The Right to Say “NO”

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Editor’s Note:

Regardless of the worthiness of any cause, you have a right say “No”.It’s very easy to say “No” when a stranger knocks on your door. Whatever they’re selling, we’re not buying.

Let’s face it — we’re all getting pretty darn good at saying “No”. In fact, for most of us, “No” has become a knee-jerk reaction to any solicitation. HOWEVER, it’s harder — and some might say it is impossible — to say “No” when the person knocking on that door is a granddaughter in a Girl Scout uniform selling Girl Scout cookies. Her advantage is called FAMILY.

It has fallen to me to make the case for you to donate $10, $25, $50 or $100 to support our PRP community which includes the following:

✽   PRP Facebook Support Group

✽  PRP Community on RareConnect

✽  PRP Survival Guide

✽  PRP Alliance, Inc.
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The opportunity to say “YES”

Has our PRP Community supported YOU or a loved one? Have you had a question answered? Have we been able to turn fear into hope?
If the answer is No”, then please OPT OUT of any future fundraising emails from the PRP Alliance. However, if your answer is “Yes”, then please consider a donation to the PRP Alliance.

How will we use the money you donate?

Our budget is admittedly austere. Patient organizations for psoriasis and eczema have million dollar budgets with paid staff. Imagine, with only $3,600 we can jumpstart a year of building awareness, supporting research and helping our worldwide PRP community meet the challenges of body, mind and spirit. Unfortunately, the financial demands we face are front-loaded during the first three months of 2018.
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✽  Annual dues ($250): National Organization of Rare Disorders (NORD) and the Coalition of Skin Diseases (CSD)

✽  Communication Technology ($1,340): Constant Contact (communication) and ReadyTalk (video conferencing)

✽  Advocacy Events ($2,010):  Annual meeting of the American Academy of Dermatology (February 16-19) in San Diego and Rare Disease Week on Capitol Hill (February 25-March 1) in Washington, DC .

A community of PRP patients and caregivers

Whether patient or caregiver, active or in remission, we are on the same journey. YOU have not been alone.
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We itch together
We shed together
We cry together
We listen to each other
We share. We care.
We learn from one another
We are family
Thus far, 47 fellow PRP patients and caregivers have donated $2,810 (78% of our goal). These donations range from $10 to $220 with an average of $60. Quite frankly, any amount you can donate will be very helpful in allowing us to achieve our goals.
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✽  If you need more information to either inspire or justify a donation CLICK HERE

 If you already know that you want to make a donation via PayPal CLICK HERE

✽  If you prefer sending a check, please make it payable to “PRP Alliance” and mail to: PRP Alliance, 1500 Commerce Drive, Plano, TX 75093-2640, USA. The PRP Alliance is a 501(c)(3) is a. tax-exempt patient organization. ID# 47-3868578

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THANKS to those who have already donated…

Jandina G (Portland, OR)
Richard L (Lansdale, PA)
Janice & Ernie F (Plover, WI)
Jessica H (Stone Mountain, GA)
Janet B (Forest Lake, MN)
Carel R (Surrey, British Columbia, Canada)
Cheri H (Omaha, NE)
Frank G (Fairview, NC)
Ronald O (Sharpsville, IN)
Derek S (Otterville, Ontario, Canada
John K (Weston, CT)
Mike W (Park City, UT)
Eve J (Byron Bay, NSW, Australia)
Leslie L (Colorado Springs, CO)
Vincent F (Cape Coral, FL)
Sylvia C (Wayne, NJ)
Susan O (Amesbury, MA)
Chelsea B (Othello, WA)
Helen M (Madison, WI)
Phil S (Chester, England, UK)
Anita R (Pearl, MS)
Corrine P (Toronto, Ontario, Canada)
Eric S (San Luis Obispo, CA)
Wayne M (Kyabram, Victoria, Australia)
Jerry R (Sausalito, CA)
David H (Scottsdale, Tasmania, Australia),
Suzanne M (LaPlata, MD)
Paula M (Los Angeles County, CA)
Debra W (Winston-Salem, NC)
Martin W (Wolverhampton, England, UK)
Vickie B (San Diego, CA)
Sandi H (Turtle Creek, PA)
Aziz T (Laurens, SC)
 Diane H (Minneapolis, MN)
Donna S (Beaumont Hills, NSW, Australia)
Diedre H (Houlma, LA)
Tierney R (Virginia Beach, VA)
Bill M (Plano, TX)
Tierney R (Virginia Beach, VA)
Elaine A (Reno, NV)
Sara K (Miami, FL)
Cay & Bill B (Berthoud, CO)
Darcy L (Colorado Springs, CO)
Cami S (San Jose, CA)
Sharlene C (Australia)
Linda L (Somerset, CA
Roberta K (Sicklerville, NJ)
Deborah W (Royal Palm Beach, FL
Marion M (Dublin, Ireland)
Jan and Malcolm T (Ringwood, NJ)
Joan P (Portland, OR)
Mark & Paul A (Boston, MA)
Joyce & Stephen T (location n/a)
Daina B (West Sacramento, CA)
Jeff S (Dayton, OH)

