Top Triggers of Sickle Cell Crisis: What Every Patient Should Avoid

Sickle Cell Disease (SCD), or sickle cell anaemia, is a hereditary blood disorder that affects millions of people across Africa, including Uganda, Nigeria, Ghana, Zimbabwe, Kenya, and Tanzania. An episode of pain caused by sickle-shaped red blood cells (RBCs) blocking blood flow in small blood vessels is called a crisis. Sickle cell crises can lead to extreme amounts of pain, organ damage, fatigue, and other serious complications.

According to the WHO,

"Sickle cell crises in Africa are primarily triggered by infections (especially malaria and bacterial illnesses), dehydration, and extreme temperature shifts. Because sub-Saharan Africa accounts for nearly 80% of global cases, with newborn prevalence up to 3% in countries like Nigeria and DR Congo, avoiding these triggers is critical."

Not all crises can be prevented; however, knowing the common triggers can reduce their frequency and severity. There are several environmental and lifestyle factors that can trigger a crisis for someone who has SCD, including changes in temperature, dehydration, exposure to illness or infection, or physical or emotional stress. By avoiding these triggers, people with SCD can improve their quality of life and experience a more consistent state of health.

What Is a Sickle Cell Crisis?

A sickle cell crisis (also called a vaso-occlusive crisis) occurs when the abnormal shape of RBCs makes them inflexible and allows them to clump together, thereby preventing the delivery of oxygenated blood to the bodyâ€™s organs and tissues.

Symptoms include:

Pain in bones, chest, abdomen or joints
Swelling of hands and feet
Fatigue and weakness
Shortness of breath
Fever
Dizziness

Pain crises can last hours to days or weeks in some cases may necessitate hospitalisation.

Dehydration: One of the Most Common Triggers

Dehydration causes the blood to thicken, making it easier for sickled cells to cluster together and block blood vessels. When the body lacks sufficient water, blood volume decreases, which causes sickle cells to cluster more easily.

In many African countries, many people can become dehydrated due to high temperatures and limited access to clean drinking water. How to avoid dehydration:

Drink water often all day long
Drink more when it is hot
Have a water bottle while travelling
Limit consumption of sugary and caffeine-containing beverages
Be aware of how much water you drink when you are active

Children and adults with sickle cell disease are at greater risk for dehydration than others and should be closely monitored for signs of dehydration.

Heat: A Major Sickle Cell Crisis Trigger

Extreme heat is compounded by dehydration, which increases the need for fluid to cope with the added stress of high temperatures; individuals are at greater risk of a crisis. Heat management is particularly prevalent in many areas of Africa, where high temperatures persist year-round. Heat-Related Safety Tips

Stay indoors during the hottest parts of the day
Wear lightweight, breathable clothing
Use any available shade
Increase fluid intake during heat waves
Avoid performing demanding outdoor activities during peak temperatures

Cold Weather and Sudden Temperature Changes Triggers

Cold temperatures can also trigger sickle cell crises, despite the fact that they cause a rise in temperature. Due to their narrowness, blood vessels can become constricted in cold climates, reducing blood flow and increasing the risk of blood vessel blockage.

Sudden temperature fluctuations, even in warmer regions, can be contributed to by wet weather, air conditioning systems, or contact with cold water. Prevention Tips:

Dress appropriately for cold weather
Keep warm clothing for your children during the rainy season
Avoid jumping into very cold water unless your doctor advises otherwise
Always, when necessary, use blankets and warm clothing

When Physical Activity Becomes Too Much

Regular exercise is beneficial for overall well-being, but intense exertion can trigger a sickle cell crisis. High-intensity physical activity can also increase the body's oxygen demand and lead to dehydration (both of which contribute to sickle cell crises).

People with sickle cell disease do not need to stop exercising completely. They should still exercise; however, they must do activities that are consistent with their fitness and health condition. Exercising Safely:

Choose moderate-intensity activities, such as walking or riding a stationary cycle
Take lots of breaks while exercising
Drink plenty of water before, during and after you exercise
Do not exercise in very hot conditions
Stop exercising if you feel dizzy, have chest pain or experience unusual fatigue

How Infections Trigger Pain Crises

Infections are a major cause of sickle cell crises and can progress to significant complications very quickly. Infection-prone illnesses, like the flu and pneumonia, can create significant increases in the body's inflammatory response and cause stress on the body.

