tv Victoria Derbyshire BBC News July 4, 2019 10:00am-11:01am BST
hello, it's thursday, it's 10 o'clock, i'm joanna gosling... patients‘ lives are being put at risk because of delays giving them treatment for sepsis — that's the warning as new figures show that nearly a quarter of patients aren't being treated quickly enough. so, it's difficult for health professionals to spot it first time, every time. what they do need to do is to work in partnership with their patients, listen to their patients, and look for sepsis. and if they do that, most of the time it can be spotted. shortly, we'll be talking a mum — whose one—year—old son died when doctors misdiagnosed his sepsis. is pride too commercialised? thousands are expected to march through london this weekend — but is the event being hijacked by business in a cynical bid to boost profits? also, we'll be speaking to the family of a boy with a terminal disease.
they're accusing health bosses of using max as a bargaining chip after they refused to fund potentially life—saving drug therapy. where do you have to go today? school. and do you like school? yeah. do you like school? yes. yes, you do. because you have lots of fun at school, don't you, max? hello... welcome to the programme. we're live until 11 this morning. are you guilty of using your mobile at work to update your social media or text your mates? one company boss has banned his employees from using their personal phones apart from at lunchtime. they're asked to hand them in at the start of the day and they get them back at the end. what do you reckon? would you be happy with this? and how much time do you actually spend on personal stuff at work? do get in touch on all the stories we're talking about — use the hashtag victoria live.
if you're emailing and are happy for us to contact you — and maybe want to take part in the programme — please include your phone number in your message. if you text, you'll be charged at the standard network rate. first, julian has the news. joanna, good morning. a bbc investigation has suggested that patients‘ lives are being put at risk because of delays in treatment for sepsis. it's estimated that 50—thousand people in the uk die from sepsis every year, but the disease is called a hidden killer because it is so hard to spot. hospitals are meant to put patients on an antibiotic drip within an hour when it's suspected, but figures show a quarter of patients in england are waiting longer. we'll have much more on this in our progamme shortly. the two tory leadership candidates have been challenged urgently to bring forward plans to tackle the social care crisis, if they become prime minister. borisjohnson is calling for a cross—party approach to the issue, whilejeremy hunt has said more money is needed for care, after a committee of peers called for an immediate £8bn cash injection and a move to a free,
nhs—based, system. both candidates are campaigning ahead of ballot papers being sent out in the election this week to decide which man will become prime minister. the children's commissioner for england, anne longfield, has called for the next prime minister to provide more funding to help vulnerable children. new research from the children's commissioner's office estimates that one in five children living in england are growing up in difficult situations. it also says that more than 800,000 children aren't getting support and are being let down by services. the government of gibraltar has seized a supertanker suspected of breaking sanctions against syria. a detachment of royal marines from 42 commando joined the operation to board the vessel, called grace one, in the early hours of this morning. they'd been flown out from the uk specially at the request of the gibraltar authorities, who said there was reason to believe the ship was carrying crude oil
to a blacklisted refinery. china is warning the uk not to interfere in its domestic affairs after the foreign secretaryjeremy hunt threatened "serious consequences" if freedoms in hong kong are watered down. tensions between the uk and china have been rising since mr hunt backed demonstrators during large pro—democracy protests in the territory. beijing's ambassador in london has accused the uk of having a "colonial mindset". commercial passenger flights are returning to carlisle lake district airport for the first time in more than 25 yea rs. scottish airline loganair will connect the cumbria airport, owned and operated by the stobart group, with london southend, belfast city and dublin. the first flight, to dublin airport, took off this morning after a blessing from the archdeacon of carlisle. andy murray will make his highly anticipated return to wimbledon today when he competes in the men's doubles. murray, 32, missed last year's
singles with a hip injury, which he feared could end his career. frank lampard is returning to chelsea football club as manager. lampard, 41, has left championship side derby county to take over at stamford bridge where he spent 13 years as a player. he says he wants to work hard, bring further success to the club — and cannot wait to get started'. that's the headlines, now back tojoanna in the studio thank you. in the uk, 52,000 people lose their lives to sepsis every year. this is more than breast, bowel and prostate cancer combined. sepsis is triggered by infections in the blood. it worsens when the immune system goes into overdrive and can cause organ failure and death. hospitals are meant to put patients on an antibiotic drip within an hour when sepsis is suspected but figures obtained by the bbc show that nearly a quarter of sepsis patients at hospitals in england haven't been given antibiotics, within this time frame.
