Sickle Cell Disease
Sickle Cell Disease

Sickle Cell Disease How Sickle Cell Disease May Affect Your Health

The sickled cells that give the disease its name can lead to pain throughout the body and serious damage to organs, such as the heart and kidneys. It is important to be aware of the possible complications of sickle cell disease and know when to seek emergency care.

Some complications may be systemwide (affecting many parts of your body at the same time). Other complications affect specific parts of the body.

Complications affecting your whole body

  • Acute pain crisis: Also known as a sickle cell or vaso-occlusive crisis, this can happen without warning when sickled cells block blood flow. People describe this pain as sharp, intense, stabbing, or throbbing. Pain can strike almost anywhere in the body and in more than one spot at a time. Common areas affected by pain include the abdomen, chest, lower back, or arms and legs. A crisis can be brought on by high altitudes, dehydration, illness, stress, or temperature changes. Often a person does not know what triggers the crisis.
  • Vitamin deficiency: People with sickle cell disease are at higher risk for nutrient and vitamin deficiencies — especially vitamin D, omega 3, vitamin C, and zinc deficiencies — that can also cause a crisis or complications.
  • Chronic (long-term) pain: Chronic pain is common, but it can be hard to describe. It is usually different from crisis pain or the pain that results from organ damage.
  • Delayed growth and puberty: Because of anemia, children who have sickle cell disease may grow and develop more slowly than their peers. They will reach full sexual maturity, but this may be delayed.
  • Infections: The spleen is important for protection against certain kinds of infections. If you have sickle cell disease, a damaged spleen raises your risk for certain infections, including chlamydia, Haemophilus influenzae type B, salmonella, and staphylococcus.
  • Joint problems: Sickling in the hip bones and, less commonly, the shoulder joints, knees, and ankles can reduce oxygen flow and result in a condition called avascular or aseptic necrosis, which severely damages the joints. Symptoms include pain and problems with walking and joint movement. Over time, you may need pain medicines, surgery, or joint replacement.
  • Blood clots: Changes in how the blood travels through blood vessels can result in blood clots. Swelling of your arms or legs, pain or tenderness not caused by injury, sudden shortness of breath, weakness in a specific area of the body, or changes in thinking may be some signs of blood clots.
  • High blood pressure: People with sickle cell disease can develop high blood pressure, which increases the workload on the heart and blood vessels and worsens high-risk complications, like stroke and kidney disease.
  • Pregnancy problems: Pregnancy can raise the risk of high blood pressure and blood clots in people who have sickle cell disease. The condition also increases the risk of miscarriage, premature birth, and low birth weight babies. Learn more about pregnancy and sickle cell disease.

Complications affecting specific parts of your body

  • Acute chest syndrome: Sickling in blood vessels of the lungs can deprive the lungs of oxygen. This can damage lung tissue and cause chest pain, fever, and difficulty breathing. Acute chest syndrome is a medical emergency.
  • Enlarged spleen: The spleen is an organ that helps your body fight infection and remove unwanted material. It filters your blood and destroys old blood cells. In people who have sickle cell disease, red blood cells may get trapped in the spleen. This makes the spleen quickly grow larger than normal. With red blood cells trapped in the spleen, fewer are available to circulate in the blood, and this can lead to severe anemia. A large spleen may also cause pain in the left side of the belly.
  • Eye problems: Sickle cell disease can injure blood vessels in the eye, most often in the retina. This is called sickle retinopathy. Blood vessels in the retina can overgrow, get blocked, or bleed. This can cause the retina to detach, which means it is lifted or pulled from its normal position. These problems can lead to vision loss.
  • Gallstones: When red blood cells break down, in a process called hemolysis, they release hemoglobin. Hemoglobin then gets broken down into a substance called bilirubin. Bilirubin can form stones called gallstones that get stuck in the gallbladder. The gallbladder is a small sac-shaped organ beneath the liver that helps with digestion.
  • Heart problems: These can include coronary heart disease and pulmonary hypertension. Frequent blood transfusions can lead to heart damage from iron overload.
  • Kidney problems: Sickle cell disease may cause the kidneys to have trouble making the urine as concentrated as it should be. This may lead to a need to urinate often and to bedwetting or uncontrolled urination during the night. These problems often start in childhood. Over time, scarring in the filters of the kidney can lead to other complications, such as protein or blood in the urine. There is a higher risk of kidney failure, changes to the kidney, and kidney cancers. Routine monitoring of urine and blood pressure is important.
  • Leg ulcers: Sickle cell ulcers are sores that usually start small and then get larger and larger. Some ulcers will heal quickly, but others may not heal and may last for long periods of time. Some ulcers return even after healing. People who have sickle cell disease usually do not get ulcers until after age 10.
  • Liver problems: Sickle cell intrahepatic cholestasis is an uncommon but severe type of liver damage that happens when sickled red cells block blood vessels in the liver. This blockage prevents adequate levels of oxygen from reaching liver tissue. These episodes are usually sudden and may happen more than once. Children often recover, but some adults may have chronic problems that lead to liver failure. Frequent blood transfusions can lead to liver damage from iron overload.
  • Priapism: Priapism is an unwanted and sometimes prolonged painful erection. This happens when blood flow out of the erect penis is blocked by sickled cells. Over time, priapism can cause permanent damage to the penis and lead to impotence. Priapism that lasts for more than 4 hours is a medical emergency.
  • Stroke or silent brain injury: Silent brain injury, also called silent stroke, is damage to the brain without outward signs of stroke. This injury is common and can be detected on magnetic resonance imaging (MRI) scans. Silent brain injury can lead to difficulty in learning, making decisions, or holding down a job. Annual TCD ultrasound, supplemented with blood transfusion, can prevent stroke in children.

Serious anemia complications

People who have sickle cell disease usually also have mild to moderate anemia. At times, however, they can have severe anemia. Severe anemia can be life-threatening.

Severe anemia in a newborn or child who has sickle cell disease may be a result of one of two conditions:

  • Aplastic crisis: An aplastic crisis is usually caused by a parvovirus B19 infection, also called fifth disease or slapped cheek syndrome. Parvovirus B19 is a very common infection, but in sickle cell disease, it can cause the bone marrow to stop producing new red cells for a while, leading to severe anemia.
  • Splenic sequestration crisis: When red blood cells get trapped in the spleen, fewer are available to circulate in the blood and the spleen can become enlarged. This can lead to severe anemia that causes a pain crisis.

Aplastic crisis and splenic sequestration crisis most commonly occur in newborns and children who have sickle cell disease. Adults who have sickle cell disease may also experience episodes of severe anemia, but these usually have other causes. Babies and newborns who have severe anemia may not want to eat and may seem very sluggish.

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