Utah’s Somer Love Battles CF with Kindness and Strength

By Larry Luxner of CF News Today

Somer Love is sitting at her favorite local hangout, the Landmark Grill in suburban Midvale, happily devouring her lunch. Looming on the horizon to the east are Utah’s majestic Wasatch Mountains, but at more than 10,000 feet above sea level, the range is strictly off-limits to Love — as is nearby Park City and its famous ski resorts, at 7,000 feet above sea level.

That’s because cystic fibrosis (CF) has left Love with only 27 percent of normal lung function.

“I haven’t been to Denver since I was a kid, because it’s harder to breathe there. Even Salt Lake City, at 4,500 feet, is hard,” she told Cystic Fibrosis News Today in a recent interview. “Growing up I was fine, because my lungs were healthier. But I think living in Utah at this higher elevation actually helped strengthen my lungs.”

Love, 39, moved here from New Jersey with her parents when she was nine months old. At 11 months she was diagnosed with CF after her mother had heard that babies who have a salty taste when kissed should be taken for testing.

“We were outside playing in a playpen, and I tasted salty,” she said. “My mom called our pediatrician for a sweat test. I was super healthy and fat. The doctor was probably thinking ‘crazy lady from Jersey.’ But she talked him into doing it, and when the results came back, he called my mother crying.”

Growing up, Love received treatment from the Intermountain Cystic Fibrosis Center located at Primary Children’s Hospital and University of Utah Hospital in Salt Lake City.

Both centers are accredited by the Cystic Fibrosis Foundation (CFF) and serve mostly patients from Utah, Idaho, and Wyoming — with some coming from as far away as Montana and Nevada.

Love’s first hospitalization was at age 6; she began taking enzymes at 8, because no drugs were available back then. In eighth grade, she got her first nebulized medication.

‘Love to Breathe’

Because of her disease, she quickly developed a strong sense of activism and self-reliance.

“I’ve always been a really positive person. This stems from my parents, with whom I’m very close,” Love said. “They started the local CF chapter here and were on the board. They did everything in their power to help me. When you’re faced with such a challenge, you can either overcome it, or do nothing and let it overcome you.”

She added: “CF is basically my life, especially now that my disease has progressed. From the minute I wake up till the minute I go to bed, I’m constantly thinking about it. I literally think about every breath I take. I’m actually grateful for CF; it’s a blessing in an ugly disguise. It makes me appreciate things more.”

No surprise, then, that the website Love launched in 2001 is called “Love to Breathe” — a play on her last name.

Among other things, the site contains a blog with nearly 700 entries, a compendium of facts about CF, and dozens of photos, videos, and original paintings. There’s also a page dedicated to the CF activist’s #LoveToBreathe tokens; she’s sent out more than 10,000 of these little medallions to CF patients and supporters in all 50 U.S. states and more than 70 countries.

“I wanted parents to have a place to go for their newly diagnosed children,” she said. “There’s a lot of negativity out there on the Internet, and I wanted people to see somebody being positive. I’m an artist, so I started by sending my paintings to fundraisers all over the country.”

Love said the Internet is especially helpful to CF patients due to the isolating nature of the disease. “We cannot be around any other CF patients, so we connect online,” she said. “My website is a personal endeavor, a way for me to spread love and CF awareness.”

Even so, her main focus is on staying healthy. That means spending six hours a day doing her airway clearance and taking all her medications — about 60 to 80 pills daily. Her morning treatments are one-and-a-half to two hours each, and she must sterilize her “neb cups” afterward.

The transplant option

Love has been taking Symdeko (tezacaftor/ivacaftor and ivacaftor) twice a day ever since the combo therapy — developed by Vertex Pharmaceuticals — was approved in February 2018 by the U.S. Food and Drug Administration. But she must eat 10 to 20 grams of fat beforehand.

“Because of our gastrointestinal problems, we don’t absorb nutrients well, so one of the perks of CF is that we can eat whatever we want,” she said. Even so, Love is a vegetarian. She usually eats five small meals daily, with breakfast being her main meal — and she snacks all day long.

Speaking of food, each November, Love’s parents host an event called Taste of Utah. Tickets cost $200 each, and 30 restaurants around the state participate. Last year, the annual fundraiser generated just over $400,000 toward CF research.

Yet, Love herself doesn’t travel much. In fact, she said, “I’m never gone from my house for more than four hours. There are some days I can’t even get out of bed. I also have asthma, which makes things difficult.”

In addition, Love regularly experiences catamenial hemoptysis, a rare hormonal condition that causes her to cough up blood a couple of days before her period. And although she doesn’t have CF-related diabetes or liver disease, motherhood, she said, isn’t a good option for her.

“I grew up knowing that I wasn’t going to have kids,” she said. “I’ve had many friends with CF who have kids, and their health takes a huge decline. Also, I could never be the kind of mom I would want to be. When your child is sick, your first instinct is to cuddle them. But if we get sick, it’s a two-week stay in the joint.”

With the median age of survival for newborns with CF now in the early 40s, Love realizes that a double lung transplant is in her future — though the prospect of such a serious and potentially risky operation is daunting.

