• Storytelling and poetry in the time of coronavirus.

      Barrett, Elizabeth; Dickson, Melissa; Hayes-Brady, Clare; Wheelock, Harriet (2020-05-14)
      The coronavirus crisis occurs at a time when many clinicians have already experienced burnout. One in three Irish doctors were suffering from burnout in the 2019 National Study of Wellbeing of Hospital Doctors in Ireland; rates are also high in Irish Psychiatry. We present a perspective on the use of narrative in medicine and recognise that storytelling, and the patient history are very much at the heart of medicine. Clinician storytelling, such as Schwartz Rounds and Balint group work, has very much come to the fore in Irish Psychiatry and in training. Projects such as MindReading have explored overlaps between clinicians, humanities experts and experts by experience. We give an overview of some approaches from the movement around narrative in medicine to bolster this. We explore why clinicians write as ways to support identification, catharsis and a way to process experiences. Clinicians and patients may also use literature and poetry to promote coping. The historical context and practical strategies are highlighted, particularly with reference to poetry use during the current crisis.
    • Key Performance Indicators in Paediatric Anaesthesia

      Doody, K; Barry, D; Holmes, C (Irish Medical Journal, 2019-07)
      Currently no national guidelines on performance measurement exist for paediatric anaesthesia in Ireland1. The purpose of this study was to ascertain if we are achieving Key Performance Indicators (KPIs) in areas of post-operative nausea and vomiting (PONV) and post-operative pain when compared to international standards.
    • Caring for Caregivers: An Evaluation of Schwartz Rounds in a Paediatric Setting

      Silke, A; Rushe, H; Keating, K; Thurstan, R; Barrett, E (Irish Medical Journal, 2019-06)
      Schwartz rounds (SR) are a multi-disciplinary intervention that aim to support clinical and non-clinical healthcare professionals in their work. Temple Street Children’s University Hospital (TSCUH) is the first paediatric hospital to introduce SR. SR are a popular intervention, with numerous sites adopting them in the US and the UK. First introduced in Ireland in 2015, they were piloted at sites in Galway University Hospital and Blackrock Hospice. SR have since spread to 15 other sites across Ireland, including regional hospitals, children’s hospices and ambulance services. 2 Only one paper has been published on the topic of SR in paediatric hospitals. This paper hopes to highlight the potential for SR in the paediatric context by evaluating the views of staff who attended SR at TSCUH.
    • Primary External Ventricular Drains in the Management of Open Myelomeningocele Repairs in the Neonatal Setting in Ireland

      Finnegan, R; Kehoe, J; McMahon, O; Donoghue, V; Crimmins, D; Caird, J; Murphy, J (Irish Medical Journal, 2019-05)
      The aim of this study is to outline the role of primary external ventricular drains (EVD) in the management of open myelomeningoceles in the neonatal setting in Ireland.
    • Is It Time To Review The Vaccination Strategy To Protect Adults Against Invasive Pneumococcal Disease?

      Corcoran, M; Mereckiene, J; Murchan, S; McElligott, M; O’Flanagan, D; Cotter, S; Cunney, R; Humphreys, H (Irish Medical Journal, 2019-03)
      Pneumococcal conjugate vaccines (PCVs) have reduced the predominant serotypes causing invasive pneumococcal disease (IPD). We assessed the impact of the paediatric 7- and 13-valent pneumococcal conjugate vaccines (PCV7 and PCV13) among older adults. We compared serotype-specific incidence rates from 2007/08 to 2016/17, expressed as incidence rate ratios (IRR). Introducing PCV7 and PCV13 into the childhood immunisation programme resulted in a decline in these serotypes in adults ≥65 years of age, with PCV7 serotypes decreasing by 85% (IRR=0.11, 95%CI: 0.05-0.22, p<0.0001) and PCV13 serotypes not included in PCV7 (PCV13-7), decreasing by 9% (IRR=0.68, 95%CI: 0.40-1.16, p=0.134). However, there was a significant increase in serotypes only found in the 23-valent polysaccharide vaccine, PPV23-PCV13: IRR=2.57, 95%CI: 1.68-4.03, p<0.0001, and non-vaccine types (NVTs), IRR=3.33, 95%CI: 1.75-6.84, p=0.0001. The decline of IPD associated with PCV7/13 serotypes and the increase in PPV23-PCV13 serotypes indicates clear serotype replacement. Increasing PPV23 uptake could still reduce the burden of disease for this population.
    • cgMLST characterisation of invasive Neisseria meningitidis serogroup C and W strains associated with increasing disease incidence in the Republic of Ireland.

