Monthly Archives: June 2021

Estimated reading time 9 minutes, 16 seconds.

As we approach the finish line of Canadas Future Fighter Capability Project (FFCP) the competitive process to replace the legacy CF-18 Boeing, Lockheed Martin, and Saab are scrambling to demonstrate why their proposals are the best fit for Canada and the Royal Canadian Air Force (RCAF). With capability, affordability, and economic benefits to Canada playing unequal, but important roles in the process, Canada must make the right decision to ensure the RCAF can continue to deliver on a proud tradition of excellence in tactical fighter capability today, and well into the future. But after years of covering this story, a common-sense solution has emerged; we can no longer afford the Lockheed Martin F-35. Allow me to explain.

Capability is the highest point-getter in the Request for Proposal (RFP), and many would argue it should be. All three fighters boast an incredible array of next-generation capabilities. An honest assessment of technical progress reports, historical timelines, and field-demonstrated performance will expose who is delivering, who isnt, and where the risks lie. The evidence in this regard is apparent and overwhelming.

Still, affordability is not considered by the RFP to be as important as capability; but the link is undeniable, especially in a post-COVID economy.

The last defense review Strong, Secure, Engaged determined Canada needed more fighter aircraft to protect its sovereignty and deliver on its defense partnership agreements than initially projected. That number grew from 65 to 88 fighters, and Canadas current competitive fighter replacement process must deliver this quantity of aircraft as a mandatory requirement. This is the point where the Lockheed Martin F-35 option could fall out of contention.

Canadas military procurement programs have a long history of being plagued by cost overruns and delays. But with no guarantees on costs, and numerous examples of allied nations abandoning their original F-35 order numbers, practical reality tells us the F-35 is simply unaffordable. The Dutch and British have cut their orders for F-35s nearly in half; the Italians by a third; and there is now credible talk of the United States Air Force (USAF) reducing its original planned F-35 intake by 40 percent. It appears the USAF has recently seen the value in upgrading proven and affordable airframes with the latest technologies with the introduction of the Boeing F-15EX and big talk about a next-generation F-16 effort that would fall short of any stealth capabilities. The United States Navy (USN), the second-largest air power in the world, has chosen to invest heavily in the Boeing Block III Super Hornet and will operate nearly three times the Super Hornets than it will the F-35C. The USNs massive investment and commitment to the Super Hornet will ensure it remains tactically relevant for several decades to come.

There is also no relief in operational costs with the F-35 option. Depending on your source, it will cost $36,000 per hour (on the low end) to operate the F-35, with an effort to reduce its cost per flight hour to $25,000 by 2025. Lockheed officials are on record indicating that this particular claim is using fiscal 2012 dollars, which puts you right back to where you started at $36,000 an hour using an average annual inflation rate of 2.63 percent.

The debate over the F-35s fiscal challenges or its tactical relevance vis-a-vis Canadas requirements are vastly overshadowed by its technical problems. In a post-COVID economy, it is simply unaffordable given the funding envelope of the FFCP. The economic benefits of this fighter replacement effort will matter now more than ever as Canada looks to recover from the new economic realities left behind by the global pandemic.

Of the three offerings, Boeing and Saab appear to be in a two-horse race to offer Canada affordable solutions that come with some significant economic benefits. Saab is the only company to offer a built in Canada, by Canadians solution. But there is little detail of what that actually means over the life of the procurement program. This would be the first time since Canadair manufactured the CF-5 Freedom Fighter that a fighter jet would be manufactured in Canada, which would be a significant win for the Canadian aerospace sector. However, given the small order of 88 fighters for Canada plus the (at most) several hundred Gripens that are projected to be in service worldwide would there be enough in economic spinoffs and job creation over the duration of the program for Canada when compared to, say, Boeing?

Working in Boeings favor is the fact that the USN is expected to operate at least 600 Super Hornets and Growlers for several decades to come.Several hundred more Super Hornets will be operated by other air forces around the world, which would potentially push the number of Super Hornets in service to more than 800. That could potentially mean more economic spinoffs for Canadian aerospace companies.

According to the economists at Ottawa-based Doyletech Corp, Boeing claims it will deliver $61 billion to the Canadian economy and create 250,000 jobs over the life of the project through a contractual guarantee.