dr ron daniels is founder and ceo of the uk sepsis trust well, sepsis is not always easy to spot. it can arise in someone of any age, it can arise as a consequence of any infection. so it is difficult for health professionals to spot it first time, every time. what they do need to do is to work in partnership with their patients, listen to their patients, and look for sepsis. and if they do that, most of the time it can be spotted. we can speak now to melissa mead, whose one—year—old son william died when doctors misdiagnosed his sepsis. and john mccarthy who went to hosptial after getting an inocuous bump on the leg — which he lost after developing sepsis and we hope thatjoint can join us shortly. melissa, thank you for joining us, your one—year—old son william died, it must have been absolutely horrendous for you and yourfamily. absolutely horrendous for you and your family. tell us what happened. william was one—year—old when he
died, in the period five weeks before his death we'd been back and forth to the doctor on numerous occasions because he'd had a cough. we were always sent a weight saying it was a viral cough and in the 36 hours before he died, he deteriorated significantly and we took him straight back and he was displaying some of the red flag symptoms of sepsis but simply, the doctor wasn't thinking about sepsis, he sent us home and he didn't tell us he sent us home and he didn't tell us anything about sepsis or what to look out for, or fort worth looks like. when you say the red flag symptoms, obviously those symptoms that at the time you were completely unaware of? exactly. iwas ignorant because i didn't know what sepsis was. seemingly these insignificant symptoms and isolation are not worrying but together, they are catastrophic and i think as ron just said in the piece before, if i was telling the doctor that william wasn't right, that means he's just
not right and i should be listened to as his parent, as a person that knows him. so there was an inquiry into william ‘s death and the report said that if there'd been a medic, even at the last moment when you called up the nhs iii even at the last moment when you called up the nhs 111 system, if a medic had been on the other end of the line he might have been saved but as it was, it was a call, someone answering but as it was, it was a call, someone answering the call he was going through inputting questions into a computer. yes, that's right but even after that, we spoke to another doctor who was a trained physician. and he completely didn't put two and two together, we talked about the symptoms and he just said the best place for him was at home in bed. and he completely didn't think about sepsis at all. when you here today in the news that obviously, so many people are dying of sepsis and the response in
hospitals is still not adequate, how do you feel? disappointed. i feel really sad for the people that this is going to affect. i mean, ultimately hospitals do know what to do when someone has sepsis or when someone presents do when someone has sepsis or when someone presents with suspected sepsis but in order to treat sepsis, you first got to think about it and that's the problem, we've got to get those health care professionals on the front line to think it, suspect it and treat it and that's where the disconnect is. it's now, your son died in 2014, we are at five years on. how much do you think has changed since then in terms of the awareness and the thinking first about sepsis? i think there has been significant strides in sepsis in
treating sepsis and suspecting it. 0bviously treating sepsis and suspecting it. obviously we are seeing cases, the numbers rise but that doesn't necessarily mean there are more cases, it might mean the public presents much more quickly, they are listening to it, they know about it, doctors are treating it and diagnosing sepsis which we weren't seeing several years ago. but four yea rs seeing several years ago. but four years ago, if i'd gone out and given a talk about sepsis, not one of the people in the audience had heard of it. and now, when i go out and give a talk about sepsis everyone puts their hand up saying, i have heard of it but very few can explain what it is or what it looks like and hopefully, you know, as time goes on we'll be in a better position, it's snowball effect. what do you say and someone says snowball effect. what do you say and someone says i know what it is, i'm in the same camp, i know what it is but i don't know what the signs are to look out for so what would you tell people, what advice? certainly i would go on the nhs website, the sepsis trust website to have a look at the signs and symptoms but if you've got a loved one are you
yourself are suffering from normal infective symptoms, the symptoms that william had were lack of urine output, he had a very high but also a very low temperature towards when he passed away. he had quite cool hands and feet. and he was quite clammy. and he wasjust hands and feet. and he was quite clammy. and he was just very lethargic, difficult to rouse. but again, in adults, it can present slightly differently so i would a lwa ys slightly differently so i would always say to people go on the sepsis trust website and you know, be informed so you know what to look for. the symptoms that you describe their sound serious and would obviously be great cause for concern whether you think it might be sepsis or not but how early did those signs kick in, what were at the very first science? he was displaying the symptoms the day before he died. 36 hours before he died. he was displaying those symptoms when we saw the doctor. the difficulty was
that there was an element of human error in william ‘s case. so not only was william, the basic observations weren't carried out with william, the doctor didn't even suspect it at all. so you would hope that had we seen a doctor that might have taken his temperature, might have taken his temperature, might have taken his heart rate, his respiratory rate, all of those would have been alarming at that point and that doctor might have been able to send us straight to hospital for treatment. but simply put, he wasn't thinking about it so he didn't then look for the signs of it. there is also the case that once people get very aware of symptoms and get overly worried about things and jump to conclusions, what would you say about how vigilant people should be, when it is the time to get concerned and speak up and say i think this is sepsis? it's really important to get
