Sickle Cell Crisis

Imagine dropping something into the sink – maybe it’s just a tiny grape, no big deal, you let the water wash it away. But what if it’s a big piece of Lego? Suddenly, you’ve got a problem. That’s similar to what happens in a sickle cell crisis.

Normally, your red blood cells are round and flexible, allowing them to move smoothly through your body. But in sickle cell disease (SCD), some cells become curved and hard, like a sickle. These rigid cells can get stuck in small blood vessels, causing blockages in organs like your lungs, belly, and joints.

More than 50% of people with SCD experience acute chest syndrome, where the small vessels in the lungs are affected. This can lead to low oxygen levels and long-term lung complications.

During a sickle cell crisis, these stuck cells slow or block blood flow, depriving parts of your body of oxygen, which causes intense pain lasting from hours to weeks. However, there are ways to reduce your risk of a crisis, and even if one occurs, you may be able to manage it at home.

Causes sickle cell crises

Sickle cell crises can be triggered by various factors, and often the exact cause remains unknown. However, there are several potential triggers to be aware of:

1. High altitudes: Activities such as mountain climbing that expose you to high altitudes can trigger a crisis.
2. Temperature changes: Moving from a warm environment to a cold one without adequate protection, such as going from a cozy house into a cold winter day without proper bundling up, can also be a trigger.
3. Infections: Illnesses or infections can sometimes lead to sickle cell crises.
4. Dehydration: Not drinking enough fluids and becoming dehydrated can increase the risk of a crisis.
5. Stress: Emotional or physical stress can also serve as a trigger for a sickle cell crisis.

Symptoms of sickle cell crises

The symptoms of a sickle cell crisis can vary widely from person to person, but the most common sign is sudden and often intense pain. This pain can take on different characteristics like dull, stabbing, throbbing, or sharp sensations. It can strike without warning and its severity and duration can differ from one crisis to another. While some individuals may experience crises sporadically, others might face them monthly.

The pain can manifest anywhere in the body and may occur in multiple locations simultaneously. Common areas include the arms, legs, belly, chest, and for young children, the hands and feet. Additionally, some other symptoms you might experience during a crisis include:

• Breathing difficulties, such as shortness of breath or pain while breathing.
• Overwhelming fatigue.
• Headaches or dizziness.
• Painful erections in males.
• Weakness or difficulty moving certain body parts.
• Yellowish discoloration of the skin, known as jaundice.

Treatments of sickle cell crises

Treatment options for sickle cell crises include several medications and home remedies to manage pain and prevent further complications.

1. Medications:
   • L-glutamine oral powder (Endari): This medication has been shown to help prevent crises and reduce hospitalizations.
   • Hydroxyurea (Droxia, Hydrea, Silkos) and voxelotor (Oxbryta): These drugs work by preventing the formation of abnormal red blood cells, thus reducing the frequency of painful crises.
   • Crizanlizumab-tmca (Adakveo): This medication helps prevent blood cells from sticking together and blocking small blood vessels, which can cause pain and organ damage.
2. Developing a crisis management plan with your doctor is crucial. If hospitalization is necessary, ensure you bring your plan with you.
3. Home remedies:
   • Stay hydrated: Drinking plenty of fluids is essential to prevent dehydration, which can trigger crises.
   • Over-the-counter pain relief: Your doctor may recommend ibuprofen or acetaminophen to manage mild pain. Ensure you consult your doctor to determine the safest option for you, especially if you have kidney issues.
   • Heat therapy: Applying a heating pad or taking a warm bath can provide relief.
   • Massage and physical therapy: These methods may help alleviate pain and improve mobility.
   • Mental health support: Counseling, meditation, and seeking support from loved ones can play a crucial role in managing overall well-being.
4. Seeking medical attention:
   • If home remedies are insufficient for managing pain, seek care in an emergency room where stronger pain medications can be administered.
   • Hospitalization may be necessary until the pain is adequately controlled.

Prevention of sickle cell crises

Preventing sickle cell crises involves a combination of lifestyle adjustments and maintaining overall health. While there’s no foolproof method, you can take steps to reduce the likelihood of experiencing a crisis:

1. Avoid cold water: Swimming in cold water can trigger a crisis, so it’s best to steer clear of it.
2. Stay warm: Dress warmly in cold weather or in air-conditioned environments to prevent temperature-related triggers.
3. Stay hydrated: Drink plenty of water to prevent dehydration, which can contribute to crises.
4. Choose commercial airlines: When flying, opt for commercial airlines that control air pressure, as planes without this feature could pose risks.
5. Limit alcohol: Limit alcohol consumption, as excessive drinking can exacerbate symptoms.
6. Manage stress: Find healthy ways to manage stress, such as through relaxation techniques or therapy.
7. Maintain overall health by:
   • Avoiding sick individuals: Try to avoid close contact with people who are sick to reduce the risk of infections.
   • Quitting smoking: Smoking can worsen sickle cell symptoms, so it’s important to avoid tobacco use.
   • Exercising wisely: Engage in regular exercise, but avoid excessive strain. Intense weight training, for example, may put too much stress on the body. Remember to stay hydrated during exercise.
   • Seeking prenatal care: If you’re pregnant or planning to be, seek prenatal care promptly to manage any potential complications.
   • Managing other health conditions: Work with your doctor to manage any coexisting health conditions, such as diabetes, effectively.
   • Staying updated on vaccinations: Ensure you receive all recommended vaccinations to prevent infections.
   • Addressing sleep issues: Inform your doctor if you experience sleep problems like snoring, as these may impact your overall health.
   • Practicing good hygiene: Wash your hands frequently to reduce the risk of infections.

By incorporating these preventive measures into your daily routine and maintaining good overall health, you can help reduce the frequency and severity of sickle cell crises.

Sources

1. CDC: “Sickle Cell Disease.”
2. FamilyDoctor.org: “Sickle Cell Disease.”
3. Mayo Clinic: “Sickle Cell Anemia.”
4. American Family Physician: “Practical Tips for Preventing a Sickle Cell Crisis.”
5. National Health Service (U.K.): “Sickle Cell Disease.”
6. KidsHealth: “What Is a Sickle Cell Crisis?”
7. National Institutes of Health: “What Are the Signs and Symptoms of Sickle Cell Disease?,” “How Is Sickle Cell Disease Treated?”