PRP Alliance to Update 2013 Biopsy Poll

From the Editors: During the summer of 2013, a total of 487 PRP patients were polled by email and given an opportunity to participate in a first-of-its-kind PRP Biopsy Poll. Remarkably, within a 3-week period, a total of 256 invitees (52.6%) shared their biopsy experiences and/or recollections.The primary objective of the PRP Biopsy Poll was to better understand the role played by biopsies in the diagnosis of PRP from the perspective of a PRP patient

2013 PRP Biopsy Poll

It was a simple poll with only two questions.

(1)  How many biopsies have you had that supported a PRP diagnosis?
(2)  How many biopsies have you had that failed to confirm PRP?

Based on the results, however, a benchmark for a future biopsy-related survey was established.

✽    Diagnosis with no biopsy ordered: 7.8%
✽    Diagnosis with no contradictory biopsy: 45.3%
✽    Biopsy supported diagnosis: 23.4%
✽    Diagnosis with no confirming biopsy: 23.4%

2018 PRP Biopsy Survey

Since participation in the 2018 PRP Biopsy Survey will require a completed PRP Census form, we will have more dots to connect.

✽    Elapsed time from onset to diagnosis
   Misdiagnoses that preceded the PRP diagnosis
   Adult onset versus juvenile onset (based on onset age)
   Duration based on onset date and current status

Questions to ask dermatopathologists

While we are surveying PRP patients and caregivers, we should recruit an advisory committee of dermatopathologists. The following questions might be a good place to start.

✽    How should a biopsy be performed to increase the likelihood of a timely PRP diagnosis? Is there a right way? Wrong way? Better way?
✽    What specific signs or indicators should a dermatopathologist be looking for as they review the biopsy slides?
✽   Are the characteristics of PRP really so elusive that they cannot be easily seen?
✽   How important is it for the dermatologist who performs the biopsy to specifically instruct the dermatopathologist to “consider” or “look for” PRP.

Path Forward

The ongoing status of the 2018 PRP Biopsy Survey  will be update in future issues of the PRP community newsletter —  On the Road….

For additional information about diagnosing PRP, go to Chapter 2 of the PRP Survival Guide.

PRPA Plans Biopsy & Efficacy Surveys: OTR025.4 

PRP Outreach to US Teaching Hospitals

 

From the Editors: Most PRP patients and caregivers are given very little guidance after their PRP diagnosis is rendered. Referrals to Dr. Google and Dr. Yahoo are quite standard, Balderdash! The PRP Alliance has self-financed an outreach effort to 87 of the dermatology departments of our nation’s teaching hospitals advocating a standard referral to GARD and NORD.

What’s standard treatment protocol?

When a dermatologist diagnoses a rare skin disease, the patient is prescribed an appropriate therapy. Predictably, the initial treatment plan will include systemic and topical therapies as appropriate. However, there’s something that most, if not all patients are missing: a referral to the Genetic and Rare Diseases Information Center (GARD).  

Without a rare skin disease referral to GARD…

A newly diagnosed patient leaves the dermatology clinic with a treatment plan, prescriptions to fill at the local pharmacy, the required paperwork and an appointment to return at a later date. For patient and caregivers, their journey has begun with little more than the name of the rare skin disease and a tacit referral to Dr. Google and Dr. Yahoo. As questions arise in the days and weeks that follow, the information unearthed via search engines is too often redundant, insufficient, outdated, and typically written for an audience of healthcare professionals.

What happens with a referral to GARD?

GARD, a program of the National Center for Advancing Translational Sciences, is funded by the National Institutes of Health. The office supports a variety of programs to serve the needs of patients who have any one of approximately 7,000 rare diseases known today. Their rare disease database includes information on each of 597 rare skin diseases.

Who can GARD help?

✽  Your patients diagnosed with a rare skin diseases
✽  The people who care for your patients: parents, family members, and friends
✽  Doctors, nurses, and other healthcare providers
✽  Scientists who are studying rare skin diseases and need information for their research

How will PRP patients and caregivers will find what they seek?

