Dataset Information

Patient and caregiver experiences of living with acute hepatic porphyria in the UK: a mixed-methods study.

ABSTRACT:

Background

This study used quantitative and qualitative research methods to analyze how acute hepatic porphyria (AHP) affects patients with varying annualized porphyria attack rates. The overall impact of AHP on patients and caregivers, including their quality of life, was explored. The nature and treatment of acute attacks, experiences of long-term heme arginate treatment and access to other appropriate treatment, and the extent of and treatment for chronic symptoms were also investigated within this study.

Methods

Patient and caregiver data were collected via an online survey of members of the British Porphyria Association, followed by an optional 1-h telephone interview.

Results

Thirty-eight patients and 10 caregivers responded to the survey. Of those, 10 patients and three caregivers completed follow-up interviews. Overall, 19 patients (50%) had experienced an acute attack within the previous 2 years, and the severity and types of symptoms experienced during or between acute attacks varied considerably. There were no clear definitions among patients for 'mild' or 'severe' attacks. Treatments and treatment settings used to manage attacks also varied. Following unsatisfactory care experiences at hospitals, some patients reported avoiding further hospital services for later attacks. Therefore, using settings of care as a measure of attack severity should be avoided. Ninety-four percent of patients also experienced chronic symptoms, which were as varied as acute attacks. Pain was the predominant chronic symptom and was managed with opioids in severe cases. Regardless of AAR, porphyria heavily impacted the daily lives of patients and caregivers. Although patients experiencing frequent attacks generally endured a greater impact on their daily life, patients with less frequent attacks also experienced impacts on all domains (social, leisure activities, relationship with family, relationships, psychological wellbeing, finances, employment, and study). Caregivers were most affected in the finance, relationships with family, and employment domains, and just over half of the caregivers reported a moderate impact on their psychological wellbeing.

Conclusions/implications

The burden of illness with AHP is high across all patients, regardless of frequency of attacks, and AHP negatively affects patients and caregivers alike.

SUBMITTER: Gill L

PROVIDER: S-EPMC8074407 | biostudies-literature |

REPOSITORIES: biostudies-literature

ACCESS DATA

Similar Datasets

Project description:IntroductionAwareness of epidemiological and clinical consequences of atrial fibrillation (AF) has increased, as have disease-related costs. Less attention has been paid to patient-related issues, such as understanding how symptoms, different therapies, and lifestyle adjustments affect daily life. We aimed to describe patients' experiences of living with AF.MethodsThe study design used a parallel convergent mixed methods approach. Patients with AF were included in the SMURF study and referred for catheter ablation. Patients completed questionnaires on symptoms, health-related quality of life, depression, anxiety, and perceived control and were interviewed. The datasets were analysed separately using inductive content analysis and descriptive statistics. Data were merged to obtain a final interpretation.ResultsNineteen patients were interviewed and 18 completed questionnaires. Twelve of the patients were male, mean age 60 years (45-75 years). Inductive qualitative analysis revealed three categories: (i) symptoms and concerns limiting life, (ii) dimensions of worries, and (iii) strategies for management. The most common symptoms were tiredness, weakness/fatigue, and breathlessness during activities, and the most pronounced negative impacts on health-related quality of life (HRQOL) were physically related, shown in the ASTA questionnaire. The most negative SF-36 scores were found in role limitations due to physical health problems and vitality. HADS revealed five patients with some degree of anxiety and four with some degree of depression. Patients had lower scores on perceived control than perceived helplessness in CAS. Patients' perceived control was higher than their families', and families experienced more helplessness.ConclusionsThe mixed methods design deepens our understanding of challenges faced by patients. Patients experienced a limited ability to perform activities of daily living due to AF which created different kinds of worries that encouraged the use of various strategies to manage their lives. Healthcare providers need to be aware that relationships between patients and their relatives can change, and therefore they need to be supported and integrated into the care system.

