Introduction

Chronic conditions, including communicable and noncommunicable diseases, certain mental disorders, and ongoing physical impairments, constitute persistent health problems that require some degree of healthcare management. Individuals with chronic conditions need to learn how to self-manage the effects of their illness over the long term [1], using strategies that must be incorporated into their daily routine. To assume new responsibilities related to the self-management of chronic conditions, individuals must actively partner with healthcare professionals [2].

HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) is a spinal spastic paraparesis that is caused by a human T-lymphotropic virus type 1 infection. Osame et al. proposed HAM as a new clinical entity in Japan [3], and Gessain et al. demonstrated HTLV-1 seropositivity of patients with TSP in the Caribbean [4]. The results of additional studies confirmed that HAM and TSP were the same clinical entity and, in 1988, the World Health Organization named this condition HAM/TSP [5]. HTLV-1 is transmitted by breastfeeding, sexual intercourse, blood transfusion, and needle sharing; approximately 10 million people worldwide are HTLV-1 carriers, of whom between 0.1% and 2% have HAM/TSP [6,7]. In Japan, approximately 3000 individuals have been diagnosed with HAM/TSP. Typical symptoms of HAM/TSP include chronic spastic paraparesis-induced gait disorders, weakness of the lower limbs, bladder disturbance, constipation, and sensory symptoms, such as tingling, pins and needles, and burning [8]. Treatment is available for the reduction of spinal inflammation caused by corticosteroids and interferon-α; however, there is currently no curative treatment for HTLV-1 itself [7]. For symptoms such as lower-limb spasticity and bladder dysfunction, symptomatic treatment with drugs is administered. Without no established curative therapeutic strategy, individuals with severe HAM/TSP must change not only their daily behaviors but also their entire way of life. Our previous study found that people with HAM/TSP experienced a “feeling of loss of self-control” over their body, HAM/TSP as a disease, and their future during the progression of symptoms. It is important to support the attempts of these individuals to manage the problems caused by HAM/TSP as a chronic condition and provide an opportunity for self-management of persistent symptoms as this may help them find positive meaning in their illness and improve their quality of life.

Self-management has gained importance in chronic illness care [9] and is defined as “learning and practicing skills necessary to carry on an active and emotionally satisfying life in the face of a chronic condition” [10]. Self-management science has developed partly because acute care models do not satisfy the needs of people with chronic conditions [1]. Research has already described and evaluated self-management interventions in various areas and confirmed the effectiveness of self-management programs in participants who significantly improved their self-management behaviors [11]. The self-management approach is effective for supporting not only lifestyle-related diseases but also progressive neurological disorders, and the Taxonomy of Everyday Self-management Strategies (TEDSS) was developed as one of the few self-management frameworks from the perspective of people living with neurological conditions [12]. The most common neurological disease studied in the context of self-management is multiple sclerosis (MS) [13], and a systematic review showed evidence that supports the value of programs designed to promote self-management of MS and other neurological diseases [14]. Considering the common symptoms, uncertainty of disease course, and progressive disability, research on self-management in MS is very informative for individuals with HAM/TSP; therefore, we reviewed published studies on self-management of MS to explore the significance and potential of self-management for people with HAM/TSP [15]. The results showed that a detailed investigation into self-management of people with HAM/TSP from the patient’s perspective is required to develop effective self-management interventions that reflect the often unique nature of the disease for each individual.

According to Lorig and Holman, although many concerns are shared across different diseases, behaviors, and populations, there are intergroup and interindividual differences in the needs [11]. The best self-management support for people with neurological disorders should go beyond standard advice and must be customized to each individual’s reality, life context, and disease trajectory [16]. People with HAM/TSP have unique experiences owing to their complex backgrounds; furthermore, the number of patients is small, there is no cure, the disease is infectious, and it can manifest with diverse symptoms. Specifically, HAM/TSP is not socially recognized and is likely to be misunderstood because of the interindividual variations in symptoms. Moreover, because HAM/TSP is an infectious disease, stigma toward viral infections may arise. A recent study about the lived experience of people with HAM/TSP in Iran found that they had “reduced self-sufficiency and social dignity,” which included three main categories “disruption of desirable personal and social life,” “reduced perception of role competencies,” and “obligatory unpleasant lifestyle changes” [17]. However, to the best of our knowledge, no research on the self-management of patient with HAM/TSP in Japan has been reported thus far. There is limited information regarding the process by which individuals with HAM/TSP self-manage their chronic health condition. Consequently, nursing care may not reflect the opinions of the patients with HAM/TSP. Therefore, the investigation of self-management among people with HAM/TSP is a priority. Accordingly, this qualitative study aimed to explore the self-management strategies of individuals with HAM/TSP in Japan.

