The authors conclude here that when withdrawing treatment in PICU is considered parents' refusal
to consent can cause additional suffering as clinicians tend to extend burdensome treatment beyond
what they think is reasonable to allow parents time to reconsider. Moreover, both parents and
clinicians try to avoid approaching the courts for a decision.
On the basis of these findings the authors suggest that limiting parental authority by using the concept of parental assent instead of consent could lead to an expeditious resolution in cases of disagreement and should be the focus of further research.
This suggestion is not supported by the parental quotes used in this article. Indeed, one of the parent's objection to a court decision stems from his opinion that the decisions regarding withdrawal of treatment should be the domain of the parents. Limiting parental authority might therefore lead to increased adversarial relationships between the treating team and parents especially when parental views are overruled.
Some quotes in this article as well as other research show that parents at the end of their child's life need time to
often extensively research alternative treatments 'because you just need to have looked and
exhausted every avenue'. Rather than limiting parental authority, it may thus be better to start the
discussion regarding end of life care, including withholding treatment earlier....
The authors conclude here that when withdrawing treatment in PICU is considered parents' refusal
to consent can cause additional suffering as clinicians tend to extend burdensome treatment beyond
what they think is reasonable to allow parents time to reconsider. Moreover, both parents and
clinicians try to avoid approaching the courts for a decision.
On the basis of these findings the authors suggest that limiting parental authority by using the concept of parental assent instead of consent could lead to an expeditious resolution in cases of disagreement and should be the focus of further research.
This suggestion is not supported by the parental quotes used in this article. Indeed, one of the parent's objection to a court decision stems from his opinion that the decisions regarding withdrawal of treatment should be the domain of the parents. Limiting parental authority might therefore lead to increased adversarial relationships between the treating team and parents especially when parental views are overruled.
Some quotes in this article as well as other research show that parents at the end of their child's life need time to
often extensively research alternative treatments 'because you just need to have looked and
exhausted every avenue'. Rather than limiting parental authority, it may thus be better to start the
discussion regarding end of life care, including withholding treatment earlier.
This would allow parents time to explore the presence or absence of other treatment options whilst
their child is still relatively well. Given that the majority of children that die in PICU due to
withholding treatment suffer from a chronic illness(1) and referral to palliative care services
reduces the number of children dying in hospital(2), I would argue that earlier referral to paediatric palliative care services might well prevent the conundrum during PICU admissions described in this article.
1. Burns JP, Sellers DE, Meyer EC, Lewis-Newby M, Truog RD. Epidemiology of death in the
PICU at five U.S. teaching hospitals*. Crit Care Med. 2014 Sep;42(9):2101–8.
2. Fraser LK, Miller M, Draper ES, McKinney PA, Parslow RC, on behalf of the Paediatric
Intensive Care Audit Network. Place of death and palliative care following discharge from
paediatric intensive care units. Arch Dis Child. 2011 Dec 1;96(12):1195–8.
Is Bexsero® (MenB vaccine) effective in preventing invasive meningococcal disease? Experience of a tertiary hospital in the UK. Novel meningococcus serogroup B vaccine (Bexsero®) was introduced in UK national immunisation programme on 1 September 2015. All babies born from July 2015 were offered the vaccine alongside other routine immunisations and all babies born in May 2015 were offered Bexsero® as a one-off catch-up. Bexsero® is estimated to protect against 73–88% of MenB strains causing invasive meningococcal disease (IMD) in England and Wales1,2. Among the diseases preventable by immunisation, IMD remains a high public profile illness deserving the most rigorous consideration because of its rapid and severe onset, high mortality rate and burden of sequelae. Epidemiological data suggest that infants in the first year of life experience the highest risk of infection peaking at around 5 months and declining thereafter. We continue to observe IMD in the first year of life despite the introduction of Bexsero® in our national immunisation programme (Table 1). This retrospective data was obtained as part of service evaluation at Central Manchester University Hospital Foundation Trust from our microbiology department. We are one of the biggest integrated Children's hospitals in the UK providing a wide range of services for the North West region and have over 220,000 patient visits each year. The epidemiological year starts from July to June, rath...
Is Bexsero® (MenB vaccine) effective in preventing invasive meningococcal disease? Experience of a tertiary hospital in the UK. Novel meningococcus serogroup B vaccine (Bexsero®) was introduced in UK national immunisation programme on 1 September 2015. All babies born from July 2015 were offered the vaccine alongside other routine immunisations and all babies born in May 2015 were offered Bexsero® as a one-off catch-up. Bexsero® is estimated to protect against 73–88% of MenB strains causing invasive meningococcal disease (IMD) in England and Wales1,2. Among the diseases preventable by immunisation, IMD remains a high public profile illness deserving the most rigorous consideration because of its rapid and severe onset, high mortality rate and burden of sequelae. Epidemiological data suggest that infants in the first year of life experience the highest risk of infection peaking at around 5 months and declining thereafter. We continue to observe IMD in the first year of life despite the introduction of Bexsero® in our national immunisation programme (Table 1). This retrospective data was obtained as part of service evaluation at Central Manchester University Hospital Foundation Trust from our microbiology department. We are one of the biggest integrated Children's hospitals in the UK providing a wide range of services for the North West region and have over 220,000 patient visits each year. The epidemiological year starts from July to June, rather than January to December. Most forms of IMD, like many infectious diseases, display seasonal variation and reach a peak during the winter. Looking at epidemiological years means that every winter peak always falls within one year rather than being divided between two consecutive calendar years. ‘Unvaccinated’ cases (Table 1) had all routine vaccinations apart from Bexsero® (before September 2015). The ‘Vaccinated’ group includes those who ‘supposedly’ received Bexsero® (after September 2015). The assumption was made that all patients born from July 2015, received Bexsero®; their vaccination records were not checked. There are uncertainties about how effective it will be in protecting against invasive meningococcal disease3. Good active population-based sentinel surveillance would allow gathering important information also about the capability of the new vaccine to disrupt carrier chain and reach herd immunity.
