Friday, 10 June 2016

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



DAY 10: Skin Involvement 

Scleroderma Awareness Month 2016 
Raynaud's, Autoimmune Rare Disease 


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 here  

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. 

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! and so, 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 published here, in my Column with Scleroderma News. 
June 2016. 

To read my articles: 


Rare Disease Day 2018, Research - Taking Part in Clinical Trials, Click here   

March Autoimmune Disease Awareness Month 2018, Click here   

Global patient video, Click here      

If we only had more RESEARCH investment for Scleroderma, Raynaud's, Autoimmune Rare Disease, Click here   


Scleroderma Awareness Month 2017 Patient Profiles Campaign, Click here     
 
Scleroderma Awareness Month 2017 Patient Profiles Campaign Patient Index, Click here        
The Pandora’s Box of the rare autoimmune disease Scleroderma, Raynaud's and Cancer, Click here



Key Event Dates 2018, Click here     

Importance of an early diagnosis, Click here     

Taking Part in Clinical Research Trials, Click here    

The Importance of a Multi Disciplinary Medical ‘Dream Team’, Click here  

Expert Specialist Centres, Click here      

My Skin is Cured from Scleroderma, Click here      

UK Guidelines for Managing and Treating Scleroderma, Click here      

Fatigue, Click here      

Mobility, Click here       


Raynaud's, Click here    


World Scleroderma Day 29th June 2016, Click here

World Scleroderma Day 29th June 2017, Click here        



RARE DISEASE DAY:

The theme to this years Rare Disease Day is Research

Rare Disease Day 2018 – Research, Taking Part in Clinical Trials. Scleroderma, Raynaud's, Autoimmune Rare Disease, Click here     

2016 Rare Disease Day Patient Voice
2016 Rare Disease UK Parliamentary Reception 

2017 Rare Disease Day Flashback  

More unmet clinical needs  

Rare Disease Day 2016



Rare Disease Day is a fantastic opportunity for the entire rare disease community to shine a spotlight on their reality, combining as one unified voice. Where, at least one commonality presides –  
Medical Research provides the brightest light
for the illumination of the rare disease patients’ plight. 

I highlighted other areas of medical research interest within Week 3 of my Patient Profiles Campaign for Scleroderma Awareness Month 2017. 

VIDEO



Although rare disease patients are few in number, eg. 2.5 million scleroderma patients worldwide, (the World Scleroderma Foundation), the commonalities and golden hallmark for each rare disease patient are the same: 

Early Diagnosis
Expert Specialist Centres
Access to Innovative Medicines

Where MEDICAL RESEARCH investment is VITAL.

Research is the key. Abstracts from 2016 World Congress, Click here


Scleroderma Awareness Month Campaign 2016, Click here

View video, here

Preamble - here


The Family Day at the Scleroderma Unit, The Royal Free Hospital is taking place on the 19th May 2018. Browse the program, here

This year, I am celebrating 20 years of being a patient at this world leading expert specialist research centre. 

Sept 2017


I am eternally grateful to the global scleroderma trail blazers Dame Prof Black and Prof Chris Denton, whose commitment and dedication to unlocking the scleroderma enigma, is nothing other than, superhuman.
I am truly humbled and inspired by their work ethic. I am wholly appreciative for Prof Denton’s continued medical expertise and support, especially during my barrister qualifying years, 1997 - 2004. 

1st March 2004, I qualified as a self employed practising barrister. Further to having been told in 1997, by my diagnosing doctor, that I was looking at a 15month prognosis. 

I very much hope to utilise my professional skills and qualifications along with my patient experience, to help achieve the #SclerodermaFreeWorld dream, hoping to improve understanding and best practice, in the meantime.
  
For latest updates follow: 

Facebook Page:

Twitter: @SclerodermaRF  @RaynaudsRf  


#SclerodermaFreeWorld           #RaynaudsFreeWorld
#ADAM #Scleroderma
#Raynauds
#RareDisease  

Living the dream, scleroderma style.  


Please DONATE to help fund medical research at The Scleroderma Unit,The Royal Free Hospital, London.  



100% of your monies will be used for medical research purposes only. Thank You. 
#HOPE 


 

Last Update: April 2018




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