Sickle cell pain crises can be extremely severe and painful. Doctors use different treatments, such as pain relievers, and prevention strategies to help people manage these pain crises.

A sickle cell pain crisis is due to the genetic condition sickle cell disease, and it occurs when blood cells clump together and restrict blood flow.

Sickle cell disease is an inherited condition that causes a person’s red blood cells to be sickle or crescent shaped.

It is the most common genetic disorder in the United States and is most prevalent among people of sub-Saharan African, South Asian, Middle Eastern, and Mediterranean heritage.

Sickle cell disease can cause extremely painful episodes, known as sickle cell pain crises.

This article will describe these crises, their causes, and their symptoms. It will also discuss treatment options for sickle cell pain crises, preventive strategies, and when to contact a doctor.

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In sickle cell disease, red blood cells that are typically the shape of rounded disks are instead crescent or sickle shaped. According to the American Society of Hematology, these cells break apart easily, clump together and stick to the walls of blood vessels. This can block blood flow and cause extreme pain.

When there is a blockage in blood flow, it can lead to problems such as anemia, eye problems, stroke, heart problems, and infections.

A sickle cell pain crisis can begin suddenly and last from several hours to several days. A person might feel throbbing, dull, sharp, or stabbing pain in their back, legs, arms, chest, or stomach.

Sickle cell pain crises are often so painful that an individual experiencing one will need medical attention at the hospital.

However, many individuals feel unable to get the care they need because doctors do not fully understand sickle cell disease. In 2015, only around 20% of family doctors reported feeling comfortable caring for people with sickle cell disease.

Learn more about sickle cell disease.

When a person can feel a sickle cell pain crisis starting, they should drink lots of fluids and take over-the-counter (OTC) pain medication. They should also speak with a doctor before taking OTC medication to treat a pain crisis.

OTC medications include:

  • acetaminophen (Tylenol)
  • aspirin
  • ibuprofen (Advil or Motrin)
  • naproxen sodium (Aleve)

Other ways a person might be able to manage their pain include:

  • using a heating pad
  • having a hot bath or massage
  • getting plenty of rest
  • doing activities that distract from the pain

For mild to moderate pain, a doctor might prescribe mild opioid pain relief, such as:

For more severe pain, doctors might prescribe strong opioid pain relief, such as:

A person might take these medications alongside nonopioid pain relief, such as ibuprofen and acetaminophen.

Doctors may also treat a person with intravenous (IV) fluids, which healthcare professionals administer through a vein. In 2019, the Food and Drug Administration (FDA) approved a new IV medication, Crizanlizumab-tmca (Adakveo), which helps reduce pain crises. This medication prevents blood cells from sticking to blood vessel walls and restricting blood flow. It has approval for use in individuals over 16 years of age.

In some cases, doctors may recommend a blood transfusion.

Learn more about Adakveo.

The symptoms of a sickle cell pain crisis can vary depending on the cause of the crisis. Symptoms include:

  • pain in the arms or legs
  • pain in the chest or abdomen
  • swelling in the hands or feet
  • fatigue

If a person experiences respiratory distress, fever, and chest pain, this could indicate acute chest syndrome, and they should seek medical attention.

A sickle cell pain crisis occurs when sickled red blood cells block small blood vessels that carry blood to the bones. There are several common triggers, which include:

  • stress
  • dehydration
  • smoking, which can trigger acute chest syndrome
  • strenuous exercise
  • exposure to extremes of cold or heat
  • infections
  • being at high altitudes, where there is less oxygen available

A person can talk with their doctor on how to avoid triggers. It is also important to note that a person may not be able to know what has triggered a sickle cell pain crisis.

It is difficult to know what causes a pain crisis, and there is often more than one trigger. The American Academy of Family Physicians recommends the following ways to prevent a pain crisis:

  • maintain good general health
  • reduce or avoid stress
  • drink alcohol in moderation
  • quit smoking if a person smokes
  • exercise regularly with moderate intensity
  • drink at least eight 12-ounce glasses of water daily during warmer months
  • treat infections as soon as they occur
  • avoid extremes of temperature
  • if traveling by air, only travel in commercial airplanes that have pressurized cabins with steady oxygen levels
  • get all recommended vaccinations

If a person is experiencing frequent crises, they can speak to a hematologist about treatments that can help prevent episodes.

For most people, a sickle cell pain crisis will resolve on its own. In more severe cases, a person will need to contact a doctor or visit the hospital emergency room.

Sickle cell disease is a lifelong condition that causes challenging symptoms. Although scientists are developing better forms of treatment, such as gene therapies, sickle cell disease remains very serious.

A pain crisis can occur suddenly, so it is important to know where to get help at any time. The Centers for Disease Control and Prevention (CDC) recommend calling a doctor right away if a person experiences sudden vision issues or pain that will not resolve with home treatment.

Individuals should go to the emergency room if they have:

  • a fever above 101°F (38.3°C)
  • breathing difficulties
  • chest pain
  • abdominal swelling
  • severe headache
  • sudden weakness or loss of feeling and movement
  • seizure
  • painful erection of the penis lasting more than 4 hours

A sickle cell pain crisis occurs when sickle-shaped blood cells clump together or lodge in the walls of blood vessels, which restricts blood flow and may cause excruciating pain.

A person can use OTC medications and other methods to treat a pain crisis at home. If the pain continues, doctors might prescribe stronger painkillers, while blood transfusions may be necessary in more severe cases.

Certain situations may trigger a pain crisis, such as being dehydrated, stressed, or having an infection. However, a person can take steps to maintain their health and prevent pain crises.

If an individual cannot manage their pain at home, they should contact a doctor right away. In the event of more serious symptoms, such as fever and difficulty breathing, they should call 911 or seek immediate medical attention.