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.”

Insufficient antibiotics available for cystic fibrosis patients: Study

Turns out, the majority of patients with cystic fibrosis may not achieve blood concentrations of antibiotics sufficiently high enough to effectively fight bacteria responsible for pulmonary exacerbations, thus leading to worsening pulmonary function.

Cystic fibrosis, a genetic condition that affects about 70,000 people worldwide, is characterised by a buildup of thick, sticky mucus in patients’ lungs. There, the mucus traps bacteria, causing patients to develop frequent lung infections that progressively damage these vital organs and impair patients’ ability to breathe.

A recent study led by researchers at Children’s National Health System shows that it’s impossible to predict solely from dosing regimens which patients will achieve therapeutically meaningful antibiotic concentrations in their blood. The findings were published online in the Journal of Pediatric Pharmacology and Therapeutics.

These infections, which cause a host of symptoms collectively known as pulmonary exacerbations, are typically treated with a combination of at least two antibiotics with unique mechanisms. One of these drugs is typically a Beta-lactam antibiotic, a member of a family of antibiotics that includes penicillin derivatives, cephalosporins, monobactams and carbapenems.

Although all antibiotics have a minimum concentration threshold necessary to treat infections, Beta-lactam antibiotics are time-dependent in their bactericidal activity. Their concentrations must exceed a minimum inhibitory concentration for a certain period. However, study’s lead author Andrea Hahn explained that blood concentrations of Beta-lactam antibiotics aren’t typically tracked while patients receive them.

Since antibiotic dosing often doesn’t correlate with cystic fibrosis patients’ clinical outcomes, Dr. Hahn and other researchers examined whether patients actually achieved serum antibiotic concentrations that are therapeutically effective.

In addition, all the patients underwent pulmonary function tests at the start of their exacerbations and about once weekly until their antibiotic therapy ended.

Using the data points, the researchers constructed a model to determine which patients had achieved therapeutic concentrations for the bacteria found in their respiratory secretions. They then correlated these findings with the results of patients’ pulmonary function tests. Just 47 per cent of patients had achieved therapeutic concentrations. Those who achieved significantly high antibiotic exposure had more improvement on their pulmonary function tests compared with patients who didn’t.

Paradoxically, they discovered that although each patient received recommended antibiotic doses, some patients had adequately high serum antibiotic concentrations while others did not.

Another way to ensure patients receive therapeutically meaningful levels of antibiotics is to develop new models that incorporate variables such as age, gender, and creatinine clearance–a measure of kidney function that can be a valuable predictor of metabolism–to predict drug pharmacokinetics.

Using findings from this research, Dr. Hahn adds, Children’s National already has implemented an algorithm using different variables to determine antibiotic dosing for patients treated at the hospital.

Original article here.

Antibiotic plus probiotic combination may kill off superbugs

By 

Every year, over 2 million people in the United States develop infections that are resistant to treatment, and approximately 23,000 people die as a result.

These statistics have prompted the Centers for Disease Control and Prevention (CDC) to deem drug resistance “one of the biggest public health challenges of our time.”

Therefore, researchers are hard at work trying to develop ingenious ways of tackling so-called superbugs — bacteria that have become immune to antibiotic treatment.

Lately, researchers have added probiotics to their arsenal against superbugs. Probiotics are beneficial bacteria found in foods, such as yogurt, kefir, pickles, or miso soup.

Only a month ago, for example, a study suggested that simply consuming probiotics on a regular basis could reduce the need for antibiotics, thus helping to curb the drug resistance crisis. Continue reading Antibiotic plus probiotic combination may kill off superbugs

Check out Jerry Cahill’s latest Cystic Fibrosis Podcasts

Cystic Fibrosis Podcast 196: Managing CF as a Track Athlete

In Jerry Cahill’s latest video podcast, we meet Teena Mobley – a Long Island University graduate and former track athlete. Diagnosed at 9 years old, Teena’s life changed as she adjusted to her new medication schedule while staying dedicated to fitness and health. She shares how exercise helps her manger her illness, remain positive, and continue to believe in herself.
This video podcast was made possible through an unrestricted educational grant from the Allergan Foundation to the Boomer Esiason Foundation.