      Mulhall, Robert M; Bennett, Desiree E; Bratcher, Holly B; Jolley, Keith A; Bray, James E; O'Lorcain, Piaras P; Cotter, Suzanne M; Maiden, Martin C J; Cunney, Robert J (2019-01-01)
      Since 2013 MenC and MenW disease incidence and associated mortality rates have increased in the Republic of Ireland. From 2002/2003 to 2012/2013, the average annual MenC incidence was 0.08/100,000, which increased to 0.34/100,000 during 2013/2014 to 2017/18, peaking in 2016/17 (0.72/100,000) with an associated case fatality rate (CFR) of 14.7%. MenW disease incidence has increased each year from 0.02/100,000 in 2013/2014, to 0.29/100,000 in 2017/18, with an associated CFR of 28.6%. We aimed to characterise and relate recent MenC isolates to the previously prevalent MenC:cc11 ET-15 clones, and also characterise and relate recent MenW isolates to the novel 'Hajj' clones.
    • Hyperammonaemia in Neonates and Young Children: Potential Metabolic Causes, Diagnostic Approaches and Clinical Consequences

      Giva, S; Finnegan, J; Ihidero, P; Maguire, G; Power, B; Knerr, I; Monavari, A (Irish Medical Journal, 2019-01)
      Hyperammonaemia is a metabolic disturbance characterized by accumulation of ammonia in the blood. Entry of ammonia into the brain via the blood-brain barrier leads to hyperammonaemic encephalopathy. The causes of hyperammonaemia in paediatric patients vary. We present 3 cases of hyperammonaemia in critically ill children in whom an inborn metabolic disorder was identified and provide insights into the phenotypes, diagnostic approaches and management. In children with acute overwhelming illness and progressive neurological deterioration plasma ammonia measurement should be included in the urgent diagnostic work-up. We here raise the awareness that hyperammonaemia is a metabolic emergency requiring prompt recognition and treatment to avoid subsequent complications.
    • Enterovirus and parechovirus meningitis in infants younger than 90 days old in the UK and Republic of Ireland: a British Paediatric Surveillance Unit study.

      Kadambari, Seilesh; Braccio, Serena; Ribeiro, Sonia; Allen, David J; Pebody, Richard; Brown, David; Cunney, Robert; Sharland, Mike; Ladhani, Shamez (2018-12-08)
      Objectives: This study aimed to prospectively collect detailed clinical information for all enterovirus (EV) and human parechovirus (HPeV) meningitis cases in infants aged <90 days in the UK and Ireland. Participants, design and setting: Prospective, active national surveillance during July 2014 to July 2015 through the British Paediatric Surveillance Unit. Reporting paediatricians completed questionnaires requesting information on clinical presentation, investigations, management and outcomes at hospital discharge and after 12 months. Main outcome measures: To describe the clinical burden of EV and HPeV meningitis in infants aged <90 days. Results: During the 13-month surveillance period, 703 cases (668 EV, incidence0.79/1,000 live- births; 35 HPeV, 0.04/1,000 live-births) were identified. The most common clinical presentations were fever (EV: 570/668(85%); HPeV: 28/35(80%)), irritability (EV: 441/668(66%); HPeV: 23/35(66%)) and reduced feeding (EV: 363/668(54%); HPeV 23/35(66%)). Features of circulatory shock were present in 27% (182/668) of EV and 43% (15/35) of HPeV cases. Overall, 11% (76/668) of EV and 23% (8/35) of HPeV cases required intensive care support. Nearly all cases (678/703, 96%) were confirmed by cerebrospinal fluid (CSF) PCR, with 52% (309/600) having normal CSF white cell count for age. Two infants with EV meningitis died (2/668, 0.3%) and four survivors (4/666, 0.6%) had long-term complications at 12 months' follow-up. Infants with HPeV meningitis survived without sequelae. Overall 189 infants had a formal hearing test and none had sensorineural hearing loss. Conclusion: The incidence of laboratory-confirmed EV/HPeV meningitis in young infants is more than twice that for bacterial meningitis. Less than 1% will develop severe neurological complications or die of their infection. Further studies are required to formally assess long-term neurodevelopmental sequelae.
    • Heterozygous loss-of-function variants of MEIS2 cause a triad of palatal defects, congenital heart defects, and intellectual disability.