REFERENCES
1.Frosi G, et al., Bactericidal antibody against a representative epidemiological meningococcal serogroup B panel confirms that MATS underestimates 4CMenB vaccine strain coverage. Vaccine, 2013.
2.Vogel, U., et al., Predicted strain coverage of a meningococcal multicomponent vaccine (4CMenB) in Europe: a qualitative and quantitative assessment. Lancet Infect Dis, 2013. 13(5): p. 416-25.
3.Andrews SM, Pollard AJ. A vaccine against serogroup B Neisseria meningitidis: dealing with uncertainty. The Lancet infectious diseases. 2014;14(5): 426–34. doi: 10.1016/S1473-3099(13)70341-4 [PubMed]
Lemer has very usefully carried out a 5 year review of policy implementation. Policy is only as good as the receivers at the other end and these change frequently along with an ever changing political and economic landscape.Thus the exercise is valuable in not only taking stock but also reminding those in power of an independent review process with recommendations which should transcend governments. Sadly , the focus on funding education of the workforce (recommendations 10-12) do not appear to have been a priority and without this foundation, we will not move ahead sufficiently fast with a child and family friendly service. The Children and Young Persons Outcomes Framework is similarly the result of much work in a previous government and must not be allowed to whither on the vine. Perhaps we should regularly remind policy makers in the current administration of the value of persistence with other such initiatives which have a broad professional consensus and can be dusted off and re-badged as necessary to tempt politicians to move the goal posts a little closer to what is required to optimize child health? Lets see how far we have got in another 5 years.
We would like to thank the authors of the Pavone paper for their interest in our paper (1,2). We are sorry for not quoting their paper in our study report but do confirm that we were aware of it (2). In our introduction we selected several papers to quote in order to introduce the uncertainty with respect to the need to record 1, 2 or 3 nights of overnight oximetry and the Pavone paper was not one we selected. The Pavone paper claims excellent night to night consistency in oximetry and that only one night of oximetry measurement is necessary while our study did not find this to be the case (2).
While we agree that the Pavone study used a pulsed oximeter with some superior properties (Radical Masimo) compared to that which we used (Nonin 9600) we do not believe that this is one of the most important reasons why our results differ from the Pavone study.
We believe the main reasons for differences between the two papers include;
1] Different primary aims - our study was aimed to determine whether doing 2 or 3 nights oximetry would increase the chances of getting adequate traces to make a report. We therefore included all studies (whether satisfactory or not). In the Pavone study only those with 2 nights each with > 6 hours satisfactory tracing were included and about one third of the children initially identified were therefore excluded. We do not know what then happened to these children – i.e. whether further studies had to be rescheduled. Clearly selec...
We would like to thank the authors of the Pavone paper for their interest in our paper (1,2). We are sorry for not quoting their paper in our study report but do confirm that we were aware of it (2). In our introduction we selected several papers to quote in order to introduce the uncertainty with respect to the need to record 1, 2 or 3 nights of overnight oximetry and the Pavone paper was not one we selected. The Pavone paper claims excellent night to night consistency in oximetry and that only one night of oximetry measurement is necessary while our study did not find this to be the case (2).
While we agree that the Pavone study used a pulsed oximeter with some superior properties (Radical Masimo) compared to that which we used (Nonin 9600) we do not believe that this is one of the most important reasons why our results differ from the Pavone study.
We believe the main reasons for differences between the two papers include;
1] Different primary aims - our study was aimed to determine whether doing 2 or 3 nights oximetry would increase the chances of getting adequate traces to make a report. We therefore included all studies (whether satisfactory or not). In the Pavone study only those with 2 nights each with > 6 hours satisfactory tracing were included and about one third of the children initially identified were therefore excluded. We do not know what then happened to these children – i.e. whether further studies had to be rescheduled. Clearly selecting only those with ‘good’ adequate tracings could improve repeatability. However, as stated in our paper, we did not find this to be the case when we analysed the repeatability for those with 3 adequate nights recording.
2] Pavone in their study excluded children with complex conditions (ie children with neuromuscular, complex or genetic conditions were not included). In our study this group represented close to 50% of those studied (Down Syndrome, Neuromuscular disorders and craniofacial structural problems, neurological disorders). We were especially worried about differential repeatability in children with Down Syndrome when compared with neuromuscular disorders. It is thus quite possible that better correlation will be noted in a specific group of otherwise normal children who also have performed well and allowed 2 nights of ‘good’ recordings to be made.
3] A 3rd and very important difference between our paper and that off Pavone relates to the method of statistical analysis for the continuous variables. We were particularly interested in determining the agreement between measures addressing the question whether one night could be replaced by the value of the second night and what the repeatability was. We used Bland & Altman 95% limits of agreement with plots and the intraclass correlation. In the Pavone paper only Pearson and Spearman correlation coefficients are quoted for relating night 1 with night 2 continuous variables and thus we were unable to estimate how good the agreement between these measures actually was. Bland & Altman have previous shown that two measurements of the same quantity (as occurs in repeated measures) are highly likely to closely correlate and especially so if a wide range of values are included (3). It is now well known that while repeated measures variables may correlate well they often do not agree to the extent that one value could be used to replace another (3). Eye balling the scatter plots (with regression lines) in the Pavone paper would not suggest that agreement was necessarily good. However, this is impossible to determine without seeing the Bland & Altman plots (with 95% limits of agreement) or the scattergrams (night 1 versus night 2) with the line of identity included rather than the regression line.
