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

What Is a Sickle Cell Crisis?

Sickle cell crises happen when a person has sickle cell disease, a hereditary blood disorder affecting children. In sickle cell disease, the red blood cells (RBCs) get hard and become sickle-shaped, blocking the blood flow, leading to anemia and painful episodes referred to as sickle cell crises.

In sickle cell disease, the common problem is sudden, severe pain called pain crises, which is treated at the hospital. But sickle cell crisis is a broad term. It contains various serious conditions like pain crises, organ involvement, infection, chest issues, swollen hands and feet, and so on.

When there are sickle cell crises, pain will happen in any part of the body, but is usually seen in the arms, legs, chest and back. Young children often get swelling and pain in their hands and feet early in life.

Sickle cell crisis is diagnosed by genetic testing, a blood test that finds an abnormal type of hemoglobin called hemoglobin S (HbS).

What Are the Types of Sickle Cell Crises and Their Differences?

There are various types of sickle cell crises. The sickle cell crisis types and how they differ are as follows:

1. Vaso-Occlusive Crisis: This is a more commonly seen type of sickle cell crisis. It results in moderate to severe pain that is on and off and varies from one person to another. Children below age six often have pain and swelling in their hands and feet (called dactylitis). Some kids also get a fever.

2. Splenic Sequestration Crisis: This happens when blood gets trapped in the spleen, making it swell suddenly. The trapped blood causes a sudden drop in hemoglobin (Hb) among children under six. This leads to abdominal pain, pale skin, rapid heartbeat, and feeling faint or tired. If it is not treated on time, it can lead to shock or even death.

3. Aplastic Crisis: This happens when the bone marrow temporarily stops making red blood cells, often due to a virus like parvovirus B19. It causes sudden paleness, weakness, and a severe drop in Hb levels. The body cannot keep up because red blood cells in sickle cell disease do not last as long. It usually lasts about a week or so and is managed with rest and blood transfusions if needed.

4. Acute Chest Syndrome: It is a life-threatening lung complication of sickle cell disease and is a major cause of death. It happens after a pain crisis or due to infection, low oxygen, or fat particles from bone getting into the lungs. It causes fever, chest pain, breathing trouble, cough, and low oxygen levels. It is treated with oxygen, antibiotics, and blood transfusions.

5. Hemolytic Crisis and Hypermolysis: This crisis happens when RBCs break down too soon, causing a sudden drop in Hb. It is worse in people with G6PD (glucose-6-phosphate dehydrogenase) deficiency. A special type called hyperhemolysis syndrome may happen after blood transfusion, where both old and new RBCs are destroyed. It results in fatigue, jaundice, and severe anemia. It is managed by stopping transfusions and possibly giving steroids or immunoglobulin.

What Are the Symptoms of a Sickle Cell Crisis?

The symptoms of sickle cell crisis vary based on which part of the body is affected, but below are a few symptoms to look for:

• Sudden, intense pain that occurs anywhere but generally in the arms, legs, back, and chest.

• Fever.

• Breathing issues.

• Chest pain.

• Cough.

• Wheezing.

• Low oxygen levels.

• Fatigue and tiredness.

• Abdominal pain.

• Paleness.

• Rapid heartbeat.

• Jaundice.

• Pain around the belly or chest is referred to as girdle syndrome. It is rare.

What Causes or Triggers a Sickle Cell Crisis?

The cause of sickle cell crisis is sickle cell anemia or disease. Sickle cell disease is a condition you are born with. It happens because of a small change in a gene on chromosome 11. This change causes your red blood cells (RBCs) to become hard, sticky, and shaped like a crescent or sickle. These sickle-shaped cells do not move well through blood vessels and get stuck, blocking blood flow. This is what leads to sickle cell crisis, which causes pain and other symptoms. But what triggers a crisis? Sometimes, the reason is not clear; more than one thing may be involved. Here are a few common triggers that lead to sickle cell flare-ups:

1. Inadequate hydration.

2. Changes from hot to cold weather.

3. Places with low levels of oxygen or high altitudes.

4. Flu or other infections.

5. Pregnancy.

6. Exercising too much.

7. Smoking or vaping.

8. Stress.

9. Some vitamin deficiencies.

How Is a Sickle Cell Crisis Diagnosed?

To diagnose sickle cell crisis, doctors do blood tests to check for an abnormal type of hemoglobin called HbS. If HbS is found, genetic testing is done to confirm the diagnosis. HbS is an abnormal hemoglobin that causes RBCs to be hard and sickle-shaped, leading to a vaso-occlusive type of crisis. This helps to find out if a person has the disease or carries the sickle cell trait.