Methods

Results

Seven people with HAM/TSP who consented to participate in the study were interviewed once each, and each interview lasted 58–134 (average, 80) min. No participant required a second interview. After the interviews, three participants returned the open-ended questionnaire; thus, the descriptions were treated as data. Of the seven participants, four were male and three were female, and the median age of the cohort was 71 (IQR, 66.5–73) years. Here, participants were recruited directly through the HAM/TSP patient association, and thus, we were unable to access participants’ medical records. Therefore, the diagnostic process leading to the HAM/TSP diagnosis, including any past or ongoing treatments, were confirmed through interviews with the participants themselves. The participants’ diagnosis of HAM/TSP was confirmed during interviews based on their statements that they had undergone cerebrospinal fluid testing for diagnosis, had applied to the government for medical care of patients with intractable diseases as HAM/TSP patients, and had participated in HAM/TSP clinical trials. The median duration since HAM/TSP diagnosis was 15 (IQR, 13–18) years. All but one participant had received corticosteroid or interferon-α therapy. Two participants were taking muscle relaxants such as baclofen, one participant was receiving drug therapy for urinary frequency, and two participants were taking alpha-1 blockers for voiding symptoms. The Osame Motor Disability Score (OMDS) [20], which assesses motor disability in HAM/TSP from 0 (normal walking and running) to 13 (complete bedridden), ranged from 4 (need support while using stairs) to 10 (crawls with hands), and four participants with OMDS scores of 6 or higher used a wheelchair for mobility. Six participants had bladder dysfunction and one required intermittent self-catheterization. In addition, six participants each had bowel dysfunction and sensory disturbance. To ensure confidentiality and prevent patient identification, the individual characteristics of the participants (Participant A to Participant G) will not be published.

From an analysis of the data, we identified 6 core categories, 14 categories, and 39 subcategories as self-management strategies that involved activities to manage their chronic conditions caused by HAM/TSP (Table 1). The six core categories were: “Attempting to maintain body functions”, “Acquiring new ways to complement body functions”, “Engaging to maintain an independent daily life”, “Ensuring a specialized treatment environment for HAM/TSP”, “Taking control of HAM/TSP”, and “Moving forward as people with HAM/TSP.” Each core categories, categories and subcategories are explained briefly with illustrative quotations from the study participants below.

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Table 1. Core Categories, Categories and Subcategories.

https://doi.org/10.1371/journal.pntd.0014055.t001

Discussion

In the present study, we found 6 strategies for managing the chronic conditions caused by HAM/TSP. These strategies address the needs of patients with HAM/TSP to self-manage their unique chronic conditions. In an overview of the 6 core categories and 14 categories describing self-management strategies, three characteristics of HAM/TSP – lack of an established curative treatment, low social awareness of the disease, and its infectious nature – had a significant impact on the self-management of individuals with HAM/TSP.

Medication management is an important component of self-management [13]. In neurological disorders, taking medications is an important self-management strategy to follow the treatment regimen, and Audulv et al. have identified “Managing medication and treatment plan” as a sub-domain of the domain “Disease controlling strategies” in the self-management strategies of individuals with neurological conditions in the TEDSS [12,16]. However, for people with HAM/TSP who do not have an established curative treatment, medication adherence was not a priority self-management strategy of “Taking control of HAM/TSP.” Corticosteroids and IFN-α are the main treatments for HAM/TSP-induced inflammation in the spinal cord at the present stage [21]. All but one participant in this study received one of these therapies, although some participants chose not to continue treatment after receiving the treatment once. One participant regretted not receiving treatment during the early stages of HAM/TSP after the diagnosis. As treatments may require hospitalization and potentially have side effects, the decision to receive medication is a consideration for people with HAM/TSP. “Self-determination regarding treatment,” under the core category of “Taking control of HAM/TSP,” has become a self-management strategy for individuals with HAM/TSP in the context of their medical condition and social situation. Regarding medication management, two participants were taking muscle relaxants for lower-limb spasticity, and three participants were taking medications such as alpha-1 blockers for urinary dysfunction. However, the study results did not identify the use of these medications as a self-management strategy. For participants, maintaining motor function through rehabilitation or finding their own ways to manage residual urine or urinary incontinence may have been readily recognizable self-management strategies. However, it cannot be denied that taking muscle relaxants and medication for urinary dysfunction creates a certain stable state that helps maintain walking and urinary functions. Therefore, we consider it necessary to explore self-management strategies thoroughly for medication management regarding symptomatic treatment going forward.

