Neuromyelitis optica spectrum disorder (NMOSD), also known as Devic disease, is an
inflammatory disorder of the central nervous system. The primary symptom of NMOSD is
attacks of inflammation and damage in the optic nerves and spinal cord. Such attacks may
cause rapid onset of eye pain or blindness, limb weakness, numbness, or partial
paralysis, shooting pain or tingling in the neck, back or abdomen, loss of bowel and
bladder control, and prolonged nausea, vomiting or hiccups. These attacks may have
periods of remission and relapse, with relapses most commonly occurring years to months
apart. Monophasic NMO is a less common variant of NMOSD, and is characterized by a
single, severe attack that may last days or weeks. When a patient is first diagnosed with
NMOSD, it is unclear whether or not they will have relapses. The psychological burden of
NMOSD is documented in the empirical literature, with a focus on experience of
depression, pain, sexual dysfunction, sleep issues, stigma, and impact on partners.
Between 30% and 50% of individuals with NMOSD have been found to endorse clinically
significant depressive symptoms on the Beck Depression Inventory. Compared with patients
with Multiple Sclerosis (MS), NMOSD patients are twice as likely to receive a formal
diagnosis of Recurrent Major Depressive Disorder.
Pain, bowel and bladder dysfunction, visual impairment, reduced sexual function, and
inability to work, were found to most negatively impact emotional well-being and quality
of life among people with NMOSD. It is estimated that chronic pain affects over 80% of
NMOSD patients, including patients without recent relapse still commonly reporting
moderate or severe pain. Pain severity is the strongest negative predictor of quality of
life, and the most common symptom of concern voiced by patients to their physician.
Sexual dysfunction, specifically reduced libido, decreased orgasm and erectile
dysfunction have been reported by 75% of males and 75% of females living with NMOSD.
Sleep disturbance is common in NMOSD, with 64% of NMOSD patients identified as poor
sleepers, with correlations with anxiety, depression, pain, disability, and disease
duration. Similarly, 71.4% of NMOSD patients endorsed fatigue, with correlations with
sleep disturbance, depression, pain, and quality of life.
Further contributing to the psychological burden of life with NMOSD, 60% of patients
reported being affected by NMOSD-related stigma. Embarrassment due to physical
limitations, perceived exclusion, avoidance/ostracism, and blame for their illness were
deemed the most impactful targets of stigma.
The psychological burden is also well-defined by patients with NMOSD and their loved ones
in online forums, blogs, and social media. In addition to the domains cited in the
literature, patients and loved ones often discuss anxiety, delayed diagnosis, physical
disability and impact on relationships as hallmark characteristics of life with NMOSD.
Despite the many studies suggesting that life with NMOSD is marred by many psychological
stressors, including increased depression, there does not appear to be any psychosocial
intervention to date to help patients and loved ones cope with this burden. Furthermore,
narratives from patients across media describe profound psychological burdens that go
untreated.
Given the prevalence of depression, stress, stigma, and physical impediments associated
with NMOSD, it makes sense that patients and their loved ones would resort to
avoidance-based coping (distraction with television, avoiding talking about illness,
avoiding reminders of illness) to manage these issues. Compared with patients with
multiple sclerosis, and with healthy controls, patients with NMOSD were more likely to
use mental disengagement strategies, while both NMOSD and MS patients were more likely to
use acceptance and behavioral disengagement strategies, compared with healthy controls.
However, while avoidance-based coping may provide an effective short-term escape from the
psychological burden of NMOSD, it is considered a maladaptive coping strategy in the
longer term, as avoidance can be associated with medication nonadherence, missing clinic
visits, failure to inform medical providers about symptoms, and can be associated with a
paradoxical increase in depression and anxiety.
While the economic burden of NMOSD, including financial cost of treatments, loss of
income due to disability, and associated financial pressure on caregiver/loved ones is
well known, the psychological impact of NMOSD on caregivers and loved ones remains
under-studied to date. Based on PHQ-9 score, 21.1% of loved ones of NMOSD patients were
found to be experiencing mild, moderate, or moderately severe depressive symptoms.
However, partners did not endorse clinically significant "burden", anxiety, or
depression. Rather, partners endorsed pressure to take on new roles both inside and
outside of the home, during NMOSD relapses, with both male and female partners
identifying challenges related to gender role shift.
Partners endorsed limiting hobbies and activities to prioritize the patient's health,
particularly during a relapse, along with frustration about accommodating NMOSD symptoms
in public spaces, helplessness that they cannot "fix" their partner's problems, isolation
due to lack of support, and anxiety about their partner's well-being and about their own
health. Male partners reported "hardly expressing thoughts and feelings about NMOSD", and
reported that prior to study participation, they had "never been asked how they feel
about NMOSD." Lack of caregiver support and avoidance-based coping are associated with
caregiver strain and burnout. Given the physical impediments associated with NMOSD,
caregivers are often responsible for managing the patient's medication regimen and
transporting patients to their infusion appointments. Caregiver burnout can
understandably negatively impact patient treatment engagement.
Therefore, the investigators propose a caregiver-assisted, NMOSD-specific mental health
intervention. Given physical limitations associated with NMOSD, and increased comfort
with telehealth over the past few years, the investigators propose a telehealth-delivered
intervention will be most accessible and effective. Acceptance and Commitment Therapy is
one potential intervention for reducing internalizing symptoms, increasing purpose in
life, and reducing avoidance-based coping among people with NMOSD and their caregiver
loved ones. ACT is a "third wave" behavioral therapy which balances encouraging life
changes in the service of one's values (purpose in life) with a strong acceptance
component. This novel, experiential, contextual talk therapy is an empirically supported
treatment for anxiety and depression, substance use, and has proven successful in
managing chronic pain, somatic problems, HIV, Pancreatic Cancer and Cystic Fibrosis.