Understanding sickle cell anemia

Sickle cell anemia (SCA), also known as sickle cell disease, is an inherited red blood cell (RBC) disorder. It’s the result of a genetic mutation that causes misshapen RBCs.

SCA gets its name from the crescent shape of the red blood cells that resemble a farm tool called a sickle. Usually, RBCs are shaped like discs.

RBCs transport oxygen to your body’s organs and tissues. SCA makes it harder for RBCs to carry enough oxygen.

Sickle cells can also get caught in your blood vessels, obstructing the flow of blood to your organs. This can cause a painful condition known as a sickle cell crisis. It can also contribute to the development of a range of complications.

Read on to learn more about these complications and how you can reduce your risk of developing them.

SCA causes blood to have less oxygen, and this usually isn’t severe enough to cause organ damage. But if a sickle cell gets stuck in a blood vessel and blocks the flow of blood to an organ, it can cause permanent damage to organs, including the kidneys, liver, and spleen.

While organ damage isn’t reversible, you can slow down the process if you catch it at an early stage. That’s one reason why regular doctor checkups are important for people with SCA.

Acute chest syndrome results from sickle cells obstructing the blood vessels that lead to your lungs.

Its symptoms include:

  • coughing
  • chest pain
  • difficulty breathing

If you have SCA and notice these symptoms, seek immediate medical treatment. Acute chest syndrome can be life-threatening

Hand-foot syndrome, sometimes called dactylitis, happens when sickle cells block the blood vessels of the hands or feet. For some, this might be the first noticeable symptom of SCA.

It’s marked by painful swelling in the hands or feet. It can also cause a fever in some people.

Treating hand-foot syndrome usually involves a combination of drinking more fluids and pain medication.

RBCs support your body’s growth by providing oxygen and other nutrients necessary for development. When they don’t contain oxygen and nutrients due to SCA, it can result in slower growth rates in children and a later onset of puberty in teenagers. In males, it can also lead to infertility.

Over time, the small blood vessels that supply blood to your eyes can become blocked with sickle cells, causing damage to your retina. Some people also develop extra blood vessels due to reduced oxygen. Both of these can contribute to vision loss.

This why doctors strongly recommend that people with SCA follow up with yearly ophthalmic exams.

When your liver breaks down RBCs, your body makes a substance called bilirubin. Sickle cells break down at a faster rate than typical RBCs, resulting in more bilirubin. Too much bilirubin can form gallstones in the gallbladder, a small organ that stores bile and helps with digestion.

Symptoms of gallstones include:

  • pain in the upper right part of your abdomen
  • pain in the center of your abdomen just below your sternum
  • back pain between your shoulder blades
  • pain in right shoulder
  • nausea and vomiting

In some cases, gallstones can be dissolved with medication. In others, they may need to be surgically removed.

The spleen is an organ responsible for filtering the blood to remove cellular waste, maintaining fluid balance, and activating white blood cells for the immune system. Splenic sequestration happens when the splenic vessels become blocked by a large number sickle cells.

Symptoms of splenic sequestration include:

  • pale lips
  • fast breathing
  • extreme thirst
  • rapid heartbeat
  • sudden weakness
  • pain in left abdomen

Splenic sequestration requires immediate treatment, usually with a blood transfusion. If it happens regularly, you may need to have your spleen removed.

The spleen also helps to filter blood and fight off potentially harmful bacteria. Sickle cells can damage the spleen, making you more susceptible to infections, including the flu, pneumonia, and meningitis

These types of infection can quickly become serious in those with SCA, so it’s important to see a doctor if you have:

  • a fever
  • body aches
  • coughing
  • fatigue

Leg ulcers are open sores in the skin of your leg. People with SCA are more prone to developing them.

Symptoms of a leg ulcer include:

  • swelling
  • aching sensation in legs
  • feeling of heaviness in the legs
  • irritated skin surrounding the open wound

Leg ulcers are treated with compression bandages and topical ointments. In some cases, you may need an antibiotic to prevent or treat an infection in the wound.

A blockage in any of the blood vessels in your brain can lead to a stroke. This is a serious condition that can have lasting consequences.

Seek emergency treatment if you experience:

  • slurred speech
  • inability to raise one arm
  • drooping on one side of the face
  • numbness, often on only one side of the body
  • difficulty walking or moving your arms
  • confusion
  • memory problems
  • difficulty speaking or understanding spoken language
  • headache
  • loss of consciousness or coma

SCA complications aren’t always preventable. But a few important lifestyle changes can reduce your risk or lessen their severity.