In addition, individuals with HAM/TSP engaged in the self-management strategy of “Ensuring a specialized treatment environment for HAM/TSP,” which may be a characteristic type of “Disease controlling strategy” for people with HAM/TSP for whom no curative treatment has been established. In the absence of a cure, it is important to ensure that the treatment environment for people with HAM involves not only “Ensuring medical treatment by HAM/TSP specialist” but also “Supporting research on the treatment of HAM/TSP.” Besides the strategy of “Taking control of HAM/TSP,” the identification of a strategy for “Ensuring a specialized treatment environment for HAM/TSP” is considered one of the characteristics of self-management among people with HAM/TSP.

In the results of this present study, “Attempting to maintain body functions,” and “Acquiring new ways to complement body functions” were important self-management strategies for HAM/TSP-affected body functions. For people with HAM/TSP for whom no curative treatment has been established, the “Management and prevention of symptoms and complications,” which was a sub-domain of “Disease controlling strategies” in the TEDSS [12,16], constitutes an important self-management strategy. Individuals with HAM/TSP achieved prevention, control, and limitation of symptoms, complications, and disease progression through self-management strategies, such as controlling impaired excretory function in unique ways, introducing self-catheterization to prevent urinary tract infection owing to residual urine retention, and training to maintain motor function. Furthermore, individuals with HAM/TSP attempted to control the disease’s impact on daily life and maintain their previous routines through a self-management strategy of “Engaging to maintain an independent daily life alongside the “Taking control of HAM/TSP” strategy. The participants attempted to become agents of their own control over the physical functions, symptoms, and daily lives that are affected by HAM/TSP. Bishop et al. found that self-management is strongly associated with perceived control and that both perceived control and self-management mediate the relationship between the impact of MS and the subjective quality of life among individuals with MS [22]. Thus, enabling people with HAM to manage and prevent symptoms and complications, accompanied by the perception that they are in control of the effects of HAM/TSP, may be an important self-management strategy that affects their subjective quality of life.

The lack of public awareness of HAM/TSP necessitates the incorporation of self-management strategies right from the time that an individual is diagnosed with HAM/TSP. For a common disease, a diagnosis provides answers to the current and future conditions. On the one hand, for people with HAM/TSP, strategies are needed to understand the situation caused by the unfamiliar with HAM/TSP. “Acting to understand HAM/TSP” within the core category “Taking control of HAM/TSP” would be characterized as a preparatory and proactive strategy for people with HAM/TSP to confront their potential chronic conditions and make decisions about how they will cope with their HAM/TSP in the future. On the other hand, the lack of awareness of HAM/TSP is similar in medical settings, and “Ensuring a specialized treatment environment for HAM/TSP” is an important self-management strategy for people with HAM/TSP. Coler-Reilly et al. conducted an epidemiological study of HAM/TSP in Japan by using a registry system and found that the median duration from onset to diagnosis of HAM/TSP was 5 (range, 1–12) years [23]. The limited awareness of HAM/TSP among general physicians in HTLV-1-non-endemic areas in Japan makes it difficult for individuals with HAM/TSP to reliably receive an accurate diagnosis. Furthermore, Zihlmann et al. pointed out that primary healthcare teams are generally unaware of HTLV-1 and do not recognize the health problems associated with the infection and consequent disease; this gap creates uncertainty regarding the medical knowledge of the team [24]. Early diagnosis and medical treatment by HAM/TSP specialists are indispensable for patients with HAM/TSP, as it affects disease progression and prognosis. Therefore, “Ensuring a specialized treatment environment for HAM/TSP” can be an important self-management strategy that also affects “Attempting to maintain body functions,” “Engaging to maintain an independent daily life,” and “Taking control of HAM/TSP.” The Comprehensive Measures for HTLV-1 was endorsed by the Japanese government in 2010 and the medical system in Japan is relatively well developed [25]. However, as participants in this study mentioned, survey results indicate that people with HAM/TSP are forced to bear the burden of receiving highly specialized and effective treatment, such as commuting several hours to see a specialist [26]. “Ensuring a specialized treatment environment for HAM/TSP” is an essential self-management strategy for people with HAM/TSP; however, there are limits to what individuals with HAM/TSP can manage on their own, and social challenges remain.

