Barbara Lash

As discourse on Sickle Cell Disease (SCD) evolves, attention increasingly turns to the critical role of lifestyle choices, particularly physical inactivity, in influencing the disease’s trajectory and the overall quality of life for those affected. The benefits of moderate physical exercise are well-documented; however, individuals with SCD face unique challenges that often prevent them from engaging in regular physical activities. A significant barrier is the increased risk of triggering crises, which account for more than 230,000 SCD-related hospital admissions annually in the United States, resulting in an estimated financial cost of $2.4 billion (Lanzkron et al., 2010; Ballas and Lusardi, 2005). Consequently, a sedentary lifestyle is notably common among this population. Therefore, the question arises: what are the consequences for individuals with SCD who lead such a lifestyle?

Firstly, a sedentary lifestyle contributes to increased blood viscosity (Copolla et al., 2004; Nader et al., 2019), a condition that exacerbates the sickling process of red blood cells. Exercise promotes better circulation and plays a critical role in reducing blood viscosity, which in turn lowers the risk of crises. These crises are likely to decrease in individuals with SCD who engage in regular, moderate physical activity. Thus, the absence of exercise not only forfeits this protective benefit but also enhances the propensity for such debilitating episodes.

Lifestyles characterized by low levels of physical activity are linked to a higher risk of heart disease and increased mortality rates (Kruger et al., 2003; Piel et al., 2017; Melo et al., 2021). The efficiency of oxygen transport throughout the body plays a pivotal role in heart health, and regular exercise markedly enhances this process. For individuals with conditions such as Sickle Cell Disease (SCD), a deficiency in physical activity can exacerbate cardiovascular efficiency. Given the already impaired capacity of sickled cells to transport oxygen, any further decrease in oxygen delivery can precipitate more severe health complications (Nyante et al., 2019) which could include acute chest syndrome (ACS) and Community-Acquired Pneumonia.

The connection between physical inactivity and sickle cell disease (SCD) highlights deep-seated health challenges that go beyond the disease’s immediate effects. For example, a sedentary lifestyle, which is prevalent among individuals with SCD, often leads to obesity, which can exacerbate other health conditions. Intriguingly, even though young individuals with SCD tend to burn more calories at rest, a significant portion—ranging from 19% to 22% are overweight or obese (Chawla et al., 2013; Zivot et al., 2017), mirroring obesity trends in the general US population (Hales et al., 2017). Within this segment, 16% are children and 26% are adolescents (Mitchell et al., 2009). These figures shift the focus from the historically emphasized issues of undernutrition and stunted growth within the Sickle Cell Disease (SCD) community to underscore a vital area of concern: weight management for these patients. The rising incidence of overweight and obesity, especially among young people with SCD, is alarming. This trend increases their risk of developing conditions such as hypertension and stroke, which could further burden their already at-risk cardiovascular system. Additionally, obesity in SCD patients is associated with reduced mobility, and inactivity leads to reduced bone density, a predisposing factor for osteoporosis and fractures (Sadat-Ali et al., 2004; Shah et al., 2004; Miller et al., 2006; Ozdemir et al., 2016). These individuals need to be sensitized and encouraged to adopt regular exercise to enhance the overall quality of their lives and steer them away from secondary complications.

Exercise training is universally acknowledged for its anti-inflammatory effects that benefit not just healthy individuals (Pedersen & Hoffman-Goetz, 2000; Pinto et al., 2020) but also those with various health conditions such as respiratory, cardiovascular, and metabolic diseases (Yang & Chen, 2003; Halle et al., 2004; Zoppini et al., 2006; Hamer et al., 2012). This benefit underscores the vital role of physical activity in the management of Sickle Cell Disease which is synonymous with inflammations. Advanced exercise tests that access the combined functionality of the heart, lungs, and muscles under peak exertion do not exacerbate vascular inflammation or edema in children with SCD (Liem et al., 2015). This confirms the safety and potential advantages of judiciously designed exercise regimes in SCD care which cannot amplify major health concerns. Therefore, identifying a balanced, moderate exercise regimen is crucial in effectively managing SCD.

In summary, while the potential for exercise to trigger sickle cell crises and other complications cannot be dismissed, the overarching benefits of a tailored, moderate exercise regimen far outweigh the risks when appropriately managed. The adverse effects of a sedentary lifestyle on individuals with SCD ranging from increased blood viscosity and reduced oxygen delivery to compromised immune function, and poor pain management highlight the critical need for integrating physical activity into the comprehensive care plan for SCD. Such an integration requires a collaborative effort between healthcare providers and patients to devise exercise strategies that are safe, effective, and tailored to individual needs.

