Published45 minutes agoShareclose panelShare pageCopy linkAbout sharingImage source, Philip AlexanderBy Catherine SnowdonBBC NewsPaul Alexander was six years old when he woke, terrified, to find himself inside a large metal tube, with only his head sticking out.He couldn’t move to feel what was trapping him, and when he tried to call for help, he discovered he couldn’t make a sound.Paul had survived a serious bout of polio, but had been left quadriplegic. After an emergency tracheostomy operation, he was unable to breathe without the iron lung machine that now encased his small body.When he died recently at the age of 78, Paul had spent more than seven decades using his iron lung, longer than anyone else in history. Image source, GoFundMeBut what was it that marked him out from so many of his peers, and kept him going?Polio ran riot in the late 19th and early 20th centuries, killing and maiming scores of children.In hospital in Texas in 1952, Paul was surrounded by other children in similar predicaments. According to the World Health Organization, one in 200 polio infections leads to irreversible paralysis. Among those paralysed, 5-10% die when their breathing muscles become immobilised.Uncertain futureAfter two years in hospital, doctors began to cast doubt on Paul’s future.That led his parents to make the brave decision to take him and his iron lung home, to live out his days in peace. But instead of dying, Paul went from strength to strength, once he was home in his parents’ care.The iron lung uses a negative pressure system. Powered by a motor, its bellows suck air out of the cylinder, creating a vacuum around the patient’s body and forcing the lungs to expand and take in air. When the air is let back in, the same process in reverse makes the lungs deflate. The device needs a source of energy to function.During power cuts, the bellows had to be pumped by hand; neighbours would come to help with the job. Paul’s father designed an alarm bell that Paul could ring using his mouth if he needed urgent attention. Over time Paul learnt to consciously breathe by gulping down air, using his throat muscles to force air into his lungs. He called it frog-breathing.His younger brother, Philip, explained to the BBC that the promise of a puppy gave Paul the incentive to be brave, and attempt to spend time out of his iron lung to learn the complicated glossopharyngeal breathing technique, the medical term for frog-breathing.”He was scared of course of choking to death,” says Philip. “They told him if you last three minutes, you’ll get the dog that you want.” And he did.Image source, Rotary Club of Park Cities DallasAs his confidence and strength grew, he was able to spend increasingly longer periods out of the iron lung. This allowed him to begin to experience a bit more of life. He was allowed to venture into the neighbourhood in his wheelchair with childhood friends, returning to the iron lung when he was tired.”He was just a normal brother to me. We fought, we played, we loved, we partied, we went to concerts together – he was just a normal brother,” says Philip.Paul finished school at home and went on to earn a college degree before setting his sights on law school.Image source, University of TexasPhilip recalls Paul’s time at the University of Texas in Austin as being “incredible”. His parents helped Paul move in with his iron lung, and then he was on his own – with limited help at first, after the carer he had hired failed to show up.”He didn’t really have any caregivers. He was in the dorm and different people just took care of him accidentally. They pushed him around campus [in his wheelchair],” says Philip.Paul went on to practise law in downtown Dallas. He had to deal with the surprise of clients on entering his office and seeing him in his iron lung.”It’s not an easy thing to see, just a head sticking out,” says Philip. “People immediately go into shock. I saw that happen a lot.”Paul lived alone for much of his adult life, no mean feat for someone unable to take care of his basic human needs like using the toilet or getting a drink on his own.This video can not be playedTo play this video you need to enable JavaScript in your browser.Philip says he became the master of his own domain, helping people to help him.”He needed a unique type of care. Not even professionals are trained to take care of a quadriplegic in an iron lung,” says Philip of the huge responsibility that came with supporting his brother.”Most of the care was basic – shaving and feeding for example. But to move him you needed to be careful not to jam his finger and so on.” There was no instruction manual for those who answered Paul’s adverts for caregivers. “They learned as they went,” says Philip. “And many left after