Sickle Cell Crisis: Types, Causes, Symptoms, Treatment and Management

Key takeaways:

• Sickle cell crisis happens when sickle-shaped red blood cells block blood flow, causing pain and complications.
• Types of Sickle cell crises include pain crisis, lung crisis, anemia crisis, spleen crisis, and blood breakdown crisis.
• Causes of Sickle cell crises include dehydration, infections, cold or hot weather, stress, low oxygen, smoking, alcohol, and certain medicines.
• Symptoms of Sickle cell crises include severe pain, swelling, trouble breathing, dizziness, tiredness, yellow skin or eyes, vision issues, and chest pain.
• Treatment includes pain relief, IV fluids, oxygen, blood transfusions, and medicines like hydroxyurea.

Sickle cell crisis is a painful condition that occurs when abnormally shaped red blood cells obstruct circulation in small blood vessels. This obstruction limits the oxygen reaching various tissues, causing sharp and severe pain, typically in the chest, back, arms, or legs.

The discomfort can persist for several hours or even days, with the intensity differing from one person to another.

Sickle cell crises are a frequent complication of sickle cell disease (SCD) and may happen repeatedly in some individuals.

Individuals with sickle cell disease have a mutation in the HBB gene, resulting in the production of an abnormal type of hemoglobin called HbS. When oxygen levels decrease, HbS makes red blood cells rigid and take on a crescent shape.

These misshapen cells tend to stick together and obstruct small blood vessels, which limits oxygen flow to tissues and causes intense pain, known as a vaso-occlusive crisis.

Various factors can trigger a sickle cell crisis, such as dehydration, cold exposure, infections, stress, and high altitudes.

Reduced fluid levels or cold temperatures slow blood flow, causing sickled cells to cluster and block vessels. Infections and physical strain further increase the risk. Symptoms may include severe pain, swelling, fever, fatigue, breathing difficulty, or jaundice.

How often does sickle cell crisis occur?

The frequency of sickle cell crises differs for each person. Some may experience episodes only a few times a year, while others have them more often or rarely at all. How often they occur depends on factors like hydration, stress, infections, and overall health management.

For those with more severe sickle cell disease, crises can happen monthly or even more frequently, while others might go long periods without one.

Preventative steps like drinking plenty of water, avoiding temperature extremes, managing stress, and taking prescribed medications such as hydroxyurea can help reduce how often crises occur.

Types of sickle crisis

Sickle cell crises occur in various types. Some of these types are listed below:

Vaso-occlusive crisis (pain crisis)

This is the most common type of sickle cell crisis. It occurs when sickle-shaped red blood cells clump together and block small blood vessels, restricting oxygen flow to tissues.

This lack of oxygen leads to sudden and intense pain, which can affect various parts of the body, including the chest, back, arms, and legs. The pain can range from mild to severe and may last for several hours or even multiple days, requiring medical attention in some cases.

Hemolytic crisis

This happens when sickle-shaped red blood cells break down at an abnormally fast rate, causing a rapid decline in hemoglobin levels.

This sudden loss of red blood cells leads to symptoms such as jaundice, which results in yellowing of the skin and eyes, dark-colored urine due to excess hemoglobin breakdown, and feelings of weakness or extreme fatigue.

In severe cases, this crisis can worsen anemia and may require medical intervention to restore healthy blood levels.

Splenic sequestration crisis

This type occurs when sickled red blood cells get trapped in the spleen, causing it to enlarge and reduce circulating red blood cells.

This leads to severe anemia, fatigue, rapid heartbeat, and abdominal pain. More common in children, it can progress to life-threatening shock if untreated, requiring urgent medical care.

Hyper-hemolytic crisis

This is a rare but serious condition in which red blood cells are destroyed at an accelerated rate, outpacing the body's ability to produce new ones. This rapid breakdown causes a significant drop in hemoglobin levels, leading to worsening anemia.

Common symptoms include jaundice, which results in yellowing of the skin and eyes, dark urine due to excess hemoglobin breakdown, and severe fatigue. In some cases, the condition may worsen quickly and require urgent medical treatment to prevent complications. 

Causes of sickle cell crisis

Some common causes and triggers include:

• Dehydration: When the body is dehydrated, red blood cells are more likely to stick together and block blood flow.
• Infections: Even small infections can cause swelling and lower oxygen levels, leading to a crisis.
• Cold weather: Exposure to cold or sudden temperature changes can tighten blood vessels, making blockages more likely.
• Stress: Feeling physically or emotionally stressed can increase the number of sickle cells in the blood.
• High places: At high altitudes, the air has less oxygen, which can trigger a crisis.
• Too much exercise: Strenuous activity reduces oxygen in the body, raising the risk of a crisis.
• Medical procedures: Surgeries or certain health conditions can lower oxygen levels or cause dehydration, leading to a crisis.
• Pregnancy: During pregnancy, the body makes more blood, which can increase the chance of a crisis.
• Alcohol or smoking: Both can dehydrate the body and lower oxygen in the blood.
• Not enough rest: Poor sleep or extreme tiredness can weaken the body, making it easier for a crisis to happen.