Comparatively, Lockheed Martin will not commit to a contractual guarantee, but suggests the F-35 could yield $16 billion in economic benefits should its fighter be selected.

The Canadian aerospace sector needs support now more than ever, and the FFCP represents an important opportunity to provide that support.

So, where do we land? There is no question that all three fighter aircraft in the running would usher in a well-needed next-generation fighter capability for the RCAF. After covering Canadas fighter replacement program for more than a decade, the choice is becoming more evident given the attributes of the remaining contenders.

The passionate, and often heated, bar banter will continue about the technical merits of each contender, and what fighter aircraft is right for Canada. But when it comes down to dollars and cents, there are only two choices that provide a minimum risk to the Canadian taxpayer, and would generate solid fiscal support to Canadian aerospace for decades to come: the Boeing Block III Super Hornet and the Saab Gripen E. Each of their capabilities are as different as their economic benefits to Canada.

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Making dollars and sense of replacing the CF-18 - Skies Magazine

Touchline Talk

Inter Miami were unfortunate to fall 1-2 to Orlando City SC at the DRV PNK Stadium on Friday, June 25. Despite the result, the boys put in a spirited performance that had head coach Phil Neville praising the teams performance throughout the match.

I thought we were absolutely outstanding. I feel really proud of the team. I think when you talk about what you want to see from the team, this is exactly what I saw tonight, said Neville.

This level of performance within the squad is now expected to be the status quo for Inter Miami, according to Neville.

I think theres a level of performance, a level of attitude now thats non-negotiable. When we have that kind of attitude and we have that total commitment, you get the performance like we did tonight.

The players that are willing to run, the players that have the right attitude, players with energy, players that were hungry, players that would scream for us to spring back. Thats the level now that weve got to expect.

One player that truly demonstrated what Neville now expects out of his players is Blaise Matuidi, who he made sure to praise for his stellar midfield performance.

I did think that Blaise Matuidi was fantastic, I thought it was his best game in an Inter Miami shirtI think he performed at a level tonight that showed why he is a World Cup winner.

Neville especially praised Matuidis commitment and willingness to improve his performance level that culminated in a stellar night for the Frenchman.

Hes been so honest, took so much accountability in terms of his performance level that needs to be better. I always felt that it would be better because hes a top professional and he does things right every single minute of every day. Tonight I thought he and Victor [Ulloa] dominated the midfield.

Matuidi believed that he had a point to prove, not only to himself, but to the team as well.

I work hard in training, I am a player with experience so I knew I had to be better, so this night I wanted to prove that [for] the team and for myself, said Matuidi

Ultimately, Neville believed that the atmosphere within the stadium elevated the players and he hopes that the supporters can be proud of the effort of each Inter Miami player.

Nights like tonight, there was something special in the air. It felt like a playoff game Ive got to say. Every game feels like that here because [of] the atmosphere. I think if you were an Inter Miami supporter tonight, I think you can be massively proud of the effort that the boys put in. We just cant wait to play the next game against Montreal, concluded Neville.

Continued here:

Neville: I feel really proud of the team - Inter Miami CF

D.C. United took twenty-seven dang shots on CF Montreal, who had virtually nothing to offer as a reply, and yet walked off the field in Ft. Lauderdale with just one point, and zero goals. There are good things to talk about here: United has three straight shutouts, and has not conceded in 315 minutes. They created plenty of chances. But, you know...you gotta score!

Its tempting to just toss that paragraph out and literally thats the whole article, because everyone that saw the game knows why it was frustrating, but at this website we are nothing if not verbose. So, with that in mind, me (good morning its Jason, you probably knew that by now), Ryan, Adam, and a Special Guest are going to dissect this game as best we can.

They really should have! Nate Scott kind of, but actually Jason Anderson using his words because its the most salient point to make about this game

When a team takes 27 shots and doesnt score, usually that means the main striker had a bad night. And since were coming off of a game where Ola Kamara had a frustrating performance that caused at least some fans to pine for a new option up front (I dont really agree, but I get their argument), that sounds bad.

