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A list of my holiday musts, and links to funny holiday vids...

First though, at last here is the link to the free short story I just posted on my website. You are the first to see it!

go here to read 'the Jolly Lodger' - based on a shaggy dog story. le...

oh and here's a heads up for Kipling fabs - the Early Bird Order Link - here's the link to Saturday's TSV bag - the Florizel medium bag from Kipling, so you can get in early! 

Fabulous holiday! You know how when you think about going away there's always that moment where you think 'shall we just go back to the same place again?' Well I'm quite adventurous in some ways, but tend to play it safe and revisit destinations sometimes. Do you? So it was nice to go to the Sunrise Beach hotel to see what the family have been raving about for years! Here's the link to last week's QVC blog with some pics and info, and stand by for the next QVC one which will appear on Friday afternoon, with tons more pics and links to videos.

One pic tho, is of me completely covered up in kaftan, with an alternative use for a cold beer! I don't drink beer, but the cold glass was really handy - literally - for putting my hot hands on the icy glass.

My skin condition had just begun to pain me a little, and I knew I'd used up all my sun minutes and had to stay out of it. It's affected me all my life, and means I can GO in, but not STAY in the sun. 

And here's the link to the Back to You blog about EPP I've just done, with the video explaining exactly what it is. And just what life has been like to not be able to be like other people all my life. In some ways it's helped me become who I am - weirdly. Just click on the link and watch the video to hear my story. Only 250 people in the country have it - told you I'm a weirdo! lol.

 

Holiday Essentials 

So here are my 'musts' for a fab hol - some of which are based on my own skin's needs, but would help others who are sensitive in the sunshine. Others I just cannot do without!

Avon Skin so Soft - anti mossy oil spray - can't believe how effective this is at deterring mossy bites. The one morning I came down without it I got bitten at breakfast. It isn't greasy and spreads amazingly well with no foul odour like deet. Do try it. more here.

Liz Earle Tonic Spray - OMG so good for cooling you down! Take little one away on plane too. more here.

ABC tea tree blemish stick - a little first aid box in a stick - especially good for stopping infection of scratched mossy bites, itching etc. I get mine from Amazon if QVC are out of stock.

 And of course, Ultrasun, loads of spfs on QVC. Plus Gatineau tan accelerator - one of the best - sinks straight in and moisturises too. It's fab for skins that have issues tanning - use each night for two weeks prior to going away. More on my QVC blog tomorrow afternoon (Friday 23rd - go here when it's up.)

The other thing I find invaluable is L-Tyrosine tablets, from eg Holland and Barrett. L-Tyrosine is the natural amino acid in our bodies that helps us make melanin. I use all year round really. 

Plus of course, long white sleeves, a long kaftan, long white skirt, spf gloves and a hat! Musts for my suitcase whenever I face a sun holiday...

Anyway the wedding was lovely, a good time was had by all, and it's back to business as usual! I'm looking forward to my few days off next week tho, as there's no one down at the guest house in Devon. It'll be great to relax - yeh, right! Hopefully I'll get lots of writing done of my own tho. email us This email address is being protected from spambots. You need JavaScript enabled to view it. if you'd like to find out more about our lovely retreat in the heart of North Devon.

Book of the Week  a lovely dual romance set in the horsey world, a touch of Jilly Cooper with a hint of Dick Francis I'm told - by fellow Choc Lit author Lisa Hill.  Go here to read more.

have a lovely week! 

best wishes

debs

x

ps the tickets are released for the September 16th concert in Torrington, Devon - go here to book yours. Come for the weekend if you can, as on the Sunday 17th we'll be opening the doors to Retreats for You for another vintage pop up tea rooms afternoon too. do come! more here.

Also - ps Fancy a Stay in Devon? Would you like to come down? I’ve got a new date for QVC viewers,  if you are one, and fancy a fab full board stay – you can inspect the new kitchen and decide if Daisy and Gracie have indeed got trimmer! More info here, or just email me This email address is being protected from spambots. You need JavaScript enabled to view it.">This email address is being protected from spambots. You need JavaScript enabled to view it.  . It’s 21st August and can include yoga/walking and wholesome food – and wine! £90 pppn if you book soon – do come?

ps That blog on emotional bullying  is worth reading – click here – it’s now had over 6100 views and am very glad it’s helping so many people when they find out they’re not alone. Do have a read and share it.

pps  check out all my paperbacks together! Very proud. More about how to get your copy is here.