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Cystic Fibrosis Podcast 197: Moving from Middle School to High School

In Jerry Cahill’s latest cystic fibrosis video podcast, he spoke with Aidan Neville – a 13-year-old with CF who started 9th grade this year. He has two siblings, 22 cousins, and many more who make up his support system. While Aidan loves to stay active – surfing, playing football, and basketball – his exercise sometimes takes a backseat to his disease.
Because he is often on IVs and has had over 24 sinus surgeries, Aidan’s biggest concern about starting high school is missing class and that possibly negatively impacting his chances to go to the college of his choice.
Tune in to learn more about Aidan and his journey from middle school to high school.
This video podcast was made possible through an unrestricted educational grant from Gilead to the Boomer Esiason Foundation.

Telavancin Promising Potential Treatment Option for MRSA in Cystic Fibrosis Patients

By Kristi Rosa

Responsible for several issues ranging from skin infections and sepsis to pneumonia and bloodstream infections, methicillin-resistant Staphylococcus aureus continues to plague patients in the health care and community setting, as well as the providers who treat them.

When acquired in patients with cystic fibrosis, clinical outcomes are known to be even worse, affecting several organs—primarily the lungs—and resulting in an increased rate of declined respiratory function as well as infections that can have severe, and sometimes deadly, consequences.

Now, however, for the first time, investigators have found that telavancin—a drug that is currently used to treat skin infections and hospital-acquired pneumonia—has potent in vitro activity and low resistance development potential when used against S aureus isolates in patients with cystic fibrosis, making it a promising potential treatment option for this population.

“Telavancin (TLV) is a lipoglycopeptide antibiotic approved by the US Food and Drug Administration in 2009 for the treatment of complicated skin and skin structure infections and in 2013 for the treatment of cases of nosocomial pneumonia, however its application for the treatment of CF-MRSA pneumonia infections was not known, so our studies are contributing to extending the application of TLV for CF treatment,” Adriana E. Rosato, PhD, associate professor in the department of Pathology and Genomic Medicine at Houston Methodist Research Institute told Contagion®. “We were also inspired by the fact that CF patients have a short life time—until 40 to 50 [years]—so our priority is to contribute to better treatment in this patient population.”

Dr. Rosato and her team hypothesized that TLV might be a promising treatment option for CF-patient-derived MRSA and MSSA infections, as in vitro studies have shown that TLV has activity against MRSA.

To prove this, the investigators screened a total of 333 strains of CF patient-derived S aureus of the wild-type or small-colony-variant phenotype, collected from both adults and children at 3 different cystic fibrosis centers: Houston Methodist Research Institute, UW Health and the Center for Global Infectious Disease Research. TLV was found to display activity against all 333 strains collected.

When testing the activity of the drug against 23 MRSA strains, the investigators observed intermediate resistance to ceftaroline (CPT)—a new beta-lactam antibiotic that targets PBP 2a in MRSA—in 20 of the strains, and high-level resistance to CPT in 3 of the strains. The authors note that although high levels of resistance to CPT is rare, intermediate resistance is more common in patients who have chronic infections.

“Among all strains, the TLV MIC90 was 0.06 mg/liter, i.e. 8-fold lower than the daptomycin (DAP) and CPT MIC90 and 25-fold lower than the linezolid (LZD) and vancomycin (VAN) MIC90,” the authors write.

Using time-kill experiments, the investigators assessed the in vitro effectiveness of TLV compared with DAP, VAN, and CPT. They found that TLV showed activity against all tested strains and displayed rapid bactericidal activity as well. The activity profile for the drug at a free serum concentration of 8 mg/liter showed that TLV performed better than VAN (16 mg/liter), LZD (10.4 mg/liter), and CPT (16 mg/liter).

The investigators also set out to determine the fate of mutation selection that could be projected by the potential prolonged use of TLV in patients with cystic fibrosis. To do this they looked at 3 specific strains: AMT 0114-48, WIS 664, and TMH 5007. They found that due to the ease of mutation selection which had been noted in control strains, TLV mutant resistance is independent of the CF patient background of the strains.

“We demonstrated that TLV has bactericidal activity against the S aureus strains tested, including those against which CPT and LZD displayed reduced activity, which might provide TLV a significant advantage over the drugs currently used to eradicate those strains and prevent future exacerbations,” the study authors write.