Well, lets look at who got the shots tonight:

This is really three problems in one. Obviously no one finished well in this game, so thats the big story that we already covered. Kamara wasnt able to get open often enough, which is another one, but to his credit he was often the runner occupying Montreals defenders (particularly on the looks Arriola, Perez, and Paredes had). United was also not able to manufacture chances cut out for Kamaras skill set, a problem they had last year when his channel-running style wasnt really ever put to use.

United will have to address all three of these, and theres not a lot you can do about them on the training ground. Tell them to practice finishing isnt really a cure when you think of how many shots (tens of thousands!) a professional forward has taken in their life up to right now, versus how many shots theyll take at training on Friday and Saturday (30 or so). The next couple of days probably wont make everyone demonstrably better at shooting.

The best training solution, and it might take more than just a couple of days, is to work on how to draw teams out of their lower blocks, and how to open organized teams up (i.e. not Miami) for the kind of service Kamara has tended to put away. Getting Kamara, who even after the Miami game is finishing well when you look at the data, a bigger share of the shots means getting more goals. JA

Adding onto Jasons point on chances, Ill Yes, and on a couple of things: there were three chances Wednesday that were dangerous; Perezs first-half chance, Arriolas shot at Clment Diop, and Julian Gressels box shot he took on the short hop.

Past that? Not a lot, and its borne out below:

So I think a couple of things are going on: a dusting of pressing (with the resulting shots outside the box) combined with hitting a lot of Montreal bodies (nine United shots were blocked), is creating a little angst, which some aforementioned fine-tuning can help with.

Another thing that would help is that under Hernn Losada, Arriola, Kamara, and Edison Flores have played together a total of zero minutes this season, and Arriola and Kamara have played together barely more than a third of the season. There should be a big resource for attacking quality coming back into the fold in the near future, so well see what United can do with its three most expensive attacking acquisitions out there at once. Ryan Keefer

United were playing on three days rest against a team that had been off for the better part of a month. And both teams showed it Uniteds starters faded from fatigue in the heat and humidity of Quebec South Florida, and the Impact Foot Boys (Garons de Foot? Can we just call them Les Garons?) lacked a rhythm.

For United, shots were off. Crosses sailed. Passes were imprecise. Players legs and feet just werent quite doing what they were telling them to. Los Capitalinos had 27 shots, and could have had more if theyd been less wasteful in turning promising attacks into shooting opportunities.

But, speaking as somebody with two young kids who knows from tired, the evidence isnt just physical. When youre tired, your decision-making facility fades at least as quickly as your physical abilities. Your thoughts and reactions get slower. And we saw so many times Wednesday night where a DC player is on the ball with a runner in space, and he stays on the ball for a touch or two too long, and then just keeps staying on the ball or passing it safely not to a more advanced player.

Losada could have been quicker on the draw on his subs. Maybe some additional squad rotation would have helped, and allowed him to bring some of his top-choice starters in to run at a tired, shorthanded defense. In any event, fatigue was a big story against the not-Montreal not-Impact, and well see on Sunday whether the sequel plays out against NYCFC. Adam M. Taylor

All of Uniteds substitutes in this game were attack-minded: A forward for a wingback, fresh legs in central midfield, an attacker for a wingback, fresh legs up front, and fresh legs up front again.

It didnt work, but United got major life from Griffin Yow, Yamil Asad, and Yordy Reyna. Yes, Montreal was fatigued, and anyone on either team that wasnt gassed was going to have an advantage. But the context here is what those three were able to do from a technical perspective: more success dribbling, more invention, better service.

Yow came back from injury a while ago and has only in the last two games cracked the gameday squad to get minutes. Asad has spent four straight games heading into this one in uniform as an unused sub. Reynas situation is more or less like Yows. All three have had a very hard time getting themselves into the mix so far, and weve just had to accept that in training the coaching staff has not seen enough.

So given their opportunity, this trio made a genuine difference, and that has real value. In particular, Reynas cleverness and technical ability might be a welcome addition up front, where the balance has been more towards work rate since Flores went down. Its not clear what Losada plans to do with the game against NYCFC Sunday, but hell surely be spending some serious time mulling over some rotation in the attack. JA

United took 27 shots in this game, and none went in. 27! Last year, per FBRef, United had 12 different stretches where they took 27 or fewer shots spread across three full games, and as bad and unlucky as last year was, only one of those spells a trio of games from September 23 to October 3 saw them fail to score.

