Category Archives: Cf

DetailsCategory: Proteins and PeptidesPublished on Friday, 04 September 2020 10:27Hits: 134

Study Stopped Enrolling in March 2020 Due to Limitations Caused by COVID-19

AUSTIN, TX, USA I September 03, 2020 I Savara Inc. (Nasdaq: SVRA), an orphan lung disease company, today announced the decision to discontinue ENCORE, a 48-week, open-label, non-controlled Phase 2a exploratory clinical study of Molgradex for the treatment of nontuberculous mycobacterial (NTM) lung infection in people living with cystic fibrosis (CF). Molgradex, an inhaled formulation of recombinant human granulocyte-macrophage colony-stimulating factor (GM-CSF), was also evaluated for the treatment of NTM in non-CF patients in the Phase 2a OPTIMA study that completed in March 2020.

The decision to discontinue ENCORE was based on confounding factors that compromised the ability of the study to achieve its primary purpose of investigating the efficacy of Molgradex on NTM sputum culture conversion to negative. Such factors included the impact of COVID-19 on patient recruitment and continued participation in the study as well as the availability of the new triple-combination CFTR modulator, approved during the treatment period of ENCORE, that has become a preferred treatment option for many CF patients. Study recruitment was terminated at the end of March with 14 patients enrolled out of a total target of 30. Additionally, nine out of 14 patients were on the triple-combination CFTR modulator. Eight patients started it during the study and one patient was on the triple-combination modulator from baseline. The decision to discontinue the study was not based on safety concerns.

Due to the early discontinuation of the study, not all patients have completed the planned 48-week treatment period. Based on preliminary data as of September 2020i from 12 patients who progressed at least beyond 20 weeks of treatment, five patients on the triple-combination CFTR modulator achieved a sputum culture conversion, defined as at least three consecutive sputum samples without growth of NTM. All of those patients had started the triple-combination modulator during the study prior to culture conversion. Sputum culture conversions were not observed in patients who were on Molgradex without the triple-combination modulator.

Discontinuing the exploratory ENCORE study is very disappointing and I extend our sincere gratitude to the patients who participated, especially during the trying times of this pandemic, said Badrul Chowdhury, Chief Medical Officer, Savara. Many factors contributed to this decision, most notably the effects of COVID-19, which resulted in small patient numbers and operational disruptions at clinical sites. The approval of the triple-combination CFTR modulator, which transformed the standard-of-care for this patient population, further confounded our ability to evaluate the potential effect of Molgradex in this study as we are unable to differentiate which drug was primarily responsible for the observed culture conversions. While the results of ENCORE are interesting, additional controlled studies would be required to accurately understand the therapeutic potential of Molgradex in combination with the triple-combination modulator treatment.

Based on the results of the exploratory ENCORE and OPTIMA studies, the Company will continue to focus development efforts of Molgradex on its lead indication, autoimmune pulmonary alveolar proteinosis, and does not plan to conduct further development activities related to Molgradex in NTM.

About Savara

Savara is an orphan lung disease company with a pipeline comprised of three investigational compounds, all of which use an inhaled delivery route. Our lead program, Molgradex (molgramostim nebulizer solution), is an inhaled granulocyte-macrophage colony-stimulating factor (GM-CSF) in Phase 3 development for autoimmune pulmonary alveolar proteinosis (aPAP). Apulmiq is an inhaled liposomal ciprofloxacin in Phase 3 development for non-cystic fibrosis bronchiectasis (NCFB). AeroVanc is an inhaled vancomycin in Phase 3 development for persistent methicillin-resistant Staphylococcus aureus (MRSA) lung infection in people living with cystic fibrosis. Savaras strategy involves broadening its pipeline through indication expansion, strategic development partnerships and product acquisitions, with the goal of becoming a leading company in its field. Our management team has significant experience in orphan drug development and pulmonary medicine, identifying unmet needs, developing and acquiring new product candidates, and effectively advancing them to approval and commercialization. More information can be found at http://www.savarapharma.com. (Twitter: @SavaraPharma, LinkedIn: http://www.linkedin.com/company/savara-pharmaceuticals/).

SOURCE: Savara

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Savara to Discontinue Exploratory ENCORE Study Evaluating Molgradex for the Treatment of NTM Lung Infection in People Living With Cystic Fibrosis (CF)...