the message and education right. 0bviously, the message and education right. obviously, we don't want hundreds of thousands of people to get to accident and emergency with a high temperature because not everyone with a high temperature will suffer from sepsis. it's about getting the education, helping people to make more informed decisions about their care, what they are experiencing. making sure we get the most sick people to the right places, people that have infections should be going to the gp but those that are suffering with sepsis symptoms need to be going to accident and emergency are ringing 999. melissa, stay with us, we can talk tojohn, i mentioned he went to hospital after getting an innocuous pump on the leg, that it developed into sepsis and he lost his leg. john, thanks for joining and he lost his leg. john, thanks forjoining us. tell us more about what happened. basically, iwas forjoining us. tell us more about what happened. basically, i was on a day out at cheltenham races, or my family and friends, it's an annual sort of event. during the day i knocked my leg and it was one of
those sort of feelings, i knew that it was going to be sore the next day and it was a couple of days later it got even worse. the skin started to get moles, there was a rash developing and i was starting to get the flu or at least what i thought was flu. this got worse, the following day i thought there is something wrong here and i knew instinctively, intuitively, something wasn't quite right sol got a friend of mine to take me to my local accident and emergency. i packed an overnight bag thinking i am going to be kept in, i went to a&e, i was there an hour and a half, they took x—rays, asked questions, don various things and the doctor said to me, you have cellulitis, here's an antibiotic, take that over the next seven days and see your gp. just as i was leaving the hospital he drew a crayon mark above where my right knee used to be and he said if you're rash develops and goes above that mark, then come back and that
was the one thing that stuck in my mind, the carjourney, i was feeling a little bit silly, i was thinking, i was overreacting thinking i was going to be captain but at the back of my mind was this crayon mark that was strong on my leg. the following day was worse, the leg was starting to seep and friends and family said go to the hospital, said we've got to give these antibiotics time. the next morning a puddle formed in my bed where my right foot was, i felt ifi bed where my right foot was, i felt if i was going to put weight on my leg it would snap, i went to the hospital, this time i entered accident and emergency in a wheelchair, my brother put me in, i was on a waiting room for about one minute when a nurse came over and spotted the puddle at the bottom of my foot. and i went, the same doctor who treated me the first time, a different response, what i recall this time was that i got put on a gurney and then got took through, put on a ward and it was three months later i left hospital. i'd had my right leg amputated above the
knee, suffered multiple organ failure, very lucky to have survived. it is so frightening hearing you talk about what happened to you and how quickly that too cold. what have you learned about it since because she said initially, the doctor said it was cellulitis. talk us through what happened in the sense of the medical progression of what was going on with you. with myself, the one thing i look at is that there are six signs of sepsis, it's your skin models, you don't go to the loo for a day, breathlessness and this is where your neck can constrict, extreme shivering, confusion. all six signs i was exhibiting when i presented to the hospital on the first occasion. now if i'd known about those signs i could have asked could it be sepsis andl could have asked could it be sepsis and i probably would not be sitting here now talking to you with the
amputation. unfortunately, none of us amputation. unfortunately, none of us need the signs and i think when i we nt us need the signs and i think when i went into the hospital, accident and emergency is busy, doctors aren't quite looking for the signs of sepsis. and it was missed. to be honest, it was missed. when i went back again it was probably missed again. it was left much to lead, they never got the antibiotics and to be in time and when i've come away, and it took me about a year afterwards, this happened three years ago, about a year afterwards to come to terms and realise that this is a national problem, i wasn't alone and i wasn't isolated. what i try and do now is create that awareness in the public, so this doesn't happen, so it's at the forefront of your mind and at the forefront of your mind and at the forefront of your mind and at the forefront of all medical professionals, nurses, gps, doctors and a&e so we can stop these 52,000 deaths happening every year. and a quarter of a million people such as myself. lots of people getting in
touch, claire said my son died from sepsis on the 5th of december aged 23, the only warning signs were he felt a little unwell, not enough to be alarmed and visit the hospital. my be alarmed and visit the hospital. my heart is broken. june says what is so terrifying about this cruel condition with its rapid deterioration and it could so easily be just flu, no time at all really for a clinician to diagnose, i'm so sad for your heartbreak. another viewer says it needs more staff and resources and the service has suffered because of funding cuts. gary says can't even get an appointment for my doctor, when you're told, you are told we have no appointments. melissa, john saying he is campaigning in the same way you are to raise awareness, what, for you, is your measure of success, what do you want to achieve? for you, is your measure of success, what do you want to achieve ?|j think, asjohn what do you want to achieve ?|j think, as john does, what do you want to achieve ?|j think, asjohn does, i go out and i speak to health care professionals and the public. i think a measure of success is that someone would message you and say, because of you are something you've done the work
of the charity, might son ‘s life, he still has his life. that is really, really important because what we are able to do is give people the gift of life by knowledge because you know, with sepsis, knowledge really is power. thank you both very much. melissa and john, thank you for your time. still to come. we'll hear from the father of max — an eight—year old boy who suffers from a rare fatal illness — he's accusing health bosses of using his son as a ‘bargaining chip', after they refused to fund drug therapy that could prolong his life. and is pride becoming too commercialised? as central london prepares to become awash with rainbows this saturday, some say the event is being hijacked by business in a cynical bid to boost profits. it's highly likely that someone in your family needs extra help — maybe your grandparents or your parents.
