Friday, 10 June 2016

DAY 10: The Skin, Raynaud's Scleroderma, Rare Disease, Scleroderma Awareness Month 2016

DAY 10  Scleroderma Awareness Month: Skin Involvement 

One of the major organs affected by Scleroderma is the thickening of the skin, as the name implies, Sclero meaning thick, derma meaning skin. 

This is usually the first tell tale symptom to prompt a diagnosis. Skin involvement can vary depending on the type of scleroderma, with the diffuse type involving most, if not the entire, body. 

See Day 1 Scleroderma Awareness Month for the level of involvement in each different type, information.

As well as,  Day 4  Scleroderma Awareness Month, Symptoms.

Skin involvement is used as one of the criteria required for the classification of a scleroderma diagnosis, as by the ACR / EULAR globally recognised and accepted guidelines. DAY 6  Scleroderma Awareness Month: ACR / EULAR guidelines

Skin involvement is measured using the Modified Rodnan Skin Score test, (mRSS) where thickness of the skin and its location on the body, are the focus. 

Steen and Medsger reported in Arthritis Rheum, 2001, that ‘improvement in skin thickening in systemic sclerosis is associated with improved survival’. Which of course is encouraging, however, skin thickness can not be regarded as a direct indicator of internal organ involvement. 

Although, it can be used as a helpful aid upon further medical diagnostic tests being carried out to confirm internal organ involvement severity, if any. See DAY 5 Diagnostic Tests. 
Clinical trials involving scleroderma include the reduction in skin thickness / fibrosis as an endpoint to investigating whether a drug can be used to treat scleroderma. 

Currently, most scleroderma treatment options which are available, are focussed on reducing the fibrotic process, with the skin being the easiest non invasive test indicator for confirmation. 

For more information on skin thickness and testing click  

From a patient perspective – having thick, tight, itchy skin is extremely painful and debilitating. The sensation is similar to feeling as though your body outer layer has shrunk a few sizes too small to house your body. This makes for routine blood tests to be impossible due to skin thickness and so a portacath / portavein can sometimes be an alternative option, as seen in today’s image. 

When I presented with my initial diagnosis, symptoms back in the Autumn of 1997, the skin on my hands, arms, thighs and chest was extremely tight, sore, sensitive, thick and itchy. I was prescribed methotrexate but decided to try homoeopathy and reflexology first. 
Within 5 months I returned to the hospital unable to stretch my arms or legs out as my skin was so tight. This was accompanied by immense exhaustion and joint swelling and pain. 

This time, I took the methotrexate, and then prednisolone, and then hydroxycholoroquin, and then cyclosporine and then tacrolimus, with little symptom relief effect, other than horrendous side effects including nausea, lack of appetite, fatigue
In December 1998, I arrived at The Royal Free hospital to see Prof Black. She stopped all of my medications and subjected me to 5 rounds of iv cyclophosphamide, combined with 2g mycophenolate mofetil. 

Prof Denton then took over my medical care and continued with the mycophenolate 2g /day regime until 1st March 2004 when I qualified as a self employed practising barrister, and I decided to decrease the dose with viewing to stopping completely. 

The 29th June 2004 was the last day I appeared in Crown Court wearing my wig and gown, as I had to accept that the 60 hour a week role was too demanding for my body. And, as it has turned out, randomly, this date, was subsequently made World Scleroderma Day! andso it is quite meaningful and poignant for me!

To read my blog post about World Scleroderma Day click here  

I am overjoyed that my skin is now at 95% reversal, and I am now 12 years chemo / immunosuppressant free. Although, I no longer have skin tightening or thickening, my skin is extremely sensitive with the outer layer being quite thin, similar to tissue paper, with my sweat glands having been altered also. 

Not long after diagnosis, I started a daily skincare regime which has proved to be essential for me, to help ease my skin symptoms. This involves starting my day by bathing in liquid paraffin, and then moisturising with a thick emollient. I use Oilatum Plus in the bath, and Hydromol cream for my body. I have tried all sorts of different skincare product combinations, but have found that this is the best combination which works for me. 

I am not able to have a shower, as the process takes out too much moisture from my skin – even if I use baby oil! A warm bath also helps ease my constant musculoskeletal pain and joint pain. 

On some days, just performing my skincare routine, can be the most that I can do for that day. To read my article ‘Ah great, another day to spend in bed, said no Raynaud’s scleroderma patient’ click here  

My skincare routine has been the springboard for me creating a scleroderma awareness presence in the media, possibly at the expense of my marital status haha: 

All in all, current treatments are only successful with reducing skin fibrosis if the patient presents at the clinic early enough to be responsive to the treatment, and even then it is hit and miss, as there are no two patients who display the exact same set of symptoms, and may not respond to the treatment. 

The more modern treatments such as mycophenolate are more specific in their action and are known as ‘biologics’, which have also been shown to be better tolerated and have an improved side effect profile. 

However, these treatments are only targeting symptom progression and are not a cure. The biologic treatments are the key to unlocking the mystery of the autoimmune disease holy grail – in my view!
An edited version of this article was originally published here in my Column with Scleroderma News. 

To read my articles:  
BSR and BHPR guideline for the prescription and monitoring of non-biologic disease-modifying anti-rheumatic drugs, Raynaud's, Scleroderma, Rare Disease click here 
The importance of an early diagnosis click here 
the role of medical research click here
the importance of expert specialist centres click here
diet and nutrition click here
taking part in research trials click here
the UK guidelines for management and treatment of Scleroderma click here
my skin is cured from scleroderma click here 
skin involvement click here
skin tests click here
skin cancer in scleroderma click here 
Full time job of managing the symptoms click here  
EULAR guidelines for treatment of systemic sclerosis scleroderma, click here  

Please DONATE to help fund medical research at the Scleroderma Unit at The Royal Free hospital where 100% of your donation will be used to fund vital medical research. Thank You.
Living the dream, scleroderma style, hoping for a cure
#SclerodermaFreeWorld #RaynaudsFreeWorld 
29th June World Scleroderma Day. Read more here
Scleroderma Awareness flashback 2016. Read more here  
Scleroderma Awareness 2016 Campaign Index Click here  
Follow Raynauds Scleroderma Awareness Global Patients Facebook Page for latest updates 

To view the Facebook page Scleroderma Awareness Month 2016 campaign images Click  here
To view Facebook page Photo Album ‘Wellbeing Side to living with Raynaud’s and Scleroderma’ Click  here  

Please use the icons below to share this article #SclerodermaAwareness

No comments:

Post a Comment