Kidney Diagnosis Guide

Below, you will find information on the top 10 causes of kidney failure. Talk with your doctor about what you learn and work as a team to stay as healthy as you can. Here are the conditions we cover:

• Type 2 Diabetes
• High Blood Pressure
• Diseases of the Kidney Filters - Glomerular Disorders
• Type 1 Diabetes
• Kidney Inflammation
• Cystic Diseases, Genetic Diseases, and Birth Defects
• Secondary Illness/Vasculitis
• Kidney Tumors
• Diseases of the Kidney Tubules (Tubular Necrosis)
• Kidney Disease After Pregnancy

Type 2 Diabetes¹

Type 2 diabetes is the number one cause of kidney failure in the U.S. Each year, more than 40% of kidney failure is due to type 2 diabetes.²

Diabetes can damage the tiny blood vessels inside your kidneys that filter wastes and water out of your blood. A healthy kidney has a million or so nephrons, or filters. Each nephron has a glomerulus — a tangled ball of blood vessels that keeps in cells what you need and lets out what you don’t. Diabetes can harm the glomeruli. The vessel walls thicken and leak. An early sign that this is going on is microalbuminuria — tiny amounts of protein in your urine. Protein is a large cell that only leaks out when something is wrong. At a later stage, your urine might be foamy or bubbly. This means a bigger loss of protein.

What You Can Do

There is a lot of good news if you have diabetes. In fact, 30 years ago, nearly 1 in 3 people with diabetes could expect to have kidney failure. Today, it’s just 1 in 10.³

• Don’t panic! It takes at least 10 years for kidney damage to occur — and if you have had diabetes for 25 years and don’t have kidney damage, you most likely never will.⁴
• Ask for a urine test for microalbumin once a year. This test will show if you have tiny amounts of protein in your urine. If so, it is a warning that your kidneys are at risk and you need to protect them. Even if you have protein in your urine, it can take 5-10 more years to lose enough function to have symptoms. So, you may have time to take action.
• Get your hemoglobin A1c level checked each quarter. This 3-month average of your blood sugar levels is a great way to see how you are doing over time. Diabetes Guidelines for doctors recommend checking it every 3 months.
• Check fasting and post-meal blood sugars. New blood glucose monitors let you test painlessly on your arm — instead of your finger. Learning what makes your blood sugars go up or down will help you stay in control.
• Keep your blood sugar in control with diet, exercise, and medications. The better your control, the more you protect your kidneys. This advice is even more vital if you have metabolic syndrome, a group of risk factors like:
  • Fat around your middle
  • High triglycerides and bad (LDL) cholesterol
  • High blood pressure
  • High levels of blood clotting factors
  • High level of c-reactive protein in your blood

This syndrome makes problems with small blood vessels much more likely, including vessels in the eyes and kidneys.⁵

• Take baby steps. Lifestyle changes are hard to make. Don’t try to change lots of things all at once, choose one and do it for a month or two, then add one more. Reward yourself for successes — even small ones. Keep trying if you don’t succeed the first time. You can do it!
• Keep a journal with your health history and your blood test results. This tool will help you track your results over time to see how you’re doing.
• Your best bet to prevent or slow kidney damage is to keep your blood pressure low (120/80 or less). If you have high blood pressure, take your pills as prescribed. Ask your doctor to change your drug if side effects or costs keep you from taking the one(s) you have.
• Ask about an ACE-inhibitor or ARB. Even if your blood pressure isn’t high, these two classes of blood pressure pills can help protect your kidneys when you have diabetes.

Questions to Ask Your Doctor if You Have Type 2 Diabetes

1. Am I doing all I can to protect my kidneys?
2. If not, what more can I do?
3. Is one of my blood pressure drugs an ACE-inhibitor or an ARB? (If not, should I be taking one to protect my kidneys?)
4. What is my level of kidney function now?
5. Has my kidney function changed over time, and if so how much?
6. Based on where I am right now, what can I expect for my kidneys in the future?

