Caring for the sickle cell patient requires a lot of nursing skill and paying strict attention to many signs and symptoms of the disease. Sickle cell anemia is a serious illness in which the body makes sickle-shaped red blood cells. “Sickle-shaped” means that the red blood cells are shaped like a “C.” Normal red blood cells are disc-shaped and look like doughnuts without holes in the center. They move easily through the blood vessels. Red blood cells contain the protein hemoglobin. This iron-rich protein gives blood its red color and carries oxygen from the lungs to the rest of the body. Sickle-shaped cells don’t move easily through the blood vessels; they’re stiff, sticky and tend to form clumps. The clumps of sickle cells block blood flow in the blood vessels that lead to the limbs and organs. Blocked blood vessels can cause pain, serious infections and organ damage.
Sickle cell anemia is an inherited disease. People who have the disease inherit two copies of the sickle cell gene – one from each parent. Sickle cell anemia is most common in people whose families come from Africa, South or Central America (especially Panama), Caribbean islands, Mediterranean countries (such as Turkey, Greece, and Italy), India, and Saudi Arabia. In the United States, it’s estimated that sickle cell anemia affects 70,000–100,000 people, mainly African Americans.
The most common signs and symptoms are linked to anemia and pain. Other signs and symptoms are linked to the disease’s complications. The most common symptom of anemia is fatigue (feeling tired or weak). Other signs and symptoms of anemia include shortness of breath, dizziness, headache, coldness in the hands and feet, pale skin and chest pain. Sudden pain throughout the body is a common symptom of sickle cell anemia. This pain is called a “sickle cell crisis.” Sickle cell crises often affect the bones, lungs, abdomen and joints. A sickle cell crisis occurs when sickled red blood cells form clumps in the bloodstream. This causes pain and organ damage. The pain from sickle cell crises can be acute or chronic, but acute pain is more common. Acute pain is sudden and can range from mild to severe. The pain usually lasts from hours to a few days. Chronic pain often lasts for weeks to months. Chronic pain can be hard to bear and mentally draining. This pain may severely limit daily activities.
Nurses need to be very aware that the effects of a sickle cell crisis on different parts of the body can cause a number of complications. Sickle cells can block the small blood vessels in the hands or feet. This condition is called hand-foot syndrome. It can lead to pain, swelling and fever. One or both hands and/or feet may be affected at the same time. Obvious things to control are pain and fever. The swelling may need to be treated with a diuretic. Tape measure readings should be performed and changes need to be monitored on both the hands and the feet.
In some cases, the spleen may trap cells that should be in the bloodstream. This causes the spleen to grow large and leads to anemia. If the spleen gets too clogged with sickle cells, it won’t work right. This can cause the spleen to shrink. It is referred to as a splenic crisis. If this happens, nurses may need to administer blood transfusions until the body can make more cells and recover.
Both children and adults who have sickle cell anemia have a hard time fighting infections. This is because sickle cell anemia can damage the spleen, an organ that helps fight infections. Infants and young children who have damaged spleens are more likely to get infections that can kill them within hours or days. Pneumonia is the most common cause of death in young children who have sickle cell anemia. Meningitis, influenza and hepatitis are other infections that are common in people who have sickle cell anemia. Therefore, we must administer IV antibiotics and other drugs for hepatitis and/or meningitis when ordered.
Acute chest syndrome is a life-threatening condition linked to sickle cell anemia. It’s similar to pneumonia. The condition is caused by an infection or sickle cells trapped in the lungs. People who have this condition usually have chest pain and fever. They also often have abnormal chest x-ray results. Over time, lung damage from acute chest syndrome may lead to pulmonary arterial hypertension (PAH). In this case, nurses need to observe for the most common symptoms, which are shortness of breath and problems with breathing. Oxygen may need to be started as well.
Two forms of stroke can occur in people who have sickle cell anemia. One form occurs when a blood vessel in the brain is blocked. The other form occurs when a blood vessel in the brain bursts. A stroke can cause learning disabilities and/or lasting brain damage, long-term disability, paralysis or death. As with any stroke, nurses must carefully monitor all vital signs on a regular basis and a head-to-toe assessment must be completed daily.
Sickle cells also can clog the small blood vessels that deliver oxygen-rich blood to your eyes. This can damage the retinas-thin layers of tissue at the back of your eyes. Without enough blood, the retinas will weaken. This can cause serious problems, including blindness. As healthcare professionals, we need to check the patient’s eyesight and pay particular attention to any visual disturbances the individual may express.
When red blood cells die, they release their hemoglobin. The body breaks down this protein into a compound called bilirubin. Too much bilirubin in the body can cause stones to form in the gallbladder. Here we need to watch for symptoms of gallstones. The classic symptom to watch for is a steady pain that lasts for 30 minutes or more in the upper right side of the abdomen, under the right shoulder or between the shoulder blades. The pain may happen after eating fatty meals. In addition, the patient may display symptoms such as nausea, vomiting, fever, sweating, chills, clay-colored stools or jaundice.
Leg ulcers can occur in the sickle cell patient. We need to observe for sores that begin as small, raised, crusted areas on the lower third of the leg. Leg ulcers occur more often in males than in females, and they usually appear between the ages of 10 and 50. The cause of sickle cell ulcers isn’t clear and the number of ulcers can vary from one to many. As with any other abnormal body signs, these need to be reported immediately to the doctor for additional treatment.
Multiple organ failure is rare, but serious. It happens if you have a sickle cell crisis that causes two out of three major organs (lungs, liver or kidney) to fail. Nurses need to be on the lookout for fever, changes in mental status and loss of interest in surroundings. This is a medical emergency and one that needs stat (immediate) attention. Sickle cell is one of the most complex disorders I have ever worked with. The disease is a very serious illness and nurses need to be keenly aware of the multiple signs and symptoms that go along with caring for the sickle cell patient.