Remember: We’re family and we need you.

A Review on PRP

From the Editor — On January 4, 2018, a well-documented overview of pityriasis rubra pilaris written by Drs. Dingyuan Wang, Vanessa Cui-Lian Chong, Wei-Sheng Chong and Hazel H. Oon was published online by the American Journal of Dermatology. CAUTION: Section 7 on TREATMENT is worth a careful review by all PRP patients and caregivers.

This scientific article is not written for patients or non-medical caregivers. It should, however, be shared with dermatologists.Just ask: “Have you read the online Review on Pityriasis Rubra Pilaris in the American Journal of Dermatology?”

1   Introduction
2  Clinical Manifestations
3  Pathogenesis
4  Associated Conditions
5  Diagnosis
6  Differential Diagnosis
7  Treatment
8  Conclusion

A Review on PRP

PRP Community Resources

Message from the Editor

The onset of my version of PRP began in early August 2012 with the sudden appearance of a small, red spot on my right temple near my hairline. Within a week the “inconsequential” spot had grown in size — enough to prompt a call to my dermatologist.  By early September the side of my face, from my forehead to jaw, had been engulfed by red.

Initially misdiagnosed with seborrheic dermatitis, it took three months, the clinical observations of a more seasoned dermatologist, and a “supporting” biopsy before an “official”  diagnosis of PRP was rendered. My treatment plan was initiated in early December with slow-paced success. It took another seventeen months before I was med-free and symptom free.

Whether you are a PRP patient or caregiver, you are on a journey that few can understand. There is, however, a PRP community of fellow travelers who have faced the same challenges of body, mind and spirit that you face today. The journey from onset to remission should not be undertaken alone.

Bill McCue


Over the past five years there has been a dramatic expansion of resources available to support PRP patients and their caregivers. The PRP community must become more proactive in building awareness of these resources during 2018among two constituencies:

✽  PRP patients and caregivers

✽  Healthcare professionals responsible for the proper treatment of PRP

As a PRP patient or caregiver it is critical that you understand these resources and share there availability with your own dermatologist, general practitioner, podiatrist, opthamologist, ENT specialist, mental health specialist, etc.

1.  Genetic and Rare Diseases Inforation Center  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 including a recently updated overview of PRP. This is a “Must Read” for every PRP patient and caregiver.

❏  Read and share the GARD PRP Report

2.  National Organization of Rare Disorders     On September 20, 2017, NORD replaced the 1,500-word PRP Report (circa 2007) with a significantly more comprehensive, 5,000-word  2017 revision developed by the PRP Community. This overview is written in lay language and a “Must Read” for every PRP patient and caregiver.

❏  Read and share the NORD PRP Report 

3.  PRP Survival Guide     Since its launch in 2013, this ever-expanding repository of information now includes over 400 PRP-specific articles written in lay language on topics specifically related to the diagnosis and treatment of PRP as reported by PRP patients and their caregivers.

❏  Read “How to use the PRP Survival Guide”

4.  PRP Facebook Support Group     Founded in April 2013 by a PRP patient from British Columbia, Canada, membership in this Closed Group has grown steadily. Today over 1,170 PRP patients and caregivers ask questions, share our PRP-related experiences and provide insights only a fellow sufferer can offer. Our mantra: what works for one doesn’t work for all. We are in this together.

❏  Join the PRP Facebook Support Group

5.  PRP Community in Rare Connect     Established in April 2015 as an initiative of EURORDIS, the international equivalent of the National Organization of Rare Disorders. 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.

❏  Join the PRP Community on RareConnect

6.  PRP Alliance     From a domain name registered in February 2013, the PRP Alliance has evolved into a 501(c)(3), nonprofit, patient advocacy organization with a mission is to advocate 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.