Links to Learn More

• American Diabetes Association - Find information, recipes, tips, local events, books, and more at https://www.diabetes.org.
• Diabetes Risk Calculator - The American Diabetes Association has a tool to check your risk for diabetes or its complications. Free registration is required. https://www.diabetes.org/phd/profile/default.jsp
• Type 2 Diabetes Information - The American Diabetes Association has info about type 2 at http://www.diabetes.org/type-2-diabetes.jsp
• National Library of Medicine - You can look up many topics on Medline Plus, the official consumer website of the National Library of Medicine. Here’s a link to their information on diabetes and kidney disease: http://www.nlm.nih.gov/medlineplus/ency/article/000494.htm
• Online Support Groups - Join an email listserve for type 2 diabetes, at http://health.groups.yahoo.com/group/Type-2-Diabetes/. Note: This listserv has about 300 emails per week. Another listserv is available for all diabetes, at: http://health.groups.yahoo.com/group/diabetes/. This listserv has more than 500 emails per week. The American Diabetes Association offers message boards, at: http://community.diabetes.org/n/pfx/forum.aspx?nav=messages&webtag=adatype2

High Blood Pressure

High blood pressure  — or hypertension — is the number two cause of kidney failure in the U.S. Each year, about 27% of all kidney failure is caused by high blood pressure.² Even if you can’t feel it, having high blood pressure can harm your kidneys. It also raises your risk of stroke, blindness, and heart attack. Each beat of your heart forces blood through your blood vessels. When blood pressure is normal or low, this force is harmless. But when it’s high, the vessels become thicker — and less flexible. No-one is quite sure how the damage occurs. One theory is that the thicker vessels bring less blood flow to the kidneys.⁶ People who are over age 55, African American, or have a family member with high blood pressure are more likely to have high blood pressure. The only way to know for sure if your blood pressure is high is to have it checked. Your blood pressure should be less than 140/90. If you also have diabetes, it should be less than 130/80.⁷

What You Can Do

High blood pressure is something you can do a lot about, through diet, exercise, and medication. With good control, you can protect your kidneys. Here’s how:

• Check your own blood pressure. You will find that it varies with the time of day and amount of stress you are under. For about $25-$75, you can buy an accurate blood pressure monitor to use at home. Ask your nurse to suggest a model and show you how to use it.
• Keep track of your blood pressure in a notebook. Bring the notebook along for your doctor visits.
• Lose weight if you’re heavy. Weight loss helps bring high blood pressure down. Your doctor can refer you to a dietitian who can give you tips.
• Eat more fruits and vegetables. The DASH (dietary approaches to stop hypertension) eating plan is proven to reduce high blood pressure — and it tastes great!
• Limit salt in your diet. Some people are very sensitive to salt, and it will raise their blood pressure. Since it’s hard to know who does and doesn’t have this problem, it’s wise for all of us to eat less salt. Canned or dried soups, processed meats (e.g., bacon, salami, Slim Jims ® ), “Helper ® ” foods, and anything pickled tends to be very high in salt — look for “sodium” on the label. Avoid salt substitutes, though — if your kidneys are damaged, these can harm you.
• Get moving! Talk to your doctor about doing moderate activity for about half an hour most days of the week. It doesn’t have to be formal “exercise” — mowing the lawn, dancing, gardening, even vacuuming count. You want to be up and moving around. Staying active helps keep your blood vessels flexible and makes your heart stronger.
• Practice meditation. Calming your mind can lower your blood pressure. Try taking 15 minutes each day to sit quietly in a comfortable chair with your eyes closed. Play soft music. Breathe in and out slowly and pay close attention to each breath. It’s that easy.
• Take your blood pressure pills just as they are prescribed. Some people think they can feel when their blood pressure is high, and then they take a pill. But blood pressure drugs need to be in your system all the time to work. After all, you never know when something will stress you. If the drugs make you dizzy, cause erectile problems, or cost too much, talk to your doctor. There are many blood pressure pills out there, and another one might work better for you.
• Check for sleep apnea. If your partner tells you that you snore loudly and stop breathing during the night — then restart with a snort, you may have sleep apnea . This is a condition where you are not getting enough air in your lungs when you sleep. It can cause hard-to-treat high blood pressure and other health problems, but can be treated. ⁸ Talk to your doctor about getting a sleep study.

Questions to Ask Your Doctor if You Have High Blood Pressure

1. What blood pressure monitor do you recommend for me to use at home?
2. How often should I check my blood pressure?
3. What level of blood pressure do you see as a good target for me?
4. Can you refer me to a dietitian, and will my insurance plan cover it?
5. Is it safe for me to do moderate activity?

