PCD survey – Confidential
(CHILD VERSION 1.0, NOVEMBER 2002)
Not much is known about some aspects of the condition known as PCD (primary
ciliary dyskinesia; Kartagener’s syndrome; immotile cilia syndrome). This
questionnaire is therefore trying to find out basic information about several aspects
of the condition and its effect on people. These include:
!
Some questions about your PCD, how and when it was diagnosed, and
whether or not anyone else in the family also has PCD. This is the first
section of the questionnaire.
!
The overall effects of PCD upon health and everyday activities. This uses a standard
questionnaire used in much medical research which is called the SF-36.
!
The effects of PCD upon the chest, and the occurrence of any respiratory symptoms. Some
people with PCD develop chest infections, and we would like to know how common this is.
This part of the questionnaire is called the Respiratory Symptom Questionnaire, and is also a
standard questionnaire used in medical research which was developed at St. George’s Hospital
in London.
!
People with PCD often have their body organs reversed, and in particular their heart can be on
the right side. Although that is well known, there is very little information about whether they
are more likely to be left-handed or show other reversals of ‘lateralisation’. The section called
the ‘Laterality Questionnaire’ uses some standard questions for assessing handedness and
related behaviours.
!
The section marked ‘Background questions’ uses some brief, standard questionnaires to find a
little more about the sort of person you are.
!
The final section of the questionnaire contains two very important sections:
"
"
Two consent forms. There are two forms which give us permission to use the
information provided for our research. It also makes clear what we will and will not do
with the data.
One form is for the parent or guardian to sign, and this must be signed in every
case.
The second consent form is for the person with PCD. If they are old enough, and
have themselves completed some or all of this questionnaire, then they should
also sign this form.
Your contact information. The PCD Family Support Group has kindly agreed to help us
with this study, but they are, quite correctly, concerned to protect the privacy of those
taking part. This questionnaire has therefore been mailed to you by the PCD Family
Support Group, and at present we do not know your name, address or other contact
details. We would however like to be able to get back in touch in the future, in order to
to ask some further questions. If you are willing to help us further we would be grateful
if you could provide us with your contact details.
We do hope you will be able to help with this research project. Should you have any further questions
about the questionnaire then please feel free to contact me, either by e-mail at i.mcmanus@ucl.ac.uk,
by telephone on 020-7679 5390 (sec 020-7679 5333), by fax at 020-7436 4276, or by post at the
address below.
Department of Psychology,
University College London,
Gower Street,
London WC1E 6BT.
Professor Chris McManus MA, MD, PHD, FRCP
Important
This questionnaire is mostly suitable for children between the ages of about five and fifteen, although
some questions will be answerable for younger children. The questions have however been adapted
from those designed for adults, and will not always been answerable by those who are younger.
Older children (12-15) will probably be able to answer all of the questions themselves,
perhaps in some cases with a little help from a parent or guardian.
Although children aged about 8-11 will probably not be able to complete the questionnaire
themselves, we hope that with the help of an adult they will be able to provide answers to
many of the questions.
Children under the age of about 8 will probably not be able to answer many of the
questions. Nevertheless, we would be grateful if an adult could try and complete as many of
the questions as possible on behalf of the child. All information provided will be of interest.
Firstly, some questions about you, your family, and about PCD.
1.
What is your date of birth? _______(day) ________ (month) _________ (year)
2.
What is your sex? Male / Female
3.
How old were you when PCD was diagnosed?
4.
What were the symptoms that led to PCD being diagnosed? Please give brief details.
5.
Do you have any other medical problems apart from PCD? No / Yes
If Yes, please give brief details
6.
On which side of the body is your heart? Left / Right.
If your heart is on the right-hand side:
a.
Has this been confirmed by a Chest X-ray? No / Yes
b.
To your knowledge are all of your body organs reversed (situs inversus)? No / Yes
7.
Does anyone else in your family have PCD? No / Yes
a.
If Yes, please tell us a bit more about them.
i.
How old are they?
ii.
What is their relationship to you?
iii.
How old were they when they were diagnosed with PCD?
iv.
Is their heart on the right or the left side?
8.
Please tell us a bit more about your family?
a.
How many brothers do you have? ______ What are their ages? __________________
b.
How many sisters do you have? ______ What are their ages? __________________
c.
What year was your mother born? ______
i.
How many brothers and sisters does/did she have? Brothers ____ Sisters_____
d.
What year was your father born? _______
i.
