PRP Parents Respond

From the editor…

41 years ago my daughter was born in a hospital near Laguna Beach, overlooking the Pacific Ocean. It seemed a fitting place for her to start a new life. She came home a few days later where family and friends assembled to celebrate her birth. Then the phone rang. The voice simply stated that we needed to bring our newborn to the Orange County Medical Center … immediately.

We arrived at the emergency room within the hour and were greeted by a TEAM of healthcare professionals. A high-ranking doctor, a geneticist, psychologist, social worker and a registered nurse introduced themselves. Over the next few hours we learned that Marisa had tested positive for phenylketonuria. They called it an “inborn error”. Initially is seemed more like a “inborn terror”.

PKU is a rare metabolic disorder affecting 1 in 50,000. With an estimated 6,400 PRP patients in the U.S. it easily qualifies as a rare disease… like PRP. Over the next dozen years I became an expert on PKU. I joined a PKU support group and attended conferences.  A good thing I did since my son Matt — now 34 — was born with PKU too.

I don’t have a child with PRP, but I understand the mental terror of a journey into the unknown — a journey that can overwhelm a parent at the end of a day when sleep seems impossible and hope remote. That is why the PRP Survival Guide will soon have a chapter devoted to PRP Parents & Kids.

Our goal is to provide a resource for the NEXT PRP parent who comes knocking on our gates.  We need to be ready. It will take a  month or two to get it right.

The following EDITED conversation was initiated on June 11, 2017 by Jane G, a mother with a recently diagnosed child. The exchange with other PRP parents illustrates the important role PRP parents play within the PRP Facebook Support Group. As we say: Those who care — share, so that others may learn.

PRP parents are generally not healthcare professionals. However, they willingly share what has worked for their and what has failed.

Source: https://www.facebook.com/groups/15865278115/permalink/10155309037303116/

Feel free to add your comments using “Leave a Reply” at the end of this webpage.

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Jane G — Vancouver, British Columbia, Canada
Hello everyone! I’m brand new to this group. Dermatologists suspect that my two-year-old son has juvenile onset PRP, but they don’t know for sure. They say that the only way to find out for sure is to wait to see how he responds to the steroid creams. Is that true?  Has anyone else’s kids been diagnosed this young?

What a scary week for us. Not knowing what’s happening to our baby — and the doctors not knowing either. We were brought to isolation because they didn’t know if it was infectious or contagious, and then still not have a diagnosis.

Poor thing soooo itchy, and his hands and feet are so cracked they are bleeding in the creases. The skin around his eyes, in his ears, nose and mouth, and bum are shedding as well. As well as random red bumps all over his legs and back.

I guess I’m just looking for a little assurance that he’s going to be OK. How long does it last? Will it come back? What causes it?

We live in Vancouver, British Columbia. All the doctors we’ve seen were on an emergency basis . Just a few days ago so I don’t know much. I still don’t know for sure that PRP is what he has, but from my research online, and based on the dermatologist’s suspicion, it’s sounding more like the correct diagnosis.

Michelle K — Roseburg, OR
My son was recently diagnosed with PRP by biopsy. He just turned 3 May 1st…. he has had skin issues since he was 6 weeks old and only gotten worse despite all of the steroid creams and misdiagnosis of eczema. The new dermatologist we took him to put him on a strict regimine. We are using Amlactin lotion 3 times a day then at night time we do a water bath 6 days a week with no soap. 1 day a week with soap. Followed by triamcinolone cream 1% on all of his spots then lather him in Vaseline head to toe before bed. It’s been 3 weeks and he has no spots left…… I started to notice a difference the 1st week. I can’t wait for our 1 month follow up with the dermatologist next week…. hang in there momma!!!! Be persistant!!

Jane G
So interesting, my son has had weird skin rashes since he was a baby too and was just told eczema.. And everytime he has a cold his skin rashes worse, gets bumps and hives.

Michelle K
I urge you to keep on the doctors.  Pityriasis rubra pilaris is a very rare skin disease.. A lot of them have no idea how to treat it. Do as much research as you can. I would ask for a biopsy, if I were you, At least with a biopsy you can start getting somewhere.

For the longest time I thought my son’s skin rashes were food related.. My son has a horrible leaky gut as well. I take him to a naturopathic doctor as well. It is interesting to get both points of view. We are going to be starting a yeast treatment for his gut health in a couple months if he is still itching without the rashes…. so far he is…

Jane G
Oh wow. My son has had GI issues since he was in infant. He’s actually scheduled to have a colonoscopy because of how much blood is in his poop.