Living with Sickle Cell Disease (SCD) involves navigating a complex landscape of symptoms, among which pain emerges as a notably daunting challenge. Sickle cell crises, a defining feature of Sickle Cell Disease (SCD), present as episodes of acute pain and significantly affect the quality of life for individuals with the condition. Traditional pain management strategies for Sickle Cell Disease (SCD) have primarily focused on pharmacological interventions, utilizing a variety of analgesics. However, emerging evidence highlights the complementary role of exercise in mitigating pain associated with SCD. Research explores the mechanisms by which exercise acts as a potent tool in managing sickle cell pain, revealing both physiological and psychological benefits that underpin this approach. 

Exercise plays a role in managing inflammation and reducing painful episodes in individuals with Sickle Cell Disease (Rossi et al., 2023). Through regular, structured physical activities, such as treadmill walking, brisk walking, step exercises, stationary cycling, and light jogging, the body’s inflammatory response is dampened, and the frequency of the pain crises is decreased. This is because inflammation is a precursor for the painful crises that define SCD and reducing it can lead to fewer and less severe crises. This is crucial because the clumping of blood platelets is averted, a situation that could otherwise aggravate inflammation. (Benatti et al., 2015; El-Kader & Al-Shreef, 2018). However, the choice of exercise is critical, especially considering the physical constraints of individuals with SCD. High-impact exercises, such as jumping jacks, hurdling, and navigating obstacle courses, may be less suitable for individuals with a higher center of gravity, a trait commonly seen in African Americans. Moreover, these activities may primarily exert excessive stress on the body without offering significant cardiovascular and muscular benefits, rendering them suboptimal for managing Sickle Cell Disease effectively.

Children with Sickle Cell Anemia face a significantly elevated risk of experiencing a stroke (Bodapati et al., 2017) due to decreased activity in the parasympathetic nervous system, which is responsible for relaxation. This reduction in activity causes an increase in inflammation by inhibiting the body’s natural anti-inflammatory mechanisms (Martelli et al., 2014), thereby raising the chances of blood vessel damage and blockages, both major contributors to stroke. Managing this risk is paramount and participating in gentle exercises, such as running or cycling, has been shown to offer considerable benefits (May et al., 2015; Sá et al., 2016). These activities not only improve heart function (Berger et al., 2019) but also help reduce the likelihood of stroke by enhancing cardiac performance and curbing complications. However, it’s crucial to customize these activities to meet the specific health needs of individuals with SCA by avoiding overly strenuous exercises. 

Connes et al. (2011) highlighted that increased exercise intensity might escalate the risk of red blood cell deformation in individuals with Sickle Cell Disease. However, the undeniable overarching benefits of engaging in regular, moderate exercise are clear. Just like in the general population, regular physical activity leads to long-term cardiovascular adaptations and enhances aerobic conditioning through structural and functional changes within the body (Blomqvist et al., 1983). This improvement is primarily due to a significant elevation in maximum oxygen consumption during peak exertion, serving as a crucial indicator of cardiovascular health and efficiency across various demographics and health conditions. For instance, engaging in aerobic training at 70% of one’s maximum effort for 45 minutes, three times a week, markedly enhances cardiovascular functionality (Murias et al., 2010). These findings highlight that well-designed exercise programs can offer significant health benefits to SCD patients by improving oxygen distribution throughout the body.

Individuals with Sickle Cell Disease often have a reduced number of blood vessels in their muscles which in turn, affects the body’s oxygen supply. Engaging in moderate exercise can stimulate the growth of new blood vessels in these muscles, thereby enhancing blood and oxygen flow, improving muscle functionality, and boosting overall physical capabilities (Merlet et al., 2019; Preidt, 2020). This improved circulation plays a crucial role in minimizing the risk of complications associated with SCD, potentially preventing organ damage. Thus, moderate exercise is an essential strategy for managing SCD symptoms and improving the quality of life for those affected.

In summary, creating exercise programs to manage pain in Sickle Cell Disease necessitates a personalized approach. Achieving this involves participating in physical activities that are both safe and beneficial while recognizing the unique challenges individuals with SCD face. Choosing low-impact, moderate-intensity exercises tailored to individual health requirements and consulting with healthcare professionals specialized in SCD enables individuals to safely enhance their endurance and strength. The relationship between exercise and pain management opens new possibilities for improving the well-being of those suffering from SCD. Incorporating a regimen of physical activity into a comprehensive pain management plan, which includes analgesics, can significantly affect the lives of those affected by this condition.

