First Steps

You've been diagnosed, read up on the basics of PKD. Now it's time to take those first steps towards becoming your own healthcare advocate. The following information has been developed to help you and your family feel more confident about the ins and outs of a PKD diagnosis.

 

Check out this article - 10 Things Every Nephrologist Should Know About PKD UPDATED!. Print it off and take it to your next doctor visit.

 

Quick Notes on PKD

Important information in one quick click.  Quick Notes on PKD are designed to provide accurate and timely information in a short and easy to understand format.  Choose the topics of interest to you and read, print or take to your next doctor visit.

Quick Notes on PKD: How to Help Maintain Kidney Function
Quick Notes on PKD: 9 Tips to Managing a Healthy Diet and Lifestyle

 

Common Questions

Click a question below to show or hide the answer. You can also download a document of all questions and answers in this section here.

What questions should I ask when choosing a nephrologist or renal clinic for my healthcare?

  • Does your clinic primarily treat ADPKD and/or ARPKD patients?
  • Is a pediatric nephrologist available?
  • Do you offer any other services (like diagnostic services or disease management)?
  • Is genetic counseling an option?
  • Is nutrition counseling available?
  • Do you offer dialysis services?
  • Are transplant services available? For children as well?
  • How many transplants are performed each year?
  • Does your clinic have access to clinical trials for PKD patients? If so, which ones?
  • Do you have access other medical specialties, like hypertension or hepatology?
  • How often should I get brain scans?

How often should I see my nephrologist?

Most ADPKD patients should check with a physician for frequency of check-ups.  Each individual’s need for a visiting schedule will depend on the degree of kidney dysfunction.  Based on this, your doctor should be able to help you establish a more personal schedule. A visit of once a year will guarantee at least an annual check of blood pressure and screening for the presence of proteinuria by urine dipstick.  Serum creatinine concentrations should be checked at each of these visits as well.  This is the minimum that a patient with polycystic kidney disease should do with regard to regular follow-up.  This includes individuals with normal renal function and those with more advanced disease.  If other conditions such as hypertension or abnormal renal function are also present, then it is important to be seen more than once a year.  For all patients with hypertension, it is worth obtaining a home blood pressure monitoring device so that blood pressures can be checked at regular intervals.  Individuals with renal function that is less than normal (clearance < 70 ml/min) most likely should be seen at least twice a year to include a review of their dietary intake as well as an assessment of their adherence to a diet and their creatinine level. 

Is genetic testing available?

  • DNA testing is available for ADPKD and ARPKD and may be used to evaluate kidney donors from ADPKD families, identify atypical presentation of cystic kidney disease and may also be useful for pre-natal testing.
  • Linkage testing requires family participation. At least two (but preferably more) family members are needed, and the doctor must know the clinical status of the participants.
  • For a list of facilities conducting genetic tests for ADPKD and ARPKD around the world, click here.

What are kidney function tests?

Creatinine, a breakdown product of muscle excreted by the kidney, is the best routine test of kidney function. As kidney function becomes impaired, creatinine levels rise. It’s important to note that young, muscular individuals may make more creatinine, while elderly adults may have less creatinine.

Glomerular Filtration Rate (GFR) is a measure of how well the kidney is filtering. GFR can be calculated using several formulas and by a 24-hour urine collection.  

What do the numbers mean?

Creatinine: Normal is approximately 0.6-1.3 milligrams/dl

  • Normal value depends on the lab and your muscle mass
  • Higher means declining kidney function

GFR: Normal is approximately 100 milliliters/minute. Anything lower than this indicates a declining kidney function.

How frequently should my kidney function be checked?

It depends:

  • If you don’t have high blood pressure and kidney function is normal: At least once per year
  • If you have high blood pressure or kidney function isn’t normal: At least twice per year
  • If you are approaching kidney more: More often, check with your doctor

Am I progressing if my creatinine is stable?

You may still be progressing even if your creatinine and GFR are stable. The CRISP study has demonstrated continuous renal enlargement as an indicator for disease progression. Imaging may be able to show this progression.

How will I know when I need a transplant or dialysis?

