IMD WATCH
Anita MacDonald (Birmingham Children`s Hospital)
Karen Van Wyk (Royal Manchester Children`s Hospital)
Rachel Skeath (Great Ormond Street Hospital)
Pat Portnoi (Dietitian to the Galactosaemia Support Group).
DEVELOPMENT OF EDUCATIONAL TEACHING PACKAGES (TEMPLE) ON INHERITED METABOLIC DISEASES For parents and health professionals in the UK. There are many rare inherited metabolic disorders (IMD). These can be complex, may be managed with diet therapy with or without medications and some patients are at acute risk of acute metabolic decompensation. Increasing numbers of infants are being diagnosed by newborn screening in the first few weeks of life. However, as we still only test for a narrow range of conditions, some infants will present acutely unwell in the neonatal period. The diagnosis of a metabolic disorder is overwhelming for parents and families and they are unlikely to have previously heard of the condition. To a family, the name of the condition is complex and they may be unsure as to how to pronounce the condition or spell it correctly. Internet information may be sparse and alarmist and initial explanations of the condition may use terminology that sounds like biochemical gobbledygook. Families may feel isolated and the entire experience is very frightening with many concerns about the future. There is very little professional information available that is clear and accurate and not often written in a language that everyone understands. For disorders of protein metabolism, it is not uncommon for professionals to adapt information written for the most common disorder, phenylketonuria (PKU), but families rarely receive well produced information about their child’s specific condition unless it is via a patient society or commercial company. In 2012, a set of structured, modular teaching slides for parents/caregivers
following a positive newborn screening test was developed by Drs Wendel and Burgard from Heidelberg, Germany. This was a comprehensive package which helped to guide medical doctors to ensure that any teaching given to parents/caregivers was delivered in a consistent and standardised manner using materials produced by considered methodology. The educational package was titled TEMPLE (Tools Enabling Metabolic Parents LEarning) and the information was very structured, designed for use primarily in Germany. It covered a range of IMD and was organised so that the health professionals had a systematic plan for teaching about each disorder. It even gave health professionals guidance on the best seating plans for clinic rooms. Although this was a commendable endeavour, the information was complex and unsuitable for lay people if they lacked background knowledge or biochemical skills. In 2014, the British Inherited Metabolic Disorders Group (BIMDG)dietitians group examined the work by Wendel and Burgard and with permission from Nutricia (who had bought the rights to the teaching package) decided to adapt the original teaching package into ‘friendly’ and understandable teaching slide sets and booklets that could be used as a basis to explain different IMD to parents. In keeping with the philosophy of the creators of the first teaching package, it was important to approach the ‘content’ of the teaching package in a manner that would help standardise how complex IMD are explained to families in the UK. Even when working
www.NHDmag.com December 2017/January 2018 - Issue 130
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