4] Pavone compared agreement between the two nights McGill Score (MOS) which is used to quantify the severity of Obstructive Sleep Apnoea (OSA). We did not use this as a significant proportion of our children especially those with neuromuscular disease were being studied for sleep disordered breathing with potential hypoventilation rather than detecting OSA. It would not have been appropriate to use the MOS score in this situation.
Unfortunately, for these reasons, we do not find that the Pavone paper provided reassurance that a single nights oximetry is adequate when screening children for the variety of sleep disordered breathing problems that we studied.
Finally, while the mean duration of overnight oximetry in our study was 8.4 hours (8.3 hours in the Pavone study) we included > 4 hours of technically satisfactory trace as adequate as our lower limit of acceptability. We agree that this may differ from the ATS recommendations but we think this is an important point for further discussion – and ask the question: Should we be discarding traces that are of good quality but only of 5.5 hours, 5 hours or even 4 hours duration in this group of children? We do not have the answer as to what is the minimum duration of good quality trace that should be regarded as useable. Instead of using a rather arbitrary value of > 6 hours our team considered that 3 hours was too short but that 5 hours was likely adequate and as a group agreed that if the trace was > 4 hours we would like and want it to be reported. It would be interesting and important to determine whether reporting studies of > 4 hours duration would accurately increase the diagnostic pick up in children who present with possible OSA.
References
1. Night-to-night consistency of at-home nocturnal pulse oximetry testing for obstructive sleep apnea in children. Pavone M, Cutrera R, Verrillo E, Salerno T, Soldini S, Brouillette RT. Pediatr Pulmonol. 2013 Aug;48(8):754-60
2. Night-to-night variation of pulse oximetry in children with sleep-disordered breathing.
Burke RM, Maxwell B, Hunter C, Graham D, O'Donoghue D, Shields MD. Arch Dis Child. 2016 Dec;101(12):1095-1099
3. Bland JM, Altman DG. Statistical methods for assessing agreement between two methods of clinical measurement. Lancet 1986;1:307–10
The title of the paper and the majority of the introduction imply that the study is about adolescents with CFS/ME. However, the final sentence of the introduction undermines that objective: “As children in our study were not examined by a physician, we have used the term ‘chronic disabling fatigue’ (CDF) rather than CFS/ME to indicate chronic fatigue that is disabling.”
Those children may have had a variety of different diseases that cause prolonged fatigue, yet we are led to believe that a study of their collective conditions can somehow add to the body of literature on a specific disease process. CFS/ME is a highly contentious disease with a great deal of conflicting evidence and hypotheses; answers as to its exact nature and cause are as yet to be determined. By publishing a study of patients who are so poorly defined as to be undefined, Archives of Disease in Childhood has further muddied already murky waters. The addition of this study to the body of literature is not only unhelpful, but is actively detrimental to the pursuit of answers for patients with this highly disabling disease.
How are ADC or the authors able to justify publishing a study that to all appearances is about CFS/ME, yet fails to properly assess if any of the study participants actually have CFS/ME?
Dear Editor,
We woud like to respond to one of the issues raised in the audit of high
flow nasal cannula (HHFNC) recently published (1). As the authors observed, although evidence for efficacy is lacking,
clinical pactice has rapidly expanded the indications for respiratory
support on the ward using HHFNC. We have observed a number of cases
where commencement of HHFNC may have delayed referral to the PICU
service, and are concerned that this may have affected the level of
respiratory support required on subsequent admission to PICU. Humidified high flow nasal cannula (HHFNC) provides heated and
humidified air/oxygen flow to support respiratory function in sick
infants and children. Flow rates may be up to 60L/min, and are usually
titrated at 1-3L/kg depending on clinical work of breathing (WOB). The
concentration of oxygen may be adjusted to maintain oxygen saturations
within the normal range for each child. Pediatric units providing this
therapy, usually do so within agreed guidelines. Some units mandate
admission to the Pediatric Intensive Care Unit (PICU), and some
administer HHFNC on the ward. There is some evidence that the use of
HHFNC may reduce the need for PICU admission and more advanced
respiratory support (2). However, studies to date have not stratified
infants further, into categories of risk of failure of therapy (3). Yet
infants with significan...
Dear Editor,
We woud like to respond to one of the issues raised in the audit of high
flow nasal cannula (HHFNC) recently published (1). As the authors observed, although evidence for efficacy is lacking,
clinical pactice has rapidly expanded the indications for respiratory
support on the ward using HHFNC. We have observed a number of cases
where commencement of HHFNC may have delayed referral to the PICU
service, and are concerned that this may have affected the level of
respiratory support required on subsequent admission to PICU. Humidified high flow nasal cannula (HHFNC) provides heated and
humidified air/oxygen flow to support respiratory function in sick
infants and children. Flow rates may be up to 60L/min, and are usually
titrated at 1-3L/kg depending on clinical work of breathing (WOB). The
concentration of oxygen may be adjusted to maintain oxygen saturations
within the normal range for each child. Pediatric units providing this
therapy, usually do so within agreed guidelines. Some units mandate
admission to the Pediatric Intensive Care Unit (PICU), and some
administer HHFNC on the ward. There is some evidence that the use of
HHFNC may reduce the need for PICU admission and more advanced
respiratory support (2). However, studies to date have not stratified
infants further, into categories of risk of failure of therapy (3). Yet
infants with significant comorbidities may present to the emergency
department of the district general hospital (DGH), and receive HHFNC
therapy (1).