but an ageing society and the number of those living with debilitating and degenerative conditions, such as dementia, has put a huge strain on resources. today, the conservative leadership contenders borisjohnson and jeremy hunt have been challenged to spend billions of pounds on social care in england. here's our political guru, norman smith. norman, social care is so often the thing that comes up and people say we've had enough of talking about brexit, social care is the pressing issue, so what is being said about it today? it's a huge issue, you are right. it's been bubbling along really for years and years and yea rs, really for years and years and years, we've had endless reports and enquiries and promises and manifesto commitments and bluntly, nothing has been done. both the tory leadership contenders have said, ok, we recognise it's a serious problem, we will make it a priority. today, we had a report that says we don't need another inquiry, but we need now is action. and this report by a group
of peers suggests basically that we should make social care, the care of the elderly, the disabled, the vulnerable, we should make it like the nhs. so it's free for your personal care, you'd have to pay for accommodation if you went into a ca re accommodation if you went into a care home or something like that but you get your actual care free and that will be funded by general taxation, just like the nhs. the big question though is will anything actually happen because you have to say, we've been round and round in circles on this and i wonder if we are actually beginning to get towards a tipping point because you just sense amongst many mps there is almost a sense of shame that we have allowed this to go on, year after year after year. the difficulty is that to carry through this sort of reform is a massive, gargantuan undertaking and would require huge amounts of political energy and time and determination and courage. and with brexit, you know, it's going to
bea with brexit, you know, it's going to be a huge issue whatever happens in 0ctober be a huge issue whatever happens in october the 31st. will any future prime minister really have that sort of space to push through this reform even though everyone is crying out for something to be done? what else are they saying they'd like to do? we are entering the end of the leadership contest and a lot of things are being thrown about. because this is a contest where the electorate is very small, only 150,000 tory party members, inevitably the contenders and pitch their appeal to those members and to date we have both meant making appeals which no doubt will go down very well with tory party members. borisjohnson very well with tory party members. boris johnson saying very well with tory party members. borisjohnson saying there should be more bobbies on the beat and jeremy hunt saying he would allow another vote on repealing the ban on fox hunting. interesting jeremy hunt proposal because that's a contentious idea and his pitch is that he is the tory party candidate who would appeal to a broader range of voters, fox hunting is not going
to help him because in pulling its hugely unpopular and you got the sense this morning that mr hunt was thrown back a bit. let's listen. the law is not going to change on fox hunting, there isn't a majority in the house of commons and i don't see theirever being one. the house of commons and i don't see their ever being one. i was restating the position in our manifesto from 2017 that there should be a free vote. if it ever looks like that majority would change but it would be my priority as prime minister, we've got brexit, the social care system which had been talking about, huge economic priorities that are going to have to be addressed. as for boris johnson, he's reiterated his idea of recruiting an additional 20,000 police officers. that would make up for the number cut under david cameron and theresa may and he said particularly he would put police officers in rural areas were therefore been steeper cuts. but you are kind of left with the thought that although fox hunting and bobbies on the beat probably go down
well with tory voters, the issue of social care probably matters to many more ordinary voters. norman, thank you very much. just got another comment to bring it on sepsis, from agp comment to bring it on sepsis, from a gp recently retired and a&e doctor david. he said when working recently in a&ei david. he said when working recently in a&e i frequently saw treatment, generally due to the failure of the triage nurse recognising an incoming patient by ambulance is usually given intravenous paracetamol if they have a high fever. this lowers they have a high fever. this lowers the fever and the patient arrives feeling somewhat better. it's always documented in the ambulance handover but often ignored, i would regularly see a patient with a long delay in the circumstances usually labelled unwell. i got to fear seeing this triage diagnosis. the father of a boy with a terminal disease is accusing health bosses of using his son as a ‘bargaining chip' after they refused to fund his
potentially life—saving drug therapy. max sewart is a happy and social eight—year—old boy from somerset. but he has batten disease — an ultra—rare illness which roughly 40 children in the uk sufferfrom. it will very quickly rob him of his eyesight, speech and finally, his breathing. in a moment we'll speak to max's dad — who wants access to a drug made by an american company, but which nice — the health body which licenses medicine here in the uk — says is too expensive and has uncertain long term effects. first let's hear a little from max — we spoke to him and his brother felix before they went off to school this morning along with their mum ivana who helped max with the interview because of his worsening condition... what do you like now, max? what do you like? catboy. catboy? who's the fastest creature in the whole world? catboy. yeah. is it a catboy? and who's the catboy? me. max is the catboy. me, too. and felix. do you, like, run really, really fast? ican do... do you like running really fast, max and felix? i can do it really fast. do you? watch me! felix is doing a demonstration. and who is your brother, max? felix.
and is he the best brother in the whole world? do you like having fun together, max? yeah. you have lots of fun. and you like playing. what else do you like? boats. you like boats. where did you go yesterday? on a boat. 0na boat? i love going on coaches... especially when. .. and you went on a coach as well, didn't you? wasn't that good fun? we can speak now to max's dad, simon sewart... and health economist karl claxton, was is also a founding member of the nice appraisal committee, and he was involved in developing methods at nice. thank you both for coming in. simon, it must be so tough dealing with the situation that you are in with the condition that your son has? it's extraordinarily tough to have a diagnosis of batten disease, it's awful. it's a horrendous disease, to
them, having had that diagnosis four months ago to actually spend the last four months expending all our energy fighting the decision by nice and nhs england not to fund this incredibly successful drug which will stabilise his condition has been exhausting and the toll on our family is showing. it's a really rare condition, i think one or possibly a couple of kids diagnosed every year, possibly a couple of kids diagnosed every yea r, 40 possibly a couple of kids diagnosed every year, 40 in this country so tell us more about the condition and what will happen over time. tell us more about the condition and what will happen over timem surrenders, children start of healthy, parents think they have a healthy, parents think they have a healthy child, at the age of two or three they start getting seizures, they start losing the ability to talk, walk, they lose their eyesight, they go blind. they lose the ability to swallow, they have a feeding peg directly into the stomach and eventually they lose the ability to breathe and they die about the age of ten or 11, normally. you said you've only got the diagnosis four months ago. max
isa the diagnosis four months ago. max is a typical, he has a slower progression of the disease so it will end at the same way without treatment but it will happen slower, we don't know how slowly, but slower than a classic kind of case. that a drug that you mentioned that you've been fighting to get funding for, what difference would it make, is it life prolonging, potentially life—saving? life prolonging, potentially life-saving? the key is to get on it really early, as soon as you are diagnosed. if you are a sibling because it is inherited, if your sibling as the disease they may need to get on the drug really quickly before they have any symptoms. so just to interrupt, you have felix as well. he's fine, luckily, we had to wait three months for that but he's 0k. lots of families have two or three children with the disease. so we can clearly see in the child, —— at the trials, many of them are stabilised, their condition, their
siblings particularly, some of them are symptom—free whereas at the same age their older siblings are not in great shape. when you know the difference it can make and you're told you can't get it, how you feel? so, clearly, once you research and you realise how great this enzyme is, likei you realise how great this enzyme is, like i said, we spent four months to this point fighting for the disease, we feel like we've been led on a not so merry dance by the national health service england, they've kind of dangled the carrot ofan they've kind of dangled the carrot of an individual funding they've kind of dangled the carrot of an individualfunding request they've kind of dangled the carrot of an individual funding request for max, they've encouraged us to do that, they know it's going to take time, during that time axis declining and on tuesday we had the news that was declined by the national health service england. i've been on television quite a lot over the last four months and i've tried to keep my composure and might my manners and i've done that
because of a deep belief in british justice and british values and i believe that matt hancock and simon stephens will hear me and which are my faith and my belief in those values and thatjustice and when we heard on tuesday that it had been declined and i realise we have been played a game by the national health service england, i feel that's happened. i've kind of going to lose my composure a bit and i feel com pletely let my composure a bit and i feel completely let down particularly by people in power who have promised to help us, matt hancock promised to help us, matt hancock promised to help us, matt hancock promised to help us and he hides behind his office, he said he has no legal power, he's the head of the department of health, he has power, if he doesn't have power he can make power to do it. let's bring in coral. 0ut power to do it. let's bring in coral. out of these decisions taken, who has the power?