Recognizing the unique physiological characteristics of African American women is crucial for crafting customized exercise programs, particularly for those managing Sickle Cell Disease (SCD). For instance, African American women, on average, possess a breast size of 168.4 cm², significantly larger than the 121.7 cm² average found in white women (Stuedal et al., 2008). A larger average breast size can lead to increased discomfort during physical activities, owing to heightened breast motion. Addressing this and other challenges necessitates the development of exercise routines that specifically aim to minimize high-impact movements and alleviate discomfort. Additionally, the challenge of finding sports bras that offer adequate support is significant is a challenge for these women, and even when such bras are available, they often come with a high price tag. Tailoring exercise strategies and support solutions to the physiological and financial realities of African American women with SCD will not only improve their comfort during physical activities but also enhance their overall care in the management of SCD.

Conventional pain management is the standard care approach in sickle cell disease. However, integrating cognitive and behavioral therapies as complementary approaches marks a significant advancement in treatment strategies. Interventions designed to bolster the capacity of women living with sickle cell disease to manage pain more effectively have been demonstrated to improve the quality of life for both children and adults (Gil et al., 1997; Thomas, Dixon, & Milligan, 1999). Moreover, the use of comprehensive treatment manuals in these therapies highlights their success and utility in clinical practice (Anie, 2005).

Given that Sickle Cell Disease (SCD) presents a range of severity levels, each necessitating a customized approach to exercise, the key to effective pain management often lies in the skillful and thoughtful integration of both physical and mental exercises, whether for mild, moderate, or severe cases. This balanced strategy will enhance the overall quality of life for these women.

Drawing on the 2020 study by Dehkordi et al., which highlighted the benefits of water-based exercises for beta-thalassemia patients—a condition closely related to SCD—incorporating water aerobics into the comprehensive treatment plans for SCD patients could be highly beneficial. This inclusion, alongside traditional treatments such as blood transfusions and medication, offers a multifaceted approach that not only enhances crucial blood metrics and life quality but also bolsters social bonds and mitigates depression. Customizing this strategy for SCD patients means adopting water exercises performed at waist depth, avoiding swimming, or submerging the head to respect cultural sensitivities. The suggested regimen recommends 40-minute sessions that begin with a 5-minute warm-up, such as pool walking, to ready the body. This leads to a 30-minute main phase of aerobic and resistance exercises, carefully selected to optimize health benefits without overburdening the body. The session wraps up with a 5-minute cooldown, involving gentle activities like walking, stretching, and simple movements to transition the body back to its resting state smoothly. This comprehensive and considerate exercise plan is specially designed to meet the unique needs of those with SCD, carefully navigating the risk of potential crises.

Building upon the comprehensive approach of water-based exercises, the endurance training program using a cycle ergometer is another customized exercise that women grappling with SCD can utilize. This moderate-intensity program, consisting of three 40-minute sessions each week, bolsters muscle strength and enhances the health of small blood vessels within the muscles, addressing issues related to the body’s blood microcirculation (Merlet et al., 2019). The exercise starts with a warm-up then progresses to a constant-load endurance exercise and concludes with a cool-down and light stretching. Safety is emphasized through careful monitoring of perceived exertion. This program carefully considers not only the physical challenges associated with SCD but also the overall well-being of individuals. Importantly, the availability of affordable cycle ergometers makes this exercise regimen financially accessible, ensuring that the cost does not become a barrier for women with SCD seeking to improve their physical health.

Yoga and low-impact aerobic exercises are another beneficial mental intervention for women with Sickle Cell Disease paralleling their effectiveness in easing symptoms akin to those observed in multiple sclerosis (Alphonsus et al., 2019). Engaging in regular yoga and low-impact aerobic routines not only helps in mitigating symptoms but also enhances both physical and mental health. This improvement potentially translates into reduced healthcare costs by minimizing the necessity for frequent therapeutic interventions and hospital admissions. Incorporating yoga and similar types of gentle exercises into a thrice-weekly schedule improves overall well-being, including reductions in fatigue and pain intensity.

The journey to alleviate the physical challenges of Sickle Cell Disease in African American women through exercise demands a customized, and comprehensive strategy. Focusing on exercise programs specifically designed for the unique physiological, cultural, and financial circumstances of these women unlocks new possibilities for health and comfort. This approach goes beyond merely addressing the direct physical symptoms of SCD; it also fosters mental strength and emotional wellness. As refinements and expansions to these exercises continue, the vision of a more manageable life with SCD through physical activity becomes ever more realistic. Therefore, these tailored exercise programs stand as beacons of hope and empowerment that can guide African American women with Sickle Cell to live a life where the disease does not hinder their ability to live fully and vibrantly.

