JCW raise £30k at Annual Black Tie


Daniel Evans

On Friday January 3rd 2020, people from across the global JCW Group came together for our annual Black Tie event at The Ned in London. 

One of the biggest dates in the JCW calendar, we celebrated the year's top performers with our annual awards and we had a number of outstanding sales performances that smashed previous company records.

We also celebrated the collective business growth as we grew our headcount by 30% and company performance grew by 90% year-on-year. 

But it was the charity raffle and auction that was the highlight of the evening.

A company-wide raffle and a competitve auction at the event saw us raise an incredible amount for charity. 

The generosity from everyone across the business was incredible. A big thanks to everyone for helping us celebrate an incredible year with such a memorable evening.

The JCW Group raised than £30,000 on the evening for the Myotubular Trust!

The term myotubular myopathy is commonly used for the x-linked form, whilst the term centronuclear myopathy is mainly reserved for the other forms.

The commonest form is x-linked myotubular myopathy, affecting mainly boys, and is also the most severe. It usually presents in the newborn period and there are associated breathing and swallowing difficulties in addition to the general muscle weakness. The other autosomal forms are either dominant or recessive in inheritance, are usually (but not always) milder and vary widely.

In normal muscle development the central nucleus of a muscle cell moves to the outside edge of the cell. In myotubular and centronuclear myopathy the nucleus remains in the centre of the muscle cell, hence the name for this group of conditions. The central nucleus can be seen under a microscope when a piece of muscle is examined, and therefore the first step in diagnosing the condition is to have a muscle biopsy. This is usually confirmed by DNA blood analysis to identify the exact gene mutation.

In x-linked myotubular myopathy the protein myotubularin which is present in normal body cells, is either missing or severely reduced.  In the other autosomal forms of centronuclear myopathy different proteins are affected, such as dynamin 2 and amphiphysin 2. The role of each of these proteins in normal muscle development is not fully understood yet, but when they are affected by these genetic mutations, the muscles don’t work as they should. Sometimes this weakness is apparent immediately from birth and sometimes symptoms may be more apparent in later childhood or adulthood.

As well as having general muscle weakness, many of those affected by x-linked myotubular myopathy in particular, need mechanical ventilation to stay alive and need to be fed straight into the stomach via a feeding tube. Many are medically very fragile and need nursing care 24/7. Put very simply, a cold can kill someone affected by myotubular myopathy as their cough and swallow function is so weak. Sadly many do not survive their first year of life.

Find out more about the Myotubular Trust here.

Some photos from the evening:

View more photos of our Black Tie event on our Facebook page here.