Data Visualization Technical Notes
Sex is displayed as the sex that was assigned to the participant at birth. Hemophilia is a genetic disorder that involves mutations of genes located on the X chromosome; thus, it is important to evaluate sex differences in hemophilia based on an individual’s chromosomes (XY vs. XX), which do not change after birth, even in individuals who change their gender later in life.
Race is displayed as the following race categories: White, Black or African American, Other races, and More than one race or Unk (unknown). The category “Other races” includes American Indian/Alaska Native, Asian, and Native Hawaiian or Other Pacific Islander. The category “More than one race or Unk” reflects a person who belongs to more than one race category or a person for which no race information was provided.
Ethnicity is displayed as the following categories: Hispanic, Latino/a, or Spanish Origin; Not Hispanic, Latino/a, or Spanish Origin; or Unk (unknown). The category “Hispanic, Latino/a, or Spanish Origin” includes participants of Cuban, Mexican, Puerto Rican, South or Central American, or other Spanish culture or origin, regardless of race. The categories “Not Hispanic, Latino/a, or Spanish Origin or Unk” include participants who are not of Cuban, Mexican, Puerto Rican, South or Central American, or other Spanish culture or origin, or participants for which no ethnicity information was provided.
For the HTC Population Profile (PP) data, year of visit and year of birth are collected. Age is calculated by subtracting the year of birth from the year of visit. Although participants may be represented in multiple years of HTC PP, age in this chart represents each unique participant’s age at their most recent visit.
For the Registry data, date of visit and date of birth are collected. Age is calculated by subtracting the date of birth from the date of visit and dividing that number by 365; leap year (if applicable) was taken into account for the calculation. Although participants may be represented in multiple years of the Registry, age in this chart represents each unique participant’s age at the time of enrollment.
Participants (both HTC PP and Registry) were grouped into the following age categories: <2 years, 2–10 years, 11–19 years, 20–44 years, 45–64 years, and 65+ years.
Patient Geographic Information
The five-digit zip code of the participant’s primary residence at the time of enrollment is used to determine their state of residence.
Insurance is grouped into the following categories based on the primary health insurance held by the participant at the time of enrollment: Private, Public, and Other/Uninsured/Unknown. “Private” includes participants with any type of commercial insurance provided through private or public companies and paid for by employers or by individuals; this includes military health care. “Public” includes participants on Medicare, Medicaid, state-sponsored insurance plans, and Indian Health Services. “Other/Unknown/Uninsured” includes participants with insurance not otherwise described as public or private (e.g., single service plans paid for by the individual that provide coverage for only one type of service or treatment for a specific condition); this category also includes participants for whom it is not known whether they had any health insurance or the type of health insurance is unknown and participants without insurance plan coverage for medical costs.
Education is recorded as the highest level of formal education completed by the participant, and is grouped into the following categories: None or Pre-elementary, Primary/Secondary, High School or equivalent, Some Post-Secondary, 4-Year College Degree (Bachelors), Advanced Degree, and Other or Unknown. “None or Pre-elementary” includes participants with no formal education, including children too young to attend a formal education program and participants attending pre-kindergarten or kindergarten. “Primary/Secondary” includes participants who have completed grades 1–11. “High School or equivalent” includes participants who completed grade 12, the General Educational Development (GED) test, or equivalent. “Some Post-Secondary” includes participants who attended college but did not complete a degree, completed a 2-year degree program, or attended any post-high school or post-GED technical, vocational, or trade program. “4-Year College Degree (Bachelors)” includes participants who completed a 4-year program of study at a college or university. “Advanced Degree” includes participants who completed a program of study at a college or university beyond the Bachelors level, culminating in a Masters, Doctorate, or professional degree. “Other or Unknown” includes participants with any other level of formal education that does not fall into one of the above categories and those participants whose education level is not known.
Employment status is grouped into the following categories: Employed full-time, Employed part-time, Not employed – Child or Student, Not employed – Disabled, Not employed – Retired, Not employed – Other or Unk (unknown), and Employment status unknown. “Employed full-time” includes participants working 35 or more hours per week and “Employed part-time” includes participants who work less than 35 hours per week. “Not employed – Child or Student” includes participants not working at all because they are a child or are a college student; all participants younger than 18 years old were grouped into this category. “Not employed – Disabled” includes participants who do not work and are known to have qualified for disability income and are, therefore, prohibited from working by law. “Not employed – Other or Unk” includes participants who are homemakers and do not work outside of the home at all; participants who do not work and have no other listed reason for not working (this would include participants who are able to work but who have been laid off or cannot find work); participants who do not work at all and are of retirement age (usually >55 years old); and participants who do not work at all for a reason that is known but not otherwise listed. “Employment status unknown” includes participants whose employment status is unknown.
