Cystic fibrosis updates, research & treatments

waltky · Aug 23, 2012

Could help asthma patients too...

'Brush' offers clues to fighting lung disease
23 August 2012 - In people with cystic fibrosis, mucus is not properly cleared from the lungs

Scientists say the discovery of an internal "brush" that helps clear lungs of unwanted matter could help them understand more about lung diseases. A team from the University of North Carolina found that the brush-like layer pushes out sticky mucus and the foreign bodies it contains. Writing in Science, it says that could help identify what goes wrong in cystic fibrosis, asthma and similar diseases. UK lung experts said the work aided understanding of how lungs function. The mucus, which helps collect inhaled pollutants, emerges as a runny nose and a wet cough.

Until now, most experts believed a watery substance acted as a lubricant and helped separate mucus from the cells lining airways. But this did not tally with the fact that mucus remained in its own distinct layer. The researchers used imaging techniques to examine what was happening within the lungs. They were able to see a dense meshwork of human bronchial epithelial cell cultures. The brush-like layer consists of protective molecules that keep sticky mucus from reaching the cilia and epithelial cells, thus ensuring the normal flow of mucus.

'Flood' risk

Dr Michael Rubinstein, who led the study, said: "The air we breathe isn't exactly clean, and we take in many dangerous elements with every breath. "We need a mechanism to remove all the junk we breathe in, and the way it's done is with a very sticky gel, called mucus, that catches these particles and removes them with the help of tiny cilia." "The cilia are constantly beating, even while we sleep. "In a co-ordinated fashion, they push mucus, containing foreign objects, out of the lungs, and we either swallow it or spit it out. "These cilia even beat for a few hours after we die. If they stopped, we'd be flooded with mucus that provides a fertile breeding ground for bacteria." The team says the brush layer protects cells from the sticky mucus and acts as a "second barrier" in case viruses or bacteria penetrate the mucus.

But in conditions such as cystic fibrosis or chronic obstructive pulmonary disease (COPD), the "brush" fails to work properly, they suggest, becoming squashed and trapping mucus, which then becomes stuck to cells. Dr Rubenstein said: "The collapse of this brush is what can lead to immobile mucus and result in infection, inflammation and eventually the destruction of lung tissue and the loss of lung function." He said the findings should help inform new research into such lung conditions. Prof Stephen Spiro, vice-chairman of the British Lung Foundation, said: "Mucus has a complex biological make-up and forms a vital part of the lungs' defence mechanism against potentially harmful or irritating substances, which are inhaled as small particles. "Research such as this helps our understanding [of] how this system works, and of the complex mechanisms deep within our lungs which protect us from the atmosphere we breathe in."

http://www.bbc.co.uk/news/health-19357090
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waltky · Oct 29, 2012

Such ignorance from so-called 'educated' people...

Boy kicked out of school because he has gene for cystic fibrosis
A California boy has been ordered to transfer to another middle school because he carries the gene for cystic fibrosis, even though he doesn't actually have the incurable, life-threatening and non-infectious disease. His parents have gone to court to fight the move.

Their son, 11-year-old Colman Chadam, was told last week that hed have to transfer from Jordan Middle School in Palo Alto, Calif., to a school three miles away because he posed a risk to another student at school who does have the disease, according to TODAY. I was sad but at the same time I was mad because I understood that I hadnt done anything wrong, Colman told TODAY. He added: It feels like Im being bullied in a way that is not right.

An inherited condition, cystic fibrosis causes the body to create a thick mucus that clogs the lungs and can lead to life-threatening lung infections. About 30,000 American adults and children have the disease and patients have an average life expectancy in the late 30s. While it is not contagious, doctors say people with cystic fibrosis can pose a danger to each other through bacterial cross-contamination if they are in close contact. In general, we would prefer that there not be more than one cystic fibrosis patient in a school, Dr. Thomas Keens, the head of the cystic fibrosis center at Childrens Hospital Los Angeles, told TODAY.

The districts assistant superintendent, Charles Young, told NBC News that officials relied on medical authorities who said a literal physical distance must be maintained between patients and that the "zero risk option" was to transfer Colman. But Colmans parents, who are home-schooling him while they await a court hearing next week, say the school is overreacting. Why take a child whos new to the district, whos just making friends, whos just building a support network, whos just getting to know his teachers, whos been well his whole life ... why stigmatize him? his father, Jaimy Chadam, said on TODAY.