let me be clear, there are reasons to be outraged at this, that outrage should not be directed at nice, who try to do the best job they can, it should be elsewhere, particularly because there is a solution. nice does an excellent job at assessing all the evidence and assessing all the cost and benefits. the cost of this is half £1 million per year, per patient. is there a threshold at which point nice says, that is too much, we will not consider it? what is the most that will be considered pain? the way that nice works is that when it comes to rare drugs, it will think about 100, 200, possibly £300 per quality adjusted life year gained, that is a measure of health which combines the impact we will have, on length of life and quality of life but it is the job of nhs england to make sure that new drugs,
if approved nice by don't do harms to the nhs. £500,000 in the nhs, we have good evidence of the impact, thatis have good evidence of the impact, that is at least three additional avoidable deaths, it is 12 years of survival... when you talk through it like this in terms of statistics and money, was there ever any chance that nice was going to fund it? they have given very clear indications that there was a strong chance. i'm not here to speak for nice, but in terms of their methods, as they currently are, there was very little chance this was going to be funded, unless the manufacturer was willing to offer confidential rebates which would make that affordable for the nhs, in the sense... i can see you are getting frustrated. in response to what carl is saying and to the processes , to what carl is saying and to the processes, first of all, we need to
save this drug is available in over 20 countries in europe, when they make their appraisal of these drugs, economics is not a key factor in that. they look at it, and we look at this, to do research in rare disease, is incredibly risky, it is difficult to get a successful trial. that is why these drugs are so expensive. they cost as much money to make as any other drug, at the end... it is the research and development cost? yes, and they can only sell it to 16 children in england, so, they are selling it, they have offered the nhs less than they have offered the nhs less than the list price of 500,000 but let's assume they sold it at that, that is assume they sold it at that, that is a turnover of £8 million a year. if you look at that, if you look at a drug company selling a rare drug for £10, toi drug company selling a rare drug for £10, to1 million patients a year, they are making a turnover of £10 million. which is the greedy
company? basically, carl says he is anchored with the drug companies, you are... no, no, i'm not angry... the cost for them to develop and research that drug is huge, same as any other drug, they can only sell it to 16 patients in this country. in terms of moving forward, what happens now, have you any further records? no, we haven't, in england, the appraisal system in my opinion is not fit for purpose, i believe that it is not fit for purpose, i believe thatitis is not fit for purpose, i believe that it is a false economy, these drug companies are no longer going to come to england, to do rare disease research, they will go to germany, italy, these countries where they know at the end of the research, the drugs will be bought by the health service, and children will be looked after. we need to get back to the core issue, i'm watching my son die. there are other children dying, there are children have died waiting to get access to this. lots of children die a horrific death,
mothers, fathers, sisters, brothers, have watched this, because there was no treatment for this, now we have a treatment, it works brilliantly, it may only be for two or three years because there is gene therapy trials which would be a cure for the disease. suddenly we have this, it should be celebrated and it is not, it has been denied, and children are dying, my son is dying. that is the key thing we need to look at. the appraisal system looks at economics, does not look at the impact on the family and the devastation that it causes. i cannot describe it, it is really difficult. another parent has got in touch, to say, as the mother ofa got in touch, to say, as the mother of a child who sadly passed away at the age of seven, with cln2 i find it beyond heartbreaking that this drug is not being provided, it is horrendous having a child that is perfectly normal, to watch them from the age of three slowly and painfully degenerate in front of
your very eyes, it is truly torturous, the decision has to be turned around asap, time is the enemy. best wishes. nhs england is the only country in the whole of europe, that sees it this way, every other country understand a rare disease is going to be expensive, cost a lot of money to develop, they consulted too few patients. am i right in thinking you would consider moving abroad? we don't know if that is possible, if we live and work in holland, we have access to the health care system, we are paying our way there, that is not an easy thing to do, it has taken a long time to get max happy at school and give him the support to settle him in. to do that is going to be horrendous for the family, it is not an easy thing to do and we don't know how possible it is. there is a chance he will not get access to this enzyme, and this is a real injustice, as far as i'm concerned, i will not stop fighting even if he
doesn't get access and draws his last breath in our arms as we hold him,i last breath in our arms as we hold him, iwill last breath in our arms as we hold him, i will still keep fighting. simon stevens hides behind his policies, and his appraisal system, matt hancock hides behind, "i don't have any legal powers", that is unbelievable. if max was their son, this would not be happening, i know that. maybe that is something you could ask them. we do very much appreciate you coming in, your family are beautiful, we saw them earlier, we wish you all the best. thank you, simon, and thank you, carl, and thank you for your comments, viewers, very welcome to sendin comments, viewers, very welcome to send in your comments, the usual ways of getting in touch. this saturday will see the pride parade in london taking centre stage with thousands of the lgbt community and allies descending on the capital. but some people are unhappy that
