The Impact of the COVID-19 Pandemic on the Perception of Health and Treatment-Related Issues among Patients with Phenylketonuria in Poland-The Results of a National Online Survey.

Dariusz Walkowiak,Dorota Korycińska-Chaaban,Jarosław Walkowiak,Michał Patalan,Joanna Jagłowska,Joanna Zarębska,Renata Mozrzymas,Agnieszka Chrobot,Bożena Didycz,Ewa Starostecka,Bożena Mikołuć,Łukasz Kałużny

doi:10.3390/ijerph18126399

Abstract

There is agreement that the pandemic has affected the healthcare system and behaviour of patients. This study aims to identify problems encountered by patients with phenylketonuria (PKU) and their parents/caregivers during the six-week pandemic lockdown in Poland (15 March to 30 April 2020). To determine the factors that influenced health and treatment-related issues, as well as the respondents’ perception of the impact of the pandemic, study participants were asked to complete a non-validated online questionnaire comprising 31 questions (including 27 single-choice, two multiple-choice and two open-ended ones). A total of 571 patients or their parents completed the questionnaire, with 9.5% of respondents not performing any blood phenylalanine (Phe) test in the analysed period, 21.3% declaring a blood Phe increase, and 15.3% a decrease. Increased problems in contacting the doctor or dietitian were reported by 26.1% of subjects, whereas 39.3% of them felt restricted access to dietary products. Most (63.4%) participants were satisfied with remote contact with their PKU clinic. Better compliance was associated with higher odds of acceptance of remote contact and of reporting fewer problems with contacting the doctor, and with lower odds of missing Phe testing. Self-reported high stress was associated with higher odds of reporting the limited availability of low-Phe products and Phe-free formulas, as well as with increased Phe concentrations and non-PKU-related health problems. These patients also had poor dietary compliance and experienced more problems in contacting specialists. Health and treatment-related problems experienced during the pandemic lockdown were related to a higher intensity of stress in patient’s family and worse therapy compliance before the pandemic. Previous experience of remote visits resulted in a better perception of this method of contact. It seems that this form of communication should be popularized and improved to increase therapy effectiveness in case of different limitations in the future. Special attention should be paid to vulnerable patients who may be at extra risk when the provision of standard care is affected.

Highlights

When diagnosed by newborn screening and treated immediately, patients with phenylketonuria (PKU; OMIM 261600) essentially show normal development, neuropsychological deficits and behavioral, emotional and social issues have been reported [1,2,3]
The present study aimed to identify problems encountered by PKU patients and parents/caregivers during the six-week pandemic lockdown and determine the factors that influenced health and treatment-related issues in this group of patients
Phe levels within the recommended target for age; more than half stated that their results had not changed during the pandemic, according to over 20% of respondents, Phe blood test results worsened in this period

Summary

Introduction

When diagnosed by newborn screening and treated immediately, patients with phenylketonuria (PKU; OMIM 261600) essentially show normal development, neuropsychological deficits and behavioral, emotional and social issues have been reported [1,2,3]. The social burden associated with having a chronic disorder and strict dietary treatment, especially for patients with severe forms of PKU, could affect their healthrelated quality of life (HrQoL) [4]. Cotugno et al [5] found lower HrQoL for children and adolescents with PKU, while Landolt et al [6] reported impaired positive emotional functioning in a group of PKU children and adolescents. PKU population compared to the general population, for rates of depression and anxiety in the overall PKU population [7,8]. Jahja et al [9] reported that adolescent and adult PKU patients demonstrated poorer social cognition and had poorer social skills than controls, with a tendency towards lower or delayed autonomy [10] and emotional difficulties related to maintaining a PKU diet [11]. Any additional external factor might aggravate the observed problems

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