Saturday, 4 June 2016

DAY 4: Scleroderma, Raynaud's Symptoms, Scleroderma Awareness Month 2016, Autoimmune Rare Disease.


DAY 4 - Symptoms

The symptoms of Scleroderma can differ from patient to patient, with no two patients displaying the exact same set of symptoms. 

This makes diagnosis and treatment more challenging to meet the needs of the individual patient.

In all types of scleroderma (see Day 1 Scleroderma Awareness Month, for the different types), the skin is affected. 

In the diffuse form, all parts of the body can be affected, including soft tissue, organs and other complications as a result.

Stiff, puffy fingers along with thick, tight itchy skin, difficulty in swallowing, stomach cramps and pain, with swollen joints and immense tiredness, are the typical symptom hallmarks of the diffuse scleroderma patient.

Early diagnosis is crucial to prevent potential life threatening damage. 

Treatments are targetted at symptom suppression only, with a cure still to be discovered. Organ transplant is a final option, if the patient fits the relevant criteria, including being strong enough to withstand the procedure.

I have lived with diffuse Scleroderma for 19 years, since 1997. Although, it is likely that I presented with some of the symptoms some time before diagnosis, I just put them down to being a normal 24 year old loving life, with a busy social life and professional career.

However, my hands turned stiff and non functional, similar to arthritic symptoms, prompting me to seek medical intervention.

Diffuse scleroderma can be extremely aggressive in its progression, attacking the body’s organs and causing irreversible damage. 

Chemotherapy and immunosuppressive agents help at suppressing the symptoms with organ transplant and stem cell transplant being the final treatment / management options, see above. 

Early diagnosis is crucial to attempt to prevent lasting potentially life threatening damage. 

And, even then, not everyone will respond to the pharmaceutical treatments which are currently available.

I see myself as being an extremely lucky diffuse scleroderma patient. Lucky in that I have minimal internal organ damage, and I am still around to shout all about what scleroderma can do. 

My full time job is managing my symptoms, which on some days is easier than others. 

I am eternally grateful for the medical expertise and care from Prof. Dame Black and Prof. Christopher Denton, who quite literally saved my life. 

My diagnosing Doctor had given me a prognosis of 15 months in September 1997. In December 1998, I had my first consultation with Prof Dame Black and then Prof Denton, who I ‘knew’ would help me get better. 

I spent the first seven years taking chemotherapy / immunosuppressants. I am now 12 years free from this regime. I have reduced the 24 tablets a day, back in my hey day, to just 2 tablets a day. 

I take Bosentan for my Raynaud’s, and digital ulcers. My hands are the best that they have been since before diagnosis, having taken Bosentan continuous for three years plus now, and very importantly to me- ulcer free! 

I have a strict daily routine for managing and respecting my diffuse symptoms, including a strict diet which includes daily fresh juices made by me, in my pharmacy, which is my kitchen! 

I am a living example that this diagnosis can be managed on a long term basis, and for this, I know, I am truly blessed, even if, I am now a scleroderma parrot.

 

 




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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