This 1st series blog will be followed by thalassaemia in series 2 and their link to occupational therapy. This blog came about sickle cell and thalassaemia are not well talked about and their characteristics can easily be mistaken for extreme exhaustion or laziness to name a few
I have decided to write about sickle cell because it has been a personal challenge, and despite its prevalence, it does not receive the attention, funding, or public discourse it deserves. In addition to remaining under‑researched compared with other chronic conditions, many clinicians report limited training or confidence in managing it.
What is sickle cell?
Sickle cell disease (SCD) is an inherited haemoglobin disorder that continues to pose a significant global health concern (GOV.UK, 2025). In over 7.7 million people, there are approximately 515,000 babies born with the disease each year, and an estimated 376,000 deaths in 2021, affecting mainly sub-Saharan Africa (WHO, 2025). As a monogenic blood disease, haemoglobinopathies arise when individuals inherit mutated haemoglobin genes from both parents, who are often asymptomatic carriers (Franco et al., 2024).
What causes sickle cell disease
SCD is caused by a mutation in the β-globin (HBB) gene, leading to the production of abnormal haemoglobin S (HbS) (Inusa et al., 2019; Eleftheriou et al., 2025). Under conditions of low oxygen, red blood cells become rigid and adopt a characteristic sickle or crescent (half-moon) shape. These distorted cells are prone to causing vaso-occlusion, blockages in small blood vessels which results in chronic haemolytic anaemia, tissue ischaemia, and widespread organ damage (Inusa et al., 2019; Elendu et al., 2023).
The burden of sickle cell
Globally, the burden of SCD is substantial. The World Health Organization estimated that 7.74 million people were living with SCD in 2021, with the highest prevalence in sub-Saharan Africa (WHO, 2025). Mortality data further reveal the severity of the condition: approximately 81,100 deaths occurred among children under five in 2021, making SCD the 12th leading cause of death in this age group. Notably, traditional mortality recording systems significantly underestimate its impact, with total deaths estimated to be up to 11 times higher than reported figures (WHO, 2025). Broader estimates suggest that over 30 million individuals worldwide are affected, reinforcing SCD as one of the most prevalent inherited blood disorders globally (Joseph et al., 2025).
Pain Mechanisms
Pain in SCD is not only physical but also neurophysiological. Research indicates that both painful stimuli and the anticipation of pain can trigger autonomic nervous system responses, including vasoconstriction (Khaleel et al., 2017). In some cases, individuals may experience syncope during severe pain episodes, potentially linked to vasovagal responses. This highlights the complex interplay between the nervous system and pain perception in SCD. For a deeper understanding of how the nervous system processes pain, perspectives such as polyvagal theory provide useful insight into these mechanisms.
To find out more check out Polyvagal Theory on YouTube
Implications for Health
Physical and medical impacts
SCD has profound physical consequences, many of which are chronic and progressive. Pain is the hallmark symptom, with vaso-occlusive crises causing severe, often debilitating discomfort in the limbs, chest, or back (Tolu & Van Doren, 2022). From a personal perspective, I experience overwhelming and unpredictable pain which can disrupt my daily life.
Chronic anaemia is another key feature, resulting in reduced oxygen-carrying capacity of the blood. With this, I experience persistent fatigue, shortness of breath, weakness, pallor, significantly limiting physical activity and my coping mechanism involves chewing lot of ice cubes as I am constantly dehydrated, water on its own feels hard and smell of iron when trying to drink. Over time, repeated vaso-occlusion (where sickled red blood cells clog blood vessels, blocking blood flow to tissues and organ) leads to cumulative organ damage, affecting the spleen, kidneys, liver, heart, and joints (Manwani & Frenette, 2013).
Neurological complications are also common. Children with SCD face a heightened risk of ischaemic stroke, while adults may experience haemorrhagic events (Shah et al., 2024). Acute chest syndrome, a life-threatening pulmonary complication, further contributes to morbidity and mortality, in addition to a splenic dysfunction, which increases susceptibility to severe infections such as pneumonia and meningitis (Shah et al., 2024).
Growth and developmental delays are often observed in children, reflecting the systemic impact of chronic illness. These physical challenges illustrate how SCD is not a single-condition illness but a multi-system disorder requiring comprehensive care.
Psychological and emotional impacts
Beyond its physical burden, SCD has significant psychological implications. Studies report high rates of depression (39%) and anxiety (38%) among individuals living with the condition (Osunkwo et al., 2021). The unpredictability of pain crises can lead to chronic stress, fear, and a sense of loss of control.
From a lived-experience perspective, this uncertainty often affects mental well-being as much as physical health. The inability to plan ahead due to the sudden onset of pain episodes can lead to social withdrawal, isolation, and reduced quality of life. Everyday activities, education, and employment may be disrupted, reinforcing cycles of disadvantage.
Social and life impacts
SCD also carries broader social consequences, where educational attainment may be affected by frequent absences due to illness, while career progression can be hindered by ongoing health challenges (Sickle cell in school, n.d.).
In adulthood, individuals may face additional concerns related to relationships, family planning, and financial stability.
Reproductive health issues also extend to sexual wellbeing. Women, in particular, report reduced sexual satisfaction, often due to pain (dyspareunia) associated with vaso-occlusive episodes (Fajewonyomi & Adeyemo, 2007) with pregnancy associated with increased risks, including miscarriage and preeclampsia. (Jain et al., 2019). Men may experience priapism a prolonged and painful erection, while both men and women may face infertility linked to hypogonadism (Smith-Whitley, 2014).
These aspects of SCD are often under-discussed in clinical settings, yet they are central to quality of life. Addressing them requires a holistic, patient-centred approach that goes beyond medical management to include psychological support, education, and open conversations about intimacy and wellbeing.
In summary, sickle cell disease is a complex, lifelong condition with far-reaching consequences across physical, psychological, and social domains. While advances in medical care have improved survival, significant challenges remain in managing pain, preventing complications, and addressing the broader impacts on quality of life. Integrating clinical care with patient education, psychosocial support, and awareness is essential in improving outcomes for individuals living with SCD. Importantly, incorporating lived experiences into academic and clinical discourse provides a more comprehensive understanding of the condition, bridging the gap between theory and reality.
This is a 3-series blog including sickle cell, thalassemia, and the link to OT intervention
About the Author
I’m Sialou, a final-year MSc Occupational Therapy student currently on placement with JB Occupational Therapy. I focused on sickle cell and thalassaemia two inherited blood disorder, through this blog to raise awareness of the negative impact on health for individuals living with either of both disorders. As a person living with sickle cell, I aim to reflect lived experience through an occupational therapy lens.
Helpful sickle cell links for more information
Sickle Cell Society
54 Station Road
London
NW10 4UA
020 8961 7795
Helpline Mon – Fri 9am – 5pm (except bank holidays) Mon & Tue: 07842 245 980; Wed: 020 89617795; Thurs & Fri: 07809736089
- Confidential email for helpline and general enquiries: info@sicklecellsociety.org
- https://www.facebook.com/SickleCellUK
- info@sicklecellsociety.org
- https://www.instagram.com/sicklecelluk/
- https://www.youtube.com/c/SickleCellSocietyUK
Sickle Cell & Thalassaemia Support Project: https://www.sctsp.org.uk/
Tel: 01902444076
- Email: info@sctsp.org.uk
Community Family Support Service by SCTSP: 01902382288
- Admin: admin.fss@sctsp.org.uk
- Address: Sickle Cell & Thalassemia Support Project
- Paycare House, George Street, Wolverhampton, WV2 4DX
Sickle cell in school.(n.d.).
Sickle Cell In School – Sickle Cell Care Manchester
