PKD Life - Summer 2022 - 18

Advocating from the
HEART
A
Parents of kids with ARPKD share how they ensure
their children get the care and support they need.
By Andrea Atkins
ll Michele Karl wanted when her son
went to the school nurse's office was a
simple phone call. But school officials
saw it as overkill. " We'll notify you if it's
related to his kidneys, " she was told, but not each
time he goes to the nurse.
Karl wasn't satisfied. Would the school nurse
know that if Max came in with a headache, it might
be because he was dehydrated, and that being dehydrated
is dangerous for children with autosomal
recessive polycystic kidney disease (ARPKD)?
Would the school nurse, who knew very little about
PKD, even know if his complaints were kidneyrelated?
Karl's fight went all the way to the school
superintendent, who ultimately sided with her.
" This is ridiculous, " he said. And Karl added the
nurse notification into Max's 504 plan, which under
law guarantees accommodations for his health.
Advocating for a child who has ARPKD can seem
like a never-ending battle. The disease can encompass
other bodily systems and arise in unexpected
ways, many of them serious. Further, patients can
look perfectly fine on the outside, while inside
their kidneys are at war. Lastly, PKD, and especially
ARPKD, is a little-known, little-understood disease,
which means that family members often have to be
the ones explaining-even to medical personnel.
No wonder moms like Karl, of White Plains, New
York, and Melissa White, of Salem, Massachusetts,
feel like warriors. " If I don't advocate for Steven,
no one else will, " says White, whose 3-year-old
son has ARPKD, which was evident at his birth
and has led to more hospitalizations in his short
life than she can count. Steven has a team of doctors
that includes renal specialists, nutritionists,
18 PKD LIFE * SUMMER 2022
a gastroenterologist, hepatologist, pulmonologist,
dermatologist, and an asthma specialist. He has
underdeveloped lungs, heart problems, and issues
with his bowels.
" When he gets sick, " White says, " he gets really
sick. " He has been on as many as four different
blood pressure medicines (he's down to two now).
He receives physical therapy and occupational therapy.
White feels she is constantly educating the rest
of the world about ARPKD, since most have never
heard of it and cannot tell by looking at her son that
anything is wrong. In the face of these challenges,
White says, the medical team at Boston Children's
Hospital has " been like family to us " and has helped
her through many scary moments with her son.
It can be challenging, both moms agree. But it
can be easier if you follow some of these steps:
Communicate often: In addition to Max,
now 22, Karl's 16-year-old son, Gabe, has ARPKD.
She emails each of his teachers at the start of
every school year to explain the disease and
his accommodations.
Communicate kindly: Karl says she often takes
a breath before she speaks because, although she
may be frustrated or annoyed when people don't
understand her sons' needs, she wants to be kind
and compassionate with those who care for her
children. Aware that she is modeling advocacy for
her sons, who will have to take care of themselves
someday, she tries to set the best example she can.
Keep notes: Karl keeps them on her phone;
White uses a notebook. Either way, having notes
makes it easier to access information that might be
helpful. And before every doctor's visit, write down
your questions so you don't forget anything.
If your
gut is
telling you
something
is wrong,
trust the
feeling.
PKD Life - Summer 2022

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