Navigating life with sickle cell anemia

Living with sickle cell anemia means being proactive when it comes to self-care and pain management.

Sickle cell disease affects approximately 100,000 Americans, with the condition being most prevalent among Black people and some Hispanics. Sickle cell disease, also referred to as sickle cell anemia, refers to a group of inherited blood disorders characterized by sickle-shaped red blood cells that can block blood flow to vital organs and tissues, causing serious complications.

Those living with sickle cell anemia will benefit from education on how to manage their condition effectively, not just from a medical standpoint, but a psychological one, which is the case with many lifelong medical conditions.

What is sickle cell disease?

Sickle cell disease (SCD) refers to a group of inherited blood disorders that affect how red blood cells work. Normally, these cells are soft and round, but in SCD, they become stiff, sticky and shaped like a crescent or sickle. This unusual shape makes it harder for the cells to move through small blood vessels. That can block blood flow and cause significant pain, anemia and even organ damage.

SCD is caused by a mutation in the gene responsible for making hemoglobin, the protein that carries oxygen through the body. Because it's genetic, the condition is present from birth. While there's no cure yet, treatments can help reduce symptoms and prevent complications, improving quality of life.

What are the symptoms of sickle cell disease?

The symptoms of sickle cell disease often begin to appear when a child is five to six months old. The signs vary from person to person and may change over time. However, common early symptoms of sickle cell disease include:

• Anemia
• Extreme fatigue
• Jaundice (yellowing of the skin or eyes)
• Painful swelling of the hands and feet

A sickle cell crisis, or pain crisis, is one of the hallmarks of SCD. It happens when the sickled cells get stuck in a blood vessel and stop or slow blood flow. Vaso-occlusive crisis (VOC) is the most common sickle cell crisis and occurs when the sickled cells block the capillaries or other small blood vessels, reducing blood flow to the tissues and organs. This can cause severe and sudden pain, often in the hands, feet, chest, back and joints. Some people have multiple pain crises in a year, while others may only have a few.

Long-term complications from sickle cell disease can lead to an increased risk of infections, organ damage and vision problems.

How is sickle cell disease treated?

There is no cure for sickle cell disease, but medication can help manage symptoms and reduce the sickling of red blood cells. Blood transfusions can also help treat and prevent complications from sickle cell disease, such as stroke. Transfusions increase the amount of red blood cells that are not affected by SCD to help reduce symptoms. Sometimes, only a single transfusion is needed, but some people may need up to 100 units of blood each year.

Hydroxyurea is an oral medication that reduces the frequency of pain crises and can reduce the need for blood transfusions and hospitalizations. Medications such as L-glutamine and crizanlizumab can also reduce the frequency of pain crises. Over-the-counter pain medication such as acetaminophen can help treat mild to moderate pain, while prescription medication may be necessary for more serious pain.

Doctors may also prescribe daily penicillin, an antibiotic, to help lower the risk of infection in children younger than five. However, adults with sickle cell anemia may take penicillin throughout their lives if they have had pneumonia or if they have had their spleen removed.

Management strategies for sickle cell disease

Living with sickle cell anemia means being proactive when it comes to self-care and pain management. Besides medication, several management strategies can help address symptoms and reduce pain:

• Try heat therapy. Warm baths or compresses can help relax muscles and reduce pain. Heat therapy is especially helpful for joint and muscle pain.
• Stay well hydrated. This is critical for preventing sickled blood cells, which can reduce the risk of pain crises. Hydration not only helps improve blood flow, but it also can help manage fatigue and boost energy levels. Aim for eight to 10 glasses of water a day, and drink more during physical activity, hot weather or during a pain crisis. Avoid alcohol and caffeine, which can cause dehydration.
• Schedule regular check-ups. Having blood work done regularly, as well as staying up to date with vaccinations, is essential for those living with sickle cell anemia. SCD can lead to a weakened immune system and increase the risk of infection.
• Find an outlet. Find ways to reduce stress and improve overall well-being, like physical activities and personal practices, such as meditation. Talk therapy and building a social network or support group are great ways to alleviate the stress of living with sickle cell anemia.

Tips for caregivers

Recognizing early signs of pain crises and knowing when to seek medical attention is essential to ensure your loved one gets the care they need. An emergency action plan containing all pertinent information can be helpful for sharing with healthcare professionals or family members.

Your loved one should know their rights as a patient, but you may need to stand up for them in the event they are unable to speak for themselves. Educating yourself about their condition can help you be a better advocate. It can be helpful to have your own support system as well. Counseling, therapy or a support group can help you navigate the challenges and emotions involved with caring for someone with SCD. Your local hospital may have support services for families affected by SCD, and organizations such as the Sickle Cell Disease Association of America provide helpful resources.

Living with sickle cell anemia presents unique and ongoing challenges for patients and their families, but with the right tools, support and care, it is possible to lead a full and meaningful life.