away from the rainbow flags and sequins — the event is being hijacked by businesses. even downing street has been keen to get in on the action. the normally sombre—looking front door has been given a multi—coloured floral flourish in recognition of a pride reception. so has pride become too corporate? we can talk now to peter tatchell, who's been campainging for lgbt rights for years, harry gay from lgbt homeless shelter the outside project, rhammel 0'dwyer—afflick from pride in london who are organising saturday's event, and emma meehan from the lgbt foundation who get funding from corporate sponsors. lots of rainbow and colour here, peter, the original pride in 1972 was attended by 2000 people, a lot to celebrate, but you have concerns around it as well. there is a general consensus in the lgbt community that right knee some kind of corporate sponsorship to pay for
it, but i think there is some concern that it is getting out of control and the event has become too commercialised. big culprits are the only one to come forward, putting forward extravagant, expensive floats, they dominate the parade and community groups are overshadowed. there is also concerned that while undoubtably, some businesses definitely do great work supporting their lgb plus staff and customers, others perhaps also see this as a marketing opportunity. in part it is all about pr and branding for their particular product, and that, again, is against the principle of pride. -- lgb+. is against the principle of pride. —— lgb+. when you talk about pr and branding, do you suspect rainbow washing, at all, in some of this, that the message does not necessarily align with behaviour? i will give you an example, some while back, marks and spencer introduced a special rainbow lg bt
back, marks and spencer introduced a special rainbow lgbt sandwich... what was in that? laughter they decided to give money to a very good lgb+ charity but the amount was derisory, if they were sincere about the commitment, they would have promised to give all profits on that toa promised to give all profits on that to a charity. it is better than nothing. yes, it is better than nothing, but when companies support pride, they really need to back it up, and a lot of it feels quite tokenistic. this is an issue that a lot of people are increasingly feeling concerned about. harry, peter was saying about the floats, the big corporate ones, sponsoring the big corporate ones, sponsoring the majority of the floats, is there room for everyone? there isn't room for everyone, and there is also the financial aspect. i work for a grassroots homeless organisation, i will spend the rest of the year running a homeless shelter for the
lgbti+ community, we always look to the guest and see what they want to do, they are at the centre of everything we do and for them, being visible, marching in pride, is important. we spent £500 on the float, because we used to run the shelter out of the tour bus, we wanted to take that on the parade and have it like a centre so people could hear about the service we are running at pride. and then we got confusing things back from pride in london, saying that we should make it colourful, make it look appealing, have the guests in matching outfits... just two, sort of, brand it up, and make it visibly nice. the reality is it is a homeless shelter and also the reality is we don't have enough money to spray paint the bus. —— just to sort of brand it up. what do you say, sounds like style over substance, you are from pride in london. it is important that we recognise that in order for us to move forward globally, notjust for
the country, that private companies need to play more than a passive role in moving things forward. before we talk about the other corporate brands, the message to harry's charity and what they were doing, obviously it is a charity that does really good work, spending limited money on the float, they wa nted limited money on the float, they wanted to be part of it and the message came back, maybe it is not bright and buzzy enough.|j message came back, maybe it is not bright and buzzy enough. i can't comment on the specifics, i don't get involved specifically on the operational side of things but what i would say is that everyone is welcome, in the sense there are people walking without floats, we don't dictate to people what they have on their floats, we have a code of conduct where we expect people to stay within that, we don't want people to be derogatory to other elements of the community, etc. we wouldn't prevent someone, i don't know of anyone preventing somebody
because it was not bright enough. i would say, there are people who have left the parade for whatever reason, in past years, and come back, and i would hope at some point they would feel comfortable to come back, it is important we have all elements of our community as part of this. we talk about culprits and their roles. there are large culprits involved but more than 60% of people involved are nonprofits, unions, small businesses, maybe even individuals who want to come out and make a statement. i want to bring in emma, lg bt foundation you statement. i want to bring in emma, lgbt foundation you are from gets funding from corporate sponsors, how do you see the commercialisation of it? as peter has said, it is about the investment brands are making and whether that is backed up by other meaningful action. lgbt foundation with relationships with a number of corporates, smirnoff have allowed us to expand our community safety
