The Impact of Chronic Pain, Faulty Interoception and Appropriate Interventions on Mental Health in Sickle Cell Disease

Sickle cell disease, mental health, and interoceptive dysregulation

Sickle Cell Disease (SCD) is widely acknowledged as a devastating physical disease associated with increased symptom severity, contributing significantly to the global burden of disease study findings (Chukwuka Elendu et al., 2023). The excruciating pain crises, organ damage, and shortened lifespan rightfully demand medical attention, with particular consideration for associated personality disorders and post-traumatic stress disorder. However, a crucial, overlooked piece of the puzzle is jeopardizing the well-being of SCD patients: disrupted body-mind communication, exacerbated by psychiatric comorbidities, mood disorders, and the misfiring of the autonomic nervous system.

The Case of Alexia: This experience highlights the chronic pain and mental health narrative.

“Ever since I was a child, it wasn’t just the pain,” shares Alexia, a thirteen-year-old battling SCD. “It was this feeling of being on high alert, overwhelmed even when I knew I ‘should’ be coping. Doctors kept saying it was about the stress of the disease, depression, and anxiety, but medications and therapy weren’t enough.”

Alexia’s experience is not an anomaly; it is common among those with chronic pain conditions and several psychiatric disorders, often highlighted in health studies, specifically those examining patients with major depressive disorder. Many SCD patients struggle with seemingly inexplicable mental health challenges, often related to anxiety disorders, major depressive disorder, and depressive states, as noted by the Institute of Mental Health (Oudin Doglioni et al., 2021). The conventional explanation focuses on the psychological toll of a chronic illness. Still, it’s important also to consider psychiatric disorders and mood disorders, as highlighted by a recent 2024 systematic review and meta-analysis (Horsburgh et al., 2024). However, emerging science suggests a deeper physiological issue often at play: interoceptive dysregulation, possibly contributing to psychiatric disorders and eating disorders and affecting brain structure.

This hidden struggle is not merely a theoretical concern; it often requires interventions like cognitive behavioral therapy, sensory integration, physical and occupational therapy, and other interventions targeting the autonomic nervous system and the effects of stress on the interoceptive system. A comprehensive scientific review titled ‘The Relationship Between Pain and Interoception: A Systematic Review and Meta-Analysis’ confirms a strong link between chronic pain and impaired interoception, highlighting the need to consider psychiatric and cognitive factors. Functional magnetic resonance imaging was utilized to explore the neural pathways involved in processing interoceptive signals, aiming to measure interoceptive awareness using a multidimensional assessment of interoceptive awareness. This means that the repeated pain crises characteristic of SCD likely throw the body’s internal signaling system into disarray, contributing to both the physical and psychological burdens of the disease.

Understanding Interoceptive dysregulation

Acknowledging its connection to cognitive and affective disorders can help manage symptoms of chronic pain conditions, type 2 diabetes mellitus, and stress response. While the term ‘interoception’ might be new to some, it describes how our bodies and brains constantly communicate about what’s happening inside us, which is vital for understanding psychiatric conditions. Think about everything from your heartbeat and breathing to feelings of hunger or temperature changes, as well as behavioral responses to stimulus and their effects on various brain regions; interoception refers to these internal states and their impact on the body and mind. Scientists have been fascinated by this body-mind connection for centuries, and their work reveals just how important interoception is for our emotions, decision-making, and even our sense of self (Khalsa et al., 2018). Understanding this complex system is an exciting frontier for research with direct implications for improving health and well-being, particularly in the context of psychiatric and cognitive disorders and overall health outcomes.

SCD: The Perfect Storm for Dysregulation and Mood and Anxiety Disorders.

• Repeated Trauma of Pain Crises: Addressing both the physiological and psychiatric aspects, including the stress response and the role of the prefrontal cortex, is key to understanding associated psychiatric conditions. Especially in early life, severe pain can rewire the nervous system, making it hypervigilant to even minor internal changes, which could be a risk factor for psychiatric disorders and cardiovascular disease.
• Chronic Stress on the Body: Evaluating the impact on both the physiological systems and the behavioral aspects, including correlates in the central nervous system and the role psychiatry plays in mood and anxiety disorders. Constant inflammation, medication side effects, and compromised organ function send confusing signals to the brain, further taxing the system and potentially exacerbating mood and anxiety disorders and even conditions like sickle cell disease.
• Misinterpretation: Patients feeling distressed with no obvious trigger are often mislabeled as having poor coping skills or pre-existing mental health conditions when they may be experiencing a stress response linked to post-traumatic stress disorder.

The Disconnect

Understanding interoception could bridge the gap in treatment for mood and anxiety disorders and potentially for sickle cell disease. This is why traditional talk therapy often falls short for SCD patients due to the complexity of their stress response, major depressive disorder, and psychiatric comorbidities. Their struggles stem from a misfiring bodily alarm system, not just conscious thought patterns, often linked to the stress response and further exacerbated by conditions like anxiety and depression. The disorder is associated with physiological stress.

The Path to Holistic Healing

Integrating insights from interoception and collaborating with mental health professionals, especially for managing sickle cell disease. Alexia’s breakthrough came when her mother, an advocate for her care, insisted her medical team consider interoceptive dysregulation and requested an occupational therapy referral, highlighting the importance of addressing potential interoceptive causes of psychiatric disorders. A shift towards calming her nervous system through occupational therapy, in addition to her medical and holistic approach, is finally bringing relief with improvements in her chronic condition and mental health outcomes.

Call to Action for Healthcare Providers of Sickle Cell Patients

Incorporate an understanding of comorbid conditions such as anxiety disorders, major depressive disorder, depressive states, somatic symptom disorder, and substance use disorder.