We have retrospectively audited admissions of infants to PICU with a
diagnosis of bronchiolitis over the past 24 months to assess the impact
of HHFNC, Synagis administration and standard PICU care on outcome. As
part of this quality audit we have observed a number of cases which
suggest the HHFNC may have increased the requirement for sedation and
intubation, and increased the duration of ventilatory support required.
We present three cases from this audit, and suggest changes to practice
which may improve outcome when utilizing HHFNC in very young infants.
Ethics approval was waived as an audit of existing practice. Case 1
12 day old ex-premature infant, born at 34+5, 2.4kg. Home for 5 days,
presented to the emergency department with occasional cough, brief
apneas and cyanosis. First SpO2 81% on room air. Commenced HHFNC 2L/kg
50% oxygen. RSV positive. 16 hours after admission had prolonged apnea
with bradycardia. Lifeless in parents arms, resuscitated and transferred
to PICU. Intubated and ventilated for 6 days.
Case 2
4 week old infant post-operative hypoplastic left ventricle, stage I
palliation, norwood. Had received Synagis before discharge home. Poor
feeding and cough for 24 hours. Presented to ED and admitted to Cardiac
ward on HHFNC 2L/kg, FiO2 0.25. Marked WOB and cold peripheries. Oxygen
saturations dipping into the 60's, oxygen titrated up overnight. Kept
fasting and commenced intravenous fluids. Significant WOB continued,
flow increased to 3L/kg, 40% oxygen. Cold peripheries. Infant
increasingly lethargic. Arterial blood gas 28 hours after admission
showed pH 7.17 pO2 3.4, pCO2 10.7, BE -17, lactate 8. Admitted to PICU,
sedated and intubated after fluid administration. Transfused 20ml/kg red
cells, ventilated for 8 days. RSV positive.
Case 3
9 week old 3.2kg infant post-operative tracheoesophageal fistula repair,
VACTERL syndrome. Presented to ED with cough, profuse nasal secretions
and significant work of breathing (WOB). HHFNC 2L/kg and nasogastric
feeds commenced with initial improvement of WOB. 50 hours later, infant
still had increased WOB. Coughing spells provoked vomiting at the end of
each feed. Venous blood gas showed pH 7.1, PO2 5.1, PCO2 11.0, BE -12,
lactate 4. He was reviewed by PICU team who noted respiratory rate of 16
breaths per minute. Transferred to PICU and intubated immediately.
Ventilated for 7 days, followed by 2 days of non-invasive ventilation.
From the above cases, and other infants reviewed during our audit of
bronchiolitis admissions to PICU, we concur with Jones et al that
further study of HHFNC in some groups of infants and children is
required (1). The patients we have particular concerns about are:
-Infants less than 48 weeks post-conceptual age (PCA).
-Ex-premature infants who are <48 weeks PCA
-Infants post cardiac surgery
-Young infants post major surgery
-Young infants who present with increased PCO2 on their first blood gas
analysis.
-Young infants who present with a significant oxygen requirement >
50%
Implementation of following measures may be considered to increase the
safety and efficacy of ward-based HHFNC in these infants:
-Increased level of monitoring for the first 2 hours after starting
HHFNC. If no clinical improvement, admit to PICU.
-If PCO2 is increased at presentation, admit to PICU for non-invasive
PS/CPAP (BIPAP).
-If HHFNC is still required after 24 hours of support, admit to PICU.
Until we have robust evidence to support the widespread use of HHFNC on
the infant wards and in the DGH, we should aim to reduce inappropriate
and prolonged use of HHFNC in very young infants, especially those with
co-morbidity, by ensuring that locally agreed clinical guidelines are
being adhered to - we also need to consider the possiblity that HHFNC
may not be a panacea for all patients (4).
References:
1. Jones AJ, Mathew S, Wong SW, Patel M, Equi A, Sukhani S, Mambiar S,
Ramnarayan P. A regional audit of high-flow nasal cannula therpay use
for bronchiolitis in London district general hospitals. Rch Dis Child
online Jan 24 2017. http://adc.bmj.com/content/early/2017/01/24/archdischild-2016-312462
2.Beggs SI, Wong ZH, Kaul S, Ogden KJ, Walters JA. High flow nasal
cannula therapy for infants with bronchiolitis. Cochrane Database Syst
Rev 2014 Jan 20;(1):CD009609
3.Riese J, Fierce J, Riese A, Alverson BK. Effect of a hospital-wide
hgh-flow cannula protocol on clinical outcomes and resource utilization
of bronchiolitis patients admitted to PICU. Hosp Pediatrics
2015;5(12):613-8
4.Kang BJ, Koh Y, Lim CM et al. Failure of high-flow nasal cannula
therapy may delay intubation and increase mortality. Intensive Care
Medicine 2015;41:623-32
We thank Professor Wright for her comments, and we welcome the opportunity to provide some clarification and further analysis.
