Thursday, 31 May 2018

CALCINOSIS - Unmet Clinical Need. Scleroderma Awareness Month, June 2018. Scleroderma, Raynaud’s, Autoimmune, Rare Disease

 CALCINOSIS  - Unmet Clinical Need 

Scleroderma Awareness Month, June 2018
Scleroderma, Raynaud’s, Autoimmune, Rare Disease

www.justgiving.com/fundraising/sclerodermaunit-royalfree
#SclerodermaFreeWorld #RaynaudsFreeWorld

Today’s topic is the debilitating, painful symptom of calcinosis.  



It is important to note that not every scleroderma patient will experience calcinosis, since no two scleroderma patients present with the exact same set of symptoms. 

Calcinosis is more prevalent in the diffuse subset of scleroderma. 

The intake of calcium in the diet does not have a direct correlation with calcinosis. 

As can be seen in the diagram, calcinosis is not specific to certain areas of the body, it can appear anywhere, but usually, at sites of soft tissue injury or pressure points, which includes the gums. 

The lumps of solid calcium can lie underneath the skin, being visible only by X-Ray, and are hard to touch, if near the outer surface. 

Calcinosis is extremely painful, especially with tight, scleroderma-like skin. The lumps will eventually work themselves to the surface of the skin, where they can be pushed out. This process can take years. 

Added complications can occur if the area becomes infected, requiring antibiotic intervention. Which of course, brings along added gastro-intestinal challenges. 

In my experience, the excision of a calcium lump is extremely painful, leaving a hole in the flesh, from where the lump has been. 

On a few occasions I have almost passed out with the pain when a lump finally becomes loose and can be removed, leaving a huge hole which eventually fills with blood. Deep breaths, help at this point!  

Removal is no guarantee that the calcium will be gone forever from that site, as the removal site can form more calcium. 

I have pitting and scars around my knees, nose, elbows, toes and fingers from where calcium lumps have been. 
The calcium can also be a soft, chalky-like liquid underneath the skin, which can be excruciatingly painful, if touched. 

Similar, to the lumps of calcium, the softer lumps can take years to work themselves to the surface of the skin for removal. 

This process being hindered by tight skin and a compromised immune and circulatory system, due to the nature of the scleroderma disease process.  




Although calcinosis is not a direct life threatening symptom when comparing to severe internal organ involvement, such as heart failure or renal crisis, it is a huge unmet need of the scleroderma patient. 

Currently there are no adequate treatments or medications for calcinosis. 

Some patients report a marginal improvement when taking the antibiotic Minocycline, personally, I saw no improvement. 

I have also spoken with patients who had undergone surgery for calcium removal, with the calcium only returning in time. I am not brave enough for this option being a reality for me! 

On a personal observational level, the calcium lump or liquid appears as sites of injury and pressure points, with tight skin, adding to the make-up ingredients. 

I have also noticed that Raynaud’s episodes / blue fingers and toes can help the calcium forming. 
Prof Herrick and her team at Salford Royal Hospital in Manchester, here in the UK, have a special interest in calcinosis research. 

With their most recent publication being published in the Scandinavian Journal of Rheumatology at the start of the year, concluding that:
‘The thumb is affected by calcinosis more than other digits, followed by the index finger. 

This observation provides insight into the pathogenesis of SSc-related calcinosis, which may relate more to repetitive trauma than to ischaemia.’ 

To learn more about Prof Herrick and her team, Click here

In my view, investment in research is needed as a matter of emergency to investigate the formation of solid calcium / bone-like lumps taking over scleroderma patient body. 

I am very much hoping that the Factor XIII trial being carried out by the clinical trials team at the Royal Free Hospital will reveal the calcinosis antidote. 

 
 
Read  Day 13   Scleroderma Awareness Month 2016. 


 

An edited version of this article was published here, in my Column with Scleroderma News. 
June 2016. 
 


More Current Unmet Clinical Needs: 

GUM RECESSION, TOOTH EXTRACTS, Click here 

SOFT TISSUE, ULCERS, Click here  

TELANGIECTASIA, Click here 

SCLERODACTYL HANDS, Click here   

INVISIBLE DISABILITY, Click here 

RAYNAUD'S, Click here 

GASTROINTESTINAL, Click here



Prof Chris Denton presenting a plaque to Kevin's daughter, Michelle, and wife, Debby, for funds raised in Kevin's memory for medical research at the Unit. Thanks to Michelle for the photo.
  
'Scleroderma Awareness Month 2018: Medical Research Fundraising, RIP Kevin East', Click here  


Global Patient Profiles Campaign Index, Click here


Importance of Medical Research and Awareness Raising, Click here


NIHR Video: 'My Experience of Clinical Trials', Click here 


Week 3, 2017 Patient Profiles: Medical areas of current interest, Click here


Week 2, 2017 Patient Profiles: Progress in Medical Research, Click here 


Week 1, 2017 Patient Profiles: Introduction to multi complex disease, Click here


Why I chose global Patient Profiles for my 2017 / 2018 Campaign, Click here 
 

2017 Global Patient Profiles Campaign Introduction, Click here 
 

'Being your own awareness advocate', Click here
 

Scleroderma Awareness Month Flashback 2017, Click here 


Scleroderma Awareness Month Flashback 2016, Click here 


My 2016 Campaign, A to Z: Scleroderma and Raynaud's diagnosis, Click here 
 

2016 Campaign Video, Click here 


March 2018 Autoimmune Disease Awareness Month, Click here 


World Scleroderma Day 2017, 29th June, Click here 


World Scleroderma Day 2016, 29th June. Origins of the date, Click here 


World Scleroderma Day 2015, 29th June. 


James Carver, myself, Prof Chris Denton

I had the pleasure of being a presenter and part of the European co-hort who gave a presentation at the European Parliament, Brussels, in honour of World Scleroderma Day 2015.  

I am immensely grateful to James Carver MEP for organizing this event in memory of his late wife Carmen, who sadly passed from Scleroderma.  

To view the presentation, Click here 


To view my article 'Why Global Collaboration is Important', Click here


All patients included within the global patient profiles campaign, highlight the URGENT need for an investment in medical research, as well as: 


Early Diagnosis 
Expert Specialist Centres 

Access to innovative medicines 

ALL being, crucial factors for best practice management and care.  

To read my articles:
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   
This year, I am celebrating 20 years of being a patient at the Scleroderma Unit, The Royal Free Hospital - a world leading expert specialist, research centre. 
Sept 2017

Prof Chris Denton and I, 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. Read more, here
For latest updates follow: 
Facebook Page:
Twitter: @SclerodermaRF  @RaynaudsRf  
#SclerodermaFreeWorld           #RaynaudsFreeWorld
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: June 2018

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