Innovation in Spinal Muscular Atrophy treatment

In this episode, Dr Felicity Cooksey speaks to Dr Kate McCann about adverse childhood experiences. Dr Kate McCann is a community child health consultant and member of the Trauma Informed Strategy Group in Somerset. We discuss how adverse childhood experiences may impact a child’s development and the importance of recognising and responding to childhood trauma. Recommended Resources: TED Talks • Dr Nadine Burke Harris – TED talk: How childhood trauma affects health across a lifetime. • Molly Wright- TED talk: How Every Child Can Thrive by Five Articles• Felitti VJ, Anda RF, Nordenberg D, Williamson DF, Spitz AM, Edwards V, Koss MP, Marks JS. Relationship of childhood abuse and household dysfunction to many of the leading causes of death in adults. The Adverse Childhood Experiences (ACE) Study. Am J Prev Med. 1998 May;14(4):245-58.• Nelson CA, Scott RD, Bhutta ZA, Harris NB, Danese A, Samara M. Adversity in childhood is linked to mental and physical health throughout life. BMJ. 2020 Oct 28;371:m3048. Books• The Body Keeps the Score by Bessel van der Kolk• The Deepest Well by Dr Nadine Burke Harris• Goodnight Mister Tom by Michelle Magorian• The Secret Garden by Frances Hodgson BurnetWebsites• The Bucharest Early Intervention Project. Available at https://www.bucharestearlyinterventionproject.org/• Center on the Developing Child at Harvard University https://developingchild.harvard.edu/ • EHCAP- Mindful Emotion Coaching https://mindfulemotioncoaching.co.uk/ • UK trauma council https://uktraumacouncil.org/• Oxford Brain Story https://www.oxfordbrainstory.org/

This episode, the Paedshub Pod team got together for our annual roundup. Felicity Cooksey, Leila Ellis, Lorna Hack and Paulina Connolly discuss 12 articles that we enjoyed over the past year from ADC. Happy New Year!   Articles & related resources: 1. Barriers to initiating and implementing palliative and end of life care for children with life-limiting conditions- A mixed methods study in a UK Children’s Hospital. https://adc.bmj.com/content/107/Suppl_2/A299.1   2. Pharmacokinetic and pharmacodynamic data from the NEOLEV1 and NEOLEV2 studies.  https://adc.bmj.com/content/109/10/854   3. Family poverty, neighbourhood greenspace and perinatal outcomes. https://adc.bmj.com/content/109/12/1017   4. Near-fatal and fatal asthma and air pollution: are we missing an opportunity to ask key questions? https://adc.bmj.com/content/109/8/616   5. Fifteen-minute consultation: The prepubescent gender-diverse child: how to answer parents’ questions.  https://ep.bmj.com/content/109/3/106 Additional resources: https://cass.independent-review.uk/home/publications/final-report/   6. Children born to subfertile couples, how are they doing? Evidence from research. https://adc.bmj.com/content/early/2024/04/07/archdischild-2023-326023   7. Delivery room dextrose gel for preterm hypoglycaemia (the GEHPPI study): a randomised placebo-controlled trial. https://fn.bmj.com/content/early/2024/11/17/archdischild-2024-327313   8. Fifteen-minute consultation: Group A streptococcal pharyngitis, diagnosis and treatment in children. https://ep.bmj.com/content/109/5/210 Additional resources: FeverPAIN score: https://www.mdcalc.com/calc/3316/feverpain-score-strep-pharyngitis#evidence   9. Nasogastric tube feeding under physical restraint of children and young people with mental disorders: a comprehensive audit and case series across paediatric wards in England. https://adc.bmj.com/content/109/8/649   10. Recognition, prevention and management of ‘digital harm’.  https://ep.bmj.com/content/109/3/120 Additional resources: https://www.ceopeducation.co.uk/ https://www.childline.org.uk/info-advice/bullying-abuse-safety/online-mobile-safety/report-remove/?gclsrc=aw.ds&gad_source=1&gclsrc=ds https://www.parentalminds.org.uk/wp-content/uploads/2023/12/LMHS-Thrive-Directory-181223.pdf https://learninghub.nhs.uk/Catalogue/Internet-safety-suicide-self-harm https://mindedforfamilies.org.uk/Content/parenting_in_a_digital_world/#/id/5e30acc07c501d4bd0554ad5 https://www.nspcc.org.uk/keeping-children-safe/online-safety/ https://www.childrenscommissioner.gov.uk/digital/5-a-day/ https://uniquevoice.org/online-safety/   11. Achieved oxygen saturations and risk for bronchopulmonary dysplasia with pulmonary hypertension in preterm infants. https://adc.bmj.com/content/109/11/941   12. Fifteen-minute consultation: Empowering children, young people and families through shared decision-making: a practical guide.  https://ep.bmj.com/content/109/6/264     Our entertainment recommendations: Books: Midnight's children by Salman Rushdie The Salt Path by Raynor Winn Lessons in Chemistry by Bonnie Garmus Comic: The Weight of Expectation ⁠https://actwithlove.co.uk/woe⁠ Films & Series: Squid Games (Netflix) Film: The Green Book (2018)

