Does maternal knowledge and parent education affect blood phenylalanine control in phenylketonuria?

MacDonald, Anita; Davies, Paul; Daly, Anne; Hopkins, V.; Hall, S K; Asplin, D.; Hendriksz, Christian J.; Chakrapani, Anupam

doi:10.1111/j.1365-277x.2008.00891.x

Cited by 44 publications

(59 citation statements)

References 11 publications

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“…Overall outcome in children depends on parental ability, their discipline, motivation, organization skills and overall coping ability [31] to continually apply and supervise the dietary regimens. Caring for 1 affected child with an IEM together with healthy children may be particularly taxing, sometimes testing the personal conviction about the need for the severity of the dietary treatment for their child.…”

Section: The Patient’s Family Characteristicsmentioning

confidence: 99%

Adherence Issues in Inherited Metabolic Disorders Treated by Low Natural Protein Diets

et al. 2012

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Common inborn errors of metabolism treated by low natural protein diets [amino acid (AA) disorders, organic acidemias and urea cycle disorders] are responsible for a collection of diverse clinical symptoms, each condition presenting at different ages with variable severity. Precursor-free or essential l-AAs are important in all these conditions. Optimal long-term outcome depends on early diagnosis and good metabolic control, but because of the rarity and severity of conditions, randomized controlled trials are scarce. In all of these disorders, it is commonly described that dietary adherence deteriorates from the age of 10 years onwards, at least in part representing the transition of responsibility from the principal caregivers to the patients. However, patients may have particular difficulties in managing the complexity of their treatment because of the impact of the condition on their neuropsychological profile. There are little data about their ability to self-manage their own diet or the success of any formal educational programs that may have been implemented. Trials conducted in non-phenylketonuria (PKU) patients are rare, and the development of specialist l-AAs for non-PKU AA disorders has usually shadowed that of PKU. There remains much work to be done in refining dietary treatments for all conditions and gaining acceptable dietary adherence and concordance, which is crucial for an optimal outcome.

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Section: The Patient’s Family Characteristicsmentioning

confidence: 99%

Adherence Issues in Inherited Metabolic Disorders Treated by Low Natural Protein Diets

et al. 2012

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“…Features of these cases might also suggest ‘risk factors’ for chronically poor adherence, including young and single parent/s, lack of parental higher education [11], and lack of extended family support. Other known risk factors include alcoholism, drug abuse and mental health problems; it is known in the UK that between 50% and 90% of parents of children on social workers' child care caseloads have one or more of these issues, affecting their parenting capacity [12].…”

Section: Discussionmentioning

confidence: 99%

“…It is established that blood Phe control rarely improves if it has been unacceptable in the first 3 years of life [11], and children with PKU are likely to gain most benefit from social service intervention in pre-school years. Therefore, early warning signs of potential issues with long-term blood Phe control described above should be quickly addressed by working closely with families and other agencies.…”

Section: Discussionmentioning

confidence: 99%

When should social service referral be considered in phenylketonuria?

Rijn

Ahring

Bélanger-Quintana

et al. 2015

Molecular Genetics and Metabolism Reports

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Lifelong low-phenylalanine (Phe) dietary management is the foundation of care in phenylketonuria (PKU). However, strict monitoring of food intake places a burden on patients and their caregivers, and adherence to the required diet frequently decreases in later childhood and adolescence. Rarely, parents of children with PKU refuse to recognise the importance of treatment and follow-up for this chronic condition. Here, two case studies are presented that document consideration of placement of children into foster care or kinship homes as a last resort to improve persistently high Phe concentrations. In the first case, social service referral led to a 3-year-old girl being placed in a kinship home with her grandparents, resulting in excellent Phe control thereafter. In the second case, discussion with the parents of possible placement of a 12-year-old child into foster care was sufficient to have a positive effect on Phe control. A staged approach for managing intractable non-adherence in PKU is proposed.