This is the same sport where this same United team could play Montreal, back when they had a good name, take one shot in 90 minutes, and win 1-0. Maybe tonight was just the debt from that game coming due.

Im writing this after knocking back a couple of post-game beers, chatting with some friends, and listening to an episode of How Did This Get Made?, so Ive had some time to unwind. This game was obviously designed to torment us, because we all know that in soccer, if a team keeps shooting, at some point the ball finds its way in somehow. Its hard to score goals, but its not this hard!

But also, as we said earlier this season after some similarly frustrating games, the balls gonna go in the net. If United can replicate this level of pressing mastery and suffocate future opponents, and they can carve out this many shots, theyre going to win a lot of games. It sucks that they didnt win this game, but its still kind of wild to think that United has become a team that can torture us in this particular way, after years where 27 shots was like one months worth of work. JA

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Five thoughts on D.C. United not finding a way through against CF Montreal - Black And Red United

D.C. United had more than their fair share of chances, but had to settle for a frustrating 0-0 draw with CF Montreal tonight in a game played Montreals temporary Florida home. Despite attempting 27 shots, a lack of attacking precision throughout proved costly as the Black-and-Red let Montreal who had Zorhan Bassong sent off just before halftime esceape.

Coming off of a 1-0 win over Inter Miami on Saturday, Hernn Losada named an unchanged starting eleven. Both teams effectively pressed each other in the early going, making chances hard to come by. However, on a simple flick-on from Ola Kamara, United should have gone in front in the 15th minute. Kamara popped the ball up over the Montreal back three, and Adrien Perez raced into the box. Perez adroitly cut the ball away from the defense, but his shot was too close to Clment Diop to open the scoring.

Diop denied Perez again just three minutes later, glancing his powerful shot over the bar after Paul Arriola picked Perez out with an excellent pass from the right.

United were taking charge of the game, with Kamara putting the ball into an empty net after break upfield thanks to more good work from Arriola only for the offside flag to correctly call the play back in the 27th minute.

Just as the half drew to a close, Zorhan Bassong already on a yellow card for a foul ten minutes earlier committed an inexplicable handball to slow Perez down after the United forward flicked the ball past him. Referee Drew Fischer had little option but to pull a second yellow card, leaving the Black-and-Red with a man advantage for the second straight game.

Despite out-shooting Montreal 10-1, the United entered the locker room stuck with a 0-0 scoreline. D.C. created an immediate big chance to start the second half, with Arriola picking Kevin Paredes out with a perfect low cross to the back post, but with no defenders nearby, Paredes scuffed his shot. A similar sequence played out in the 52nd minute, with Julian Gressel getting past Mustafa Kizza to cross. This time Paredes got full power on his effort, but it cannoned off a Montreal defender, allowing the home side to scramble it away.

United had yet another excellent chance sparked by an Arriola turn at midfield in the 58th minute. DCU surged forward in a four-on-three, with the play moving to the left for Paredes. The United homegrown slid the ball back across, and with Kamara drawing defenders, Arriola was wide open, but his shot allowed Diop to extend his legs and make a kick-save.

The Black-and-Reds profligacy nearly cost them in the 67th minute, with Djordje Mihailovic curling a shot just wide after Victor Wanyama turned an innocent looking throw-in into a good piece of set-up play.

Still, most of the looks were Uniteds, but time and again a lack of precision cost them either in the build-up or on the shot. Losada used every attacking player he had in uniform off the bench, including Yamil Asad making his first appearance in over a month.

After a pretty convincing handball shout after one of those subs, Moses Nyeman, had a shot handled by Wanyama was turned down by Fischer, the chances started to pick up. An 85th minute header from an Asad corner seemed very promising, but Diop did enough to push the effort away, while Yordy Reynas slashing run saw him send Yows cross just barely over the bar in the 87th.

Diop, taking a stoppage-time goal kick, fell and required extensive treatment. Montreal considered putting forward Mason Toye in goal, but Diop soldiered on with a heavy limp, and United couldnt muster a shot to test whether he was up to make any difficult saves.

The draw wont feel good, but it does push United (4W-1D-5L) up into 6th place in the Eastern Conference. A draining week for United continues this Sunday, as they stay on the road. NYCFC will play host at Red Bull Arena, with the game set for a 6:00pm kickoff.