In this episode Dr Lorna Hack speaks to Dr Anirban Majumdar about Spinal Muscular Atrophy, and the remarkable advances made in managing this condition in the twenty-first century. Dr Majumdar is a Consultant Paediatric Neurologist at the Bristol Royal Hospital for Children, and delivers the regional Neuromuscular Service. We discuss: the genetic basis of SMA, the targets of disease modifying treatments, the pros and cons that guide treatment choice, and monitoring of treatment efficacy. Article: https://adc.bmj.com/content/108/Suppl_2/A199 Dr Majumdar’s book recommendations: ‘Yellowface’ by R. F. Kuang and ‘The Spanish Civil War’ by Antony Beevor.

In this episode, Dr Felicity Cooksey talks with Dr Marie Monaghan about idiopathic intracranial hypertension. Dr Monaghan is a general paediatric consultant with a specialist interest in epilepsy, based at Bristol Royal Hospital for Children. She co-authored a paper titled ‘Consensus recommendations for the assessment and management of idiopathic intracranial hypertension in children and young people’, which was published in Archives of Disease in Childhood. Dr Monaghan discusses her experiences of working abroad, why cases of idiopathic intracranial hypertension are predicted to rise and how to approach a suspected case. Link to the full article: ⁠Consensus recommendations for the assessment and management of idiopathic intracranial hypertension in children and young people | Archives of Disease in Childhood (bmj.com)⁠

In this episode, Dr Leila Ellis welcomes Nicola O'Donnell to the podcast to discuss psychosocial support for childhood cancer survivors. Nicola is a Health Psychologist and is completing the final year of a PhD with the University of York exploring psychoeducation interventions for young people with retinoblastoma funded by the Childhood Eye Cancer Trust (CHECT). Resources: An overview of Acceptance and Commitment Therapy⁠ ⁠An overview of Compassion Focused Therapy⁠ Charities: ⁠The Teenage and Young Adult Cancer Trust (TYAC)⁠: TYAC is part of the Royal Marsden Cancer Charity and offers specialised support for teenagers and young adults with cancer, including counselling  Lennox Children’s Cancer Fund: offer family-wide support including a 24-hour phone line, local community support in your area, and grants for professional counselling ⁠Place2Be⁠: offer children and young people’s mental health support at school Maggie's Centres⁠: Maggie's Centres have have several locations across the UK and provide free practical, emotional, and social support to people with cancer, including children and teenagers Rainbow Trust⁠: offer whole family support including a space to listen to family’s fears and anxieties, helping to explain illnesses, diagnoses and treatments, support through grief and bereavement ⁠Winston’s Wish⁠: helps children, teenagers and young adults when experiencing grief ⁠Together for Short Lives⁠: provide a phone line for any parent, carer or professional who looks after or knows a child or young person who is expected to have a shorter life expectancy ⁠Child Bereavement UK⁠: support families when a child grieves or when a child dies. Including a helpline, or face-to-face support in certain locations Support lines: IAM (Teenage Cancer Trust)⁠: emotional and clinical support tool for young people who’ve had cancer ⁠Connect (Teenage Cancer Trust)⁠: supportive online community for young people going through or recovering from cancer Young Lives vs. Cancer: phone 0300 303 5220 or email [email protected] Macmillan: call 0808 808 00 00 or chat online Child Death Helpline: phone 0800 282 986 for free telephone service for anyone affected by the death of a child Young Minds: text ‘YM’ to 85258 for free 24/7 support The Mix – Crisis Messenger: text THEMIX to 85258 for free 24/7 crisis support across the UK Samaritans: phone 116 123 or email [email protected] for 24/7 free support Shout: text ‘SHOUT’ to 85258 for free 24/7 support NHS psychological therapies (IAPT) (18+): self-referral for free talking therapies Book recommendation: A Heart That Works by Rob Delaney Please let us know what you thought of this episode. Your feedback will help us keep the podcast relevant to you. Let us know what you think about the format, length, choice of articles or anything else. Perhaps you’d like to suggest an article for a future episode? Here are your options for getting in touch: Leave a comment below Use our feedback form (which allows you to give anonymous feedback) Email us at [email protected]

