Complications of Sickle Cell Anemia
Disclaimer: Not medical or professional advice. Always seek the advice of your physician.
Sickle cell anemia is one of a group of inherited disorders that affect hemoglobin. The disease can have life-threatening consequences.
In sickle cell anemia, red blood cells look like a C-shaped tool called a “sickle”. As a result, abnormal red blood cells block blood vessels, inhibiting their ability to carry adequate oxygen to organs and tissues.
If the cells do not receive enough oxygen, people risk developing dangerous symptoms and complications.
Anemia
Decreased number of healthy red blood cells in people with SCA causes signs of low oxygen: paleness, weakness, inactivity, dizziness, shortness of breath, physical development delays, jaundice, dark urine. The destruction of red blood cells leads to excess levels of iron in the body, which deposits in various organs and disrupts their functioning.
Acute Pain Crisis
This is the most common complication of SCA. The crisis is caused by the accumulation of sickle-shaped red blood cells in the vessels. Mutations in hemoglobin cause stiffening of erythrocytes and blockage in blood flow. In this case, episodes of extreme pain can occur anywhere in the body.
The crisis has a sudden onset, and it is impossible to prepare in advance. Patients with sickle cell anemia experience 3 to 10 episodes of severe pain per year.
Acute Chest Syndrome
It is the second most frequent complication in patients with SCA. Deformed red blood cells cluster together and obstruct blood flow in the small vessels of the lungs. Acute chest syndrome often follows an acute pain crisis.
Children with acute chest syndrome may experience abdominal pain, fever, and cough. Adults present with severe chest pain, cough, and shallow breathing. The condition is also accompanied by a drop in oxygen level in the blood that often leads to pulmonary infarction. Acute chest syndrome requires immediate medical attention.
Stroke
Sickle cell anemia increases the risk of a stroke. It is estimated that 25% of patients with this condition have a stroke by age 45. They may also experience silent strokes when sickle-shaped red cells block small blood vessels in the brain.
Splenic Enlargement
Sickle-shaped erythrocytes get trapped and gradually build up in the spleen. Over time, the spleen suffers damage and stops working.
As a result, people become more susceptible to infections, pneumonia, severe COVID-19, and other infectious diseases. Viral infections can also affect the production of red blood cells, worsening the clinical course of anemia.
Splenic inflammation is characterized by weakness, blanching of lips, rapid breathing, fast heartbeat, thirst, abdominal pain.
Liver Enlargement
Blood vessels in the liver become blocked because of clusters of sickle-shaped red blood cells. Chronic inflammatory processes lead to gradual enlargement of the liver. Adult patients may also develop liver failure.
Visual Impairment
Visual impairment can occur when blood vessels in the retina become blocked with sickled red cells. Since the retina sends visual information to the brain, its damage can lower the quality of vision.
Gallstones
There is an increased incidence of gallstones in patients with sickle cell anemia. Healthy red blood cells circulate for about 120 days. Their breakdown produces a substance called bilirubin. It is an orange-yellow pigment.
Sickle erythrocytes break up too easily and survive for only 20 days. When they massively die, it causes a sharp increase in bilirubin levels. The excess pigment forms into clots and eventually into stones in the gallbladder. Gallstones can pose an unpredictable threat to health. In most cases, gallstones do not cause any symptoms at all. But sometimes, they trigger inflammation and obstruction of bile flow.
Kidney Inflammation
Lower oxygen concentration in the kidneys affects their ability to filter blood. Every 6th patient with sickle cell anemia has chronic renal failure. Therefore, adults and children with SCA should be regularly tested for kidney disease in order to prevent serious complications.
Joint Pain
Deformed red blood cells deliver less oxygen to the joints. Eventually, patients develop chronic pain or joint inflammation. The pain is often described as sharp, intense, stabbing, or throbbing. It can affect multiple parts of the body during an attack.
Leg Ulcers
Leg ulcers are caused by poor blood circulation. They often form around the ankles. This type of complication tends to occur in patients aged 10 to 50 years. Most ulcers can heal in several months, while others take longer.
Avascular Necrosis (AVN)
Avascular necrosis ( aseptic necrosis, osteonecrosis) is a disease that causes bone tissue death due to poor blood supply. In the case of SCA, necrosis occurs when sickle cells block the blood flow that feeds bone tissue. Osteonecrosis can ultimately lead to the destruction of bone. The most common site for AVN is the hip joint, but it may also occur in other areas of the body.
This type of complication progresses without noticeable symptoms, and most patients experience pain only when the bone is already seriously damaged.
Movement Disorders
When sickle cells block the vessels that supply various parts of the brain and spinal cord, people may develop neurological complications. They experience a loss of feeling or sensation in the limbs and body, as well as the ability to move. In some cases, sickle cell anemia increases the risk of a stroke.
Priapism
Priapism is a disorder of prolonged and persistent penile erection that is unrelated to sexual interest or stimulation. This erection can last for more than 4 hours. Subsequently, priapism becomes a contributing factor to male infertility.
Growth Impairment and Pubertal Delay
Children with sickle cell anemia often have a short torso and disproportionately long arms, legs, fingers, and toes. They may not grow as fast as their peers. They also tend to reach puberty a couple of years later.
Besides, patients with sickle cell anemia often suffer from depression and sleep disturbances caused by chronic pain and medication.
Sickle Cell Anemia Clinical Trials
Visionaries Clinical Research is currently recruiting volunteers to participate in Phase 1 clinical trial to evaluate the efficacy and safety of an investigational drug in patients with sickle cell anemia. This study will include patients aged between 18 and 65 years (inclusive).