How many brothers and sisters does/did he have? Brothers ____ Sisters_____
2
_________ (years) _________ (months)
The SF-36 questionnaire
Please answer all the questions. Some questions may look like others, but each one is different. Please
take the time to read and answer each question carefully by putting a tick in the box that best represents
how you feel.
How to answer the questions
Do not answer this question, which is only an example.
For each question you will be asked to tick one of the boxes on the line. For instance:
How strongly do you agree or disagree with each of the following statements?
Strongly
agree
a) I enjoy listening to music
Agree
Uncertain
Disagree
Strongly
disagree
U
b) I enjoy reading magazines
U
In this case the person filling in the questionnaire agrees that they enjoy listening to music but
YOUR HEALTH IN GENERAL
1.
In general, would you say your health is:
Excellent
2.
Very good
Good
Fair
Poor
Compared to one year ago, how would you rate your health in general now?
Much better now than
one year ago
Somewhat better now
than one year ago
About the same
as one year ago
3
Somewhat worse now
than one year ago
Much worse now
than one year ago
3.
The following items are about activities you might do during a typical day. Does your
health now limit you in these activities? If so, how much?
Yes, limited
a lot
Yes, limited
a little
No, not limited
at all
a) Vigorous activities, such as running, lifting heavy
objects, participating in strenuous sports
b) Moderate activities, such as moving a table, riding a
bicycle, or throwing a ball
c) Lifting or carrying shopping
d) Climbing several flights of stairs
e) Climbing one flight of stairs
f) Bending or kneeling
g) Walking more than a mile
h) Walking several hundred yards
i) Walking one hundred yards
j) Walking in the house
k) Sitting or lying still
l) Bathing or dressing yourself
4.
During the past 4 weeks, have you had any of the following problems with daily activities as
a result of your physical health?
Yes
No
a) Cut down on the amount of time you spent on your activities
b) Accomplished less than you would like
c) Were limited in the kind of activities
d) Had difficulty performing activities
(for example, it took extra time)
5.
During the past 4 weeks, have you had any of the following problems with regular daily
activities as a result of any emotional problems (such as feeling depressed or anxious)?
Yes
No
a) Cut down on the amount of time you spent on the activities
b) Accomplished less than you would like
c) Didn’t do the activities as carefully as usual
6.
During the past 4 weeks, to what extent have your physical health or emotional problems
interfered with your social activities with family, friends, school or other groups?
Not at all
Slightly
Moderately
4
Quite a bit
Extremely
7.
How much bodily pain have you had during the past 4 weeks?
None
8.
Very mild
Moderate
Severe
Very severe
During the past 4 weeks, how much did pain interfere with your normal activities, at home
or at school?
Not at all
9.
Mild
A little bit
Moderately
Quite a bit
Extremely
These questions are about how you feel and how things have been with you during the past
4 weeks. For each question, please give the one answer that comes to closest to the way you
have been feeling. How much of the time during the past 4 weeks:
All of
the
time
Most
of the
time
A good
bit of
the time
Some of
the time
A little
of the
time
None of
the time
a) did you feel full of life?
b) have you been very nervous?
c) have you felt so down in the dumps
nothing could cheer you up?
d) have you felt calm and peaceful?
e) did you have a lot of energy?
f) have you felt downhearted and depressed?
g) did you feel worn out?
h) have you been happy?
i) did you feel tired?
10.
During the past 4 weeks, how much of the time have your physical health or emotional
problems interfered with your social activities (like visiting friends, relatives, etc.)?
All of the time
11.
Most of the time
Some of the time
A little of the time
None of the time
How TRUE or FALSE is each of the following statements for you?
Definitely
true
a) I seem to get sick a little easier than other people
b) I am as healthy as anybody I know
c) I expect my health to get worse
d) My health is excellent
5
Mostly
true
Don’t
know
Mostly
false
Definitely
false
Respiratory Symptom Questionnaire
Some people with Primary Ciliary Dyskinesia (PCD) have troubles with their breathing. This
questionnaire is designed to help us understand whether you have any such problems. If you do not
have any problems with your breathing then just indicate that in your answers.
12.
Please describe how often breathing or respiratory problems or other problems associated
with PCD have affected you over the last four weeks.
Almost
everyday
Several
days a
week
A few
days a
month
One
day or
so
Not
at
all
a. I have coughed ...
b. I have brought up phlegm (sputum) ...
c. I have had shortness of breath ...
d. I have had episodes of wheezing ...
e. I have had headaches...
f. I have had indigestion or heart-burn (reflux) ...
g. I have had a runny nose and nasal congestion ...
h. I have had a sore throat
i. I have had pain over my sinuses ...
j. I have had a fever ...
k. I have had earache or hearing problems ...
