Appendix 2: Supplementary questionnaire

A.  For all patients,

  1. Is there a prior history of urinary tract infections?
  2. How many?
  3. At what ages?
  4. What age was he/she toilet trained?

B.  For those with late onset (juvenile) Pompe disease and having no current problems,

  1. Did he/she have any problems prior to diagnosis of Pompe?
  2. If so, Please elaborate
  3. Did ERT help symptoms?

C.  For others,

  1. Does laughing/giggling cause urinary leakage?
  2. If no, next question
  3. If yes,
  4. Quantify amount of leakage
  5. Small spot in undergarment
  6. Medium amount
  7. Large amount- consistent with full bladder emptying
  8. Quantify frequency
  9. More than once a day
  10. Daily
  11. 3-6 times per week
  12. 1-2 times per week
  13. Less than weekly
  14. Was this the only type of urinary incontinence that caused you to answer other than “never” and “I don’t pee in my underwear” on questions 1-2 on questionnaire?
  15. Does other activity cause a small amount of urinary leakage? These include crying, coughing, sneezing, jumping, running, etc.
  16. If no, next question
  17. If yes,
  18. Quantify amount of leakage
  19. Small spot in undergarment
  20. Medium amount
  21. Large amount- consistent with full bladder emptying
  22. Quantity frequency
  23. More than once a day
  24. Daily
  25. 3-6 times per week
  26. 1-2 times per week
  27. Less than weekly
  28. Was this the only type of urinary incontinence that caused you to answer other than “never” and “I don’t pee in my underwear” on questions 1-2 on questionnaire?
  29. Do these activities cause leakage from the back side (anus) of:
  30. Gas (flatus, fart)?
  31. Stool (poop)?
  32. Is there dribbling of urine after going to the bathroom?
  33. No
  34. Yes
  35. Quantify amount of leakage
  36. One or two drops
  37. Small spot in undergarment
  38. Medium amount
  39. Quantify frequency
  40. More than once a day
  41. Daily
  42. 3-6 times per week
  43. 1-2 times per week
  44. Less than weekly
  45. Did increasing the dose of ERT change symptoms of urinary control?
  46. No
  47. If yes, please elaborate