Project description:Background In an acute hospital setting, diabetes can require intensive management with medication modification, monitoring and education. Yet little is known about the experiences and perspectives of nursing/midwifery staff and patients. The aim of this study was to investigate diabetes management and care for patients with diabetes in an acute care setting from the perspectives of nursing/midwifery staff and patients. Methods A convergent mixed-methods study design. Patients with diabetes (Type 1, Type 2 or gestational diabetes) recruited from a public health service in Melbourne, Australia completed a survey and nurses and midwives employed at the health service participated in focus groups. Descriptive statistics were used to summarise the survey data. Thematic analysis was used for the free-text survey comments and focus group data. Results Surveys were completed by 151 patients. Although more than half of the patients were satisfied with the diabetes care they had received (n = 96, 67.6%), about a third felt the hospital nursing/midwifery staff had ignored their own knowledge of their diabetes care and management (n = 43, 30.8%). Few reported having discussed their diabetes management with the nursing/midwifery staff whilst in hospital (n = 47, 32.6%) or thought the nurses and midwives had a good understanding of different types of insulin (n = 43, 30.1%) and their administration (n = 47, 33.3%). Patients also reported food related barriers to their diabetes management including difficulties accessing appropriate snacks and drinks (n = 46, 30.5%), restricted food choices and timing of meals (n = 41, 27.2%). Fourteen nurses and midwives participated in two focus groups. Two main themes were identified across both groups: 1. challenges caring for patients with diabetes; and 2. lack of confidence and knowledge about diabetes management. Conclusions Patients and nursing/midwifery staff reported challenges managing patients’ diabetes in the hospital setting, ensuring patients’ optimal self-management, and provision of suitable food and timing of meals. It is essential to involve patients in their diabetes care and provide regular and up-to-date training and resources for nursing/midwifery staff to ensure safe and high-quality inpatient diabetes care and improve patient and staff satisfaction. Supplementary Information The online version contains supplementary material available at 10.1186/s12912-022-01022-w.

Project description:BackgroundDuring the early stages of the COVID-19 pandemic, concerns were raised about reduced attendance at hospitals, particularly in paediatric emergency departments, which could result in preventable poorer outcomes and late presentations among children requiring emergency care. We aimed to investigate the impact of COVID-19 on health-seeking behaviour and decision-making processes of caregivers presenting to paediatric emergency services at a National Health Service (NHS) Trust in London.Materials and methodsWe conducted a mixed-methods study (survey and semi-structured interviews) across two hospital sites between November-December 2020. Data from each study were collected concurrently followed by data comparison.ResultsOverall, 100 caregivers participated in our study; 80 completed the survey only, two completed the interview only and 18 completed both. Our quantitative study found that almost two-thirds (63%, n = 62) of caregivers attended the department within two days of their child becoming ill. Our qualitative study identified three major themes which were underpinned by concepts of trust, safety and uncertainty and were assessed in relation to the temporal nature of the pandemic and the caregivers' journey to care. We found most caregivers balanced their concerns of COVID-19 and a perceived "overwhelmed" NHS by speaking to trusted sources, predominantly general practitioners (GPs).ConclusionCaregivers have adapted their health-seeking behaviour throughout the pandemic as new information and guidance have been released. We identified several factors affecting decisions to attend; some existed before the pandemic (e.g., concerns for child's health) whilst others were due to the pandemic (e.g., perceived risks of transmission when accessing healthcare services). We recommend trusted medical professionals, particularly GPs, continue to provide reassurance to caregivers to seek emergency paediatric care when required. Communicating the hospital safety procedures and the importance of early intervention to caregivers could additionally provide reassurance to those concerned about the risks of accessing the hospital environment.

Project description:IntroductionAcute hepatic porphyria (AHP) is a family of rare metabolic diseases characterized by potentially life-threatening acute attacks and, in some patients, chronic debilitating symptoms. While patients with frequent or recurrent attacks (three or more attacks annually) are known to have reduced health-related quality of life (HRQoL) as most aspects of daily living are impacted, limited data exist in patients with sporadic attacks. This research aims to identify porphyria-related symptoms between attacks, characterize the frequency, severity, and bothersomeness of these symptoms, and more generally understand the burden of this disease in patients who experience attacks sporadically.MethodsPatients with AHP with sporadic attacks (AHP-SA) (at least one porphyria attack in the past 2 years, but no more than two attacks per year in the previous 2 years) were recruited, via outreach performed by patient advocacy groups, for participation in qualitative telephone interviews. Interviews were conducted using a semi-structured guide and were audio-recorded, transcribed, anonymized, coded, and analyzed to determine if saturation was reached.ResultsA total of 14 participants with AHP-SA were interviewed (mean age 45 years, 100% female). The most frequently reported chronic symptoms were fatigue, pain, heartburn, and constipation. The most frequently experienced chronic impacts were difficulty performing daily activities, difficulty exercising, negative impact on work, need for a special diet, anxiety, and depression. Beyond these chronic symptoms and impacts, participants also frequently described flares in their porphyria that were severe, did not qualify in their minds as an acute attack, but were nonetheless more severe than their typical chronic experience.ConclusionPatients with acute hepatic porphyria who experience sporadic attacks face significant chronic symptoms and impacts that frequently require significant pharmacological and clinical treatment. The reported severity of these symptoms and impacts suggests that the humanistic burden of AHP-SA is substantial and may lead to a significant decrease in health-related quality of life in these patients between acute attacks. The presence of flares that do not reach the level of what is considered an acute attack by patients is a unique finding of this study not reported elsewhere and requires additional investigation.