ADPKD Patient Survey

Hello:
You are invited to participate in our survey on the real-life impact for people living with ADPKD. In this survey, approximately 700 people will be asked to complete a survey that asks questions about ADPKD. It will take approximately 30 minutes to complete the questionnaire.
The beginning of the questionnaire is designed to collect information about each participant to allow us to ensure that we can analyse the answers you give us in the best possible way. When you have finished completing these initial questions you will move on to the main content of the questionnaire. Although some questions may seem like duplicates or to collect similar information please answer them.
At the end of the initial section you may receive a message to say that at this time you are not able to continue. This is because we have strict criteria for participants.
Your participation in this study is completely voluntary. There are no foreseeable risks associated with this project. However, if you feel uncomfortable answering any questions, you can withdraw from the survey at any point. It is very important for us to learn your opinions.

Your survey responses will be strictly confidential and data from this research will be reported only in the aggregate. Your information will be coded and will remain confidential. If you have questions at any time about the survey or the procedures, you may contact Dirk Grygo at +494060534586 or by email at the email address specified below.

Thank you very much for your time and support. Please start with the survey now by clicking on the Continue button below.

* Please input your unique 5 digit number (this will have been given to you by your recruiter)

Introduction

* S1
Please indicate which country you are resident in

* S2. Have you been diagnosed with ADPKD?

Yes

* S3. When were you diagnosed with ADPKD?
If you are not sure of the exact date, please select the 1st of the month

* Years (enter 0 to 99)

* Months (enter 0 to 12)

* S5 Do you know your latest Glomerular Filtration Rate (GFR)% measure?
If yes, please state. If no, please select Don't Know

(1)GFR above 90mL/min/1.73m²

(2)GFR of 60 to 89 mL/min/1.73m²

(3)GFR of 30 to 59 mL/min/1.73m²

(4)GFR of 15 to 29 mL/min/1.73m²

(5)GFR less than 15 mL/min/1.73m²

Don't Know

* S6 Which of the following age groups do you belong

* S7 What is your exact age in years?

* S8 What is your gender

Male

Female

* S9 Has ADPKD been diagnosed in your family history?

Yes

Not sure (some family history of kidney disease related illnesses, but ADPKD has not been specified)

* S10.1 Please tell us which other family members have been diagnosed (Select all that apply)?

S10.2 If yes,

	Number
how many siblings have been diagnosed?

how many daughters / sons have been diagnosed?

The 5 stages of kidney disease. Please read.

The medical term for reduced kidney function is chronic kidney disease (CKD). CKD means that your kidneys are damaged. As a result, your kidneys may not work as well as they should to clear waste materials from the body and to maintain a normal balance of fluids and chemicals in the body. Various conditions can cause CKD, including polycystic kidney disease (PKD).

• PKD is a genetic condition that causes many cysts (fluid-filled sacs) to develop in the kidneys. The most common type is an inherited condition called autosomal dominant polycystic kidney disease (ADPKD).
• ADPKD tends to be defined by stages that reflect the effect the disease is having on the kidneys. In medical terms this is generally called CKD progression.

There are 5 stages of CKD progression:

• Stage 1: Normal kidney function with possibly blood in the urine, kidney cysts or some family history of the disease
• Stage 2: Mildly reduced kidney function, accompanied by other symptoms (as for stage 1)
• Stage 3: Moderately reduced kidney function
• Stage 4: Severely reduced kidney function
• Stage 5: Very severe kidney failure (sometimes call established renal failure) usually on or starting dialysis

Most people with mild to moderate (stage 1 to 3) do not have any kidney failure symptoms. Symptoms tend to develop when CKD becomes severe (stage 4) or worse. The symptoms can include: feeling tired; a poor appetite; weight loss; itchy skin; muscle cramps; swollen feet; puffiness around the eyes; being pale due to anaemia; feeling sick. As the kidney function declines from stage 4 to 5, you are likely to feel more unwell.
If you have already received a kidney transplant please consider yourself to be back at stage 1 CKD unless your symptoms dictate otherwise. We will take your transplant into consideration when we analyse the results.

* S11 Considering the previous definitions, which stage would you say you are currently within?

* S12.1 Are you currently in dialysis?

Yes

* S12.2 Which type of dialysis are you currently on?

Haemodialysis

Peritoneal Dialysis

* S13. Have you had a kidney transplant?

Yes

* S14. What is your employment status?

* Prior to diagnosis with ADPKD

* After diagnosis with ADPKD

1. Diagnosis

The 5 stages of kidney disease - recap.
The medical term for reduced kidney function is chronic kidney disease (CKD). CKD means that your kidneys are damaged. As a result, your kidneys may not work as well as they should to clear waste materials from the body and to maintain a normal balance of fluids and chemicals in the body. Various conditions can cause CKD, including polycystic kidney disease (PKD).