Box Score

MLS Regular Season - Game 10CF Montreal 0D.C. United 0

Lineups:Montreal (3421): Clment Diop; Aljaz Struna, Rudy Camacho, Zorhan Bassong; Zachary Brault-Guillard (Clment Bayiha 75), Samuel Piette (C) (Emanuel Maciel 66), Victor Wanyama, Mustafa Kizza (Kamal Miller 53); Djordje Mihailovic, Ahmed Hamdi (Sunusi Ibrahim 53); Bjorn Johnsen (Mason Toye 53)

DCU (343): Bill Hamid; Andy Najar, Brendan Hines-Ike, Donovan Pines; Julian Gressel (Griffin Yow 73), Russell Canouse, Felipe Martins (Moses Nyeman 67), Kevin Paredes (Nigel Robertha 67); Paul Arriola (C) (Yamil Asad 81), Ola Kamara (Yordy Reyna 81), Adrien Perez

Bookings:Montreal - Piette 19, Bassong 35, Bassong 45 (sent off), Diop 87, Wanyama 95+DCU - Paredes 59, Najar 63, Nyeman 71

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D.C. United lets 10-man CF Montreal off the hook in 0-0 draw - Black And Red United

Added on June 25, 2021Kurt VerlinGR Supra , Limited edition , Special Edition , Supra A91-CF Edition , Toyota Supra , u.s. market

The Supra may be one of the most iconic Japanese nameplates in the world, but Toyota isnt beyond creating a few special edition models just for its North American market. Every example of the new GR Supra A91-CF Edition will be sold only in the United States rejoice, JDM fanboys.

Of course, there wont be that many models to begin with. The special-edition Supra will have a limited run of 600 units, meaning it will be more likely to end up in a collectors garage than in the hands of a wide-eyed Supra lover who grew up watching too many Fast and Furious movies.

Still, no matter who ends up at the wheel, the GR Supra A91-CF Edition is undeniably cool. It has lots of exposed carbon fiber trim, unique 19-inch matte black wheels, and a red Brembo brake package. The interior is two-tone leather and Alcantara in black and red.

The best perk, at least from the perspective of someone who would want to track the thing, is the complimentary one-year membership to the National Auto Sport Association. Said membership entitles owners to a one-day performance driving event with expert instructors who can show you how to properly extract all that the Supra has to give.

Thats about it for the list of features. As for the 2022 GR Supra in general, its largely unchanged from the 2021 model, which last year got a substantial power increase. For the new model year, the GR Supra gets a newly available red interior and standard heated seats, but thats about it. If youre looking for a reason to buy a 2022 GR Supra over the 2021 model, the A91-CF Edition is it.

Kurt Verlin was born in France and lives in the United States. Throughout his life he was always told French was the language of romance, but it was English he fell in love with. He likes cats, music, cars, 30 Rock, Formula 1, and pretending to be a race car driver in simulators; but most of all, he just likes to write about it all. See more articles by Kurt.

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New Toyota GR Supra A91-CF Edition is Exclusive to the U.S. - The News Wheel

Rian Murphy was diagnosed with cystic fibrosis as a child andnever expected to live into his 30s, but Health Canada's recent approval of a new breakthrough drug treatmenthas theWindsor, Ont., dad hopefulhe'll be spending many more years with hisson.

"It's a massive step going forward for cystic fibrosis patients such as myself," said Murphy about the triple-combination therapy Trikafta. "At the end of the day, it's a big, big window of opportunity for us to look down the road, future-wise."

On Friday, Trikaftawasapproved for use in patientsage 12 and over who have aminimum of one of the CF F508del gene mutations.

Cystic Fibrosis Canada (CFC) calls Trikaftaa "transformational" therapy that couldtreat up to 90 per centof Canadianswith theprogressive, genetic disease, whichaffects the lungs and digestive system, and is the most common fatal genetic disease in children. TheCFCestimates one in every 3,600 children is born with the disease, and over4,370 Canadiansattend specialized clinics.

Over time, the CFC says, Trikafta could reduce severe lung disease by 60 per cent andthe number of deaths by 15 per cent,and increase life expectancy by several years, the CFC says in quoting research.Clearing the airways from mucus buildup is important in CF care.

