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Symptoms and Complications
Sickle Cell Vaso-occlusive Crises  Stroke Acute chest syndrome Liver complications Fever & Infections Spleen complications Prolonged painful non-sexual erections Pain crisis in the hands and feet Multisystem organ failure
Anemia chronic hemolytic anemia
Bone complications avascular aseptic necrosis
Chronic pain
Headaches
Delayed puberty
Fatigue
Gallstones
Jaundice
Kidney problems
Leg sores ulcers
Organ failure
Vision problems retinopathy
Brain Lungs Liver Spleen Genitals Hands & Feet Multisystem Organ Failure

Learning about the signs and symptoms associated with sickle cell can help you and your loved ones work with your healthcare team to address long-term complications.

Impact of Sickle Cell on the Body

Sickle cell affects everyone differently. People with sickle cell may experience symptoms differently, and these symptoms can change over time.

Although sickle cell is present at birth, most newborns don’t experience problems until they are 5 months old. This occurs because fetal hemoglobin protects their red blood cells from sickling. However, over time, the fetal hemoglobin is replaced by hemoglobin sickle, which begins to clump together and cause red blood cells to sickle. The American Academy of Pediatrics recommends children under the age of 5 receive certain vaccinations and medications to help prevent invasive pneumococcal disease.

It’s important to note that although a person may not be experiencing symptoms, the silent damage of sickle cell is progressive, and frequency of symptoms may increase with age.

Acute Complications and Vaso-occlusive Crises

People with sickle cell may experience problems early on, while others may not experience symptoms and complications until later. Acute symptoms may come on suddenly and last for a short period of time. However, the underlying damage may be ongoing and requires monitoring and management with your healthcare team.

In people with sickle cell, sickle-shaped red blood cells can damage blood vessels and block blood flow throughout the vessels of the body. This blockage, known as vaso-occlusion, can prevent your organs and tissues from getting the oxygen they need and cause vaso-occlusive crises. Vaso-occlusive crises include pain crises, acute chest syndrome, and stroke—these can cause excruciating pain or even death.

“I had to be put into a medically induced coma because of how bad the pain was.”

— Trey, Living With Sickle Cell

Pain crises are unpredictable and extremely painful, lasting anywhere from a few hours to a few weeks, and are the most common reason for hospitalization. Frequency, duration, and intensity of pain crises varies with age and peaks in young adulthood.

In addition to causing pain, the reduced blood flow and blockage can impact your entire body and cause:

  • BRAIN
    Stroke (overt and silent)

    Blood flow to the brain becomes blocked by sickled red blood cells preventing the brain from getting the oxygen it needs to function
  • LUNGS
    Acute chest syndrome (ACS)

    Occurs when not enough oxygen is getting to the lungs when the blood vessels of the lungs are blocked due to sickling. If a person with sickle cell has a drop in oxygen levels, a cough, or difficulty breathing—particularly with a fever—they should be checked for acute chest syndrome
  • LIVER
    Liver complications (hepatic sequestration)

    Trapped red blood cells prevent blood flow through the liver and cause the liver to enlarge over a short period of time
  • SPLEEN
    Fever & Infections

    Damage to the spleen affects its ability to filter blood and increases the risk of a bacterial infection. If you have a temperature of >101.3°F, call your doctor
  • Spleen complications (acute splenic sequestration)
    Sudden trapping of red blood cells in the spleen causing the spleen to grow over a short period of time and can cause blood pressure drops and severe illness

  • GENITALS
    Prolonged painful non-sexual erections (priapism)

    Red blood cells become trapped in the penis causing painful erections
  • HANDS & FEET
    Pain crisis in the hands and feet (dactylitis)

    Blocked blood vessels cause painful swelling of the hands and feet, especially in young children
  • MULTISYSTEM ORGAN FAILURE
    A catastrophic vaso-occlusive crisis from a severe trigger leading all organs to fail due to sickling in the whole body
The blockages and damage from sickled blood cells that cause acute symptoms can cause organ damage and can progress to organ failure, and sometimes cause early or sudden death.
Areas of the body potentially impacted by chronic sickle cell complications
Photo of Trey, living with sickle cell
 

I would say the pain varies. Sometimes it’s just like a pressure—just being squeezed or bones are breaking or being stressed. Sometimes it’s pointy, like a thousand needles kind-of-pain…"

I would say the pain varies. Sometimes it’s just like a pressure—just being squeezed or bones are breaking or being stressed. Sometimes it’s pointy, like a thousand needles kind-of-pain…"

TREY

Living With Sickle Cell

Chronic Symptoms and Complications

Even when you are not in pain, ongoing vaso-occlusion and damage to your blood vessels from sickled red blood cells can cause damage to your body leading to chronic (arising over a longer period of time) complications including organ damage or even failure. These longer-lasting complications can include:

Symptoms Definition 
Anemia (chronic hemolytic anemia) Sickled red blood cells break apart and die faster than the body can replace them, reducing the amount of oxygen throughout the body. This shortage of red blood cells can also cause fatigue
Bone complications (avascular aseptic necrosis) Sickled red blood cells in bones such as the thigh bones can decrease oxygen flow to the bones and severely damage the joints
Chronic pain Ongoing back, joint, and bone pain when nerve endings get damaged from having recurrent pain episodes
Headaches Occur due to low blood counts, but if severe, can be a sign of a migraine or even a stroke caused by blockages from sickled cells in the blood vessels
Delayed puberty Chronic anemia may cause slower sexual maturity
Fatigue Anemia, depression, and/or use of pain medications may cause lack of energy and tiredness
Gallstones Broken down red blood cell products form into stones in the gallbladder causing pain on the right side of the abdomen, especially after eating
Jaundice A large number of red blood cells break down and release bilirubin, causing the skin and the whites of the eyes to yellow
Kidney problems Trapped red blood cells prevent blood flow to the kidneys, causing damage, limiting its ability to filter blood and concentrate urine. This may cause nighttime wetting as well as chronic kidney disease (CKD), and can potentially lead to kidney failure (renal failure)
Leg sores (ulcers) Painful sores that often appear on the lower part of the leg and grow over time
Organ failure Repeated blockages and long-term damage from sickled cells eventually cause an organ to completely stop working
Vision problems (retinopathy) Blocked or bleeding blood vessels caused by damage from sickled cells can lead to retinal detachment and vision loss

Chronic complications are a significant cause of illness in adults with sickle cell. Because chronic complications may go unnoticed and can cause damage to the organs and other parts of the body, it is important to work with your doctor to develop a care plan. Learning how to monitor and manage these complications may help limit their progression, and help you maintain your overall health as best as you can.

Recognizing the impact your symptoms have on your health now and in the long term may help you when discussing comprehensive care with your healthcare team. Take a short quiz to see how sickle cell impacts you.

What Can You Do?

Speaking up helps. Spark a conversation with your doctor to make sure you are receiving comprehensive care that includes careful monitoring and management
for your sickle cell.
Every voice, no matter how big or small,
can spark a conversation and action to
help drive change for sickle cell.


Recognizing how sickle cell impacts your
life may help spark important
discussions with others.
How does sickle cell affect you?