Links to Learn More

• Kidney School - Heart Health and Blood Pressure Module - Free online learning tool takes 30 minutes ( http://www.kidneyschool.org/mod_13/mod13_01.shtml) or download it and read it at your convenience: http://www.kidneyschool.org/pdfs/KS-Module_13.pdf
• Take the High Blood Pressure Quiz - Test your knowledge at http://www.americanheart.org/presenter.jhtml?identifier=3021399
• Your Guide to Lowering Blood Pressure - Free booklet from the National Heart, Lung, and Blood Institute has recipes, tips, and more. Download the pdf (http://www.nhlbi.nih.gov/health/public/heart/hbp/hbp_low/hbp_low.pdf) or order a single copy mailed to you at: http://emall.nhlbihin.net/product2.asp?source=&sku=5232
• Follow the DASH Diet - Read the free booklet on eating right to lower your blood pressure at: http://www.nhlbi.nih.gov/health/public/heart/hbp/dash/new_dash.pdf
• Blood Pressure Medication Info - Learn about types of blood pressure drugs from the American Heart Association: http://americanheart.org/presenter.jhtml?identifier=159
• ACE Inhibitors - Consumer Reports tells you which ones are good buys at http://www.crbestbuydrugs.org/drugreport_DR_ACEI.html (Download free pdf full report).
• Cold and Flu Medications for People with High Blood Pressure - Some over-the-counter drugs can raise your blood pressure. Find out what to watch out for at: http://www.americanheart.org/presenter.jhtml?identifier=580
• What is Sleep Apnea? Read this article from the National Heart, Lung, and Blood Institute to learn more about this condition and how it is treated. http://www.nhlbi.nih.gov/health/dci/Diseases/SleepApnea/SleepApnea_WhatIs.html

Diseases of the Kidney Filters — Glomerular Disorders

Problems with glomeruli (tiny blood vessels that filter wastes and water out of the blood) are the number 3 cause of kidney disease. Ten types cause 8.2% of kidney failure in the U.S.² We’ll tell you about the 5 most common ones, below:

1. Glomerulonephritis (GN) is inflammation of the glomeruli that can be a short or a long-term problem. You may have dark brown or foamy urine, and high blood pressure is common with GN. GN is also called Bright’s disease.
2. FSGS ⁹(focal segmental glomerulosclerosis) scars the glomeruli. Protein and blood then leak into the urine, which may be foamy or pink. The cause of FSGS is not known. A needle biopsy of your kidney may find FSGS — or may not catch it on the first try. About half of people with FSGS will have kidney failure ¹⁰. A fast-moving type can cause kidney failure in 2-3 years; for most, it takes 5-20 years.
3. IgA nephropathy (Berger’s disease) causes protein (immunoglobulin A) to deposit in the kidney blood vessels. No one knows why, though it can run in families. IgA nephropathy tends to cause high blood pressure and swelling. It moves quite slowly; about 1 in 4 will go on to kidney failure over 25 years.¹¹
4. Membranous nephropathy (MN) thickens the walls of the glomerular blood vessels. It may occur on its own or as a result of other disease. Blood clots are more likely, so blood thinners may be prescribed. MN often goes away for years — or even for good. About 1 in 5 will have kidney failure.¹²
5. MPGN (membranoproliferative glomerulonephritis) causes immune cells to deposit in the kidney blood vessels. This changes the vessels so they don’t work as well. It can cause high blood pressure, swelling, and dark urine that looks like tea or may be cloudy. About half the time, MPGN will go on to kidney failure in 10 years or so.¹³ Cases that are mild at the start may be less likely to cause kidney failure.¹⁴

What You Can Do

Glomerular disorders often affect young people with many years ahead of them. It’s key to take steps to avoid heart disease, which can go along with kidney problems. You’ll see a number of tips below:

• Consider immune suppressant drugs for FSGS, MPGN, and IgA nephropathy. About half the time, drugs to suppress the immune system can slow kidney damage in FSGS.¹⁵ These drugs include steroids and some cancer fighting drugs. When protein is spilled into the urine, these drugs may be of use. ¹⁶
• Reduce cholesterol to help fight membranous or IgA nephropathy. Both of these diseases can raise cholesterol levels by acting on the liver. Statin drugs to lower cholesterol can help protect your kidneys — especially if you have heart disease, too. ¹⁷
• Keep your blood pressure low - this will reduce the stress on your kidneys.
• Ask your doctor about limiting salt and fluids - this can help fight edema , swelling or shortness of breath caused by a build up of fluid in your tissues.
• Eat a lower protein diet to reduce waste build up - Americans tend to eat a lot of protein. In some cases, cutting back a bit can ease the burden on your kidneys. Ask to talk to a dietitian — you need good nutrition, too.
• Avoid known kidney toxins

Questions to Ask Your Doctor if You Have a Glomerular Disease

1. What is the name of my condition?
2. Can you tell whether my disease will progress to kidney failure or not? When?
3. Will immune suppressing drugs be useful in my case?
4. If so, what are the side effects of these drugs?
5. Would an ACE-inhibitor be a good choice to help me control my blood pressure?
6. Are there alternative treatments, like fish oil, that might help my problem? (Some studies have found it useful for IgA nephropathy, but others have not.)
7. Can you check the level of uric acid in my blood? (High levels predict that IgA nephropathy will progress.)