Reliable information about rare or genetic diseases is often hard to find. Your patients will have a roadmap to the resources they need from this dependable source.

GARD website
PRP patients and caregivers have online access to reliable information about their newly diagnosed rare disease to include symptoms, cause, diagnosis, treatment, research, daily life and support organizations.

GARD “Learn More” Opportunities
Links to resources where patients can find more information, e.g. the National Organization of Rare Disorders.

GARD Information Specialists
Whether by phone, fax or email, the public has access to current, reliable, and easy-to-understand information in English or Spanish.

✽  Toll-free Telephone: 888 205-2311
✽  Email:  GARDinfo@NIH.gov/

Scope

The target audience for the Rare Disease Awareness Initiative includes “leaders” at teaching hospitals in the U.S. with a dermatology department, e.g., Department Chair. We will also reach out to leadership in the following:

✽  American Academy of Dermatology
✽  Society of Dermatology Physician Assistants
✽  Dermatology Nurses’ Association

Methodology

The Rare Disease Awareness Initiative is organized around a “Referral Packet” containing the following:

✽  Cover letter
✽  PRP Referral Protocol
✽  GARD brochure
✽  NORD PRP Report (16 pages)

Who to thank

✽  The Genetic and Rare Diseases Information Center for the services they routinely provide as well as brochures for the 100 “Referral Packets” 
✽  The National Organization for Rare Disorders for allowing the PRP Community to take a leadership role in rewriting the NORD PRP Report 
✽  A dozen members of PRP Facebook Support Group who donated $1,100 to make the Rare Disease Awareness Initiative a reality, e.g., printing, postage, etc.

 

PRP Outreach to US Teaching Hospitals: OTR025.3 

PRP Awareness Month Begins Today


November 1, 2017
Every patient organization needs an Awareness Day they can call their own. We celebrate Rare Disease Day on the last day of February and share the spotlight with 7,000 other rare diseases. However, PRP Awareness Month gives us 30 days to crow like a banty rooster, shake our tail feathers and get more than our fair share of attention. — Bill McCue

My first PRP Awareness Day was November 28, 2012 and quite “unofficial” by any standards. It began with a phone call from my dermatologist’s office. I was told that the red spot on my forehead on August 8 — nearly four months prior — had been the onset of pityriasis rubra pilaris. Like most PRP “newbies”, I spent the next few days searching the internet and learning what little there was to learn about PRP. What I found was redundant, insufficient, outdated, and typically written for an audience of healthcare professionals.

On November 1, 2013, I celebrated PRP Awareness Day once again. This time I sent out over 1,500 emails to PRP patients and caregivers and called the effort the PRP Worldwide Census. It had taken me several months during the previous summer to review over 29,000 emails in the PRP Support Group Archive going back to November, 1997.  As I  collected email addresses I also harvested “core” information about the onset, diagnosis, and treatment of PRP. It took six months to solicit 500 responses. That was the genesis of the PRP Community Database.

November 6, 2014 was the first “official” observance of PRP Awareness Day. The date was selected to commemorate the signing of the Rare Diseases Act of 2002 that set the threshold for a rare disease. A rare disease or disorder is defined in the U.S. as one affecting fewer than 200,000 Americans. Using the European standard, however, the U.S. threshold would be only 160,000. Today there are more than 7,000 known rare diseases, and it is estimated that about 30 million Americans are affected by them.

November 6-8, 2015 was the first “official” observance of PRP Awareness Weekend since the 6th fell on a Friday. The “Big Event” would be a global  Meet & Greet. Whether face to face or using technology (FaceTime, Skype and ooVoo) we pitched the idea to the PRP Facebook Support Group.  Other than a few face-to-face Meet & Greets and a spike in Facetime encounters, the effort missed it’s mark. Lesson learned.

November 1-30, 2016 was the first “official” observance of PRP Awareness Month.  Perhaps all we needed was 30 days rather than a weekend or a day. Sunday, November 6th was selected to host a 24-hour GoToMeeting gathering. Only the predictable and dependable cadre of seasoned PRP travelers stopped by to chat.