Primary diagnosis is grouped into the following categories: Hemophilia A, Hemophilia B, von Willebrand disease (VWD) type 1, VWD type 2, VWD type 3, VWD type other or unknown, Rare Factor Deficiencies, and Platelet Disorders and Other. “Hemophilia A” includes participants diagnosed with Factor VIII (FVIII) deficiency and “Hemophilia B” includes participants diagnosed with Factor IX (FIX) deficiency. “VWD type 1” includes participants diagnosed with VWD type 1 or type 1C; “VWD type 2” includes those diagnosed with VWD type 2A, type 2B, type 2M, type 2N or type 2, type unknown; “VWD type 3” includes participants diagnosed with VWD type 3. “VWD type other or unknown” includes participants categorized as VWD type other or VWD type unknown. The category “Rare Factor Deficiencies” includes participants with the following diagnoses: Factor I deficiency (including dysfibrinogenemia, hypofibrinogenemia, afibrinogenemia, and unspecified hereditary Factor 1 deficiency); Factor II deficiency; Factor V deficiency; combined Factors V & VIII deficiency; Factor VII deficiency, Factor X deficiency; Factor XI deficiency; Factor XIII deficiency; Alpha-2 antiplasmin deficiency; and PAI-1 deficiency. “Platelet Disorders and Other” includes the following diagnoses: gray platelet syndrome; Glanzmann thrombasthenia; Bernard-Soulier syndrome; Hermansky-Pudlak syndrome; inherited thrombocytopenia; platelet function disorder; platelet storage pool disease; platelet release defect or other hereditary functional platelet disorder; blood coagulation disorder with impaired clot retraction time; blood coagulation disorder with prolonged bleeding time; or blood coagulation disorder with prolonged coagulation time.
If the participant is a hemophilia carrier, the diagnosis is reported under FVIII or FIX deficiency according to the factor mutation carried. If the participant has more than one bleeding disorder, the primary bleeding diagnosis is assigned according to the following algorithms:
- Primary diagnosis for participants who have hemophilia plus another bleeding disorder is reported as hemophilia (FVIII or FIX as appropriate).
- Primary diagnosis for participants who have both FVIII and FIX deficiency is reported as whichever deficiency is more severe (has the lower baseline factor level); if equally severe, they are reported as FVIII.
- Primary diagnosis for participants who have VWD plus another bleeding disorder is reported as VWD (unless the other disorder is hemophilia; in which case, they are reported as hemophilia).
- Primary diagnosis for participants who have more than one other clotting factor deficiency is reported as whichever deficiency has the lowest baseline factor level.
- Primary diagnosis for participants who have a clotting factor deficiency and a platelet disorder or a connective tissue disorder is reported as the clotting factor disorder.
Severity of Hemophilia
Severity of hemophilia is grouped into the following categories: Severe, Moderate, Mild, and Unk (unknown)/Missing. For both the Registry and HTC PP, the baseline clotting factor activity result which best represents the participant’s inherent clotting factor activity is collected. “Severe” includes participants with less than 1% baseline clotting factor activity; “Moderate” includes participants with 1–5% baseline clotting factor activity; and “Mild” includes participants with greater than 5% baseline clotting factor activity. “Unk/Missing” includes participants whose severity is unknown or missing.
The severity of hemophilia chart is only displayed for participants with a primary diagnosis of hemophilia A or hemophilia B; however, the denominators for the percentages displayed in this chart are all participants, including those with non-hemophilia diagnoses. Thus, this chart presents the percentages of all persons in the Registry or HTC PP who have hemophilia A or B of a particular severity. A note in the chart description displays the number of participants with non-hemophilia diagnoses: “Number of people who are not affected by Hemophilia in Registry: X and HTCPP: Y.” To calculate the percentage of persons with hemophilia A or B with a particular severity, divide the number of persons with that severity (obtained from the mouse-over description or from the .csv file for Severity of Hemophilia) by the number of persons with hemophilia A or B (obtained from the Primary Diagnosis element).
History of hepatitis B virus (HBV), hepatitis C virus (HCV), and human immunodeficiency virus (HIV) infection is displayed for Registry participants ages 18 years and older as “Yes,” “No,” and “Unk (unknown)/Not applicable.” For HBV and HCV, “Yes” is recorded if the participant has been diagnosed with hepatitis B or C infection, respectively, regardless of whether the infection has since resolved or if the participant is currently HBV or HCV viral load negative or viral load positive. For HIV, “Yes” is recorded if the participant has been diagnosed with HIV infection or AIDS during their lifetime. “No” is recorded if the participant has been tested for the virus but has not been diagnosed with HBV infection (active or resolved), HCV infection (active or resolved), or HIV infection or AIDS. “Unk/Not applicable” is recorded if the participant is younger than 18 years old, if the results are unknown or indeterminate, or the participant has never been tested.
History of vaccination for hepatitis A and hepatitis B is displayed for Registry participants as “Yes,” “No,” and “Unknown.” For hepatitis A and hepatitis B, “Yes” is recorded if the participant’s medical record indicates a history of vaccination for hepatitis A or hepatitis B virus or if the participant (or the participant’s parent, in the case of minors) reports vaccination. “No” is recorded if the participant’s medical record indicates that the participant has not been vaccinated for hepatitis A or hepatitis B virus or if the record does not address hepatitis A or B vaccination and the participant/parent reports that they have not been vaccinated for this virus. “Unknown” is recorded if the participant’s record does not address hepatitis A or hepatitis B vaccine and the participant/parent does not know.
Weight status is grouped into the following categories: Obese, Overweight, Normal, Underweight, and NA (not applicable). Body mass index (BMI) was calculated for all of the Registry participants ages 2 years and older using the most recent weight and height reported at enrollment. A corresponding weight status was assigned based on established national guidelines, which are based on a person’s BMI for adults1 or age and BMI for children and teens.2 “NA” includes children younger than 2 years old.