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“…Por outro lado, mesmo descrevendo adequadamente a forma como o tratamento deveria ser realizado e suas conseqüências, a grande maioria dos participantes admitiu não seguir regularmente a instrução de oferecer diariamente o medicamento à criança. Tal resultado sugere a importância de o profissional, no caso o médico, se certificar sobre o entendimento de cada cuidador a respeito das informações recebidas durante a consulta, em especial sobre o caráter de cronicidade do HC, como apontado no estudo realizado sobre fenilcetonúria (MacDonald et al, 2008).…”

Section: Discussionunclassified

“…Fatores que dificultem a adesão no início do tratamento podem determinar a saú-de futura dessas crianças. Por outro lado, um cuidador bem esclarecido sobre as necessidades reais do tratamento indicado aumenta as chances de adesão ao tratamento da criança (Ferreira, 2006;Garcia et al, 2007;MacDonald et al, 2008;Rand & Weeks, 1998).…”

Section: Discussionunclassified

Adesão ao tratamento do hipotireoidismo congênito segundo relato de cuidadores

Oliveira¹,

Ferreira²

2010

Psicol. Reflex. Crit.

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ResumoEsta pesquisa procurou identificar variáveis que interferem na adesão ao tratamento segundo relato de cuidadores de crianças com Hipotireoidismo Congênito atendidas em um centro de referência no Estado do Pará. Participaram 50 cuidadores primários, com idade entre 17 e 55 anos, com diferentes graus de parentesco com a criança. As variáveis relacionadas à adesão foram identificadas por meio de entrevistas com os cuidadores, seguindo roteiro estruturado. Os resultados indicaram que a maioria dos cuidadores desconhece sobre características da doença, etiologia, diagnóstico, tratamento e prognóstico, independentemente da idade, escolaridade, grau de parentesco e tempo em que a criança está no Programa, dificultando a adesão ao tratamento. Para a maioria das crianças, o Teste do Pezinho foi realizado com atraso, indicando prejuízos ao tratamento. Palavras-chave: Adesão ao tratamento; Psicologia pediátrica; Hipotireoidismo congênito. AbstractThis study aims at identifying variables which interfere on the entry into the treatment according to reports of caregivers of children suffering from Congenital Hypothyroidism seen at the Center of Reference in Pará State. Fifty main caregivers, with different degrees of kinship to the children and ranging from 17 to 55 years old, took part in this study. The variables related to the treatment entry were identified by means of interviews with the caregivers following structured pilot instructions. Results showed that most of the caregivers are not aware of the characteristics of the disease, its etiology, diagnosis, treatment and prognostic, regardless the age, school degree, kinship and amount of time the child is on the Program, which makes the treatment entry more difficult. In most children, the Newborn Screening Test was not done in the right time, fact that brought damage to the treatment. Keywords: Treatment entry; Pediatric psychology; Congenital hypothyroidism.O hipotireoidismo se caracteriza pela produção diminuída de hormônios tireoidianos ou, raramente, como um estado clínico resultante da produção de uma ação inadequada destes hormônios em nível celular. Os hormônios tireoidianos são importantes reguladores metabólicos durante a vida toda; porém, durante o início da vida é que sua atuação é fundamental para o desenvolvimento do indivíduo (Murahovschi, 1994;Setian, 2002).Desde antes do nascimento, os hormônios tireoidianos agem sobre o desenvolvimento do Sistema Nervoso Central. A ausência destes hormônios provoca uma diminuição dos processos metabólicos e do desenvolvimento cerebral normal. Esta diminuição metabólica é reversível quando feita a administração terapêutica destes hormô-nios. Desse modo, o Hipotireoidismo Congênito (HC) é de recuperação satisfatória uma vez que sejam restabelecidas as funções metabólicas mediante a utilização dos hormônios. Porém, para que esse sucesso seja alcança-do, é necessário o diagnóstico precoce e a intervenção terapêutica tão logo seja diagnosticada a patologia no recém-nascido (Murahovschi, 1994;Setian, 2002).A maioria da...

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Does maternal knowledge and parent education affect blood phenylalanine control in phenylketonuria?

Abstract: Blood phenylalanine control within the first 3 years of age, poor parental educational achievement at school level, and unsatisfactory maternal dietary knowledge may all influence longer-term blood phenylalanine control in children.

Cited by 44 publications

References 11 publications

Adherence Issues in Inherited Metabolic Disorders Treated by Low Natural Protein Diets

Adherence Issues in Inherited Metabolic Disorders Treated by Low Natural Protein Diets

When should social service referral be considered in phenylketonuria?

Adesão ao tratamento do hipotireoidismo congênito segundo relato de cuidadores

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