PRP Parent Coalition

From the editor…

Every so often Christine Günther and I lament the fact that PRP parents have been unable to create a community within the PRP Facebook Community. That doesn’t stop us from raising the issue in hopes that a PRP Parent Coalition might become an outcome. So here we go again.

The PRP Juvenile Onset Community

There is a limit to what we know. about the PRP Juvenile Onset Community, a subset of the PRP Community Database. Over the past four years the PRP Community Database has been evolving. Data gathering began in 2013 with the methodical review of over 29,000 email messages shared by members of the PRP Support Group during the period November 1997 and April 2014. Core data was “harvested” and entered into a secure and confidential database. The data included:
 
✽  Name of patient or caregiver
✽  Email address
✽  Location
✽  Onset date
✽  Onset age
✽  Misdiagnoses
✽  Biopsy history
✽  Diagnosis date
✽  Name of the dermatologist diagnosing PRP
✽  Name of the dermatologist treating PRP
✽  Type of Onset: Adult versus Juvenile
✽  Current status (Remission Date if in remission)
PRP Parents and Kids
The consensus among dermatologists is that 45% of all PRP cases are juvenile onset. Let’s take a closer look.
 

Classical Juvenile Onset PRP  (Type 3)
✽   Usually occurs between 5 and 10 years old.
✽   This form accounts for about 10% of all cases of PRP or 80 “active” patients in the U.S.
✽   Remission can occur sooner than Classic Adult Onset, Type 1.
✽   The average Type III remission is one year.
✽   Odds: One in 4 million.

Circumscribed Juvenile Onset PRP (Type 4)
✽   Occurs in pre-pubertal children.
✽   Usually confined to palms, soles, knees and elbows.
✽   This form accounts for about 25% of all cases of PRP or 200 “active” in the U.S.
✽   It occurs in pre-pubertal children (age less than 14).
✽   Not a long-term affliction.
✽   Odds: One in 1.6 million.

Atypical Juvenile Onset PRP (Type 5) —
✽  Sometimes inherited; occurs at birth or early in childhood.
✽  This form accounts for about 5% of all cases of PRP of 40 “active” in U.S.
✽  Most cases of familial PRP belong to this group.
✽  It has an early onset and runs a chronic course.
✽  Odds: One in 8 million.

What do we actually know?
The PRP Community Database has identified 1,520 PRP patients that are “trackable”. By “trackable” we mean that they fall into one or more of the following categories:
 
(1)  Member of the PRP Facebook Support Group
(2)  Member of the PRP Community on RareConnect
(3)  No affiliation but have a valid email address on file.
 
When it comes to PRP patients who have been diagnosed with Juvenile Onset PRP, the number drops dramatically to 149. However, there is a glimmer of hope in the fact that 822 PRP patients in the PRP Community Database have yet to indicate whether they are Adult Onset or Juvenile Onset. There may be more “wee ones” already in the database. and more to be found. Here is some of what we know about PRP Parents and Kids:
 
✽  BY AFFILIATION: 87 are PRP Facebookers, 36 are PRP RareConnecters, 62 have no affiliation (but do have a valid email address)
 
✽  BY AGE — While 46 of the “kids” are 15 or older, 102 are 14 years old or less.
 
✽  BY STATUS — 64 are active, 29 are in remission, 29 n/a
 
✽  BY LOCATION: 81 live in the USA, 49 are international
The Data Gathers Dust
The data in the PRP Community Database is gathering dust. In all probability, dermatologists may not even know the PRP Community Database even exists. Even more disconcerting is that if they knew, they might not care. It is the wrong kind of data — not scientific enough for their palette. It is not a bona fide patient registry.
Pityriasis rubra pilaris is an über-rare skin disease that does not represent a significant market or patient population. There are only an estimated 816 PRP patients in the U.S.
What is the Path Forward for the PRP Juvenile Onset Community?
 
Your guess is as good as mine. A survey? A GoToMeeting Conference? Formation of a PRP Parent Coalition? Reach out to the dermatologists treating PRP kids and share information. The ball is back in your court…parents.
 
Please use “Leave a Reply” to make suggestions.
PRP Parent Coalition

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