We reported Z-scores rather than percentiles, although some comments on approximate percentiles can be made. Assuming that Z scores of -1.96 and -3 represent approximately the 2.5th and 0.2nd centiles respectively, 36/101 children were below the 2.5th centile, and 17/101 were below the 0.2nd centile for weight. Additionally, our mixed effects model (accounting for multiple measurements) modelling the group trend over time estimated the mean weight Z score at 11 years to be -1.63 (approximately 5th centile).
Despite the overall short stature of the group, 24/101 children had a BMI Z score of less than -1.96. So, by this approach, their weight was low even after taking into account stature. We agree that we cannot infer causality from this observational study, but we believe a proportion of the stunted growth is explained by low weight. We are exploring other measures of malnutrition, such as skin-fold thickness.
Whilst our patient numbers are small, they do give some weight to the argument that PEG feeding halts the progression of malnutrition. We investigated the rate of decline of weight after PEG insertion. In a mixed effects model with a random intercept for individual patients, the rate of wei...
We thank Professor Wright for her comments, and we welcome the opportunity to provide some clarification and further analysis.
We reported Z-scores rather than percentiles, although some comments on approximate percentiles can be made. Assuming that Z scores of -1.96 and -3 represent approximately the 2.5th and 0.2nd centiles respectively, 36/101 children were below the 2.5th centile, and 17/101 were below the 0.2nd centile for weight. Additionally, our mixed effects model (accounting for multiple measurements) modelling the group trend over time estimated the mean weight Z score at 11 years to be -1.63 (approximately 5th centile).
Despite the overall short stature of the group, 24/101 children had a BMI Z score of less than -1.96. So, by this approach, their weight was low even after taking into account stature. We agree that we cannot infer causality from this observational study, but we believe a proportion of the stunted growth is explained by low weight. We are exploring other measures of malnutrition, such as skin-fold thickness.
Whilst our patient numbers are small, they do give some weight to the argument that PEG feeding halts the progression of malnutrition. We investigated the rate of decline of weight after PEG insertion. In a mixed effects model with a random intercept for individual patients, the rate of weight Z score decline was 0.01 units per year, in contrast with 0.1 per year in the un-operated population, although low numbers prevent a meaningful statistical comparison. Given the ultra-rare nature of the disease, a randomised trial is effectively impossible and probably anyway unethical.
The multifactorial aetiology of wasting is unique in A-T. The disease compromises varying components of chronic inflammation, poor feeding ability, and increased calorie consumption due to dystonic and athetoid movements. The prognosis in A-T is truly appalling, and therefore we aim for the best possible quality of life. As well as showing that PEG feeding halts the progression of malnutrition (albeit in small numbers of children as compared to controls) and making feeding safer in the presence of possible aspiration, one consistent theme (not reported in our paper but consistently reported by parents in our clinic and previously published by other groups(1)) is significant caregiver satisfaction and reduction in very lengthy meals times. This has a very significant impact in improving the quality of life of carers and patients.
It is well recognised in other chronic paediatric diseases, such as cystic fibrosis and chronic kidney disease that nutrition is a predictor of overall quality of life. Currently, we have very little to offer patients with A-T in terms of intervention to prevent the development of malignancy or neurological progression, but we believe by extrapolating from other similar patient groups, we can reduce the risk of death from pulmonary failure. Key to this is prevention of wasting.
All long term invasive medical technologies interfere with the human condition of childhood, and we wholeheartedly agree that a PEG is life-changing and should always be carefully considered on an individual basis. However, given the poor weight gain in many A-T children, we believe it is important to discuss whether a PEG would be useful early, and consider placement prior to the respiratory deterioration of the child.
Yours sincerely
Emma Stewart1, Andrew P Prayle2, Alison Tooke1, Sara Pasalodos3, Mohnish Suri3, Andy Bush4,5,6, Jayesh M Bhatt1
Author affiliations:
1 Nottingham Children's Hospital, National Paediatric Ataxia Telangiectasia Clinic, QMC, Nottingham, UK
2 University of Nottingham, School of Clinical Science, Queens Medical Centre, Child Health, Nottingham, UK
3 Nottingham Clinical Genetics Service, National Paediatric Ataxia Telangiectasia Clinic, Clinical Genetics Service, City Hospital Campus, Nottingham, UK
4 Imperial College, London, UK
5 National Heart and Lung Institute, London, UK
6 Royal Brompton & Harefield NHS Foundation Trust, London, UK
1. Lefton-Greif MA, Crawford TO, McGrath-Morrow S, Carson KA, Lederman HM. Safety and caregiver satisfaction with gastrostomy in patients with Ataxia Telangiectasia. Orphanet J Rare Dis. 2011;6:23.
I agree with Dr. Goldwater that an undetected prodromal respiratory infection can suddenly fulminate and cause acute anoxic encephalopathy. In such an instance, there may not be time for visible pulmonary histological pathology to form. Then if a lung culture is not performed or gives sepsis-negative results, the cause may be coded as SIDS rather than an ARI. See Farber S. Fulminating streptococcus infections in infancy as a cause of sudden death. N Engl J Med 211:154-158, 1934 and Mage et al. .Front Neurol. 2016 Aug 23;7:129. doi: 10.3389/fneur.2016.00129. eCollection 2016. PubMed ID 27602017
I wonder if this brief report by Harvey et al. highlights where we are going wrong. Firstly, the lack of response to the QIP may just reflect the fact that we have such limited ability to influence outcomes when it comes to childhood obesity. If you are working in a busy CAU it seems pointless doing things that are not going to produce a positive outcome.