In the last three years, half of Canadians who died of cystic fibrosis were under age 34.

"I never thought about retirement. Inever thought about those things because my whole life I was told you're never going to make it until you're 20, you're 30," said Murphy, who with wife Diane are parents to their year-old son Logan.

Three years ago, Murphy lost significant lung function andwas hospitalized for threeweeks at St. Michael's Hospital in Toronto. He hadbeen on and off intravenousantibiotics for months.

"On an average day, I'm doing about two to three hours of masks, and vests and physiotherapy, not including all the pills I take," he said.

"I'm 34 years old. If I can obtain this drug [Trikafta] and take it for the recommended period of time to get the results, it would be like me turning back the clock."

There's no cure for CF. While other therapies work to address the symptoms, Trikafta helps the defective protein function more effectively.

With Health Canada's approval, doctors can now prescribe Trikafta.

But aswith a couple of other drugs for CF, provincial insurance coverage for Trikaftaremains a concern for patients, Kelly Grover, president and chief executive officer of CFC, saidin a release.

"We turn to the provinces next. They must immediately fund Kalydeco and Orkambi, which have been in negotiations for more than a year, and fund Trikafta as soon as possible. Provincesend the wait and save lives."

The pan-Canadian Pharmaceutical Alliance (pCPA) isa regulatory body that negotiates drug prices on behalf of the provinces.

In astatement released shortly after Health Canada announcedapproval of Trikafta, thepCPAsaid it has agreed to negotiate prices for the CF drugsOrkambi and Kalydeco, and Trikafta might be added to the agreements, pending a positive regulatory and health technology assessment recommendation.

CBC reached out to the Ontario government for comment, and in an email, the Ministry of Health said the province "recognizes that the cystic fibrosis community is anxious for access to new and effective treatments such as Trikafta," and "will continue to work productively through the established review and pCPA processes."

In the meantime, Murphy and his wife have started a fundraiser, hoping to raise enough money so hecan eventually access the drug.

"If I can get a couple months, that's huge," he said.

His wife Diane, who'sactively involved with CFC, as well as a petition and Instagram groups calling for the Ontario government to fund Trikafta, encourages the public to send letters to the province.

Shehas hopes of her husband "watching our child grow up."

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'Like turning back the clock': Windsor dad with cystic fibrosis among patients seeking access to new therapy - CBC.ca

Let us know what you think and join the conversation at the bottom of this article

Jordy, who recently came out as non-binary and who was last years Young Scot Awards winner, was diagnosed with cystic fibrosis (CF) when they were two-years-old.

In February last year, the drag star, whose shows in previous years have centred on complications that come with having CF, was on a waiting list for a lung transplant.

But that same month, the 26-year-old started taking a newly-discovered drug Kaftrio on compassionate grounds to see if it would help.

Kaftrio is a three-drug combination taken daily which can treat 90 per cent of people with the genetic condition, and was released on the NHS in June last year.

Now, about 18 months on from taking their first pill, Jordy said the drug has been life-changing.

They noticed a small cough develop within three hours of taking the medication, which continued for several weeks, bringing up mucus that can collect on CF sufferers lungs.

Its a miracle, they said, still sounding amazed by the improvement in their health.

I went from puffing, constantly, especially after doing my drag shows, to being able to run up the stairs with ease.

For more than two decades of their life, Jordy relied on a PEG tube - a feeding tube installed into the stomach to allow fluids and nutrition into the body, bypassing the mouth - to keep weight on.

But, thanks to Kaftrio, they have been able to have it removed this week a rare procedure, Jordy said, for someone living with CF.

I was so nervous about getting it done because I have had this tube my whole life, its helped keep me alive, they said.

But I am the healthiest I have ever been and, I cant believe I can say this but, I dont need it anymore to maintain a healthy weight.

Jordy said by the summer of last year, just months after starting the treatment, their lung function improved from 26 per cent to 37 per cent.

Their weight also went from 57kg in February 2020 to 67kg about a year later, which is what they weigh now and is a healthy weight for their height, Jordy said.

It might not sound like a huge jump, they said, but the difference has felt enormous in terms of how I go about my day-to-day.