Lets all agree not to waste a single synapse remembering the first-ever match between Atlanta United and Inter Miami CF. Tyler Wolff, Ill grant you the lone exception, MLS debut and all. For everybody else, purge every last second of those 90 minutes.

This particular scoreless draw will be remembered solely for the news that broke before the ball was even kicked: a year and a half after becoming MLSs record incoming transfer, Pity Martinez is headed to Saudi Arabia for a reported $18 million. Atlanta United are mashing the reset button. Vaarwel, Frank de Boer. Adios, Pity. Tu siguiente, Ezequiel?

Well see.

Same two words define the Inter Miami experience right now. What is this teams identity? Well see. Who will be the much-discussed third Designated Player? Well see. Will they every wear a primarily pink jersey? Well see. Can they capture the hearts of South Floridians without a gameday experience or wins? Well see.

One team is rebuilding, the other just building. Either way, there are lots of short-, medium- and long-term questions to be answered. Here are three.

Remember when I Baeranteed Barco would drop a double-double in goals and assists in 2019 on Extratime? Me neither. Never happened.

This definitely happened on Wednesday night after an ineffectual 62 minutes from the Argentine earned him an early hook.

Hmmmmm, where have I seen that before?

No word on whether Barco kicked any seats upon arrival to the bench, but theres no doubt that his production for the Five Stripes hasnt matched the hype, price tag or international pedigree. Its hard to imagine thats going to suddenly change in the midst of a pandemic, with an interim head coach and without the Martinez duo (Pity and Josef) that was supposed to make him look like a $30 million player.

So now what? Will Barco be part of the house-cleaning? Is there a taker out there, at a number that makes sense for Atlanta? And if not, can Darren Eales and Carlos Bocanegra coax a manager to the club who can revitalize the young mans career? Do you wait for the Olympics and hope for a value/form bump? Is that bump ever coming? Well see.

What does it say that neither Pity nor Barco settled with Tata Martino out the door?

Enter The Athletics Felipe Cardenas

Sounds like the answer to that question then is the player, as Eales also confirmed the club will be looking to add a player in this window prior to Wednesday night's match.That is unless Atlanta is conducting a high-profile coaching search with almost no leaks or peeps from South America, where theyre reportedly sniffing around. If thats indeed the way it goes down, it simply reinforces whats been pretty clear of late: the soccer decisions are made by technical director Carlos Bocanegra.

Thats his job, after all, but the seemingly unilateral nature of those decisions was clearly a point of contention for De Boer in the divorce. Maybe Atlanta already have their next manager on the down low. Maybe theyre consulting with him as they seek out their next big name. Maybe not. I dont know. What I do know is that a gap between the coach and the players blew up the locker room and the project once and thats one too many times for a club like Atlanta.

Think back to what made Miguel Almiron and Josef Martinez so special. It was, in some significant part, their relationship with Martino and the freedom, faith and responsibility that he instilled in them that led to an MLS Cup triumph, dump truck full of pounds from Newcastle and goals on goals (plus, a long-term commitment) from the Venezuelan.

That vibe and Martino may be irreplicable, but 2020 is basically a wash already. Best to make sure the big pieces all fit together. The next big spend needs to be more Miggy than Pity.

Sounds like general manager Paul McDonough thinks so.

So, after dozens of rumors, it seems the Argentine striker will be the teams third DP, along with Rodolfo Pizarro and 20-year-old winger Matias Pellegrini. Should it come to pass, the 32-year-old Higuain would join 33-year-old Blaise Matuidi in making free moves from Juventus. At the very least, in a pandemic with no gate revenue, that seems like smart business. Why pay transfer fees when the immediate future is so uncertain?

Best-case scenario? The Argentine is a South Florida Robbie Keane. Miami need goals. They need some guile when it comes to chance creation. Theres some disagreement about what sort of DP would best facilitate those things, but its hard to argue that Higuain wouldnt be a huge help in those departments, especially once Matuidi adds some dynamism to the midfield. They need to set a culture, too. A couple World Cup finalists (one winner) with trophy hauls Inter can only dream about ought to help there.