a day or two. I remember going around once and asking a couple of assisted living centres if they could care for him, and the looks on their faces were priceless.”Paul had one carer who was in his life for decades. When Kathy Gaines died, her passing left Paul bereft.Philip says he always saw himself as the back-up carer for his brother, but he admired the support system Paul built: “He had a lot of wonderful friends, some really beautiful people in his life.”Help neededOne of those people came into Paul’s life in a moment of dire need.In 2015, his iron lung began to leak. As the machines were by now extremely rare, it was a race against time to find someone able to carry out the vital repair job to make it airtight once more.After a plea was posted on social media, responses came in from around the world. But the solution turned out to be much closer to home.Ten miles down the road from Paul’s apartment in Dallas, was the mechanical durability business Environmental Testing Laboratory, owned by Brady Richards.Mr Richards had come into possession of two iron lung machines at a building clearance and he recalls how one day a paramedic walked in asking: “Is this the place I can find iron lungs?”The medic had been transporting Paul to and from hospital, as carers fought to keep him alive in the failing iron lung. As the situation became more desperate, word of mouth had brought her to Mr Richards.Image source, Brady Richards”I did not have any idea about Paul Alexander at that time,” says Mr Richards. On learning about the emergency, he immediately set about rebuilding one of the broken machines he had in his warehouse. He built some parts from scratch and scavenged others from other devices, learning as he went along: “Iron lungs are very robust machines. They are built to last. “It’s a simple machine so I managed to work it out. You can increase and decrease the breathing rate and the pressure. Paul always liked the settings up high.”Once the refurbished machine was ready, Mr Richards swapped it for the damaged one at Paul’s apartment. He wouldn’t accept any money for his work.But that wasn’t the end of the story. Later that night Mr Richards received a call from Paul’s carer to say the machine wasn’t working.Mr Richards returned to Paul’s apartment and quickly realised the neck collar wasn’t on properly and had come loose.”Paul was saying ‘I’m fine, I’m OK’ as we worked to correct the problem,” recalls Mr Richards. “The truth was, he was turning blue.”In moments of desperation like these, Paul had access to more portable breathing devices, which used a different sort of technology to the iron lung.”Positive pressure respirators can make the patient feel like they have their head stuck out of the car window. Not everyone likes that sensation” explains Dr Patrick Murphy, the clinical lead consultant at the Lane Fox respiratory unit at St Thomas’ Hospital, in London.Paul never moved full-time to these mask-based devices. Another polio survivor, living in York, in northern England, did make the switch.Image source, James PorteousJames Porteous, 78, was infected with polio in the same year as Paul. He was initially placed in an iron lung. He recovered to a point where he could live his life without significant breathing support, but as is often the case for polio survivors, as he aged he began to need more help. He now uses a respirator mask for about 17 hours a day: “I don’t remember much about being in the iron lung. I was also placed in a head-to-toe plaster cast initially as the thought was that this would prevent my limbs from becoming deformed,” says Mr Porteous, who is the regional president of the British Polio Fellowship. Mr Porteous had a long career, first in stockbroking and then in various senior roles for Rowntree’s, which became part of Nestlé. He married and has four daughters. He travelled the world and in 2001 he was awarded an MBE for services to the community.Image source, James Porteous”I could have sat all my life with a rug over my knees, but I decided to just get on with it. I’m tired these days and I don’t get out much any more, but life is good. I have a nice family and good friends. One thing I’d still like to see achieved in my lifetime is the complete eradication of polio around the world,” he says.Ending polio globally was also Paul’s ambition. He wrote about it in his 2020 memoir, which he typed himself, using a pencil attached to a stick gripped in his mouth to reach the computer keyboard.Philip says it was after the book was published that he fully realised what an inspiration his brother was to people around the world: “His personality had a lot to do with how much he was admired. He had that great big smile and he was such a welcoming, warm person. He