Sickle cell disease (SCD) is an inherited red blood cell (RBC) disorder. It’s the result of a genetic mutation that causes misshapen RBCs.
SCD gets its name from the crescent shape of the RBCs, which resembles a farm tool called a sickle. Usually, RBCs are shaped like discs.
RBCs transport oxygen to your body’s organs and tissues. SCD 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.
Pain from a sickle cell crisis tends to be felt in the:
A sickle cell crisis can begin suddenly and last for days. Pain from a more severe crisis may persist for weeks to months.
Without proper treatment, a sickle cell crisis can lead to potentially serious complications, including organ damage and vision loss.
Experts don’t fully understand the reasons behind a sickle cell crisis. But they do know that it involves complex interactions between RBCs, endothelium (cells lining the blood vessels), white blood cells, and platelets. These crises usually occur spontaneously.
The pain occurs when sickled cells get stuck in a blood vessel, blocking the flow of blood. This is sometimes referred to as sickling.
Sickling may be triggered by conditions associated with low oxygen levels, increased blood acidity, or low blood volume.
Common sickle cell crisis triggers include:
- sudden change in temperature, which can make the blood vessels narrow
- very strenuous or excessive exercise, due to shortage of oxygen
- dehydration, due to low blood volume
- high altitudes, due to low oxygen concentrations in the air
- other medical conditions, such as diabetes
It’s not always possible to know exactly what caused a particular sickle cell crisis. Many times, there’s more than one cause.
Not all sickle cell crises require a trip to the doctor. But if home treatments don’t seem to be working, it’s important to follow up with a doctor to avoid any other complications.
Some sickle cell crises are manageable with over-the-counter pain relievers, such as:
- acetaminophen (Tylenol)
- ibuprofen (Advil, Motrin)
- naproxen sodium (Aleve)
Other ways to manage mild pain at home include:
- heating pads
- drinking plenty of water
- warm baths
If you have severe pain or home treatments aren’t working, see a doctor as soon as possible. They’ll likely start by checking for any signs of an underlying infection or dehydration that might be triggering the crisis.
Next, they’ll ask you some questions to get a better idea of your pain level. Depending on your pain level, they’ll likely prescribe some medication for relief.
Options for mild to moderate pain include:
- nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen
- codeine, alone or in combination with acetaminophen (Tylenol)
- oxycodone (Oxaydo, Roxicodone, OxyContin)
Options for more severe pain include:
- morphine (Duramorph)
- hydromorphone (Dilaudid, Exalgo)
- meperidine (Demerol)
There are several medications that you can take if you experience frequent acute pain episodes with SCD. Hydroxyurea, glutamine, and crizanlizumab are FDA approved to reduce the rate of acute pain episodes when administered in acute care settings (such as the ER or infusion centers). If you experience frequent acute pain episodes from your SCD, talk with your doctor about these options.
A sickle cell crisis should be treated right away to avoid long-term issues. It’s important to make sure you know who to call and where to go for medical treatment because a sickle cell crisis can come on suddenly.
Before you have a pain crisis, talk to your regular doctor to make sure that information in your electronic medical record (EMR) is updated. Keep a printed copy of your pain management plan and a list of all your medications to take with you to the hospital.
You should seek medical attention right away if you have SCD and any of the following symptoms:
- unexplained, severe pain in your back, knees, legs, arms, chest, or stomach
- fever above 101°F (38°C)
- unexplained severe pain
- 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
When you visit an emergency department, make sure to do the following:
- Inform the staff right away that you have SCD.
- Provide your medical history and a list of all the medications you’re taking.
- Ask the nurse or doctor to look up your EMR.
- Give the staff your regular doctor’s contact information.
You can’t always prevent a sickle cell crisis, but certain lifestyle changes can help to reduce your risk.
Here are some ways to help lower your risk of having a sickle cell crisis:
- Take all medications recommended by your doctor.
- Try to drink about 10 glasses of water a day, adding more in hot weather or during exercise.
- Stick to light or moderate exercise, avoiding anything strenuous or extreme.
- Dress warmly in cold weather, and carry an extra layer just in case.
- Limit time spent at high altitudes.
- Avoid mountain climbing or flying in an unpressurized cabin (noncommercial flights) above 10,000 feet.
- Wash your hands often to avoid infection.
- Get all recommended vaccinations, including a flu vaccination.
- Take a folic acid supplement, which your bone marrow needs to make new RBCs.
- Pay attention to and manage stress.
- Avoid smoking.
A sickle cell crisis can be very painful. While mild pain can be treated at home, more severe pain is a sign you should see a doctor. If not treated, a severe sickle cell crisis can deprive organs, such as the kidneys, liver, lung, and spleen, of blood and oxygen.