Still, I cant help but think a pair of over-30 players on free transfers wasnt exactly what Inter Miami was billed to be. You can measure MLSs growth via your reaction to this move. In 2010, it would have been the only news that mattered. In 2020, were sort of like, Yeah, that seems like it could work, but its kind meh, too.

Short- and medium-term, I think itd work out. It wont be easy to be like Keane, who joined a Supporters Shield and MLS Cup winning team with an established culture, but Higuain seems genuinely excited about the opportunity to play and compete in MLS. Hed arrive with eyes wide open thanks to the counsel of his older brother, Federico. He still finds the back of the net, even if those 30-plus goal seasons at Napoli and Juve are a couple years in the past.

And hey, maybe its another hold-on-how-did-that-happen TAM signing! Almost certainly not, but those parachute payments are no joke in Turin. Good money if you can get it.

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What's next for Atlanta United and Inter Miami CF? | Andrew Wiebe - MLSsoccer.com

Patients with cystic fibrosis (CF) who had pulmonary exacerbations treated with concomitant azithromycin and tobramycin had worse outcomes than those treated with tobramycin alone, according study results published in the Annals of the American Thoracic Society.

Researchers screened medical records in the CF Foundation Patient Registry-Pediatric Health Information System for patients aged 6 to 21 years with CF who were hospitalized for pulmonary exacerbations and treated with tobramycin. These records were retrospectively reviewed to determine if differences in outcomes existed between patients treated with concomitant azithromycin and tobramycin or tobramycin alone.

Of the 10,660 patients included in the dataset, there were 2294 (totaling 5022 pulmonary exacerbations) who were treated with tobramycin. Of those 5022 exacerbations, 2247 were treated with concomitant azithromycin and tobramycin.

When azithromycin was used to treat patients, there was a significantly lower absolute improvement in percent predicted forced expiratory volume in 1 second (ppFEV1). Additionally, those patients had lower odds of returning to 90% of baseline ppFEV1, (odds ratio, 0.79; 95% CI, 0.68-0.93; P =.003) and a shorter time to next pulmonary exacerbations requiring intravenous antibiotics (hazard ratio, 1.22; 95% CI, 1.14-1.31; P <.001) compared to tobramycin without concomitant azithromycin.

Concomitant [azithromycin] and [intravenous] tobramycin use for in-hospital pulmonary exacerbation treatment was associated with poorer clinical outcomes than treatment with [intravenous] tobramycin without [azithromycin], the study authors wrote. These results support the hypothesis that an antagonistic relationship between these two medications might exist.

Reference

Cogen JD, Faino AV, Onchiri F, et al. Effect of concomitant azithromycin and tobramycin use on cystic fibrosis pulmonary exacerbation treatment. Ann Am Thorac Soc. Published online August 18, 2020. doi:10.1513/AnnalsATS.202002-176OC

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Cystic Fibrosis Outcomes With Concomitant Azithromycin and Tobramycin Use - Pulmonology Advisor

Dutch truck manufacturer DAF Trucks has doubled the range of its all-electric CF Electric truck to over 200 kilometres. Since the vehicle is significantly lighter with the new battery generation, the payload has also increased.

The introduction of a new battery generation also means a weight reduction of 700 kilograms that the manufacturer says is directly reflected in a higher payload. The CF Electric with extended range will be launched on the market this month and is expected to be delivered from early 2021. The electric motor will remain the same, as the drive still has an output of 210 kW and a maximum torque of 2,000 Nm.

The battery now has a gross capacity of 350 kWh, of which 315 kWh can be used. Previously the gross energy content was 170 kWh. According to DAF, the dimensions of the electricity storage unit have remained the same. The battery is also conditioned to remain within its optimal working window of 25 to 40 degrees, which supports the batterys durability and constant performance level.

Assuming an appropriate charging station, the CF Electric should be able to cover over 500 kilometres per day. With 250 kW charging power, the complete charging process takes 75 minutes according to heavy transport manufacturer based in Eindhoven in the Netherlands. If the battery is charged during loading or unloading of the payload or during a drivers break, the so-called daily driving distance should be possible. The CF Electric is not actually intended for long-distance traffic but is optimised for urban areas in logistics for supplying supermarkets or as a waste collection vehicle.

daf.com

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DAF Trucks double the range of the CF Electric - http://www.electrive.com

ConFlat (CF) fittings are flanges which utilize metal seals to attain vacuum conditions. The combination of a stainless-steel knife-edge flange and a copper gasket (OFHC) makes for a nearly impenetrable vacuum barrier.

