How a routine well-child check ended in a von Willebrand disease diagnosis

WhenOlivia Pipinich

went in to Sanford Children’s for her two and a half year well-child check, her parents Tim and Katie Pipinich assumed it would be like any other appointment. Unfortunately, that was not the case.

A few hours after her initial appointment, Katie received a call asking for Olivia to come back in as her blood tests were off.

After the second round of tests, Katie received yet another call. “It was at 5:30 p.m., which I knew was not a good sign. I was told there were abnormalities with Olivia’s blood tests and that she wanted us to go in at 9 a.m. to the new medical center for an iron infusion.”

While receiving the infusion, things took an unexpected turn, resulting in an extended stay at Sanford Medical Center Fargo.

“As she was getting infusions and fluids, she just started to crash,” Katie says. “She started to look worse and worse and we were told we couldn’t go home. We ended up staying there for three days.”

After various tests, questions and days spent in the hospital, Olivia was diagnosed with von Willebrand disease. “Von Willebrand disease is a genetic bleeding disorder caused by a missing or defective clotting protein called von Willebrand factor,” Jana Suder, a nurse practitioner specializing in hematology at Sanford Health, says.

“There can be bleeding around the brain and spinal cord with a traumatic injury and a high risk for excessive bleeding after surgeries, dental procedures and deliveries. If not appropriately managed, the bleeding can lead to severe iron deficiency anemia, hypovolemic shock, and even death.”

Due to the nature of this disease, von Willebrand is something that will affect Olivia as she grows up and becomes more active. “She can play most sports and participate in any activity but some modifications may be needed to keep her safe,” Suder says. “For instance, she’ll need to wear a helmet when riding bike to protect her from a head injury. She will also need to be careful with high contact sports and most likely require taking medication prior to these activities. If she develops an in- jury, she will need to be evaluated urgently to make sure we get any bleeding under control before problems arise.”

Because von Willebrand can be unpredictable, Olivia sees Suder for regular blood draws at Sanford Health Center for Bleeding and Clotting Disorders located at the Roger Maris Cancer Center in Fargo.

“Olivia uses a mask and gets nitrous gas any time she has a blood draw because if her heart rate is elevated, even in the stress of a normal blood draw, it can skew the numbers,” Katie says. “It’s extremely hard to read, so she has to be very calm.”

Before going in for an appointment, Olivia’s doll, Ellie, helps her to understand what happens when she gets her blood drawn. “Ellie is actually a medical play doll. She comes with a suitcase that has the nitrous gas mask for the doll, so we practice on her before she gets her blood drawn,” Katie says.

Ellie has been an important piece in helping Olivia remain calm before blood draws. “I can’t even imagine what it was like for a three-year-old brain,” Tim says. “We asked, ‘Can we put this on Ellie like you use your mask?’ And then it was a lot less scary for her.”

Although the Pipinich’s would rather not spend their days at the hospital, their experience at Sanford Health has helped make appointments manageable. “Olivia has always felt comfortable here,” Katie says. “Our experience at Sanford has been really good; I can’t imagine it going any better.”

As Olivia grows, she will continue to require yearly visits to the specialized bleeding disorders clinic, but Tim and Katie have remained positive. “I have my moments where I freak out about really small, insignificant things, but it’s easy to be happy when she’s so happy,” Katie says. “If she’s good, we’re good.”

“It’s really cool to see how resilient, strong and brave a little two and three year old can be,” Tim says. “To see her spark and spirit return has been really nice.”