There’s a lot to think about when you find out that you have autosomal dominant polycystic kidney disease (ADPKD).

When do you start treatment?

Will your daily life change?

How will it affect your kids?

That last one can be really tough. But Jillian Warejko, MD, a pediatric kidney specialist at Yale Medicine, says it’s important for kids to learn what to expect.

“There’s a lot of power in knowing your own health history,” Warejko says. “So we try to teach kids to empower themselves and advocate for themselves.”

You should tailor your conversation based on your child’s developmental age. And you may want to space your talks out over time.

Here are some topics to get you started. 

How You Get It

If your kids are teenagers or young adults, you can break down the basics of this autosomal dominant genetic disease. Warejko explains that a faulty gene on a non-sex chromosome causes ADPKD. That means that whether your child is male or female, there’s a 50% chance they’ll get this mutated gene from you. And if they have the disease, there’s a 50% chance they’ll pass it on to their kids.

You may need to give younger kids less information. You can still bring up ADPKD. But use age-appropriate terms that aren’t scary.

“You don’t want kids to be fearful,” Warejko says. “Fear can make people not want to go to the doctor.” 

In her practice, Warejko describes ADPKD like this:

“It sounds like a couple of family members have had this illness called kidney disease. And the reason you’re visiting with me today is because we want to make sure that, if you have kidney disease too, we’re doing everything in our power to make sure you stay as healthy as possible.”

She also tells kids that it’s best to treat this disease early, when their kidneys are “good” and still work like normal. And the best way to do that, she says, is to “make sure that you’re checking in with us.”

Symptoms to Watch For

If your kids have ADPKD, they might not have any problems when they’re children or young adults.

“The disease can be relatively quiet in those early years,” says Neera Dahl, MD, PhD, a kidney specialist with Yale Medicine.

With that said, Dahl thinks parents and kids should know what to watch for.

Tell your child to speak up if they notice:

  • Back or side pain
  • Blood or pain when they pee
  • Any other urine changes 
  • Frequent headaches

Encourage your kids to keep track of their health details.

“If the doctor tells them their blood pressure is a little high, take down that number,” Warejko says. “Everyone has a piece of ‘paper’ wherever they go because our phones have ‘notes.’”

You can also share what the disease feels like for you. But keep in mind that your child may not have the same experience.

“The tricky thing about ADPKD is that everyone is a little bit different, even among family members,” Warejko says. “But I always encourage families to be open with one another.”

When and How to Get Diagnosed

Children may only need regular wellness visits once a year. But their doctor should check for high blood pressure or blood in the urine.

“If those are off, that’s a reason to investigate further,” Dahl says. “Otherwise, it’s OK to monitor kids to see how they’re doing.”

Warejko stresses the importance of annual checkups throughout the late teens and early 20s. Sometimes young adults who might have ADPKD skip these screening tests, she says, because they feel healthy. Though they could have high blood pressure or cysts and not know it.

“One of the hardest parts about kidney disease is you don’t feel it until you feel it,” she says.   

To diagnose ADPKD, you may get:

Imaging tests. The most common one is an ultrasound, Dahl says. It’s a painless test that can take pictures of the urinary tract or kidneys. Other imaging tests include a computed tomography (CT) scan or magnetic resonance imaging (MRI). 

Genetic testing. This is a surefire way to know if you have ADPKD. But it might not be the right choice for everyone, especially young kids. “There’s a lot of debate about what it means to genetically test someone who’s under the age of 18, when they’re not at an age that they can fully consent,” Warejko says. “Not every person wants to know what their genetic predisposition is.”

Ask your doctor to refer you to a genetic counselor. They can help you figure out if this is something you or your family might want to do.

Pre-implantation screening. This is for your adult kids who want to start a family. People who use in vitro fertilization (IVF) can get a special test to check for ADPKD in fertilized eggs. It’s not 100% accurate. But it can be a choice for people who want to take steps to lower the odds that they’ll pass on the gene, Dahl says.

Set a Good Example

You can teach your kids some healthy habits to protect their kidneys. If they ask why, tell them you want to help them achieve their goals. That’s what Warejko does. She asks kids what they want to be when they grow up. Then she tells them, “I want you to be successful, and part of that is being healthy.”

Her tips include:

  • Drink plenty of water.
  • Eat a low-salt diet.
  • Get enough physical activity.
  • Eat lots of fresh fruits and vegetables.
  • Avoid processed and fast foods.

Warejko knows it’s not easy for everyone to make these healthy changes. Some families may not have access to fresh produce or live in a neighborhood where it’s safe to go for walks. That’s why she makes an effort to brainstorm with kids and parents. She’ll help them find ways to get healthy food or exercise in their home. “I try to specifically ask, ‘What are the restrictions we need to work inside?’”

Life With ADPKD

Treatment has come a long way in recent years.  In 2018, the FDA approved tolvaptan (Jynarque), the first drug to treat adults with ADPKD. It can slow down cyst formation on the kidneys.

“I think tolvaptan is going to change the trajectory and we’re not going to see as many people ending up with kidney failure,” Warejko says. “My hope is that snowball will continue to roll downhill.”