❏  Learn more about the PRP Alliance

On November 1, 2017, as part of the annual observance of PRP Awareness Month, the PRP Alliance executed mailing to the dermatology departments of 88 teaching hospitals in the U.S. This was the first salvo in a year-long Rare Skin Disease Referral Initiative seeking to link PRP patients with the PRP resources listed above.

The Last Word

PRP patients and caregivers who join the PRP community discover that they are not alone. YOU are not alone. WE are on this journey together.

Bill McCue, Editor PRP Survival Guide 1500 Commerce Drive Plano, TX 75093-2640 Email: bill.mccue@prpSurvivalGuide.org Website: prpSurvivalGuide.org

 

2018 Path Forward

                 

Yale University Commits to PRP Patient Registry

From the Editor…

With the support of the National Institutes of Health and the Foundation for Ichthyosis & Related Skin Types (FIRST), Yale University School of Medicine is building the first Patient Registry for ichthyosis and related skin types. A patient registry is the cornerstone of research. Investigators researching PRP, for example, will have access to the patient data they need to experiment, discover, and achieve medical breakthroughs. Without patient registries, the process of scientific discovery in medical treatments can be slowed, halted, or even dismissed.

The PRP community has been given the opportunity to participate in the building of a PRP Patient Registry. The PRP Alliance supports this effort and encourages every PRP patient to participate.


Step One — Decide to Participate

Ted Zaki is the go-to person for the PRP Patient Registry. A senior medical student at the Yale School of Medicine, Ted  is currently working as a research fellow in the laboratory of Dr. Keith Choate, director of research of the Yale Medicine Department of Dermatology.   Besides maintaining the National Registry for Ichthyosis and Related Skin Types (including PRP) run by the Foundation for Ichthyosis and Related Skin Types (FIRST), Ted is interested in studying quality of life in patients with various skin conditions, particularly PRP.

All you need to know about Patient Registries is available on the FIRST website. Check out Frequently Asked Questions.

If you are interested in sharing your experience living with PRP, please email Ted addressing the questions below for enrollment in the National Registry for Ichthyosis and Related Skin Types. Remember, PRP is one of the “related skin types”. Once enrolled, you will be invited to participate in the the Department of Dermatology’s ongoing quality of life project.

Step Two — Answer Yale’s Five Questions

  How old were you when you first noticed PRP symptoms?
   Where on your body did the symptoms first appear?
   Does anyone else in your family have PRP?
   Have you ever had a biopsy done? If so, did it support the diagnosis of PRP? If you have access to the biopsy report, please send a copy.
   Have you ever had genetic testing done? If you have access to the report, please send a copy

Step Three — Send photos

Please send photos of your skin — whatever you are comfortable sharing. preferably of your PRP during a flare. Your photos will be reviewed by Dr. Choate and he will determine your eligibility for inclusion in the PRP Patient Registry. (See Sample)

Sample PRP Patient Registry Application

I wanted to make sure that Ted and Dr. Choate knew that I was a card-carrying PRP patient. I wanted to be included in the Yale University’s PRP Patient Registry.

✽  Name: William McCue
✽  DOB: 05/20/46
✽  Current age: 71
✽  Gender: Male
✽  PRP Onset Date: August 8, 2012
✽  PRP Onset Age: 66
✽  PRP Onset Symptom(s): dime-size red spot on right temple
✽  Differential diagnosis: seborrheic dermatitis
✽  PRP diagnosis: November 28, 2012 by Michael Golden, MD, Plano, TX
✽  Treating dermatologist: Arturo Dominguez, MD, UT Southwestern, Dallas, TX
✽  Initial Treatment Plan:

▸  Acitretin (25mg to 50mg)
 Triamcinolone (body)
▸  Desonide (face/ears)
 Clobetasol and urea lotion (palms and soles)
 Hydroxyzine (itch relief)

✽  Outcome: In remission as of April 4, 2014
✽  Biopsy history4 biopsies

▸  First three were inconclusive
▸  Fourth biopsy supported PRP

✽  Familial history: None
✽  Genetic testing:  Yes — TJU (no results to share) 

Now It’s Your Turn…

It’s easy to procrastinate, but why not answer the five questions and send the answers, together with your photos, to Yale now? Send what you have to Ted at theodore.zaki@yale.edu. If you have any questions, he is the go-to guy at Yale for the PRP Patient Registry. Call him at (203) 737-4675

