Sickle cell crisis prevention living

Overview

Sickle cell disease is an inherited blood disorder that changes the shape of red blood cells. Normally, red blood cells are round and flexible. In sickle cell disease, they become stiff and shaped like a sickle, or crescent. These abnormal cells can get stuck in small blood vessels, blocking blood flow. This blockage causes sudden, severe pain called a sickle cell crisis. A crisis can also damage organs over time.

Sickle cell disease is one of the most common inherited blood disorders in the world. It affects millions of people, especially in parts of Africa, the Middle East, India, and South America. It also affects people of African, Caribbean, and Mediterranean descent living in other countries, such as the United Kingdom and the United States.

Sickle cell disease is present from birth. A child must inherit two sickle cell genes, one from each parent, to have the disease. People with one sickle cell gene have sickle cell trait, which usually causes no symptoms. The disease mainly affects people whose ancestors are from Africa, the Caribbean, the Middle East, India, and parts of the Mediterranean.

Symptoms

Common symptoms

• Sudden, severe pain in the chest, back, arms, legs, or belly
• Swelling and pain in the hands and feet, especially in children
• Feeling very tired or weak
• Yellowing of the skin and eyes, called jaundice
• Frequent infections
• Shortness of breath

Symptoms in children

• Painful swelling of the hands and feet, called dactylitis
• A swollen belly that feels hard
• Delayed growth and development
• Pale skin and feeling very tired
• Frequent colds or infections

Symptoms in older adults

• Long-term or chronic pain, especially in the joints and bones
• Leg ulcers that are slow to heal
• Damage to the kidneys, lungs, or eyes over time
• Gallstones
• Feeling very tired due to anemia

Causes

Main causes

• A genetic change in the gene that tells the body how to make hemoglobin, the part of red blood cells that carries oxygen.
• Sickle cell crises are triggered when sickled cells block blood vessels. This can happen during infections, dehydration, extreme cold, high altitude, or intense physical stress.

Risk factors

• Having both parents carry the sickle cell gene
• Being born with sickle cell disease is the only true risk factor for crises.
• Everyday factors like not drinking enough water, getting too cold, overexertion, and stress can trigger a crisis.

When to see a doctor

See a doctor urgently if:

• You have any of the emergency symptoms listed above, such as difficulty breathing or sudden severe pain.
• Your pain medicine does not help, and the pain is getting worse.
• You have a fever of 101°F (38.5°C) or higher.
• You cannot keep fluids down because of vomiting or stomach pain.

Book a routine appointment if:

• Regular checkups with your doctor to monitor your blood counts and organ function.
• If you notice new symptoms like tiredness, jaundice, or leg sores, make an appointment.
• Discuss your pain plan with your doctor even when you feel well.

Diagnosis

Sickle cell disease is usually diagnosed at birth through newborn screening. A simple blood test can show whether the baby has sickle cell disease or sickle cell trait. In countries without newborn screening, it may be diagnosed later when symptoms appear.

Tests that may be done

• A complete blood count (CBC) to check for anemia and red blood cell shape
• A hemoglobin electrophoresis test, which looks at the types of hemoglobin in your blood
• A genetic test to check for the sickle cell gene

What to expect at your appointment

Your doctor will explain your test results and what they mean. If you or your child has sickle cell disease, your care team will build a care plan with you. This plan will include vaccinations, medicines (if needed), and guidelines for managing pain and preventing crises. Many clinics offer education and counseling to help you understand the disease.

Treatment

There is no everyday cure for sickle cell disease, but many treatments can help prevent crises and reduce complications. The aim is to manage pain, protect organs, and keep you healthy. Your care team will tailor your treatment plan to your needs.

Self-care at home

• Drink plenty of water every day to stay well hydrated.
• Keep warm and avoid sudden temperature changes.
• Get enough rest and sleep.
• Prevent infections by washing hands often and staying away from sick people.
• Avoid smoking and second-hand smoke.
• Learn your personal triggers and do your best to avoid them.

Medical treatments

Doctors may prescribe medicines to reduce the number of crises, such as a medicine that helps the body make more fetal hemoglobin. They may also use antibiotics to prevent infections, especially in children. Blood transfusions can help treat severe anemia and prevent stroke in some people. Always talk to your doctor about which treatments are right for you. Never take any medicine without your doctor’s advice.

When is surgery considered?

Some people with sickle cell disease may need surgery to treat complications. For example, surgery to remove the spleen if it is damaged, or surgery to replace a damaged hip. A stem cell transplant (also called a bone marrow transplant) is a treatment that can possibly cure the disease, but it is not an option for everyone. Your doctor will explain the risks and benefits if surgery is considered.

Living with this condition

Living with sickle cell disease means learning to manage your health every day. Follow your care plan, go to regular appointments, and take any prescribed medicines as directed. Keep a pain diary to track when crises happen and what triggered them. This helps you and your doctor make a better prevention plan.

Lifestyle tips

• Drink 8 to 10 glasses of water a day, more if it is hot or you are exercising.
• Dress warmly in cold weather and avoid cold pools or icy environments.
• Do gentle exercise like walking or swimming, but avoid very intense workouts.
• Do not smoke or use recreational drugs.
• Limit alcohol, as it can cause dehydration.
• Manage stress with relaxation techniques, talking to friends, or hobbies.

Diet and exercise

Eat a balanced diet with plenty of fruits, vegetables, whole grains, and protein. Your doctor may recommend a vitamin supplement, especially folic acid, which helps your body make new red blood cells. Stay physically active, but pace yourself. If exercise causes pain or shortness of breath, stop and rest. Always ask your doctor what level of activity is safe for you.

Mental health and emotional wellbeing

Living with a chronic illness can cause stress, anxiety, and sadness. It is normal to feel this way sometimes. You do not have to face it alone. Talk to your doctor about how you are feeling. Some people find therapy or counseling helpful. If you ever have thoughts of harming yourself, seek crisis help immediately.

Prevention

The disease itself cannot be prevented because it is inherited. But you can prevent many sickle cell crises by staying hydrated, avoiding extreme temperatures, preventing infections, and knowing your triggers. With proper care, many people go for long periods without a crisis.

Vaccines

Vaccinations are very important for people with sickle cell disease because the spleen, which helps fight infections, may not work well. Make sure you and your child receive all recommended vaccines, including the pneumococcal, flu, and COVID-19 vaccines. Ask your doctor for the full vaccination schedule.

Screening programmes

Newborn screening detects sickle cell disease early, which is very important. If you are planning a family, you can ask your doctor about genetic screening to see if you or your partner carries the sickle cell gene. This information can help you make informed decisions.

Complications

If left untreated

• Damage to the spleen, which increases the risk of severe infections
• Stroke, especially in children
• Acute chest syndrome, a serious lung condition that can cause breathing problems
• Kidney damage or failure
• Eye problems that can lead to blindness
• Leg ulcers that are difficult to heal
• Gallstones

Long-term outlook

There is good reason to be hopeful. With early diagnosis, good medical care, and careful daily habits, many people with sickle cell disease live full, active lives. New treatments are improving quality of life, and research into better therapies, including gene therapy, is continuing. You are more than your condition. With a strong support team and a clear plan, you can manage the challenges and focus on the things that matter to you.

Find support

International organisations

• Sickle Cell Society (UK) ↗
• Sickle Cell Disease Association of America ↗

Local organisations

• Your local sickle cell and thalassaemia center · Available in many countries, ask your doctor or local health service

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