The power of technology

I am lucky to have the job I have. I am a training consultant for an organisation that is focused on technology. First if all I am lucky because my boss and the organisation have been very understanding regarding time off, hospital visits, etc. I am supposed to work from the office 3 days a week. The office is 150 miles away in Newcastle so that involves staying over in a hotel, which can be difficult. I am trying to maintain this pattern as much as possible but there have been weeks where I've not made it. 

Being part of a technology focused organisation has really helped. I can attend meetings remotely, daily office banter can take place via Skype and I can deliver training online. Thanks to technology I delivered a couple of training sessions on Friday where I retained my air of authority and respect while wearing bandages for socks (covering the cream on the soles of my feet) and an old track suit while being completely covered in 50/50 paraffin emollient (goo).  

Changes

I saw the dermatologist yesterday and he decided to change my treatment:
No more light treatment
Neotigason Capsules (acitretin) reducced from 50 to 20 Mg a day
175 mg of neoral a day

A bad end to the good holiday

After the great holiday things started to go wrong on the flight back when I was shedding quite a bit and my face was flakey especially around the eyes. I was feeling dehydrated and it was a while before I could get any water on the plane. I couldn't get at the little bit of cetreban I had left which was stored in my bag. Also my legs which had started swelling a few days befor were now really uncomfortable. 

When we landed I added some Emoliant as soon as I could. The drive back from the airport was difficult as I was now really tired, my legs were really sore and my night vision was a bit blurry. I got home and got straight to bed where I shuddered uncontrollably for some time. I can't explain what causes the shuddering. I think it's related to the tiredness. I did get to sleep but woke up at 4am scratching. After trying to control it and get back to sleep I gave up and got out of bed at 7. There was lots of skin tone n the bed and a lot more when I rubbed down in the bathroom. 

A great weekend holiday

I was dreading this weekend as I was going on a weekend away with friends to Lisbon. It was booked before this skin thing started and I did consider cancelling, several times, including the day before we flew when I had a very itchy & flakey night. 

I had a great time.  So glad I went.  

Some things I was worried about:

 Sun. It was sunny all the time with temp around 30 degrees. I was very careful and kept covered as well as applying skin sensitive factor 50. Sun cream for sensitive skin stinks of vomit.  I didn't burn or blister. 

Heat sensitivity. I wasn't painfully hot in the day or shivering cold at night. 

Anyway here's a general update 

• Skin shedding.  Some relatively good days and a couple of days where I have been crispy/ crunchy/ flakey. But ive definitely not shed as much as I usually do at home. The bed sheets haven't been as full of flakes as usual. The flight back however was bad. Flakes on a plane 
• Scalp.  A few unpleasant flaky days. 
• Palms of hands. Within 24 hrs of being on holiday the palms cleared to be really good, although by the 3rd day they were fairly peel-y again. 
• feet. Had swollen and then calmed down before the holiday. By the 3rd day of the holiday they had swollen again making walking along the uneven cobbled streets quite uncomfortable. Soles of feet were peeling quite a bit one night. 

• Eyes. My eyes were really irritating all weekend. Often drying out and full of goo. Also lots of flakey skin around the eyes and tight skin below the eyes. Glasses & sun glasses which seemed constantly covered in cream and/or flakes really didn't help. 
• Itchiness. Minimal itching since being on holiday 
• Tiredness. I didn't have as much energy as my friends & I had a couple of early nights but I wasn't too bad. 

Gross

Contents of my bed this morning+ a mornings shake down = 15 grams of dead skin

Weekly update

• Skin shedding there have been some good half days with minimal shedding and my skin looked good (no wrinkles).  Lots of bad days with heavy shedding all over. Falling down my face, and off arms.
• Scalp.  Last few days scalp has become full of flakes
• Palms of hands constantly peeling, quite sore
• feet Ankles and feet swollen, top of feet peeling and red. Soles of feet peeling but not sore
• Eyes lots of flakey ness around eyes.  Skin tight below eyes, pulling lower eyelids down, eyes dry because of this.  Bits of goo footing around the eyes. 
• Itchiness constant but not unbareable 

Scabby head

Recently my scalp has become very flakey so the nurses at the hospital have massaged in some greasey ointment which needs to stay in for a few days. Both the flakes and the ointment are revolting 

Swollen feet and ankles

What are the odds?

Here are some statistics about PRP.  

The chance of getting PRP is about 1 in 400,000 (Clinical Dermatology, By Richard Weller, John A. A. Hunter, John Savin, Mark Dahl).  That is about one person in a population the size of Malta.

