Kayla Gregory is sixteen and one-in-a-million. She has Type 3 VWD. If you or someone you love has this rare disease, Kayla’s journey may sound familiar; her experiences close to home. Interviewing her mother Kari, I am struck by how eloquently she speaks of Kayla’s life with Type 3 VWD. So far, they have made this journey together; proof that being one-in-a-million does not mean having to go it alone. Kari is both proud and possessive of these sixteen years and all I picture is a Lioness.

Yes, Kari is a Lioness in this story.

Lioness Facts¹

Out of all female animals, Lionesses are the most admired.

Role of Lioness

Providing for the “pride” (the family) is the main job; to hunt prey for the rest. Lacking a mane they make it impossible to be noticed by other animals. They protect by searching for the safest place for their cubs and make sure to teach [them] important survival skills.

The Bottom Line

Lionesses are said to be the bravest. It’s no wonder lion’s day comes the next day after woman’s day. If you want a true definition of a courageous and let Lioness be your role model, and imitate how they roar!

Kari is a full-time mother of three, and an attorney with a leading integrated energy company. She and husband Chris first grasped the severity of Kayla’s Type 3 VWD diagnosis when she was six weeks old. Kari checked in with her father, a hospital pharmacist, when Kayla woke up with spontaneous bruising on her feet and legs. Alarm bells signaled. Living in San Francisco at the time, they had rich medical options; CPMC (California Pacific Medical Center) or UCSF (University of California San Francisco). These initial experiences set the stage for Kari’s future education and role as patient advocate.

She became a sponge for all things VWD. There was the obvious fear of the unknown. There were important medical decisions to be made. She had questions, “do I want a relationship with a softer, kinder hospital or one in which there is a deep bench of experts in the field of severe bleeding? How and where do I learn more about what’s happening with my daughter? Where does my pediatrician fit in?”

Her journey as Lioness had begun.

It was in these initial days of stress and strain that she and Chris met Dr. Marion Koerper. Now retired, Koerper was a renowned hematologist who would inform and transform their world. As Kari asserts, “the first thing she did was take the leading book on hematology out of my Dad’s hands.” [Today, this is comparable to a doctor begging us not to Google a symptom in search of a cure!] “What she did do, however, is reassure us that Kayla could live a very normal life. She would not recommend [Kayla] going out for wrestling or tackle football, and [Kayla] may require factor infusions on a regular basis; but she would be OK and could do all that we had dreamed for her.”

From initial confusion, Kari emerged with comfort. She was already distinguishing the importance between care and experienced care, and she was introduced to the critical role that HTC’s (Hemophilia Treatment Centers) would play in the ongoing support for Kayla.

The Lioness Protects

There have been major hospitalizations that have required Lioness grit. Thankfully these have been few and far between.

At 9 months, Kayla experienced a brain bleed from a short fall onto a carpeted surface. For fourteen days doctors poked and probed this fragile infant and parents agonized. Ten years later Kalya fell to a severe infection from her port which left her in kidney failure. Kari had noticed her picking nervously at her port in recent weeks and had chided her about it, but the damage had been done. Hospitalized and in the ICU over the Thanksgiving holiday, Kari was full throttle, insisting and advocating for the experience of the attending physicians, or as Kari likes to call them, “the gray-haired doctors who could tell Kayla was not recovering as expected and something was seriously wrong.” Kari had developed a deep understanding and vocabulary around the idiosyncrasies of VWD. She had long acknowledged not all physicians come with the same level of expertise in treating severe bleeding disorders. This was both Lioness grit and patient advocacy operating in high gear. Of these tense days she says “this was absolutely no time to be shy about insisting on the senior team.”

That next year, Kari would proactively advance the standard of care for her daughter. Kayla’s pediatrician had warned that her first menstruation was imminent, and Kari was determined to have a plan in place. She worked to identify a gynecologist who would be willing to help them, discovering that gynecologists willing to work with girls Kayla’s age were few and far between. Kari’s persistence led her to a pediatric gynecologist at Stanford and together, they created a plan that would start with hormonal pills and then quickly move to an IUD if necessary. Fortunately, they were ready for the IUD since the hormonal pills were not sufficient and Kayla did not stop bleeding once she started menstruating. The IUD has been life-changing and allowed Kayla to gain independence and control over her periods for years going forward. Kari reflects, “her 14-year-old unaffected sister only wishes she could be so lucky!”