Links to Learn More

• Learn about glomerulonephritis from the Mayo Clinic at: http://www.mayoclinic.com/health/glomerulonephritis/DS00503
• The Nephcure Foundation - a non-profit organization seeking a cure for FSGS. http://www.nephcure.org
• Are you under age 40 with FSGS? You may be able to take part in a clinical trial. Visit: http://www.fsgstrial.org/welcome.html
• FSGS on-line support groups - Join an email listserve for FSGS, at http://health.groups.yahoo.com/group/FSGSFocalSegmentalGlomerulosclerosis/ or a different one at: http://health.groups.yahoo.com/group/FSGS/
• Read about Membranous Nephropathy from the National Library of Medicine at: http://www.nlm.nih.gov/medlineplus/ency/article/000472.htm
• Are you over age 13 with Membranous Nephropathy? You may be able to take part in a clinical trial : http://www.clinicaltrials.gov/show/NCT00050713
• The Foundation for IgA Nephropathy - a non-profit organization offering support for people who have IgA nephropathy. http://www.igan.ca/
• IgA nephropathy on-line support group - Join an email listserve, at http://health.groups.yahoo.com/group/iga-nephropathy. Note: This listserv has about 125 emails per week.

Type 1 Diabetes

Type 1 diabetes is the number 4 cause of kidney failure in the U.S. Each year, about 4% of kidney failure is due to type 1 diabetes.² Diabetes can damage the tiny blood vessels inside your kidneys that filter out wastes and water. A healthy kidney has a million or so nephrons, or filters. Each nephron has a glomerulus — a tangled ball of blood vessels that keeps in cells you need and lets out what you don’t. Diabetes first harms the glomeruli. The vessel walls thicken and leak. An early sign that this is going on is microalbuminuria — tiny amounts of protein in your urine. Protein is a large cell that only leaks out when something is wrong. At a later stage, your urine might be foamy or bubbly. This means a bigger loss of protein.

What You Can Do

There is a lot of good news if you have diabetes. In fact, 30 years ago, nearly 1 in 3 people with diabetes could expect to have kidney failure. Today, it’s just 1 in 10.³

• Don’t panic! It takes at least 10 years for kidney damage to occur — and if you have had type 1 diabetes for 25 years and don’t have kidney damage, you most likely never will.⁴
• Ask for a urine test for microalbumin once a year - this will show if you are spilling tiny amounts of protein. If you are, this is a warning that your kidneys are at risk and you need to protect them. (While you’re at it, set up an eye check visit, too.) Even if you have protein in your urine, it can take 5-10 more years to lose enough function to have symptoms. So, you may have time to take action.
• Get your hemoglobin A1c level checked each quarter . This 3-month average of your blood sugar levels is a great way to see how you are doing over time. Diabetes Guidelines for doctors recommend checking it every 3 months.
• Check fasting and post-meal sugars. New blood glucose monitors let you test painlessly on your arm — instead of your finger.
• Keep your blood sugar in control with diet, exercise, and medications. The better your control, the more you protect your kidneys. Using an insulin pump can make it easier to stay in control.
• Keep a journal with your health history and your blood test results - this tool will help you track them over time to see how you’re doing.
• Your best bet to prevent or slow kidney damage is to keep your blood pressure low (120/80 or less). If you have high blood pressure, take your pills as prescribed. Ask your doctor to change your drug if side effects or costs keep you from taking the one(s) you have.
• Ask for an ACE-inhibitor or ARB. Even if your blood pressure isn’t high, these two classes of blood pressure pills can help protect your kidneys when you have diabetes.

Questions to Ask Your Doctor if You Have Type 1 Diabetes

1. Am I doing all I can to protect my kidneys?
2. If not, what more can I do?
3. Is one of my blood pressure drugs an Ace-inhibitor or an ARB? (If not, should I take one to protect my kidneys?)
4. What is my level of kidney function now?
5. Has it changed over time, and if so how much?
6. Based on where I am right now, what can I expect for my kidneys in the future?