However my biggest concern is the statement: "How paediatricians act has a large impact on parents: we cannot expect them to prioritise their child’s obesity if we do not do the same." This appears to be the “nanny state” at work. The fact that parents are not recognising their children’s obesity, if this is really the case given the publicity this topic is receiving, is the main problem. This idea that patients are completely dependent on professionals to bring about change influences the outcome for many chronic conditions. Best results are obtained when patients (and carers) are actively involved in the management of the disease and are equipped to influence outcomes. This can only come about through education.
My personal experience is that I cannot remember ever seeing an overweight child maintain any significant weight loss. The lack of parental recognition of the fact that their child is overweight is a major problem. I am not sure how long the comment "your child is overweight" stays with parents after they leave the clinic. Do parents feel that an overweight child reflects well on...
I wonder if this brief report by Harvey et al. highlights where we are going wrong. Firstly, the lack of response to the QIP may just reflect the fact that we have such limited ability to influence outcomes when it comes to childhood obesity. If you are working in a busy CAU it seems pointless doing things that are not going to produce a positive outcome.
However my biggest concern is the statement: "How paediatricians act has a large impact on parents: we cannot expect them to prioritise their child’s obesity if we do not do the same." This appears to be the “nanny state” at work. The fact that parents are not recognising their children’s obesity, if this is really the case given the publicity this topic is receiving, is the main problem. This idea that patients are completely dependent on professionals to bring about change influences the outcome for many chronic conditions. Best results are obtained when patients (and carers) are actively involved in the management of the disease and are equipped to influence outcomes. This can only come about through education.
My personal experience is that I cannot remember ever seeing an overweight child maintain any significant weight loss. The lack of parental recognition of the fact that their child is overweight is a major problem. I am not sure how long the comment "your child is overweight" stays with parents after they leave the clinic. Do parents feel that an overweight child reflects well on them in that they are providing adequate amounts of food. The importance of food in our distant history must still have an influence. For the vast majority of the population, as recently as 100 years ago, food was often scarce and you ate whatever was put in front of you in case there was nothing for a while. That behaviour is now a problem, when food is freely available.
The only way this is going to change is when parents take the lead. The aim has to be to prevent children becoming overweight. Once it has happened it appears next to impossible to correct. This is a problem that only parents are in a position to manage and they must take responsibility for it, not medical professionals.
The paper by Yang et al1 provided an interesting epidemiological picture regarding the healthcare use in the year before correct diagnoses are confirmed for childhood cancer, type 1 diabetes mellitus (T1DM) and other immune diseases. Despite the presence of known clinical presentations associated with these diseases, diagnoses are not usually made until after couples of medical visits, except in cases with T1DM. Nearly two thirds of newly diagnosed T1DM patients presented emergent diabetic ketoacidosis. This rate was similar to that reported in a single-center Taiwanese study2 but still much higher than those in the US and Europe.3,4 This finding raised a question whether diabetic ketoacidosis at diagnosis of T1DM was a result of missed recognition for diabetic symptoms. In this regard, I am surprised that common urological symptoms, such as proteinuria and polydipsia2, found in Taiwanese T1DM patients were not included in the ICD-9 codes grouped for the urogenital problems, although the data showed an increase in urogenital problems shortly before the diagnosis of T1DM.1 From a clinical perspective, it is also crucial to know how the access to healthcare before diagnosis differ between those with and without diabetic ketoacidosis.3 If the analysis can be stratified by this factor, we may better evaluate the performance and impact of pre-diagnostic outpatient visits on subsequent healthcare for T1DM. There is always room for improvement in terms of increasing awareness of...
The paper by Yang et al1 provided an interesting epidemiological picture regarding the healthcare use in the year before correct diagnoses are confirmed for childhood cancer, type 1 diabetes mellitus (T1DM) and other immune diseases. Despite the presence of known clinical presentations associated with these diseases, diagnoses are not usually made until after couples of medical visits, except in cases with T1DM. Nearly two thirds of newly diagnosed T1DM patients presented emergent diabetic ketoacidosis. This rate was similar to that reported in a single-center Taiwanese study2 but still much higher than those in the US and Europe.3,4 This finding raised a question whether diabetic ketoacidosis at diagnosis of T1DM was a result of missed recognition for diabetic symptoms. In this regard, I am surprised that common urological symptoms, such as proteinuria and polydipsia2, found in Taiwanese T1DM patients were not included in the ICD-9 codes grouped for the urogenital problems, although the data showed an increase in urogenital problems shortly before the diagnosis of T1DM.1 From a clinical perspective, it is also crucial to know how the access to healthcare before diagnosis differ between those with and without diabetic ketoacidosis.3 If the analysis can be stratified by this factor, we may better evaluate the performance and impact of pre-diagnostic outpatient visits on subsequent healthcare for T1DM. There is always room for improvement in terms of increasing awareness of T1DM symptoms, ensuring early diagnosis, and preventing complications among healthcare sectors and affected families.
References
1. Yang TO, Huang W, Chen M, et al. Childhood cancer, type 1 diabetes and other immune diseases: healthcare visits in the year before diagnosis in Taiwan Archives of Disease in Childhood Published Online First: 08 February 2017. doi: 10.1136/archdischild-2016-311762
2. Chen YC, Tung YC, Liu SY, et al. Clinical characteristics of type 1 diabetes mellitus in Taiwanese children aged younger than 6 years: A single-center experience.
