Table 1 Summary of PKDD and PKDIA itemsa
Description | Score range | |
|---|---|---|
PKDD itemb | ||
1 | Describe how tired you were at its worst today | 0–10 |
2 | Describe how tired you felt after finishing your daily activities (e.g., work, social, leisure, physical or household activities) today | 0–10 |
3 | Describe your jaundice (how yellow your eyes and/or skin appeared) when you looked in the mirror today | 0–4 (None–very severe) |
4 | Describe your bone pain at its worst today Option: I have never experienced bone pain | 0–10 |
5 | Describe your shortness of breath during moderate (e.g., walking on an incline or upstairs) physical activity you did today Option: I avoided this activity because it was too difficult for me to do moderate physical activity Option: Not applicable, because I did not have the opportunity to do moderate physical activity | 0–10 |
6 | Describe your energy level at the beginning of your day (after being awake for one hour) | 0–10 |
7 | Describe your energy level at the end of your day (right now) | 0–10 |
PKDIA itemc | ||
1 | Start things you wanted to get done | 0–10 |
2 | Finish things you wanted to get done | 0–10 |
3 | How often were you bothered by your appearance because of your PK deficiency over the past 7 days? | 0–10 |
4 | How often did you get unwanted attention because of your PK deficiency over the past 7 days? | 0–10 |
5 | How often did your PK deficiency interfere with your ability to do household activities (e.g., chores, cleaning, laundry) over the past 7 days? | 0–10 |
6 | How often did a lack of energy due to your PK deficiency interfere with participating in social activities (e.g., doing something together with friends) over the past 7 days? | 0–10 |
7 | How often did your PK deficiency interfere with leisure activities (i.e., hobbies or things you do for fun in your free time) over the past 7 days? | 0–10 |
8 | How often did you feel your relationships with friends or family were negatively affected because of your PK deficiency over the past 7 days? | 0–10 |
9a | Did you work or go to school over the past 7 days? Option: Yes Option: No, because it was too difficult for me to go to work or school Option: No, because I am not currently working or in school for reasons unrelated to my PK deficiency | Yes/no |
9b | [If yes to Question 9a] How often did your PK deficiency interfere with your ability to perform to your full potential at work or school over the past 7 days? | 0–10 |
10 | How often did you have difficulty concentrating because of your PK deficiency over the past 7 days? | 0–10 |
11a | Did you perform moderate (e.g., walking on an incline or up stairs) physical activity over the past 7 days? a. Yes b. No, because it was too difficult for me to do moderate physical activity c. No, because I did not have the opportunity to do moderate physical activity | a/b/c |
11b | [If yes to Question 11a] How often did you have difficulty performing moderate (e.g., walking on an incline or up stairs) physical activity because of your PK deficiency over the past 7 days? | 0–10 |
12 | How much additional rest or sleep did you feel you needed because of your PK deficiency over the past 7 days? | 0–4 (None–a lot) |