13.
During the last 4 weeks, how many severe or very unpleasant episodes of breathing or
respiratory problems have you had?
No episodes
14.
1 episode
More than 3
episodes
3 or more days
1 or 2 days
Less than a day
Over the last 4 weeks, in an average week, how many good days (with few breathing or
respiratory problems ) have you had?
Every day
16.
3 episodes
How long did the worst episode of breathing or respiratory problems last? Go to the next
question if you didn't have a severe episode.
A week or more
15.
2 episodes
Nearly every day
3 or 4
1 or 2
None
How would you describe your breathing or respiratory problems ? Please fill in one circle
only.
Causes me no problem
Causes me a few
problems
Causes me a lot of
problems
6
The most important
problem I have
17.
These are more questions about cough and shortness of breath.
True
False
Not
applicable
Coughing hurts
Coughing makes me tired
I am short of breath when I talk
I am short of breath when I bend over
My coughing or breathing disturbs my sleep
I become exhausted easily
18.
These are questions about other effects that breathing or respiratory problems may have. If
you do not have a particular problem then please tick ‘Not applicable’.
Definitely
Agree
Disagree Definitely
Not
agree
somewhat somewhat disagree applicable
My coughing or breathing is
embarrassing in public
My breathing problem is a nuisance to my
family, friends, or neighbours
I panic or get afraid when I cannot
catch my breath
I feel that I am not in control of my
breathing or respiratory problems
I do not expect my breathing or respiratory
problems to get any better
I have become frail because of my breathing or
respiratory problems
Exercise is not safe for me
Everything seems too much of an effort
I have sometimes felt I had to hide from other
people the fact that I had PCD
I have sometimes felt embarrassed in public
because of having PCD
I have sometimes avoided situations where
people might find out that I had PCD
I have sometimes felt worried about
other people’s reaction to PCD
7
19.
Are you using any of the following treatments to help with the symptoms of PCD?
At
present
In the
past
Never used
Regular antibiotics
Bronchodilators (inhaler or tablets)
Mucolytics to thin the sputum
Physiotherapy
Breathing exercises and techniques
Expectorants
Antacids or other drugs for heartburn or reflux
20.
If you are receiving any treatment or medication for breathing or respiratory problems,
please answer the following questions. If you are not receiving medication, please tick ‘not
applicable’.
True
False
Not
applicable
My treatment does not help me very much
I get embarrassed using my treatment in public
I have unpleasant side effects from my treatment
My treatment interferes with my life a lot
21.
These are questions about how your activities might be affected by breathing or respiratory
problems. Answer True if the statement applies to you because of your breathing
problem, and otherwise answer False
True
I take a long time to get washed or dressed
I cannot take a bath or shower, or I take a long time to do it
I walk slower than other people my age, or I stop to rest
Jobs such as helping around the house
take a long time or I have to stop to rest
If I walk up one flight of stairs, I have to go slowly or stop
If I hurry or walk fast, I have to stop or slow down
My breathing problem makes it difficult to do things such as
walking up hills, carrying things up stairs,
dancing, or playing light sports
My breathing problems make it difficult to do things such as
carrying heavy loads, walking fast, playing tennis, or swimming
My breathing problems make it difficult to do things such as riding
a bike uphill, running, swimming fast or underwater,
or playing sports such as football or netball
8
False
Not
applicable
22.
How does PCD affect your daily life?
True
False
Not
applicable
I cannot play sports or active games
I cannot go out for entertainment or recreation
I cannot go out of the house to go shopping
I cannot do household chores
I cannot move far from my bed or chair
I sometimes feel it makes people reject me
Sometimes it makes it more difficult for me to make friends
It is sometimes difficult telling people about my condition
Sometimes I prefer not to let other people know that I have PCD
Sometimes it is difficult having a rare and unusual condition
23.
Overall, how would you describe the effect of PCD on you?
Causes me no
problems
24.
Causes me minimal
problems
The most important
problem I have
It stops me from doing
one or two things I
would like to do
It stops me from doing
most of the things I
would like to do
It stops me from doing
everything I would like
to do
Overall, how would you describe the effect of PCD on you?
Causes me no
problems
26.
Causes me a lot of
problems
Please indicate how overall you think that PCD affects you by ticking one of the boxes.
It does not stop me
from doing anything
I would like to do
25.
Causes me a
few problems
Causes me minimal
problems
Causes me a
few problems
Causes me a lot of
problems
The most important
problem I have
Please indicate how overall you think that PCD affects you by ticking one of the boxes.
