Sickle cell anemia impairs your body’s ability to circulate oxygen. Since every part of your body must have oxygen in order to live, the effects of sickle cell anemia can be deadly or result in severe organ and tissue damage. The anemia that sickle cell disease causes is a shortage of cells that carry oxygen in the blood, and it is caused by something called sickling.
What is Sickling?
Sickling is the tendency of red blood cells to elongate, conforming to the abnormal shape of sickle hemoglobin. When this occurs, the crescent-shaped red blood cells cluster together in the smaller blood vessels and bones and cause blockages. Sickling and its effects can cause death, severe pain, and swelling in the extremities and abdomen, as well as many other complications.
Episodes of sickling and pain are commonly referred to as a sickle cell crisis, a sickling crisis, or a pain crisis. The underlying cause of the crisis is the anemia caused by the sickle cells either dying too soon or too many of them dying at the same time, or clogging up your blood vessels.
What are Sickling Symptoms?
Commonly experienced symptoms during a sickle cell crisis include:
- Anemia
The most common symptom of anemia is fatigue. While many patients chronically experience anemia, more severe symptoms of anemia may be experienced during a sickle crisis. These include: shortness of breath, headaches, dizziness, pale skin, and even yellow discoloring of the skin.
- Pain
When sickle cells block circulation, patients with sickle cell anemia commonly experience the pain and swelling previously mentioned. When this occurs in the hands and feet, it is called hand and foot syndrome, and children experience this symptom the most. Males commonly experience priapism, or painful erections that can last hours if not days. The Centers for Disease Control and Prevention states that an erection accompanied with severe pain lasting more than one hour requires urgent medical attention.
- Spleen Sequestration
Under normal conditions, the spleen acts as a filter to reduce infections and remove abnormal blood cells. With sickle cell anemia, the spleen can trap too many of your blood cells, a cause for anemic conditions. When this happens, you may need a blood transfusion to increase the amount of red blood cells, and your spleen may become enlarged. Over time, your spleen may become stiffened and damaged from the effects of sickling and may need to be surgically removed.
- Acute Chest Syndrome
Sickling can cause blockages in the lungs in patients with sickle cell anemia. Sudden chest pain may result, as well as shortness of breath and a feeling of tightness or pressure in the chest. Acute chest syndrome can be linked with infection and can be fatal.
- Pulmonary Hypertension
When the pulmonary vasculature becomes obstructed from sickling, the pressure increases and triggers the heart to pump harder to get blood to and through the lungs and on to the rest of the body. Symptoms of pulmonary hypertension may include: shortness of breath, chest pain, fatigue, and over time, an enlarged heart. Patients with sickle cell anemia should seek emergency care for pulmonary hypertension.
- Gallstones
Sickle cell anemia causes red blood cells to die approximately 100 days sooner than healthy red blood cells. When these cells die, the sickle hemoglobin is broken down into bilirubin. When bilirubin levels are in excess, deposits can build up in the gall bladder. These deposits are called gallstones, and they can cause pain, nausea and vomiting, fever-like symptoms, and jaundice (yellow discoloration of the skin). Many patients with sickle cell anemia eventually undergo gallbladder surgery.