According to studies, "Several commonalities also emerged across the regions. Although individuals in sub-Saharan Africa were younger on average, the proportion who reported experiencing 1 to 4 vaso-occlusive crises in the past 12 months was comparable across settings (56% in sub-Saharan Africa, 54% in other LMICs, and 50% in HICs). Additionally, fever and infections emerged as common complications, while fatigue, headache, and bone aches were the three most frequently reported disease symptoms."

Children suffering from sickle cell disease are at a greater risk because they will not have the same immune system functioning efficiency. Reducing the Risk of Being Infected

Washing your hands often
Keeping vaccinations current
Avoiding the close proximity of others who are sick
Seeking medical care for fevers as early as possible
Following any preventive medications prescribed by your doctor

In someone with sickle cell disease, a fever must not be ignored and may indicate a serious infection.

The Risk of Low Oxygen Levels

When people are exposed to low-oxygen conditions (e.g., at high altitudes), red blood cells are more likely to become sickle-shaped, which can be hazardous for those diagnosed with sickle-cell anaemia. Precautions to be taken:

Before travelling to high-altitude destinations, it is important to talk to your doctor
Be aware of possible signs and symptoms such as dyspnoea (shortness of breath) or extreme fatigue
Follow your doctor's advice regarding travel and exercise

Why Treatment Adherence Matters in SCD

For a number of patients with sickle cell anaemia, medications are used to manage symptoms, prevent complications and decrease the risk of crisis.

Patients who miss medications or do not show up for their visits are at increased risk of developing health problems. Staying on schedule:

Take medications exactly as prescribed
Use reminders or medication organisers so that you do not skip doses
Make regular appointments for check-ups
Report any new symptoms to your medical provider
Always keep a copy of your medical history

A Simple Sickle Cell Crisis Prevention Checklist

Patients and their caregivers can help reduce their risk of a sickle cell crisis by developing a set of daily behaviours. Here are some guidelines to help minimise the risk of a crisis:

Drink plenty of fluids
Avoid extreme temperatures for long periods
Get enough sleep and rest
Manage stress in an effective way
Exercise moderately, but donâ€™t exert yourself
Keep your body from being infected (be aware of proper handwashing and the importance of vaccinations)
Eat a healthy and balanced diet
Take any prescribed medications as directed
See your doctor regularly

Go to the hospital immediately if you experience any of the following symptoms:

Severe pain, or pain that gets worse over time
Fever
Chest pain
Difficulty breathing
Weakness on one side of the body
Excessive fatigue
Signs of dehydration
Sudden swelling that persists

Early medical intervention can help reduce the severity of complications and improve the chances of a positive outcome.

Conclusion

While sickle cell crises can be painful and serious, many environmental triggers are preventable. The most common causes of a crisis are dehydration, exposure to extreme heat, exposure to infections, emotional stress, lack of sleep, overexertion, and not receiving medical attention.

Understanding these triggers and developing good health habits can be extremely helpful to people throughout Africa who are living with sickle cell disease. Staying hydrated, minimising stress, preventing infection, getting routine medical care, and being aware of environmental factors can help reduce the number of pain episodes (crises) experienced by an individual with sickle cell disease, enabling a more active and healthy lifestyle.

Prevention of pain episodes continues to be one of the best long-term ways for individuals living with sickle cell disease to manage their disease.

Reference

Frequently Asked Questions

A sickle cell crisis often causes sudden pain in the bones, joints, chest, abdomen, or limbs. Some patients may also experience swelling, fatigue, fever, shortness of breath, or dizziness. If the pain is severe or accompanied by fever or breathing difficulties, seek medical attention immediately.

Yes. Dehydration is one of the most common triggers of a sickle cell crisis because it makes the blood thicker and increases the likelihood of blood vessel blockages. Drinking enough water every day can significantly reduce the risk of pain episodes.

Yes, moderate exercise is generally beneficial. Activities such as walking, cycling, or light fitness routines can help maintain overall health. However, overexertion, dehydration, and exercising in extreme heat should be avoided, as they can trigger a crisis.

You should seek urgent medical care if you experience severe or worsening pain, fever, chest pain, difficulty breathing, signs of dehydration, sudden weakness, or unusual swelling. Early treatment can help prevent serious complications.

Yes. Both physical and emotional stress can increase the risk of a crisis by placing extra strain on the body. Managing stress through adequate sleep, relaxation techniques, regular physical activity, and emotional support can help reduce the frequency of pain episodes.