Newsletter #9

George Pantziarka TP53 Trust - Newsletter

Whole-body MRI

The only way to describe current access to whole-body MRI for people with LFS in the UK is scandalous. That could finally now be changing..

Right now it remains a post code lottery, with people getting it in some places and not in others. While it is improving in some places, in others it's exactly the same as it has always been - even though annual whole-body MRI is the agreed standard of care recommended for people with LFS.

We have been working with the NHS England Highly Specialised Services with a plan to roll it out across the whole country. This meant agreeing a service schedule defining what screening centres would have to provide, the costings (NHS tariff) had been agreed in consultation with radiologists. We had got to the stage where all of this was going to be published so that centres would be able to apply for the funding to provide the service. And then we had the general election and shortly after that everything stopped - completely.

First the sign-off for approving the call to tender was put on hold - we were told that no new spending was being approved, even though we had been repeatedly told that the funding was there. Then recently Keir Starmer announced that NHS England was being abolished. This came out of nowhere and seems to have taken everyone by surprise. We've been left totally in the dark, as have most people working in the NHS. It had taken us months and months of work and just when we thought we were making progress it had stalled and there was no sign that it would start up again.

However, after some intense lobbying behind the scenes we have just heard from NHS Highly Specialised Commissioning that the funding for this has been prioritised in '25/26. The procurement team is expected to pick this up again at the beginning of May, having missed our previous target of September '24.

While it's a delay, this is good news compared to so many other projects according to our source. Whole-body MRI is not a luxury, it's key to the right to life and health for people with LFS and we are grateful that the lobbying and pressure has given us this cautious good news. We will keep everybody posted if there are further developments.

MILI Update

We are happy to report that more centres are opening for the MILI trial - which is testing metformin, a drug used in type II diabetes, as a cancer prevention drug for adults with Li-Fraumeni Syndrome.

Currently there are seven centres able to recruit patients to the trial: Leicester, Southampton, Aberdeen, Nottingham, Cambridge, Guy’s Hospital London, and Oxford. Three more are in preparation: Manchester, Edinburgh and Northwick Park.

As a reminder, people recruited to this trial will receive either screening (including whole-body MRI) or screening and metformin. The trial is randomised, which means that people recruited are randomly picked for either the screening or screening + metformin arm. Randomisation is designed as the fairest way to test whether a treatment works or not. If everyone received the drug there would be no way of knowing whether it is working or not. The only way is to have two equal groups of participants so that if there is a difference in outcomes you can be more certain that it's down to the drug and not some other factor.

There is also a new MILI website which has got the latest site information on it. Also planned is a list of FAQs, which will answer the most common questions that people interested in joining have asked. Invitations to join the trial are being sent by the cancer genetics clinics at the MILI recruitment sites - they will be contacting all of their eligible LFS patients in good time.

If you are asked about joining - please keep in mind that regardless of which arm you are selected for, taking part in the trial will benefit you and the rest of the LFS community. We all want to know whether this drug really does reduce cancer risk or not - and the only way to do that is for large numbers of people to actively participate.

Learn more

What's new with us?

We'd like to say a big thank you to all those who volunteered to help student Natalie Wilson with her project on occupational choices in adults with LFS.

We look forward to hearing about the results of this project from Natalie at LFS UK 2025 in September.

Trust ambassador Chris Stevens, who we'll hear from below, is also involved in a community charity, called A Shining Light.

Set up in 2022 following the death of his son, Luke, A Shining Light raises awareness and funds for Teenage Cancer Trust. This year would have been Luke's 21st, so Chris and the team have planned 21 events throughout the year in his memory. From climbing the Yorkshire Three Peaks, to taking part in the York Dragon Boat challenge it's going to be a year to remember and you can donate below.

Donate

Our chairman, Pan Pantziarka, was at the ESMO Sarcoma conference and workshop to represent the patient perspective on sarcoma research issues.

During his presentation he took the opportunity to highlight that March 20th was International LFS Awareness Day.

Meet the Trustees

In this section we'll get to know one of our trustees a little better - this time we're speaking to Chris Stevens.

How did you get involved at the Trust?

I got involved with the Trust at LFS 2022, after my daughter Cassie was diagnosed with LFS. This followed the loss of her brother, Luke, in 2021 from an osteosarcoma. I've known I've had LFS since my mum passed away in 2007 and my brother and I were tested. At that time, certainly in Yorkshire, there was little understanding of LFS and we were advised that there was no real benefit to our two small children of having them screened. But once Luke passed away I wanted to ensure I had access and awareness of all the research and trials possible.

What does your role at the Trust entail?

As an ambassador, I make myself available as a sounding board for people who may be newly diagnosed, or wish to understand LFS more from someone who lives with it. I do have to say that there appears to be a lack of willingness for clinics to recommend patients to communicate with the Trust and the ambassadors, but hopefully this will change.

What do you enjoy most about working with the Trust?

The people and their passion. Everyone is so committed to increasing the awareness and improving the outcomes for those of us with LFS. I was honoured to have been asked by Pan to get involved with and speak at the LiNCR conference in Oxford last year. To listen to and meet with so many clinicians and experts with a shared goal to improve the lives of LFS patients was heart-warming, if not a little frustrating to learn that they all face the same bureaucratic obstacles in trying to do so.

What is your greatest accomplishment?

A cliche but raising two fantastic children. Cassie graduated from York with a degree in Business and Marketing and is now set on her career with Heineken. Though we only had Luke for 17 years, he lived a full life and I'm sure would have done well in whatever field he chose, which would likely have involved sport.

What do you do in your spare time?

My wife, Joanne, and I like to travel as much as we can. We also have a caravan which we've just pitched up in North Yorkshire for the year, so look forward to spending time up there with our two Miniature Schnauzers.

What are you reading at the moment?

I like to lose myself in action novels, usually when travelling, and love Dan Brown. He's been very quiet over the past few years, so I'm presently reading the Ben Hope stories by the prolific author, Scott Mariani.

New sacroma paper

LFS is now firmly on the agenda amongst sarcoma specialists.

A new paper has just been published in the journal eClinicalMedicine entitled 'Genetic predisposition in sarcomas: clinical implications and management'.

The paper, which includes our chairman Pan Pantziarka as an author, sets out the state of play as regards treatment for sarcoma patients with LFS and other cancer predisposition. It also stresses the need for future research to improve outcomes for patients and to plug the many gaps in knowledge that still exist.

Meanwhile, there is now a task force looking specifically at LFS and osteosarcoma working within the FOSTER (Fight OSteocarcoma Through European Research) consortium. Again, aimed at laying out what we know and don't know about LFS and this bone sarcoma, considered one of the 'core' LFS cancers. The Trust is playing a part here too, making sure that the views of LFS patients are represented and included in all of the work.