Self-Efficacy, Perceived Barriers to Care, and Health-Promoting Behaviors Among Franco-Americans Across Cardiovascular Risk Factors: A Cross-Sectional Study.

PURPOSE: To assess the prevalence of perceived barriers to accessing health care services, self-efficacy, and health-promoting behaviors among Franco-Americans as a higher-risk group for familial hypercholesterolemia (FH), stratified by cardiovascular risk factors. DESIGN: Cross-sectional survey based on components of the Health Belief Model. SETTING: Administered in-person at a Franco-American cultural center and online through mailing lists and social media platforms in the Northeastern United States. SAMPLE: Franco-Americans and French Canadians (n = 170). MEASURES: Demographic and clinical characteristics (i.e. high cholesterol, prior heart attack or stroke, family history of atherosclerotic cardiovascular disease (ASCVD), diagnosis of FH), perceived barriers to accessing health care services, self-efficacy, and health-promoting behaviors (i.e. taking lipid-lowering medications, seeing a cardiovascular specialist). RESULTS: In a cohort of Franco-Americans, 42 (25%) had both high cholesterol and family history of ASCVD. Among Franco-Americans with both cardiovascular risk factors, 22% had low self-efficacy and only 16% had discussed FH with their physician. Individuals with both risk factors were significantly more likely to report a concern over a future diagnosis as a barrier to accessing health care services when compared with those with neither risk factor (36% vs. 15%, p = 0.014). Overall, other prominent barriers to care included knowledge of when to seek help (27%) and a distrust in medicine (26%). CONCLUSION: Franco-Americans report significant barriers to accessing health care services. Our findings strengthen the case for developing focused public health strategies to raise awareness for FH, particularly among high-risk subpopulations with unmet cardiovascular needs.

Familial Hypercholesterolemia and the Founder Effect Among Franco-Americans: A Brief History and Call to Action.

Familial hypercholesterolemia (FH) is an inherited disorder characterized by chronically elevated low-density lipoprotein cholesterol levels and an increased risk of premature atherosclerotic cardiovascular disease. FH has been shown to disproportionately affect French Canadians and other ethnic populations due to the presence of a founder effect characterized by reduced genetic diversity resulting from relatively few individuals with FH-causing genetic mutations establishing self-contained populations. Beginning in the mid-1800s, approximately 1 million French Canadians immigrated to the Northeastern United States and largely remained in these small, tight-knit communities. Despite extensive genetic- and population-based research involving the French-Canadian founder population, primarily in the Province of Quebec, little is known regarding Franco-Americans in the United States. Concurrent with addressing the underdiagnosis rate of FH in the general population, we propose the following steps to leverage this founder effect and meet the cardiovascular needs of Franco-Americans: (1) increase cascade screening in regions of the United States with a high proportion of individuals of French-Canadian descent; (2) promote registry-based, epidemiological research to elucidate accurate prevalence estimates as well as diagnostic and treatment gaps in Franco-Americans; and (3) validate contemporary risk stratification strategies such as the Montreal-FH-SCORE to enable optimal lipid management and prevention of premature atherosclerotic cardiovascular disease among French-Canadian descendants.

L'hypercholestérolémie familiale (HF) est un trouble génétique caractérisé sur un mode chronique par des taux élevés de cholestérol à lipoprotéines de faible densité et un risque accru de maladie cardiovasculaire athéroscléreuse prématurée. L'HF touche de façon disproportionnée les Canadiens français et d'autres groupes ethniques par suite d'un effet fondateur caractérisé par une faible diversité génétique résultant d'un nombre relativement peu élevé d'individus ayants des mutations génétiques responsables de l'HF établissant des populations isolées. À partir du milieu des années 1800, environ un million de Canadiens français ont émigré dans la région nord-est des États-Unis et y sont pour la plupart demeurés au sein de communautés relativement restreintes et fortement homogènes. En dépit d'une vaste recherche génétique et populationnelle visant la population fondatrice canadienne-française, principalement dans la province de Québec, on sait peu de choses sur les Franco-Américains aux États-Unis. Parallèlement à une démarche ciblant le sous-diagnostic de l'HF au sein de la population générale, nous proposons les mesures suivantes pour tirer parti de cet effet fondateur et répondre aux besoins cardiovasculaires des Franco-Américains: 1) augmenter le dépistage en cascade sur les régions des États-Unis comptant une forte proportion de personnes d'ascendance canadienne-française; 2) promouvoir la recherche épidémiologique fondée sur les registres pour obtenir des estimations précises de la prévalence et cerner les lacunes touchant le diagnostic et le traitement chez les Franco-Américains; 3) valider les stratégies contemporaines de stratification du risque comme celle fondée sur le Montreal-FH-SCORE pour permettre la prise en charge optimale de la lipidémie et la prévention des maladies cardiovasculaires athéroscléreuses prématurées chez les descendants canadiens-français.