Links to Learn More

• American Diabetes Association - Find information, recipes, tips, local events, books, and more at https://www.diabetes.org.
• Diabetes Risk Calculator - The American Diabetes Association has a tool to check your risk for diabetes or its complications. Free registration is required. https://www.diabetes.org/phd/profile/default.jsp
• Type 1 Diabetes Information - The American Diabetes Association has info about type 1 at http://www.diabetes.org/type-1-diabetes.jsp
• National Library of Medicine - You can look up many topics on Medline Plus, the official consumer website of the National Library of Medicine. Here’s a link to their information on diabetes and kidney disease: http://www.nlm.nih.gov/medlineplus/ency/article/000494.htm
• Online Support Groups - Join an email listserve for type 1 and 2 diabetes, at http://health.groups.yahoo.com/group/diabetes. (Note: This list has about 500 messages per week). The American Diabetes Association has message boards for adults with type 1 diabetes at: http://community.diabetes.org/n/pfx/forum.aspx?nav=messages&webtag=adatype1

Kidney Inflammation

Types of kidney inflammation (nephritis), are the number 5 cause of kidney failure in the US. These problems are rare: all told, each year they cause less than 5% of kidney failure. Here are some of the problems that can cause the kidneys to become inflamed:

• Interstitial nephritis - this is swelling of the spaces between the renal tubules that form urine. It is most often a short term problem caused by a drug allergy or side effect.¹⁸ Sometimes it can cause chronic kidney failure. Symptoms may include fever, confusion or sleepiness, rash, swelling, more or less urine than normal, and blood in the urine.
• Pyelonephritis is a kidney infection. It can occur if a bladder infection moves up into the kidneys. This is more likely if you have a kink or blockage in a blood vessel or ureter (tube that brings urine from a kidney to the bladder) that causes reflux — urine that backs up into your kidney. Pyelonephritis can cause a high fever, sweating, and chills; flank or back pain; changes in urine color or odor; pain when urinating; and confusion.¹⁹
• Kidney stones are sharp, pointy crystals made of substances found in the urine. If you have had one, you know that they are quite painful to pass  — and you are more likely to have another.²⁰ Kidney stones are a very rare cause of kidney failure, but it can happen.

• Gout is a common, painful form of arthritis caused by high levels of uric acid in the blood. Early on, most people have a year or more between attacks, so gout is only treated when it is active. In time, attacks can become more frequent, so drug treatment may help. Gout is a rare cause of kidney failure, but uric acid in the blood can form crystals that deposit in the kidneys, or can cause kidney stones.²¹

What You Can Do

Most forms of kidney infection or inflammation can be prevented or treated. You can:

• Check out new drugs. Before you take a drug for the first time, do a little homework. Look them up at http://www.rxlist.com, or ask the pharmacist if there are side effects or warnings you should know about.
• Watch for allergies. When you take a new drug, note any changes in your body. Report new symptoms to your doctor and ask if they might be due to a drug.
• Limit use of NSAIDs. These pain pills with ibuprofen, naproxen, or aspirin or COX-2 inhibitors can harm the kidneys in high doses or at low doses for long time spans.
• If you must take NSAIDs for a long time, ask for a urine protein test. Protein in the urine can be a sign that NSAIDs are causing kidney damage. This can often go away if it is caught early.
• Prevent bladder infections. Most kidney infections start in the bladder. You can help stop bladder infections by drinking plenty of fluids, wiping from front to back after using the bathroom, and urinating after sex.
• Have your urine checked after bladder infections. If you have infections that keep coming back, be sure that they are gone after treatment by asking for a urine culture. If they keep happening, ask your doctor to test you for reflux.
• Drink plenty of fluids to prevent kidney stones. Prone to stones? Keeping your urine dilute by drinking lots of fluids can help prevent them, keep them smaller, or make them pass more easily.
• Learn what your kidney stones are made of. Keep any stones you pass, and ask for a 24 hour urine test afterward so your doctor can prescribe a treatment that will work for you.
• A healthy lifestyle reduces gout. If you keep your weight and blood pressure normal, have no more than 1-2 alcoholic drinks per day, and keep your cholesterol down, you’ll make gout less likely.²²

Questions to Ask Your Doctor if You Have Kidney Inflammation

1. How many NSAID pills can I take before I should worry about my kidneys?
2. Should I take any special precautions when I take NSAIDs? Will drinking a full glass of water after each pill help?
3. What side effects should I be aware of with this drug?
4. I have had ___ bladder (or kidney) infections in ___ (time). What tests do you suggest to rule out reflux from a kink or blockage?
5. What should I eat or drink (or avoid) to prevent future kidney stones?
6. What should I eat or drink (or avoid) to prevent future gout attacks?

