Sickle Cell Crises

People with sickle cell disease experience a build-up of red blood cells (due to their long, thin, sickle shape) that can block capillaries, disabling them from carrying oxygen to tissues (hypoxia) causing tissue cells to become damaged and die. This painful process is what's known as "Sickle Cell Crisis," which is also another name for sickle cell disease due to its frequency. It's Sickle Cell Crisis that makes living with Sickle Cell Disease so difficult for patients. In this blog post I describe, in-depth, a lot of the symptoms related to pain crises and how they are treated. For a quicker glimpse into sickle cell crises, you can scroll down and watched the video I embedded.

Severe pain is the most common sickle cell emergency. This pain can start out of nowhere though there are common situations that can lead to a sickle crisis, including:
• Dehydration
• Infection
• Fever
• Hypoxia
• Bleeding
• Exposure to cold or heat
• Drug or alcohol use
• Pregnancy
• Stress

Acute Sickle Cell Crisis (crises that occur infrequently) is divided into four different patterns depending on where the crisis occurs in the body:
• Bone Crisis - an acute or sudden bone pain (usually in the arm or leg) causing tenderness in the area. Sometimes patients with bone crisis have crises in the same bone in future episodes.
• Acute chest syndrome - acute chest pain comes suddenly, sometimes accompanying by coughing up blood. Often occurs simultaneously with a slight fever. A symptom is shortness of breath. Over time people can develop chronic sickle cell lung disease as the acute lung crises lead to scarred lungs and other related problems.
• Abdominal crisis: this crisis results in intense, constant and sudden pain in the abdomen area. It might not be concentrated in one specific area and possible be accompanies by to nausea, vomiting, and diarrhea.
• Joint crisis: this could develop without significant history of trauma focused in one or multiple joints concentrating mostly in the bony parts of joint. Pain also restricts the range of motion.

In addition to these particular crises, other problems can throughout the body, including:
• Central nervous system damage. Did you know 2/3 of all sickle cell stroke occur in children ~8 years old? In fact, 10% of people with sickle cell disease have strokes or other brain bleeding when under age 10. Repeat strokes occur in 2/3 of survivors within 3 years of the first stroke.
• Eyes - sickle cell disease can cause thickening if the blood and narrowing of eye blood vessels. Often Retinopathy (disease of the retina) and retinal detachment can occur as well as cause vision problems. Jaundice, a common side-effect of sickle cell also can cause the whites of eyes to yellow.
• Kidneys - kidney damage occurs in nearly every sickle cell disease patient.
• Genitals - priapism is common, affecting about 40% of men with the disease. Severe episodes can lead to impotency. Check out the new possible medication to treat priapism here.
• Weakened immune system - this leads to increased rates of infect, especially in the lungs, kidneys, bones, and central nervous system.
• Blood problems - people with sickle cell disease can develop anemia. This leads to shortness of breath, light-headedness, and chronic fatigue

When to seek medical care:
If a condition develops, it is important for people with sickle cell disease to see a doctor (preferably a specialist) right away. If this isn't possible, going to the ER is the bets bet. Contact a doctor:
• If pain is not relieved by prescribed pain medication or if the pain is different than previous episodes
• If nauseous, vomiting, diarrhea, losing a lot of fluid, the inability to drink and keep it down. Dehydration is very common among people with sickle cell disease. Patients might need to get an IV from a doctor or hospital to replace lots fluids.
• If infection seems to be starting. Even if a person is already on antibiotics to prevent infection, it's still important to see a doctor. Some of the most common infections are from flu viruses, pneumonia, and salmonella (a type of bacteria).

Often doctor or hospital visits are quick as patients are given more fluids and pain medication. Because it is so important and easy, patients really should not hesitate to seek care. Waiting to receive care will probably make a condition worse and require hospitalization for treatment. A trip to the ER or outpatient treatment center can treat many cases of sickle cell crisis.

When to visit the ER:
• uncontrollable pain (even with the use of narcotics)
• continued fluid loss and dehydration (if vomiting)
• uncontrollable fever
• chest pain or shortness of breath
• severe abdominal pain

Because prevention of crises is often difficult, the best thing for a patient is to know when to seek medical help.

What medicines are administered?
For a pain crisis, pain medications (usually narcotics) will be prescribed and IV fluids are important. With the diagnosis of an infection, antibiotics are prescribed. In cases of anemia, if there is a significant decrease in red blood cells, a blood transfusion may be needed.
For chronic therapy, regularly scheduled transfusion therapy is required to decrease symptoms of acute chest syndrome, incidences of stroke, and severe pain crises. Long-term transfusion therapy, however, has its own set of risks that should be considered as well. In addition, bone marrow transplants are promising for the same percentage of people with sickle cell disease that are approved for the treatment. Patients should discuss this serious procedure thoroughly with their doctor.

Because so many body systems are involved with sickle cell crises, and crises are so common, patients should seriously consider having a follow-up with a hematologist.

Want to learn more about sickle cell?
Check out this video from nursing-emergencies.com:

Join the new WEGO Health Sickle Cell Disease Group
Visit: Sickle Cell Foundation
Read more info on emedicine's Sickle Cell Page
Check out Wellsphere's Sickle Cell Crisis content
Health Central has a lot of content on Sickle Cell too.