What tests are done to diagnose sickle cell crisis? Here are the tests that are done to diagnose sickle cell crisis:

• Blood tests involving complete blood count to check red and white blood cells, platelet count, and reticulocyte count show how well the bone marrow is making new RBCs. Metabolic panel and liver function tests are also done to check organ health and body functioning.

• CRP (C-reactive protein), procalcitonin, and blood cultures are done to test for infections and inflammation.

• Chest X-rays are done for acute chest syndrome.

• Abdominal ultrasound for stomach pain and spleen involvement.

• MRI (magnetic resonance imaging), CT (computed tomography), or Doppler tests are done to look for stroke, bone issues, or blocked blood flow.

• Arterial blood gas is done to check oxygen levels if a person has breathing difficulty.

How Is Sickle Cell Crisis Treated?

Management of sickle cell crisis mostly involves symptom management, preventing severity, and stabilizing a person. Sickle cell crisis treatment options include treating a painful crisis. Is a sickle cell crisis painful? A pain crisis is the most common reason people with sickle cell disease go to the hospital. Here is how sickle cell crisis pain management is done:

• Quick pain relief is provided with Morphine through an IV (intravenous) or IM (intramuscular).

• For some people, a PCA (patient-controlled analgesia) pump may be used (a device that lets them control their pain medicine safely).

• Check for vital signs like oxygen and breathing regularly.

• Once the pain is managed, patients are given oral analgesics (painkillers).

• If pain does not get better, hospitalization is recommended. Sometimes, blood transfusion also helps.

• Staying hydrated is important with IV fluids to keep the blood flowing smoothly and prevent more sickling of red cells.

• Acute chest syndrome is a serious lung problem that needs immediate care. It is treated with oxygen, antibiotics, pain management, IV fluids, and a blood transfusion.

• Patients are also encouraged to do incentive spirometry (a breathing exercise) and drink fluids.

• If blood gets trapped in the spleen, to prevent shock, it is treated urgently with fluids and a blood transfusion.

Long-term management includes the following:

• Hydroxyurea to protect against sickling and reduce pain episodes, hospital visits, and transfusions.

• L-glutamine to manage oxidative stress.

• Voxelotor, a medicine that helps RBCs hold on to oxygen better, reduces hemolysis and treats anemia.

• Crizanlizumab is a monthly infusion medicine that blocks sticky cells from clumping and blocking blood vessels and reduces the pain episodes.

Curative options are bone marrow transplant and gene therapy.

How to Prevent a Sickle Cell Crisis?

Sickle cell crisis cannot be completely avoided, but can be prevented by taking care of your health and avoiding the triggers, such as smoking, keeping oneself warm, limiting alcohol intake, which can cause dehydration, drinking water, and hydrating. Taking folic acid supplements helps in making new red blood cells.

What Are the Complications of Sickle Cell Crisis?

There are several sickle cell crisis complications that occur in this condition, many of which become worse over time due to repeated sickling of red blood cells and ongoing damage to organs and tissues. One of the most common and dangerous complications is infection. Because the spleen, which helps fight infection, becomes damaged early in life, people with sickle cell crisis are at high risk for life-threatening infections such as Streptococcus pneumoniae, Neisseria meningitidis, and Haemophilus influenzae type b. In some regions, additional threats include malaria, hepatitis B, and Salmonella.

Pulmonary complications in acute chest syndrome, strokes, delayed hemolytic reactions, and iron overload are also complications of sickle cell crisis.

What Does It Mean to Live With a Sickle Cell Crisis?

Living with sickle cell crises means learning to manage pain, prevent complications, and take care of your overall health daily. It is also important to avoid triggers and stay hydrated. For children, regular doctor visits and up-to-date vaccinations are needed to prevent infections.

When a crisis happens, getting early treatment for sickle cell crisis for pain and breathing issues prevents worsening of the crisis. Also, Hydroxyurea reduces the episodes of sickle cell crisis.

Conclusion:

Sickle cell crisis is a serious and painful complication of sickle cell disease that affects many parts of the body and leads to life-threatening issues if not treated on time. This crisis happens when the blood vessels get blocked due to sickle-shaped red blood cells, leading to pain, organ damage, and other complications like infections, stroke, and acute chest syndrome. It is essential to stay away from triggers, stay hydrated, practice infection prevention, and prompt treatment and care can reduce sickle cell flare-ups and severity.

A Keynote/Takeaway From iCliniq:

Sickle cell crises are top medical emergencies that require immediate action and care. They are common in children, and preventive strategies such as routine vaccinations, infection control, hydration, and regular follow-ups can reduce crisis episodes. iCliniq doctors can help you guide through symptom management, prevention, vaccinations, and infection control.