1 PRP, 2 PRP, 3 PRP…

And as long as you are in the “sharing information” mode, please complete your PRP Worldwide Census form (if you haven’t already done so). Please go to: http://survey.constantcontact.com/…/a07eer5a6spj94cb3…/start

For those who need to know why the PRP Worldwide Census is so important to the PRP community, please go to http://prpsurvivalguide.org/…/prp-worldwide-census-datapoi…/

OTR26 — Yale University Commits to PRP Patient Registry

TJU Announces PRP Center of Excellence


From the Editor…

In an open letter to the PRP Community, Drs. Uitto, Keller and Ross announced the establishment of the nation’s first PRP Center of Excellence, located at Sidney Kimmel Medical College at Thomas Jefferson University Philadelphia, PA. What does this mean to the PRP community? We have asked questions based on the comments from the PRP community and will update accordingly.

“It is with great pleasure that we write to you to announce the first PRP Center of Excellence at the Department of Dermatology & Cutaneous Biology of Thomas Jefferson University in Philadelphia, PA. This Clinic seamlessly integrates laboratory and clinical sciences to improve patient care and research to advance our understanding of this devastating disease.”

 Will there be more that one PRP Center of Excellence at TJU?

 Is the Center of Excellence a clinic or more than a clinic?

 What is the difference between laboratory sciences and clinical sciences?

“At the PRP Center of Excellence, each patient will have a personalized consultation to review the prior diagnoses, biopsies and treatments with the world’s leading clinical dermatologists, dermatopathologists and basic science researchers in the field of dermatology. Your appointment will include whole body photography to document one’s progress during care, interdisciplinary review of diagnostic data (biopsy and other tests), and access to direct enrollment into clinical research.”

 How much time is allotted for the “personalized consultation”?

“Together with one of our clinical dermatologists, treatment decisions at the PRP Center of Excellence will be based on evidence-based algorithms that draw from research and expert opinions of a variety of medical disciplines to provide you with the greatest chance of successfully and safely treating your PRP.”

✽  How will the PRP Center of Excellence facilitate access to pharmaceuticals that have yet to be approved for PRP by the Federal Drug Administration?

In addition, at Thomas Jefferson University, patients have access to the largest network of tertiary referral center services in the region. This is particularly important because PRP is often accompanied by other diagnoses that can affect quality of life, one’s health and treatment decisions. It is this attention to the whole body that will allow you to heal. Specifically, patients can obtain appointments with therapists, psychologists and psychiatrists within the same building as the Dermatology Clinic to seek care for the depression and anxiety that can often accompany PRP. Patients will also be able to make appointments with Will’s Eye Hospital at Thomas Jefferson University for specialized ophthalmology care as the incidence of damaging conditions of the eye, such as ectropion (outward turning of the eyelid) and keratoco unctivitis (severely dry, pain lly irritated eyes), is increased in PRP. In addition, patients can schedule appointments with other nationally recognized fields such as Jefferson cardiology, endocrinology, gastroenterology, hematology, neurology, oncology, nutrition and integrative medicine to manage any accompanying health concerns.

✽  As a matter of official treatment protocol, will the PRP Center of Excellence refer PRP patients to the following PRP support resources:

▸  National Organization of Rare Disorders: PRP Report
▸  Genetic and Rare Diseases Information Center: PRP Report
▸  PRP Alliance, Inc.
 PRP Survival Guide
▸  PRP Facebook Support Group
▸  PRP Community on RareConnect

The PRP Center of Excellence marks another achievement of the PRP Community in advancing access, quality and value of care. We look forward to continuing to serve the Community and hope to see you soon! To schedule your consultation with Drs. Keller and Ross, please call (215) 955-9295.

✽  Is there an expectation that PRP patients beyond Southeaster Pennsylvania, e.g., New York, New Jersey, Maryland and Delaware will access the resources of the PRP Center of Excellence?

✽  Is there an opportunity for a Second Opinion regarding diagnosis and treatment?

✽  Will other healthcare professionals be able to “consult” with the PRP Center of Excellence?

