Correction to: Medical and financial burden of acute intermittent porphyria

Neeleman, Rochus A.; Wagenmakers, M. A. E. M.; Koole-Lesuis, Rita H.; Mijnhout, G. Sophie; Wilson, J. H. P.; Friesema, Edith C. H.; Langendonk, Janneke G.

doi:10.1007/s10545-018-0216-x

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“…As a result, previous studies have stratified patients by frequency of attacks, known as the annualized attack rate (AAR) and defined as annualizing the number of AHP attacks over a specified time period. For instance, patients may be stratified as those with an AAR of either ≥ 3 or > 4 attacks per year (‘recurrent attacks’) and those with fewer or no attacks [ 11 – 13 ]. Those with recurrent attacks represent approximately 3–5% of the overall patient population [ 9 ].…”

Section: Introductionmentioning

confidence: 99%

“…Several studies have characterized the disease and disease burden of patients with AHP [ 2 , 3 , 7 , 11 – 14 ], with a focus on patients experiencing recurrent attacks [ 3 , 11 , 12 ]. Disease characteristics in patients having fewer attacks are less well understood [ 13 ]. This study used both quantitative and qualitative research methods to build on the existing research to analyze the effects of AHP on patients with different frequencies of AAR.…”

Section: Introductionmentioning

confidence: 99%

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

Gill

Burrell

Chamberlayne

et al. 2021

Orphanet J Rare Dis

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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.

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Section: Introductionmentioning

confidence: 99%