Fibrodysplasia Ossificans Progressiva

Jim Shannon Excerpts

Thursday 7th September 2023 Commons Chamber

[Jim Shannon (Strangford) (DUP)]

It is a pleasure to follow the right hon. Member for Hemel Hempstead (Sir Mike Penning) and the hon. Member for Blaydon (Liz Twist). I thank them for their contributions, particularly the right hon. Gentleman for setting the scene.

FOP is an extremely rare condition, but it is important that the matter is aired in the Chamber and the right hon. Gentleman has done that well. He is probably right that the relevant Minister is not here. That is no disrespect to the Minister who is here—we know that he is an honourable person, who will respond positively within his remit. If another Minister needs to take the matter on board, we look to the Minister who is here to make the case persuasively to them, and hopefully we will get a response.

When the right hon. Member for Hemel Hempstead asked me whether I would come along to support him, I automatically said that I would, because the debate is about a health issue, health is one of my portfolios and I wanted to understand the condition. I have always had a particular interest in rare diseases. Long before I came here, I was a member of the Northern Ireland association for rare diseases. I am therefore particularly interested in the subject.

I will be honest: I knew little about FOP. I had to research it, and the right hon. Member for Hemel Hempstead gave me some pointers. It was so interesting to read about it and to learn how rare it is and how little we know about it. FOP is an exceptionally rare genetic condition, where soft tissue develops into bone, creating a second skeleton. The right hon. Gentleman illustrated the condition clearly when he described the bruise, the bump, the jag or the discomfort. Around 70 known individuals are diagnosed in the UK, and the disorder has been described as impacting “one in a million.” That gives an idea of the rarity of the condition.

The House is tasked with highlighting the issue and raising awareness, and I hope that the debate will do that. As far as I am aware, there are only two known cases in Northern Ireland, and they are twin sisters. I do not have their permission to name them, and I would not do that, but there has been public information about the case.

[Sir Mike Penning]

My hon. Friend has touched on the really important point of the lack of diagnosis. If we know that one in a million children will be born with it, the calculation for the world population is pretty obvious. We are nowhere near identifying the numbers that should be out there. In my constituency and in any other hon. Member’s constituency, there are probably children who have been born with the condition. However, the length of time it takes to get a diagnosis—because the test is not part of the programme—is the most important thing. The condition can be tested for if there is a will.

[Jim Shannon]

The right hon. Gentleman has made a simple request about diagnosis. Perhaps awareness can be raised of the simple test, which it is so important to do. I understand that the twins in Northern Ireland are rightly keen to raise awareness of the issue.

Such rare diseases are often ignored. Most people—including me before this debate—have little or no idea what it is or, more importantly, what we can do to raise awareness. But today we can use our position as MPs to highlight the issue, with the co-operation of the Minister and the shadow Minister. I cannot for a moment imagine what it must be like to grow up with a condition about which there is little or no information, not to mention what it would have been like when the twins were children, when there was no cure or treatment for the condition.

Early and correct diagnosis is key to changing the life of someone suffering with FOP, as the right hon. Gentleman said. This debate is a request for hope and for progress for our constituents. The purpose of this debate is funding, with the hope of a potential trial of the existing drug Saracatinib, originally developed by AstraZeneza as a cancer drug. The underlying issue is that if the STOPFOP trial is not completed, the money spent on it will be wasted. Given the progress of trials and the advancement in medication, it is right that every effort should be made to try to find that money to ensure that the investigation into that treatment takes place.

[Sir Mike Penning]

Like all clinical trials and all things in health, there are other things the trial could help with. I am not a scientist, but it has been put to me that while we are looking at clinical trials into FOP, there may be help for osteoporosis—brittle bones—and skeletal damage, particularly that suffered by the military. Even though we are talking about a tiny percentage of the population with FOP, the population with brittle bones is huge. It seems that there is very little cure for it apart from taking calcium tablets. If we get the principle right on what is causing the bone growth, perhaps the research can be extended past FOP and we can help millions of people in other areas.

[Jim Shannon]

The right hon. Gentleman is absolutely right. Whenever trials take place there are always benefits, although perhaps not the intended ones. None the less, the focus can be larger, whether it be brittle bones or whatever. What was originally a cancer drug has been found to be beneficial to those with FOP.

As initial funding for the trial is running out, the main asks are to ensure that additional funding is allocated, while ensuring that secured funding extends to allow the trial to include children, especially the screening of new-born infants—as the right hon. Gentleman has referred to. Many have said that early and correct diagnosis is key to changing the life of someone with FOP, so I cannot imagine how the trial could not extend to newborns and extremely young children.

Raising awareness is how we will improve treatment for the condition. I have been made aware that there are only three knowledgeable FOP clinicians in the whole of the United Kingdom of Great Britain and Northern Ireland. That leaves patients often finding themselves treated by doctors with little or no FOP knowledge, which is rather disappointing, but focuses attention on those three clinicians. Like other conditions, patients must battle to be heard. Being aware of what to look out for is crucial: shortened or turned-out toes in young children raise concern, but if combined with tumour-like swellings, FOP is almost certain. It has also been raised that many patients are given biopsies and misdiagnosed with cancer. Others have had limb amputations, which perhaps was not the right way forward, but a response to not being quite sure what the problem is.

Having better access to a wider pool of experts would make a huge difference in diagnosing and treating people correctly. The charity FOP Friends, based in Oxford, is fantastic at supporting families. I have also seen some of the social media pages of parents of children who have FOP. Their work is absolutely incredible.

[Sir Mike Penning]

I thank the hon. Gentleman for giving way—thank goodness we have plenty of time to debate this issue. As he alluded to, there was a petition on social media, which was signed by well over 100,000 people. The Government’s response—I should have mentioned this to the Minister—is that they have funded research into FOP, but I am afraid that does not appear to be the case. They have funded research into rare diseases, not FOP. That is probably crucial when it comes to the public’s belief in what we do in this place.

[Jim Shannon]

The Minister took note of what the right hon. Gentleman said, so I have no doubt that there will be a response. The Minister has a genuine interest in the subject and I hope we will all be encouraged by what will have been said.

The parents are the main drivers of the campaign and the effort going into it is truly incredible. Many different people are making an effort with FOP Friends, whether the families, clinicians, those involved in clinical trials, ourselves as Members of Parliament, and, ultimately, the shadow Minister and the Minister.

To conclude, I thank the right hon. Member for Hemel Hempstead again for raising this issue with us today. He speaks so highly of his constituents. He does it all the time, by the way, but he did it again today. He has indicated to me that he is not running for Parliament again. We will miss his constant and compassionate commitment, interventions and speeches in this Chamber. He does not always do what his party wants him to do, but he always does what is right and that is what I admire about him as an individual.

It is important that we do all we can to help those with this condition to cope. We must do more to fund research into this trial. I sincerely look forward to hearing about developments in the future. To give those with FOP a better quality of life just like the rest of us, we need the Government and the Minister, from whatever Department, to help deliver just that.