      Verheije, Rosalind; Kupchik, Gabriel S; Isidor, Bertrand; Kroes, Hester Y; Lynch, Sally Ann; Hawkes, Lara; Hempel, Maja; Gelb, Bruce D; Ghoumid, Jamal; D'Amours, Guylaine; et al. (2018-10-05)
      Deletions on chromosome 15q14 are a known chromosomal cause of cleft palate, typically co-occurring with intellectual disability, facial dysmorphism, and congenital heart defects. The identification of patients with loss-of-function variants in MEIS2, a gene within this deletion, suggests that these features are attributed to haploinsufficiency of MEIS2. To further delineate the phenotypic spectrum of the MEIS2-related syndrome, we collected 23 previously unreported patients with either a de novo sequence variant in MEIS2 (9 patients), or a 15q14 microdeletion affecting MEIS2 (14 patients). All but one de novo MEIS2 variant were identified by whole-exome sequencing. One variant was found by targeted sequencing of MEIS2 in a girl with a clinical suspicion of this syndrome. In addition to the triad of palatal defects, heart defects, and developmental delay, heterozygous loss of MEIS2 results in recurrent facial features, including thin and arched eyebrows, short alae nasi, and thin vermillion. Genotype-phenotype comparison between patients with 15q14 deletions and patients with sequence variants or intragenic deletions within MEIS2, showed a higher prevalence of moderate-to-severe intellectual disability in the former group, advocating for an independent locus for psychomotor development neighboring MEIS2.
    • Potential Coverage of the 4CMenB Vaccine against Invasive Serogroup B Isolated from 2009 to 2013 in the Republic of Ireland.

