An important part of managing sickle cell disease is recognizing the triggers that can bring on a pain crisis. Some common triggers include:

• Alcohol
• Being too hot or too cold
• Not getting enough sleep
• Not drinking enough water
• Poor nutrition, such as eating processed or sugary foods
• Stress

Our team provides education to help you pinpoint your triggers and identify steps you can take to avoid them. Making changes in your lifestyle isn't always easy, so we support you and connect you to resources that can help. 

Routine visits help us ensure your treatments are working and detect any changes in your health. Depending on your situation, we may see you every one to four months. 

A blood transfusion gives you a boost of healthy red blood cells that can help relieve symptoms and prevent complications. Your doctor may recommend blood transfusions occasionally or routinely to:

• Ease chronic pain
• Prevent a severe complication from happening again, such as a stroke, acute chest syndrome or multiorgan failure
• Treat severe anemia

We provide transfusions in our infusion center using blood provided by Memorial's blood bank. Our blood bank is rigorous about testing donor blood to ensure it’s safe for people with sickle cell disease. 

For severe symptoms, we may use a specialized type of transfusion called red blood cell exchange (apheresis). This treatment removes some of your sickled red blood cells before replacing them with healthy red blood cells. 

Certain medications can decrease the frequency of pain crises and may help you live longer. These drugs are called disease-modifying because they target specific processes inside your cells that cause symptoms. They can help:

• Decrease sickling
• Prevent pain crises
• Protect organs from long-term damage
• Reduce hospital visits
• Improve daily energy and quality of life

The main disease-modifying medications available for sickle cell disease include:

• Hydroxyurea: A pill you take daily
• Crizanlizumab: A monthly infusion you receive at our infusion center
• L-glutamine: A powder you mix with a soft food or beverage 

Taking the right medications can make a difference in your life now and for years to come. Our on-site pharmacist provides medication monitoring to make sure your medications are working properly and that you're taking the correct dose. We also offer convenient options for medication pick-up and delivery if you can't get to the pharmacy.

Researchers across the U.S. are investigating new disease-modifying medications. When possible, we offer access to these medications through clinical trials. If you're eligible for a clinical trial, our team will let you know and help you understand the benefits and risks. Participation in a clinical trial is always voluntary. 

As part of your care, you receive a personalized pain plan outlining the pain management strategies that work best for you. That plan may include powerful opioids, such as morphine, oxycodone, or fentanyl.

Over time, opioids can lose their effectiveness. If that happens, or you're looking for an alternative, a combination of treatments may be effective. Other pain management strategies include: 

• Acetaminophen and nonsteroidal anti-inflammatory drugs, such as ibuprofen 
• Disease-modifying medications
• Medications for nerve pain (gabapentin, duloxetine)
• Safer opioid options (buprenorphine) 
• Non-medication options, such as heat, massage, hydration, relaxation breathing, physical therapy and counseling

At our infusion center, we offer patient-controlled analgesia (PCA) to deliver opioid medications. You may need PCA to relieve severe pain during a sickle cell crisis when oral medications aren't effective. 

1. We set up an IV attached to a pump. 
2. You control the amount of medication you receive by pressing a button. 
3. A lockout period prevents overdosing, and a nurse monitors you for safety. 

One of the main benefits of PCA is faster and steadier pain relief. It also gives you more control over your comfort. 

This type of pain starts suddenly, lasts hours to days and often affects the chest, back, arms, legs, or joints. 

The goal for acute pain crises is to treat pain quickly and prevent complications. Strategies to try include:

• Take prescribed medications right away
• Avoid triggers that could make pain worse
• Drink water or electrolyte fluids
• Use warm compresses

If pain doesn't improve or you're having chest pain or trouble breathing, call our office or go to the emergency department. All Memorial emergency departments have access to your medical chart and personalized pain plan. They also have protocols in place to ensure people with sickle cell disease receive rapid care. 

This type of pain lasts three months or more and often feels like a deep ache, burning, tingling, or stiffness. If you have chronic pain, it's important to take your long-term medications consistently. 

Other strategies that can help include:

• Blood transfusions and red blood cell exchange
• Disease-modifying medications
• Physical therapy to improve movement and reduce stiffness
• Relaxation techniques, counseling and support groups