Considering Mental Health Implication for Patients with Primary Progressive Multiple Sclerosis
By: Gabriela Rose Bevacqua-Collins, Applied Clinical Psychology masters program Class of 2024
Multiple Sclerosis is an autoimmune disease that attacks the myelin sheath on the axons of the central nervous system (CNS). Patients with MS lose immune homeostasis that triggers an inflammatory response which targets the myelinated axons in cortical regions of the brain and spinal cord (Baranzini & Oksenberg, 2017; Buecken et al., 2012; Højsgaard Chow et al., 2018; Holland et al., 2023; Lamb et al., 2022; Methley et al., 2016; Pérez-Miralles et al., 2019; Sparaco et al., 2021). This results in lesioning across regions of the CNS, reduction in cortical thickness, and an inability for neurons to communicate with each other, producing progressive and irreversible neurological dysfunction (Baranzini & Oksenberg, 2017; Buecken et al., 2012; Højsgaard Chow et al., 2018; Holland et al., 2023; Khan et al., 2011 Lamb et al., 2022; Methley et al., 2016; Pérez-Miralles et al., 2019; Sparaco et al., 2021). The onset of the disease typically presents itself between 20 to 50 years of age and is diagnosed through MRI imaging (Baranzini & Oksenberg, 2017; Højsgaard Chow et al., 2018; Holland et al., 2023). Primary Progressive Multiple Sclerosis (PPMS) is considered the most severe form of MS and the symptoms can include weakness, problems with mobility, vision issues, speech disorders, spasticity, paresthesia, changes in cognition, respiratory complications, bowel and bladder dysfunction, pain, fatigue and dysphagia (difficulty swallowing; Holland et al., 2023; Khan et al., 2011; Rosiak &Zagożdżon, 2017). In addition to the physical and neurocognitive complications resulting from the progression of the disease, individuals with PPMS often have to endure mental health difficulties.
PPMS is distinguishable from both relapsing remitting (RRMS) and secondary progressive (SPMS) forms of multiple sclerosis. RRMS is defined by episodes of worsening MS symptomologies interwoven with periods of remission in which symptoms remit (Holland et al.,2023; Lamb et al., 2022). RRMS can sometimes transition to a SPMS classification where symptoms begin to progress with or without periods of remission (Holland et al, 2023). PPMS, on the other hand, is characterized by an initial rapid decline in functioning which steadily progresses without any periods of remissions. (Buecken et al., 2012; Højsgaard Chow et al., 2018; Holland et al., 2023; Khan et al., 2011; Lamb et al., 2022; Pérez-Miralles et al., 2019; Sparaco et al., 2021). Only 10% of people with MS (pwMS) have PPMS (Holland et al., 2023; Khan et al., 2011; Lamb et al., 2022). Women are three times more likely to have MS however, men are more likely to have PPMS than other forms of MS (Holland et al., 2023).
Compared to patients with RRMS, previous research has shown that individuals with PPMS are four times more likely to report poorer quality of life (Rosiak &Zagożdżon, 2017). Over 40% of patients cannot walk and need assistance in daily living; patients with PPMS are eight times more likely to be unable to walk than those with RRMS (Holland et al., 2023; Kratz et al., 2016; Simmons et al., 2010). While RRMS has several pharmacological interventions which increase the length of remission period, no treatments have been shown effective for patients with PPMS. Ocrelizumab, designed to slow the progression of disease, is the only medication for PPMS that has been approved by the FDA, but it is not effective for all individuals with PPMS (Khan et al, 2011; Lamb et al., 2022; National MS Society, n.d.). Treatment of PPMS therefore is limited to rehabilitation, neuropalliative care, and management of symptoms (Khan et al, 2011).
