Living with Sickle Cell
Living with sickle cell disease (SCD) presents unique challenges, both in the short term and over a lifetime. As a chronic illness that primarily affects the shape and function of red blood cells, SCD leads to a variety of complications, from pain crises to organ damage, all of which can have a significant impact on a person’s daily life.
In this article, as part of our September series for Sickle Cell Awareness Month, I will explore the realities of living long-term with SCD. We’ll discuss the daily struggles faced by individuals, the long-term physical effects, and provide valuable insights into managing pain and maintaining mental health. Additionally, we’ll offer tips for leading a healthy lifestyle with SCD, while addressing and debunking the stigmas that still surround the disease.
How does Sickle Cell Affect the Body long term
Sickle cell disease (SCD) affects the body long-term in multiple ways due to the recurring blockage of blood flow caused by abnormally shaped red blood cells. These sickle-shaped cells are stiff and can stick to vessel walls, restricting oxygen delivery to organs and tissues. Over time, this leads to cumulative damage across several systems and patients can deal with many chronic issues:
- Chronic Pain: Recurrent Vaso-occlusive crises, where sickle-shaped cells block blood vessels, lead to severe episodes of pain. Over time, patients can develop chronic pain, often requiring ongoing pain management.
- Organ Damage: The repeated lack of oxygen can damage major organs over time, including kidneys, lungs, heart, liver, and spleen
- Stroke and Neurological Complications: SCD increases the risk of stroke, particularly in children. Repeated blockages in blood flow to the brain can cause cognitive impairments, learning difficulties, or more severe brain damage.
- Bone Damage: Avascular necrosis (bone death) can occur in joints like the hips and shoulders, leading to chronic pain, decreased mobility, and joint replacement surgeries.
- Growth and Development: Children with SCD often experience delayed growth and puberty due to the chronic strain on their bodies and nutritional deficiencies from anemia.
- Vision Problems: Sickle cells can block blood vessels in the eyes, leading to conditions like retinopathy or, in severe cases, blindness.
- Increased Risk of Infections: Due to spleen damage, SCD patients have a weakened immune system, making them more vulnerable to bacterial infections such as pneumonia, meningitis, and sepsis.
- Fatigue and Anemia: Sickle cells have a much shorter lifespan than normal red blood cells, leading to chronic hemolytic anemia. This results in persistent fatigue, weakness, and reduced tolerance for physical activity.
Managing pain and fatigue
Managing pain and fatigue in sickle cell disease (SCD) requires a comprehensive approach that combines pharmacological treatments with holistic strategies to address both acute vaso-occlusive crises and chronic pain.
In an earlier part of the series (Link to part 2 and 3), we covered pharmaceutical approaches to pain management for sickle cell disease (SCD), including pain medications, NSAIDs, hydroxyurea, L-Glutamine, and blood transfusions. This article will shift our focus to home and holistic care strategies.
Holistic (Non-Drug) Pain and Fatigue Management
- Hydration: Staying well-hydrated helps reduce the sickling of red blood cells, potentially lowering the risk of vaso-occlusive crises. SCD patients should aim for high fluid intake, especially during crises.
- Heat Therapy: Warm compresses or baths can help relax muscles and reduce pain in affected areas. Heat therapy is particularly useful for joint, or muscle pain caused by sickling cells.
- Physical Therapy and Exercise: Gentle stretching, yoga, and low-impact exercises can help improve circulation, reduce stiffness, and promote joint mobility. Physical activity should be carefully monitored to avoid overexertion.
Mind-Body Techniques for pain and fatigue management
- Cognitive Behavioral Therapy (CBT) helps patients manage the psychological aspects of chronic pain by changing negative thought patterns and teaching coping skills.
- Relaxation Techniques such as deep breathing, meditation, and guided imagery help reduce stress and muscle tension, which can exacerbate pain.
- Biofeedback can teach patients how to control certain physiological functions, like heart rate and muscle tension, to reduce pain perception.
- Acupuncture This traditional Chinese medicine technique has been used to manage chronic pain by stimulating specific points in the body. While evidence is limited, some SCD patients report relief from acupuncture.
- Massage Therapy can help alleviate muscle pain, reduce stress, and improve overall relaxation. It may also enhance blood circulation.
Prevention and Long-Term Strategies for pain and fatigue
- Avoiding Triggers: Identifying and avoiding triggers for pain crises—such as extreme temperatures, dehydration, or overexertion—can help minimize painful episodes.
- Take medications and prescribed: Follow your healthcare provider’s recommendations and take medications consistently. Attend regular check-ups to monitor your condition and adjust treatments as necessary.
- Regular Monitoring: Routine check-ups with healthcare providers and managing underlying health issues can help prevent complications that lead to pain.
Combination Approach
For optimal pain management in SCD, it’s essential to combine both pharmacological and holistic treatments. Medications can help control acute and severe pain, while lifestyle changes and non-drug strategies offer long-term benefits and improve the patient’s overall quality of life. Each patient may respond differently, so treatment plans should be individualized based on the severity of the disease and the patient’s preferences.