The Lioness and the Learning Curve

Sixteen years ago, baby Kayla could not fathom the arc of Kari’s journey. Or the beginning of her own. As Kari asserts “VWD doesn’t put a stamp on your forehead giving you instructions of what to do, when and how and there is so much to be known and learned about this disease.” Kayla’s pediatricians had no experience with Type 3 VWD and much of the burden of care has fallen on Kari and Chris.

The Lioness was both learning, and teaching, survival skills.

Kari connected with local Hemophilia organizations. She researched bleeding disorders and read voraciously about Von Willebrand Disease. She found camaraderie among parents facing similar challenges although it would be several years before meeting someone else with Type 3 VWD. She learned everything about ports and peripheral access; valuable tools for protecting Kayla’s future. She absorbed tips for negotiating the school system and health care systems and navigating emergency and hematology rooms. The Lioness became fearless in connecting up with social workers, pharmaceutical reps, and home health care companies providing factor and nursing support. When Kayla was seven, their pharmaceutical rep invited them to the first-ever Von Willebrand conference where Lioness and Cub met, for the first time, a handful of others with Type 3 VWD. They were encouraged to push for attendance at the National Hemophilia Foundation’s Annual Meeting later that year. While Kari enjoyed the camaraderie, she was disappointed with the lack of focus on the severe form of the disease; surfacing new worries and concerns for how she could best support Kayla’s future. The floodgates had been broken open and there was new urgency for answers.

The Lioness Waits

In retrospect Kari was waiting for 2017 and the first conference devoted exclusively to supporting people with Type 3 VWD. This would be followed by the formation of VWD Connect Foundation, subsequent conferences, and Kari’s appointment to its Board as Patient Representative, on which she served from 2021-2023.

Besides feeling like destiny, this also seems confirmation of her sixteen-year parallel travels with Kayla. Today, Kari IS a dictionary of knowledge about Type 3 VWD. She has empathy for parents struggling with infants and young children. She knows how to navigate the system of healthcare and insurance and is ready to help others traverse this landscape and advocate for their children and loved ones. Most importantly she can explain this complex and complicated disease in terms most can wrap their brains around. Today, Kari participates in the Foundation’s ongoing Parent Connection, bi-weekly Book Club meetings, monthly Trivia nights and other virtual events. She continues to be a veteran member of the community and a welcoming voice for new members at annual conferences.

The Lioness and the “Cub”

This story would not be complete without some words about the “Cub”. Growing up Kayla thankfully did not gravitate towards wrestling or tackle football. She did try a bit of tap dancing and ballet though. Before leaping to on-pointe (and the potential for more ankle bleeds), she instead decided to focus on her love of the pool and discovered the thrill of competitive swimming in breaststroke and IM (individual medley). She fully intends to keep the action going in college.

When asked how Kayla lives with the unpredictability associated with severe Type 3, Kari radiates pride. “She’s completely comfortable with it. She understands all its severity and risks. She learned to face them head on at an early age and doesn’t allow them to detract from what she enjoys most. She loves to talk to friends about it and explains it all. She has the tools to do it. She knows she has the support of doctors, social workers, home health aides and the medical community. What’s more, she has the vocabulary and explanations of how it affects her — and she’s not going to be marginalized.”

The journeys of Kari and Kayla have been interconnected in many ways and co-dependent in all the best ways. Actively engaged with VWD Connect Foundation I asked Kari for three adjectives to define the work of the organization. Not missing a beat, Kari ascribes “powerful, impactful, and revolutionary”.

Roared like a true Lioness.

¹ Source: savannaaddict.com | animal welfare association

Cindy Connelly is a marketing strategist and communications writer living in Boston.

To find out how you can be a part of VWD Connect Foundation, please visit our Donation page and consider becoming a volunteer.