Sincerely,

Jouni Uitto, MD, PhD, Professor and Chair
Matthew Keller, MD, Associate Professor
Nicholas Ross, MD, Resident-Physician

The announcement was made to the PRP Facebook Community on November 13 and generated an immediate response including 52 LIKES and 38 COMMENTS including the following (as of 11/14/5:17 PM CST):

Teri R — Ft. Wayne, IN
Awesome

Eric S — San Luis Obispo, CA
That is terrific news! Though I am in remission I am glad to know that there is a place specializing in this condition for future sufferers.

Marion M — Ireland
Wonderful news and it will help so many. Well done to all who made it happen.

Margarita B
…find a medicine that puts an end to the PRP!

Anne E — UK
Fantastic News!

Leslie L
So happy to hear this!!! It is wonderful news!! THANK YOU SO MUCH to all who made this possible!!!Abbie C — San Antonio, TXThis is great.Chris U — England, UK
Great news.

Phil P — England, UK
Congratulations to all from over the Pond. PRP on PRP

Alan C — England, UK
This has the potential to be an excellent resource! Well done to all involved in helping and supporting this development!

Cathy H — China Grove, NC
Wow this is wonderful….

Sandi H
This is great news. Thank you for sharing this!

Marianne B  — San Diego, CA
Please tell me how this is going to help anyone if they don’t live in or near Pennsylvania?

Linda B — England

Or even further afield!

Tierney R — Virginia Beach, VA
Travel. I had to drive 5 1/2 hours and spend the night. I raised money through GoFundMe.

Marianne B — San Diego, CA
I will have to drive two weeks and Linda B  will need a ship.

Burt U — Kenedy, Texas
Outstanding

Guy F — Scaggsville, MD
Excellent news! I was treated by Dr. Keller up until April 2017 with significant clearing and I am participating in TJU’s PRP study. I have nothing but good things to say about TJU Dermatology!

Norma M — Chicago, IL
Wonderful news! It’s a start. More centers of excellence will likely follow

Craig W — Charlotte, NC

Epic news!

Connie D — Maitland, FL
Yippee!!!!!!!

 

If you have any question regarding the PRP Center of Excellence at Thomas Jefferson University, please “Leave a Reply” below and we will find the answer for you.

 

OTR26 — TJU Announces PRP Center of Excellence

Thank you for subscribing…

It was Thursday, October 1, 2015 when I posted the 24th and final issue of On the Road… on the PRP Alliance website. After writing 598 pages of “PRP stuff” I was ready to shift my focus to the PRP Survival Guide. No more self-imposed, end-of-the-month deadlines for me. Besides, I was in remission.

It has been over two years and I am still in remission — but eager to publish a newsletter for the PRP community.

I urge every PRP patient and caregiver who subscribes to On the Road…  to take the time to read what we publish and share your personal insights. PRP patients and caregivers live with PRP day in and day out. You are the subject matter expert for your version of PRP. We need to tell all our stories.

Bill McCue, Editor

TJU & Yale Seek Research Partners

From the Editors: The PRP Community had long lamented the lack of PRP research until Thomas Jefferson University began genetic research in 2012. Three years later TJU initiated a clinical research ieffort that continues today. Recently Yale University has committed to genetic research, a PRP patient registry, and a Burden of Skin Disease study.

Why participate in PRP research at Yale and TJU?

Like many rare disease communities, the PRP community laments the snail’s pace at which PRP research progresses. What should we expect —  it took 61 years just to get the name right. But with your help, we can be part of research that goes beyond description and uncovers the genetic basis for PRP, creates effective treatments, and possibly discovers a cure. Be part of PRP history.

1828
James Shooter became the world’s first patient with what the medical community would eventually call pityriasis rubra pilaris.

1835
Claudius Tarral wrote about the case in “Traite theorique et pratique des maladies de la peau” (Treatise on Skin Diseases), considering it a variant of psoriasis.

1856
Marie Guillaume Alphonse Devergie published the most complete description of PRP. In fact, it was considered to be the “original description” of PRP

1877
Dr. Richaud was the first to recognize PRP as a distinct entity, in his publication “Etude sur le pityriasis pilaris.”

1889
Ernest Bernier presented nine cases in a 120-page article and forever fixed the name of the disease as pityriasis rubra pilaris.

It’s November 1, 2017 and in the 128 years since Mr. Shooter’s onset of pityriasis rubra pilaris. The PRP community has information researchers want.

✽  To learn more about PRP Research opportunities  CLICK HERE

 For a more detailed history PRP (1828-1889CLICK HERE

OTR025.5 TJU & Yale Seek Research Partners

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