programme into soho, to put volu nteers programme into soho, to put volunteers onto the streets of soho to make sure lgb people are having a safe night out. if friends can show that it safe night out. if friends can show thatitis safe night out. if friends can show that it is notjust about sticking a rainbow flag on something for the months ofjune and july and there is a desire to invest in social change to make the country a better place for lgbt to make the country a better place for lg bt people, to make the country a better place for lgbt people, we should welcome brands wanting to become part of that wider network of allies. how would they do that, you mention the sandwich and the proportion of profits being too small. one of the issues is, the city authorities impose an artificial limit of 30,000 people in the parade. last year, 50,000 people wanted too much, 20,000 were turned away. that is com pletely 20,000 were turned away. that is completely against the spirit of pride. in the 1990s, over100,000 people marched, nowadays, the city of london has imposed draconian limits. plus, they slapped pride
with huge charges, so westminster city council demands money for road closures, barriers, suspension —— pride. of parking bays. a victim of its own success? know, that is not imposed on the anti "brexit" march, the march for the nhs, pride is being singled out. and that is why pride it is being., to some extent, to go to the city authorities are punishing them financially and exploiting them financially. —— pride why is being forced, to some extent. westminster are crushing pride financially even though they say that they are lgb friendly. the other role that culprits play in pride is, is actually in order for us pride is, is actually in order for us to make this an accessible —— corporates is to make this accessible. things like providing bottle for the thousands of volunteers who are going to be out there in the heat, they need
something. that is controversial, of a plastic? they are not, and it is to avoid exactly that, so people do not bring single use plastic bottles. things like the family area, not bottles. things like the family area , not everyone bottles. things like the family area, not everyone wants to be in the ground, we need a family area thatis the ground, we need a family area that is more quiet and, safe, for some families. we need a viewing platform for those who have accessibility needs and might want to see billy porter on the main stage and cannot be among the crowd. all of that cost a significant amount of money. we do have to provide some costs to show that the event is as safe as possible but it also helps us go the extra mile. event is as safe as possible but it also helps us go the extra milem new york, we went to the reclaim pride march and there were no corporates, no police, self organised, joyous, fabulous parade, in the true spirit of stonewall 1969. i don't think all these add—ons are necessary. 1969. i don't think all these add-ons are necessary. we are out of time... the reality is that we have
an area promoting in the community and in orderto an area promoting in the community and in order to do that it cost money. promoting bame in the community. would you do reclaim pride here? i would like it to be reorganised, pride has hundreds of volu nteers reorganised, pride has hundreds of volunteers that do a fantasticjob, i would like to bring attention to them. —— pride. do you remember back in 2000 the explosion on—board the russian nuclear submarine, the kursk which caused it to sink to the bottom of the sea? 95 people died in the explosion, but 23 survived and were trapped in the submarine. the royal navy immediately offered to help, but russia were resistant and by the time the british team were ready to go down, it was too late. the story of the rescue attempt is portrayed in a new film starring colin firth. he plays david russell, the real life navy officer who headed up the british rescue team. in a moment we'll be talking to him, but first let's take a look at clip from the film.
let me be frank, we have the divers and the equipment to start bringing those meant to the surface in a matter of hours. we don't know their condition, i implore you to accept the help, if there are survivors we can bring them to the surface very quickly. i believe we have been very clear: when we reconvene, if your help is needed, we will let you know. we look forward to your call. david russell who was played by colin firth there is with us. how does it feel, watching you on screenplay by colin firth? quite surreal! i don't think anyone ever expect anything they have done in their life to be in a film, but then to be portrayed by colin firth... quite bizarre. it caused endless amusement in my family. i'm sure! you were, of course, involved in what was an extraordinary real—life
event, that is why it is being made into a film. the situation was com pletely into a film. the situation was completely shocking and tragic, talk us completely shocking and tragic, talk us through what was happening at the time from your perspective, when what you first aware of what was happening? i learned of the incident in the morning, monday, straight after the weekend, when it happened. we very fortunately had all our rescue assets in trucks on the way to portsmouth, we were going to do an exercise, in the mediterranean. everything was ready, instantly we said, we can help, we have a very capable submarine rescue system and will use a thing called nr—5, a submersible, we thought we should help and so we offered through the normal channels, the commander in chief, and by lunchtime, we offered help to the russians. the russians we re help to the russians. the russians were still not acknowledging what had happened. various stories coming
out through the media, "there's not a problem", "the problem has contained, caused by a collision with a western submarine", whatever it was. they were not being honest. you were aware that something had happened, explosions had been reported and you were aware that the clock was ticking. as always, as sub—mariners in particular, you know there is a submarine in those circumstances on the bottom of the ocean, very circumstances on the bottom of the ocean, very dark and cold, you have no power. the water is rising inside the compartment. a limited amount of time to do things. we can see a clip from the film which shows the moment that explosions happen, and to say while you are watching at home, you mightfind it while you are watching at home, you might find it upsetting.