Symptoms: what sickle cell crisis feels like

Sickle cell crises can be extremely painful and vary from person to person. The main symptom is sudden, severe pain, but other symptoms may also occur. They include:

• Swelling and tenderness, especially in the hands and feet.
• Extreme fatigue and weakness due to reduced oxygen supply.
• Shortness of breath and rapid heartbeat, sometimes accompanied by chest pain.
• Fever and increased risk of infections, which can worsen the crisis.
• Yellowing of the skin and eyes (jaundice) due to the breakdown of sickled cells.

Complications of sickle cell crisis

Complications of sickle cell crisis include:

• Stroke – A lack of proper blood flow to the brain may result in a stroke, potentially causing lasting damage.
• Acute chest syndrome – This serious and potentially fatal lung condition is marked by chest pain, difficulty breathing, and fever.
• Organ damage – A continuous lack of oxygen can progressively lead to failure of the kidneys, liver, or spleen.
• Infections – The spleen’s weakened function makes individuals more susceptible to severe bacterial infections.
• Leg ulcers – Inadequate blood circulation may result in painful, slow-healing sores on the legs.
• Blindness – Poor blood flow to the eyes can impair vision and may eventually lead to complete loss of sight.
• Priapism – Restricted blood circulation in the penis can cause prolonged and painful erections.
• Gallstones – The excessive breakdown of red blood cells increases the likelihood of gallstone formation.
• Severe anemia – The rapid destruction of sickled red blood cells can lead to a dangerously low red blood cell count.

Treatment and management

Sickle cell diagnosis is not the end of the world. There are management options that can help the individual to live a relatively normal lifestyle.

Managing and treating sickle cell crisis

Effective management of a sickle cell crisis involves a combination of medical interventions and self-care practices to alleviate pain, prevent complications, and enhance overall health.

Medical interventions

Medical interventions aim to manage the symptoms and improve the overall quality of life. They do this by:

• Pain management – Mild pain can be treated with over-the-counter medications like ibuprofen, while severe pain may require prescription opioids.
• Blood transfusions – In critical situations, transfusions help increase healthy red blood cells and oxygen levels.

Medications

These medications help to manage the symptoms and improve the overall quality of life.

• Hydroxyurea – Increases fetal hemoglobin to lower the number of pain episodes.
• Voxelotor – Helps prevent red blood cells from deforming into a sickle shape.
• Crizanlizumab – Reduces the risk of blood vessel blockages and painful crises.
• Antibiotics and vaccines – Protect against infections that can trigger complications.

Advanced treatment options

While these are expensive, they provide a more permanent solution to Sickle cell.

• Bone marrow transplant – The only known cure, mainly recommended for severe cases.
• Gene therapy– Gene therapy for sickle cell disease involves modifying or replacing the faulty hemoglobin-producing gene to restore normal red blood cell function. FDA-approved treatments like Casgevy (CRISPR-based) and Lyfgenia (gene addition therapy) offer potential cures.

Surgical intervention

Surgery is sometimes needed to manage complications of sickle cell disease (SCD), even though it is not a main treatment.

Common procedures include joint replacement for bone damage, splenectomy to remove a damaged spleen, and cholecystectomy to take out the gallbladder when gallstones develop.

Other surgeries, like removing the tonsils to help with breathing problems or eye surgery for vision issues, may also be needed. Special care before and after surgery—such as staying hydrated, managing pain, and getting enough oxygen—helps lower the risk of complications.

Self-care and lifestyle adjustments

These lifestyle adjustments will also help to manage the symptoms and improve the overall quality of life.

• Stay well-hydrated – Drinking sufficient water helps keep the blood from thickening and reduces the risk of blockages.
• Avoid temperature extremes – Dressing warmly in cold weather and staying cool in hot weather can prevent triggers.
• Reduce stress – Practicing relaxation methods like meditation and deep breathing can help prevent crises.
• Maintain a balanced diet – Eating iron rich foods in case of iron deficiency, folic acid, and essential nutrients supports red blood cell health.
• Exercise in moderation – Light physical activity is beneficial, but excessive exertion should be avoided.
• Prevent infections – Regular vaccinations and good hygiene habits lower the risk of infections.
• Avoid smoking and alcohol – Both can constrict blood vessels and worsen dehydration.
• Regular medical checkups – Visit your doctor consistently rather than just undergoing occasional health checks helps detect and prevent complications early.

When to seek emergency medical care for sickle cell crisis

Immediate medical attention is necessary if any of the following symptoms occur during a sickle cell crisis:

• Intense and unbearable pain that does not improve with home remedies or medication.
• Trouble breathing, rapid breaths, or chest pain, which could indicate a serious lung complication.
• A high fever exceeding 101°F (38.3°C), suggesting a severe infection that requires urgent care.
• Persistent headache, sudden weakness, or confusion, which may be signs of a stroke.
• Blurred vision, sudden eyesight changes, or partial or complete loss of vision.
• Severe stomach pain, swelling, or tenderness, which could indicate organ damage.
• A prolonged and painful erection lasting more than four hours, which may cause permanent harm.
• Extreme tiredness, dizziness, or weakness, possibly due to severe anemia.
• Noticeable paleness or yellowing of the skin and eyes, which could signal worsening anemia or liver issues.

Wrap up

While sickle cell crises can be a painful experience, there are treatment and management options to help manage the symptoms. Sickle cell patients should also take note of the lifestyle changes to reduce the occurrence of the crisis.