14Appendix

© Van Genderen et al., 2005, UMC Utrecht

All rights reserved. No part of this document may be reproduced, stored in a retrieval system, or transmitted in any form or by any means, without the

prior permission of the author.

When using this questionnaire, please use the following references:

Van Genderen FR, Van Meeteren NLU, Van der Bom JG, Heijnen L, De Kleijn P, Van den Berg HM, Helders PJM. Functional consequences of haemophilia in adults: the development of the

Haemophilia Activities List. Haemophilia 2004; 10: 565-71.

Van Genderen FR, Westers P, Heijnen L, De Kleijn P, Van den Berg HM, Helders PJM, Van Meeteren NLU. Measuring patients' perceptions on their functional abilities: validation of the

Haemophilia Activities List (HAL). Haemophilia Submitted.

Haemophilia Activities List

Introduction This is the Hemophilia Activities List, or HAL. In this questionnaire several activities are listed that could be difficult for people with hemophilia. The aim of this questionnaire is to see how easy it is for you to do these activities General comments When answering the questions, it is only your own experience that counts. You should tick the box behind the question that best reflects your own situation. For every activity, you are asked whether you had any difficulty in performing that activity due to hemophilia. There are six different response options. Answer each question by ticking the box that describes your situation. Example: In the past month, did you have any difficulty due to hemophilia with:

n/a Impossible

Always

Mostly

Sometimes

Rarely

Never

Using public transportation (bus,train, subway) 8 1

2 3 4 5 6

For every question you are required to tick one box. The “n/a” response option (“not applicable”) can be used if you never (have to) perform that specific activity. The “n/a” option is only available for some activities. The difference between the “Impossible” and “Always” response option, is that with “Always” you are in fact able to perform that activity, but with problems and with “Impossible” you are unable to perform that activity. It is very important that you answer all questions. Even when a question seems irrelevant to you, or when you have no opinion relating to the question, please tick the box that describes your situation most closely. It will take 5-10 minutes to finish this questionnaire.

Van Creveldkliniek / Dep. Rehab. Med.

Haemophilia Activities List

Lying down/ sitting / kneeling / standing

In the previous month, did you have any difficulty, due to hemophilia, with:

Impossible

Always

Mostly

Sometimes

Rarely

Never

Sitting down (e.g. on a chair or couch) 1

2 3 4 5 6

Rising from a chair with armrests 1 2 3 4 5 6

Rising from a chair without armrests 1 2 3 4 5 6

Kneeling / squatting 1 2 3 4 5 6

Bending forward 1 2 3 4 5 6

Kneeling for a longer period of time 1 2 3 4 5 6

Squatting for a longer period of time 1 2 3 4 5 6

Standing for a longer period of time 1 2 3 4 5 6

September 2005

Van Creveldkliniek / Dep. Rehab. Med.

Functions of the legs

In the previous month, did you have any difficulty, due to hemophilia, with:

Impossible

Always

Mostly

Sometimes

Rarely

Never

Walking short distances (less than 1 kilometer / 15 minutes) 1

2 3 4 5 6

Walking long distances (more than 1 kilometer / 15 minutes) 1

2 3 4 5 6

Walking on a soft surface (e.g. on the beach or through the woods) 1

2 3 4 5 6

Walking on an uneven surface (e.g. cobblestones, high sidewalks) 1

2 3 4 5 6

Strolling / (window-)shopping 1 2 3 4 5 6

Climbing up the stairs 1 2 3 4 5 6

Climbing down the stairs 1 2 3 4 5 6

Running (e.g. in order to catch the bus) 1

2 3 4 5 6

Jumping 1

2 3 4 5 6

Haemophilia Activities List

Functions of the arms

In the previous month, did you have any difficulty, due to hemophilia, with:

Impossible

Always

Mostly

Sometimes

Rarely

Never

Lifting heavy objects 1 2 3 4 5 6

Carrying heavy objects in the arms 1 2 3 4 5 6

Fine hand movements (e.g. closing buttons) 1

2 3 4 5 6

Reaching above your head (to pick something up from a high shelf) 1

2 3 4 5 6

Use of transportation

In the previous month, did you have any difficulty due to hemophilia with:

n/a Impossible

Always

Mostly

Sometimes

Rarely

Never

Riding a bicycle 8 1 2 3 4 5 6

Getting in and out of a car 8 1 2 3 4 5 6

Using public transportation (bus, train, subway) 8 1

2 3 4 5 6

September 2005

Van Creveldkliniek / Dep. Rehab. Med.

