Sunday, 16 April 2017

The Skin, Scleroderma Raynaud's, Autoimmune Rare Disease - Nicola Whitehill

 
 The Skin
Scleroderma Raynaud's, Autoimmune Rare Disease



The skin is the largest organ in the body and is affected in most scleroderma patients. 

The exception to this rule is the limited subset version of scleroderma patients.

See Day 1 of my Scleroderma Awareness Month 2016 campaign.



At the time of my diffuse scleroderma (dcSSc) diagnosis in 1997, I presented with extremely tight skin across my chest, arms, hands, face, legs … all over my body. My hands and fingers were puffy with tight, itchy, shiny skin and painful joints. I was unable to make a fist and could not remove bottle tops, the lids of jam jars, or the like, and had other symptoms (joint pain, gastro-intestinal, pain, lethargy, fatigue, and so on).

I discussed this on Day 4  


After years of chemotherapy and immunsuppressants — I am now 12 years cytotoxic drug free — my skin has reversed to 97% normal healthy skin, according to my last medical assessment in May 2016, with the dedicated scleroderma world expert Professor Chris Denton.

The Modified Rodnan Skin Score (mRSS) is the standard measurement assessment test used to identify scleroderma skin activity.  

A skin score is important because:
  • Skin score often peaks within one year of dcSSc onset
     
  • There is an association between severe skin involvement and burden of organ-based complications
  • Death or major organ-based morbidity occurs in 50% of dcSSc cases within 3 years of disease onset
  • Stabalization or improvement in skin sclerosis within 2 years of diagnosis may confound studies designed to prevent skin score worsening
  • Latent Trajectory Modelling identifies distinct subgroups defined by skin score
To learn more about this, Click here

I have a daily symptom management routine that includes bathing and moisturising my skin. 

For the bath, I use liquid petroleum (Oilatum Plus) and then, after towel drying, I use Hydromol emollient cream all over my body.


I use sensitive skin skincare laundry products and only wear clothing that is soft against my skin. Sadly, my Armani jeans have remained at the bottom of my wardrobe since diagnosis, as denim is too heavy against my skin, causing my inner Bond girl to go AWOL.

As with most things scleroderma-related, nothing is that simple, and although I have regained the elasticity and texture of healthy skin, I am challenged with skin-related symptoms caused by the scleroderma, such as calcinosis, telangiectasia, Bowen’s disease, and a possible basal cell carcinoma on my forehead.

I discussed these skin challenges on Days 10, 18, 19, 27 and 28 of Scleroderma Awareness Month. 

The mystery of the calcinosis/basal cell carcinoma (BCC) will be investigated in two weeks time, which I am trying to remain calm about, having had a very impromptu, if brief, meltdown in a phone call with the local dermatologist’s secretary to book the appointment last week. 

I had spoken with this secretary a few years ago, when this calcinosis/BCC mystery commenced its journey, and she remembered me, so luckily she understood that I was having an uncharacteristic, albeit embarrassing, reaction, and she was most kind and supportive. I have already purchased the chocolates to take to her on the appointment day!

I know that most of my fellow patients will wholly understand my little blip when booking another medical appointment due to another complication of scleroderma. 

I also know that I am very blessed to have gotten to year 19 without a skin biopsy, and I am in awe of my fellow (much braver than I) patients, who have been subjected to skin biopsies when their skin is tight and inflamed.

The small skin patch of Bowen’s on my left knee is thought to have been caused by sun damage. I cannot stress enough how important it is to wear sun protection, especially if you are taking — or have taken — any of the chemotherapy and immunotherapy drugs widely used for scleroderma symptom suppression. 

I went into more detail about this in 'The Magic of Summer'.


I discussed my annual monitoring tests, here 


As with the management of most of my Raynaud’s and scleroderma symptoms – prevention is better than cure, since there is no cure.

So, I now avoid being in any direct sunlight in the hope of preventing further unwanted scleroderma skin complications, as, I want to feel good ~ living the dream, scleroderma style.



An edited version of this article was published here, in my Column with Scleroderma News.  August 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   

Becoming a Patient Research Ambassador for the NIHR, 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    


Diet and nutrition, Click here     


Raynaud's, Click here    



World Scleroderma Day 29th June 2016, Click here

World Scleroderma Day 29th June 2017, Click here   


Why Global Collaboration is important to the Rare Disease Patient, 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  
  





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

Links

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. No wages or admin costs. Thank You.
#HOPE 


 

Last Update: April 2018




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