The explicit cause of MS is still unknown, however, research has proposed potential etiological factors of the disease. The current consensus is that no one genetic locus or one environmental factor is responsible for MS, but it is rather a combination of a genetic predisposition and environmental triggers (particularly viral infections contracted in early childhood) that results in the disease presentation (Baranzini& Oksenberg 2017; Holland et al., 2023; Khan et al., 2011; Marrie 2004). Additionally, the prevalence of MS increases as you move north from the equator, however, this finding has its limitations because the majority of research into MS has been conducted in these geographical locations (Marrie 2004). The complexities of the diagnosis and the unidentified etiology is in part due the disease’s rarity, heterogeneity of symptomatology, and a three decade long history of misdiagnosis following its discovery by Jean-Martin Charcot in 1868 (Talley, 2005). It was not until neurology was established as a specialty in the late 1920s and the rapid congregation of people in urban centers during the industrial revolution that MS was diagnosed with any regularity (Talley, 2005). The need for clinical categorization of the phenotypic variety within MS diagnoses was accounted for in 1996 with the formation of three main types of MS: RRMS, SPMS, and PPMS (Klineova et al., 2018). Now PPMS is often diagnosed as RRMS initially, but the diagnosis is switched to PPMS once there is a steady progression of disease-related symptoms and there is no evidence of periods of remission (Holland et al., 2023; Perez-Miralles et al., 2019; Sparaco et al., 2021).
In the context of current research, it is unknown whether psychological symptoms are linked to the demyelination, lesioning, and volume loss in cortical areas (i.e., a part of the disease progression) or are they related to the social and interpersonal experiences after initial diagnosis (Holland et al., 2023; Methley et al., 2016; Sparaco et al., 2023). Anxiety occurs in 36% of individuals with PPMS and there is evidence of clinically significant levels of Generalized Anxiety Disorder and Panic Disorders (Methley et al., 2016; Sparaco et al., 2021). Schizophrenia, Bipolar Depression, and Obsessive Compulsive Disorder also occurs within this population (2.5% to 16.2%) however, there is not enough research to establish a definite link between MS and these disorders (Sparaco et al., 2021). While these three psychiatric disorders are less common compared to rates of anxiety within the MS population, Schizophrenia, BPD, and OCD all occur at higher rates among pwMS compared to the general population (Marrie et al., 2013; Sparaco et al., 2021).
Perhaps the most profound mental health implication to a PPMS diagnosis is depressive psychopathologies. More than 50% of patients with PPMS experience depressive symptomologies (Højsgaard Chow et al., 2018; Holland et al., 2023; Methley et al., 2016; Perez-Miralles et al., 2019; Sparaco et al., 202; Wakefield et al., 2013). The lack of treatment to stop or slow disease progression has generated feelings of hopelessness within PPMS community members (Holland et al., 2023). Patients with PPMS are two times more likely to complete suicide than individuals without a MS diagnosis (Sparaco et al., 2021). Additionally, the relationship between low quality of life and depression has been linked to disease-related progressive cognitive dysfunction, fatigue, and physical disability. It is these latter three symptoms which feed the multi-directional relationship between stigmatization, depression, and poorer quality of life (Broersma et al., 2018; Cadden et al., 2018; Cook et al., 2016; Højsgaard Chow et al., 2018; Methley et al., 2016; Perez-Miralles et al., 2019). More than 75% or individuals with PPMS experience some form of internal or external stigma, i.e., the societal or self rejection based on the devaluation of an individual based on their phenotypic departure from the norm (Broersma et al., 2018; Cadden et al., 2018; Cook et al., 2016; Perez-Miralles et al., 2019). For pwMS stigma most routinely takes the form of feeling isolated or left out of activities in addition to being self-conscious about their chronic illness and their resulting physical limitation as an artifact of internalized ableism (Broersma et al., 2018; Cadden et al., 2018; Cook et al., 2016; Perez-Miralles et al., 2019).
