early diagnosis. If you carry the sickle cell trait, a specialist can explain how it may affect your children.

Complications

If left untreated

• Damage to organs, such as the lungs, liver, kidneys, or eyes
• Increased risk of serious infections
• Strokes or brain damage
• Acute chest syndrome — a serious condition where sickle cells block blood flow in the lungs
• Frequent severe pain that affects daily life and mental health

Long-term outlook

The outlook for sickle cell disease has greatly improved in recent years. Many people live into their 50s, 60s, and beyond with good quality of life. Advances in treatment and care mean that pain episodes can be better managed, and serious complications are less common. Your team will help you create a plan so you can stay as healthy and independent as possible.

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