“I’m not quite there yet,” she said. “You can’t be too healthy, and you can’t be too sick. But I would absolutely opt for a transplant when the time is right.”

For Use in CF, New 2-in-1 Powder More Effective Against Resistant Bacteria

https://cysticfibrosisnewstoday.com/2018/12/18/new-powder-inhalation-more-effective-killing-antibiotic-resistant-bacteria/?utm_source=Cystic+Fibrosis&utm_campaign=c31e0676bf-RSS_MONDAY_EMAIL_CAMPAIGN&utm_medium=email&utm_term=0_b075749015-c31e0676bf-71418393

By Albert Molano

Originally published in Cystic Fibrosis News Today

12/18/2018

Purdue University researchers have invented a new way of delivering two antibiotics (colistin and ciprofloxacin) deep into the lungs of cystic fibrosis (CF) patients, enabling much more effective killing of antibiotic-resistant bacteria without exposing patients to high systemic doses of these therapies.

“We are providing a promising option to fight the global crisis of antimicrobial resistance,” Qi (Tony) Zhou, PhD, assistant professor at Purdue’s College of Pharmacy, who led the research team, said in a press release.

Respiratory infections caused by multidrug-resistant bacteria in CF patients can be deadly because they are resistant to most available antibiotics. In addition, therapies given intravenously (by injection into the vein) or orally have a hard time reaching the lungs, potentially requiring high doses for the therapy to be effective.

Last-resort antibiotics can still be effective, but these can be very toxic if they are given systemically. Colistin, for example, can damage the kidneys.

The existing alternative is to nebulize the antibiotics and deliver them directly into the lungs, which increases their local concentrations while reducing the risk of systemic toxicity. However, this requires expensive and complicated delivery devices, and prolonged administration times.

Now, Zhou’s team has succeeded in combining two antibiotics — colistin and ciprofloxacin — into a single particle that can be delivered as a dry powder.

According to the team, with this new formulation, more than 60 percent of the antibiotics reach the lungs, as opposed to only 10 percent with a jet nebulizer. In addition, the dry formulation offers improved chemical stability, and it is easier to use than conventional inhalation products.

“It has been a worldwide challenge to incorporate two antibiotics with different chemical properties into a single particle. Our novel formulation allows for a much more effective killing of drug-resistant bacteria in the deep lungs as two synergistic antibiotics can be simultaneously delivered to the same infection site,” Zhou said.

The team believes this technology can be applied to several antibiotic and compound combinations, potentially saving tens of thousands of lives from several deadly lung infections, including those affecting CF patients and people with ventilator-assisted pneumonia.

The Purdue Office of Technology Commercialization has filed a patent for the new technology, and researchers are looking for partners to continue its development.

An Ode to Friends with CF – Guest Blog by Amy Sylvis

By: Amy Sylvis

Yesterday, I didn’t want to go to the gym to workout. This time of year, I have bad allergies, the aspergillus in my lungs tends to cause me issues, my sinuses get angry and I just generally don’t feel like working out while my airways feel so tight.

Working out can sometimes remind me of the lung damage I have. One day I am able to run 5 minutes straight (ya, that’s a good day for me!), and some days running for 2 minutes straight is a huge struggle. That can make me feel like I’m falling short in my CF care – like I’m at fault for not being able to exercise as well as I could a few days earlier. But I’m insanely compliant and have been my whole life, so I know the truth is that some days with CF my lungs just aren’t as open as other days. Nature of the beast.

I’m also just not generally a very athletic person. I know that working out- forcing air deep in to my lungs and in to my small airways –  keeps me healthier than anything else available to me on this planet. Studies back this up as well – exercise inhibits epithelial sodium channels (eNac), which leads to hydration of airways and makes it easier to cough up mucus.

Sometimes this knowledge isn’t enough to get me to the gym, though.

Yesterday was one of those days that I’m so grateful for my friends with CF. I’m a strong believer in surrounding myself with people who are better than I am – it’s a real ego destroyer, but it’s also an amazing life hack for self-improvement, no matter what the goal. While on the treadmill, I used an app called Marco Polo and spoke to two friends of mine with CF – both who are amazing runners and make sure to get in exercise daily themselves. I complained to them about how crappy I was feeling the past few days. I told them how my lungs were feeling so tight and I could hardly run at all compared to a week ago.

My goal every time I run is 30 minutes. Whether it’s running 5 minutes and walking 3, then repeat; or running 2 minutes, walking 2, then repeat – I’m on that hamster wheel for 30 minutes.  I need to have a goal and structure to survive the nightmare that is running. Ha.

“Run for longer today,” one of my friends said when she messaged me back on Marco Polo.

“WHAT???” I replied with an angry face in my video.  

“On days you feel worse, work out longer than you normally would,” she emphasized.

I almost threw my phone across the gym. I was so miserable I didn’t think I could even make it to 30 minutes, let alone longer.

Then I remembered – the woman I was talking to has half the lung function I do. Half. She’s a mom, takes care of 2 kids by herself, and she can run farther and longer than I can. If she can do it, so can I.