Self care

In the previous month, did you have any difficulty, due to hemophilia, with:

Impossible

Always

Mostly

Sometimes

Rarely

Never

Drying your whole body 1 2 3 4 5 6

Putting on a shirt, sweater etc. 1 2 3 4 5 6

Putting on sock and shoes 1 2 3 4 5 6

Putting on a tie or closing the top button of a shirt 1

2 3 4 5 6

Going to the toilet 1 2 3 4 5 6

Household tasks

In the previous month, did you have any difficulty, due to hemophilia, with:

n/a Impossible

Always

Mostly

Sometimes

Rarely

Never

Going out shopping (for food, drink etc.) 8 1

2 3 4 5 6

Washing the dishes, cleaning the sink 8 1 2 3 4 5 6

Cleaning the house 8 1 2 3 4 5 6

Other household tasks (ironing, making the beds) 8 1

2 3 4 5 6

Doing odd jobs (both in and around the house) 8 1

2 3 4 5 6

Gardening 8 1 2 3 4 5 6

Haemophilia Activities List

Leisure activities and sports

In the previous month, did you have any difficulty, due to hemophilia, with:

n/a Impossible

Always

Mostly

Sometimes

Rarely

Never

Playing games (outdoors, e.g. with your children) 8 1

2 3 4 5 6

Sports 8 1 2 3 4 5 6

Going out (theatre / museum / movie theatre / bar) 8 1

2 3 4 5 6

Hobbies 8 1 2 3 4 5 6

Dancing 8 1 2 3 4 5 6

Going on a holiday (active) 8 1 2 3 4 5 6

Going on a holiday (“passive”; beach-/hotel holiday) 8 1

2 3 4 5 6

September 2005

Van Creveldkliniek / Dep. Rehab. Med.

Adaptations and using an aid

To do some activities, you might need some adaptations or an aid. This does not apply to acute bleeding episodes, when you or more or less forced to use crutches to be able to walk. In the following questions we ask you some things regarding those adaptations or aids.

Do you own a car with adaptations?

No, I don’t have a car

No, I don’t have adaptations in my car

Yes, I own a car with (multiple responses are allowed):

Electronic windows

Power steering

Automatic gearbox

The ability to sit in a wheelchair inside your car

Brake and/or accelerator on the steering column

Other, namely: ………………………………………………………...

Other, namely: ………………………………………………………...

Other, namely: ………………………………………………………...

Do you use aids when performing certain activities?

No, I don’t use any aids

Yes, I use (multiple responses are allowed):

A crutch (1 crutch / cane)

Crutches (two)

Wheelchair

Rollator

Other, namely: ………………………………………………………...

Other, namely: ………………………………………………………...

Other, namely: ………………………………………………………...

Haemophilia Activities List

Thank you for completing the questions on activities. To finish this questionnaire, please provide us with some personal information in the box below. The information you provide will be handled strictly confidentially. Today’s date : .................................................................... Your date of birth : .................................................................... What type of haemophilia do you have? Haemophilia type* 1 Haemophilia A

2 Haemophilia B Severity* 1 Mild

2 Moderate 3 Severe

* Please tick the appropriate box

Thank you very much for your cooperation

September 2005

Van Creveldkliniek / Dep. Rehab. Med.

Scoring system Scores can be calculated for each of the seven domains of the HAL. Additionally, three component scores can be calculated (Activities involving the Upper Extremities, Basic activities involving the Lower Extremities and Complex activities involving the Lower Extremities) as well as an overall score. Before summarizing the individual item scores, recoding is required (see Table 1); a higher raw score represents more functional limitations; possible scoring ranges are given (Table 2). Normalized scores for the domains, components, and the full questionnaire can also be obtained. Missing values are controlled for and the possible scores range from 0 to 100, where 0 represents the worst possible functional status and 100 the best possible functional status (Table 3).