A clinical trial is currently underway to assess the pharmacokinetic profile of TLV in patients with cystic fibrosis, who usually need dose adjustment because of an increase in the volume of distribution and clearance.

“[The next step for our research is] to perform in-vivo analyses studies that could lead to translational application/clinical trial,” Dr. Rosato added. “However, we are limited in research funds to continue our investigations.”

Original article here.

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

I Have Cystic Fibrosis, and CF Has Me

This Lung Life By Ella Balasa

I hear others say “I have CF. CF doesn’t have me.” This may be an accurate statement for some, the small percentage of patients who are not limited by this disease. Those who climb mountain peaks, work 60 hours a week, and raise three children. They could say this statement is true. They conquer everything, despite CF.

I am not one of these patients. I am optimistic, though. I’m optimistic that one day I will sprint faster than you (with transplanted lungs). I’m optimistic that I will leave this world having made some kind of impact on those around me, and maybe others that I am unaware of. But with this DNA in the cells of my lungs, I can’t do it all.

I’ve had significant events and minute moments in my life that have been affected by CF, although it’s not always apparent to the world around me. However, I don’t claim that CF has altered my life for the worst. Instead, I show the reality.

CF had me most recently when I was planning to go to the Cystic Fibrosis Research Inc.’s Family Education Conference. Being a director for the U.S. Adult Cystic Fibrosis Association, I wanted to connect with fellow CF directors and hear about the amazing new research the CF community is eager to benefit from. Unfortunately, due to CF infection guidelines and the bacteria I harbor in my lungs, I posed a risk to other CF patients, so I was restricted from attending.

Recently, as my form of exercise, I have been playing tennis. CF has me when it grasps my airways after just a few serves. I feel my lungs expanding but not getting enough air, exhausted from a previous sprint of just a few feet. I watch as the ball spins toward the far corner of the court. In my mind, my legs are in the air moving toward it, but in reality, they have just elevated the sole of my foot for the first step. The muscles are depleted of oxygen, waiting for the next burst for them to spring into action, but it never comes. Instead, they continue straining with what little reserve they have, for one-quarter of their potential. The quarter that comes from the lungs that function at one-quarter of what they should.

CF dictated the direction my life would take when upon graduation I was offered my dream job, but I didn’t take that career path. Spending four hours a day on breathing treatments, attending frequent doctor’s appointments, having occasional hospital stays and health insurance factors, as well as maintaining a social life and community involvement weren’t conducive to a full-time working schedule. Choosing not to advance in my career as my peers did made me feel left behind. Instead, keeping my health as the focus, I chose part-time employment.

CF has me when I have an exacerbation and lots of congestion in my lungs. On occasion during these times, I’ve taken the flight of stairs from the basement out into the sunshine after work. After a few steps outside, I feel the absence of air in my lungs. I gasp and then panic. Continue the article here. 

AIT’s Inhaled Nitric Oxide Shows Potential in Fighting Bacterial Infection Prevalent in CF Patients

By Alice Melao

Inhaled nitric oxide (NO) was shown to be an effective antibacterial agent against Mycobacterium abscessus infection in preclinical studies, as well as in a pilot clinical trial, according to AIT Therapeutics.

The company discussed the latest data on its NO product in two poster presentations during the 3rd Annual World Bronchiectasis Conference held recently at Georgetown University in Washington, D.C.

NO is a small molecule that is an important mediator of immune defense mechanisms against infections. The compound has been shown to have broad-spectrum antibacterial activity against several strains of bacteria that often infect patients with underlying lung diseases, including cystic fibrosis (CF).

Continue reading AIT’s Inhaled Nitric Oxide Shows Potential in Fighting Bacterial Infection Prevalent in CF Patients

Positive Data from the CARE CF 1 Clinical Study of Oral Lynovex in Cystic Fibrosis Exacerbations

NovaBiotics Ltd (“NovaBiotics”) announces that its oral therapy for cystic fibrosis (CF), Lynovex®, has met the study objectives of the CARE CF 1 clinical trial.

