What is a Sickle Cell Crisis?

What Is a Sickle Cell Crisis?

At some point in your life, you’ll accidentally drop something into the sink. If it’s small and of little value, like a grape, it may not be an issue. You let the water run and flush it through. But if your kid slips a big piece of Lego down the drain, you’re going to have some bigger problems when it gets stuck in your pipes.

That’s kind of like what happens during a sickle cell crisis. Red blood cells are usually round and have some give to them -- their shape lets them move easily throughout your body. But when you have sickle cell disease (SCD), some cells are curved -- like a sickle--and hard. Because they aren't as flexible--or deformable- they can get stuck in small blood vessels. This can happen in lots of organs like your chest, belly and joints. That's when you have.... More than 50% of people with SSD, experience acute chest syndrome because of the small vessels in the lungs. This can lead to acute hypoxia (low oxygen levels) as well as long term complications in the lungs.That’s when you have a sickle cell crisis.

The stuck cells slow or even totally block blood flow, so some parts of your body don’t get the oxygen they need. That can cause intense pain that lasts anywhere from a few hours to a few weeks. But you can take steps to lower your chances of a crisis. And even when one comes on, you may be able to care for yourself at home.

Symptoms

The most common sign is pain that might be dull, stabbing, throbbing, or sharp, and seems to come out of nowhere. How severe it is and how long it lasts varies with different people and different crises. Some people have crises here and there, while others may have them every month.

You might feel the pain anywhere in your body and in more than one place, but it’s often in your:

• Arms and legs
• Belly
• Chest
• Hands and feet (more typical in young children)
• Lower back

You may also have:

• Breathing problems (shortness of breath or pain when breathing or both)
• Extreme tiredness
• Headache or dizziness
• Painful erections in males
• Weakness or a hard time moving some parts of your body
• Yellowish skin color (jaundice)

Causes

Typically, you won’t know why you had a crisis, and there may be more than one cause. Possible triggers include:

• Being at high altitudes (mountain climbing, for example)
• Changes in temperature, like if you go from a warm house into a cold winter day and you haven’t bundled up
• Infections
• Not having enough to drink (dehydration)
• Stress

Treatment

Thetr are a few drugs that can help. The drug called L-glutamine oral powder (Endari) has proven to help prevent these crises from occurring and thus preventing hospitalizations. Hydroxyurea (Droxia, Hydrea, Silkos) and voxelotor (Oxbryta) prevent abnormal red blood cells from forming. This reduces the number of painful crises from sickling blood cells. Crizanlizumab-tmca (Adakveo) helps stop the blood cells from sticking together and blocking small blood cells, which can not just be painful, but can damage organs.

Your doctor can help you come up with a plan for how to handle a crisis. If you need to go to the hospital for treatment, make sure to take your plan with you.

Often, you can treat the pain at home. When a crisis first starts, your doctor will likely suggest you drink plenty of liquids and take an over-the-counter pain medicine, such as ibuprofen or acetaminophen. Talk to your doctor to see what’s safe for you. For example, if you have a kidney problem, acetaminophen might be the better choice. For more severe pain, your doctor may give you a stronger medicine.

You can also try a heating pad, hot bath, or a massage. Physical therapy may provide some relief, too. And don’t forget to tend to your mind. Counseling, relaxation methods like meditation, and seeking support from family and friends are key steps in keeping yourself well.

If you can’t manage the pain at home, go to an emergency room, where they can give you stronger pain medicine. You may need to stay in the hospital until the pain is under control.

How Can I Prevent a Crisis?

There’s no sure way, but you can lower your odds:

• Avoid swimming in cold water.
• Dress in warm clothes when it’s cold out or when you’re in air-conditioned buildings.
• Drink plenty of water.
• Fly only on commercial airlines. Planes that don’t control air pressure could cause you problems.
• Limit how much alcohol you drink.
• Manage your stress.

It also helps to keep yourself as healthy as possible:

• Avoid being around people who are sick.
• Don’t smoke.
• Exercise, but drink plenty of liquids and don’t push too hard. Activities like intense weight training may put too much stress on your body.
• Get prenatal care right away if you’re pregnant or you’re planning on it.
• Manage any other health conditions you may have, like diabetes, with your doctor’s help.
• Stay up to date on your shots and vaccines.
• Tell your doctor if you have any sleep problems, like snoring.
• Wash your hands often.

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