      Mulhall, Robert M; Bennett, Desiree; Cunney, Robert; Borrow, Ray; Lucidarme, Jay; Findlow, Jamie; Jolley, Keith A; Bray, James; Maiden, Martin C J; Moschioni, Monica; et al. (2018-08-22)
      Neisseria meningitidis is a common cause of bacterial meningitis in children and young adults worldwide. The 4CMenB vaccine (Bexsero), developed to combat meningococcal serogroup B (MenB) disease, contains subcapsular antigens that may induce immunity against strains of N. meningitidis, regardless of serogroup. Owing to differential levels of expression and peptide diversity in vaccine antigens across meningococcal strains, the meningococcal antigen typing system (MATS) was developed to estimate the potential MenB strain coverage of 4CMenB. Prior to introducing the 4CMenB vaccine into routine use, we sought to estimate the potential 4CMenB coverage against invasive MenB strains isolated in the Republic of Ireland (RoI) over four consecutive epidemiological years. MATS was applied to a panel of 105 invasive MenB strains isolated during July 2009 to June 2013. Sequence data characterizing the multilocus sequence typing (MLST) alleles and the major 4CMenB target peptides were extracted from isolate genome sequence data, hosted in the Bacterial Isolate Sequencing database (BIGSdb). MATS data indicated that 4CMenB may induce protective immunity against 69.5% (95% confidence interval [CI95%], 64.8% to 84.8%) of circulating MenB strains. Estimated coverage was highest against the most prevalent disease-causing lineage, cc41/44, where the most frequently observed sequence types, ST-154 and ST-41 (21% of isolates, collectively), were typically covered by three antigens. No significant temporal trends were observed. Overall, these data provide a baseline of strain coverage prior to the introduction of 4CMenB and indicate that a decrease in invasive meningococcal disease (IMD) is predicted following the introduction of 4CMenB into the routine infant immunization schedule in the RoI.IMPORTANCE The meningococcal antigen typing system (MATS) is an enzyme-linked immunosorbent assay (ELISA) that measures both the levels of expression and the immune reactivity of the three recombinant 4CMenB antigens. Together with PorA variable-region sequence data, this system provides an estimation of how susceptible MenB isolates are to killing by 4CMenB vaccine-induced antibodies. Assays based on subcapsular antigen phenotype analyses, such as MATS, are important in situations where conventional vaccine coverage estimations are not possible. Subcapsular antigens are typically highly diverse across strains, and vaccine coverage estimations would require unfeasibly large efficacy trials and screening of an exhaustive strain panel for antibody functional activity. Here, MATS was applied to all invasive meningococcal serogroup B (MenB) strains isolated over four consecutive epidemiological years (n = 105) and predicted reasonably high 4CMenB vaccine coverage in the Republic of Ireland.
    • Bringing the Board of Directors on Board with Quality and Safety of Clinical Care

      Temple Street Children’s University Hospital; HSE Quality Improvement Division (Health Service Executive, 2018-08)
      The genesis of this project was about bringing the Temple Street Children’s University Hospital Board of Directors on a journey, which would result in the Board holding the hospital Executive accountable for the quality of clinical care delivered. It was a collaboration between the Board, the Project Team and the HSE Quality Improvement Division. Governing Boards of healthcare organisations are responsible for their organisations’ performance (HSE 2017). Prior to this project Temple Street Children’s University Hospital (TSCUH) Board of Directors received operational information on access, efficiency, human resources and finance indicators through a monthly balanced score card report, while the quality indicators were reported quarterly. Data on the score card were presented using a red, amber and green speedometer with an associated line chart, which demonstrated if the desired target was achieved.
    • Further Reductions in Road-Related Deaths and Injuries in Irish Children

      Garry, E; Donnelly, J; Heffernan, S; Mc Garvey, C; Nicholson, AJ (Irish Medical Journal, 2018-04)
      The aim was to study road-related injuries and fatalities in under 15-year-olds in three time periods (1996-2000, 2004-2008 and 2009 -2013 respectively) to assess whether progress has been made via cross-sectoral efforts (legislation, public awareness campaigns and police enforcement) to reduce this injury toll in Ireland. For road traffic collisions where an injury has occurred, police assistance is required and at the time a detailed CT 68 form is completed by the attending police officer and sent to the Road Safety Authority for analysis. Details regarding the severity of injury, light and road conditions and safety measures such as seat belt or car restraint use, seat position and helmet use if a cyclist is involved are recorded. Injuries were sub-classified as fatalities, serious (detained in hospital, fractures, severe head injury, severe internal injuries or shock requiring treatment) or minor. All data for the three time periods was entered onto an SPSS database. A concerted national campaign re road safety media campaign allied to random breath testing, penalty points for driving offences, on the spot fines for speeding and greater police enforcement took place over the 17-year timeframe and continues to this day. When results were compared between the three cohorts, total injuries dropped from 5928 (1996-2000) to 3903 (2009-2013).Fatal injuries dropped from 163 to 43 with car occupant fatalities fell from 69 to 17 between 1996-2000 and 2009-2013. Serious injuries dropped from 347 in the first cohort to 201 in the third cohort. Minor injuries fell from 5,063 to 3,659 between first and last cohort. Pedestrian injuries dropped from 1719 to 1258 with pedestrian fatalities decreased from 61 (1996-2000) to 21 (2009-2013) and serious pedestrian injuries decreased from 261 down to 129. Cyclist fatalities saw the most significant fall (76%) with a dramatic reduction in fatalities from 25 down to 6. A national road safety campaign, greater police enforcement and a cultural change has seen road-related deaths and injuries in children drop very significantly (by over 70%) over the three time periods (spanning 1996 to 2013) and this campaign should continue.
    • New Frontiers in the Treatment of Spinal Muscular Atrophy

