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Living with sickle cell disease (SCD) can mean experiencing symptoms that are painful, unpredictable, and not always visible to others. You may have had times when your pain was underestimated, or your concerns were dismissed.
SCD is an inherited blood disorder that causes red blood cells (RBCs) to be abnormal. These abnormal RBCs can cause anemia (too few healthy red blood cells) and can also block blood flow throughout the body, leading to pain, fatigue, and a wide range of other symptoms.
Learning what symptoms to watch for may help you describe what you’re experiencing, recognize when something has changed, and decide when to seek care.
Sickle cell disease starts in the blood, but it can affect nearly every part of the body. It makes red blood cells become sickle-shaped, sticky, and less flexible. These abnormal cells can block blood vessels and cause a variety of symptoms.
Sickle-shaped RBCs break down much faster than normal cells, with sickle cells surviving only 10 to 20 days. Because the body cannot replace them as fast as they are being destroyed, most people develop chronic anemia.
Together, blocked blood flow and chronic anemia are responsible for many disease symptoms.
Signs, symptoms, and complications of sickle cell disease can include:
Pain is a common reason people with SCD seek medical care. Sickle cell pain is unpredictable and can be severe and debilitating. It can interrupt sleep, work, school, relationships, and everyday plans.
Sickle cell pain may feel different from one episode to the next. Some people experience acute pain that comes on suddenly and lasts for a short time.
Others live with chronic pain that continues on most days for months or longer. It’s also possible to experience both types of pain.
Acute pain in SCD is called a pain crisis or a vaso-occlusive crisis. A pain crisis can cause intense throbbing, stabbing, or sharp pain. Pain crises can be very severe and often lead to an evaluation in the emergency department or hospitalization.
The pain can occur in any part of the body, but common locations include the arms, legs, chest, stomach, and lower back. Young children often experience painful swelling of the hands and feet during a pain crisis. A fever can accompany a pain crisis and needs prompt medical attention.
Pain crises can start out of nowhere, but there are known triggers, including:
Fatigue may also be a warning sign that a crisis is approaching.
Most people with SCD experience a pain crisis by the time they are 6 years old. Pain patterns can change as you get older.
Because everyone’s pain experience and treatment needs are different, work with your medical team to develop an individual pain plan (or pain action plan). This plan outlines how to treat your acute pain when you go to the emergency department or are hospitalized. This individualized pain plan should be reassessed yearly and updated for you and your team to reference.
Chronic pain associated with sickle cell disease is pain that occurs on most days for at least six months. Although it can occur at any age, it’s more common in adults. It can be related to long-term organ or nerve damage.
Some people develop chronic pain as a result of nerve damage caused by inadequate blood supply. This kind of pain is called neuropathic pain.
Chronic pain can significantly impact people with sickle cell disease. On top of the severe pain experienced, it can be difficult to receive proper care.
Adults with sickle cell disease have often received poor care for chronic pain. Most adults with SCD are people of color. For many years, the medical field falsely claimed that people of color didn’t feel pain as strongly as white people, according to the New England Journal of Medicine.
Today, healthcare professionals may still downplay sickle cell pain and ignore what their patients are feeling, which can have severe emotional effects. One traumatic experience can make later pain episodes feel much more frightening and distressing.
When people with SCD don’t receive pain relief, the brain may become more alert to pain signals. This can cause people to notice pain more often and experience more distress from it.
Fatigue is an ongoing state of exhaustion that can severely impact daily activities and quality of life. Fatigue can be hard to explain, and it may get less attention than pain crises, even when it affects daily life just as much.
Over time, some people and families may start to see constant tiredness, poor sleep, brain fog, or long recovery time after pain as “normal” parts of life with SCD. But these symptoms still matter, especially when they affect school, work, parenting, relationships, or daily routines.
There are a few reasons people with sickle cell disease experience fatigue.
Because sickled red blood cells break down quickly, the body can’t replace them fast enough. This leaves fewer red blood cells in the body, causing anemia. Fatigue is a major symptom of anemia.
In addition to fatigue, anemia can also cause:
Experiencing pain at night can make it difficult to get a good night’s sleep. Additionally, people with SCD commonly experience sleep disorders. Any disruptions to sleep can lead to fatigue.
Some symptoms and complications of SCD may receive less attention than pain and fatigue.
SCD can damage the brain even when there are no obvious signs of a stroke. This is known as a silent brain injury or silent stroke. Problems with learning and decision-making may follow, affecting school or work.
Many people aren’t aware that sickle cell disease can cause nocturnal enuresis (bedwetting). This can feel embarrassing, but it’s a known symptom of SCD and not a child’s fault. Bedwetting often improves with age.
Sickle cell disease can affect the kidneys’ ability to concentrate urine. These problems can start in childhood and continue into adulthood. Kidney damage can progress over time and lead to kidney failure.
Regular monitoring of your kidney health, including urine tests for protein, is important because early treatment may help slow kidney damage.
People with SCD commonly experience anxiety and depression. Living with a severe, unpredictable illness can be exhausting. Barriers to accessing care can add to this stress.
Constant fear of experiencing a pain crisis, worrying about complications, barriers to accessing treatment, and physical limitations are among some of the reasons people with SCD often experience anxiety and depression.
Anxiety and depression are associated with more daily pain and a lower quality of life.
Sickle cell disease can cause symptoms relating to sexual health, including:
These symptoms can be hard to talk about, but they’re common enough in SCD that your care team should take them seriously. Treatment may help and can sometimes prevent complications.
Sickle cell disease can cause severe symptoms and complications. For many people with SCD, going to the emergency department can bring worry about being dismissed, misunderstood, or not treated quickly enough. In addition, the medical team treating you may have limited experience treating sickle cell disease.
Knowing which symptoms need urgent attention can help you recognize when you need emergency care, even when getting that care feels difficult or exhausting.
Seek emergency medical care right away if you experience:
Call 911 for possible stroke symptoms, such as sudden weakness or numbness, trouble speaking, seeing, or walking, or confusion.
Sickle cell symptoms vary from person to person, and some can become dangerous. Staying connected with a healthcare team can help, but getting the right care isn’t always easy. Transportation, insurance, cost, limited access to SCD specialists, and past experiences of not being believed can all make care harder to manage.
If something feels new, worse, or different from your usual symptoms, reach out to your healthcare team promptly. For the emergency signs listed above, seek care right away. You deserve care that takes your symptoms seriously.
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