The Experience of Living With Retinitis Pigmentosa: An In-depth Exploration of Patients’ Challenges

Abstract

Background: Retinitis Pigmentosa (RP) is a genetic disease that causes irreversible progressive impairment of vision and eventual blindness due to the formation of pigment cells in the retina. Therefore, this disease is one of the problems that can reduce the abilities of patients in different dimensions and cause various injuries in different dimensions of physical, mental, psychological, social and economic for these people due to visual dysfunction. Hence, this disease threatens the health of these patients in every way. Given the genetic and incurable nature of this disease, it is important to examine the health challenges of Retinitis Pigmentosa patients. Therefore, this study was conducted to explain the health concerns of patients with Retinitis Pigmentosa. Retinitis Pigmentosa is a genetic disease that causes irreversible progressive impairment of vision and eventual blindness due to the deposition of retinal pigment cells. Therefore, this disease is one of the problems due to impaired visual function that can reduce the capabilities of patients in various dimensions and cause them various injuries in various physical, mental, psychological, social and economic dimensions. Therefore, this disease always threatens the health of these people in every way. Due to the genetic nature and incurability of this disease, it is important to investigate the health challenges of RP patients, so this study was conducted to explain the health concerns of RP patients. Progressive visual impairment is one of the problems that threatening the health of patients and has the negative effects on their abilities. This study aimed to explore the health concerns of patients with Retinitis Pigmentosa (RP). Methods: The present study was an inductive qualitative method that was performed with the conventional content analysis approach. The study was conducted from March 2016 to March 2017 and 11 patients with non-syndromic Retinitis Pigmentosa participated. The study was conducted at the Retinitis Pigmentosa Patient Support Institute located in Tehran, Iran. Participants were selected by purposive sampling. Data were collected through semi-structured face-to-face interviews. Twelve interviews (one person was interviewed twice) were conducted for at least 30 minutes to 120 minutes ) 70 minutes on average). Data collection continued until data saturation. Data were analyzed using conventional content analysis; so that based on the technique of continuous comparison, subcategories were composed of each category of similar code. Then, by comparing all the subcategories with each other, new categories were created from each category of similar categories. As the comparison progressed, all similar categories came together to form larger categories. MAXQDA software version 2018 was used to organize the findings. Rigor of data was evaluated based on four criteria: credibility, dependability, confirmability and transferability. Results: The main category of "Attrition of personal and social abilities on the dim road of life with RP" was extracted as the main category of study findings. This category means the gradual and progressive damage and destruction of force, mastery and capacity in all physical, mental, psychological and social dimensions of the patient and consequently the need for various individual supports in society. This phenomenon creates progressive visual weakness. This category consisted of two subcategories "perceived health threat" and "perceived support needs". The subcategory "perceived health threat" means the weakening of the condition and damaged abilities in various physical, mental, psychological and social dimensions, which the person has perceived; this subcategory was derived from the sub-subcategories "gradual decline in physical function", " Sustained fluctuations in mental health" and "The weakening of social capability". In addition, the subcategory of "perceived support needs", which means feeling the need for various supports from others following the limitations caused by the disease, were extracted from the sub-subcategories of "need for socio-economic support", "need for educational support”, "need for psychological support" and "need for a personal assistant ". Conclusions: Retinitis Pigmentosa Patients face with the problem of abilities impairment in various physical, mental, and social dimensions and need for support in society and need to receive comprehensive support from different social groups. Therefore, progressive visual impairment always negatively affects the independence and health of Retinitis Pigmentosa patients on a large scale. Therefore, in order to facilitate the management of various problems, maintain their independence, and promote their health, it is necessary to formulate a context-based wholistic care program through multidisciplinary cooperation by identifying their health needs and challenges. Achieving this goal requires more cooperation between different fields of health such as medicine, nursing, rehabilitation, psychology, and social worker. Keywords: Retinitis Pigmentosa, Visual impairment, Health, Care, Qualitative research