Towards developing an ethical framework for decision making in long-term ventilation in children

Ray, Samiran; Brierley, Joe; Bush, Andy; Fraser, James; Halley, G; Harrop, Emily; Casanueva, Lidia

doi:10.1136/archdischild-2018-314997

Cited by 22 publications

(49 citation statements)

References 32 publications

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“…The ethical implications of prolonging life with long term ventilation in the context of commencement of an experimental therapy need to be weighed carefully against those of redirection of supportive care. Indeed, long term ventilation may be requested as a bridge to emerging or experimental therapies and its use in this setting is difficult to evaluate 37 . An ethics committee should be called upon to meet to assess the individual clinical case considering the opinion of the infant's parents and available second opinions provided by other clinical teams.…”

Section: Genotype-phenotype Correlationsmentioning

confidence: 99%

The natural history of infantile mitochondrial DNA depletion syndrome due to RRM2B deficiency

Keshavan

Abdenur

Anderson

et al. 2020

Genetics in Medicine

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Mitochondrial DNA (mtDNA) depletion syndrome (MDDS) encompasses a group of genetic disorders of mtDNA maintenance. Mutation of RRM2B is an uncommon cause of infantileonset encephalomyopathic MDDS. Here we describe the natural history of this disease. Methods Multinational series of new genetically confirmed cases from six paediatric centres. Results Nine new cases of infantile-onset RRM2B deficiency, and 22 previously published cases comprised a total cohort of 31 patients. Infants presented at a mean of 1.95 months with truncal hypotonia, generalised weakness and faltering growth. Seizures evolved in 39% at a mean of 3.1 months. Non-neurological manifestations included respiratory distress/failure (58%), renal tubulopathy (55%), sensorineural hearing loss (36%), gastrointestinal disturbance (32%), eye abnormalities (13%), and anaemia (13%). Laboratory features included elevated lactate (blood, CSF, urine, MR spectroscopy), ragged-red and cytochrome c oxidase-deficient fibres, lipid myopathy and multiple oxidative phosphorylation enzyme deficiencies in skeletal muscle. Eight new RRM2B variants were identified. Patients with biallelic truncating variants had the worst survival. Overall survival was 29% at 6 months and 16% at 1 year. Conclusion Infantile-onset MDDS due to RRM2B deficiency is a severe disorder with characteristic clinical features and extremely poor prognosis. Presently management is supportive as there is no effective treatment. Novel treatments are urgently needed.

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Section: Genotype-phenotype Correlationsmentioning

confidence: 99%

The natural history of infantile mitochondrial DNA depletion syndrome due to RRM2B deficiency

Keshavan

Abdenur

Anderson

et al. 2020

Genetics in Medicine

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“…An ethical framework for decision making in pediatric long term ventilation has been proposed (28). This approach can provide appropriate space for the interaction between the involved patients, their families, healthcare providers, health authorities and the broader society.…”

Section: Ethical Dilemmamentioning

confidence: 99%

Noninvasive Ventilation in Palliative Care and Ethical Dilemma

Krivec

Caggiano

2020

Front. Pediatr.

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Significant difference exists between validated indications for noninvasive ventilation (NIV) use in children and current real life practice. Lately, dedicated centers have reported exponential growth of NIV use in children and adolescents. Upper airway obstruction, neuromuscular diseases, chronic lung/thoracic conditions, and central respiratory drive failure remain the most prevalent indications. However, the need to alleviate respiratory failure related distress has been increasingly recognized in several other conditions. Palliative care in children with life limiting disorders is a complex continuum of activities. In order to provide the most appropriate care for the patients and their families, the management often oscillates between actively curative and purely supportive actions. Despite unprecedented therapeutic advancements, several neurologic, metabolic, hemato-oncologic, respiratory, and other rare diseases remain with no curative options. Besides, attentiveness to relive suffering, awareness, and availability have moved the boundaries of NIV use toward conditions formerly not considered suitable for such care. Still, NIV has limitations and can, if sustained in inappropriate circumstances, fail to provide relief. A structured professional frameshift should be available for support and ethical guidance in order to provide confidence to patients, families and all the involved caregivers.

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“…Nasogastric feeding has long been a community treatment, but the rare provision of parental nutrition in the community as a bridge to bowel transplant has somehow evolved to a destination therapy. Similarly, long term ventilation – largely a bridge to recovery as recently as the first few years of the millennium, is now a destination for children without prospect of recovery (Fine‐Goulden et al , ; Ray et al , ). Soon, renal replacement therapy and mini‐ventricular assist devices will complete the home‐ICU!…”

Section: Change In Medical Interventionsmentioning

confidence: 99%

Challenges in managing parental expectations in paediatric care

Pré

Brierley

2018

Br J Haematol

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Given the recent number of controversial cases featured in the media, it is timely to consider the management of parental expectations in paediatric care. What is sadly overlooked in the controversy is the usual excellent working relationship formed by treating child health professionals, parents (those with parental responsibility) and children themselves in tackling really tough situations. We are able to do more and more to tackle previously deadly diseases, and yet the burden of our treatments is undeniably higher. At the same time, social changes and ready access to information have led to a necessary reduction in medical paternalism, and more empowered and knowledgeable patients. We review how things have become very challenging in this arena in the UK over the last decades, and consider the issues surrounding disagreements and conflict. On the background of population shifts, religious diversity and populism, parental expectations now vary dramatically given the same situation. The challenge is still met by communicating, listening with empathy but finally by adhering to the basic tenet of Article 3 of the UN Convention of the Rights of the Child -'The best interests of children must be the primary concern in making decisions that may affect them.'

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Towards developing an ethical framework for decision making in long-term ventilation in children

Cited by 22 publications

References 32 publications

The natural history of infantile mitochondrial DNA depletion syndrome due to RRM2B deficiency

The natural history of infantile mitochondrial DNA depletion syndrome due to RRM2B deficiency

Noninvasive Ventilation in Palliative Care and Ethical Dilemma

Challenges in managing parental expectations in paediatric care

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