Get moderate exercise

It’s important for both adults and children with SCA to get regular exercise.

The Centers for Disease Control and Prevention (CDC) recommends that people with SCA get a total of 150 minutes of moderate aerobic activity, such biking or walking, per week. You might consider breaking that total recommended time into five 30-minute sessions weekly.

The CDC also suggests performing light strengthening activities, such as lifting weights, at least two days a week.

While it’s important to be active, try to avoid heavy exercise or strenuous activities, as these can cause breathing difficulties.

Eat a balanced

To help your body make more red blood cells, eat a diet rich in colorful fruits, vegetables, and whole grains. Try to limit your consumption of refined sugars and fried foods.

You might also want to consider taking a take a folic acid supplement. Bone marrow requires folic acid to make new red blood cells.

Drink water

You should drink plenty of fluids throughout the day, especially in hot weather or while exercising. Dehydration increases your risk of a sickle cell crisis. Aim for 8 to 10 glasses of water every day. Plan to have a few more if it’s warm or you’re going to be exercising more than usual.

Manage stress

Stress can also trigger a sickle cell crisis. While it’s impossible to avoid all forms of stress, some practices for managing stress include:

  • staying organized and planning your day
  • taking time to relax and rest
  • getting adequate sleep
  • breathing exercises
  • practicing yoga or tai chi
  • writing in a diary
  • talking to a friend
  • listening to music
  • going on nature walks

Try to keep tabs on how you feel throughout the day. This can help you identify situations that make you feel stressed so you can work on avoiding or reducing them.

Be aware of temperature and altitude

There’s less oxygen in the air at higher altitudes. This lack of oxygen can trigger a crisis. If possible, you avoid traveling to high-altitude areas.

If you have SCA, you should also try to avoid sudden temperature changes, like jumping into a pool or lake of cold water. When you go outside, make sure you’re dressed appropriately for the weather and consider keeping an extra layer handy.

Reduce your risk of infection

Remember, people with SCA may have a higher risk of infection. As a result, it’s important to take steps to reduce your exposure to viruses, bacteria, and fungi.

Reduce your risk by:

  • washing your hands often, especially after going to the bathroom and before eating
  • avoiding contact with people who have an active infection and spending time in crowded environments
  • cooking and storing food, especially meat, properly to prevent food poisoning
  • making sure you’re up to date on your vaccinations, including a flu vaccination
  • taking any antibiotics as prescribed by your doctor
  • taking extra precautions when traveling abroad, such as only drinking bottled water or bringing along antibiotics if recommended by your doctor
  • avoiding interactions with reptiles, including turtles, snakes, and lizards, as they can carry a harmful Salmonella bacteria

You should contact your doctor immediately if you think you have an infection. Early treatment may prevent a full-blown sickle cell crisis.

Avoid smoking

While smoking’s bad for your health in general, it’s extra risky if you have SCA. It can increase your risk for acute chest syndrome, which can be life-threatening in some cases.

It can also contribute to the development of:

  • a sickle cell crisis
  • leg ulcers
  • pneumonia

Ready to quit smoking? Here’s what you need to know.

If you have SCA, it’s important to see a doctor as soon as you think you might be having complications. The earlier you can treat the issue, the better your chance of preventing long-term issues.

SCA complications can come on suddenly, so make sure you know who to call and where to go for medical treatment. Consider giving this information to close friends and family as well.

You should seek medical attention right away if you have any of the following signs and symptoms:

  • fever above 101°F
  • unexplained, severe pain
  • dizziness
  • stiff neck
  • difficulty breathing
  • severe headache
  • pale skin or lips
  • painful erection lasting more than four hours
  • weakness on one or both sides of the body
  • sudden vision changes
  • confusion or slurred speech
  • sudden swelling in the abdomen, hands, or feet
  • yellow tint to the skin or whites of the eyes
  • seizure

Regular checkups with a doctor are also essential in preventing serious problems. Babies with SCA should see a doctor every three months. Children aged 2 and older, as well as teens and adults, should see their doctor at least once a year, even if they aren’t having any symptoms.

Sickle cell anemia can cause a range of complications, but there are several things you can do to reduce your risk of developing them. Make sure to check in with your doctor at least once a year so you can get a head start on treating any issues that come up.