An important lesson has been learned over the past five years. If you run the same flag up the flagpole, the same people are going to salute. We’re going to try something different.

  ✔︎  Revive the PRP Community newsletter
  ✔︎  Launch the PRP Worldwide Census
  ✔︎  Execute the Rare Skin Disease Referral Initiative
  ✔︎  Initiate PRP Surveys
  ✔︎  Recruit participants for PRP research
  ✔︎  Promote legislative advocacy
  ✔︎  Support the PRP Alliance.

 

 

PRP Awareness Month Begins Today: OTR025.1

1,621 Invited to Share PRP “Core” Data

From the Editors: The PRP Community Database currently tracks 1,622 PRP patients who are now members of the PRP Facebook and/or RareConnect Communities, or are unaligned but have a valid email address. The 2017 PRP Worldwide Census begins today with a simple goal: confirm the data we have and add data we are missing.

 

If you are a PRP patient or caregiver, you have something we need.

 We can’t force you to give it to us.
 We don’t have the funds to buy it from you.
 And we would never try to steal it from you.

All we can do is ask you to share and hope you say “Yes”.

Sharing data: Are you a skeptic?

Most PRP patients/caregivers appreciate the importance of sharing data to help PRP researchers better understand pityriasis rubra pilaris. Because your information is personal and private, it is important that we safeguard any data you share. We hope that our data collection methodology encourages your enthusiastic participation in the upcoming PRP Worldwide Census.

Confidential Data — While the PRP Alliance is not required to comply with federal regulations regarding the security of protected health information (HIPAA Security Rule), the PRP Community Database is in full compliance. The data we collect about your PRP journey and the data for other PRP patients (active and in remission) is both encrypted and password protected. Moreover, the data cannot be accessed via the Internet.

De-identified Data — When shared with PRP researchers or published on the PRP Survival Guide website, your data is de-identified. Simply stated, the PRP Community Database reports numbers, not names. When your PRP-related data is published or shared, that data is disassociated with your name. There is complete and total anonymity.

The Value of Data in the Aggregate — Our PRP-related data has the greatest value when combined with the data of others.  It is the aggregation of data that will ultimately enlighten PRP researchers.

Sharing of Data — The PRP community will be notified when information contained in the PRP Community Database is shared with PRP researchers, e.g., Thomas Jefferson University, Yale University School of Medicine, etc. Such notification will include the reason why the de-identified data is being shared.

Still skeptical?


What is “Core” data?

The following datapoints represents core data. With this information we can build a profile of the worldwide PRP community.

 Elapsed time between onset and diagnosis
 Elapsed time between onset and remission
 Onset age by decade of life
 Misdiagnoses
 Biopsies as a diagnostic tool
 Survey PRP patients in remission
 Survey PRP children based n onset age

Name: Bill McCue
Even though we ask for the FIRST NAME and LAST NAME, we NEVER publish NAMES. WE ONLY report NUMBERS, e.g., How many “this” and how many “that”.

Location: Plano, TX
We are NOT looking for street addresses or ZIP Codes/Postal Codes)

Email: papa.bill@mac.com
The email address is the only way we can verify that the information we receive is from a valid source. We will send a confirming email to the email address of the person completing the PRP Census. If the email confirmation is undeliverable, the data will not be added to the database.

Onset Age: 66 years, 2 months
At what age did your PRP symptoms first appear?

Onset Date: August 8, 2012
What was the approximate date when your PRP symptoms first appeared? Because few people know the exact date Onset Date, only month and year is required, Do the best you can, e.g.,  August 8, 2012; August 2012, or even the “Summer of 2012”.

Misdiagnosis: seborrheic dermatitis
What misdiagnoses preceded your “official” PRP diagnosis? We are only concerned with the misdiagnoses of your dermatologist, not your GP. Options include psoriasis, eczema, seborrheic dermatitis, allergic reaction and other (specify)

Biopsy History: 3 biopsies inconclusive. 4th biopsy supported PRP
What role did biopsies play in your diagnosis of PRP?