If you are followed by a nephrologist regularly, blood work will be followed regularly. Your nephrologist will not only be looking at the creatinine level, but also how you are feeling (signs and symptoms) and other electrolyte imbalances. However, you may not experience any symptoms until renal function significantly declines.

Renal replacement in the form of dialysis is typically recommended when the GRF is about 10 percent. However, this does require planning, discussion with your nephrologist about dialysis modalities and what will work best for you and placement of dialysis access in advance.

Transplantation also requires planning. For a cadaver transplant, a patient is usually placed on the waiting list when the GFR is less than or equal to 20 milliliters per minute. A patient is evaluated for a cadaver transplant when the GFR is at about 25 milliliters per minute. A living or unrelated transplant requires time and planning, the evaluation of both the donor and the recipient and a GFR of about 30 milliliters per minute.

What are the signs of kidney failure?

  • Fatigue
  • Poor appetite
  • Nausea/vomiting
  • Trouble concentrating (in severe cases, confusion)
  • Dry, itchy skin, especially if phosphate is high
  • Funny taste in your mouth; food tastes funny, metallic
  • Muscle cramps at night
  • Swelling in feet and ankles

How can I prolong my kidney health?

To prolong your kidney health, you must first maintain good overall health through diet, exercise, not smoking and controlling risk factors for cardiovascular disease, including hypotension. Hypertension is very common in ADPKD. The average age for hypertension in PKD patients is around 30, even before kidney function is often affected

Why is blood pressure control important?

High blood pressure may further damage and scar the kidney and is also a risk factor for heart disease and stroke.

What is the best blood pressure medication?

  • Increased activity of the renin angiotension system associated with ADPKD
  • ACE inhibitors and Angiotensin receptor blockers block the system so these are the first choices.

How low should my blood pressure be?

Although there is some debate about this issue, as a rough rule, blood pressure should be about 130/80 or lower. The HALT PKD study may shed some light on this issue by comparing the effects of having a blood pressure at 130/80 versus 110/70.

What kind of medications should I avoid?

In general, you should avoid any medication that could harm your kidneys or affect your blood pressure. This includes: 

  • Non-Steroidal Anti-inflammatory Agents, such as Advil, Motrin and Aleve: If you have pain, use can use safe doses of Tylenol or Ultram.
  • Cold or allergy pills with pseudafed: These can raise your blood pressure.
  • Over-the-counter diet pills: These may contain diuretics or stimulants.
  • Herbal supplements: These have not been studied in kidney disease and could be dangerous if you have renal dysfunction. They are not regulated by the government and do not have to meet requirements for purity, safety and effectiveness. Herbal supplements may also include contaminants, such as heavy metals and potassium salts. They may also have diuretic effects and interact with prescription drugs.
  • Bactrim: This prescription drug may decrease creatinine secretion.

As always, you should consult your physician/nephrologist before taking any over-the-counter medications and supplements. He or she can help you weigh the risks and benefits, depending on your degree of renal dysfunction.

What are the top 10 things I should ask my nephrologist?

Most patients wished their nephrologist knew a little more about PKD … especially when they are first diagnosed.  To help provide nephrologists a solid understanding of the basics of the disease, Dr. Theodore Steinman has put together a short primer, "Top Ten Things Every Nephrologist Should Know about PKD," patients can take to their nephrologist. 

Dr. Steinman is a Clinical Professor of Medicine at Beth Israel Deaconess Medical Center and Harvard Medical School as well as a current member of the PKD Foundation Board of Trustees and a past Chair of the PKD Foundation’s Scientific Advisory Committee. Click here for a PDF document you can print and take with you to the doctor or read below ...

When interacting with your nephrologist it is important that the physician discuss issues that you and family members face with autosomal dominant polycystic kidney disease (ADPKD).   Autosomal recessive PKD issues will not be addressed in this article.  Your nephrologist needs to listen to you and address your concerns.  The issues below are not necessarily in order of importance, but reflect an overall approach to the understanding and treatment of PKD.

1. Genetics of  PKD (Incidence is 1:500 – 1000 live births)

Inherited:  Each offspring of an affected parent has a 50% chance of inheriting the disease.

