PRISM-SMA -Parent and Caregiver Survey Section 1 Demographics Question Title * 1. How old are you? Question Title * 2. What is your gender? Female Male I'd prefer not to say Question Title * 3. What type of SMA has the person you care for been diagnosed with? SMA Type 1 SMA Type 2 SMA Type 3 SMA Type 4 Question Title * 4. Where in the UK do you live Scotland Northern Ireland North East North West Yorkshire and Humber West Midlands East Anglia South West South East London Wales Question Title * 5. What is your highest/current educational level? Primary School Secondary School 6th form/College University Question Title * 6. What is your current occupation, if working? Question Title * 7. Is English your first language? Yes No. If it is not please let us know what your first language is below. Question Title * 8. How would you describe your ethnicity? Feel free to leave blank if you would prefer not to say. Question Title * 9. Has the child you care for received any of the following medications? Tick all that apply. Nusinersen Risdiplam Zolgensma They have received no medications Please let us know if these are ongoing, or the order you received these medications Section 2: Current Clinical Needs Question Title * 10. What are the current respiratory needs of the person you care for? No respiratory support Respiratory support only when unwell Overnight NIV only Day and night NIV Permanent ventilation Question Title * 11. Do they require any help maintaining their airway, for example a tracheostomy? If so, please note the support they are using. No Yes (please specify) Question Title * 12. Do they require support managing their secretions? No support required Suction as required or when unwell only Regular suction required daily (less than once every 2 hours) Regular frequent suction required daily (more than once every 2 hours) Nasal Suctioning Question Title * 13. Do they use a cough assist? Yes - Regularly Yes - Occasionally No Question Title * 14. How are they currently feeding? Fully orally fed. Modified oral feeding (i.e., modified textures but fully orally fed) Combined oral feeding & gastrostomy (PEG), nasogastric (NG) or jejunostomy (JEJ). Fully enteral feeding (no oral feeding) Question Title * 15. What is their level of function in their lower limbs? Please select the option closest to their ability. Walking independently (without equipment) Standing independently (without equipment) Walking with assistance (with equipment or support from another person) Crawling on hands and knees Sitting with support – (sitting for more than 10 seconds, either using equipment, for example spinal brace, or propping with hands or pillows) Sitting independently – (for more than 10 seconds using no equipment) Question Title * 16. What is their current level of function in their upper limbs? Able to touch top of head with hands Able to bring hands above head Able to raise hands above shoulder Able to bring a small, filled cup with hands to mouth. Able to bring hands to mouth Able to hold pen or pick up small object or drive powered chair or use phone keypad. If none of these options describe what your child is able to do, please feel free to enter in free text below Question Title * 17. How is their speech currently? Everyone can understand them all the time. It can be hard for some people to understand them, this varies with the situation and who they’re talking to Only people who know them really well can understand their speech and sometimes they use an augmentative and alternative communication (AAC) device or system. They use an augmentative and alternative communication (AAC) device or system as their main way of talking to people They don’t use speech or augmentative and alternative communication (AAC) device or system to let people know what is on their mind. If none of these options describe what your child is able to do, please feel free to enter in free text below Question Title * 18. Please rank the following areas of health that are most important to you as a parent/carer in the last 7 days. (1 = most important 6 = least important). Question Title * 19. Please rank the following areas of health that were most important to you as a parent/carer in the first year of diagnosis (1 = most important 6 = least important). Question Title * 20. Have we missed something? Please add in any other areas you feel are most important to their health below and let us know where that ranks in the options above. Question Title * 21. : How would you consider YOUR CHILD’S quality of life in the past month? 1 -Lowest possible quality of life 4 7 - Best possible quality of life Clear i We adjusted the number you entered based on the slider’s scale. Question Title * 22. If you would like to clarify, please feel free to use free text below question. Question Title * 23. How would you consider YOUR quality of life in the past month? 1 - Lowest possible quality of life 4 7 - Best possible quality of life Clear i We adjusted the number you entered based on the slider’s scale. Question Title * 24. If you would like to clarify, please feel free to use free text below question. Section 3: Discussions at time of diagnosisIf you were present in the initial diagnosis and information given about the condition soon after diagnosis please answer the following questions, if not tick N/A Question Title * 25. Were you given sufficient information about the condition at time of diagnosis? Yes No N/A Question Title * 26. Please let us know what areas of information you felt were missed. Question Title * 27. Were you given enough information of what the outcome for your child would be without drug treatments like nusinersen, risdiplam or zolgensma? Yes No N/A at the time of my child’s diagnosis N/A Question Title * 28. Please let us know what areas of information you felt were missed. Question Title * 29. Were drug treatments like nusinersen, risdiplam or zolgensma available in the UK at the time of your child’s diagnosis? Yes No Not sure Question Title * 30. Were you given sufficient information about available non-drug management (e.g. breathing and feeding support or postural/orthopaedic management) of the condition? Yes No N/A Question Title * 31. Please let us know what areas of information you felt were missed Question Title * 32. Were you informed of all possible drug treatments available on the NHS (not under research) at that time? Yes No N/A Question Title * 33. Did you understand the drug treatment options presented enough to make an informed decision? Yes No N/A Question Title * 34. Did you understand the possibility of the choice of no drug treatment and what this may entail? Yes No N/A Question Title * 35. Were you informed of the possibility of not engaging in other supportive management for SMA (e.g. breathing and feeding support or postural/orthopaedic management) and what this may entail? Yes No N/A Question Title * 36. If a new drug treatment for SMA became available on the NHS subsequently after your diagnosis, was information about the drug treatment given adequately? Yes No N/A Question Title * 37. Did you feel you participated in the health decision making process for SMA? Yes No N/A Question Title * 38. Feel free to elaborate. Question Title * 39. How confident are you that the management decisions made for your child or person you care for were the right decisions at the time? Very confident (I definitely would not change the decision if asked today) Confident (I am likely not to change the decision if asked today) Unsure (I cannot decide if I would change the decision if asked today) Not confident (I am likely to change the decision if asked today) I would change the decision if asked today. N/A or unable to answer. Question Title * 40. If the diagnosis was made much earlier (a few days after being born and potentially with very minimal symptoms of SMA), such as through newborn screening, would this change your decision, and if so how. Yes No N/A Question Title * 41. Please let us know your thoughts on why you selected your answer. Question Title * 42. Throughout this process, were you offered any psychological support from the teams looking after your child? Yes No N/A Question Title * 43. Was the concept of palliative care discussed/explained or introduced during your initial consultations? Yes No N/A Question Title * 44. In your view, is it the family that make the final decision about treatment for their child or is it the health care professionals, or in partnership together? The family make the final decision The healthcare professional makes the final decision It is made in partnership between families and healthcare professionals. Question Title * 45. Has the treatment met your expectations? Yes No N/A Question Title * 46. If no, please let us know why. Question Title * 47. Would you be interested for a researcher to contact you for participating in a qualitative interview to yield more information? This will likely take 1 hour and can be in-person or virtually. If yes, provide an email address or phone number and one of the research team will be in touch. Thank you for completing this survey. Once we have reviewed everyone’s answers, we will post a summary of the findings on the SMA UK website. Next