For the seal, the integrity of the knife-edge on the stainless-steel flange is vital. The hardness of the stainless steel coupled with the softer copper gasket means that there are few configurations that would provide equally airtight capabilities. The stainless-steel knife-edge cuts an indentation into the softer copper gasket, as it is the sturdier, stronger metal.

The CF knife-edge system guarantees the best performing vacuum seal, permitting vacuum levels, which are over 10-12 mbar. CF flanges use a series of bolts, unlike other vacuum seals that use clamps and elastomers.

This guarantees a strong and uniform seal over the copper gaskets. Depending on the application, CF fittings can be supplied with both through-bolt and threaded holes.

The gaskets are made from soft materials, while the majority of CF fittings are manufactured from high tensile metals like stainless steel. OFHC copper is the most common material utilized for the gaskets. Silver plated copper of aluminium gaskets provide a viable alternative where pure copper cannot be used.

The main advantages of using such robust materials are; increased durability and an elevated propensity for withstanding high temperatures. The seals can be baked to temperatures as high as 400 C, which, consequently, enables the more efficient pumping of a system.

A certain level of flexibility is required because of the varied environment that CF fittings are used in. There is a variety of standard products, with rotatable and fixed flanges. Rotatable flanges are needed for bolt holes to align and permit components to interface with each other.

CF fittings are utilized for UHV application, but not exclusively, another common application is for high temperature processes. The all metal seal will withstand temperatures up to 400 C making it a popular choice for coating systems and furnaces, amongst other applications.

Allectras team of specialists would recommend CF fittings in a high temperatures process or when the vacuum level requires it. The durability at high temperature, naturally low outgassing of metals, and the superior metal sealing technology make them the obvious choice for processes in demanding environments.

This information has been sourced, reviewed and adapted from materials provided by Allectra Limited.

For more information on this source, please visit Allectra Limited.

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ConFlat (CF) Fittings and Flanges: What Are They? - AZoM

Since Stephen Glass took over as interim head coach of Atlanta United, much of the talk has been about getting Atlanta back to their core values and style.

That means winning with energetic, entertaining andattacking soccer.

In Glass' interim reign the club are finding their feet again, allwithout Josef Martinez, of course. The process won't be linear nor easy, as Atlanta picked up a 2-0 win over Nashville SC but lost 3-1 to rival Orlando City SC this weekend. With a clash against struggling Inter Miami on Wednesday (7 pm ET | FS1 in US; MLS LIVE on DAZN in Canada), Glass forecasts his side to be the aggressors.

(Miami) have multiple threats, wide and through the middle, and we need to be on our guard," Glass told reporters on a virtual press conference Monday. "But any game where were at home with the players that weve got, we should be looking to take charge of the game.

Even without Josef, Atlanta still boast the likes of Pity Martinez, Ezequiel Barco, Matheus Rossetto and new signings Jurgen Damm and Cubo Torres. Pity scored both goals in Atlanta's win over Nashville, while Rossetto continues to find his feet.

We have a lot of players in the top half of the pitch that are good with the ball at their feet," Glass said. "Having that belief, putting that belief in the group, I think youll see it. Its a positive group.

But Pity and Rossetto are the only two of the group are the only ones fully fit at the moment.

Barco is returning from injury while Damm and Torres are working on match fitness/sharpness after their summer moves to Atlanta. Getting up to speed on the fly, without friendlies to get match minutes in your legs, isn't easy. Barco played 30 minutes off the bench while Damm made his Atlanta debut with 11 minutes against Orlando. Both players picked up assists on Atlanta's lone goal. Torres has played a total of 40 minutes over two games.

Glass expects more involvement for those three players against Miami.

The aim is theyll get a little bit more (time against Miami)," Glass said."The aim is theyll keep building. You saw the quality of these boys when they got on the pitch. We havent had a group of preseason game where you can afford to give guys 35 minutes or half a game because the results are important. But the results over the course of the season is more important than one individual result. We have to get this group of players as good as they can be for what hopefully will be a very important run in.