Although, putting on the weight has also been a bit of a nightmare because my drag clothes dont fit, I have gone up a few dress sizes.

Before they took Kaftrio, Jordy had to do five hours of treatment and physiotherapy a day to help fight pain and infection, but now, they only need to do it for two hours a week.

This drug took me off the transplant list, its stopped my lungs deteriorating and I no longer have a tube in my tummy.

"And for the first time in my life, I am writing and creating new art about being well, not ill.

Rebecca Cosgriff from the Cystic Fibrosis Trust, who Jordy has worked closely with, said: "It's always so great to hear of the improvement in health of people like Jordy, who have benefited so much from Kaftrio in the last year.

"We won't stop until everyone with cystic fibrosis has access to the best possible treatments.

Kaftrio can be prescribed to people aged 12 and over, with certain mutations of the gene that causes CF.

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Jordy Deelight: Edinburgh drag queen with cystic fibrosis speaks about effects of 'miracle drug' - Edinburgh News

LONDON--(BUSINESS WIRE)--Vertex Pharmaceuticals Incorporated (Nasdaq: VRTX) today announced it has reached a new portfolio agreement with the Italian Medicines Agency, AIFA, for the reimbursement of all of Vertexs approved medicines for the treatment of cystic fibrosis (CF), including KAFTRIO (ivacaftor/tezacaftor/elexacaftor) in a combination regimen with ivacaftor.

Italian patients ages 12 years and older with one F508del mutation and one minimal function mutation (F/MF) or two F508del mutations (F/F) in the cystic fibrosis transmembrane conductance regulator (CFTR) gene will now have access to KAFTRIO (ivacaftor/tezacaftor/elexacaftor) in a combination regimen with ivacaftor. Additionally, under the terms of the agreement, eligible patients ages two years and older with CF who have two copies of the F508del mutation in the CFTR gene (F/F) will now have access to ORKAMBI (lumacaftor/ivacaftor). Eligible patients ages 12 years and older who either have two copies of the F508del mutation (F/F), or one copy of the F508del mutation and another responsive residual function mutation in the CFTR gene (F/RF), will have broad access to SYMKEVI (tezacaftor/ivacaftor) in combination with ivacaftor. The agreement also expands access to KALYDECO (ivacaftor) for eligible patients ages one year and older. In addition, the agreement covers any new approved indication extensions for Vertexs CF medicines submitted and approved for reimbursement during the term of the contract.

Ludovic Fenaux, Senior Vice President, Vertex International, commented, This agreement is an important milestone for cystic fibrosis patients in Italy. Our medicines have fundamentally changed the way CF is treated, and we are delighted with this broad portfolio agreement which includes access for younger patients to ORKAMBI and KALYDECO and access to SYMKEVI and KAFTRIO for patients 12 years and older. I would like to thank AIFA and all parties involved for their collaboration, commitment and engagement in quickly reaching this agreement.

KALYDECO was first reimbursed in Italy in 2015, followed by ORKAMBI in 2017 for patients 12 years and older. Vertexs CF medicines are reimbursed in over 25 countries around the world including Australia, France, Germany, the Republic of Ireland, the Netherlands, Spain, Sweden, the U.K. and the U.S.

About KAFTRIO (ivacaftor/tezacaftor/elexacaftor) in a Combination With Ivacaftor

KAFTRIO (ivacaftor/tezacaftor/elexacaftor) in a combination regimen with ivacaftor 150 mg was developed for the treatment of cystic fibrosis (CF) in patients ages 12 years and older who have at least one copy of the F508del mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Ivacaftor/tezacaftor/elexacaftor is designed to increase the quantity and function of the F508del-CFTR protein at the cell surface. The latest approved EU licensed indication for ivacaftor/tezacaftor/elexacaftor was supported by positive results of three global Phase 3 studies in people ages 12 years and older with CF: a 24-week Phase 3 study (Study 445-102) in 403 people with one F508del mutation and one minimal function mutation (F/MF), a four-week Phase 3 study (Study 445-103) in 107 people with two F508del mutations (F/F), and a Phase 3 study (Study 445-104) in 258 people heterozygous for the F508del-CFTR mutation and a CFTR gating mutation (F/G) or a residual function mutation (F/RF).

