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Sickle Cell Anemia

Chronic Conditions of Sickle Cell Anemia

Patients with sickle cell disease produce misshaped blood cells that have difficulty passing through blood vessels. When a sudden, severe blockage of blood flow occurs (vaso-occlusion), sickle cell patients develop acute problems such as pain, acute chest syndrome, stroke, priapism, and sudden death.

Over long periods of time, damage to muscle, blood vessels, and organ tissues from reduced blood supply due to sickle cell blockages cause chronic conditions to develop.

Common Chronic Conditions Related to Sickle Cell Disease

Chronic Lung Disease

Chronic Lung Disease (CLD) is a general term for a group of symptoms that arise as a result of lung damage and loss of lung tissue. These symptoms include:

There are many causes for CLD such as:

The treatment of CLD in sickle cell is similar to the general population. Lung specialists can recommend medications and/or oxygen therapy.

Pulmonary Hypertension

Pulmonary Hypertension is a condition in which patients develop high blood pressure in the arteries that supply blood to the lungs. It is a common complication in sickle cell patients because it arises as a result of abnormal blood flow. This complication is a major concern because people with sickle cell disease and pulmonary hypertension have a higher risk of death as compared to people with sickle cell disease who do not have pulmonary hypertension.

The exact mechanism that causes pulmonary hypertension is not known. Recent research, though, suggests that the development of chronic lung disease as well as the release of hemoglobin with subsequent depletion of nitric oxide when red blood cells are destroyed may be responsible.

Symptoms of pulmonary hypertension include:

As blood vessels that supply the lungs narrow, this forces the heart to work harder to push blood through. Most of the time, however, patients do not have symptoms, similar to patients with systemic hypertension (high blood pressure).

An echocardiogram (ultrasound) of the heart is a noninvasive test that can diagnose pulmonary hypertension. In some circumstances, a cardiac catheterization is needed to make the diagnosis. As many as one third of adults with sickle cell disease develop pulmonary hypertension. The best treatment for sickle cell patients with pulmonary hypertension is not known. Potential treatments include:

Avascular Necrosis

Avascular Necrosis is the term used to describe the necrosis (or death) of bone tissue. This occurs often as a result of injury or disease when blood supply is cut off (avascular) partially or completely to a bony area of the body; most commonly the hip joint. The condition causes extreme pain and can result in the flattening or collapsing of the bone.

This is a concern for patients with sickle cell because of the constant risk of blocked blood supply all over the body. The treatment for avascular necrosis in sickle cell patients is largely supportive using pain medications, physical therapy, and limiting physical activity. In very advanced damage, an operation to replace the hip joint may be necessary.

Kidney Failure

Kidney failure (also called renal failure) can result from many different conditions and complications. It can occur in sickle cell patients from, first, chronic sickling and reduced blood supply to the kidneys, and second, from the use of non-steroidal anti-inflammatory drugs (NSAIDs). NSAIDs are pain medications such as ibuprofen and naproxen prescribed often to help sickle cell patients manage pain crises. They are very effective and have few side effects and are therefore widely used. However, with excessive use, NSAIDs can cause kidney damage and therefore should be avoided in sickle cell patients that have known kidney problems.

The treatment for kidney failure in sickle cell patients is similar to the general populations. Kidney specialists can recommend medications to help mild or moderate disease. In severe kidney failure, dialysis or a kidney transplant may be necessary.

Gallstones

A gallstone is a calculus (stone) that forms in the gallbladder or biliary passage and blocks the flow of bile into the bowels. The stones can also sometimes block the flow of pancreatic enzymes leading to inflammation of the pancreas (pancreatitis). Gallstones form when there is too much bilirubin in the body. Bilirubin is made when red blood cells break down. People with gallstones may have steady pain in the upper right side of the belly, under the right shoulder, or between the shoulder blades. The pain usually occurs after eating fatty meals. The patient may also have nausea, vomiting, fever, sweating, chills, clay-colored stool, and yellowish color of the skin or whites of the eyes.

Patients with sickle cell disease are at increased risk for developing gallstones because their blood cells are constantly breaking down and releasing bilirubin into the blood. The treatment for gallstones is the same in sickle cell patients as in the general population. Patients need to adopt a low fat diet and have surgery to remove the gallbladder.

Retinopathy

The retina is a thin layer of nerve tissue at the back of the eye that receives and processes visual images. When the retina does not get enough blood, it can weaken and cause problems. These problems can be serious enough to cause blindness. This condition is called retinopathy and it is a particular concern for sickle cell patients due to their impaired blood flow. The peak age of onset of retinopathy is 20-30 years.

For unknown reasons, the condition develops more frequently in patients with hemoglobin SC disease than in those with homozygous sickle cell anemia (SS). Retinopathy can continue to get worse and if it goes untreated. All people should visit an eye doctor annually and those with sickle cell may consider visiting more often particularly when abnormalities are detected because of their heightened risk of developing this complication and related conditions.

Hypertension

Hypertension is the medical term for high blood pressure. In some cases, high blood pressure can be caused by kidney problems. Patients with sickle cell disease are at increased risk for both hypertension and kidney problems because of chronic sickling in the kidneys. (See kidney failure)

Leg Ulcers

Leg ulcers are a common problem for sickle cell patients. The cause of these ulcers is unclear, but likely is the result of chronic damage to the blood vessels in the legs from sickling. We do know that sickle cell ulcers usually begin as small, raised, crusting sores on the lower third of the leg. Leg sores occur more often in males than in females and usually appear between ages 20-50 years. There can be just one ulcer or many. Some heal rapidly, but others persist for years or come back after healing. Methods that have been used with variable results include transfusions and hyperbaric oxygen.

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Last Reviewed: Jul 10, 2009

Eric H Kraut, MD Eric H Kraut, MD
Professor of Hematology
College of Medicine
The Ohio State University

Anthony D Villella, MD Anthony D Villella, MD
Formerly, Assistant Professor of Pediatrics
School of Medicine
Case Western Reserve University