152. say your prayers. i'm not a religious... shocking moment in the movie, did you... at the beginning you said you never expected, no one ever expects their lives to be turned into a movie but something like this, you know the outcome, we know everybody died. what did you initially think when you knew it was going to be turned into a movie, did you think this was a subject for a movie when the outcome is so tragic? it is very difficult to make a movie about something as tragic as this, the correspondence in moscow at the time, now in washington, wrote a
very good book, called a time to die, and that is what the book is —— microfilm is based on. it is not a happy ending, but there is a lot to be gained from watching the movie, it isa be gained from watching the movie, it is a very well done movie. by thomas vinter berg. it tells the story in a very sympathetic and a very true and honest way. —— thomas vinterberg. the secrecy, from russia's end, something we talked about, even this week there has been about, even this week there has been a submarine disaster, 14 crew members have died from toxic fumes, after a fire, russia is saying it will not be releasing details of the submarine and what the mission was, there is still secrecy, around that. different this time around. do you see echoes of kursk the and what happened then? at the time, it was their most modern and powerful
submarine, and undoubtedly full of lots of secrets. 0ne submarine, and undoubtedly full of lots of secrets. one of the reasons people think they were reluctant to accept help, they were worried about those secrets. they also think it was to do with pride, accepting help from the west. the current event, the submersible, that has had a fire on board, and 14 men, sadly, have died, has probably been something highly classified, and i think the interviews and releases from the russians make it clear that yes, they have acknowledged, this time, there is a problem and people have died, but that is it, they are not going to release anything more. david russell, immortalised by colin firth, thank you very much. kursk: the last mission is released in cinemas on 12th july william hill is closing around 700 branches in the uk, this is breaking
news, placing 4500 jobs at risk. significant fall in gaming machine reve nu es significant fall in gaming machine revenues after the government introduced changes to the way they operate. william hill closing 700 branches are you so are you guilty of posting updates on social media and texting your friends when you're at work? i was caught completely red—handed their... i was not looking at twitter, i was working!
well, some bosses have had enough. a director of a marketing company in west yorkshire confiscates phones during working hours and has also ruled out recruiting candidates who refuse to hand over their phones. in a recent video advert for a new social media marketer, he outlined what he isn't looking for in a prospective we are looking to get somebody involved who does not have a psycho boyfriend, texting you! involved who does not have a psycho boyfriend, texting you 1 million times a day, driving you mad, and driving me mad. so, the person we are looking to recruit is going to have a partner that is quite happy for them to actually get a job and work between nine and three, monday to friday. we also don't want someone to friday. we also don't want someone who is addicted to their phone, so, if the thought of you putting your phone down for three seconds and working for a company that only allows you to put your phone back on at 12 o'clock in your break times, this job phone back on at 12 o'clock in your break times, thisjob is definitely not for you. not for you whatsoever. what we are looking for is someone who is a complete winner, who wants to succeed, who likes to have a laugh, but does not take the mickey.
i don't frown on phone work if they are using the company phoned to do my work. i think they are pleased that they are not constantly updating social media, friends and fa ns updating social media, friends and fans on instagram, snapchat, twitter, facebook, they can actually doa twitter, facebook, they can actually do a job. we implemented this policy on the basis that they allowed phones on their desk but let's face it, it is an addiction, it became too much of a risk for them to do it and we were constantly telling people, get off your phones. it was annoying me, they were constantly replying to text messages from boyfriends, etc. i don't think it is
unreasonable for a boss to ask someone unreasonable for a boss to ask someone to work for them between the hours of eight and 12, they get their phone back and for a full hour they can do what they want, then they can do what they want, then they can do what they want, then they can come back to work. not unreasonable. it gives a better worklife balance, actually. unreasonable. it gives a better worklife balance, actuallylj unreasonable. it gives a better worklife balance, actually. ithink it sounds quite controlling and patronising, the idea my boss would be telling me, handing in my phone, it is like going back to school, worse than school. if people can't be trusted, then... research suggest that if you trust your employee, hire good people and trust your employees, treat them like adults, people will work like a grown—up and will get things done. if some of them... if somebody is constantly on them... if somebody is constantly on the phone, that is because they are a problematic employee, if you are someone a problematic employee, if you are someone who takes the mick, you will do it with or without a phone. what company do you do it with or without a phone. what company do you run? i am a freelance journalist, i am a sole trader. we
don't have time for this because we have so little time left on the programme. you don't employ anybody. i manage people. you are saying, you pay the wages, you have made this point online. what it is is, it sounds controlling, but when you are looking at a situation where people are spending 90 minutes a day doing their personal phone, as opposed to working for an employer... we are out of time, we have got to go, thank you forjoining us, we all have work to do, be like jarrod, not like me, don't go on your phone as much. —— gerard. was started the morning with lots of sunshine, further north, a bit more cloud and that cloud is producing a bit of rain across the far north—west, that is the south—west, you can see cloud edging its way further south, turning
sunshine a bit hazy, towards northern parts of england, throughout the afternoon, the rain really continuing in the north of scotland, particularly the north—west. further south, temperatures getting up further south, temperatures getting up to 24 to 26 celsius, further north, a little bit fresher, 15 to 18 celsius. throughout the night, continuing with a bit of rain across the west of scotland and elsewhere, clear spells, temperatures not falling below that, into friday morning, and friday itself, more sunshine expected across mainly southern areas of england, temperatures even higher, and further north, cloud around, 18 or i9 further north, cloud around, 18 or 19 degrees.
you're watching bbc newsroom live. it's 11 o'clock and these are the main stories this morning: the bookmakers, william hill, is shutting nearly a third of its shops in the uk, putting 4,500 jobs at risk. the firm blames the government's clamp—down on fixed odds betting terminals. jeremy hunt and borisjohnson are challenged to prioritise the crisis in social care if they become prime minister, as peers call for a free, nhs—based system. patients' lives are being put at risk because of delays in giving them treatment for sepsis, research by bbc news has found. royal marines in gibraltar board an oil tanker, suspected of transporting iranian crude to syria in breach of sanctions. in a rare interview, the scientist, james lovelock, warns about the rise of robots, saying humanity could soon be