Table 3.
Modules | Domain scores | PKU-QOL items (for multi-item scores) | Child PKU-QOL (40 items) | Adolescent PKU-QOL (58 items) | Adult PKU-QOL (65 items) | Parent PKU-QOL (54 items) |
---|---|---|---|---|---|---|
PKU symptoms | Self-health rated status | √ | √ | √ | ||
Headaches | √ | √ | √ | √ | ||
Stomach aches | √ | √ | √ | √ | ||
Tiredness | √ | √ | √ | √ | ||
Lack of concentration | √ | √ | √ | √ | ||
Slow thinking | √ | √ | √ | √ | ||
Trembling hands | √ | |||||
Irritability | √ | √ | √ | √ | ||
Aggressiveness | √ | √ | √ | √ | ||
Moodiness | √ | √ | √ | √ | ||
Sadness | √ | √ | √ | √ | ||
Anxiety | √ | √ | √ | √ | ||
PKU in general | Emotional impact of PKU | Unfairness having PKU | √ | √ | √ | √ |
Worries about the future | √ | √ | √ | |||
Worries about future children | √ | √ | √ | |||
Disease acceptance | √ | √ | √ | √ | ||
Self-esteem | √ | √ | √ | √ | ||
Practical impact of PKU | Burden of care for PKU | √ | √ | √ | √ | |
Burden of physician visits | √ | √ | √ | √ | ||
Maintaining activity at work | √ | √ | √ | |||
Time in administrative tasks | √ | √ | ||||
Missing work | √ | |||||
Difficulty to do other things due to the burden of care for PKU | √ | |||||
Social impact of PKU | Explain situation to others | √ | √ | √ | √ | |
Discussing PKU within family | √ | √ | √ | √ | ||
Difficulty making friends | √ | √ | √ | √ | ||
Impact on relationship with partner | √ | √ | ||||
Maintaining friendship | √ | |||||
Impact of PKU on child’s siblings | √ | |||||
Overall impact of PKU* | Items from Emotional, practical and social impact of PKU | √ | √ | √ | √ | |
Anxiety – blood tests | Anxiety having blood test in the arm | √ | √ | √ | √ | |
Anxiety having blood test in the finger | √ | √ | √ | √ | ||
Impact of child anxiety – blood tests | Impact of child anxiety of blood test in the arm on parent | √ | ||||
Impact of child anxiety of blood test in the finger on parent | √ | |||||
Anxiety – Phe levels | √ | √ | √ | √ | ||
Anxiety – Phe levels during pregnancy | √ | |||||
Financial impact of PKU | √ | √ | ||||
Information on PKU | √ | √ | ||||
Administration of Phe-free protein supplements | Adherence to Phe-free protein supplements | Frequency of supplement intake | √ | √ | √ | |
Adherence/compliance to supplement | √ | √ | √ | √ | ||
Missing supplements because of school | √ | |||||
Missing supplements because of work constraints | √ | √ | ||||
Guilt if poor adherence to Phe-free protein supplements | √ | √ | √ | √ | ||
Impact of Phe-free protein supplements on family | √ | √ | √ | √ | ||
Practical impact of Phe-free protein supplements | Embarrassment/shame taking supplements | √ | √ | √ | ||
Lack of spontaneity/freedom due to supplements | √ | √ | √ | |||
Difficulty eating out due to supplements | √ | √ | √ | |||
Difficulty travelling, transporting supplements for special event situations | √ | √ | √ | |||
Taste - Phe-free protein supplements | √ | √ | √ | |||
Management of Phe-free protein supplements | √ | |||||
Dietary protein restriction | Food temptations | Temptation/adherence | √ | √ | √ | |
Temptation not emotionally | √ | √ | √ | |||
Adherence to diet | Adherence/compliance to the diet | √ | √ | √ | √ | |
Eat forbidden things secretly | √ | √ | √ | |||
Change in diet because of school/college/university constraints | √ | √ | ||||
Change in diet because of work constraints | √ | √ | ||||
Eat forbidden food intentionally | ||||||
Management of diet | Difficulty monitoring dietary protein restriction | √ | ||||
Difficulty monitoring the amount of calories | √ | |||||
Sadness to forbid food to child | √ | |||||
Worry that child eats things secretly | √ | |||||
Hard when child has to follow diet in front of others | √ | |||||
Impact of diet on relationship with child | √ | |||||
Practical impact of diet | Burden of weighing/estimating quantity of protein in food | √ | √ | √ | ||
Lack of spontaneity/freedom due to PKU diet | √ | √ | √ | |||
Difficulty eating out due to PKU diet | √ | √ | √ | |||
Need to plan meals in advance | √ | √ | √ | |||
Time-consuming aspects of the diet | √ | √ | √ | |||
Complexity cooking | √ | √ | √ | |||
Difficulty travelling, transporting PKU food for special event situations | √ | √ | √ | |||
Social impact of diet | Feeling different because of diet | √ | √ | √ | ||
Relationship within family | √ | √ | √ | |||
Temptation emotionally | √ | √ | √ | |||
Embarrassment/shame following diet | √ | √ | √ | |||
Isolation because of diet | √ | √ | √ | |||
Cooking for others | √ | |||||
Overall impact of diet* | Items from practical and social impact of dietary protein restriction | √ | √ | |||
Overall difficulty following diet | √ | √ | √ | |||
Guilt if diet not followed | √ | √ | √ | √ | ||
Taste of low-protein food | √ | √ | √ | |||
Food enjoyment | √ | √ | √ | √ |
In bold*: Overall multi-item scores including items from different domains.
Phe: phenylalanine; PKU: phenylketonuria.