Spontaneous mutation:  It is estimated that the incident of spontaneous mutation is 4-7% of the PKD population, but an exact number is difficult to estimate.  The only way one can truly be classified as a spontaneous mutations is if both natural parents have negative ultrasounds for kidney cyst formation.

2. How do I tell if I have PKD I or PKD II ?

It is estimated that approximately 70% of the PKD population have Type I and  30% have Type II disease.  There is no difference in physical appearance between those with PKD I vs those with PKD II.  However, those with Type II disease have a more benign course with a smaller number of cysts, less severe hypertension and a slower time course to ESRD by approximately 15-20 years as compared to those with Type I PKD.  If a family member with PKD developed ESRD before age 55, then it is likely the patient has Type I PKD.  With the onset of ESRD at greater than age 70 in an affected family member, then Type II is invariably the diagnosis in the patient.  Genetic blood testing, not covered by insurance, can provide the exact answer. 

3. What is the natural course history of PKD (Type I and Type II) and can it be changed?

The natural course history of the two types is covered above.  Results from the HALT PKD Study and the Tolvaptan Study may provide  treatment options for the PKD population.  The exact role of blood pressure control will be determined by the HALT PKD Study.  As a general rule, blood pressure should be at least normalized in patients with hypertension who have PKD. 

The role of diet remains to be determined in patients with PKD.  However, we do know from the MDRD Study, that dietary protein restriction does not prevent a decline in kidney function when baseline function is < 50% of normal.  If dietary protein restriction has any impact, it is in those whose kidney function is normal or near normal when the diet is instituted.

With regards to anemia management, it is recommended that the hemoglobin be maintained at the 10-12 gm/dL range.   Correction of severe anemia can have a positive impact on progressive heart enlargement (a consequence of chronic kidney disease of many causes).

4. Complications of PKD – how to diagnose and manage:

Cerebral aneurysm: Screening should only be done in those patients with PKD who have a family history of a cerebral aneurysm or a stroke.  Otherwise, the incidence of aneurysms is not much greater than the general population and brain magnetic resonance angiogram (MRA) is not indicated.

Heart enlargement:  Enlargement of the left side of the heart (left ventricular hypertrophy) is known to occur as a consequence of chronic kidney disease and is made worse by high blood pressure.  Therefore, tight control of the blood pressure and control of anemia helps reduce the severity of this heart problem.

Kidney stones: As many as 20-30% of patients with PKD have kidney stones and approximately 50% are composed of uric acid.  A large fluid intake of at least two and one half quarts per day is recommended as treatment for those with kidney stones of any cause. 

Hematuria:  Rupture of a kidney cyst is one of the most common causes for blood in the urine.  Kidney stones can also cause bleeding.  With both circumstances pain is a common accompaniment.  Occasionally an infected cyst that bleeds can also cause blood in the urine  

5.  How to manage pain in the patient with PKD:

Understanding the cause of the pain is the most important step in deciding treatment.  It is important to determine if the pain is directly related to the cyst or mechanical back pain caused by the change in posture caused by cyst enlargement. 

6. How do I discuss with my children and loved ones about the inheritance of PKD? 

There is no clearcut answer to the question if and when children should be tested.  However, the Genetic Information Non-Discrimination Act of 2008 (GINA) is a federal law that prohibits discrimination in health coverage and employment based on genetic information.  This law generally prohibits health insurers or administrators from requesting or requiring genetic information on an individual or the individual’s family members.   Decisions regarding coverage or rates cannot be based on genetic information.  The law prohibits most employers from using genetic information for hiring, firing or promotion decisions and for any decisions regarding terms of employment.  GINA’s health coverage non-discrimination protections do not extend to life insurance, disability insurance or long-term care insurance. 

7. Transplantation and PKD

How to decide between a living donor and a deceased donor transplant.  This is a decision that you need to discuss with the transplant team at your institution.

Short and long term success with a transplant for a patient with PKD:   PKD patients do as well as any other patient with ESRD and sometimes even fare better with regard to long-term outcomes.  All the statistics will be provided by your transplant team.

