Questionnaire Report Lori Nusbaum Associate Director CJD - PowerPoint PPT Presentation

2015 CJD Foundation Family Conference Washington, DC July 10th-12th, 2015 Questionnaire Report Lori Nusbaum Associate Director CJD Foundation New York, NY 341 W. 38 th Street, Suite 501, New York, NY 10018  212.719.5900 HelpLine 1.800.659.1991  help@cjdfoundation.org www.cjdfoundation.org 

Summary • We have 3211 patients in our database • Data have been collected since 2000 • The questionnaire underwent revisions in 2004, 2008 and 2011 341 W. 38 th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org www.cjdfoundation.org 

Summary (cont.) • Before 2004, questions were in narrative form • Narratives are difficult to process for statistical analysis • 1226 patient questionnaires in total have been returned 341 W. 38 th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org www.cjdfoundation.org 

Demographics Gender (1226 patients) 47% Male 53% Female 341 W. 38 th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org www.cjdfoundation.org 

Age At Death • Youngest person, age 16; oldest, age 92 • 65 is the average age at death Patient Age 45% 40% 35% 30% 25% 20% 15% 10% 5% 0% 16-29 30-40 41-51 52-62 63-73 74-84 > 85 341 W. 38 th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org www.cjdfoundation.org 

Ethnic Origin • Of the 1226 patients 3% 1% 0% 1% Caucasian (White/Non- Hispanic) Hispanic/Latino African American 95% Native American Asian • *108 (9%) patient questionnaires with missing data. 341 W. 38 th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org www.cjdfoundation.org 

Duration of Illness • Of the 1226 patients 40% 35% 30% 25% 20% 15% 10% 5% 0% < 3 months 3 - 6 months 7 - 12 13 - 18 19 - 24 > 24 months months months months *98 (8%) patient questionnaires with missing data. 341 W. 38 th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org www.cjdfoundation.org 

Unusual Stress Prior to the Onset of Symptoms • Of the 1226 patients Yes 44% 56% No • *292 (24%) patient questionnaires with missing data. 341 W. 38 th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org www.cjdfoundation.org 

Did Patient Develop Dry Cough • Of the 1226 patients 28% Yes No 72% • *360 (29%) patient questionnaires with missing data. 341 W. 38 th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org www.cjdfoundation.org 

Diagnosing Physician 1% 8% 7% Neurologist General Practitioner Psychiatrist 84% Ophthalmologist Other • 748* (61%) patient questionnaires with missing data . 341 W. 38 th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org www.cjdfoundation.org 

Autopsy Information • Was an Autopsy performed? 36% Yes 64% No • 28* (2%) patient questionnaires with missing data. 341 W. 38 th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org www.cjdfoundation.org 

CJD/Prion Disease Confirmed • From Autopsy (764 Patients), was CJD or Other Prion Disease Confirmed? 7% Yes No Results 93% Pending • 34* (4%) patient questionnaires with missing data 341 W. 38 th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org www.cjdfoundation.org 

Type of CJD/Prion disease confirmed (Out of 676) Type of CJD/Prion disease confirmed 1% 1% Sporadic 2% 4% Familial (hereditary) Iatrogenic - Acquired 10% Variant Gerstmann-Straussler-Scheinker Fatal Familial Insomnia Sporadic Fatal Insomnia 82% PSPr (New Disease) Final Report Pending 341 W. 38 th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org www.cjdfoundation.org 

Autopsy Referral to NPDPSC • Of the 764 patients Who referred autopsy to NPDPSC 1% CJDF 1% 11% Hospice Physician 50% Health Department 35% Other Funeral Home 2% • 233* (30%) patient questionnaires with missing data. 341 W. 38 th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org www.cjdfoundation.org 

Physician Information • Was the Physician …. Informed about CJD 643 (52%) Informed about CJDF 262 (21%) Informed about NPDPSC 290 (24%) Compassionate 692 (56%) Recommended 840 (69%) 341 W. 38 th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org www.cjdfoundation.org 

CJD Listed On The Death Certificate • Of 1226 patients 19% Yes No 81% • *485 (40%) patient questionnaires with missing data. 341 W. 38 th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org www.cjdfoundation.org 

Food Consumption and Animal Exposure (1226) • Patients who consumed beef Yes 1094 (99%) No 13 (1%) * 119 (10%) patient questionnaires with missing data • Patients who consumed deer/elk Yes 574 (56%) No 459 (44%) * 193 (16%) patient questionnaires with missing data 341 W. 38 th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org www.cjdfoundation.org 

Travel Outside the US Yes 670 (62%) No 414 (38%) * 142 (12%) patient questionnaires with missing data Travel to: No. (%) United Kingdom 226 (18%) Japan 46 (4%) Eastern Europe 104 (8%) France 131 (11%) Canada 204 (17%) 341 W. 38 th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org www.cjdfoundation.org 

Surgery and Tissue/Organ Transplant History • Type of Organ Transplant No. Liver 1 Corneal 1 341 W. 38 th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org www.cjdfoundation.org 

Medical procedures • Medical Procedures of Interest Gastric (Upper GI) 42 (3%) Endoscopy 147 (12%) Colonoscopy 384 (31%) Other 65 (5%) 341 W. 38 th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org www.cjdfoundation.org 

Other Medical Procedures Spinal tap (other than for CJD 131 (11%) diagnosis) Conditions associated with the 157 (13%) thyroid Blood donor 357 (29%) Blood recipient 101 (8%) Transfusion of albumin or 22 (2%) Immunoglobulin 341 W. 38 th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org www.cjdfoundation.org 

Of Possible Interest Dental surgery 730 (60%) Eye surgery 247 (20%) Contact lenses 176 (14%) Eye drops 386 (31%) HGH treatment 13 (1%) 341 W. 38 th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org www.cjdfoundation.org 

Of Possible Interest Hormone replacement therapy 111 (20%) Females (out of 569 ) Unknown 57 (10%) Blank/No 401 (70%) Homeopathic/herbal therapy 63 (5%) IV drug use 33 (3%) Acupuncture 63 (5%) 341 W. 38 th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org www.cjdfoundation.org 

Of Possible Interest Vaccinations/ Injections 381 (31%) Tattoos 64 (5%) Piercings 340 (28%) 341 W. 38 th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org www.cjdfoundation.org 

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The CJD Foundation Gratefully Acknowledges • Families • Centers for Disease Control and Prevention • The National Prion Disease Pathology Surveillance Center • Paul Pearsall