From TungArt2024 [Image], 2024, https://pixabay.com/illustrations/elderly-caregiver-support-9514821/. Pixabay CC BY 2.0
Background: Stiff-Person Syndrome (SPS) is a rare motor disease thought to be immunological in nature that presents with rigidity, spasms, and stiffness. This chronic illness affects the patient’s quality of life (QoL) with decreased mobility, social isolation, and depression and can be associated with other immunological diseases such as Type 1 diabetes. The goal of this study was to assess the impact of SPS on QoL in various facets.
Design and Participants: Due to the rare nature of this disease, patients were recruited through the hospital who diagnosed them and a self-help group. A survey was sent to their homes, asking them to complete and return it. Medications taken investigated. Patients were classified based on severity. QOL was assessed with a short form health survey (SF-36) and depression with the Beck Depression Inventory (BDI).
Results: Thirty-two patients were contacted with a 78.1% response rate. Twenty-one patients primarily had axial stiffness versus 3 with limbs affected; 17 were anti-GAD antibody positive. The 8 patients who did not participate did not differ in demographics. Diagnosis took years to obtain (3.0 +- 2.2 years). Fifty percent had Type 1 diabetes; 7/24 had other autoimmune diseases. There was no difference between the sexes related to limb involvement, anti-GAD antibodies, other autoimmune disease, age, duration of disease, or BDI score. While women scored higher on distribution of stiffness score, it was not statistically different. Only 7 of the 24 were employed, and all but 3 were married and lived with their families. Regarding QoL, respondents scored worse in physical and social functioning; scores were not affected by the duration of disease. Ten patients had mild to moderate depression; 4 had moderate to severe depression. BDI scores had a negative correlation with the SF-36 mental health score, physical limitation, vitality, social functioning, emotional role limitations, and general mental health – all of which were statistically significant.
Commentary: Chronic illness, especially ones like SPS that affect mobility and thus socialization and mental health, are often not recognized by the public. As providers, we may only appreciate the physical limitations for which these diseases are known. The true toll they take on our patients can only be appreciated when specifically assessing the impact on QoL. From a prolonged time to diagnosis to unknown future due to rarity, these diseases can affect our patients’ mental health, resulting in a higher rate of depression as well as anxiety and substance abuse as described in other studies. Not surprisingly, worse cases of SPS have a greater impact on the patient’s QoL and high risk of depression.
Bottom Line: Patients with SPS suffer from a decreased QoL and are at higher risk of depression. As the disease progresses, their mobility is significantly hindered, affecting their physical QoL by decreasing their socialization, increasing their isolation, and increasing their risk of depression. This small sample of SPS, a rare disease, demonstrates how chronic neurological diseases with motor symptoms truly affect patients and their QoL which makes them an ideal palliative care patient.
Source: Gerschlager W, Schrag A, Brown P. Quality of life in stiff-person syndrome. Mov Disord. 2002;17(5):1064-1067.
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