J Formos Med Assoc. 2016 Aug 9. doi: 10.1016/j.jfma.2016.07.005. [Epub ahead of print]
3. Baldelli L, Flitter B, Pyle L, et al. A survey of youth with new onset type 1 diabetes: Opportunities to reduce diabetic ketoacidosis. Pediatr Diabetes. 2016 Oct 11. doi: 10.1111/pedi.12455.
4. Cherubini V, Skrami E, Ferrito L, et al. High frequency of diabetic ketoacidosis at diagnosis of type 1 diabetes in Italian children: a nationwide longitudinal study, 2004-2013. Sci Rep. 2016 Dec 19;6:38844. doi: 10.1038/srep38844.
The authors conclude here that when withdrawing treatment in PICU is considered parents' refusal
Show Moreto consent can cause additional suffering as clinicians tend to extend burdensome treatment beyond
what they think is reasonable to allow parents time to reconsider. Moreover, both parents and
clinicians try to avoid approaching the courts for a decision.
On the basis of these findings the authors suggest that limiting parental authority by using the concept of parental assent instead of consent could lead to an expeditious resolution in cases of disagreement and should be the focus of further research.
This suggestion is not supported by the parental quotes used in this article. Indeed, one of the parent's objection to a court decision stems from his opinion that the decisions regarding withdrawal of treatment should be the domain of the parents. Limiting parental authority might therefore lead to increased adversarial relationships between the treating team and parents especially when parental views are overruled.
Some quotes in this article as well as other research show that parents at the end of their child's life need time to
often extensively research alternative treatments 'because you just need to have looked and
exhausted every avenue'. Rather than limiting parental authority, it may thus be better to start the
discussion regarding end of life care, including withholding treatment earlier....
Is Bexsero® (MenB vaccine) effective in preventing invasive meningococcal disease? Experience of a tertiary hospital in the UK. Novel meningococcus serogroup B vaccine (Bexsero®) was introduced in UK national immunisation programme on 1 September 2015. All babies born from July 2015 were offered the vaccine alongside other routine immunisations and all babies born in May 2015 were offered Bexsero® as a one-off catch-up. Bexsero® is estimated to protect against 73–88% of MenB strains causing invasive meningococcal disease (IMD) in England and Wales1,2. Among the diseases preventable by immunisation, IMD remains a high public profile illness deserving the most rigorous consideration because of its rapid and severe onset, high mortality rate and burden of sequelae. Epidemiological data suggest that infants in the first year of life experience the highest risk of infection peaking at around 5 months and declining thereafter. We continue to observe IMD in the first year of life despite the introduction of Bexsero® in our national immunisation programme (Table 1). This retrospective data was obtained as part of service evaluation at Central Manchester University Hospital Foundation Trust from our microbiology department. We are one of the biggest integrated Children's hospitals in the UK providing a wide range of services for the North West region and have over 220,000 patient visits each year. The epidemiological year starts from July to June, rath...
Show MoreLemer has very usefully carried out a 5 year review of policy implementation. Policy is only as good as the receivers at the other end and these change frequently along with an ever changing political and economic landscape.Thus the exercise is valuable in not only taking stock but also reminding those in power of an independent review process with recommendations which should transcend governments. Sadly , the focus on funding education of the workforce (recommendations 10-12) do not appear to have been a priority and without this foundation, we will not move ahead sufficiently fast with a child and family friendly service. The Children and Young Persons Outcomes Framework is similarly the result of much work in a previous government and must not be allowed to whither on the vine. Perhaps we should regularly remind policy makers in the current administration of the value of persistence with other such initiatives which have a broad professional consensus and can be dusted off and re-badged as necessary to tempt politicians to move the goal posts a little closer to what is required to optimize child health? Lets see how far we have got in another 5 years.
We would like to thank the authors of the Pavone paper for their interest in our paper (1,2). We are sorry for not quoting their paper in our study report but do confirm that we were aware of it (2). In our introduction we selected several papers to quote in order to introduce the uncertainty with respect to the need to record 1, 2 or 3 nights of overnight oximetry and the Pavone paper was not one we selected. The Pavone paper claims excellent night to night consistency in oximetry and that only one night of oximetry measurement is necessary while our study did not find this to be the case (2).
Show MoreWhile we agree that the Pavone study used a pulsed oximeter with some superior properties (Radical Masimo) compared to that which we used (Nonin 9600) we do not believe that this is one of the most important reasons why our results differ from the Pavone study.
We believe the main reasons for differences between the two papers include;
1] Different primary aims - our study was aimed to determine whether doing 2 or 3 nights oximetry would increase the chances of getting adequate traces to make a report. We therefore included all studies (whether satisfactory or not). In the Pavone study only those with 2 nights each with > 6 hours satisfactory tracing were included and about one third of the children initially identified were therefore excluded. We do not know what then happened to these children – i.e. whether further studies had to be rescheduled. Clearly selec...
The title of the paper and the majority of the introduction imply that the study is about adolescents with CFS/ME. However, the final sentence of the introduction undermines that objective: “As children in our study were not examined by a physician, we have used the term ‘chronic disabling fatigue’ (CDF) rather than CFS/ME to indicate chronic fatigue that is disabling.”
Those children may have had a variety of different diseases that cause prolonged fatigue, yet we are led to believe that a study of their collective conditions can somehow add to the body of literature on a specific disease process. CFS/ME is a highly contentious disease with a great deal of conflicting evidence and hypotheses; answers as to its exact nature and cause are as yet to be determined. By publishing a study of patients who are so poorly defined as to be undefined, Archives of Disease in Childhood has further muddied already murky waters. The addition of this study to the body of literature is not only unhelpful, but is actively detrimental to the pursuit of answers for patients with this highly disabling disease.