• PKD is a genetic condition that causes many cysts (fluid-filled sacs) to develop in the kidneys. The most common type is an inherited condition called autosomal dominant polycystic kidney disease (ADPKD).
• ADPKD tends to be defined by stages that reflect the effect the disease is having on the kidneys. In medical terms this is generally called CKD progression.

There are 5 stages of CKD progression:

• Stage 1: Normal kidney function with possibly blood in the urine, kidney cysts or some family history of the disease
• Stage 2: Mildly reduced kidney function, accompanied by other symptoms (as for stage 1)
• Stage 3: Moderately reduced kidney function
• Stage 4: Severely reduced kidney function
• Stage 5: Very severe kidney failure (sometimes call established renal failure) usually on or starting dialysis

Most people with mild to moderate (stage 1 to 3) do not have any kidney failure symptoms. Symptoms tend to develop when CKD becomes severe (stage 4) or worse. The symptoms can include: feeling tired; a poor appetite; weight loss; itchy skin; muscle cramps; swollen feet; puffiness around the eyes; being pale due to anaemia; feeling sick. As the kidney function declines from stage 4 to 5, you are likely to feel more unwell.
If you have already received a kidney transplant please consider yourself to be back at stage 1 CKD unless your symptoms dictate otherwise. We will take your transplant into consideration when we analyse the results.

* Q1.1 At what age were you first diagnosed?

* Q1.2 Prior to diagnosis were you aware of any family history of ADPKD or kidney disease?

Yes

* Q1.3 What Health Care Professional provided the diagnosis of ADPKD?

* Q1.4 What were the top four emotions you experienced at this time (at diagnosis)?

Q1.5a What type of information did you receive about the disease at the time of diagnosis (Select all that apply)?

No information

Leaflet/booklet from the doctor

Conversation with the doctor only

Referred to Support Groups

Given helpline number/website address

Psychological Support / Counselling

Emotional / Practical Support

Other (please specify)

Q1.5b Where did the information come from? (Select all that apply)?

Q1.5c On a scale of 1 to 4, how adequate was the information you received (if none received please choose not at all adequate)?

	1. Not at all adequate	2. Not very adequate	3. Adequate	4. Very adequate
* Select one

Symptoms

* Q1.6a What symptoms, if any, initiated your ADPKD diagnosis?

* Q1.6b When you were diagnosed what symptoms did you have?

* Describe what happened to you/how you came to be diagnosed.

* Q1.6d What were the top four emotions you experienced when telling your family about your diagnosis?

2. HCP Relationships

Q2.1 On a scale of 1 to 4, how knowledgeable are you about ADPKD?

	1 Not at all knowledgeable	2 Not very knowledgeable	3 Knowledgeable	4 Very knowledgeable
* Level of knowledge

Q2.2 How knowledgeable are the Health Care Professionals you see in relation to ADPKD? (Please answer for all relevant personnel)

* Q2.3a Who provided you with the most information about the disease?

Select all that apply

On a scale of 1-4 (where 4 is very useful) how useful was this information?

* Q2.4 What were the top four emotions you experienced when you received this information?

3. Treatment

* Q3.1 On average, how much time is currently taken up receiving dialysis (including travel etc) per week

* Q3.2 What are the top four emotions you experience when thinking about potentially having dialysis in the future?

* Q3.3 Have you had, or are you waiting for, a kidney transplant?

Yes – I have had a kidney transplant

Yes – I am still waiting for a kidney transplant

Q3.3b On average, how much time was taken up receiving dialysis (including travel) each week?

Q3.3a How long did you wait for a kidney transplant/have been waiting for a kidney transplant (from being placed on the transplant list)?

4. Employment Issues

The 5 stages of kidney disease

Reminder

The medical term for reduced kidney function is chronic kidney disease (CKD). CKD means that your kidneys are damaged. As a result, your kidneys may not work as well as they should to clear waste materials from the body and to maintain a normal balance of fluids and chemicals in the body. Various conditions can cause CKD, including polycystic kidney disease (PKD).

• PKD is a genetic condition that causes many cysts (fluid-filled sacs) to develop in the kidneys. The most common type is an inherited condition called autosomal dominant polycystic kidney disease (ADPKD).
• ADPKD tends to be defined by stages that reflect the effect the disease is having on the kidneys. In medical terms this is generally called CKD progression.