Michelle K
Dang…. our sons sound very similar. I have had my son on a strict diet since he started eating food that is all organic. No processed foods and only water to drink. He also takes a probiotic, fish oil, vitamine D drops, and a multivitamin daily.  It has helped with that aspect. Does your son have food allergies? My son is allergic to all sorts of stuff, e.g., gluten, wheat, oats, barley, rye, dairy, eggs, garlic, cashews. He has environmental allergies as well including dust mites, grasses, and pollen, etc. I am supposed to vacuum sweep, mop, dust daily and then shampoo and wash all linens and carpet weekly for to his dust mites allergy.

Michele W — Subiaco, Western Australia, Australia

Image may contain: one or more people and closeup My son had that as a toddle. He is now 20 years old. Its on and off . He seems to be outgrowing it. We did not do drugs or even steroids — only because I did not know what it was all those years back. We were told it was an allergy or psoriasis. In the end we just used emollients. He still does. He had so many different diagnoses that I thought well, let’s wait until he’s older. I guess I was scared in those days it  would affect his growth – as it did mine due to cortisone for atopic dermatitis (eczema) as a child.

He is healthy and tall. He still gets flaking but less so. Maybe worse in Winter. It was a lot worse as a toddler. He would get deep painful fissures in his feet. Last year had different diagnosis one said PRP one said erythrodermic progressiva variabilisis and then the latest theory is Afrikaner skin disease…Oudtshoorn skin. Could be as we come from Cape Town and his father is Afrikaans. At end day though these are just labels. Because he had some kids tease him at school l suggested he say its eczema and this demystified it. Many had this also.
Plenty emollients help a lot. As l said he now very fit healthy and in a good space. I suggest also that you start when he is young to eat healthy and build good gut health, to exercise and use emollients. Now that my son older we may look into other options like biologics. Simple things help: oatmeal tied in stocking as a sponge in bath or coconut paste in bath or apple cider vinegar well diluted as a hair rinse. HoBoy, l could go on!
Christine G — Zürich, Switzerland

Hi, I know the look of this kind of red feet, hands and eyelashes VERY well.
We have an 8-year-old daughter who has PRP since she is four.

Susanne A — Melbourne, Victoria, Australia
My daughter was diagnosed when she was two years old. It’s an especially tough time because it looks like it is only the beginning! My daughter is now 10 and we succumbed to medication at the age of 8 and we haven’t looked back. Good luck stay calm be strong and allow yours self to cry. Be kind to yourself. Your son will be fine. It’s his parents who struggle

Janine R — Medinah, IL
Jane, your son looks almost spot on to how mine did at the beginning. He was diagnosed in January of this year at 3.5 years. He was started on topicals, but quickly got put on isotretinoin, which helped him clear remarkably fast. We’ve learned that GI bugs are his trigger. Every time he gets a stomach bug (which happens a decent amount due to daycare), his hands, feet, elbows and knees turn red.

His itchiness has definitely subsided. He refuses any antihistamines, so I would use cool compresses to try to calm it down. Night time was the worst.

Today he has some mild discoloration on his hands, feet, and knees. But his skin is otherwise clear and smooth.

Reach out anytime. It’s so overwhelming in the beginning, so don’t be afraid to ask anything to this group. PRP soon becomes part of your routine and it does get better.

Image may contain: one or more people and closeupGinny M — Lexington, SC
This is my 4-year-old daughter’s hand. I have PRP and so do her 12-year-old twin brothers.

We had gentic testing done by Thomas Jefferson University and learned that we didn’t have a CARD14 gene mutation. CARD14 it is believed to play a role in certain skin conditions. TJU also determined that we did NOT wehave the familial form of juvenile onset PRP. As much as I respect Thomas Jefferson University, my three children and I can’t possible have sporadic PRP. I am the first in my family to have PRP.

A bit of advice this disorder is extremely unique. What works for my one son doesn’t work as well for my other son. You have you help your son find what works best for him and your family. My one son and I flared from head to toe after antibiotics. My other son and daughter have not had full body involvement. We don’t get the flu vaccine and currently have delayed a set of the live vaccines for me daughter — mainly, because of the Stelara injection. We are not suppose to live with a person who has had a live vaccine.

Eliane S — Zug, Switzerland
We also know juvenile onset PRP. Our son is now nine years old and has had PRP for six years. We also know the red hands and feet.
Kristen T
My son is 3-1/2 years old.  was diagnosed in April, 2017 with PRP. Can you get an appointment with a pediatric dermatologist or go to a dermatologist at a teaching hospital? Can you call around to the different locations and find a doctor that does know what PRP is?The steroid creams have only been effective in quickly healing up the fissures. We have moved on to topical retinoid cream. I am interested in looking into what Michelle K is doing. My son is not in very much pain, but he gets more tired and more quickly. This happens when your body is going through something like this. I know this is terribly stressful. One day at a time.
Jane G
We were able to see a pediatric dermatologist immediately when we were in the emergency room at the children’s hospital. She’s the one who told me she suspected PRP. We have a follow up appointment in a week where I’m assuming we will do more testing.