Links to Learn More

• National Library of Medicine - read about interstitial nephritis at: http://www.nlm.nih.gov/medlineplus/ency/article/000464.htm
• National Library of Medicine - read about pyelonephritis at: http://www.nlm.nih.gov/medlineplus/ency/article/000522.htm
• What I Need to Know About Kidney Stones - read all about it from the National Institutes of Health: http://kidney.niddk.nih.gov/kudiseases/pubs/stones_ez/
• Download a free booklet on gout - the National Institute of Arthritis and Musculoskeletal and Skin Diseases offers this information: http://www.niams.nih.gov/hi/topics/gout/gout.htm
• Learn about gout from the Mayo Clinic, at: http://www.mayoclinic.com/health/gout/DS00090

Cystic Diseases, Genetic Diseases, and Birth Defects

Some diseases cause cysts to form in the kidneys. These fluid filled or solid cysts replace normal kidney function over time, and can be a rare cause of kidney failure. In the US , about 2% of kidney failure is caused by cystic diseases.² The best known one is polycystic kidney disease, or PKD. PKD cysts can form in the kidneys, liver, pancreas, or testes.²³ In medullary cystic kidney disease (MCKD), cysts form in the middle of each kidney. People with MCKD may pass large amounts of urine and need to take salt supplements. The kidneys become scarred and shrink.²⁴ Other kidney diseases run in families, like:

• Alport’s syndrome - which affects a type of collagen that makes up part of the kidneys.
• Tuberous sclerosis - which causes benign tumors in the brain and organs.
• Cystinosis - which causes crystals of the amino acid cystine to build up in the body.
• Oxalosis - which causes oxalate crystal kidney stones to form.
• Fabry disease - which causes deposits of a fatty substance in the blood and vessels.

And some kidney damage can be caused by birth defects, like DRASH syndrome; blockage in a ureter or kidney blood vessel; small, “horseshoe,” or extra kidneys; or prune belly syndrome.

What You Can Do

In most cases, while you can’t prevent or treat these problems, you can still help protect your kidneys if you:

• Keep your blood pressure in control - Avoiding the “double-whammy” of high blood pressure and any other kidney problem can help protect your kidneys. Work with your doctor to find blood pressure drugs that will work for you. Ask about diet and exercise to help blood pressure.
• Find a support group - You can get tips and stay on top of new research findings when you find others who have the same problem. Look in our Links section below for ideas.
• Learn all you can about your condition - Knowledge is power, and new research is being done all the time.
• Seek out a specialist. When you have a rare disease, you need to find a doctor who knows what you are up against and what the current research says. No doctor can stay up to date on every illness. Ask for a referral to an expert who can consult with your doctor, or find one by talking to others who have the disease.
• Treat the symptoms. You may spill protein into your urine, lose too much water or salt, or have pain that raises your blood pressure. Getting treatment for these symptoms can help take some of the stress off of your kidneys.
• Avoid known kidney toxins
• Check kidney function once a year. A test for protein in your urine and the level of creatinine in your blood can help you and your doctor see how well you’re doing.

Questions to Ask Your Doctor About a Cystic or Genetic Disease, or Birth Defect

1. Should I take an ACE-inhibitor to keep my blood pressure down with PKD? (This class of blood pressure pills can help if you have a lot of protein in your urine)²⁵
2. How likely is it that my children will inherit this disease?
3. What can I expect in the future from this condition, and how will I know?
4. What treatments are available to help my symptoms?