There are 5 stages of CKD progression:

• Stage 1: Normal kidney function with possibly blood in the urine, kidney cysts or some family history of the disease
• Stage 2: Mildly reduced kidney function, accompanied by other symptoms (as for stage 1)
• Stage 3: Moderately reduced kidney function
• Stage 4: Severely reduced kidney function
• Stage 5: Very severe kidney failure (sometimes called established renal failure) usually on or starting dialysis

Most people with mild to moderate (stage 1 to 3) do not have any kidney failure symptoms. Symptoms tend to develop when CKD becomes severe (stage 4) or worse. The symptoms can include: feeling tired; a poor appetite; weight loss; itchy skin; muscle cramps; swollen feet; puffiness around the eyes; being pale due to anaemia; feeling sick. As the kidney function declines from stage 4 to 5, you are likely to feel more unwell.
If you have already received a kidney transplant please consider yourself to be back at stage 1 CKD unless your symptoms dictate otherwise. We will take your transplant into consideration when we analyse the results.

Q4.1 Have you told/Did you tell, your employer that you have ADPKD, and in what CKD stage did you tell them?

Yes, at diagnosis

Yes, in stage 2

Yes, in stage 3

Yes, in stage 4

Yes, in stage 5

No, I have not told my employer

Non applicable: I’m not employed/was not employed at the time

Non applicable: I am self employed / was self employed at the time

* Why have you not told your employer?

* Q4.2 After telling your employer, what impact did this have on your career?

No effect on my career

My employer was sympathetic and has made effort to accomodate me

My employer was unsympathetic

I’ve had to downgrade/compromise my career

I’ve had to change careers/retrain for a new profession

I’ve become unemployable

I’m now working part-time

I’m now working as a freelancer / consultant

Other (please state)

Q4.2b Following your diagnosis, as a self employed person, what, if any, adaptations did you have to make to your working life / hours?

* Q4.3 In terms of sickness absence (since you were diagnosed), in total how many days can be attributed to ADPKD related issues (in the last year)?

Q4.4 What four emotions did you feel when you had to take time off due to ADPKD? (If your previous answer was zero please just press continue)

* Q4.5 What, if any, special arrangements has your employer put in place after you informed them about having ADPKD?

5. Social life

Note: This section contains very personal questions and of course you are not obliged to answer them, however just to remind you, this survey is completely private and confidential. If you can share your experiences with the following questions, it will help us to truly understand the difficulties an ADPKD sufferer is going through.

Q5.1 To date how has ADPKD impacted your social life?

ADPKD does not have an impact on my social life

I don’t go out with friends as much as I would like as I am more self conscious

I never go out with friends as I am more self conscious

I don’t go out with friends as much as I would like as the symptoms make it difficult, (e.g. Urinary frequency)

I never go out with friends as the symptoms make it difficult, (e.g. Urinary frequency)

Other (please state)

Q5.2 To date how has ADPKD impacted your relationships?

ADPKD does not / did not have an impact on relationships

I am / was worried about starting new relationships in case they want children

I am / was worried about starting new relationships because of the potential emotional strain

ADPKD has placed a strain on my relationship because I do not / did not want to pass the condition onto my children

Managing my ADPKD on a daily basis placed an emotional strain on my relationship

Q5.3 How do you feel about talking about ADPKD and telling others about this disease?

I am completely comfortable telling other people that I have ADPKD

It’s frustrating telling people that I have ADPKD as they don’t understand

I don’t tell people that I have ADPKD as I know they won’t understand

It’s frustrating telling people about my ADPKD as they are often not interested

I get nervous telling people about my ADPKD in case they treat me differently

I don’t like talking about my ADPKD as I find it distressing

Other (please state)

Q5.4 How has ADPKD influenced or affected your sexual relationships?

Not affected (remains the same in frequency and ease )

Some effect (more awkward/a little less frequent)

Affected (much less frequent/very difficult)

Major effect (little or no regular sex due to symptoms or impact of ADPKD)

Why do you say that?

Q5.5 In what ways has your ADPKD diagnosis affected your family members?

Q5.6 How has ADPKD influenced your thoughts about having children or having more children?

I chose to not have children/any more children because I don’t want to pass on ADPKD

I chose to not have children/any more children because I am worried about pregancy worsening my symptoms

I probably will not have children/any more children because I don’t want to pass on ADPKD

I probably will not have children/any more children because I am worried about pregancy worsening my symptoms

I may still have children/ more children

I will (I had) have children/ more children

Other (please state)

Why do you say that?

* Q5.7 If you had to describe your emotional state of mind right now. What four emotions best describes how you feel?

Q5.8 What one thing frustrates you most about ADPKD right now?

6. Health attitude

* Q6.1 What would you like for the future for ADPKD sufferers?

* Q6.2 To finish the last questions in this survey, is there anything else you think we should be aware of regarding an ADPKD sufferer’s life, psychological state and general effect on their life and health generally?