Living Well with Sickle Cell

Sickle cell disease (SCD) is an inherited condition that, if not properly managed, can lead to a serious health crisis. However, with consistent care and support, individuals living with SCD can effectively manage their condition and lead long, healthy lives. Learn more with Cone Health Chief Health Equity & Community Impact Officer Dr. Olu Jegede. Dr. Jegede works with sickle cell patients at Cone Health Patient Care Center and is a member of the North Carolina Council on Sickle Cell Syndrome.

Understanding Sickle Cell Disease

Sickle Cell Disease (SCD) is a genetic blood disorder that people are born with. The condition is most often found in African-Americans and those of descent from:

• Central America and South America.
• Middle Eastern countries, especially Saudi Arabia and the Arabian Gulf.
• South Asia, primarily India and Pakistan.
• The Mediterranean, such as from Turkey, Greece or Italy.

Unlike typical round red blood cells, those with SCD have stiff, crescent-shaped red blood cells. These abnormally shaped cells can get stuck together in blood vessels and block blood flow to organs, leading to a range of symptoms, including pain, infection, fatigue, and even life-threatening complications.

The good news is that with consistent care and support, individuals can live longer, healthier lives. Staying connected to a care team ensures that patients receive the necessary resources to maintain their best quality of life.

Diagnosis and Warning Signs

In the United States, SCD is typically diagnosed at birth through newborn screening. Early diagnosis is crucial for preventing complications through prompt care. For those not screened at birth, such as immigrants, warning signs can include:

• Severe fatigue
• Frequent pain
• Delayed growth
• Repeated infections

A simple blood test can diagnose SCD if these symptoms are present.

Managing Symptoms and Avoiding Complications

At Cone Health Patient Care Center, each patient with sickle cell disease receives an individualized care plan. These plans often include:

• Disease-modifying medications: Such as Hydroxyurea.
• Access to clinics: Including a day infusion center for managing pain without other complications.
• Vaccination updates and routine labs.

Patients are also educated on how to avoid triggers like dehydration, extreme temperatures, and stress. Addressing social barriers that might interfere with care is also a key part of the management strategy.

When to Contact Your Provider

It's vital for individuals with SCD to recognize "red flags" that warrant immediate medical attention. Dr. Jegede highlights these critical symptoms:

• Fever over 101°F
• Shortness of breath or chest pain
• Worsening fatigue or pain that doesn't subside

In such cases, patients should call their provider or go directly to the emergency department. Cone Health's infusion center and trained staff aim to reduce delays in care and prioritize saving lives.

The Role of Hydration, Rest, and Nutrition

Hydration, rest, and good nutrition are foundational to managing SCD. Drinking plenty of water helps maintain blood flow and prevent blockages. Sufficient rest and a nutritious diet support the body's recovery and help prevent complications. The care team at Cone Health Patient Care Center actively helps patients stay hydrated, emphasizing it as a continuous practice.

The Importance of Regular Check-ins

Even when feeling well, regular provider visits are crucial for individuals with SCD. Dr. Jegede explains that SCD can cause silent damage to organs, such as the kidneys, or increase the risk of stroke and lung issues, often before symptoms appear. Regular check-ins allow providers to:

• Monitor kidney function.
• Screen for stroke risk.
• Address lung health concerns.
• Adjust medications.
• Assess emotional well-being.

These frequent visits (some patients are seen monthly) help build trust and maintain a strong patient-provider relationship, ensuring proactive management of the condition.

Transitioning from Pediatric to Adult Care

The transition from pediatric to adult SCD care can be a vulnerable time for patients, who move from a highly supported environment to greater independence.

To facilitate a smooth transition, Cone Health Patient Care Center partners with pediatric teams for a "warm handoff" starting around age 16. During this period (ages 16-18), patients are invited to the adult clinic to meet providers, ask questions, and become familiar with the services, including the day hospital. This gradual approach builds confidence and connection for the young adults.

Prioritizing Mental Health

The emotional burden of living with SCD is significant. Chronic pain, fear of judgment, and disruptions to school or work can lead to anxiety, depression, and isolation. Cone Health supports patients by providing:

• Counseling through medical social workers.
• Referrals to psychiatrists for those needing more intensive support.

Family and community support, empathy, and open communication are vital. Patients are encouraged to reach out if they are struggling with their mental health, reinforcing that they are not alone.

Supporting Loved Ones with SCD

For family and friends of individuals with SCD, Dr. Jegede offers key advice:

• Be informed: Understand the nature of sickle cell disease.
• Believe their pain: Recognize that their pain is real, even if it's not always visible.
• Show empathy.
• Encourage engagement: Support them in staying connected with their care, asking questions, and attending appointments.

Next Steps

Partner with Cone Health Patient Care Center’s specialized primary care providers to better manage your health and protect your well-being. See why more than 400 adults in the Triad trust us for care at conehealth.com/sicklecell.