For complete product information, please see the Summary of Product Characteristics that can be found on http://www.ema.europa.eu.

About SYMKEVI (tezacaftor/ivacaftor) in Combination With Ivacaftor

Some mutations result in CFTR protein that is not processed or folded normally within the cell, and that generally does not reach the cell surface. Tezacaftor is designed to address the trafficking and processing defect of the CFTR protein to enable it to reach the cell surface and ivacaftor is designed to enhance the function of the CFTR protein once it reaches the cell surface.

For complete product information including dosing guidance, please see the Summary of Product Characteristics that can be found on http://www.ema.europa.eu.

About ORKAMBI (lumacaftor/ivacaftor) and the F508del Mutation

In people with two copies of the F508del mutation, the CFTR protein is not processed and trafficked normally within the cell, resulting in little-to-no CFTR protein at the cell surface. Patients with two copies of the F508del mutation are easily identified by a simple genetic test.

Lumacaftor/ivacaftor is a combination of lumacaftor, which is designed to increase the amount of mature protein at the cell surface by targeting the processing and trafficking defect of the F508del-CFTR protein, and ivacaftor, which is designed to enhance the function of the CFTR protein once it reaches the cell surface.

For complete product information, please see the Summary of Product Characteristics that can be found on http://www.ema.europa.eu.

About KALYDECO (ivacaftor)

Ivacaftor is the first medicine to treat the underlying cause of CF in people with specific mutations in the CFTR gene. Known as a CFTR potentiator, ivacaftor is an oral medicine designed to keep CFTR proteins at the cell surface open longer to improve the transport of salt and water across the cell membrane, which helps hydrate and clear mucus from the airways.

For complete product information, please see the Summary of Product Characteristics that can be found on http://www.ema.europa.eu.

About Vertex

Vertex is a global biotechnology company that invests in scientific innovation to create transformative medicines for people with serious diseases. The company has multiple approved medicines that treat the underlying cause of cystic fibrosis (CF) a rare, life-threatening genetic disease and has several ongoing clinical and research programs in CF. Beyond CF, Vertex has a robust pipeline of investigational small molecule medicines in other serious diseases where it has deep insight into causal human biology, including pain, alpha-1 antitrypsin deficiency and APOL1-mediated kidney diseases. In addition, Vertex has a rapidly expanding pipeline of cell and genetic therapies for diseases such as sickle cell disease, beta thalassemia, Duchenne muscular dystrophy and type 1 diabetes mellitus.

Founded in 1989 in Cambridge, Mass., Vertex's global headquarters is now located in Boston's Innovation District and its international headquarters is in London. Additionally, the company has research and development sites and commercial offices in North America, Europe, Australia and Latin America. Vertex is consistently recognized as one of the industry's top places to work, including 11 consecutive years on Science magazine's Top Employers list and a best place to work for LGBTQ equality by the Human Rights Campaign.

Special Note Regarding Forward-Looking Statements

This press release contains forward-looking statements as defined in the Private Securities Litigation Reform Act of 1995, including, without limitation, statements made by Ludovic Fenaux, Senior Vice President, Vertex International, in this press release and statements regarding our beliefs about the eligible patient population that will have access to our medicines, including patients that will now have access to a CFTR modulator for the first time, reimbursement coverage for future approved indication extensions of our medicines, and our beliefs regarding the benefits of our medicines. While Vertex believes the forward-looking statements contained in this press release are accurate, these forward-looking statements represent the company's beliefs only as of the date of this press release and there are a number of risks and uncertainties that could cause actual events or results to differ materially from those indicated by such forward-looking statements. Those risks and uncertainties include, among other things, that data from the companys development programs may not support an extended indication for our medicines, and other risks listed under the heading Risk Factors in Vertex's annual report and in subsequent filings filed with the Securities and Exchange Commission and available through the company's website at http://www.vrtx.com and http://www.sec.gov. You should not place undue reliance on these statements. Vertex disclaims any obligation to update the information contained in this press release as new information becomes available.

(VRTX-GEN)

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Vertex Announces New Portfolio Reimbursement Agreement in Italy Including KAFTRIO, SYMKEVI and Additional Indications of ORKAMBI and KALYDECO for...