In this episode Dr Lorna Hack speaks to Dr Dinesh Giri about obesity, it's growing prevalence and associated complications. Tertiary management for obese children is discussed, including the increasing use of medication. Dr Giri is a Paediatric Endocrine Consultant at the Bristol Royal Hospital for Children, and is part of the team who run the weight management service. Dr Giri's book recommendation: 'The Secret' by Rhonda Byrne Articles discussed: Gap in obesity monitoring in preschool children: https://adc.bmj.com/content/108/Suppl_2/A64.2 Review of paediatric fatty liver disease: https://adc.bmj.com/content/106/1/3 Waist-to-height-ratio superior to BMI in identifying obese children: https://www.nature.com/articles/s41390-024-03112-8 Resources for families: https://beezeebodies.com/resources/ Thank you for listening!

In this episode Dr Paulina Connolly welcomes Professor Ramanan to the Podcast. Professor Ramanan is a Consultant Paediatric Rheumatologist at Bristol Royal Hospital for Children, and Associate Editor of Archives of Disease in Childhood. They explore the evidence behind current treatment strategies for Kawasaki disease, reviewing the recent paper from Archives titled 'Infliximab for intensification of primary therapy for patients with Kawasaki disease and coronary artery aneurysms at diagnosis' The full link to the article can be found below: https://adc.bmj.com/content/108/10/833 Prof Ramanan also references the recent editorial in ADC 'Thinking beyond intravenous immunoglobulin for Kawasaki disease' which provides an overview of the current adjunctive treatments and supporting evidence: https://adc.bmj.com/content/108/10/781

In this episode, Dr Felicity Cooksey talks with Dr Denize Atan about nutritional blindness. Dr Atan is an Associate Professor and an Honorary Consultant in Neuro-ophthalmology at the Bristol Eye Hospital. She co-authored a review published in Archives of Disease in Childhood titled ‘Nutritional blindness from avoidant-restrictive food intake disorder- recommendations for the early diagnosis and multidisciplinary management of children at risk from restrictive eating.’ The link to the full article can be found below: https://adc.bmj.com/content/early/2023/07/05/archdischild-2022-325189 Dr Atan discusses the importance of asking about diet, why growth measurements can lead to false reassurance and the traffic light algorithm they propose for managing patients with ARFID who are at risk of visual and other neurological complications. Dr Atan’s book recommendation was ‘Lessons in Chemistry’ by Bonnie Garmus.