Links to Learn More

• PKD Foundation - request free information, learn about research, join a chapter, and more, at: http://www.pkdcure.org
• On-line PKD Support Groups - Join a PKD email listserv at: http://health.groups.yahoo.com/group/PKDCARE (about 70 emails/week).
• Are you 15-64 years old with PKD? You may be able to take part in a clinical trial: http://www.pkd.wustl.edu/pkd-tn/
• Read about medullary cystic kidney disease on Medline Plus, at http://www.nlm.nih.gov/medlineplus/ency/article/000465.htm
• Join Alport syndrome message boards at: http://forums.delphiforums.com/n/main.asp?webtag=alport&nav=start&prettyurl=%2Falport
• Are you 15-70 years old with Alport syndrome? You may be able to take part in a clinical trial: http://www.clinicaltrials.gov/ct/gui/show/NCT00309257
• Find out more about tuberous sclerosis from the US government, at: http://www.ninds.nih.gov/disorders/tuberous_sclerosis/tuberous_sclerosis.htm
• The Tuberous Sclerosis Alliance. Find information, local resources, on-line discussions, and more, at http://www.tsalliance.org/
• On-line Tuberous Sclerosis Support Groups - Join a TSC email listserv at: http://health.groups.yahoo.com/group/TuberousSclerosisChatRoom (about 20 emails/week).
• The Cystinosis Foundation. Find information, Q&As, message boards, and more, at http://www.cystinosisfoundation.org/
• The Cystinosis Research Network - offers conferences, information, and more, at http://www.cystinosis.org/
• Do you have a child with cystinosis? He or she may be able to take part in a clinical trial: http://clinicaltrials.gov/ct/show/NCT00359684
• The Oxalosis and Hyperoxyluria Foundation - offers information, discussion boards and more, at http://www.ohf.org
• Read about oxalosis from the Mayo Clinic website: http://www.mayoclinic.org/hyperoxaluria/what.html
• Fabry Support and Information Group - Find research trials, discussion boards, links, and more, at http://www.fabry.org/la
• Learn about birth defects of all kinds from the March of Dimes: http://www.marchofdimes.com/
• Prune Belly Syndrome Network - Offers a patient registry, media watch, support forum, links, and more, at: http://www.prunebelly.org

Secondary Illness

Diseases of the Kidney Filters can occur as the body’s response to illness. A number of rare kidney problems together are the number 7 cause of kidney failure in the US (they cause about 2%).² These include:

• Lupus - This autoimmune disease can flare up and affect the skin, joints, blood, and kidneys. About 1 in 3 people with lupus will have a kidney problem that needs treatment. Foamy urine (with protein in it) and swelling are the main symptoms. ²⁶
• Hemolytic uremic syndrome (HUS) - HUS is one of the most common causes of kidney failure in children. Most HUS is due to food poisoning with E coli, which causes bloody diarrhea. Kidney problems are acute (sudden), but can be long-term. ²⁷
• Scleroderma - This disease causes too much collagen to be made, which makes skin, tissues, or organs become stiff. It can affect the kidneys. ²⁸
• Vasculitis - This group of diseases causes inflamed blood vessels, from very small vessels all the way up to arteries. It includes:
  • Wegener’s granulomatosis (WG) - WG inflames blood vessels so less blood reaches some organs, like the nose, sinuses, windpipe, lungs, and kidneys. ²⁹
  • Henoch-Shonlein purpura - This disease causes purple skin spots on the legs, and inflamed blood vessels that can affect kidney function.³⁰
  • Polyarteritis nodosa (PAN) - PAN is an autoimmune disease that causes blood vessels to swell. It can affect the skin, and many organs, including the kidneys. ³¹

What You Can Do

In most cases, while you can’t prevent or treat these problems, you can help protect your kidneys if you:

• Consider immune suppressant drugs for Lupus.^32,33 These drugs include steroids and some cancer fighting drugs.
• Have an ANCA blood test every 6 weeks to follow active Wegener’s. This test can be done less often when the disease is in remission.
• Keep your blood pressure in control. Avoiding the “double-whammy” of high blood pressure and any other kidney problem can help protect your kidneys. Work with your doctor to find blood pressure drugs that will work for you. Ask about diet and exercise to help blood pressure.
• Find a support group. You can get tips and stay on top of new research findings when you find others who have the same problem. Look in our Links section below for ideas.
• Learn all you can about your condition. Knowledge is power, and new research is being done all the time.
• Seek out a specialist. When you have a rare disease, you need to find a doctor who knows what you are up against and what the current research says. No doctor can stay up to date on every illness. Ask for a referral to an expert who can consult with your doctor, or find one by talking to others who have the disease.
• Treat the symptoms. You may spill protein into your urine, lose too much water or salt, or have pain that raises your blood pressure. Getting treatment for these symptoms can help take some of the stress off of your kidneys.
• Avoid known kidney toxins
• Check kidney function once a year. A test for protein in your urine and the level of creatinine in your blood can help you and your doctor see how well you’re doing.

Questions to Ask Your Doctor About Protecting Your Kidneys from Your Other Illness

1. How do you plan to track my kidney health, and how often should I get tested?
2. Can you refer me to an expert in this disease who can work with you to help protect my kidneys and my health?
3. Will immune suppressing drugs be helpful in my case?
4. Will certain blood pressure drugs (like ACE inhibitors) protect my kidneys?
5. What treatments are available to help my symptoms?