Jennifer Chadam said her son has attended two other schools with students who have cystic fibrosis. It has never been an issue. Ever, she said. Colmans parents told the school about his condition on a form at the start of the school year, the San Francisco Chronicle reported. Colman has not suffered from lung problems, never needed treatment and had a negative result on a sweat test, the most accurate test for the disease, his parents told the Chronicle last week. They told the paper their son has never had a clinical diagnosis of cystic fibrosis.

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Richard Powell · Nov 5, 2012

Asthma has emerged as a problem in many regions of country.Therefore many center has presented their claim in fast and treatment of this problem but they fail.This post has presented few information which might help people suffering from this problem

waltky · Apr 2, 2013

The infection has been on the rise over the past decade in patients...

Cystic fibrosis bug 'can spread between patients'
30 March 2013 - A dangerous infection which is becoming more common in people with cystic fibrosis can spread between patients, UK researchers say in The Lancet.

Doctors previously thought the Mycobacterium abscessus bacteria could only be caught from water and soil. But hospitals around the world may now have to change the way patients are treated, the study says. Around 3-10% of cystic fibrosis patients in Europe and the US are infected with the hard-to-treat bug. There are around 9,000 people with cystic fibrosis in the UK although around one-in-25 people carries the faulty gene which causes the condition.

It affects the internal organs, especially the lungs and digestive system, by clogging them with thick sticky mucus which makes it hard to breathe and digest food. Researchers writing in The Lancet do not know exactly why Mycobacterium abscessus - which is distantly related to the bacteria that causes tuberculosis - is more likely to infect people with cystic fibrosis but it could be related to problems with the immune system.

It causes lung damage, and can be incredibly hard to treat with infected patients needing months of treatment with toxic drugs. Although the infection has been on the rise over the past decade, doctors always believed it could not spread between humans. But by looking at DNA from almost 170 samples of the bacterium, and using that to create a family tree, researchers found that it can indeed spread from person to person.

Infection control
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waltky · Jun 13, 2013

Mebbe dey'll find a cure in the next 10 years...

New lungs buy time but don't cure cystic fibrosis
13 June`13 WASHINGTON (AP) The 10-year-old Pennsylvania girl who fought for a lung transplant has a difficult journey ahead. The transplant isn't a cure for her cystic fibrosis, and new lungs don't tend to last as long as other transplanted organs.

But it can extend life by years, buying some time. "You're keeping them alive and hopefully well, hoping that something else will come along that will make the big difference," said Dr. Anastassios Koumbourlis, pulmonary chief at Children's National Medical Center in the nation's capital. Sarah Murnaghan, who is recovering from Wednesday's operation at the Children's Hospital of Philadelphia, made headlines as her parents challenged national policy over how children under 12 are placed on the waiting list for donated lungs. Lost in the debate over how to give out scarce organs was this broader question: How well do children with cystic fibrosis fare when they do get a new set of lungs?

Fortunately, few children get sick enough anymore to need transplants, said Dr. Stuart Sweet, pediatric lung transplant chief at Washington University in St. Louis. Treatments for the genetic disease have improved so much over the past decade that patients live much longer before their lungs start to wear out. About 30,000 Americans live with cystic fibrosis, which causes sticky mucus to build up in the lungs, leading to life-threatening infections in the lungs and problems in other organs. Only a few decades ago, children with the disease seldom survived elementary school. Now the typical life expectancy is about 37 years and growing.

A 2007 study published in the New England Journal of Medicine prompted major controversy over whether lung transplants offered enough survival benefit to be used for cystic fibrosis. Ultimately, doctors decided it did, for the right patient who is out of options. Since then, about 150 to 200 people with the disease, mostly teens and adults, have gotten lung transplants every year, according to a patient registry run by the Cystic Fibrosis Foundation. Over 80 percent of patients who get new lungs survive a year, and over 50 percent are alive after five years, the registry shows.

