Table 2 Illustrative quotes for conceptual model of child's symptoms and impacts
Theme | Concept | Quote |
|---|---|---|
Behavior | Hyperactivity | “He’s always like Jack Flash, fast as lightning. […] If you say slow down, he gets really excited. He’s always full of energy” (Patient 101) |
Unaware of danger | “When he’s excited, it’s too late. Then he also runs ahead and scurries about and talks to everyone he meets on the street. It’s […] definitely takes an effort to keep an eye on him then. So that he won’t simply run away somewhere” (Patient 305) | |
Sleep | Wakes up at night | “Last night I think he woke up about half past 11, then I got him back down, gave him a drink, then he woke up at like 2. The night before he was literally awake from half past two till about five o’clock in the morning, and then he went to sleep again, and then you’re having to wake him up because he’s tired” (Patient 103) |
Trouble to fall asleep | “We left her in her bedroom so that she would go to sleep by herself like any child, but […] she would not go to bed right away. She would go to bed late. So, the next morning, she was tired, obviously” (Patient 405) | |
Speech/ language | Verbal language | “He can’t talk, he’s never really been able to talk. […] He can sing, but he, sort of, sings the tune rather than the actual words.[…] If you asked him a question or something he wouldn’t really know what you mean. Well, if you say to him, do you want a biscuit, and if you show him the biscuit, he’ll understand what that is, he might point to something but that’s about it really” (Patient 102) |
Non-verbal language | “Speaking is still very hard for him even today. […] But he shows more with his hands what he wants. Yeah, with gestures he expresses what he wants. But he can't speak. Not the way he should” (Patient 303) | |
Pain | In pain | “She has often been hospitalized when she was in severe pain. So, she was given Valium […] When things aren’t going well, I call them [the hospital] and I say, ‘Take her for a three-day in-patient stay, under surveillance,’ and I stay with her, alone, for several days, while the dad stays here [with the other children]” (Patient 407) |
Sensitivity to pain | “It’s true that, it’s annoying, these children [with MPS IIIA], they don’t complain…you need to guess! You need to guess where they are in pain! […] It’s frustrating” (Patient 402) | |
Motor skills | Walking | “He does have trouble walking. […] He doesn’t walk, he has two speeds; one’s running and one’s standing still […]. He’s got problems with his legs so he is a little bit unstable. […] So if we go out we can’t let him—we just can’t let him walk on his own, because for one he’d just run off and two, he hasn’t—he’s just not aware of what’s happening around him” (Patient 103) |
Running | “He walked quickly. It was never running. But like the physiotherapist said, running was more for safety. The faster you move, the sturdier the movement. So, for him it was harder to walk. That's why he always ran. Of course, he always stumbled over something. Since you have to look where you're going” (Patient 302) |