Links to Learn More

• Lupus Foundation - Find information, support, advocacy and more, at: http://www.lupus.org
• Read about Hemolytic Uremic Syndrome - from the National Library of Medicine: http://kidney.niddk.nih.gov/kudiseases/pubs/childkidneydiseases/hemolytic_uremic_syndrome/
• On-line Hemolytic Uremic Syndrome support - Join an email listserv at: http://health.groups.yahoo.com/group/Hemolytic_Uremic_Syndrome/ (note: very small group)
• Scleroderma Foundation - Find information, message boards, and support at: http://www.scleroderma.org/. Join and get a quarterly magazine.
• Read a Scleroderma handout - from the National Institute of Allergy and Infectious Diseases: http://www.niams.nih.gov/hi/topics/scleroderma/scleroderma.htm
• On-line Scleroderma support - Join an email listserv at: http://health.groups.yahoo.com/group/SDWorld/
• Vasculitis Foundation - Learn about Wegener’s, Heonoch-Schonlein, Vasculitis, and many other blood vessel diseases, and find a chapter or support group, at: http://www.vasculitisfoundation.org
• Learn about types of Vasculitis from Johns Hopkins Medical Center: http://vasculitis.med.jhu.edu/whatis/whatis.html
• On-line Vasculitis Support - Join an email listserv for a number of types of vasculitis at: http://health.groups.yahoo.com/group/savvy/
• Learn about Wegeners - from the National Institute of Allergy and Infectious Diseases: http://www3.niaid.nih.gov/healthscience/healthtopics/wegener/overview.htm
• On-line Wegeners Support - Join an email listserv at: http://health.groups.yahoo.com/group/Wegeners_Granulomatosis
• Read about Henoch-Schonlein purpura - from the National Library of Medicine, at: http://www.nlm.nih.gov/medlineplus/ency/article/000425.htm
• On-line Henoch-Schonlein support - Join an email listserv at: http://health.groups.yahoo.com/group/Henoch-Schonlein/
• On-line Polyarteritis support - Join an email listserv at: http://health.groups.yahoo.com/group/PolyarteritisNodosa/

Kidney Tumors

Tumors (growths) are a rare cause of kidney failure in the US, causing about 2% per year. Most types of benign (non-cancer) kidney tumors are removed to keep them from becoming cancerous later. Kidney cancer may have symptoms of: ³⁴

• Blood in the urine (it may be red, rusty, or brown)
• Back or flank pain
• Unplanned weight loss
• Vision problems
• Paleness and feeling cold all the time
• Excess hair growth in women
• Constipation
• Swelling of one testicle

Most of these symptoms can also be due to other kidney problems. Blood tests and X-rays are needed to find out their cause.

What You Can Do

There are steps you can take to protect your kidney function if you have a kidney tumor:

• Keep as much kidney tissue as you can. It used to be the “gold standard” to remove a whole kidney when cancer was present. But for tumors up to 4 cm, it works just as well to remove only part of the kidney — and you keep more kidney function. ³⁵
• Consider immune treatment. Kidney cancer cells resist radiation and chemotherapy drugs. But drugs that spur your immune system can help shrink tumors. These drugs have side effects, and only an expert doctor should prescribe them. ³⁶
• Ask about drugs to cut off the tumor’s blood supply. Two new drugs have been approved by the FDA to treat advanced kidney cancer. Sorafenib (Nexavar ® ) ³⁷ and sunitinib (Sutent) ³⁸ are pills that can help shrink tumors. Another drug, Avastin, may also help in kidney cancer.
• Find a support group. You can get tips and stay on top of new research findings when you find others who have the same problem. Look in our Links section below for ideas.
• Learn all you can about your condition. Knowledge is power, and new research is being done all the time.
• Seek out a specialist. When you have a rare disease, you need to find a doctor who knows what you are up against and what the current research says. No doctor can stay up to date on every illness. Ask for a referral to an expert who can consult with your doctor, or find one by talking to others who have the disease.
• Treat the symptoms. You may spill protein into your urine, lose too much water or salt, or have pain that raises your blood pressure. Getting treatment for these symptoms can help take some of the stress off of your kidneys.
• Avoid known kidney toxins

Questions to Ask Your Doctor About Kidney Cancer

1. What type and stage of cancer do I have? (Writing down the name can help you seek more information)
2. What treatments can I use, based on my stage?
3. Which treatments do you recommend for me?
4. What are the side effects or risks of these treatments?
5. How can I best protect my kidney function?
6. What are the chances that the cancer will return with the treatment you suggest?