CARE CF 1 assessed the effects of two weeks of Lynovex treatment as an adjunct to standard of care therapy (SOCT) in CF, compared to placebo plus SOCT. This trial was designed to determine whether the inclusion of Lynovex capsules alongside SOCT lessened the clinical impact of exacerbations in adults with CF, as measured by symptom severity and levels of bacteria and inflammatory mediators in sputum and blood.  CARE CF 1 was a 6-arm study with the primary objectives of determining the optimal dose and regimen of Lynovex in patients with exacerbations of CF-associated lung disease and to further evaluate the safety and tolerability of Lynovex in exacerbating CF patients.  Continue reading Positive Data from the CARE CF 1 Clinical Study of Oral Lynovex in Cystic Fibrosis Exacerbations

Toothpaste ingredient may bust up cystic fibrosis biofilms

By Chris Waters and Sarina Gleason

A common antibacterial substance in toothpaste may combat life-threatening diseases such as cystic fibrosis when combined with an with an FDA-approved drug, researchers report.

Researchers have found that when triclosan, a substance that reduces or prevents bacteria from growing, combines with an antibiotic called tobramycin, it kills the cells that protect the CF bacteria, known as Pseudomonas aeruginosa, by up to 99.9 percent.

CF is a common genetic disease with one in every 2,500 to 3,500 people diagnosed with it at an early age. It results in a thick mucus in the lungs, which becomes a magnet for bacteria.

These bacteria are notoriously difficult to kill because a slimy barrier known as a biofilm, which allows the disease to thrive even when treated with antibiotics, protects them.

“The problem that we’re really tackling is finding ways to kill these biofilms,” says Chris Waters, lead author of the study and a microbiology professor at Michigan State University.

According to Waters, there are many common biofilm-related infections that people get, including ear infections and swollen, painful gums caused by gingivitis. But more serious, potentially fatal diseases join the ranks of CF including endocarditis, or inflammation of the heart, as well as infections from artificial hip and pacemaker implants.

Waters and his coauthors grew 6,000 biofilms in petri dishes, added in tobramycin along with many different compounds, to see what worked better at killing the bacteria. Twenty-five potential compounds were effective, but one stood out.

“It’s well known that triclosan, when used by itself, isn’t effective at killing Pseudomonas aeruginosa,” says coauthor Alessandra Hunt, a postdoctoral associate of microbiology and molecular genetics. “But when I saw it listed as a possible compound to use with tobramycin, I was intrigued. We found triclosan was the one that worked every time.”

Triclosan has been used for more than 40 years in soaps, makeup, and other commercial products because of its antibacterial properties. Recently, the FDA ruled to limit its use in soaps and hand sanitizers due to insufficient data on its increased effectiveness and concern about overuse. Clear evidence has shown, though, that its use in toothpaste is safe and highly effective in fighting gingivitis, and it is still approved for use.

“Limiting its use is the right thing to do,” says coauthor Michael Maiden, a graduate student in medicine. “The key is to avoid creating resistance to a substance so when it’s found in numerous products, the chances of that happening increase.”

Tobramycin is currently the most widely used treatment for CF, but it typically doesn’t clear the lungs of infection, Waters says. Patients typically inhale the drug, yet still find themselves chronically infected their whole lives, eventually needing a lung transplant.

“Most transplants aren’t a viable option though for these patients and those who do have a transplant see a 50 percent failure rate within five years,” he says. “The other issue is that tobramycin can be toxic itself.” Known side effects from the drug include kidney toxicity and hearing loss.

“Our triclosan finding gives doctors another potential option and allows them to use significantly less of the tobramycin in treatment, potentially reducing its use by 100 times,” Hunt says.

Within the next year, Waters and his colleagues will begin testing the effectiveness of the combination therapy on mice with hopes of it heading to a human trial soon after since both drugs are already FDA approved.

Just brushing your teeth with toothpaste that has triclosan won’t help to treat lung infections though, Maiden says.

“We’re working to get this potential therapy approved so we can provide a new treatment option for CF patients, as well as treat other biofilm infections that are now untreatable. We think this can save lives,” he says.

The research appears in the journal Antimicrobial Agents and Chemotherapy.

The National Institutes of Health, Cystic Fibrosis Foundation, and Hunt for a Cure in Grand Rapids, Michigan funded the research.

Source: Michigan State University