Pain Episodes and Sickle Cell Disease

What is a pain episode?

A pain episode in sickle cell disease occurs when the body’s tissues do not get enough oxygen. This is also called a pain crisis or acute vaso-occlusive pain.   

Acute pain episodes are the most common complication of sickle cell disease. Pain episodes are different from other types of pain. They occur suddenly and can last for several hours, a few days, or even weeks.

Symptoms of a pain episode

In a pain episode, the pain may feel throbbing, stabbing, dull, or sharp and range from mild to very severe.

Pain can occur in different parts of the body. Common places include the:

• Back
• Chest
• Abdomen (belly)
• Arms and hands
• Legs and feet

Pain can also move from one part of the body to another. 

In addition to acute pain episodes or pain crises, your child may have pain that has nothing to do with sickle cell disease. Your child can get headaches, sprains, and other aches and pains.  

People with sickle cell disease can also have chronic pain. Chronic pain is pain that occurs on most days and lasts for 6 months or longer. It normally becomes more severe as people get older. More than half of adults with sickle cell disease have chronic pain.   

Causes of a pain episode

Acute pain in sickle cell disease can happen when sickle-shaped red blood cells stick together and block small blood vessels.  

Normal red blood cells are round and flexible. They flow easily through the body’s blood vessels. People with sickle cell disease have red blood cells that are hard, sticky, and shaped like a banana (sickle-shaped). Sickle cells clump together and clog blood vessels. This slows the delivery of blood and oxygen to the body and can cause pain.

Possible triggers of acute sickle cell pain

Pain episodes can be hard to predict and often happen without a clear reason. Common known triggers are:  

• Dehydration
• Low oxygen levels
• Infection
• Intense physical activity  
• High altitude
• Cold temperatures
• Smoking or vaping
• Alcohol use
• Stress
• Existing pain or injury

Pain with sickle cell disease can happen at any time. Pain episodes can keep your child from school and daily activities. It is not always possible to prevent pain. But try to limit or avoid any activities or situations that trigger your child’s pain.  

Diagnosis of a pain episode

Early treatment of a pain episode is critical. You may need to seek care at your clinic or hospital if you are not able to manage your child’s pain at home. 

A health care provider will do a physical exam including a pain assessment. Your child may also have lab tests or imaging tests to learn more about the cause of pain or other symptoms.  

Be sure to let your care team know if you have given your child any pain medicines. They will need to know the type of medicine, dose, and time you gave them the medicine.

When assessing your child’s pain, your care team will want to know information about their current pain as well as their pain history. Your child will be asked to rate the pain on a pain scale if they are able. 

You may be asked questions such as:

• When did the pain start?
• What does the pain feel like?
• How severe is the pain?
• Where is the pain located?
• Is the pain constant or does it come and go?
• Does anything make the pain better or worse?
• Does your child have other symptoms in addition to pain?

Your care team might ask about your child’s pain history including:  

• Has your child had this type of pain before?
• How long does the pain usually last?
• How was the pain treated in the past? 
• How well does your child respond to pain medicines or other treatments?

Treatment of a pain episode

If your child has a pain episode, it is important to treat the pain as soon as you can. If your child’s pain is severe, seek medical care from your clinic or hospital. 

Pain medicines are the main treatment for acute sickle cell pain episodes. For severe pain, your child may need opioid medicine, such as morphine, hydromorphone, or fentanyl. 

Your child may need to be admitted to the hospital for pain control. 

Safety reminders for pain medicines

Opioid medications can be highly addictive. Your care team will take steps to help prevent addiction. This may include prescribing opioids for a brief period to limit how much and how often your child takes the medicine. Your care team will also watch for tolerance. Opioid tolerance is a condition where the body gets used to a medicine so that the medicine does not work as well. These factors are considered as part of your child’s pain treatment plan. 

You can take steps to help make sure your child takes their prescribed medicines safely:   

• Your child should only take opioid medicines when they are truly hurting. 
• Do not give pain medicine more often or in greater amounts than prescribed.  
• Only give the medicine that was prescribed for your child and give as directed. 
• Do not crush, cut, or allow your child to chew the medicine unless your doctor or pharmacist says it is OK. 
• Do not give any other prescription medicine, over-the-counter medicine, supplements, or herbal remedies without talking to the care team.  
• Do not give pain medicine at home for more than 24 hours without talking to your doctor.  
• Pain medication can cause constipation. Be sure your child drinks plenty of fluids and eats foods that are high in fiber. Give medicines for constipation as prescribed by your care team.
• Keep all medicines out of the reach of children.    

How to manage pain episodes at home

Pain treatments work differently for different people. Work with your care team to develop a pain management plan for your child. If the pain cannot be controlled or is severe, call your child’s care team.  

To help your child manage mild to moderate pain at home: 

• Give pain medicine as directed. Mild pain can be treated with ibuprofen. For moderate pain, give prescription medicines as recommended by your care team.  
• Be sure your child rests and drinks plenty of fluids. 
• Use heat therapy to increase blood flow. Apply a warm blanket or towel to the painful area. Talk to your care team before using heating pads. Do not use ice or cold therapy, as this can make pain worse.  
• Use non-medicine strategies along with pain medicines.  

Be sure that you let others who care for your child know about your child’s condition, their pain management plan, and what to do in an emergency.

Non-medicine strategies to manage pain

Here are some non-medicine strategies you can try to help manage your child’s pain. These techniques are not a substitute for pain medicine. But they can help pain medicines work better and give additional options to cope with pain.  

Find more information on managing pain without medicines.

When to call your care team

If your child has pain, don’t wait to start treatment. Follow your care team’s recommendations for treating your child’s pain. 

Go to the hospital or emergency room if your child has sudden, severe pain that does not get better with medicine. 

Seek medical care if your child has any of these signs or symptoms:

• Pain that gets worse or won’t go away
• Shortness of breath or trouble breathing
• Fever of 101°F (38.3°C) or higher
• Acts very tired or is hard to wake up
• Sudden weakness, loss of feeling, or difficulty moving a body part

Questions to ask your care team

• What are the early signs of a pain crisis? 
• Is there a pain crisis action plan we should follow?
• Are there ways to help prevent my child’s pain?
• How can I manage my child’s pain at home?
• Are there any changes needed to my child’s diet, physical activity, or fluid intake?
• What do I need to tell my child’s school, babysitter, or other caregivers about how to recognize and manage a pain episode?
• What emergency warning signs should we watch for?
• When should we go to the emergency room for care?

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