It does not stop me
from doing anything I
would like to do
It stops me from doing
one or two things I
would like to do
It stops me from doing
most of the things I
would like to do
9
It stops me from doing
everything I would like
to do
Laterality questionnaire
27.
Which hand would you use:
Always
Usually
use right use right
Slightly
prefer
right
Slightly
prefer Usually
left
use left
Always
use left
i. To hold a pen while writing a letter?
ii. To throw a ball at a target?
iii. To hold a pencil while drawing a picture?
iv. To hold a dish while drying it?
v. To turn the winder on a clock?
vi. To hold a jar while unscrewing its lid?
vii. To hold a thread while guiding
it through the eye of a needle?
viii. To hold a knife when eating
with a knife and fork?
ix. To hold a potato while peeling it?
x. At the top of a broom when sweeping the floor?
xi. To pick up a glass of water?
28.
Below are pictures of a range of tasks. Indicate the usual way in which you would carry
out each one by ticking one of the six boxes for each task.
Open can with
right hand
Always
do this
Usually
do this
Open can
with left hand
Slightly
Slightly Usually
prefer this prefer this do this
Left hand
raised to mouth
Always
do this
Usually
do this
Right hand
raised to mouth
Slightly
Slightly Usually
prefer this prefer this do this
Chin in
right palm
Always
do this
Usually
do this
Always
do this
Always
do this
Chin in
left palm
Slightly
Slightly Usually
prefer this prefer this do this
10
Always
do this
Right hand
to forehead
Always
do this
Usually
do this
Left hand
to forehead
Slightly
Slightly
Usually
prefer this prefer this do this
Baby held
on right side
Always
do this
Usually
do this
Baby held
on left side
Slightly
Slightly
Usually
prefer this prefer this do this
Counting fingers
on right hand
Always
do this
Usually
do this
Usually
do this
Slightly
Slightly
Usually
prefer this prefer this do this
Usually
do this
Slightly
Slightly
Usually
prefer this prefer this do this
Usually
do this
Always
do this
Right hand
shielding eyes
Slightly
Slightly
Usually
prefer this prefer this do this
Right foot forward,
left hand shielding
eyes
Always
do this
Always
do this
Left leg forward,
pick up with right
hand
Left hand
shielding eyes
Always
do this
Always
do this
Counting fingers
on left hand
Right leg forward,
pick up with left
hand
Always
do this
Always
do this
Always
do this
Left foot forward,
right hand shielding
eyes
Slightly
Slightly
Usually
prefer this prefer this do this
11
Always
do this
Bag held over
right shoulder
Bag held over
left shoulder
Always
do this
Usually
do this
Slightly
Slightly
Usually
prefer this prefer this do this
Hands clasped, left
thumb on top
Always
do this
Usually
do this
Hands clasped,
right thumb on top
Slightly
Slightly
Usually
prefer this prefer this do this
Usually
do this
Slightly
Slightly
Usually
prefer this prefer this do this
Ankles crossed,
right foot in front
Always
do this
Usually
do this
Usually
do this
Slightly
Slightly
Usually
prefer this prefer this do this
Usually
do this
Always
do this
Arms folded,
right forearmon top
Slightly
Slightly
Usually
prefer this prefer this do this
Hands folded,
right hand upper
Always
do this
Always
do this
Ankles crossed,
left foot in front
Arms folded,
left forearmon top
Always
do this
Always
do this
Legs crossed,
left leg in front
Legs crossed, right
leg in front
Always
do this
Always
do this
Always
do this
Hands folded,
left hand upper
Slightly
Slightly
Usually
prefer this prefer this do this
12
Always
do this
Mobile in left
hand
Always
do this
29.
Usually
do this
Mobile in right
hand
Slightly Usually
Slightly
prefer this prefer this do this
Always
do this
To which ear would you hold a mobile phone while writing? Circle one of these pictures.
.
30.
Please indicate which foot, eye or hand you would use for each of these tasks.
Always
Usually
use right use right
Slightly
prefer
right
Slightly
prefer Usually
left
use left
Always
use left
Which foot would you use to kick a ball at a goal?
With which eye would you
look through a keyhole?
With which hand do you
operate a computer mouse?
On which wrist do you wear your watch?
31.
Which diagram best shows the position
of your hand when writing?
32.
Has injury, damage or disease ever prevented you using your dominant hand? No / Yes
If Yes:
How old were you? _______ years Which hand/arm was injured or damaged? Right / Left
Was the effect temporary or permanent? Temporary / Permanent If Temporary, how long? _________
Please describe the problem briefly: _________________________________________________
33.
