What are the main symptoms or signs of Sickle cell crises: understanding the pain and how to prevent it?

Common signs vary from person to person. The article above lists the symptoms to watch for, how they progress, and when they need medical attention.

When should you see a doctor for Sickle cell crises: understanding the pain and how to prevent it?

See a doctor if symptoms last more than a few weeks, get worse quickly, stop you doing everyday things, or if you notice sudden pain, breathing trouble, fainting or bleeding.

Blood & Hematology

Sickle cell crises: understanding the pain and how to prevent it

Why red cells change shape, and what actually stops a crisis

Dr. Camille Roux

Internal medicine physician

9 min read

Medical illustration of Sickle cell crises: understanding the pain and how to prevent it

In simple terms

How a vaso-occlusive crisis happens, the triggers to avoid, and treatments that reduce their frequency.

What it is, in plain words

In sickle cell disease the haemoglobin inside red cells clumps together when oxygen levels fall. The round, flexible cell turns into a stiff crescent that blocks small blood vessels. Tissue downstream is starved of oxygen and that is what causes the severe pain of a vaso-occlusive crisis. The sickled cells also break apart early, which explains the chronic anaemia and yellowing of the eyes.

Signs to look out for

Deep pain in bones, back, chest, arms or legs, often coming in waves
Painful swelling of hands and feet in young children
Fever, which must always be treated as an emergency
Chest pain with breathlessness, a warning sign of acute chest syndrome
Persistent tiredness, yellow eyes and, over years, damage to spleen, kidneys, hips and eyes

Why it happens

Common triggers are dehydration, cold, infection, fever, high altitude, intense exercise without hydration, stress and alcohol. Many crises have no identifiable trigger at all, which is important to know so patients are not blamed for their pain.

How doctors confirm it

The disease is confirmed by haemoglobin electrophoresis, usually at newborn screening. During a crisis, doctors look for a cause such as infection and check blood count, reticulocytes, oxygen level and a chest X-ray when there is chest pain.

Treatments and solutions

Fast, adequate pain relief: paracetamol and anti-inflammatories for mild pain, and strong opioids without delay for severe crises, as under-treatment of pain is the commonest failure
Rehydration by mouth or by drip, plus warmth and rest
Oxygen if saturation falls, and urgent care for acute chest syndrome
Hydroxyurea taken daily, which raises fetal haemoglobin and clearly reduces the number of crises and hospital stays
Transfusion or exchange transfusion for severe complications, penicillin prophylaxis and vaccination in children, and stem cell or gene therapy in selected patients

Living with it day to day

Prevent crises by drinking plenty of water every day, dressing warmly, treating infections early, keeping all vaccinations up to date, taking folic acid, and having yearly checks of eyes, kidneys and, in children, brain blood flow with transcranial Doppler.

When to seek medical help

Go to the emergency department for fever above 38 C, chest pain or breathlessness, sudden weakness on one side or difficulty speaking, a painful erection lasting over two hours, or a rapidly enlarging abdomen.

This article explains general health information in simple language. It does not replace advice from your own doctor.

The words explained

Medical terms used above, in everyday language.

Chronic: A problem that lasts a long time, usually more than three months, and often comes back.
Acute: Something that starts suddenly and lasts a short time.
Inflammation: The body's reaction to injury or irritation: redness, swelling, heat or pain.
Prevention: Steps taken before a problem starts, to make it less likely.
Screening: A check-up done before you feel ill, to catch a problem early.
Vaccine: A safe training session for your immune system, so it can fight a germ later.

When should you see a doctor?

Your symptoms last more than a few weeks or keep coming back.
They get worse quickly, or stop you doing everyday things.
You notice sudden pain, breathing trouble, fainting or bleeding — seek urgent care.
You already take medication and want to change anything about it.

This article explains general health information. It does not replace advice from your own doctor.

Frequently asked questions

What is Sickle cell crises: understanding the pain and how to prevent it?: How a vaso-occlusive crisis happens, the triggers to avoid, and treatments that reduce their frequency.
What are the main symptoms or signs of Sickle cell crises: understanding the pain and how to prevent it?: Common signs vary from person to person. The article above lists the symptoms to watch for, how they progress, and when they need medical attention.
When should you see a doctor for Sickle cell crises: understanding the pain and how to prevent it?: See a doctor if symptoms last more than a few weeks, get worse quickly, stop you doing everyday things, or if you notice sudden pain, breathing trouble, fainting or bleeding.

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