 

Ps my new Till the Fat Lady Slims book is now out on Amazon here, and there’s such an amazing support system on Facebook on theTTFLS group here - do come join us!

for info about my paperbacks and ebooks for sale now, go here 

for all my social media and to keep in touch go here

Published in Debbie's News

so here's a video explaining it better than text can ever say...

click here to watch the video on youtube about what life has been like as a result of EPP

created during my holiday in Cyprus, Summer 2017

Plus go here for the list of things I find invaluable on my holidays - from tanning problems to mossies etc.

And the pic above is of me finding another use for a cold beer - I don't drink beer, but this ice cold glass really made my burning hands feel better!

luv

debs

x

 

Published in Back to You

NEWS UPDATE ON EPP (16th Nov)

Another update for you - I was sent the results of my blood tests, and it turns out that there's this measurement of how much 'protoporphyrins' are in my blood, compared to the norm. Well most people will have less than 0.2 (of this specific protoporphyrin measuremnt) - well my measurement is 14.7. That's about 73 times the norm. ~So yes, I definitely have EPP. (see below if you haven't yet read about my rare genetic condition.)

Dr Robert Sarkhany also told me that because of the odds involved and the rarity of what I've got, whilst both my kids don't have it, thank goodness, their own children only stand a one in 80 chance of getting it.

Plus mine is only mild, again thank goodness. He told me that there is a drug that's now been licensed but it costs a fortune, an implant under the skin, and by February NICE will have given their verdict on whether the 250 or so sufferers in the UK would be able to be given it by their doctor. Interesting. And he gave me a 'prescription' to go to St Thomas's hospital dispensary to ask for some thick gunky sun cream, that doesn't block 'UVA UVB' which most do - it blocks VIOLET light... Will keep you informed how I get on!

d

x

 

 

EPP update

This week I went to see the expert in EPP, my skin condition, which I first told you about a couple of weeks ago – for the full info about my first post see below left. If you don't know me well and haven’t heard, I just discovered recently about the condition that’s plagued me since I was two - where my skin gets burning hot if I stay too long in the sun.

So the first dermatologist sent me along to see Dr Robert Sarkaney, the country’s foremost expert in this rare genetic condition called EPP – erythro-poietic Protoporphyria – and the apt was this week. He is based in a wonderful relaxed calm setting in Lister Hospital in Chelsea, near our old QVC building! (Well the place it used to be, it's flats now) He then, for the first time in my life, explained to me how EPP works

 

.

I have to have inherited a gene from both my parents, that's why it is so rare, only 250 people in the country have it, he thinks, and one of them is me. Always knew I was unique! Lol.

One parent’s gene has to be faulty and the other has to be not working at all. That's how someone gets what I get. So both my parents would themselves have had no problems in the sun because they also had a second healthy gene  that was absolutely fine. He asked if anyone else has it, b ut no, I’m the ‘lucky’ person who inherited this awful skin problem.

Normally I can't go in hot sun for longer than about three or four minutes before it starts feeling like it's burning. If I stay in it too long, it swells up – the backs of my hands go all gel like and puffy and it burrrrns like crazy!  I always just thought it was sensitive skin. But it's this unusual situation under the skin where chemicals are interacting with a part of sunlight.

I’d have guessed it was something to do with UVA or UVB but apparently it’s not. They think that it's actually violet light, as in the violet end of the rainbow. What happens is that it creates a chain reaction inside my skin with a enzyme which is missing called Ferrochelatase. This has to be present to change something called ‘protoporphyrins,’ into  ‘heme’ as in haemoglobin
. But in my skin the process doesn’t work – hardly at all it turns out – so  because my protoporphyrins don't get converted  I  end up with a build up. These protoporphyrins coat  the insides of the tiny tiny blood vessels just under my skin. On the hands and feet and face the skin is skinner – Alison Young was always right! And it's because it's so thin that the violet light gets through when it's exposed to the sun for too long and this creates a catastrophic breakdown of the tiny blood vessel walls. Because there is no blood vessel any more the pain is excruciating. What also happens is that anything that should have been pumping through the blood vessel has nowhere to go, so my hands puff up because of all the fluid that should have been contained in the blood vessels. It takes two days for it to go back down again because that's how long it takes to regenerate new blood vessels.

The pain during that time is like putting your hand on an iron - it really is that hot. And now I know why. There is no known cure, but he's going to see me again after blood test to tell me more about what tiny things we can do to mitigate and lessen the photosensitivity. I told him how Ultrasun 50 definitely feels like it helps me. Maybe they’ll do something one day which blocks some of the violet light as well
, who knows. I must ask Abi Cleeve :-)

It feels good to know I'm not the only one in the country, not alone. Have you ever felt that if you discovered and illness? One of my closest Friends has something called IIH. And she and I chatted about how comforting it was just to discover what it was even though not a lot can be done about it.