That's a sobering statistic, although some people survive much longer. For comparison, well over 90 percent of people who receive a kidney transplant survive five years. "We expect it will be a long road, but we're not going for easy, we're going for possible," Sarah's family said in a statement after her surgery. Sweet said the issue isn't the cystic fibrosis but that lungs simply are difficult to transplant, no matter what the underlying disease. "The reality is that lung transplantation is not a perfect solution," Sweet said.

After all, "this is the only organ we transplant that's in contact with the outside world," added Dr. Karen McCoy, pulmonology chief at Nationwide Children's Hospital in Columbus, Ohio. For cystic fibrosis patients, the donated lungs don't contain the defective gene that caused their own lungs to clog so they won't fill with mucus again. Cystic fibrosis will continue to damage their pancreas, intestines and other parts of the body, requiring ongoing treatment to deal with nutritional problems and other symptoms.

More http://news.yahoo.com/lungs-buy-time-dont-cure-cystic-fibrosis-213114899.html
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dudeman · Jun 20, 2013

I found this transplant to be insane. If cystic fibrosis is genetic in origin, how can replacement lungs "solve" the problem? I can see a replacement for a person with a one-time inhalation or aspiration incident (i.e. environmental exposure). There should have been a lot more validation before this political load of crap was allowed to occur and displace the opinions or people knowledgeable in the medical field.

waltky · Feb 5, 2016

FDA won't allow expanded use of CF drug...

FDA rejects expanded use of Vertex's cystic fibrosis drug
5 Feb.`16 - The U.S. Food and Drug Administration has denied the expanded use of Vertex Pharmaceutical Inc's bestseller, Kalydeco, in certain cystic fibrosis patients, the company said on Friday.

The regulator rejected the company's application for using the drug in cystic fibrosis patients aged 2 or older, who have one of 23 residual function mutations. The company's shares fell 7.7 pct to $9.60 in premarket trading.

Cystic fibrosis is caused by a defective gene that disrupts the function of the lungs and digestive system, producing a build-up of thick, sticky mucus leading to inflammation and recurrent bacterial infections. Half of the people with cystic fibrosis live longer than 28 years, according to the World Health Organization.(https://bit.ly/1OqUj6Z)

http://news.yahoo.com/fda-rejects-expanded-vertex-cystic-fibrosis-drug-140802932--finance.html
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DennisTate · Feb 13, 2016

dudeman said: ↑

I found this transplant to be insane. If cystic fibrosis is genetic in origin, how can replacement lungs "solve" the problem? I can see a replacement for a person with a one-time inhalation or aspiration incident (i.e. environmental exposure). There should have been a lot more validation before this political load of crap was allowed to occur and displace the opinions or people knowledgeable in the medical field.
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A Dr. Joel Wallach is convinced that there is also a nutritional deficiency at work.

http://www.youngevity.net/category/dwj.html

DR. JOEL WALLACH , BS, DVM , ND

Dr Wallach was born and raised in Missouri , attended the University of Missouri, where he received a Bachelor's degree in Science of Agriculture.

A veterinarian for 30 years, he worked as a research veterinary pathologist with The National Institute of Health for The Center for the Biology of Natural Systems, Washington University, St. Louis, Missouri; Yerkes Regional Primate Research Center in Atlanta, Georgia, at Emory University.

Having discovered and identified the first animal models for cystic fibrosis- monkeys - he found that he could reproduce their condition at will because it was a nutritional deficiency. His find offered great promise for children with the disease, but when he made it public, the institute fired him on 24- hours notice.

Chagrined and perplexed, Dr. Wallach made the decision to go to medical school so he could treat children for cystic fibrosis himself.
Realizing that traditional medical school was likely to be as repressive as the National Institute he accepted an invitation to teach nutrition at The National College of Naturopathic Medicine in Oregon , not even knowing what a naturopathic doctor was.

When he learned that he could be a primary care physician-deliver babies, sew up chainsaw wounds, write prescriptions and get paid by insurance- all through a program that encouraged interest in nutrition, he simultaneously enrolled as a full-time student. Soon people were flocking to the college clinic.

BACKGROUND INFORMATION AND PARTIAL BIBLIOGRAPHY FOR DR. JOEL WALLACH , BS, DVM , ND

Dr. Wallach 's research has resulted in the publication of more than 70 peer review and refereed journal articles in the fields of nutrition and pharmaceutical research, and he has made major contributions to eight multi- authored text and reference books on these subjects. The following is a general summary of his educational and biomedical research credentials:
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waltky · Sep 22, 2016

Sad story of a young couple with cystic fibrosis...