How are ADC or the authors able to justify publishing a study that to all appearances is about CFS/ME, yet fails to properly assess if any of the study participants actually have CFS/ME?
Dear Editor,
Show MoreWe woud like to respond to one of the issues raised in the audit of high
flow nasal cannula (HHFNC) recently published (1). As the authors observed, although evidence for efficacy is lacking,
clinical pactice has rapidly expanded the indications for respiratory
support on the ward using HHFNC. We have observed a number of cases
where commencement of HHFNC may have delayed referral to the PICU
service, and are concerned that this may have affected the level of
respiratory support required on subsequent admission to PICU. Humidified high flow nasal cannula (HHFNC) provides heated and
humidified air/oxygen flow to support respiratory function in sick
infants and children. Flow rates may be up to 60L/min, and are usually
titrated at 1-3L/kg depending on clinical work of breathing (WOB). The
concentration of oxygen may be adjusted to maintain oxygen saturations
within the normal range for each child. Pediatric units providing this
therapy, usually do so within agreed guidelines. Some units mandate
admission to the Pediatric Intensive Care Unit (PICU), and some
administer HHFNC on the ward. There is some evidence that the use of
HHFNC may reduce the need for PICU admission and more advanced
respiratory support (2). However, studies to date have not stratified
infants further, into categories of risk of failure of therapy (3). Yet
infants with significan...
Dear Editor,
We thank Professor Wright for her comments, and we welcome the opportunity to provide some clarification and further analysis.
We reported Z-scores rather than percentiles, although some comments on approximate percentiles can be made. Assuming that Z scores of -1.96 and -3 represent approximately the 2.5th and 0.2nd centiles respectively, 36/101 children were below the 2.5th centile, and 17/101 were below the 0.2nd centile for weight. Additionally, our mixed effects model (accounting for multiple measurements) modelling the group trend over time estimated the mean weight Z score at 11 years to be -1.63 (approximately 5th centile).
Despite the overall short stature of the group, 24/101 children had a BMI Z score of less than -1.96. So, by this approach, their weight was low even after taking into account stature. We agree that we cannot infer causality from this observational study, but we believe a proportion of the stunted growth is explained by low weight. We are exploring other measures of malnutrition, such as skin-fold thickness.
Whilst our patient numbers are small, they do give some weight to the argument that PEG feeding halts the progression of malnutrition. We investigated the rate of decline of weight after PEG insertion. In a mixed effects model with a random intercept for individual patients, the rate of wei...
Show MoreI agree with Dr. Goldwater that an undetected prodromal respiratory infection can suddenly fulminate and cause acute anoxic encephalopathy. In such an instance, there may not be time for visible pulmonary histological pathology to form. Then if a lung culture is not performed or gives sepsis-negative results, the cause may be coded as SIDS rather than an ARI. See Farber S. Fulminating streptococcus infections in infancy as a cause of sudden death. N Engl J Med 211:154-158, 1934 and Mage et al. .Front Neurol. 2016 Aug 23;7:129. doi: 10.3389/fneur.2016.00129. eCollection 2016. PubMed ID 27602017
I wonder if this brief report by Harvey et al. highlights where we are going wrong. Firstly, the lack of response to the QIP may just reflect the fact that we have such limited ability to influence outcomes when it comes to childhood obesity. If you are working in a busy CAU it seems pointless doing things that are not going to produce a positive outcome.
Show MoreHowever my biggest concern is the statement: "How paediatricians act has a large impact on parents: we cannot expect them to prioritise their child’s obesity if we do not do the same." This appears to be the “nanny state” at work. The fact that parents are not recognising their children’s obesity, if this is really the case given the publicity this topic is receiving, is the main problem. This idea that patients are completely dependent on professionals to bring about change influences the outcome for many chronic conditions. Best results are obtained when patients (and carers) are actively involved in the management of the disease and are equipped to influence outcomes. This can only come about through education.
My personal experience is that I cannot remember ever seeing an overweight child maintain any significant weight loss. The lack of parental recognition of the fact that their child is overweight is a major problem. I am not sure how long the comment "your child is overweight" stays with parents after they leave the clinic. Do parents feel that an overweight child reflects well on...
The paper by Yang et al1 provided an interesting epidemiological picture regarding the healthcare use in the year before correct diagnoses are confirmed for childhood cancer, type 1 diabetes mellitus (T1DM) and other immune diseases. Despite the presence of known clinical presentations associated with these diseases, diagnoses are not usually made until after couples of medical visits, except in cases with T1DM. Nearly two thirds of newly diagnosed T1DM patients presented emergent diabetic ketoacidosis. This rate was similar to that reported in a single-center Taiwanese study2 but still much higher than those in the US and Europe.3,4 This finding raised a question whether diabetic ketoacidosis at diagnosis of T1DM was a result of missed recognition for diabetic symptoms. In this regard, I am surprised that common urological symptoms, such as proteinuria and polydipsia2, found in Taiwanese T1DM patients were not included in the ICD-9 codes grouped for the urogenital problems, although the data showed an increase in urogenital problems shortly before the diagnosis of T1DM.1 From a clinical perspective, it is also crucial to know how the access to healthcare before diagnosis differ between those with and without diabetic ketoacidosis.3 If the analysis can be stratified by this factor, we may better evaluate the performance and impact of pre-diagnostic outpatient visits on subsequent healthcare for T1DM. There is always room for improvement in terms of increasing awareness of...
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