I was still only able to run for 2 minutes straight yesterday, but I ran 2 minutes walked 2 minutes for 35 minutes, instead of my usual 30. Halfway through those painful 35 minutes, my other friend sent me a message stating that he agreed with what had already been said – suck it up and exercise for longer. (He runs several miles a day, every day, like a maniac, so he further reinforced my need to push myself in the gym).

I can’t tell you how much better I feel today. My lungs are more clear, more open, and I feel so much better both mentally and physically. I’m not sure I would have pushed myself so much yesterday if it hadn’t been for my two friends offering support when I needed it, a.k.a. a swift kick in the butt.

Growing up, I didn’t have friends with CF. I didn’t go to CF camp, I didn’t come across a lot of kids with CF at clinic, so I didn’t have the benefit of these amazing interactions when I was young. At the time, I’m not sure I saw the need. Heck, I’m not sure I saw the need even a few years ago. I had no idea what I was missing out on, though.

Having friends with CF offers a dimension to life that I never thought was possible, and that I didn’t realize I needed. I’m not saying we should all be friends with every person with CF on the planet – but finding your tribe can be life-changing. Yup, I said it. Truly life changing.

Here’s to laughing, exercising, vesting, coughing, insulin-ing and CFTR-modulating together, CF community! We are so fortunate to have each other. FIGHT ON FOREVER!

I’m Drowning – A researcher-patient’s plea for broader inclusion in cystic fibrosis trials

By: Ella Balasa

I’ve always known cystic fibrosis (CF) is a progressive disease; it destroys lung cells, tightens the small airways in the bottom of my chest, and each day takes me closer to the time when it will have ravaged my lungs. I had never really questioned if there was some way this process could be altered. I accepted that it couldn’t.

Recently, however, this has changed. The epicenter of new CF research is the development of medications that will slow, stop, and hopefully even reverse the effects and damage that CF inflicts on the body. The possibility of the cells in my lungs functioning to their full potential — with CF transmembrane conductance regulator protein function restored and working correctly, expelling chloride out of my cells, hydrating the surface of my lungs, and halting the thick sticky mucus that has caused my airways to be enveloped in a suffocating cloak for all these years — is like a feeling of being rescued when you are drowning.

Unfortunately, I am still drowning.

“I’m very sorry, Ms. Balasa, but you will not be able to be a participant in this clinical trial.” This was the response I received during one of my searches for these drug trials. Excited by the possibility of participating, finding one recruiting at my local adult clinic, I reached out to study coordinators and was informed that I met all but one criterion to participate in the studies. This specific criterion has prevented me from prior trial participation involving other investigational medications treating the symptoms of CF, including anti-infectives and anti-inflammatories.

Most CF studies, including phase I, II, and III trials, require a lung function minimum of at least 40% FEV1 (forced expiratory volume in one second). My FEV1 is 25%, so I am excluded from these trials. Many patients face a similar situation. The 40% threshold biases samples toward a young patient population, as this degenerative condition causes steadily decreasing lung function with time. Furthermore, as CF treatment has rapidly progressed and increased patients’ life expectancies, there are now more adults with CF in the U.S. than children, according to the CF Foundation Patient Registry.

As a patient who works in the science field, I started to ask myself: Where does that number come from? Should this one variable be such a deciding factor? Are we getting comprehensive results from these studies if a subset of patients is omitted? Are investigators using eligibility criteria from a prior study without determining whether the exclusions are scientifically justifiable?

To continue reading, please visit MedPage Today.

Monitoring Pulmonary Exacerbation in Cystic Fibrosis: The Hunt for Urine-based Biomarkers Begins

By Michele Wilson PhD

The buildup of mucus in the lungs is an ongoing challenge faced by people with cystic fibrosis, and knowing whether they should seek medical attention is not always clear.

Recently, Mologic – a developer of personalized diagnostics – have developed a tool which they hope will help guide people with cystic fibrosis so they can avoid unnecessary stays in hospital.

The app-embedded algorithm converts data collected from a urinary test to a traffic light result, which indicates whether a patient is stable or in need of medical intervention.

Recently, Mologic, announced that they are launching a clinical trial to assess the company’s urine-based diagnostic tool, ‘HeadsUp’.

To learn more about how this point-of-care diagnostic tool could help improve healthcare for people with cystic fibrosis, we spoke with Gita Parekh, Head of R&D at Mologic.

How do you define pulmonary exacerbation, and why is it important that it is monitored in people with cystic fibrosis? Continue reading Monitoring Pulmonary Exacerbation in Cystic Fibrosis: The Hunt for Urine-based Biomarkers Begins

Lung Plethysmography Procedure

By HH Patel, M.Pharm

Similar to spirometry and pulse oximetry, lung plethysmography is a tool within the broad umbrella of pulmonary function tests. It is a type of diagnostic procedure used to measure the amount of air in the lungs after inhalation or exhalation.

Also referred to as body plethysmography, lung plethysmography provides an idea about how efficiently the lungs are functioning. It is also especially useful in characterizing types of pulmonary abnormality and implementing appropriate treatment approaches depending on the diagnosis.

Continue reading Lung Plethysmography Procedure