made people comfortable.”Brady Richards remained involved in Paul’s life over the years. He helped Paul move apartments when needed, and serviced the iron lung regularly: “It was always a pleasure to be around Paul – he had a very upbeat and positive attitude.” Image source, NHSDr Murphy is full of admiration for the people who cared for Paul.”His parents took their son home in a machine that was at the time relatively high-end technology. He also had a tracheostomy, which calls for special care. They will have needed to be engineers, nurses and doctors. “If you speak to lots of trained doctors or nurses they won’t be confident in managing a patient with complex respiratory failure, and yet patients like Paul and their families do it at home on their own. The bravery cannot be underestimated,” says Dr Murphy.World recordLast year Paul was recognised by Guinness World Records as the person who had lived the longest in an iron lung.Philip’s admiration for his brother runs deep: “I saw him go through a lot of struggles in his life. I’m going to miss him. I called him when I needed to talk to someone about any kind of problem I had.”He says that while the rest of the world might be amazed by how long his brother survived in his iron lung, his parents would not have been: “They believed in him. They gave him so much strength and love. They wouldn’t have been shocked.” More on this story’Man in the iron lung’ Paul Alexander dies at 78Published13 MarchSuper-engineered vaccines created to help end polioPublished14 June 2023What is polio and how can you protect yourself?Published10 August 2022

Published2 hours agoShareclose panelShare pageCopy linkAbout sharingImage source, Hayley BeanBy Catherine SnowdonBBC NewsI have always been proud of donating blood. I have a relatively rare blood type (B-) and recently found out my blood is even more precious to the NHS, because it can be given to newborn babies.At my last donation session, the donor carer who was about to put the needle in my arm asked: “It must feel great being a Neo?” My baffled face prompted her to show me the bright blue tag that was waiting in the bowl to receive my bag of blood. Neo was written in large font on it. “Your blood is special, it’s going to help the tiniest of patients,” she explained.Neo stands for neonatal, which is the term used to describe a baby in the first 28 days of life.Image source, NHSBTAs my blood was collected I had a speed lesson about how blood is tested after donation. It turns out some patients – including infants – need specific blood. I wanted to learn more so I spoke to Dr Andy Charlton, a consultant in haematology and transfusion medicine at NHS Blood and Transplant.He explained that all donated blood is screened for HIV, hepatitis B, C, and E, as well as syphilis. Once that has been done, further tests and processes are carried out on some samples to ensure they are suitable for patients who have specific requirements. For instance, some people need blood that has been “washed” to remove proteins they have previously had allergic reactions to during transfusions. Common virusBlood that is destined for new babies, immunocompromised patients, pregnant women or to be transfused into a foetus in-uterine must be screened for a virus called cytomegalovirus or CMV. Part of the herpes virus family, it is very common and usually harmless, causing mild flu-like symptoms or none at all. But for some people it can be serious. In babies it can cause seizures, sight and hearing problems as well as damage to the liver and spleen. In rare cases it can be deadly.Estimates vary but it is thought that between 50 and 80% of adults in the UK have had CMV. As only about 2% of the eligible population in England currently give blood, finding enough donors who have not been exposed to the virus is crucial for supplies.The blood I donated the previous time was tested and came back clear of antibodies for CMV, meaning I had not been exposed and received the special tag. My blood will be tested for the virus every time I donate, to ensure I have not caught it in the interim.Immunity to the virus lives forever in white blood cells so if I ever catch it, my blood can no longer be given to these vulnerable patients.I am one of only 10,916 active donors in England who has CMV-free, B- blood. Over the last year 153,801 units of CMV negative blood products were requested by hospitals.Dr Charlton says demand for “specialised blood components” is increasing and urges people to come forward to donate.”We can’t thank our donors enough,” he says. “Every donation of blood is a gift of life and can save more than one person.” LifesaverNo-one understands the importance of blood donation better than Hayley Bean. Her daughter Willow’s