Furthermore, the infectious nature of HAM/TSP affects the self-management strategies. Individuals with HAM/TSP must consider the effects of HTLV-1 not only on themselves but also on their close relatives. This self-management strategy is specific to people with HAM/TSP and is not found in other neurological disorders. The results of the present study identified as a category of “Concern about the impact of HTLV-1 infection on close relatives” within the core category “Taking control of HAM/TSP.” One participant was concerned not only about transmission to close relatives, but also that if a close relative was an HTLV-1 carrier, he or she could spread HTLV-1 infection through vertical transmission, breastfeeding, and sexual intercourse, or limit those activities. This characteristic self-management strategy of people with HAM/TSP may include two intentions. The first is the proactive intention to prevent further spread of HTLV-1 infection through infection prevention actions. Preventing the spread of HTLV-1 reduces the risk of HAM/TSP and other related diseases for close relative. The second aim was to minimize misconceptions about HTLV-1 infection and its associated impact on relationships with others and social life. Zihlmann et al. found that many of their HTLV-1-infected participants tended to believe that their disease was an HIV infection or were in denial that they had HTLV-1, and that fear of exposing their infection and the destruction of family relationships that a diagnosis of HTLV-1 might cause led them to treat their diagnosis as a “secret” [24]. Davoudi et al. found that people with HAM/TSP, when they came to know of their HTLV-1 infection, experienced an impact on their desirable personal and social lives, as the fear of spreading the virus prevents them from continuing their marital lives and may even lead to divorce [17]. Although some of the participants’ narratives in the present study expressed concern about the roots of their HAM/TSP, exploring the roots of their HTLV-1 infection can cause family discord and confer a psychological burden for people with HAM/TSP if there is no proper understanding of HTLV-1 infection. Thus, HAM/TSP is prone to social stigma owing to low social awareness of the disease and the fact that it is an infectious disease; thus, self-management strategies are required to minimize this impact. Within the core category “Engaging to maintain an independent daily life” of self-management strategies identified in this study for people with HAM/TSP, “Receiving the required support” may be difficult for people with HAM/TSP to openly receive the support that they need in a society where HAM/TSP is stigmatized and precludes openness about suffering from HAM/TSP; this may affect the social and psychological self-management strategy “Moving forward as people with HAM/TSP.” In Japan, the public initiatives of Comprehensive Measures for HTLV-1 include infection-prevention measures, such as the inclusion of HTLV-1 antibody testing in antenatal pregnancy screening, and measures to increase awareness and provide information, such as the website of the Ministry of Health, Labor for providing information on HTLV-1 infection and associated diseases, and published materials to increase public awareness [26]. Although measures at the national and municipal levels are being expanded, the concerns and hardships for individuals with HAM/TSP remain endless.

Finally, we suggest care approaches that we consider useful for patients with HAM/TSP. Nurses will need to implement personalized educational sessions to help individuals with HAM/TSP develop the skills to self-manage the impact of HAM/TSP on their body functions, and thereby enable them to experience control over their HAM/TSP. We believe that the supportive role of nurses could help patients with HAM/TSP recognize that their disease is under their control and enable the identification of individualized strategies for symptom management and prevention of complications. Moreover, nurses may need to undertake self-management advocacy to help patients with HAM/TSP fully understand and make decisions regarding their treatment and participation in clinical trials, including understanding their personal preferences, coordinating with their physicians, and providing the information necessary for decision-making. Furthermore, we believe that the self-management strategy of “Concern of the impact of HTLV-1 infection on close relatives” within the core category “Taking control of HAM/TSP” should never be left to the patient alone. Nurses may need to play a role not only in providing more accessible support to people with HAM/TSP and those around them, such as guidance and counseling on understanding HTLV-1 and imparting information on correct infection-control measures, but also in national policies from the nurses’ perspective, ensuring that the dignity of people with HAM/TSP is preserved.

Limitations

An important limitation of this study was that, owing to the background of recruiting participants from HAM/TSP patient associations and the small sample size, the study’s results may not describe all aspects of self-management in people with HAM/TSP. We were forced to collect data from the recruited participants, and this made it difficult to conduct theoretical sampling. Although we were unable to increase the number of cases for saturation, we were able to identify an overview and variations in the self-management strategies that were analyzed for different cases, and we consider it worth reporting as a basic research finding in this field. Further studies are needed for a detailed exploration of each of the self-management strategies of HAM/TSP by expanding the survey to include those who were not members of the HAM/TSP patient association.

Conclusion

This study explored the self-management strategies of individuals with HAM/TSP in Japan. Consequently, 6 core categories were identified in self-management strategies for managing the chronic HAM/TSP-induced conditions. The self-management strategies of individuals with HAM/TSP had a significant impact from the 3 characteristics of HAM/TSP: lack of an established curative treatment, low social awareness of the disease, and its infectious nature. Personalized education to develop self-management skills in individuals with HAM/TSP and accessible support to prevent patients from struggling alone are needed.

Acknowledgments

We would like to thank all the participants in this study who shared their experiences with HAM/TSP and the HAM/TSP Patient Association.