Friday, 13 April 2018

Raynaud’s, Scleroderma and Digital Ulcers. Autoimmune, Rare Disease. Nicola Whitehill.

Raynaud’s, Scleroderma and Digital Ulcers 

Here in the UK, summer is gradually transforming into autumn. The decrease in temperature can be seen by the change in colour to the leaves on the trees, as well as, a change in colour to my fingers and toes. 

Even on a hot summer’s day, I struggle to maintain a healthy normal colour to my hands and feet. The smallest of breezes or a draft, is sufficient to set off my Raynaud’s symptoms.  

Hottest day of 2013, I have to wear Ugg boots and gloves
As all patients know, all too well, there is currently no cure for Raynaud’s.  

 
During the course of the last two decades, I have learnt, (the hard way, I hasten to add), that prevention is most certainly the best way for me to manage my Raynaud’s hypersensitivity.  

My nemesis season, being Winter.



Jan 2017 Vlog. 

Consequently, my reality for optimum symptom management, means me being housebound for most of the year. Intense hibernation and house arrest, are a foregone conclusion from October – April.  

A seasonal change will bring along a different set of symptoms to juggle.  

‘You should get out more’ is certainly not a viable option for me, whilst trying to maintain a constant body temperature, in the hope to prevent a Raynaud’s attack.  

In the event of a Raynaud’s attack, my entire body will go into spasm, as well as, I lose the colour to my hands and feet.   





The time taken to eventually ‘warm up’ again- bringing the colour back to my extremities, will vary from minutes to hours, depending upon the intensity of the attack. 

This will then be followed by complete exhaustion, whilst my body repletes its energy levels, which, further to my scleroderma symptoms, are minuscule at the best of times.  

 

Fatigue remains one of my biggest challenges.    



DIGITAL ULCERS     

Prior to me taking the medicine, bosentan, I would constantly have ulcers on my fingers, and sometimes, on my toes. 

I can not describe accurately, the excrutiating pain which ulcers bring. Along with, the time consuming process for them to heal. 

In the event that they do not heal, and become infected, amputation of the affected digit / area, is a realistic possibility. Hence my strict house detention rules. 
   
I included Digital Ulcers as one of the many Unmet Clinical Needs of the Scleroderma patient, for my article for Rare Disease Day 2017.   

  

Digital ulcers were also highlighted by EVERY patient, who kindly, took part in my patient profiles campaign for Scleroderma Awareness Month 2017.  





In particular:  


Deani UK  






Jan UK   




Alex UK    





Kelli from the USA   




Thanks to Kelli for this image - used extensively throughout the awareness campaign
As stated earlier, there is currently no cure for Raynaud’s, with treatments altering blood pressure, whilst widening the blood vessel, in an attempt to maintain the circulation and blood supply.  



 

Sadly, I am unable to tolerate any of the recommended ‘treatments’, as my blood pressure is too low and I experience the side effects of dizziness and nose bleeds.  

I am extremely honoured to have been a contributor with the medical team who compiled the NHS England guidelines for the treatment of Digital Ulcers, published June 2015.  

Within my advisory role to the European Medicines Agency (EMA), my suggestion that digital ulcers be included in ALL scientific clinical trial protocol has been endorsed - against much protest from pharmaceutical company representees! 

My argument being, that, for a new compound to be given the licensed indication for treatment of systemic sclerosis, a positive effect on digital ulcers must be seen, along with other biomarkers, and not just, a reduction in skin fibrosis.  



Management of Digital Ulcers – UKSSG

The Editorial contained within Vol. 2 Issue2, May – Aug 2017, of the Journal of Scleroderma and related Disorders, sets out ‘The critical need for accurately defining digital ulcers in scleroderma’ 


The research article ‘Defining skin ulcers in systemic sclerosis: systematic literature review and proposed World Scleroderma (WSF) definition, is contained within the same edition. 

Both the Editorial and the research article set out the difficulties which digital ulcers pose in their identification, management and treatment.   
 
However, the presence of this topic being discussed within the Journal, is very encouraging to the patient. 

Since, in my view, digital ulcers, albeit not life threatening per se, have not been given the medical and pharmaceutical recognition which they deserve, as by their causing complete misery and catastrophe to the Raynaud’s and scleroderma patient's day to day activities.

Living the dream, scleroderma style. 
 
October is Raynaud’s Awareness Month.


Living the dream, scleroderma style.

Please DONATE to help fund medical research at The Scleroderma Unit, The Royal Free Hospital, London where 100% of all of your monies will be used for medical research purposes only. Thank You.

#SclerodermaFreeWorld #RaynaudsFreeWorld
#RareDisease #Hope #Belief 

Follow Raynauds Scleroderma Awareness Global Patients Facebook Page for latest updates  


   





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

September 2017.  
 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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