What is a sickle cell crisis?

Sickle cell crisis is a health complication of sickle cell disease, an inherited blood disorder. It usually causes severe pain and occurs as a result of an acute blood cell reaction. Sometimes a sickle cell crisis is triggered by physical stress, such as an infection, or it can occur without a trigger.

Pain attacks are treated with painkillers. Medications are also available to help prevent sickle cell crisis. Some people with sickle cell disease experience crises frequently, while some people with the disease experience crises rarely.

Types of sickle cell crisis

Several conditions are defined as sickle cell crises, and they can cause sudden, severe, and sometimes dangerous effects.

The main common types of sickle cell crisis are:

  • Vaso-occlusive pain: This type is thought to be the most common. This happens when red blood cells form a sickle shape and block small blood vessels. This can cause extreme pain in the affected area of ​​the body.
  • Acute chest syndrome: This type occurs when small blood vessels in the lungs are blocked by sickle-shaped red blood cells. It causes shortness of breath, low oxygen levels, and can be fatal.
  • Aplastic crisis: This occurs when the bone marrow slows or stops making red blood cells, causing severe anemia (low number of healthy red blood cells).
  • Spleen sequestration: This occurs when red blood cells build up in the spleen. It can cause an enlarged spleen and severe anemia.
  • End-Organ Damage: Sickle cells can build up in the blood vessels of any organ, causing pain and organ damage. Examples include priapism (prolonged erection of the penis), intestinal ischemia (damage to the intestine due to lack of blood flow), and stroke (blocked blood flow in the brain).

Sickle cell disease has other health effects that are not considered sickle cell crisis, including anemia, a tendency to infection, the risk of serious complications such as kidney and liver damage, and avascular necrosis (a type of joint damage).

Symptoms and Complications

Sickle cell crisis can cause a variety of symptoms. These are related to the affected organs.

Symptoms may include:

  • Pain can affect the muscles, bones, and joints in the limbs, chest, or back.
  • Shortness of breath is caused by acute chest syndrome.
  • When organs are affected, generalized weakness, low blood pressure, pain, or altered consciousness may occur.
  • Aplastic anemia may cause severe weakness or loss of consciousness.

These symptoms may appear within hours or days. You can have more than one type of sickle cell crisis at a time.

A sickle cell crisis can cause lasting damage to the body that worsens with more frequent episodes.

Causes and Triggers

Several conditions can trigger a sickle cell crisis. In people with sickle cell anemia, these changes make red blood cells prone to sickling and can also cause changes in small blood vessels that make it harder for blood to flow smoothly.

Sickle red blood cells are not as smooth as healthy red blood cells. Instead, they become sticky and tend to clot and block blood vessels anywhere in the body.

Triggers of sickle cell crisis include:

  • Infect
  • Dehydration (loss of body fluids without adequate replacement)
  • low temperature
  • pressure
  • surgery or medical illness
  • hypoxia

trigger

If you have sickle cell disease, you can also experience a crisis without a trigger, and you probably won’t have a crisis every time you have a trigger.

Can you prevent a sickle cell crisis?

A sickle cell crisis can cause severe distress that takes at least a few days to resolve. Sometimes a sickle cell crisis can be dangerous. For people with sickle cell disease, preventive treatment can help reduce the severity and frequency of sickle cell crises.

Prevention includes medical interventions and lifestyle approaches. Medical interventions to treat sickle cell anemia may reduce the frequency of crises, including hydroxyurea, L-glutamine, chronic blood transfusions, and stem cell transplantation (replacement of blood-forming cells in the bone marrow).

Oxbryta (voxelotor), a drug that prevents red blood cell sickling, has received accelerated approval from the U.S. Food and Drug Administration (FDA) for clinical use in people 4 years of age and older.

Other potential treatments are being investigated in research trials, including anti-inflammatory treatments, Dacogen (decitabine) and tetrahydrouridine (which increases hemoglobin in the blood) and gene therapy (replaces defective genes in stem cells).

Lifestyle approaches to prevent sickle cell crisis include:

  • Stay hydrated, especially after exercise, during an infection, or during any medical illness.
  • Reduce your risk of infection by washing your hands frequently, avoiding close contact with infected people you know, and staying up to date on recommended vaccinations.
  • Wear gloves, hats, and other temperature-appropriate clothing as needed to maintain a stable temperature.
  • Moderate exercise.
  • Avoid high altitudes, where oxygen levels are low.

If you have sickle cell disease, there is no complete guarantee that you will never develop sickle cell disease, but reducing your risk is good for your health.

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What is the treatment for sickle cell crisis?

Generally, sickle cell crisis is managed symptomatically. Most of the time, painkillers are prescribed.

If you can get pain relief with over-the-counter (OTC) medicines, your healthcare provider will tell you how much it is safe to take. Some strong prescription drugs commonly used to treat sickle cell crisis, such as morphine, can have serious side effects, including severe constipation.

Pain control is important, and if OTC pain relievers are insufficient, your healthcare provider will give you prescription-strength oral or intravenous medications to relieve your pain.

Other treatments you may need include:

  • Intravenous (IV, intravenous) fluids
  • supplemental oxygen
  • blood transfusion

If you require hospitalization, we will closely monitor your condition and adjust your treatment as needed.

generalize

Sickle cell crisis is a complication of sickle cell disease. There are several types of sickle cell crisis, which occurs when sickle-shaped blood cells build up in small blood vessels. Attacks can cause severe pain, shortness of breath, or organ damage.

Prevention strategies that include avoiding risk factors such as infection, colds, dehydration, and stress can help reduce the risk of sickle cell crises, but they can still occur. Drugs are also available to prevent sickle cell crisis. Treatment may include pain medication, blood transfusions, or supplemental oxygen.

VigorTip words

If you or a loved one has sickle cell disease, a crisis can be painful and potentially harmful to your health. The more you know about your condition, the better you can help manage factors that can lead to a sickle cell crisis.

Talk to your healthcare provider about a plan so you know what to do if you start to experience early symptoms of sickle cell crisis. Consider joining a support group to meet with others who are going through the same situation as you. You can encourage each other and help each other with practical skills.

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