life was saved soon after birth by a transfusion of CMV-free blood.Image source, Hayley BeanDuring pregnancy, Hayley was diagnosed with vasa previa, a dangerous condition in which the blood vessels from the placenta or umbilical cord block the birth canal.The vessels are at risk of rupture at any time and, because they obstruct the baby’s passage out of the uterus, natural birth is impossible. Hayley was admitted to hospital at 32 weeks for monitoring, and a Caesarean section was planned for 35 weeks.During the operation, Willow’s blood vessels burst, causing life-threatening bleeding.Image source, Hayley Bean”All the alarms were going off and people were running around,” recalls Hayley.”They got Willow out and I waited to hear that first cry. It was the worst moment of my life. She wasn’t breathing and had gone into shock. The neonatal team had to resuscitate her. After about 10 minutes I remember finally hearing a tiny cry.” Willow was taken to intensive care after a nurse quickly took a picture to show Hayley.”All I remember was how pale and swollen she looked,” she says.Hayley finally held Willow for the first time 12 hours after she was born.Image source, Heyley BeanWillow is now a thriving four-year-old, and Hayley is eternally grateful for the treatment her daughter received.”She was in intensive care for five days but there was no permanent damage, thanks to her getting that blood transfusion,” says Hayley.”She wouldn’t be here today except for the kindness of a stranger. Someone, somewhere made the choice to give blood, and it’s thanks to them that Willow is here today.”A few days after my first Neo donation, the text I had been waiting for came through. It told me which hospital my blood had been issued to. I smiled and wished the little one well.More on this storyGen Z and millennials encouraged to donate bloodPublished17 January’It’s my duty to give blood as often as I can’Published30 December 2023

Published29 minutes agoShareclose panelShare pageCopy linkAbout sharingImage source, Getty ImagesBy Catherine SnowdonBBC NewsWhen Vertex Pharmaceuticals developed a treatment for genetic condition cystic fibrosis (CF), it was hailed around the world as a “groundbreaking” moment for patients.For the first time, the condition – which often causes patients to die before their 40s, as mucus clogs and damages their lungs – could be treated at the root cause, by bypassing the genetic errors responsible for the disease.But these treatments are thought to cost the NHS well over £100,000 a year per patient. The newest of the so-called modulator drugs, Kaftrio, has proven effective for about 90% of CF patients – but with more than 11,000 in the UK, it would, at list price, cost the NHS more than £1bn a year.And last month, National Institute for Health and Care Excellence (NICE) draft guidance deemed the drugs too expensive.Patients will continue to have access to them during the appraisal process – but parents worry young children might not be eligible if NICE recommends withdrawing funding.Image source, Marc holding a packet of Kaftrio”Parents are right to question NICE, to make sure it has the right data to assess the drug correctly, particularly given that CF is a rare condition and that the benefits of early access might not be realised for many years or even decades,” Marc Cotterill, 41, who has CF, says.”But equally, we need to protect our NHS – its budget is limited. “We can sit here and point the finger at NICE and assume they’re trying to put a price on a life – but the problem is the price itself set by Vertex Pharmaceuticals. “This drug has to be affordable and sustainable. It’s not a one-off drug. Once on it, patients need it for life in order to enjoy the benefits.” Mr Cotterill says Kaftrio “saved” his life. And he cannot remember the last time his lung capacity was as good as it is since he started the treatment – currently, 80% functioning.Extra costOther countries around the world are also arguing the price set by Vertex Pharmaceuticals for Kaftrio is too high. The US, Dutch and Canadian authorities have called for large price reductions.But the company says it is “disappointed” by the draft NICE guidance and disagrees with some of the “key evidence that NICE has selected to use, which has a significant impact on the way that our medicines are valued, particularly underestimating the costs of caring for people with CF and the impact that CF has on a person’s quality of life”. A Bristol University study, released in January, showed the average extra cost of having CF was more than £6,500 a year.Image source, Just TreatmentPatent law has allowed Vertex Pharmaceuticals to carefully guard its monopoly of these drugs.But World Trade Organization agreements and national