Accessibility to Occupational Therapy Services for Hereditary Transthyretin Amyloidosis

Abstract

This study was designed to investigate the global utilization of occupational therapy (OT) services by patients with hereditary transthyretin amyloidosis (ATTRv) in Spain. The main objective was to find out whether these patients have access to OT services and the types of interventions being offered to them, together with their satisfaction and real benefits as users. We developed an online questionnaire which was distributed to patients with ATTRv in Spain through patient associations. Seventy-four patients with a diagnosis of ATTRv residing in Spain participated in the study. Thirteen had already used OT services at least once, felt that OT interventions improved their quality of life, would recommend OT services to others, and would return to see an occupational therapist. However, 61 had never used this type of service before. Of these, 35 knew what OT is and 13 declared that they considered that OT interventions in ATTRv could be positive for them. The results suggest that the use of OT services by ATTRv patients is low, mainly because of the lack of information about the occupational profile of individuals with this disease. The low response rate obtained for the survey limits generalization, and thus further research to confirm these preliminary findings is needed.

Establishing Occupational Therapy Needs: A Semi-Structured Interview with Hereditary Transthyretin Amyloidosis Patients

“…Accordingly, diagnosis of ATTRv is likely to represent a challenging situation for occupational therapists whose profession is focused on the promotion of health and well-being through occupational interventions [ 13 ]. In line with our findings, other studies that have focused on the psychosocial impact of ATTRv suggest that the most significant impact is essentially on the quality of life and patients’ mental health once the symptoms become noticeable [ 11 ]. However, there is no relevant information available on the role of the occupational therapist in ATTRv and the usefulness of conducting a semi-structured interview [ 11 ].…”

“…The symptomatology of this rare disease is variable among patients from different geographical areas, but the most severe presentations are debilitating and life-threatening and are associated with physical, psychological, occupational, and social symptoms. ATTRv is usually characterized by neuropathic symptoms mainly related to peripheral damage—at both sensory and motor levels—and autonomic neuropathy [ 11 , 12 ]. Gastrointestinal impairment, cardiomyopathy, ocular symptoms resulting from amyloid deposition in the eye, and nephropathy frequently appear in association with neurological impairment [ 8 ].…”

“…Gastrointestinal impairment, cardiomyopathy, ocular symptoms resulting from amyloid deposition in the eye, and nephropathy frequently appear in association with neurological impairment [ 8 ]. Symptoms are progressive and disabling throughout the four main polyneuropathy stages of the disease (I, II, III, IV), with severe impairment in the IV stage [ 10 , 11 ]. The first phase is characterized by mild autonomic dysfunction and sensory impairment in the lower limbs.…”

“…The first phase is characterized by mild autonomic dysfunction and sensory impairment in the lower limbs. In the second phase, as a progressive disease, with autonomic dysfunction and sensory-motor impairment of the upper limbs, and in the third phase, dysfunction is severe, and even complete paralysis may develop in the fourth phase [ 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 ].…”

“…The limitations in daily activities faced by ATTRv patients in all stages of the disease—although to a greater extent in advanced stages (III, IV)—have been described as difficulties impairing personal independence in self-care skills and self-determination [ 12 ]. An individual’s occupational performance is essential for a balanced daily life [ 12 , 13 , 14 , 15 , 16 ]; by developing specific interventions for these patients, occupational therapy (OT) can play an important role in reducing activity limitations, improving personal autonomy, empowering patients to overcome disease-related barriers and constitutes a pioneer project, due to the fact that up to now no psychosocial interventions have been carried out on ATTRv patients [ 11 ]. OT can intervene using specific techniques and programs that help develop, maintain, or improve the performance of daily living activities in patients with a diagnosis of the disease by compensating for dysfunctions and promoting occupational health and general well-being [ 13 , 14 , 15 , 16 , 17 ].…”