      Power, CL; O’Rourke, DJ (Irish Medical Journal, 2018-03)
      Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disorder, with a current estimated incidence of 1 in 11,000 live births. Although there is a variable phenotype, 60% of patients with SMA have type 1 disease. Typically diagnosed by the age of six months, this severe form of the condition is characterised by progressive weakness and the failure to meet motor milestones. There is an early need for permanent assisted ventilation, without which the median life expectancy is less than two years. Proper maintenance and function of motor neurons in the brainstem and spinal cord is dependent on optimum production of survival motor neuron (SMN) protein. Two genes are responsible for this production: SMN1 and SMN2. SMN1 codes for approximately 90% of SMN protein; homozygous deletion or mutation of this gene results in the clinical presentation of SMA. Copy number variability has been observed with SMN2; those with a higher copy number tend to present with a less severe phenotype. Therefore, increasing the production of SMN protein by SMN2 represents a clear therapeutic approach to SMA.
    • A Cluster of Paediatric Invasive Group A Streptococcal and Chicken Pox Infections

      Ó Maoldomhnaigh, C; Butler, K; Gavin, P (Irish Medical Journal, 2018-03)
      Group A streptococcus (GAS) causes a variety of acute clinical syndromes from pharyngitis and scarlet fever commonly seen in primary care to more severe life-threatening invasive disease. Invasive GAS, categorised into three groups - necrotising fasciitis, streptococcal toxic shock syndrome and sepsis with or without an identifiable source of infection- is a notifiable disease to the Health Protection Surveillance Centre (HPSC)1. Laboratory criteria for a confirmed case require isolation of GAS from a normally sterile site. The HPSC previously reported a marked increase in the incidence of invasive GAS infections from 1.65/100,000 population in 2011 to 3.65/100,000 in 20132. The increased incidence was notable also for a 300% increase in the proportion of invasive GAS cases in children. After a slight decrease in incidence in 20153 (2.3/100000), we noted a cluster of invasive GAS cases referred to the paediatric infectious disease (PID) department of Children’s University Hospital (CUH), Temple Street, in 2016. We sought to further characterise this cluster of paediatric invasive GAS infections.
    • A Review Of Referral Patterns For Sagittal Synostosis In Ireland: 2008-2013

      Berney, M J; McGillivary, A; Caird, J; Murray, D J (Irish Medical Journal, 2018-01)
      Sagittal synostosis (SS) is the commonest form of craniosynostosis. Children with sagittal synostosis in Ireland are treated in the National Paediatric Craniofacial Centre (NPCC) in Temple Street Children’s University Hospital. This retrospective study analysed the correlation between referral patterns to the unit and age at operation. The notes of 81 patients referred over a 5 year period (April 2008 – April 2013) to the NPCC with non-syndromic SS were reviewed and demographics and referral information were recorded. Of 81 patients reviewed, 60 (74%) were referred before 6 months of age, while 21 (26%) had late referrals. Neonatologists referred 100% of infants before 6 months, paediatricians referred 71%, and GPs 64%. Later referral was associated with a more complex referral pathway, including multiple-steps of referral and unnecessary investigations. Improved clinician knowledge and emphasis on the importance of early referral may lead to a reduction in late referrals.
    • Paediatric Type 2 Diabetes Still Rare in an Irish Tertiary Referral Unit

