Joint Pain and Sickle Cell Disease

Acute and chronic pain are very common for people with sickle cell disease (SCD). Almost 1 in 3 adults with SCD have pain every day, and more than 1 in 2 have pain 50 percent of the time.^1,2

SCD causes pain because of blocked blood flow, or vaso-occlusion, by sickle red blood cells. Many complications of SCD can also cause pain. Painful episodes can affect any part of the body for any length of time. People with SCD who experience worse pain tend to have higher fatigue and difficulty carrying out daily tasks.

Some treatments can reduce the frequency of pain crises, most notably hydroxyurea. Pain is usually managed with over-the-counter pain drugs at home, but some people may need more serious treatment in a hospital.

What is sickle cell pain?

Episodes of pain are the main symptom for people with SCD. The pain varies in intensity and can last for a few hours to a few weeks. Some people have a few episodes per year, while others may have a dozen or more per year.³

People describe this pain as sharp, intense, stabbing, or throbbing. It can strike anywhere in the body and in more than 1 spot a time. Commonly affected areas include:⁴

• Abdomen
• Arms
• Chest
• Legs
• Lower back

Pain can also occur in bones.⁴

Some children and adults also have chronic pain. This can be caused by bone and joint damage, ulcers, and other causes. It can also change the brain network and affect response to treatment.^5,6

Pain leads to a lower quality of life for many people with SCD. It often disrupts sleep quality, which can cause feelings of fatigue. It can also affect the ability to carry out daily tasks, and attend school and work.^2,7,8

Why does SCD cause pain?

The acute pain crisis is the most common complication of SCD. It happens when sickled red blood cells block blood flow (vaso-occlusion). Blocked blood flow can prevent oxygen delivery to many different organs. This means acute pain crises can cause sharp pain in any part of the body.⁴Acute chest syndrome is an example of blocked blood flow that affects the lungs. It can cause severe chest pain, fever, and difficulty breathing. Acute chest syndrome is a potentially life-threatening complication of SCD. Other complications of SCD can cause different types of pain, including:⁴

• Gallstones
• Joint complications due to avascular necrosis
• Splenic sequestration

The frequency and severity of painful episodes are affected by some types of thalassemia and levels of fetal hemoglobin. Other factors like temperature, altitude, air quality, and socioeconomic status also impact the severity of pain in SCD.⁹

Depression, anxiety, and asthma are common for people with SCD. These are all linked to greater sensitivity to pain and sleep disturbance.¹

How is pain prevented and treated?

Some ways to reduce or prevent the number of pain crises include:¹⁰

• Avoiding extreme temperatures and altitudes
• Chronic blood transfusions to reduce pain episodes and stroke
• Drinking plenty of water
• Preventing infections with vaccinations and antibiotics
• Taking the drug hydroxyurea

Other drugs approved for people with SCD or drugs used in clinical trials may also reduce the frequency or duration of pain crises.¹

Most acute pain events are managed at home with non-steroidal anti-inflammatory drugs (NSAIDs) like aspirin or ibuprofen or other over-the-counter pain drugs. When home management does not work, treatment in a hospital with opioids may be necessary.¹Treatment in the ER is often delayed. People with SCD wait 25 to 50 percent longer than people without SCD who have similar levels of pain. This is mostly because of lack of understanding of SCD and discrimination in the healthcare system. People with SCD, most of whom are Black, are often labeled as “drug seekers” by ER doctors.¹¹