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Stephen Glass expects Atlanta United to take game to Inter Miami CF on Wednesday - MLSsoccer.com

CHIPPEWA FALLS A local public library will no longer require appointments for patrons starting next week, making it easier to stop in to rent a book while other COVID-19 precautions remain in place.

The limit of 10 patrons in the Chippewa Falls Public Library at any given time will be maintained, but people looking to enter the building will no longer need to schedule a time in advance.

Its going to kind of be like stores where they have someone at the front door, library director Joe Niese said about controlling the number of people in the building.

Previously, people would sign up for 50-minute time slots to use the Chippewa Falls library, but Niese said some only needed a few minutes in the building to pick up materials and check out. That prompted the library to consider jettisoning the appointment system when it is increasing its hours for fall.

Were also thinking that if we have more open hours that patronage would be spread out, he said.

Whether or not a time limit would still apply to patrons when the scheduling system goes away on Tuesday has not yet been decided.

Other area libraries are also changing the availability of their in-person services this month to allow more flexibility for patrons while also working to limit the spread of COVID-19.

Altoona Public Library is expanding its in-person hours next week, but keeping an appointment system in place.

Were trying to accommodate demand while being mindful of public health concerns, said Alyson Jones, the librarys director.

Since mid-July, Altoonas library had let patrons inside on two days a week but that will change to six days a week after Labor Day. Capacity had been limited to seven patrons per hour, but that will increase to 10 next week.

At the same time, the Altoona library will cease curbside service and instead offer short grab-and-go appointments where people can pick up their reserved materials inside the building.

While customers have appreciated the convenience of books delivered to them as they wait curbside, Jones said continuing that service would become onerous for librarians as the building gets busier and outdoor weather becomes colder.

Were trying to recognize the weather will change, she said.

Curbside service is continuing at the Chippewa Falls library, but will require appointments starting next week. L.E. Phillips Memorial Public Library in Eau Claire intends to continue offering its curbside service, which is available in the covered parking lot outside the buildings lower level.

Eau Claires public library will be raising its capacity later this month for in-person services, but still require people to schedule their visit.

On the week of Sept. 14, the current 10-person limit on people allowed in for browsing will be raised to 25 patrons. Those appointments will remain for hourlong time slots.

The Eau Claire library also has been scheduling separate 75-minute appointments for visitors to use public computers in the building. The capacity for those is going from five people at a time to 10.

Since L.E. Phillips Memorial Public Library reopened its doors to patrons on July 1, director Pamela Westby said people have been reserving time slots to use the building.

Theres definitely a demand, she said.

On some days, she said people have gone to the librarys front door to ask staff if any other patrons missed their appointments, hoping for a last-minute opening in the schedule to get time inside the building.

While the capacity is increasing to help people reserve the times they want, Westby said that reserving an appointment will still be required for public health reasons. Knowing who was in the building at a certain time would help with contact tracing in the event that a patron tests positive for COVID-19.

Westby said the library has been consulting with local health officials and is basing its decisions on indicators of coronavirus activity in the community.

Were watching the statistics from the Eau Claire City-County Health Department and trying to be mindful and smart about that, too, she said.

Area libraries closed in mid-March when COVID-19 was declared a global pandemic. In late April and early May, curbside pickup programs began, allowing patrons to get materials they reserved online while waiting in their cars outside of local libraries. Libraries reopened their doors during summer with reduced capacities, time limits, appointment systems and shorter hours in place.

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No more need to book a time at CF library, starting next week - Leader-Telegram

This afternoons interleague matchup between the Baltimore Orioles and New York Mets started off with quite a bang. Seriously, Mets outfielder Jake Marisnick was involved in a scary collision during the bottom of the second inning.

Marisnick was tracking down a long fly ball off the bat of Pedro Severino, but it was just past the reach of his outstretched glove. To make matters worse, he crashed into the wall since he lost track of his surroundings.

It was such a violent collision that Marisnick legitimately left an imprint on the wall. His sunglasses also broke due to the impact, leaving a huge scratch on his face.

New Yorks staff briefly checked on Marisnick to make sure that he was fine. Although he crashed into the wall pretty hard, the former World Series champ chose to stay in the game.