Imagine a life where each day brings an unpredictable challenge—a life with Sickle Cell Disease (SCD), an inherited condition where red blood cells warp into sickle or crescent shapes. This change goes beyond mere alteration in shapes; it heralds a turbulent journey characterized by crises, anemia, and heightened susceptibility to infections (Elendu et al., 2023; CDC). While the CDC promotes exercise as a fundamental aspect of health, for individuals with SCD, this route is marked by distinctive challenges. Understanding these complexities helps in crafting strategies that will not only promote physical fitness but also address the unique challenges seen in sickle cell populations.

Chronic painful episodes are a hallmark of Sickle Cell Disease (Piel et al. 2017). Individuals afflicted with this condition frequently experience decreased blood oxygen levels, which are caused by defective hemoglobin in their red blood cells (Quinn and Sargent (2008; Setty et al. 2003).  As a result, their bodies attempt to compensate for the low levels of red blood cells by increasing blood production. However, this effort is often inadequate, leading to recurrent anemia (Piel et al., 2017). The combination of these challenges hampers the effective transportation of oxygen to the body’s tissues (Nahavandi et al. 2004; 2009).

Over 50 % of those with sickle cell disease report pain, exhaustion, and dehydration as pivotal deterrents to physical activity (Osunkwo et al., 2021). These symptoms do not just cause immediate discomfort but also increase the risk of severe health complications, making the decision to exercise fraught with potential risks. Furthermore, the pervasive issue of fatigue critically undermines their daily living quality and the capacity to sustain physical exertion. This constellation of challenges—pain, exhaustion, dehydration, and debilitating fatigue collectively acts as a formidable barricade against the pursuit of an active and healthy lifestyle for those afflicted with sickle cell disease (Osunkwo et al., 2021).

Children living with Sickle Cell Disease (SCD) exhibit a notable disparity in their engagement in physical activities. Although a significant majority, 90%, participate in physical education classes, only about 48% engage in organized sports. Furthermore, their involvement in moderate-to-vigorous physical activities tends to be of shorter duration (Omwanghe et al., 2017). This variation in activity levels is primarily attributed to widespread concerns regarding the risks associated with physical overexertion. There exists a fear that exerting themselves too intensely could precipitate complications, given the disease’s pro-inflammatory nature (Connes et al., 2011). Such apprehensions lead to a more cautious approach towards participating in sports and intense physical activities among these children.

As previously mentioned, individuals with Sickle Cell Disease (SCD) exhibit reduced oxygen levels in their bloodstream. Consequently, their muscles often demand more oxygen during physical activities. However, due to these diminished oxygen levels, there is a heightened risk of the cells clustering together (Martin et al., 2018). Moreover, red blood cells become prone to deformation and may cause blockages in the bloodstream, particularly during strenuous activities. Such occurrences could lead to severe health complications (Connes et al., 2011; Nader et al., 2020; Sundd et al., 2019). Given these potential risks, individuals with SCD may prefer to avoid physical exertions, inclining towards a more sedentary lifestyle.

Adolescents living with Sickle Cell Disease often lead sedentary lifestyles because of their widespread lack of confidence in engaging in sports, exercises, or any form of physical activity (Shields and Synnot, 2016). This diminished confidence negatively affects their self-image. Coupled with the disease’s physical limitations, it leads to lowered self-esteem and an increased sense of vulnerability consequently driving them towards a more sedentary lifestyle.

For some individuals, the mental and emotional strain associated with the condition fosters a pervasive fear of physical exertion which is rooted in the possibility of unseen adverse effects rather than the direct experience of pain. This psychological barrier prevents the initiation or persistence of any form of exercise (Thornburg et al., 2011; Vlaeyen et al., 1995; Martin et al., 2018). The anticipation of pain, rather than its immediate presence, acts as a formidable obstacle to physical activity, underscoring the profound impact of psychological considerations on the decision-making processes regarding health and exercise.

It is essential to tackle the complex challenges that deter individuals with Sickle Cell Disease (SCD) from participating in physical exercise by adopting a holistic and personalized approach. This approach should encompass managing chronic pain, dispelling misconceptions, offering accessible resources, and enhancing awareness. By identifying and eliminating these barriers, a more inclusive and supportive environment for physical activity can be established. This ensures that individuals with Sickle Cell Disease (SCD) can lead fulfilling lives, focusing on their health and overall well-being.