One day they may have developed a drug which helps. Apparently during pregnancy it doesn't happen. It was so long ago for me,  I don't remember to be honest. But it would be great to think that at some point in the future I can possibly get a holiday booked in the red Sea or the Maldives and know I can do all the sports activities
, diving et cetera and be like a normal person. But having stayed out of the sun for so many years my skin is reaping the rewards, so I have to look on the bright side. People who know me know I always do!

This week I went to see the expert in EP p, my skin condition, for full info about my first post go here. If you don't know me well this is where my skin gets burning hot if I stay too long in the sun.

So I saw Dr Robert so Caney, the countries for most expert in this disease called a PP in a wonderful relaxed calm setting in Lister Hospital in Chelsea, near our old building! (Well the place it used to be, it's flats now) he explain to me how a PP works

I have to have inherited gene from both my parents, that's why it is so rare, only one in 250 250 people in the country have it he thinks, that's her where I am

One parents Jane has to be faulty and the other has to be not working at all. That's how someone gets what I get. So both my parents would have had no problems in the sun because they also had a second Jane that was absolutely fine, and on the lucky person who inherited this awful skin problem.

Normally I can't go in the sun for longer than about three or four minutes before it starts feeling like it's burning. I always just thought it was sensitive skin. But it's this unusual situation under the skin where chemicals are interacting with a part of sunlight.

I thought it was the usual UVAUVB. But it's not it's actually violet light, as in the violet end of the rainbow. What happens is that it creates a chain reaction inside my skin with a enzyme which is missing coffee row Kiliti is. Favourite killer tase is supposed to help produce blood cells, as in haemoglobin, the Haim part of haemoglobin, with something that we all have in our body is called protoporphyrins. But because my protoporphyrins don't get converted because there is not enough for you Sarah Katie is enzyme, I end up with a build up. These protoporphyrins line the insides of the tiny tiny blood vessels just under my skin. On the hands and feet and face the skin is dinner – Alison Young was always right! And it's because it's so thin that the violet light gets through when it's exposed to too long and creates and catastrophic breakdown of the Bblood vessel wall. Because there is no blood vessel any more the pain is excruciating. What also happens is there anything that should have been pumping through the blood vessel is now no no come on nowhere to go, so my hands puff up it's all the fluid that should have been contained in the blood vessels. It takes two days for it to go back down again because that's how long it takes to regenerate new blood vessels.

The pain during that time is like putting your hand on an iron it really is that hot. And now I know why. There is no known cure, but he's going to see me again after blood test to tell me more about what tiny things we can do to mitigate and lessen the photosensitivity. I told him how old son 50 definitely feels like it helps me. Maybe I'll do some blocks some of the violet light as well who knows. I must ask Abi Cleeve :-)

It feels good to know I'm not the only one in the country, not alone. Have you ever felt that if you discovered and illness? One of my closest Friends has something called I H I H I R I H. And she and I chatted about how comforting it was just to discover what it was even though he is not a lot can be done about it.

One day they may have developed a drug which helps. Apparently during pregnancy it doesn't happen. It was so long ago I don't remember to be honest. But it would be great to think that atat some point in the future I can possibly get a holiday booked in the red Sea or the Maldives and no I can do all the sports activities diving et cetera and be like a normal person. But having stayed out of the sun for so many years my skin is reaping the rewards, so I have to look on the bright side. People who know me know I always do!

Anyway I will keep you informed of any news.

Best wishes

Debs

x

 



Published in Back to You

So it turns out I've got a thing - it's called EPP and it's a proper 'thing.' All my life I've had to stay out of the sun, as regulars will know if you recall my blog from early July when I had to report that my 'skin had flared up' during the Rochester Castle fundraising bodyblade wobble in Kent. What happens is that if my skin is over-exposed it feels like it's on fire, even though nothing shows on the outside. Then it starts to itch as well, and then it swells up and the skin gets super tight. It takes around two days to go down.

 

When I was on a skiing hol once using a balaclava, the skin in between my eyes swelled up and my eyes were like slits. When I stupidly used a sunbed aged around 22 I had the most horrendous reaction my rings needed to be cut off my fingers. And apart from one occasion, I've never been able to do normal things in the sun. When I was a kid I had to shelter away from the sun, I felt like my needs were never made a priority as I just had to make the best of it and bought comics and books and sweets and sat away from everyone else and rarely went in the sea. (the pic above is when I was about 9, that's me behind the deck chairs in the shade. Poor Debbie.) My family know this, and just take it for granted, and so did I, until this year.