      Kernan, R; Carroll, A; Mc Grath, N; Mc Donnell, CM; Murphy, NP (Irish Medical Journal, 2018-01)
      While Type 2 Diabetes in childhood has become increasingly prevalent throughout the world, in our service we found that only 2% (7/320) of children and adolescents with diabetes aged < 16 years had type 2 diabetes. All type 2 subjects were overweight or obese and six of seven were non-Caucasian. Mean age at presentation was 12.8 years. Six patients (85%) had complications, most commonly hypertension. Although Type 2 Diabetes in children remains relatively rare in our cohort, identification of these children is important as management differs from Type 1 Diabetes.
    • Iron Refractory Iron Deficiency Anaemia: A Rare Cause of Iron Deficiency Anaemia

      McGrath, T; Cotter, M (Irish Medical Journal, 2018-01)
      We describe the case of a 17-month-old boy with a hypochromic microcytic anaemia, refractory to oral iron treatment. After exclusion of dietary and gastrointestinal causes of iron deficiency, a genetic cause for iron deficiency was confirmed by finding two mutations in the TMPRSS6 gene, consistent with a diagnosis of iron-refractory iron deficiency anaemia (IRIDA).
    • Rotavirus Gastroenteritis is Associated with Increased Morbidity and Mortality in Children with Inherited Metabolic Disorders

      Smith, A; Mannion, M; O’Reilly, P; Crushell, E; Hughes, J; Knerr, I; Gavin, P; Monavari, A (Irish Medical Journal, 2017-04)
      Rotavirus is the leading cause of infantile diarrhoea worldwide in children <5 years1. Although mortality rates are low in Ireland, certain populations are more susceptible to the associated morbidity and mortality of infection. A retrospective chart review of 14 patients with confirmed IMDs who were admitted to Temple Street Children’s Hospital between 2010 to 2015 with rotavirus infection were compared with 14 randomly selected age matched controls. The median length of stay was 7 days (SD25.3) in IMD patients versus 1.5 days (SD 2.1) in the controls. IV fluids were required on average for 4.5 days (range 0-17) in IMD patients versus 0.63 days (range 0-3) in controls. This report highlights the increased morbidity of rotavirus infection in patients with IMD compared to healthy children. This vulnerable population are likely to benefit from the recent introduction of the rotavirus oral vaccination in October 2016.
    • Caring for Obese Children- A change in Paradigm

      Doyle, S; Cahill, D; Smyth, M; Murphy, S (Irish Medical Journal, 2017-04)
      Childhood Obesity is a problem of epidemic proportions. The causes are complex and treatment results are variable with much research ongoing. We analysed the initial assessment forms of a group of patients attending the W82GO! Healthy Lifestyle service at The Children’s University Hospital, Temple Street to look at the population and their specific needs. Our analysis revealed a high proportion of emotional and behavioural problems along with bullying. This group of patients are complex and a multi-disciplinary team approach is essential in their treatment.
    • Out of sight, out of mind? A national survey of paediatricians in Ireland regarding Fetal Alcohol Spectrum Disorders

      Gill, I; Sharif, F (Irish Medical Journal, 2017-03)
      Fetal Alcohol Spectrum Disorders (FASDs) are one of the major causes of preventable developmental delay. There is no register of children with FASDs in Ireland. Up to 81% of Irish women report drinking alcohol during the periconceptual period or pregnancy. We aimed to evaluate self-reported knowledge and practice of doctors working in paediatrics in Ireland with regards to FASDs and alcohol consumption during pregnancy. We circulated a survey to all paediatric doctors in Ireland, either enrolled in specialist training or registered as trainers. Fifty-six respondents (31.3%) were unaware of the existence of FASDs. Sixty-two (34.6%) believed most patients with FASDs have dysmorphic features. Seventy-three respondents (40.8%) routinely ask about maternal alcohol consumption during pregnancy in the context of developmental delay. Thirty-one respondents (17.3%) stated that mild alcohol intake in the third trimester of pregnancy is safe. Our survey suggests prenatal alcohol exposure may not be routinely considered in the evaluation of children with developmental delay by paediatric doctors in Ireland.