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Not clear if there is a benefit to survivability between naive and steroid group #HMVUK2024

Translarna - DMD nonsense mutation -promotes read through of stop codons to support production - STRIDE registry - may appear to slow respiratory decline. #HMVUK2024

Gene therapy - not available in the UK Unable to cure DMD - Currently attempts to convert DMD to Becker MD - milder #HMVUK2024

SMA - improved survival of type 2 SMA Deletion in SMA1 gene as well as often mutation. Most people have SMA2 gene - can produce smaller amount of functional protein. -dependent on the MN2 gene copy number Therapeutic - increase SMA 1 or 2 #HMVUK2024

Spinraza - restores reading frame of SMA gene 2 -licensed for adults but studies in children - in SMA type 1 significant improvement in survival Available for SMA type 1,2 +3 via NHS England Managed Access Agreement #HMVUK2024

Risdiplam - oral treatment -theoretical risk of male infertility - adverse : rashes and GI upset No comparative data with Spinraza Improvement in scores if we time #HMVUK2024

Gene therapy - Zolgensma - to replace SMN1 Type 1 SMA -92% achieved scored with increases in numbers walking, sitting independently and survival Lots of adverse effects Needs to be given as soon as possible - needs to be less than 6months & 20kg #HMVUK2024

Michael Phelan discussing care agencies and supporting patients with complex needs #HMVUK2024

Home first approach: Nurse delegated complex care via specialist trained caters Nature, intensity, complexity and unpredictability of care should be recognised #HMVUK2024

Environments for continuing complex care - not everyone has a home to go to -nurse delegated care at home is different to nurse delivered care at home - specialist Nursing Home Placement Nurse delegated care at home in the community is often the most efficient #HMVUK2024

10 principles of complex discharge 1. Preparation 2 governance 3 identification if needs 4 establishment of environment 5 Communication 6. Family 7. Training 8. Equipment identification and provision 9. Partnership Working 10. Preparation for final discharge #HMVUK2024

Getting patients on ventilation is not simply twiddling the knobs. Upper airway complexities are becoming more apparent in failure of NIV Management of hypersalivation - not about changing the ventilator but Botox and complex saliva management Complex discharge #HMVUK2024

Please be aware of the URGENT FSN for Bivona Traches. Please find the link and look for Smiths Medical - unable to post as document is 83 pages. gov.uk/drug-device-al…

Practical recommendations for swallowing and speaking during NIV in people with neuromuscular disorders, written together with great SLT Lee Bolton (he/him) 🇪🇺🏳️‍🌈🏳️‍⚧️🕊️ and legendary PT Michel Toussaint 🤓🤩😃 actamyologica.it/article/view/4… Hot off the press🔥

Philips have discontinued the AF541 acute NIV masks and under the nose insert with immediate effect. Apart from the F and P nivairo masks what alternatives are people using? SiLVaH ACPRC Adam C Peake RC