Given the prevalent association between the stigma surrounding progressive physical disability and depression it is imperative to micro-analyze the existing theoretical models for treating individuals with disability in order to identify where stigma originates and generate a therapeutic paradigm that accounts for progressive disability. While thinking within a biomedical model might initially make sense given that PPMS is a neurological disease, this model dangerously over pathologizes the individual as something that needs to be cured within a vacuum without of any consideration of social determinants of health (APA, 2022; Smart & Smart, 2006). In addition to hitting a metaphorical wall within this model due to the lack of definitive etiology, cure, and mitigating treatment factors for PPMS, the biomedical model’s overemphasis on the individual as a solvable “problem” inevitably leads to a dehumanization of the pwMS and thereby intensifying internal and external loci of stigma surround their disease (APA, 2022; Smart & Smart, 2006). The functional model also considers biological basis for disability but situates disability as a byproduct of societal expectations for how one should function within a particular environmental or occupational context (APA, 2022; Smart & Smart, 2006). While this model appears to be an optimistic departure from the biomedical model as it seeks to view individuals holistically, it does not consider the specificity of progressive diseases. Smart and Smart discuss chronic illnesses in their overview of existing theoretical models for treating individuals’ disabilities, but treatment within the functional model requires medical stabilization in order to improve quality of life through the prevention of subsequent complications and bolster independence (Smart & Smart, 2006). However, within the context of PPMS, medical stabilization is not possible and further disability and complications are inevitable resulting in a reduction in physical independence with the need for help with daily living.
Despite the progressive disability experienced by patients with PPMS as a biological explanation, the auxiliary psychiatry symptoms and levels of stigmatizations are best conceptualized within a social model. This theoretical orientation posits that disability is a social construct stemming from a system of oppression predicated on biases that routinely benefits abled bodied individuals and subjugates individuals with cognitive and physical limitations (APA, 2022; Smart & Smart, 2006). These socially conceived biases against disabled people are then internalized by individuals within this identity group, thereby generating feelings of stigmatization (APA, 2022; Perez-Miralles et al., 2019). In recognizing the cause of the stigma as a remnant of this societal systems of oppression, therapy can endeavor to alleviate the psychological ramifications of experiencing high levels of stigma around an PPMS diagnosis and reduction in levels of functioning by battling internalized ableism (Holland et al., 2023; Perez-Miralles et al., 2019; Sparaco et al., 2021).
This model also advocates for pushing back against forms of medical categorization that further ostracize people with disability (APA, 2022; Smart & Smart, 2006). This “othering” based on medical nomenclature is ever-present within the MS community as PPMS patients refer to themselves as orphans in the community and experience a doubled sense of otherness—having MS and then having PPMS (Holland et al., 2023; National MS Society, 2010). In order to come to terms with this multilayered otherness as generated by the social context in which pwMS reside, it is imperative that the working alliance focuses on finding PPMS specific communities (Methley et al., 2016; Rosiak & Zagożdżon, 2017; Wakefield et al., 2013). By helping the patient become active member in these community-based support groups, individuals can self-define their individual and collective disability identities (APA, 2022; Methley et al., 2016; Rosiak & Zagożdżon, 2017; Smart & Smart, 2006; Wakefield et al., 2013). Along with the added benefit of reducing feelings of stigma, higher levels of social support are associated with an increase in quality of life (Rosiak & Zagożdżon, 2017). Finding a PPMS community is especially important in the case of male patients with PPMS as they are four time more likely to report lower levels of perceived social support (Rosiak & Zagożdżon, 2017). In addition to improving levels of life satisfaction, higher group identification has been associated with the alleviation of the severity of both depression and anxiety symptomologies (Wakefield et al., 2013).
