The best defense for a sickle cell crisis is prevention. Here are sickle cell crisis prevention tips for families.

Published: April 5, 2021

Sickle cell crisis prevention is important for families of children with sickle cell disease. A sickle cell crisis can come on suddenly, causing great pain for hours up to several days, as the sickled red blood cells block blood flow. Here are some sickle cell crisis prevention tips to help families.

Why does sickle cell disease cause pain?

In sickle cell disease, the sickle-shaped cells in the blood can stick together easily and block off the small blood vessels in the body. When these vessels become blocked, pain appears in the affected part of the body. A sickle cell crisis can happen anywhere in the body, but most often happens in the chest, arms and legs. Babies and younger children can experience painful finger and toe swelling during a sickle cell crisis.

While these crises can come on without warning due to illness, stress, dehydration and other issues, families can do many things to help prevent a sickle cell crisis from starting.

How to prevent a sickle cell crisis

To prevent a sickle cell crisis, families can:

• Take sickle cell medications as prescribed: Preventing sickle cell crises starts with a child taking all medications as prescribed for sickle cell disease, including disease-modifying therapies such as hydroxyurea.
• Try to limit infection exposure: Children with sickle cell disease can be more at risk for complications of infections and bacteria such as the flu and salmonella. Staying up to date on vaccines, including the flu shot, and practicing good hygiene, including hand-washing, can help limit exposure to germs that cause infections.
• Pay attention to fever: If a child with sickle cell disease has a fever of over 100.4 degrees Fahrenheit, call your child’s pediatrician. Additionally, if your child doesn’t have a fever but they are not acting like themselves or you think they seem unwell, call your pediatrician.
• Eat well and stay hydrated: Eating a balanced diet and drinking plenty of water can help children with sickle cell disease stay healthy overall and prevent dehydration. Dehydration can help to bring on a sickle cell crisis.
• Get good rest: Staying well rested can help children prevent sickle cell crises. If you have any concerns about your child’s sleep, talk to your pediatrician.
• Stay active: Children with sickle cell disease benefit from physical activity with breaks incorporated. Regular physical activity is good, but do not allow children to exercise or play so much that they become really worn out. Staying hydrated during any physical activity is important.
• Dress for the weather: Sickle cell crises can be caused by exposure to extreme temperatures, both hot and cold. Try to limit time in extreme heat or cold weather. Should you need to be out during extreme weather, make sure a child is dressed appropriately. Have access to layers for being indoors during the summer due to air conditioning.
• Manage any other conditions: Should a child have any other conditions, such as diabetes, make sure they are managed well as part of their overall health care.
• Avoid high altitudes: Should you need to fly, always travel in commercial airplanes. Should you need to travel in a smaller, unpressurized aircraft, talk to your child’s hematologist about extra precautions.
• Avoid smoking and alcohol: Teens and young adults with sickle cell disease should not experiment with alcohol or smoking. These activities can cause the onset of a sickle cell crisis.
• Try to stay positive and avoid stress: Managing any chronic condition can be difficult for children and families. Stress can cause a lot of physical reactions in the body that could trigger a sickle cell crisis. If you or your child is experiencing stress, talk to your child’s care team. They may be able to provide you with tools and resources to help manage a child’s stress as well as your own.

How to manage a sickle cell crisis

It’s important for parents to know what to do once your child experiences a sickle cell crisis. Work with your child’s pediatric hematologist to have a plan in place to manage your child’s pain. Depending on your child’s age, severity of sickle cell disease and other factors, sickle cell crises may be able to be managed at home with over-the-counter pain relievers, heating pads, warm baths and massage. Additionally, your child should have a plan in place to manage more severe flare-ups and when the crisis is an emergency.