
Sickle cell disease is an inherited genetic disorder that affects the shape of red blood cells, the body’s main delivery system for oxygen.
As blood passes through the blood vessels of the lungs, red blood cells pick up oxygen molecules, which are then carried and delivered to tissues and organs throughout the body. Red blood cells also carry carbon dioxide (a waste product produced when cells use oxygen) back to the lungs so it can be exhaled.
Red blood cells are produced in the bone marrow, the spongy tissue found at the core of many bones throughout the body. When a person has sickle cell disease, mutations in certain cells found in the bone marrow create abnormally shaped red blood cells. Instead of the round, flat, disk-or doughnut-shape of healthy red blood cells, the bone marrow produces red blood cells shaped like crescents or sickles.
Sickled cells are less effective at carrying and delivering oxygen and are more fragile than healthy red blood cells. This reduces the body’s ability to deliver oxygen to cells and tissues, causing symptoms like fatigue, weakness, and anemia.
Sickled cells can also become stuck in small blood vessels, forming blockages that obstruct blood and oxygen delivery to tissues and organs. This is called a sickle cell crisis or vaso-occlusive crisis. These episodes can be intensely painful and can lead to serious complications, including permanent damage to organs like the spleen, lungs, liver, kidneys, and brain.
Damage can accumulate with repeated sickle cell crises, and preventing these episodes is such a critical part of treatment.
Many people with sickle cell disease are prescribed medications to reduce the frequency. Additionally, habits and lifestyle strategies are an essential part of prevention.
Daily habits and strategies that can help prevent sickle cell crises
Your healthcare providers will be your best source of information about treatment and steps you can take to prevent sickle cell crises, and it’s always best to consult with a healthcare provider before making any drastic lifestyle changes, such as changes to diet and exercise routines.
With that in mind, here are some topics and triggers that you should consider and discuss:
- Hydration. You will want to drink plenty of water each day. Dehydration can trigger a sickle cell crisis. Coffee, tea, and other liquids also count, but any high-calorie/high-sugar beverages like sodas and energy drinks should be limited.
- Nutrition. People with sickle cell disease are encouraged to follow a heart-healthy diet. Eat plenty of vegetables, fruits, whole grains, healthy fats, and lean proteins. Avoid sugary foods, processed foods, and unhealthy fats. Talk to your healthcare provider about any nutritional supplements you should be taking.
- Smoking, vaping, alcohol, and recreational drugs. If you use tobacco or vaping products, quit—your healthcare provider can help. Also talk to your healthcare provider about limiting alcohol consumption and any recreational drug use.
- Travel. Air contains less oxygen at higher altitudes and lower oxygen levels contribute to cell sickling. Talk to a healthcare provider about precautions to take during flights or when visiting areas at higher altitude. Do not travel in an unpressurized aircraft.
- Temperature changes. Sudden changes in temperature and extreme heat or cold can trigger a crisis. Ease into water when swimming. Drink plenty of fluids and seek shade during hot days.
- Exercise. Ask your healthcare provider for exercise guidelines. Regular physical activity improves many aspects of health, but symptoms like fatigue can make exercise challenging for people with sickle cell. Exercise that is too intense or strenuous can also trigger a sickle cell crisis.
- Stress. Mental and emotional stress are also potential triggers. Take steps to reduce stress, such as time to relax and activities like meditation or yoga.
- Sleep. Aim for consistent bedtimes and 7 to 8 hours of sleep per night. Tell your provider if you are having trouble sleeping or if you feel sleepy during normal waking hours.
- Pregnancy. Talk to your healthcare provider about pregnancy, any plans to become pregnant, and birth control. Pregnancy can raise the risk of sickle cell complications, and sickle cell can raise the risk of pregnancy complications. Some medications used to treat sickle cell disease can cause pregnancy complications and birth defects. Hormonal contraceptives that contain estrogen can increase the risk of blood clots.
Also talk to your healthcare provider about infection risk and steps to prevent infections, including staying up to date on vaccinations.
Sources: National Organization for Rare Disorders... + 15
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