8 Tips for living a healthy lifestyle with sickle cell
Living a healthy lifestyle with sickle cell disease (SCD) involves managing the condition while promoting overall well-being. Here are some tips to help SCD patients maintain a healthier lifestyle:
- Stay Hydrated-Drink plenty of water throughout the day, aiming for at least 8–10 cups. Increase fluid intake during physical activities, hot weather, or illness.
- Eat a Balanced Diet-Focus on a diet rich in fruits, vegetables, whole grains, and lean proteins. Consider foods high in:
- Folic acid (leafy greens, beans) to help produce healthy red blood cells.
- Omega-3 fatty acids (salmon, flaxseeds) to reduce inflammation.
- Iron-rich foods (spinach, lentils) if recommended by your healthcare provider, but only take iron supplements if advised, as excess iron can be harmful.
- Exercise Regularly (with Caution)– Engage in low-impact activities like walking, swimming, yoga, or cycling. Avoid strenuous or high-intensity exercises that can trigger a crisis. Always warm up before and cool down after workouts and stay hydrated during physical activity.
- Get Enough Rest– Prioritize rest and sleep by maintaining a regular sleep schedule and ensuring quality rest each night. Adequate sleep (7–9 hours) helps the body recover and can reduce the frequency of pain episodes.
- Avoid Extreme Temperatures– Dress appropriately for the weather, avoiding prolonged exposure to hot or cold environments. Layer clothing in cold weather to maintain warmth and stay cool and hydrated in hot weather.
- Manage Stress-Practice relaxation techniques such as deep breathing, meditation, or mindfulness. Engage in hobbies or activities that help reduce stress and consider seeking support through counseling or therapy.
- Prevent Infections– Stay up to date with vaccinations, including flu, pneumonia, and meningococcal vaccines. Practice good hygiene, such as regular handwashing. Seek medical attention promptly for any signs of infection, such as fever, cough, or shortness of breath.
- Stay Informed and Advocate for Yourself– Learn about sickle cell disease, its potential complications, and the latest treatments. Advocate for yourself during medical visits and communicate your needs and concerns clearly to healthcare providers.
By integrating these lifestyle practices into daily routines, individuals with sickle cell disease can better manage symptoms, reduce the frequency of pain crises, and enhance their overall quality of life.
Mental health and Psychosocial Support
Mental health is crucial in managing sickle cell disease (SCD) due to the emotional and psychological challenges of this chronic condition. Coping with frequent pain crises, hospital visits, and the disease’s limitations can lead to emotional exhaustion, anxiety, and depression. Effective mental health support helps patients develop coping strategies, manage pain, and maintain a positive outlook, which is essential for enhancing overall well-being and quality of life.
Additionally, mental health is key for adhering to treatment regimens and fostering resilience against the disease’s uncertainties. Depression or anxiety can hinder motivation and self-care, impacting treatment adherence and healthy lifestyle choices. Integrating mental health care, along with family and psychosocial support, into SCD management helps address both emotional and physical needs, leading to more comprehensive and effective disease management.
Coping with the Stigma of SCD
The stigma surrounding sickle cell disease (SCD) often arises from misunderstandings and misconceptions. One major issue is the perception that SCD patients misuse pain medications; their frequent need for pain management can lead to accusations of drug-seeking behavior, resulting in delayed or inadequate care. Additionally, racial biases can affect patients, particularly those of African, Mediterranean, Middle Eastern, and Indian descent, leading to discrimination in healthcare settings and society. Chronic illness stigma can also cause patients to be viewed as less capable or overly dependent, leading to isolation and misunderstanding. Moreover, the mental health impact of chronic pain and frequent hospitalizations is frequently overlooked, reducing empathy and support. Addressing these stigmas requires increased awareness, education about SCD, and efforts to combat biases in healthcare and society.
Outlook: Thriving with Sickle Cell Through Balanced Care
While living with sickle cell disease (SCD) presents challenges, it is manageable with the right approach. Maintaining both physical and mental health is key to coping with the disease, as managing symptoms extends beyond just addressing physical pain. Integrating pharmaceutical treatments, such as pain medications and hydroxyurea, with non-pharmacological methods like stress management, holistic care, and lifestyle adjustments, can help control long-term symptoms. By focusing on a balanced approach to care, individuals with SCD can improve their quality of life and better manage the complexities of the disease.
About the author
Dr. Daniel Landau is a distinguished board-certified hematologist/oncologist renowned for his exceptional contributions in the field. With an illustrious career spanning across esteemed institutions like the Orlando Health Cancer Institute and the Medical University of South Carolina, Dr. Landau’s expertise shines in both genitourinary oncology and hematology. .
Engage with Dr. Landau’s expertise and ignite your curiosity on “Ask MedOnCMD” as he invites you to explore the vast landscape of oncology with a fresh outlook.