laws include flexibilities designed to ensure citizens’ right to life are not threatened by intellectual-property rights – and patients are challenging the company’s patents.In February, legal papers were filed with the South African High Court, seeking a compulsory licence that would allow the supply of more affordable generic versions of the CF drug. Petitions to revoke or suspend Vertex Pharmaceuticals’ patents have also been submitted to the governments of India, Ukraine, and Brazil.The company declined to comment on the legal processes in progress. Operating expensesResearch led by Dr Andrew Hill, of the University of Liverpool pharmacology department, found the basic production cost for these drugs was less than $6,000 (£4,790) a year per patient.But this does not include the significant costs involved in research and development (R and D).”The price of our medicines reflects their clinical value and benefits to patients, caregivers and healthcare systems,” Vertex Pharmaceuticals told BBC News. And list prices “published at the time of marketing authorisation do not reflect the final reimbursed price agreed with the local health authorities which is confidential”. “Each year, we reinvest over $1bn into developing new medicines for people with CF, and others, who today have few or no treatment options, dedicating three out of every five Vertex employees to that purpose,” the company said.”More than 70% of our operating expenses are dedicated to R and D, well above the average of the top pharma and biotech companies in the industry.”‘Hugely inflated’Understanding the price of developing a new treatment is hugely complex. But Diarmaid McDonald, the director of Just Treatment, which campaigns for lower-cost drugs for NHS patients and globally, says the industry should be more transparent. “We have no idea of the real cost of clinical trials or the other costs involved in developing these drugs – though industry figures on R and D spend are criticised as hugely inflated,” he says.”We also don’t really know what the NHS or other countries are paying for them or the details of the deals they are negotiating. “It makes it really hard to judge whether we’re getting value for money.”Expiry dateThe NHS says patients already on these drugs will continue to have access to them while the negotiation continues.But patients will nevertheless be worried about their future. Image source, CJ GUNTHER/EPA-EFE/REX/ShutterstockThose old enough will remember the last such process between NICE and Vertex, which involved Kaftrio’s predecessor, Orkambi, another CF drug. The negotiations lasted years and saw Vertex destroy nearly 8,000 packets of the drug, as they passed their expiry date before the price was agreed. Buyers clubDuring these negotiations, the NHS called Vertex Pharmaceuticals an “extreme outlier” among pharmaceutical companies because of its hard line trying to secure a high price. The government began discussing invoking what is known as a Crown use licence, which would have swept aside the company’s exclusivity rights to these drugs and allowed the UK to buy in generic, vastly cheaper versions of the treatment.In the end, Vertex Pharmaceuticals returned to discussions and a deal was finally agreed.But some patients, desperate for access to these drugs in the meantime, took matters into their own hands and created a buyers club, flying to Argentina to buy a generic version produced by a company there.In its latest financial results, Vertex Pharmaceuticals raised the amount it expects to bring in via CF product revenue to about $9.85bn. And these results were published before the announcement the UK Medicines and Healthcare products Regulatory Agency had extended the licences of Kaftrio and Kalydeco to two- to five-year-olds, which will increase the amounts needed for NHS patients.The second NICE committee meeting to discuss the comments submitted during the consultation period is on Thursday, with a final recommendation regarding NHS funding for these treatments expected next spring.Vertex Pharmaceuticals says it is “committed to working collaboratively” with the NHS and NICE to secure “long-term access” for new patients who may benefit from these treatments following the conclusion of the NICE evaluation.The company stresses that the NICE process will not impact access for patients who are already taking these medicines or who may start taking it in the near future.”Access for these patients will continue irrespective of the outcome of the NICE process,” says Vertex,More on this storyParents fear loss of cystic fibrosis ‘miracle drug’Published16 NovemberCystic fibrosis buyers’ club travels to ArgentinaPublished22 September 2019’Groundbreaking’ cystic fibrosis drugPublished17 May 2015