Questionnaire Report Lori Nusbaum Associate Director CJD - - PowerPoint PPT Presentation

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Questionnaire Report Lori Nusbaum Associate Director CJD - - PowerPoint PPT Presentation

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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

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SLIDE 1

Questionnaire Report

Lori Nusbaum Associate Director CJD Foundation New York, NY

341 W. 38th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org  www.cjdfoundation.org

2015 CJD Foundation Family Conference

Washington, DC July 10th-12th, 2015

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SLIDE 2

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. 38th 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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SLIDE 3

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. 38th 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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SLIDE 4

Demographics

341 W. 38th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org  www.cjdfoundation.org

53% 47%

Gender (1226 patients)

Male Female

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SLIDE 5

Age At Death

  • Youngest person, age 16; oldest, age 92
  • 65 is the average age at death

341 W. 38th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org  www.cjdfoundation.org

0% 5% 10% 15% 20% 25% 30% 35% 40% 45% 16-29 30-40 41-51 52-62 63-73 74-84 > 85

Patient Age

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SLIDE 6

Ethnic Origin

  • Of the 1226 patients
  • *108 (9%) patient questionnaires with missing data.

341 W. 38th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org  www.cjdfoundation.org

95% 3% 1% 0% 1%

Caucasian (White/Non- Hispanic) Hispanic/Latino African American Native American Asian

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SLIDE 7

Duration of Illness

  • Of the 1226 patients

*98 (8%) patient questionnaires with missing data.

341 W. 38th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org  www.cjdfoundation.org

0% 5% 10% 15% 20% 25% 30% 35% 40% < 3 months 3 - 6 months 7 - 12 months 13 - 18 months 19 - 24 months > 24 months

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SLIDE 8

Unusual Stress Prior to the Onset of Symptoms

  • Of the 1226 patients
  • *292 (24%) patient questionnaires with missing

data.

341 W. 38th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org  www.cjdfoundation.org

56% 44% Yes No

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SLIDE 9

Did Patient Develop Dry Cough

  • Of the 1226 patients
  • *360 (29%) patient questionnaires with missing data.

341 W. 38th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org  www.cjdfoundation.org

28% 72% Yes No

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SLIDE 10
  • 748* (61%) patient questionnaires with missing

data.

Diagnosing Physician

341 W. 38th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org  www.cjdfoundation.org

84% 7% 1% 8% Neurologist General Practitioner Psychiatrist Ophthalmologist Other

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SLIDE 11

Autopsy Information

  • Was an Autopsy performed?
  • 28* (2%) patient questionnaires with missing

data.

341 W. 38th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org  www.cjdfoundation.org

64% 36% Yes No

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SLIDE 12

CJD/Prion Disease Confirmed

  • From Autopsy (764 Patients), was CJD or Other Prion

Disease Confirmed?

  • 34* (4%) patient questionnaires with missing data

341 W. 38th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org  www.cjdfoundation.org

93% 7% Yes No Results Pending

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SLIDE 13

Type of CJD/Prion disease confirmed (Out of 676)

341 W. 38th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org  www.cjdfoundation.org

82% 10% 2% 1% 1% 4%

Type of CJD/Prion disease confirmed

Sporadic Familial (hereditary) Iatrogenic - Acquired Variant Gerstmann-Straussler-Scheinker Fatal Familial Insomnia Sporadic Fatal Insomnia PSPr (New Disease) Final Report Pending

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SLIDE 14
  • Of the 764 patients
  • 233* (30%) patient questionnaires with missing data.

341 W. 38th 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

50% 2% 35% 1% 11% 1% Who referred autopsy to NPDPSC

CJDF Hospice Physician Health Department Other Funeral Home

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SLIDE 15

Physician Information

Informed about CJD 643 (52%) Informed about CJDF 262 (21%) Informed about NPDPSC 290 (24%) Compassionate 692 (56%) Recommended 840 (69%)

341 W. 38th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org  www.cjdfoundation.org

  • Was the Physician ….
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SLIDE 16

CJD Listed On The Death Certificate

  • Of 1226 patients
  • *485 (40%) patient questionnaires with missing data.

341 W. 38th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org  www.cjdfoundation.org

81% 19% Yes No

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SLIDE 17

Food Consumption and Animal Exposure (1226)

  • Patients who consumed beef

* 119 (10%) patient questionnaires with missing data

  • Patients who consumed deer/elk

* 193 (16%) patient questionnaires with missing data

Yes 1094 (99%) No 13 (1%)

341 W. 38th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org  www.cjdfoundation.org

Yes 574 (56%) No 459 (44%)

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SLIDE 18

Travel Outside the US

Yes 670 (62%) No 414 (38%)

341 W. 38th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org  www.cjdfoundation.org

Travel to:

  • No. (%)

United Kingdom 226 (18%) Japan 46 (4%) Eastern Europe 104 (8%) France 131 (11%) Canada 204 (17%)

* 142 (12%) patient questionnaires with missing data

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SLIDE 19

Surgery and Tissue/Organ Transplant History

No. Liver 1 Corneal 1

341 W. 38th Street, Suite 501, New York, NY 10018  212.719.5900  HelpLine 1.800.659.1991 help@cjdfoundation.org  www.cjdfoundation.org

  • Type of Organ Transplant
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SLIDE 20
  • Medical Procedures of Interest

Medical procedures

Gastric (Upper GI) 42 (3%) Endoscopy 147 (12%) Colonoscopy 384 (31%) Other 65 (5%)

341 W. 38th 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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SLIDE 21

Other Medical Procedures

Spinal tap (other than for CJD diagnosis) 131 (11%) Conditions associated with the thyroid 157 (13%) Blood donor 357 (29%) Blood recipient 101 (8%) Transfusion of albumin or Immunoglobulin 22 (2%)

341 W. 38th 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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SLIDE 22

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. 38th 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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SLIDE 23

Of Possible Interest

Hormone replacement therapy Females (out of 569 ) 111 (20%) Unknown 57 (10%) Blank/No 401 (70%) Homeopathic/herbal therapy 63 (5%) IV drug use 33 (3%) Acupuncture 63 (5%)

341 W. 38th 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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SLIDE 24

Of Possible Interest

Vaccinations/ Injections 381 (31%) Tattoos 64 (5%) Piercings 340 (28%)

341 W. 38th 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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SLIDE 25

Pin Map

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SLIDE 26

The CJD Foundation Gratefully Acknowledges

  • Families
  • Centers for Disease Control and Prevention
  • The National Prion Disease Pathology

Surveillance Center

  • Paul Pearsall