Heres the collision that nearly knocked Marisnick out of the game:

Jake Marisnick just sprinted face-first into the center field fence in pursuit of Pedro Severino's triple. He literally left an imprint of his face on the wall, and appears to have broken his sunglasses.

Following a long delay, Marisnick stays in the game. pic.twitter.com/Hq3Mrq8h0r

Anthony DiComo (@AnthonyDiComo) September 2, 2020

The Mets elected to put Marisnick in the lineup today because they didnt like the matchup between Brandon Nimmo and John Means.

Marisnick hasnt seen much playing time this year. That could change though if he can string together a few solid at-bats.

At the very least, the Mets know they have an extremely tough utility player on their roster in Jake Marisnick.

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Video: Mets CF Has A Scary Crash Into The Outfield Wall - The Spun

Researchers reveal the Vav3 protein is responsible for the formation of bacterial docking stations in the airway cells which promote the recurrent lung infections seen in Cystic Fibrosis patients.

Cystic fibrosis (CF) is one of the most common genetic disorders in Switzerland, it does not have a cure and requires life-long management throughout patients shortened lifespans. This reduced life expectancy is particularly attributed to recurrent, life-threatening respiratory infections, so researchers set out to establish why CF patients are so prone to these lung infections.

The team Scientists from the University of Geneva (UNIGE), Switzerland, discovered in their study that a protein called Vav3 promotes these infections by creating what they named a bacterial docking station on surface of the airways. The scientists also demonstrated that inhibiting Vav3 could prevent these recurrent infections and suggest this could be transferred into therapeutic prospects to limit respiratory complications in patients with CF.

CF, thought to affect more than 700,000 people worldwide, is caused by a mutation in a gene for a protein involved in the secretion of mucus. The result is an abnormally thick (hyperviscous) mucus that can cause severe respiratory and digestive problems, such as airway obstructions.

Aside from its role in airway obstruction, accumulations of the hyperviscous mucus also appear to play a role in promoting persistent lung infections, primarily caused by the bacteria Pseudomonas aeruginosa. These bacteria are known for being highly resistant to antibiotics, so understanding how it anchors to the cells of the airways is highly desirable.

Marc Chanson, Professor at the Department of Cell Physiology and Metabolism of the Faculty of Medicine of the UNIGE, explained: While it is known that mucus viscosity plays a role in trapping bacteria, the reason why they anchor so easily to airways was unknown. Anchoring of Pseudomonas aeruginosa to airways cells is the starting point for these often fatal infections. Understanding this process could help preventing their occurrence.

The protein Vav3 (in green) creates bacterial docking stations on the surface (in red) of respiratory cells (nuclei in blue) that facilitate airways infection in patients with cystic fibrosis [Credit: UNIGE, laboratoire Chanson].

In their paper, the researchers compared airway cells from people with healthy cells and those affected by CF. The whole project began when we found that the protein Vav3, which had not been shown to be involved in this disease until now, was over-expressed in sick cells, commented Mehdi Badaoui, researcher in Professor Chansons team and first author of this work.

After numerous in vitro analyses, the team discovered that in the CF cells Vav3 interacts with two other proteins, fibronectin and integrin b1, creating a complex on the cell surface that acts as a bacterial docking station, promoting Pseudomonas aeruginosa infections. This is the first time that a mechanism creating a favourable microenvironment for a bacterium before it even arrives has been observed, said Chanson. This might explain the high number of chronic lung infections in people with cystic fibrosis.

The team experimented further, inhibiting Vav3 expression in CF cells enabled them to prevent the expression of the two other proteins that make up the docking station. And, indeed, the absence of this structure limits the adhesion of Pseudomonas aeruginosa, added Badaoui.

Although the exact link between Vav3 and the genetic defect that causes CF has yet to be determined, the researchers concluded that their discovery is a promising therapeutic target for limiting respiratory complications.

The paper was published inCell Reports.

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Protein promotes the respiratory complications of Cystic Fibrosis - Drug Target Review

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Social distancing has become the new normal during the pandemic.

Millions across the UK have also had to "shield" due to their high risk of needing hospital treatment if they catch coronavirus.

An end to some restrictions is in sight - shielding is due to be paused at the end of July in Northern Ireland while social distancing has been reduced to 1m (3ft) with restrictions.