(All other stats taken from DermNet NZ)

There are different types of PRP;

•       About 50% of those with PRP have Classical adult onset
•       About 5% of those with PRP have Atypical adult onset
•       About 10% of those with PRP have Classical juvenile onset
•       About 25% of those with PRP have Circumscribed juvenile
•       About 5% of those with PRP have Atypical juvenile onset
•       And there is also a HIV-associated variant

This table represents this split, with each square representing 5%:

The only types I could have are Classical adult onset or Atypical adult onset, so I think my chances of having Classical adult onset are 90.91% (10 out of 11) while my chances of having Atypical adult onset are about 9.09% (1 out of 11)

For those with Classical adult onset, 80% will have spontaneous remission within 3 years

For those with Atypical adult onset, the condition may persist for 20 years or more.

Shedding

After a 3 hr drive where I could see lots of skin fall off my face and arms, I undressed in the bathroom, rubbed my arms and legs and swept up about 9grams of skin 

Wet tshirt and the contents of my bed

No real change since last weeks update. I do have a sore ear, I went to the walk in centre who looked in it and couldn't see anything wrong. 

Still no biopsy results.  

I returned to work today, working from home, next week I plan to be in the office Tuesday to Thursday which will mean 4 nights away from home.  This will be a challenge as I will have to bring everything from bedsheets, to medication & emollients to my own DustBuster.  We shall see how it goes. 

This is my wet t-shirt look after being covered in the paraffin emollient 

This is what my bedsheets look like in a morning 

The Cruelest Things

Here are some of the cruelest things about this disease, from my point of view;

When I was 19 my mother died of leukaemia, and ever since I have worn my mothers wedding ring on a necklace.  When I have been feeling down, for whatever reason, just holding the wedding ring has helped me.  Unfortunately my skin is now so sensitive I can't wear it any more.  Just when I need it most.

I have had to move into the spare bedroom as my shedding and itching means there is no way me and my other half can share a bed at the moment.

I have 2 holidays lined up this year, one is an annual long weekend away with a group of friends, and the other is a once in a lifetime fortnight in Thailand which my other have has been planning for over a year for my 40th birthday next month.  I haven't cancelled either holiday yet but the is a BIG question mark over both.

G-string or sock?

At the moment I am going into a local hospital everyday (except Sunday's and bank hols) to be covered in 50/50 paraffin emollient.  The first few days it was tops off, covered, top back on, then everything off below the waist with both hands self consciously covering my bits.

After a few days I relaxed and it seemed more practical to whip everything off with one hand covering what needed to be covered. Then after a week or so of this one of the nurses passed me an odd wrapped package which turned out to be disposable pants. Was I supposed to be wearing these all the time?  No one told me.  Oh well, disposable pants from now on.

I have also started light therapy.  Standing in an upright tanning booth every other day.  The only bits to be covered are my ....bits.  The nurse said I could get a g-string but just tucking everything into a dark sock should be okay.  So first time the sock method worked fine,  the second time just as the lights went on the sock fell off.  STOP!!! I shouted as I didn't know whether to replace the sock, cover myself with a hand, or just get a tan on the little fella.  Thankfully they stopped while I the sock was returned to its rightful place.  But now going forward I have to decide.  Do I find a sock with a tighter grip? Or do I go g-string shopping?

This time it's personal

I stared with a timeline of symptoms which I stored on my iPad ( using the Evernote app if your interested).  The point was to show the Drs and dermatologists, and to keep track myself.  

Then I transferred this all to this blog, with backdated posts.  I did this because PRP is a rare disease and there's not much out there.  

But there are other things I want to say about how I'm feeling/dealing with PRP, and I might start posting these as well.  I feel I need to do this for me.  

So you have been warned

In the meantime here is a get well soon drawing from my nephew. Love how he spells Kev with a C

Current symptoms update

Handheld Vac which had been emptied less than 24 hours ago

• Skin shedding allot particularly, upper body.  The full size Dyson and the handheld vacuum cleaner are both required to be emptied out every 2 or 3 days
• Palms of hands were leathery now starting to peal again, and can be quite sensitive to sharp edges.
• Soles of feet are shedding a bit but not sore
• Face is very flaky, lots of hanging skin around the eyes
• Itchiness is still there and very severe allot of the time, but i no longer feel it is completely taking me over.
• Sleep is generally allot better than it was.  Most nights I am getting enough sleep. I am no longer apprehensive about going to bed.
• Nails are fine.
• Temperature control; difficult but not unbearable.  I seem to feel cold when others say it is warm.
• Shivering, for a few weeks I was shivering uncontrollably for an hour or 2 every evening.  I wasn't sure whether this was due to lack of sleep or feeling the cold.  I have not been shivering since Sunday.