How Kansas' Medicaid Drug Policy Affects My Daughter With Chronic Rare Diseases

This is my daughter, Cambria. She’s 7 and lives with two rare chronic illnesses: panhypopituitarism and adrenal insufficiency. To manage her condition, Cambria receives a nightly injection that stabilizes her blood sugar, regulates her body’s internal systems, and keeps her alive and healthy. These shots will continue throughout her life.

To tweak her medication regimen, it has taken seven-plus years of trial and error, careful observation, repeated blood work, incredibly large sums of money (even with private insurance) and multiple trips to see the best specialists in America. Though her injection “sucks,” she understands her life is at stake if she doesn’t have it.

I don’t know about anyone else, but when I was 7, all I worried about was what flavor Flintstone Vitamin I’d wind up with at breakfast. At 7, my own mortality never even crossed my mind. Today my heart was broken when Cambria questioned her own upon learning Step Therapy would have an impact on her, too.

Last June, Kansas passed a Medicaid drug policy referred to as “fail forward” or Step Therapy. Despite aggressively campaigning against this initiative, many lawmakers supported and pushed for the passage of this Medicaid policy as a “cost saving measure.” (Some of these lawmakers were even present at our 2016 NORD Rare Disease Awareness event and saw firsthand the faces of those this policy would affect.)

In a move lawmakers thought would reduce the costs of KanCare, the state’s privatized Medicaid program, Step Therapy requires KanCare members to start patients on less expensive drug therapies before moving them to a more expensive, sometimes newer therapy. In short, you must fail on a less effective medication, have medical documentation of the symptoms, and endure unknown medical effects on two different Tier 2 medications before you qualify for a clinician to review your Tier 3 request. There is no guarantee, even with your physician’s letter and documentation, that a clinician behind a desk with limited knowledge or your case will approve that higher cost medication. This is unacceptable.

Today we received a letter from Blue Cross Blue Shield Kansas City, dated January 17, 2017. The letter stated that due to changes in our prescription drug list, a result of decisions made by a BCBS committee that meets quarterly, Cambria would no longer be eligible for her current medication under the Step Therapy Plan. All changes are effective March 1, 2017, and no patient will be grandfathered in on their current treatment.

If the BCBS committee meets quarterly, it’s a safe bet this “pricing decision” was made in 2016. In waiting approximately six weeks before a major policy change before informing affected patients by mail, BCBS has been incredibly irresponsible. If you’ve ever waited for a medical authorization, you understand the agony of waiting for a treatment for yourself or a loved one.

We first learned of Cambria’s condition when she had a “crash” at home – she was literally dying right in front of us. We rushed her to the hospital where a team of 10 miraculously stabilized her for transport to the local children’s hospital downtown. During her five-day stay, we were told to initially qualify for her life-saving injection, the insurance company required a failed stimulation test. A stim test consists of baseline blood draw, a hormone injection, and repeated blood draws at specific intervals to prove your body cannot sustain.

During the test, Cambria visibly declined, leaving me pleading with nurses to stop the test. For the second time in five days, she crashed and became unresponsive. Though we were in the hospital with qualified staff, they struggled to raise Cambria’s sugars, unsuccessfully trying several treatments before stabilizing her. Watching your daughter headed toward death twice in two days is difficult, but we thought the worst had passed.

We were wrong.

We waited six long months after her stim test for Cambria’s initial authorization in 2009. At discharge, doctors advised us to wake our 4-month-old daughter every 90 minutes to give her a bottle of apple juice laced with cornstarch to maintain her blood sugars. If we missed a single feeding, we risked waking up to a dead baby. If we were lucky, she might “only” experience brain damage or a coma. If not for a doctor who called our insurance company and demanded an approval during a follow-up appointment, I’m not sure how long we would have waited for an approval.

As a private patient advocate for individuals and a public advocate for the rare disease community, I frequently hear indifferent attitudes about Medicaid decisions from families who are blessed to enjoy privatized insurance options. The “it doesn’t affect me” attitude is rampant.

BCBSKC proved today that financially driven decisions made when lives are at stake are the worst decisions – and they affect us all. I believe the Kansas Step Policy is not only harmful, causing undue pain and hardship for patients, it is irresponsible and fiscally unsound. Since there are no timelines for failure, patients can be restricted to Tier 2 medications for unknown periods of time to satisfy a clinical requirement of which there is no public documentation. The costs of treating symptoms and conditions resulting from an inferior medication are likely much higher than providing Tier 3 access in the first place. Compounding this issue is the fact that two drugs must be failed on before returning to an initial Tier 3 prescription.

Why are we touting the success of additional NIH funding and research developments at KU when we are not utilizing newer medications that already exist? Why do we even bother putting any money into research at all when our lawmakers are determined to restrict access to treatment in the name of cost savings?

On March 6, I will be in Topeka to host NORD’s Rare Disease Day Expo for lawmakers, industry stakeholders, patients and non-profits. Kansas failed in all seven areas of the NORD report card – the only state with that distinction. It is time for all of us, regardless of our participation in state programs or private insurance, to band together and show lawmakers we are people with faces and families – not cost savings.

We want to hear your story. Become a Mighty contributor here.

Thinkstock photo by Nomadsoul1

Find this story helpful? Share it with someone you care about.

Related to Rare Disease

girl looking out of tent window

Why Parents Searching for a Diagnosis Need to Know About Whole Exome Sequencing

This morning I received an email from a parent whose child was just diagnosed with a KIF1A mutation. The family has suffered through almost 10 years of misdiagnosis, agony and unnecessary (often painful) tests. These parents have been on a decade-long quest for an answer to their son’s degenerative disease. Not once in 10 years [...]
woman carrying sign that says i am not free while any woman is unfree

To the 3 Women Who Rallied For My Rare Disease at the Women's March

I write this piece on a harsh day for disabled students in our public school system as Betsy DeVos’ nomination becomes a stark reality, even as it flouts opposition from national education organizations, students with special needs and disability advocacy groups, American parents’ demands for a qualified candidate, and the demands of many American citizens. As [...]
people running in a marathon on a wetted surface

My Life With Illness Is Like Running the Boston Marathon While Sick. But I'm Winning.

Yes. I am sick. I know you can’t tell. I look just like anybody else you may pass on the street. Maybe even a little bit better? (I put a lot of effort into that, thank you). That’s OK. I actually kind of like it better this way. You see… 1) I like that I [...]
rare disease day 2017 image

10 Ways to 'Defy Rare' on Rare Disease Day's 10th Anniversary

Rare Disease Day, on Tuesday, February 28, 2017, is fast approaching!  This is the one day each year that rare disease is recognized. Rare Disease Day is organized by Eurordis, Rare Diseases Europe, and includes partners celebrating from all over the world. This is the 10th year the event. Rare Disease Day activities occur throughout February [...]