But Dr Ian Banks and his three-year-old grandson Lorcn Maguire, both from County Down, will not benefit from these changes.

They have always lived 2m apart.

Dr Banks has not held Lorcn since his grandson was diagnosed with cystic fibrosis (CF) - a genetic condition that can cause fatal lung damage.

He last touched him when his daughter, Jen Banks, visited his home to tell him about the diagnosis following a heel-prick test given to all newborn babies in the UK.

In a cruel twist of fate, the former A&E doctor has a long-term lung infection that could be deadly to his grandson.

"Ever since then, I have never come closer to him than 2m or 3m, and always downwind and never in the same room," he said.

Dr Banks said he had a good medical understanding of the need to stay 2m apart, not only with Lorcn, but also with his parents, due to the risk of cross-infection.

But he said it was heartbreaking to do so.

"Even with all that medical training, your relationship with your own child is very different," he said.

"We have been in social isolation for three years when it comes to our family.

"I can't even hug my own daughter, I haven't touched Jen since Lorcn was born.

"It's a huge strain on your relationship, particularly when you are used to touching people as humans are."

Despite the challenges, Dr Banks said Lorcn looks healthy, is a "wonderful little fella" and a credit to his parents.

"Even the tiniest things we take for granted can be lethal when it comes to CF, so you are constantly worrying over whether you are going to give this child an infection," he said.

"You never get used to it, when the virus crisis declines we will still be where we are."

Jen said the need to be apart from family was "one of the worst things" about her child's diagnosis.

She said it went against instinct not to hug her dad out of fear that it could hurt her son and that it "never felt normal" despite the passage of time.

"From the start of them being together, they have always been 2m apart," she said.

It has meant many difficult decisions around whether Jen's father or her son attend birthdays, weddings and funerals, while extended family Christmases are virtually impossible, she added.

"Family is the most important thing in most people's lives so not to be as close to them as you want to be, not to be able to share a meal in the same house, not to be able to come and visit with you, it's not ok," she said.

Jen said she vividly recalled the last touch between her father and son.

"I put Lorcn into his arms before I told him [the diagnosis] because I wanted him to feel him - love is so tactile," she said.

"I said: 'We've some bad news. Lorcn has been diagnosed with CF'.

"My dad handed him straight back into my arms. He knew immediately he could be a risk to Lorcn.

"He has never held him again, very rarely been within 6ft, never held his hand, lifted him on his shoulders, any normal things a grandfather would do with his grandson."

Dr Banks said it was an "absolutely heartbreaking" moment that had repercussions throughout their family.

Jen said there were "dark days" during which they campaigned with the CF community for access to the best treatments.

"Since he was born we have been fighting - fighting for access to the right drugs, fighting infections we can't clear, it's been awful," she said.

The situation improved when Lorcn was prescribed the life-extending drug Orkambi after it was made available in Northern Ireland at the end of 2019.

Lorcn has thrived on Orkambi but campaigning continued for Kaftrio, another groundbreaking drug that tackles the underlying causes of the disease by helping the lungs work effectively.

Health Minister Robin Swann said last week that a deal had been struck with the makers of Kaftrio, which will also become available on the NHS.

The Cystic Fibrosis Trust has described the drug as life saving and Mr Swann said the importance of the deal to affected families "cannot be emphasised enough".

Jen said the news was a "complete dream".

Although there are no clinical guidelines as yet, she hopes Lorcn will start Kaftrio when he is six - subject to the age limit being reduced from 12 - finally enjoying a more normal relationship with her father due to a reduced risk of cross-infection.

"Obviously we still need to social distance until then, but there is light at the end of the tunnel," she said.

She said she was "incredibly grateful to the NHS" and Lorcn's CF team who were "the best in the world" and added that campaigning for those who would not benefit from Kaftrio will continue.

Dr Banks described Kaftrio as a "game-changer" that should enable Lorcn's body to work normally.

"It is very hard, even with a medical background, to maintain what we are doing," he said.

"At the age of 70 I think I am allowed to be optimistic, I think the future for these children is so much brighter.

"I am looking forward to his sixth birthday because that is when he is going to start [on Kaftrio] hopefully, and that will be the first time since he was born I will be able to give him a hug."

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Cystic Fibrosis: The grandfather and grandson who must remain 2m apart - BBC News