Do I need one or both kidneys removed prior to transplantation:
Generally kidneys do not need to be removed.  However, if there is massive polycystic kidney disease that prevents placement of a kidney transplant for pure mechanical reasons, then removal of one or both kidneys may be needed.  Also, if kidney cysts are repeatedly infected, then nephrectomy may be needed.

8. Polycystic liver disease – what influences the expression of this disease in one person versus another:

Cystic liver disease is more common in females who have had multiple pregnancies, generally three or more.  However, it can also occur in males, but with less frequency.  While kidney function is affected by cystic disease, liver function is almost never impacted in patients with polycystic liver disease.  However, very enlarged livers can produce severe abdominal pain, discomfort, loss of appetite and difficulty breathing (large liver cysts press against the diaphragm). 

Does polycystic liver disease occur only in patients with kidney failure:  
Cysts in the liver generally occur in patients with severe polycystic kidney disease.  However, a certain number of patients have massive liver enlargement, disproportionate to kidney involvement.  

9. What is a healthy lifestyle in PKD?

This is the same as a healthy lifestyle in any individual.  In particular, no smoking is critical.  Weight control and a healthy diet are strongly recommended.  Exercise should be part of one’s daily routine. 

10. Current research initiatives:

Will it effect me or my children?  In the last several years there have been more clinical research projects than at any time in history.  All of these research initiatives are still underway and there is no clear-cut answer as to the results from the many studies that are being done.  Your nephrologist should be able to keep you up to date when the results are reported from these research studies.  


Common Exercise Questions

 

Can I exercise if I have PKD?

Yes, exercise is important for cardiovascular health! Activities that may be more risky include contact sports, where the kidneys might be hit accidentally, such as football, rugby, boxing, etc., and any type of activity that includes repetitive jarring or impact, such as horseback riding. There is no evidence that these activities worsen renal function, but they can result in blood appearing in the urine. Remember to always keep well hydrated when exercising.

Are sports dangerous to my kidneys?

In general, most sports do not affect kidney function. Specifically to ADPKD, there are some issues that need to be addressed with regard to the type of exercise regimen chosen. Given the unique nature of ADPKD, where kidneys are enlarged and cysts can easily rupture, there are some simple precautions to take. Contact sports where the kidneys may be traumatized (flank or lower-back impact) should either be avoided or protective pads should be worn. Examples of these types of sports include football, rugby, basketball, hockey and particularly boxing or kickboxing. Horseback riding and cross-country biking are other sports where repetitive impact has resulted in hematuria or blood in the urine, in individuals with ADPKD. 

What are the best exercises for PKD patients?

Good exercises for people with PKD include sports where impact is minimized.  These include swimming, tennis, rowing, golf, biking and, to a lesser extent, jogging or running. Clearly one’s desire for a certain sport needs to be considered when recommendations about choosing a sport are made.   


Finished with this section? Learn more about Living with PKD. Or, return to the Learning Center.

PKD National Convention

PKD National Convention: Better Together
The PKD National Convention 2014 will be held June 20-22 in Kansas City, Mo.

Learn More

Register Now

 

Find Your Local PKD Chapter

ADPKD Q&A

Your Organization

2000 Daniel Island Drive, Charleston SC 29492
Phone: 800.443.9441 | Fax: 843.216.6100
info@yourdomain.org

©2014, PKD Foundation ·The PKD Foundation is a 501 (c)(3), 509 (a)(1) public charity.

©2014, PKD Foundation ·The PKD Foundation is a 501 (c)(3), 509 (a)(1) public charity.

The PKD Foundation is a 501 (c)(3), 509 (a)(1) public charity.
National Headquarters: 8330 Ward Parkway, Suite 510, Kansas City, MO 64114. Phone: 1.800.PKD.CURE
©2014 PKD Foundation. Privacy Policy | Terms & Conditions

Founded in 1982, our vision is that one day,
no one will suffer the full effects of polycystic kidney disease.


International: Canada Canada  France France  Germany Germany  Italy Italy  Japan Japan  United Kingdom United Kingdom