Diagnosis Date: November 28, 2012
The Diagnosis Date should be as accurate as possible, e.g., November 28, 2012, November, 2012; end of November, 2012. Do the best you can.

Current Status: In remission since April 4, 2012 
Don’t worry about definitions. Given the choice between “Active” and “In Remission”, what is your current status? If “in remission”, what is your date of remission

Treatment Options (oral/injection): acitretin
All we want to know are the PRP-related drugs you were prescribed from the list retinoids, immunosuppressants and biologicals. Whether or not the drugs were successful will be the subject of a future survey. We are NOT collecting data on itch and pain relief, topical ointments, creams and lotions, sleep aids, etc. 

We are a global community. Each country has their own way of protecting a patient’s medical information. For purposes of the PRP Census, we comply with U.S. HIPAA regulations.  Even with assurances of confidentiality, data collection has never been an easy task.

Confidential Data – The data we collect about your unique PRP journey – and the data for all PRP patients (active and in remission) – is encrypted, password-protected, and not connected to the Internet.

De-identified Data – When shared with PRP researchers like those at Thomas Jefferson University and Yale University School of Medicine or published in the PRP Survival Guide, your data is de-identified. Simply stated, the PRP Community Database reports numbers, not names. Because your data is disassociated with your name, you have complete and total anonymity.
 

 

If you have any questions, Leave a Reply below. If not, your PRP Worldwide Census awaits.

 

1,621 Invited to Share PRP "Core" Data: OTR025.2

Thank You For Being Counted

 

I have been collecting PRP-related information from PRP patients and their caregivers since November, 2013. In the beginning it could take six requests before I received a completed PRP census form.

On the 1st and 15th of ever month the NUMBERS will be posted here.  The list will show the November 1, 2017 numbers and the most current numbers.

The numbers below are based on the “core data” shared by PRP patients and caregivers worldwide. The PRP Community Database is the most complete representation of our patient population. Thank you for sharing your information.

Bill McCue, Founder/President
PRP Alliance, Inc.

 


Recap as of November 1, 2017

Numbers in parentheses ( ) indicate “not providing data”

✽  Total number of PRP patients reporting:   1,629

✽  Reporting email addresses:   1,033 (596)

✽  Reporting location:   1,140 (489)

✽  Reporting onset date:  715 (914)

✽  Reporting onset age:  651 (978)

✽  Reporting diagnosis date: 694 (935)

✽  Reporting current status: 656 (973)

✽  Active:  394

✽  Remission: 262

✽  Remission date: 64 (198)

✽  Reporting standard drug therapies: 67 (1,562)

PRP Dermatology Referral Initiative

Objective
To conduct an outreach effort to dermatologists, dermatology physician assistants, and dermatology nurses.j

Problem to be addressed
When a patient is diagnosed with PRP, the dermatologist — with rare exceptions — does not refer the patient to the PRP Alliance, PRP Facebook Support Group, the PRP Community on RareConnect or to the PRP Survival Guide. The PRP patient is typically referred to Dr. Google, Dr. Yahoo or Dr. Bing.

Scope
The target audience for the Dermatology Awareness Initiative includes “leaders” at teaching hospitals in the U.S. with a dermatology department, e.g., Department Chair. We will also reach out to leadership in the following:

 American Academy of Dermatology
 Society of Dermatology Physician Assistants
 Dermatology Nurses’ Association

Message
The PRP Dermatology Awareness Initiative will advocate that patients diagnosed with pityriasis rubra pilaris (IDC-10 L44.0) be referral to the PRP-specific information to be found at:

 Genetic and Rare Diseases Information Center (GARD)
 National Organization of Rare Disorders (NORD)

Methodology
The Dermatology Awareness Initiative is organized around the First Class mailing of a “PRP Packet” containing the following:

✽  Cover letter
 PRP Referral Protocol
✽  GARD brochure
✽  NORD PRP Report (16 pages)
✽  PRP Resources Card

Based on available resources, recipients of the mailing will be contacted a second time after the conclusion of PRP Awareness Month.