Terminally ill US couple die days apart
Thu, 22 Sep 2016 - A young married couple who bonded through their shared struggles with cystic fibrosis have both died within days of each other.

A young US married couple who bonded through their shared struggles with cystic fibrosis have both died within days of each other. Katie Prager, 26, died in Kentucky on Thursday, five days after the death of her husband, Dalton Prager, 25. Katie died in bed on Thursday morning, her mother said wrote on Facebook. "I know Dalton was waiting with open arms, as well as both her grandmothers and a host of family and friends that have gone before her," she wrote. The couple married in 2011 after falling in love on Facebook. Doctors had advised against meeting in person, due to the likelihood that Dalton would pass a highly-infectious disease to Katie.

The young couple on a beach

The couple had been forced to live in separate states in recent years and were last together for their fifth anniversary in July. Katie had lived in Kentucky, while Dalton was living in Missouri. Cystic fibrosis clogs the lungs with mucus making it difficult for patients to breath. The median survival age is 40-years-old. They had each received lung transplants, which failed. Dalton had tried desperately but was unable to travel to Kentucky to see Katie one last time before his death. "He has tried so many times and he has tried so hard. Unfortunately his body is not agreeing with what he wants to do," his mother Renee Prager told the St Louis Post-Dispatch. The couple raised money for their treatment, and wrote extensively about their difficult experiences.

Lamenting how he and his wife were treated by health insurance companies, Dalton wrote: "They are turning my wife into a number, a statistic, a dollar sign. I cannot lose her! This can't be the end of our love story! "We are both ready to continue fighting but at this point we are running out of options and need your help. Please help me save my wife Katie! "We knew her time was short and she was able to do a few things that she wanted," Katie's mother Debbie wrote. "The days to follow will not be easy but I find comfort in knowing that my girl lived, she really lived." The couple were compared to the one in the book and film The Fault In Our Stars, that focuses on a young couple battling cancer.

http://www.bbc.co.uk/news/world-us-canada-37442522
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waltky · Jan 30, 2017

Wonder if it'll work for Cystic Fibrosis?...

Shark-inspired drug may help treat fibrosis, researchers say
Mon, 30 Jan 2017 - Australian researchers hope an antibody found in sharks may help treat an incurable lung disease.

Idiopathic pulmonary fibrosis (IPF) scars lung tissue, causing breathing to become progressively harder. It kills more than 5,000 people each year in the UK alone, according to the British Lung Foundation. Researchers hope a new drug, inspired by an antibody in the blood of sharks, can begin human trials next year. The drug, AD-114, was developed by researchers at Melbourne's La Trobe University and biotechnology company AdAlta.

Initial testing successfully targeted fibrosis-causing cells by creating a human protein that imitated the shark's antibody, according to Dr Mick Foley, from the La Trobe Institute for Molecular Science. "Fibrosis is the end result of a lot of different insults and injuries," he told the BBC. "This molecule can kill the cells that cause fibrosis."

Debilitating symptoms

IPF symptoms include shortness of breath, especially during exercise, which gradually gets worse, and a persistent dry cough. There is currently no cure so treatment focuses on trying to relieve symptoms and slow its progression. The US Food and Drug Administration this month designated AD-114 an "orphan drug" - a move which gives tax breaks to companies attempting to find treatments for diseases.

A wobbegong shark

Dr Foley, who is also AdAlta's chief scientific officer, said the company had raised A$10 million (£6m; $7.5m) since being listed on the Australian Stock Exchange in August. It intends to use the money to take the drug to human trials in 2018. AD-114 does not involve injecting shark blood, which the human body would reject, Dr Foley said.

Other potential uses

In laboratory tests, the drug also showed potential to treat other forms of fibrosis. This included, for example, people suffering from liver disease and age-related eyesight degeneration, Dr Foley said. He added no sharks had been harmed in the process. A single blood sample was extracted from a wobbegong shark at Melbourne Aquarium, ."It would be very nice to say one day that 'this person is alive because of what the sharks told us,'" Dr Foley said.

http://www.bbc.co.uk/news/world-australia-38792405
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