Increased IBD diagnoses using BYK-CVR

Combined, Better You Know (BYK), a self-bleeding assessment tool, and Community Voices in Research (CVR), a patient registry, can facilitate earlier, faster diagnoses of inheritable blood disorders (IBDs) in females.  Each tool informs the other: BYK directs females whose scores indicate an IBD to healthcare providers (HCPs) for confirmation of a diagnosis.  Once diagnosed, women are encouraged to enroll in CVR to share information over several years through regular surveys.  The resulting confidential, deidentified, aggregate data is funneled back into the awareness component of BYK.

In response to MIT’s call for solutions, the National Hemophilia Foundation (NHF) will shape the data from the ever-expanding cycle of BYK and CVR into educational content to be shared with advanced practice providers (APPs) and school nurses, who increasingly serve as the first point of contact for disinvested community members.  Upon identifying IBD symptoms, they can guide patients to BYK and CVR. 

An estimated 1% of females in the U.S. have an IBD, yet many are routinely mis- or undiagnosed.  IBDs, like von Willebrand disease (VWD) or platelet disorders, occur when blood lacks the proteins or platelets essential to forming clots.  In women, IBD symptoms commonly present as heavy, protracted periods.  Symptoms can also include frequent nosebleeds; heavy post-surgery bleeding; easy, large bruising; and anemia.

Because these symptoms can indicate numerous conditions, often they are attributed to diagnoses other than IBDs—or dismissed entirely.  Even among women in better-served communities (e.g., women with commercial insurances/more resources), computer modeling by researchers in 2020 suggests that even females with relative privilege appear to be regularly undiagnosed, despite presenting with "VWD or other mucocutaneous bleeding disorders."

By extension, females lacking health insurance or access to care are at greater risk of not being diagnosed, and of suffering poorer health outcomes even if a correct diagnosis is made.

To improve diagnosis of IBDs in women and girls, NHF will utilize CVR data to identify patterns among those with confirmed diagnoses.  The data collected from CVR will educate, via regionalized pilot presentations, APPs and school nurses, who are increasingly among the first providers to treat underserved populations, about the BYK self-bleeding assessment tool available to symptomatic females, as well as NHF resources.  NHF’s Research Department will develop and update presentations to be offered at local, state, regional, and national APP and school nurse meetings.  The BYK assessment tool and literature will be made available to meeting participants as digital and physical copies.  Access to specialized care for IBD consultation and potential referral will be facilitated. 

The first indicator of an IBD is heavy, protracted menstrual bleeding.  Per current CVR data, additional symptoms considered indicative include:

• Passing clots that are nickel-sized or larger during menstruation (75% of respondents);
• Decreased participation in social activities due to pain during menstruation (82%);
• Bleeding before or after childbirth (58%); and
• Bruising, muscle hematoma, or heavy periods in past six months (79%).

As APPs guide more patients to BYK/CVR, stronger patterns will emerge in CVR that will contribute to faster diagnoses of IBDs.

NHF's solution is designed for females, from underserved communities, exhibiting symptoms of inheritable blood disorders (IBDs) and the providers most likely to care for their health.

The inferiority of health care received by women in the U.S. compared to men--and compared to women in other developed countries--is well documented.  Regardless of race or ethnicity, socioeconomic status, or access, the women seeking medical help from providers are regularly dismissed and considered neurotic, bounced from practitioner to practitioner until finally a correct diagnosis is given--if a correct diagnosis is given.

Women of color, women with less income, and women in locations with fewer healthcare providers (HCPs) disproportionately bear the burden of these inequities.  As part of its creation of a patient-centered research agenda, NHF has established six working groups, including one for women and IBDs and one for underrepresented community members and IBDs, that empower patients to share their experiences with other IBD stakeholders developing equitable solutions to care.

Specific to IBDs, on average, females with VWD wait 16 years from the onset of symptoms before receiving a diagnosis and corresponding treatment.  (By comparison, males are typically diagnosed with hemophilia within 36 months of birth.)  Within that timeframe, HCPs with little to no knowledge of IBDs will recommend inappropriate solutions to manage bleeds (e.g., nasal cautery, hysterectomies) despite the existence of less invasive and more effective treatments and therapies.   

Failure to diagnose IBDs in females results in numerous consequences.  In children, VWD can lead to "lower scores for physical functioning, role function - emotional/behavioral, general health perceptions, and physical summary."  Females with a VWD diagnosis in Ontario, Canada, reported lower health-related quality of life than females without VWD.  Joint bleeds associated with VWD impact patient mobility.  Protracted, heavy periods and corresponding pain can interfere with a menstruating person's school or work attendance and impact their participation in social activities.  Likewise, undiagnosed females shoulder high, unintended healthcare costs (e.g., emergency room visits, surgeries) that, with proper diagnosis and treatment, are avoidable.  

Though, as researchers in 2020 noted, "[t]here remains a need for research on the lived mensuration-related experiences of American adolescent girls, especially girls identifying as Black, Indigenous, or People of Color," it is known that school nurses are often relied upon for menstrual pain and bleeding.  In fact, it is at school during the "period talk" that many girls first learn of their ability to menstruate.  A commonality among most U.S. youths, school and school nurses serve as necessary vehicles through which BYK/CVR data can be shared to improve IBD diagnoses, which, in turn, can lead to an increase in IBD data collection from newly diagnosed young patients through CVR.

Likewise, in 2009, The Journal of Rural Health published findings that patients "without insurance or with public insurance other than Medicare were more likely than those with private insurance to utilize [physician assistants/nurse practitioners]."

Presenting on data collected in CVR to APPs and school nurses increases the likelihood that they will be able to identify the symptoms of IBDs in women and girls from underserved communities.  Providers' awareness of the BYK assessment tool can be used to guide females to an IBD diagnosis (or rule an IBD diagnosis out) that is confirmed by specialists with sufficient IBD training, and supplemented by the resources made available by NHF.  

The aggregate data in CVR illuminates commonalities in symptoms experienced by females with IBDs.  As NHF works to improve provider awareness of IBDs, CVR data unlocks the potential for faster diagnosis of IBDs in females--in this context specifically, females from underrepresented communities relying on APPs and school nurses for primary medical care.  Awareness of IBDs, the BYK assessment tool, and the potential for better diagnosis of IBDs in females strengthens the BYK/CVR cycle, especially as diagnosed females from disinvested communities choose to share their experiences in CVR, which fosters deeper understanding of the interplay between IBD symptoms and patient underrepresentation.  

BYK and CVR were established as NHF programs within the past few years, and NHF continues to broaden their application.  This specific solution is born of the need to adequately address health disparities experienced by underserved communities in the U.S.  As NHF works closely with women and minorities with IBDs through the development of the patient-centered research agenda, piloting this phase of the BYK/CVR project is timely and can be refined by patients actively involved with NHF in shaping the future of diagnostics and care for others with IBDs.