Progressions and Possible Complications of Sickle Cell Disease

What is sickle cell disease?

Sickle cell disease is an inherited red blood cell disorder that causes hemoglobin to become irregularly shaped. Hemoglobin is a protein in red blood cells that delivers oxygen throughout the body. Normal red blood cells with hemoglobin are smooth, flexible and round. Sickle cell disease results in hemoglobin evolving into a sickle shape. They also become stiff and stick together, causing difficulty moving through blood vessels. Sickle cell disease can cause blockages in blood flow, resulting in anemia, pain, infections, organ failure, etc.

Progressions of sickle cell disease

Although life expectancy is reduced with the progression of sickle cell disease, new treatments are increasing quality of life and life expectancy. The progression of sickle cell disease can result in various events.

Sickle cell anemia

Due to the sickle cells dying at a faster rate than they can be replaced, sickle cell anemia often develops. The body is unable to receive the oxygen needed without healthy red blood cells, which can lead to chronic fatigue.

Vaso-occlusive crisis

Vaso-occlusive crisis is frequently experienced by the age of six and is the most common progression. It is characterized by moderate to severe pain and swelling of the hands and feet. The pain regularly impacts the extremities, back and chest.

Splenic sequestration crisis

When sickle cells pool in the spleen, hemoglobin can drop suddenly. This may cause hypovolemic shock, which can be life-threatening. A spleen infraction frequently occurs by the end of childhood. The spleen becomes damaged and enlarged, resulting in pain.

Aplastic crisis

Aplastic crisis involves rapidly dropping hemoglobin levels and decreased red blood cell production. This usually occurs as a result of parvovirus B19, which suppresses the bone marrow that affects red blood cell production. However, other viral infections may also trigger aplastic crisis. Weakness and extreme anemia may last seven to ten days.

Acute chest syndrome

Acute chest syndrome often follows a vaso-occlusive crisis and can be triggered by hypoxia from hypoventilation of the chest. Another possible cause is a fat embolism originating from the distal bone in a vaso-occlusive crisis. This creates a cycle of producing additional sickle red blood cells due to hypoxia. Symptoms may include chest pain, respiratory distress, fever, cough, and wheezing. It could result in death if not treated aggressively.

Hemolytic crisis

A G6PD deficiency could cause an acute drop in hemoglobin levels. Red blood cells are destroyed at a higher rate than replaced. The portion of the cells that carry oxygen is also released into the bloodstream, potentially causing kidney damage.

Possible complications of sickle cell disease

Numerous complications can arise from sickle cell disease due to blockages and the inability for red blood cells to flow normally. Potential complications include, but are not limited to, the following:

• Eye damage
• Early gallstones
• Increased infections
• Leg ulcers
• Bone damage
• Kidney damage or loss of body water in the urine
• Multiple organ failure
• Pulmonary hypertension
• Splenic sequestration
• Blindness
• Acute chest syndrome
• Stroke
• Deep vein thrombosis
• Pregnancy complications
• Priapism (painful, long-lasting erections)