This episode the Paedshub Pod team Felicity Cooksey, Leila Ellis and Paulina Connolly discuss 12 articles that we enjoyed over the past year from ADC. Happy Christmas!   Leila's recommendations from Fetal and Neonatal:   Marshall A, Ladlow OJ, Bannink C, et al. Apnoea-triggered increase in fraction of inspired oxygen in preterm infants: a randomised cross-over study Archives of Disease in Childhood - Fetal and Neonatal Edition. https://fn.bmj.com/content/early/2023/08/28/archdischild-2023-325849   Cromb D, Hall M, Story L, et al. Clinical value of placental examination for paediatricians. Archives of Disease in Childhood - Fetal and Neonatal Edition https://fn.bmj.com/content/early/2023/09/26/archdischild-2023-325674   Suonpera E, Lanceley A, Ni Y, et al. Parenting stress and health-related quality of life among parents of extremely preterm born early adolescents in England: a cross-sectional study. Archives of Disease in Childhood - Fetal and Neonatal Edition https://fn.bmj.com/content/early/2023/10/24/archdischild-2023-325429   van Hasselt TJ, Gale C, Battersby C, et al. Paediatric intensive care admissions of preterm children born <32 weeks gestation: a national retrospective cohort study using data linkage. Archives of Disease in Childhood - Fetal and Neonatal Edition. https://fn.bmj.com/content/early/2023/11/03/archdischild-2023-325970   Felicity's recommendations from Archives:   Byrne S, Sledge H, Franklin R on behalf of the CORAL Study group, et al. Social communication skill attainment in babies born during the COVID-19 pandemic: a birth cohort study Archives of Disease in Childhood 2023;108:20-24 https://adc.bmj.com/content/108/1/20   Pereyra-Elías R, Carson C, Quigley MA. Association between breastfeeding duration and educational achievement in England: results from the Millennium Cohort Study Archives of Disease in Childhood 2023;108:665-672. https://adc.bmj.com/content/108/8/665   Zhang CX, Quigley MA, Bankhead C, et al. Ethnic differences and inequities in paediatric healthcare utilisation in the UK: a scoping review Archives of Disease in Childhood 2023;108:518-524.  https://adc.bmj.com/content/108/7/518   Storch-De-Gracia P, Fernández JL, Velasco R, et al. Invasive bacterial infection in children with fever and petechial rash in the emergency department: a national prospective observational study. Archives of Disease in Childhood 2023;108:445-450. https://adc.bmj.com/content/108/6/445   Paulina's recommendations from Education & Practice:   Abdalhady M, Harrison CM. Early switch of intravenous to oral antibiotic therapy in clinically well neonates with a probable bacterial infection is as effective as a full course of intravenous antibiotics therapy. Archives of Disease in Childhood - Education and Practice 2023;108:290-291. https://ep.bmj.com/content/108/4/290   Low A, Blyth FJ. Infants reported for maltreatment had an increased risk of death from medical causes. Archives of Disease in Childhood - Education and Practice 2023;108:142. https://ep.bmj.com/content/108/2/142   Barrington J, Polley C, van Heerden C, et al. Parents’ attitudes to medical education on paediatric ward rounds. Archives of Disease in Childhood -Education and Practice 2023;108:134-138. https://ep.bmj.com/content/108/2/134   Mulholland M, Glancy C, Sweeney E, et al. An ulcerated lesion in a previously well child. Archives of Disease in Childhood - Education and Practice 2023;108:74-76. https://ep.bmj.com/content/108/1/74   Our book recommendations:  The Vanishing Half by Brit Bennett Between the World and Me by Ta-Nehisi Coates When breath becomes air by Paul Kalanithi War Doctor: Surgery on the Front Line by David Nott Thursday Murder club series by Richard Osman (for some light relief) Such a fun age by Kiley Reid Growing pains by Mike Shooter And finally our Podcast recommendations: How to Fail by Elizabeth Day This Podcast Will Kill You by Erin Welsh and Erin Allman Updyke

The BNFc recommends age-banded dosing for children prescribed oral paracetamol for non-post operative pain. This was recently challenged by Kirstie Wright and her colleagues following their study published in Archives of Disease in Childhood.  Evaluation of age-banded dosing of oral paracetamol in hospitalised children: a retrospective analysis using clinical data in a tertiary paediatric hospital | Archives of Disease in Childhood (bmj.com) In this episode, Dr Felicity Cooksey meets with Ceri Gaskell, a specialist pharmacist at Bristol Royal Hospital for Children, to explore the debate. Resources: ·      Oral paracetamol dosing in hospitalised children in the UK | Archives of Disease in Childhood (bmj.com) ·      Paracetamol | Drugs | BNFC |NICE ·      A model for size and age changes in the pharmacokinetics of paracetamol in neonates, infants and children - PMC (nih.gov) ·      UKMIQA-drug-dosing-in-childhood-obesity-1.docx (live.com) ·      Medicine dosing in childhood obesity | RCPCH Ceri’s Favourite Book: Pride and Prejudice by Jane Austen