Measuring Success
The execution of the first mailing is the first measurement of success. This has never been attempted by the PRP community. The fact that it happens will be a reason to celebrate. We will continue the advocacy of a “PRP Referral Protocol” during 2018 and measure success a second time on November 1, 2018.

PRP Dermatology Referral Initiative

Resources Supporting PRP Patients & Caregivers

PRP Alliance.Inc.

The PRP (Pityriasis Rubra Pilaris) Alliance is a 501(c)(3), nonprofit, patient advocacy organization.Our mission is to advocate for the timely and accurate diagnosis of pityriasis rubra pilaris (PRP), the implementation of more effective and accessible treatment options, and an increase in PRP-specific research. Advocacy contacts:

Bill McCue, Founder/President
Plano, TX
Telephone: (214) 205-0574
Email: bill.mccue@prpAlliance.org

Ginny Maxwell, Director, Patient Advocacy
Lexington, SC
Telephone: 803.640.5769
Email: ginny.maxwell@prpAlliance.org

PRP Survival Guide

The PRP Survival Guide is an online repository of patient-reported experiences as reported by the PRP community. It  is offered as an alternative to unstructured and random searches by newly diagnosed PRP patients using Dr. Google, Dr. Yahoo and Dr. Bing. These efforts will almost always lead to frustration and frequently to misinformation.

If we do are job properly, you will either (1) find the answers you seek or (2) send the PRP Survival Guide editor out in search of answers to questions we haven’t asked. Eight chapters provide an organizational framework for PRP patients and caregivers:

✽  Basics
✽  Diagnosing PRP
✽  Treating PRP
✽  Daily Life
✽  PRP Parents and Children
✽  PRP & Remission
✽  PRP Research
 PRP Advocacy

PRP Facebook Support Group

Founded in April 2013, the membership in this Closed Group has grown steadily to nearly 1,100 with representation on every continent. Truely the “Land of Chat”, post a question and comments flood in. Need a hug, the huggers respond. Need a friend … there’s a community of fellow travelers ready to lend an ear or a shoulder. Need information … there are nearly 1,100 members who can share what works and what doesn’t for their unique version of PRP. We learn together. We are in this together.

PRP Community on RareConnect

The PRP Alliance supports RareConnect, an initiative of EURORDIS, the international equivalent of the National Organization of Rare Disorders. Established in April 2015, the PRP Community on RareConnect is a 200+ member, multi-language, non-Facebook option serving adults with PRP and the parents of children diagnosed with juvenile onset.

RareConnect provides a “safe, easy to use platform where rare disease patients, families and patient organizations can develop online communities and conversations across continents and languages. RareConnect partners with the world’s leading rare disease patient groups to offer global online communities allowing people to connect around issues which affect them while living with a rare disease.”

Genetic and Rare Diseases Information Center

The Genetic and Rare Diseases (GARD) Information Center is a program of the National Center for Advancing Translational Sciences (NCATS) and funded by two parts of the National Institutes of Health (NIH): NCATS and the National Human Genome Research Institute (NHGRI). GARD provides the public with access to current, reliable, and easy to understand information about rare or genetic diseases in English or Spanish.

The PRP Community advocated that all patients diagnosed with PRP should be routinly referred to GARD’s PRP Repost rather than Dr. Google, Dr. Yahoo and Dr. Bing.

National Organization of Rare Disorders

The National Organization of Rare Disorders maintains a Rare Disease Database to provide brief introductions to more than 1,200 rare diseases — including pityriasis rubra pilaris. These rare disease reports are primarily geared toward patients and families. There is also, however, an important secondary audience, e.g.,  physicians, researchers, nurses, students, journalists and others who might request and benefit from such rare disease information.

The PRP Report (circa 2017) has recently replaced the 2007 version.