Has any person ever tried to change your handedness? No / Yes
If Yes: Was it? From using the right hand to using the left hand / From using the left hand to using the right hand.
How old were you when the attempt was made? _______ years
Who was the person who tried to change your handedness? _____________________
How successful was the attempt? Very successful / Moderately / Not very / Not at all successful
13
34.
Please tell us whether the following relations are (or were) right- or left-handed?
Righthanded
Lefthanded
Not sure
Mother
Father
Mother’s mother (maternal grandmother)
Mother’s father (maternal grandfather)
Father’s mother (paternal grandmother)
Father’s father (paternal grandfather)
35.
How many brothers do you have who are:
Right-handed
36.
Left-handed
Not sure of their handedness
How many sisters do you have who are:
Right-handed
Left-handed
Not sure of their handedness
Please turn to the next page
14
A few questions about yourself
These last few questions are to help us understand a little more about you and your personality. There
are no right or wrong answers to any of the questions, so please just answer them as seems most
appropriate.
37.
Below is a list of words which may describe how you see yourself or how others see you.
– Underline a word if it describes you particularly well –
like this
– Cross out a word if it describes you particularly badly – like this
– If a word neither describes you particularly well
nor particularly badly, just leave it unmarked – like this
active
adaptable
artistic
calm
careless
cheerful
conscientious
considerate
contented
co-operative
creative
curious
domineering
energetic
happy
intelligent
kind
knowledgeable
lazy
moody
neat
nervous
organised
persevering
quiet
reserved
sensitive
shy
spiteful
stable
superficial
suspicious
38.
scatterbrained self-centred
talkative
thoughtful
touchy
unimaginative unreliable unsophisticated
Please say how have you been feeling in general over the past four weeks, by ringing one
of the answers. Have you recently:
Been able to concentrate on whatever you're doing?
Lost much sleep over worry?
Better than usual
Not at all
Same as usual
Less than usual
Much less than usual
No more than usual Rather more than usual Much more than usual
Felt that you were playing a useful part in things?
More so than usual
Same as usual
Less useful than usual
Much less useful
Felt capable of making decisions about things?
More so than usual
Same as usual
Less so than usual
Much less capable
Felt constantly under strain?
Not at all
No more than usual Rather more than usual Much more than usual
Felt that you couldn't overcome your difficulties?
Not at all
No more than usual Rather more than usual Much more than usual
Been able to enjoy your normal day-to-day activities?
More so than usual
Same as usual
Less so than usual
Much less than usual
Been able to face up to your problems?
More so than usual
Same as usual
Less able than usual
Much less able
Been feeling unhappy and depressed?
Not at all
No more than usual Rather more than usual Much more than usual
Been losing confidence in yourself?
Not at all
No more than usual Rather more than usual Much more than usual
Been thinking of yourself as a worthless person?
Not at all
No more than usual Rather more than usual Much more than usual
Been feeling reasonably happy, all things considered?
15
More so than usual About same as usual
Less so than usual
Much less than usual
Consent and contact information
In this questionnaire you have provided us with important and sensitive information about yourself and
your condition. In order that we can use that information for medical research we need your signed
consent. Please read the statement below and sign it:
Because you are under the age of 16, your parent or guardian should sign the consent below.
Consent: I understand that the information provided in this questionnaire about
_______________________________ (please insert name), who is my ______________________
(please state relationship), is strictly for the purposes of medical research, that it will be kept entirely
confidential, and that the information will only be available to other individuals directly involved in
the research itself. Any information which is published will only be in an anonymised or aggregated
form, in which individuals cannot be identified. I hereby give my consent to such use of this
information.
Full name ___________________
Signature ______________________ Date_________________
If you have answered any of the questions yourself in this questionnaire, then you should also sign
the consent form below:
Consent: I understand that the information provided in this questionnaire about myself is strictly for
the purposes of medical research, that it will be kept entirely confidential, and that the information
will only be available to other individuals directly involved in the research itself. Any information
which is published will only be in an anonymised or aggregated form, in which individuals cannot be
identified. I hereby give my consent to such use of this information.
Full name ___________________
Signature ______________________ Date_________________
Contact information
This questionnaire has sent to you via the Primary Ciliary Dyskinesia Family Support Group. If in the
future you are willing for the researchers to contact you directly, then please complete the section below.
Name_____________________________________________________________________________
Name of Parent or Guardian ___________________________________________________________
Address: ___________________________________________________ Post code _______________
Telephone number ______________________ E-mail address _______________________________
Many thanks for your help with this research study