Tabel 1: Recoding Score Recode Meaning 8 0 N/A 1 6 Impossible 2 5 Always problems 3 4 Mostly problems 4 3 Sometimes problems 5 2 Rarely problems 6 1 Never problems

Table 2: Scores Score Items Score range Lying / sitting / kneeling / standing LSKS 1-8 (8) 8 - 48 Functions of the legs LEGS 9-17 (9) 9 - 54 Functions of the arms ARMS 18-21 (4) 4 - 24 Use of transportation TRANS 22-24 (3) 3 - 18 Self care SELFC 25-29 (5) 5 - 30 Household tasks HOUSEH 30-35 (6) 6 - 36 Leisure activities and sports LEISPO 36-42 (7) 7 - 42 Upper Extremity Activities UPPER * (9) 9 - 54 Basic Lower Extremity Activities LOWBAS ** (6) 6 -36 Complex Lower Extremity Activities LOWCOM *** (9) 9 - 54 Sum score SUM 1-42 (42) 42 - 252

* Items for UPPER-component: 18, 19, 20, 21, 25, 26, 27, 28, 29. (9 items) ** Items for LOWBAS-component: 8, 9, 10, 11, 12, 13. (6 items) *** Items for LOWCOM-component: 3, 4, 6, 7, 14, 15, 16, 17, 22. (9 items)

Table 3: Normalization Score Normalisatie

LSKS 100 - ((Σ1−8 - valid) * (100/(5 * valid))) LEGS 100 - ((Σ9−17 - valid) * (100/(5 * valid))) ARMS 100 - ((Σ18−21 - valid) * (100/(5 * valid))) TRANS 100 - ((Σ22−24 - valid) * (100/(5 * valid))) SELFC 100 - ((Σ25−29 - valid) * (100/(5 * valid))) HOUSEH 100 - ((Σ30−35 - valid) * (100/(5 * valid))) LEISPO 100 - ((Σ36−42 - valid) * (100/(5 * valid))) UPPER 100 - ((Σ18−21;25−29 - valid) * (100/(5 * valid))) LOWBAS 100 - ((Σ8−13 - valid) * (100/(5 * valid))) LOWCOM 100 - ((Σ3−7;14−17;22 - valid) * (100/(5 * valid))) SUM 100 - ((Σ1−42 - valid) * (100/(5 * valid))) “valid” = number of items scored within the specific domain/component. Items with “n/a”-response are to be considered NOT valid

Haemophilia Activities List

Example: A patient completed the domain of Leg Functions as follows:

Impossible

Always

Mostly

Sometimes

Rarely

Never

Walking short distances (less than 1 kilometer / 15 minutes) 1

2 3 4 5 6

Walking long distances (more than 1 kilometer / 15 minutes) 1

2 3 4 5 6

Walking on a soft surface (e.g. on the beach or through the woods) 1

2 3 4 5 6

Walking on an uneven surface (e.g. cobblestones, high sidewalks) 1

2 3 4 5 6

Strolling / (window-)shopping 1 2 3 4 5 6

Climbing up the stairs 1 2 3 4 5 6

Climbing down the stairs 1 2 3 4 5 6

Running (e.g. in order to catch the bus) 1

2 3 4 5 6

Jumping 1

2 3 4 5 6

Based on the recoded scores, the raw domain score for the LEGS domain is 30 points. The LOWBAS component encompasses the items 8-13. Item 8 scored 6 points (i.e. “Impossible”), which results in a raw component score of 6+3+4+2+3+3 = 21 points. To normalize the scores (both domain and component scores), the formulas presented in

Table 3 are used. This results in the following:

Item Score Recode Item 9 4 3 Item 10 3 4 Item 11 5 2 Item 12 4 3 Item 13 4 3 Item 14 Invalid Invalid Item 15 4 3 Item 16 1 6 Item 17 1 6

LEGS Normalized: Within the domain, 1 item is invalid, resulting in 8 valid responses out of a possible 9. This results in the following formula:

100-((Σ9−17-valid)*(100/(5*valid))) = 100-((30-8)*(100/(5*8))) = 100-55 = 45 points LOWBAS Normalized: Within the component, no items are invalid, resulting in 6 valid responses out of a possible 6. This results in the following formula:

100-((Σ8−13-valid)*(100/(5*valid))) = 100-((21-6)*(100/(5*6))) = 100 - 50 = 50 points

September 2005

Notes

Notes

Notes