Longitudinal low density lipoprotein cholesterol goal achievement and cardiovascular outcomes among adult patients with familial hypercholesterolemia: The CASCADE FH registry.

BACKGROUND AND AIMS: There are limited data from the US on outcomes of patients in specialty care for familial hypercholesterolemia (FH). METHODS: CASCADE FH Registry data were analyzed to assess longitudinal changes in medication usage, in low density lipoprotein cholesterol (LDL-C) levels, and the rate of major adverse cardiovascular events (MACE (myocardial infarction, coronary revascularization, stroke or transient ischemic attack) in adults with FH followed in US specialty clinics. RESULTS: The cohort consisted of 1900 individuals (61% women, 87% Caucasian), with mean age of 56 ±â€¯15 years, 37% prevalence of ASCVD at enrollment, mean pretreatment LDL-C 249 ±â€¯68 mg/dl, mean enrollment LDL-C 145 mg/dl and 93% taking lipid lowering therapy. Over follow up of 20 ±â€¯11 months, lipid lowering therapy use increased (mean decrease in LDL-C of 32 mg/dl (p < 0.001)). Only 48% of participants achieved LDL-C < 100 mg/dl and 22% achieved LDL-C < 70 mg/dl; ASCVD at enrollment was associated with greater likelihood of goal achievement. MACE event rates were almost 6 times higher among patients with prior ASCVD compared to those without (4.6 vs 0.8/100 patient years). Also associated with incident MACE were markers of FH severity and conventional ASCVD risk factors. CONCLUSIONS: With care in FH specialized clinics, LDL-C decreased, but LDL-C persisted >100 mg/dl in 52% of patients. High ASCVD event rates suggest that adults with FH warrant designation as having an ASCVD risk equivalent. Earlier and more aggressive therapy of FH is needed to prevent ASCVD events.

Health disparities among adult patients with a phenotypic diagnosis of familial hypercholesterolemia in the CASCADE-FH™ patient registry.

BACKGROUND AND AIMS: Most familial hypercholesterolemia (FH) patients remain undertreated, and it is unclear what role health disparities may play for FH patients in the US. We sought to describe sex and racial/ethnic disparities in a national registry of US FH patients. METHODS: We analyzed data from 3167 adults enrolled in the CAscade SCreening for Awareness and DEtection of Familial Hypercholesterolemia (CASCADE-FH) registry. Logistic regression was used to evaluate for disparities in LDL-C goals and statin use, with adjustments for covariates including age, cardiovascular risk factors, and statin intolerance. RESULTS: In adjusted analyses, women were less likely than men to achieve treated LDL-C of <100 mg/dL (OR 0.68, 95% CI, 0.57-0.82) or ≥50% reduction from pretreatment LDL-C (OR 0.79, 95% CI, 0.65-0.96). Women were less likely than men to receive statin therapy (OR, 0.60, 95% CI, 0.50-0.73) and less likely to receive a high-intensity statin (OR, 0.60, 95% CI, 0.49-0.72). LDL-C goal achievement also varied by race/ethnicity: compared with whites, Asians and blacks were less likely to achieve LDL-C levels <100 mg/dL (Asians, OR, 0.47, 95% CI, 0.24-0.94; blacks, OR, 0.49, 95% CI, 0.32-0.74) or ≥50% reduction from pretreatment LDL-C (Asians, OR 0.56, 95% CI, 0.32-0.98; blacks, OR 0.62, 95% CI, 0.43-0.90). CONCLUSIONS: In a contemporary US population of FH patients, we identified differences in LDL-C goal attainment and statin usage after stratifying the population by either sex or race/ethnicity. Our findings suggest that health disparities contribute to the undertreatment of US FH patients. Increased efforts are warranted to raise awareness of these disparities.