• Awareness: Recognize the significance of interoceptive processes in addressing disorder symptoms and mental health problems, fostering a greater interest in interoception and its implications for mood and anxiety disorders. Familiarize yourself with interoceptive dysregulation and its potential as a risk factor for psychiatric disorders and somatic symptom disorder, identified through various longitudinal studies and prospective studies. Consider it as an underlying factor, especially when anxiety or depression in SCD patients seem resistant to standard treatment, suggesting possible comorbidity with major depression and generalized anxiety disorder.
• Assessment: A comprehensive approach should include an evaluation of interoceptive signals and their impact on mental and physical health, employing a multidimensional assessment of interoceptive awareness. Simple tools exist to screen for interoceptive dysfunction, early life stress, and possible correlates in heart rate irregularities, supplementing longitudinal studies in this field, especially for individuals with type 2 diabetes mellitus. Integrate these into SCD patient evaluations, considering the comorbidity of anxiety disorders, depressive conditions, generalized anxiety disorder, and other mental disorders, as patients with major depressive disorder often experience heightened interoceptive difficulties.
• Trauma-Informed Care: Important for managing comorbid posttraumatic stress disorder, anxiety disorders, and major depression in SCD patients, incorporating cognitive behavioral therapy, as supported by the World Health Organization. Recognize that repeated medical interventions can create trauma, especially for young patients, contributing to this dysregulation and adding to mental health problems, frequently exacerbated by their response to acute stress.
• Collaboration: Mental health professionals must work together to understand the interoceptive processes in SCD patients and those with type 2 diabetes mellitus. Referrals to physical and occupational therapists specializing in sensory integration could provide a crucial missing piece in treatment plans beyond symptom management, potentially reducing psychiatric conditions.

Call to Action for Patients with Sickle Cell Disease

Randomized clinical trials have indicated that interoceptive signals can play a critical role in managing chronic pain and mental health disorders.

• You Are Not Alone: Understanding the shared experiences in managing chronic conditions like type 2 diabetes mellitus and mood disorders. If the “mental health” answers do not fit, trust your experience. Research interoception and its connection to chronic illness, psychiatric disorders, and physical health, as recognized by the National Institutes of Health, and note the increasing interest in interoception in recent studies.
• Advocate for Yourself: Educate your healthcare providers about the risks of psychiatric disorders linked to interoceptive dysregulation and the potential biomarkers involved, including those associated with the anterior cingulate cortex. Ask your doctor about interoceptive dysregulation testing and its implications for psychiatric and cognitive health, and consider enrolling in a randomized controlled trial to examine the response to acute stress further. Seek providers offering a more body-oriented approach alongside your SCD medical management to address potential psychiatric disorders that may arise, particularly as they relate to the autonomic nervous system.
• Share Your Story: Mental health professionals encourage patients to share their experiences to understand better interoceptive processes and their impact on mood and anxiety disorders. Raising awareness within the SCD community empowers others and can drive progress in the research and understanding of this complex interplay, particularly concerning cognitive and psychiatric disorders.

Addressing interoceptive dysregulation will not magically erase SCD or any comorbid mental disorders, but it can mitigate the hyperactivity of the sympathetic nervous system and improve physical health. However, optimizing the body-mind connection can significantly improve mental well-being, possibly serve as a biomarker for progress, and potentially lessen the severity of physical symptoms over time. According to Horsburgh et al. (2024), interoception refers to the body’s ability to sense its internal state, which can be notably affected in cases like sickle cell disease. Pain is not the only cause of this dysfunction. It may be linked to abnormalities in certain brain regions, such as the anterior cingulate cortex, as shown in recent health studies focusing on the effects of stress and interoception. Dysfunctional interoception itself can worsen pain perception and complicate pain treatment, particularly in chronic pain conditions and neurological disorders, necessitating improved chronic pain treatments. Giving patients the tools to feel safe within their bodies is essential for true healing—beyond pain management and into a life with less internal chaos, greater resilience, and improved interoceptive awareness.

References

Chukwuka Elendu, Amaechi, D. C., Chisom Euphemia Alakwe-Ojimba, Elendu, T. C., Elendu, R. C., Ayabazu, C. P., Aina, T. O., Oluyinka Bamidele Aborisade, & Adenikinju, J. S. (2023). Understanding Sickle Cell disease: Causes, symptoms, and Treatment Options. Medicine, 102(38), e35237–e35237. https://doi.org/10.1097/md.0000000000035237

Horsburgh, A., Summers, S. J., Lewis, A., Keegan, R. J., & Flood, A. (2024). The Relationship Between Pain and Interoception: A Systematic Review and Meta-Analysis. ˜The œJournal of Pain/Journal of Pain. https://doi.org/10.1016/j.jpain.2024.01.341

‌Khalsa, S. S., Adolphs, R., Cameron, O. G., Critchley, H. D., Davenport, P. W., Feinstein, J. S., Feusner, J. D., Garfinkel, S. N., Lane, R. D., Mehling, W. E., Meuret, A. E., Nemeroff, C. B., Oppenheimer, S., Petzschner, F. H., Pollatos, O., Rhudy, J. L., Schramm, L. P., Simmons, W. K., Stein, M. B., & Stephan, K. E. (2018). Interoception and Mental Health: A Roadmap. Biological Psychiatry. Cognitive Neuroscience and Neuroimaging, 3(6), 501–513. https://doi.org/10.1016/j.bpsc.2017.12.004

‌ Oudin Doglioni, D., Chabasseur, V., Barbot, F., Galactéros, F., & Gay, M.-C. (2021). Depression in adults with sickle cell disease: a systematic review of the methodological issues in assessing prevalence of depression. BMC Psychology, 9(1), 54. https://doi.org/10.1186/s40359-021-00543-4