Due to the lack of a cure or pharmacological interventions, treatment should also help clients generate coping tools in order to come to terms with not being in control of their disease (Holland et al., 2023). Psychological care should focus on processing the grief that results from a PPMS diagnosis. This process requires clients working through common questions such as “why did this happen to me” or “what will my life look like now?” (Højsgaard Chow et al., 2018; Holland et al., 2023; National MS Society, 2010). Alleviating psychological symptoms can not only improve the quality of life of patients with PPMS but also increase patients’ adherence to treatment of their MS (Buecken et al., 2012; Højsgaard Chow et al., 2018; Holland et al., 2023; Pérez-Miralles et al., 2019; Sparaco et al., 2021). Therapists should seek consultation from MS medical professionals to increase their own professional knowledge and seek to integrate mental health care into MS care as a model for improving overall PPMS treatment adherence (APA, 2022; Minden et al., 2023). Methley and colleagues argue for the importance of relational continuity of care in terms of thoroughly knowing your patient’s PPMS diagnosis history, disability, speech patterns, body language (Methley et al., 2016). Therefore, in the context of individual therapy, it is vitally important that the client remains with the same therapist because having to start over and re-explain the history of their PPMS diagnosis can be psychologically taxing and exacerbate internalized stigma (Methley et al., 2016).
Therapists need to have end of life discussions with their PPMS clients. Buekcken et al. (2012) found that 76% of MS patients wanted to openly discuss disease progression and 74% of individuals wanted to create a plan for when they lose their decisional capacity (Buekcken et al., 2012). However, this majority consensus was not present in the case of explicit discussions of death; the desire to speak about dying depends on each patient so clinicians should ask the client if they wish to speak about dying (Buekcken et al., 2012). The explicit discussion about death becomes a requirement if your client has suicidal ideations or is at a high risk for completing suicide. Interwoven into conversations about disease progression, the formulation of a treatment plan should safeguard against future threats against autonomy due loss of decisional capacity (APA, 2022; Holland et al., 2023; McKenzie et al., 2015; Smart & Smart, 2006). Therapists should consider working with the client’s caregivers and incorporating family therapeutic techniques in order to help the family unit honor the client’s wishes for end-of-life care (Holland et al., 2023; McKenzie et al., 2015).
It is important that therapists do not avoid these difficult, and often uncomfortable, conversations. Navigating such sensitive topics with clients requires therapists to self-regulate in order to not exacerbate clients’ feelings of stigma and potential self-consciousness about their diagnosis. It is important not to frame such discussion by considering the client with PPMS as a kind of tragic figure, as this will undoubtedly elicit negative feelings of self within the client and exacerbate the existing power differential (APA, 2022; Smart & Smart, 2006). Genuinely listening to clients’ personal reactions to discrimination should lead to a broader discussion of how individual experience intersects with larger patterns of discrimination around disability as a cultural minority group (APA, 2022; Smart & Smart, 2006). This requires the therapist to explore and identify their own implicit biases (such as internalized ableism) as members of the same system of oppression in the United States. Seeking out personal testimonials from people living with PPMS can build a greater familiarity with the disease which will in turn generate a higher degree of empathy within the clinician. Treating patients with PPMS necessitates the consideration of social determinants of health and how psychological health intersects with their progressive decline in physical and cognitive functioning. Broaching disease-related topics requires empathy and authenticity in order to help clients grapple with the implications of a lifelong disease.
Additional Resources
There are a number of additional resources that therapists can utilize to build their competency and authenticity in treating the psychological needs of patients with PPMS. Hearing testimonials from patients living with MS is an effective means of learning how members of the community cope with their lifelong diagnosis. TED Talks, in connection with the University of Nevada, invited Robin Brockelsby to share her experience with MS in her lecture “How I’m Living My Best Life with Multiple Sclerosis” (TEDxUniversityofNevada, 2020). The Multiple Sclerosis Association of America (MSAA; Multiple Sclerosis Association of America – Improving Lives Today! | MSAA) and the National Multiple Sclerosis Society (NMSS; National Multiple Sclerosis Society | Leading the MS Movement) are multifaceted resources, providing information about the disease and current treatment options, where to find support groups in your area, and upcoming community events. Multiple Sclerosis News Today (Multiple Sclerosis News Today | Symptoms, Diagnosis, Treatments) is a publication which includes articles, videos, and podcasts on topics related to living with MS, new research findings and treatments, topics of diversity and inclusion, as well as testimonials from patients.
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