Ginny M
We also do light therapy during the winter months and natural sun during the summer months.
Also if his feet crack a lot, we apply Vaseline, then a sandwich bag. They should fit him at age 2. We have moved up to gallon size bags! We then put the sock on over the bags. My boys pretended we were getting ready to blast off into space. Make a game out of the care and it will go easier.
We have a Facebook page where we share our collective journey:  https://www.facebook.com/joeynathanlauren/
Many will disagree but at the point I see no reason to put him through a biopsy. It will not change the course of treatment. In the states it is extremely hard to get the 3rd step drugs for PRP. Our doctor agrees not to biopsy the kids so she can label them as having psoriasis or arthritis for insurance purposes.
Michele W
Totally agree my son had 2 painful biopsies both inconclusive. Only the islands of sparing on his body some clue.
Ginny M
I have had nine biopsies over the years. I can recall very clearly. My punch biopsies went better than my shave biopsies done at the Mayo Clinic. They became infected and I had to get painful injections to reduce the scaring. The scars were very painful. I think I had a total of 11 but since my mom’s passing I have lost alot of my medical records.

My dermatologist is just a 2nd year doctor and she has done more for me than 40 years of my mom touring the states with me in hopes of finding a cure. Now we have the internet, the PRP Survival Guide and the PRP Facebook Support Group. My kiddos and I don’t have to waste countless hours on doctor visits.
Janine R
I totally agree about the biopsy. It’s painful and unnecessary, especially for young children who can’t consent on their own. I think the best thing you can do is get on the waiting list for a pediatric dermatologist at an academic center.

Allie W — Cleveland, OH
Looks just like my son’s PRP diagnosed age 3. He is 9 years old now. After many things we have been off meds for a year and he is holding his own. Not worse and  some improvement.

Adita F — Arnhem, Netherlands
My son was showing his first rash around 6 months. He was misdiagnosed as eczema, psoriasis and icthyosis for couple of months.

He was diagnosed as PRP from biopsy result that was confirmed by CARD14 mutation from genetic analysis. He is now 3 year and using acitretin in low dose.

I would suggest to ask for biopsy before going further with treatments, because personally many outside factors can also worsening the symptoms and clouding the therapy effect. Biopsy is a more objective tool for diagnosis because all those diagnosis (eczema, ichthyosis, psoriasis) look different under microscope. While macroscopically/from outside visually, all these diagnoses look the same as redness and scaling, especially if it is already widespread.

i wish i had done it earlier, since my son could not tell if the treatment was painful for him (he has other developmental condition). I wish you good luck and you can always private message me anytime.

Christine G —
Our daughter had a biopsy when she was four. She had a Narcosis with nitrous oxide. I could talk to her the whole time and she was relaxed and pain free.

No fears, no panic and no pain – went fine for her. Of course mommy was far away from relaxed!
Michelle K
Christine G. Jacob did very well with the biopsy also. It is just a matter of personal preference. I was tired of doctors jerking us around when I knew there was more to it than eczema and he was suffering on a daily basis. The five-minute procedure and an answer was well worth it to our family. Since the “official” PRP diagnosis he is doing so much better with appropriate treatment. Good luck to you! I’ll be praying for all of your family’s and the doctors helping you along your journey. It’s not an easy one. Just hope you keep your head up and stay strong in getting the answers that satisfy your son’s needs… blessings.
Christine G
Jane G — in case you want to read Molly’s story follow this link
https://www.rareconnect.org/…/molly-s-prp-journey-part…
Melissa C — United Kingdom
Hi! My little one was diagnosed in February at the age of 15 months. Though it has improved massively, its still very much there. She’s very good at letting us cream her and is now beginning to understand when we tell her to stop itching. We don’t use steroids mainly because we are still waiting to see the pediatric dermatologist (been 20 weeks since referral, heart breaking).
At first we assumed diet. Cut all milks, gluten, anything processed. Got allergy tested, only milk seemed a problem. Got retested four weeks ago and her reaction to milk is now hardly there but her reaction to dog is very high – we do actually have a dog but he’s meant to be a hypoallergenic breed (pure fluke!).The worst part is clothing – what do you all dress your children in during summer whens its super hot? I’m struggling as she itches too much for any part of her to be showing! Which brings me to the question – is sun good or bad for PRP skin? And baths, how many helps… daily/weekly? You can literally ask constant questions and still be non the wiser! Good luck with your journey.
 
PRP Parents Respond

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