I'd been genning up on how gut bacteria affects everything and remembered that the only time I felt like I could go in the sun was, yes, with my regular triple whammy of high once a day spf, plus L-tyrosine tablets (they work on the melanin from the inside out) and tan accelerator (it works on the melanin from the outside in) BUT I'd also been vegan for three months and was eating clean for another seven before I went to Cyprus for my brother's wedding. It was the first and so far only time I've been able to be in the pool with my kids on a summer holiday. So maybe there's a connection with nutrition. So I went to see a specialist.

The dermatologist called Dr Flohr, at Guildford Nuffield, asked me a very pertinent question. Why was this the first time I'd ever come to an expert? I was stumped by that. Have you ever had it where you just accept that's the way life is and have stopped questioning it? I guess that was me. It had been part of me, my reality, for so long, it didn't really occur to me to challenge it, it was just sensitive photosensitive skin, right? Till the gut thing. So here I was. And he looked me in the eye and said 'I think you've got this.'

It was EPP.

Erythropoietic protoporphyria (EPP) is one of a group of genetic diseases called the porphyrias. EPP is due to an inherited deficiency of the enzyme ferrochelatase. Reduced activity of this enzyme causes a build-up of the chemical protoporphyrin in the skin; resulting in photosensitivity i.e. the skin is damaged by light. There is no known cure, but you can reduce the photosensitivity with certain supplementation of enzymes etc. More here.

He swung the monitor round and showed me the page and I read words that resonated so strongly they of course could be writing about me. I was shocked. “First symptoms usually appear in infancy or early childhood and present as an uncomfortable or painful burning sensation of the skin after sun exposure. It occurs most often on the tops of the hands and feet, face and ears. In most cases visible changes to the skin are mild. The affected skin may become red and swollen and blistered.”

But at least I don’t now have the worst version of the condition – “Later there are pitted scars and sometimes crusty thickened skin, particularly over the cheeks, nose and knuckles of the hands.” Although I used to get a nose scab every summer when I was young. Plus my knuckles are not attractive – nor are my hands – I always said I have man’s hands, my dad’s hands, and now I know why. Hence you’ll often see me on jewellery shows using whole hand shots rather than close ups of my hands with the jewellery on. Don’t suppose you’ve ever noticed but that’s why. When the condition flares, after the swelling starts going down again and the burning dissipates, I’m left with a rough, sensation-deprived surface on my knuckles in particular. God, now I know why.

If this is indeed what I have, and it seems as though it is, then I’m rare.

The treatment possibilities include –

Lifelong photosensitivity is the major problem for EPP uncomplicated by liver disease, which about 10% may go on to get due to the protoporphyrin build up.

  • The obvious stuff I already do, eg avoid unnecessary exposure to sunlight and wear protective clothing and wide-brimmed hats. Sunscreens, especially formulations containing zinc oxide or titanium dioxide that reflect visible light. (I told the doctor about tan accelerators which he hadn’t heard about, I don’t think, and when I said Ultrasun he nodded his head.)
  • Consider tinting windows.
  • Other strong sources of light may also cause symptoms, including fluorescent and halogen lights. (I’ve had this before and thought I must be imagining things.)

But I’ll be looking into taking some of these for sure – maybe my vegan time included lots of carrots!:-

  • Oral beta-carotene (a food precursor of vitamin A found naturally in tomatoes and carrots) is thought to help reduce photosensitivity in some people. The dose for children is 30 to 150mg per day (1 to 5, 30-mg capsules) and for adults it is 30 to 300mg (1 to 10, 30-mg capsules).
  • Cysteine 500mg twice daily reduces photosensitivity
  • Colestyramine reduces photosensitivity and hepatic protoporphyrin content.
  • Narrowband UVB phototherapy increases melanin content and induces skin thickening so may reduce sun sensitivity.
  • Afamelanotide, an α-melanocyte stimulating hormone given by subcutaneous implantation, has been reported to provide good clinical effectiveness and safety in EPP. It is approved under exceptional circumstances by the European Medicines Agency for treatment of EPP (October 2014).

So there you go. And there I go. So now I am being referred to the country’s foremost expert , a colleague of Dr Flohr, who apparently did his doctorate in THIS SUBJECT!

I feel a bit weird since it’s like suddenly I’m not so strange, there are others like me out there. I spoke with a mate who has IIH and who felt exactly as I felt when she was first diagnosed. It’s not something to be proud of  cos everyone wishes they were ‘normal.’ BUT it feels relief to know there’s a course of action. I will keep you informed.

Best wishes

Debs

x

 ps coming soon - the latest update from my Till the Fat Lady Slims group - some super before and afters, stories and NSVs (non scales victories!)

 

 

 

Published in Back to You