Idiopathic Pulmonary Fibrosis Support Group - Living the South African Experience

To become a member of the Support Group, you must register. There is a box on the top right side of the homepage. Click on the bold link, "Click here" below the login button and complete the registration. Remember that all the info you supply here is strictly confidential.

You can then go to the"navigation" block on the left of the page and exercise one of the following options:

Discussion Forum- Open a new thread
Guest Book - post a message
Blog system - create a blog

Introduction

"We can all be angels to one another. We can choose to obey the still small stirring within, the little whisper that says, Go. Ask. Reach out. Be an answer to someone's plea. You have a part to play. Have faith. We can decide to risk that He is indeed there, watching, caring, cherishing us as we love and accept love. The world will be a better place for it. And wherever they are, the angels will dance."-Joan Wester Anderson

The idea for the creation of this website has originated from a strong need to bring fellow PF sufferers together in a forum where we can communicate with each other in an informal manner, sharing experiences, exchanging knowledge and just spontaneously support each other. The name chosen for the website may need some explanation:

.
Living with pulmonary fibrosis - It is not only the patient/sufferer who lives with the disease, but there are also a support group around him who also have to live with the patient and his disease on a daily basis and who offers care and support to him in one form or another. This group generally consists of a spouse, close family, friends and neighbours, therapists, nursing staff and medical practitioners. The input of all these various support groups will be highly appreciated in this forum so that we may be able to expand our knowledge of dealing with the disease.

The South African experience – I have scoured the world- wide web over a long period of time and could not find a web based support group or communication forum for South African PF sufferers. In the process I have joined several overseas forums. However pleasant it was to communicate with those folks, they have different experiences relating to medical aid, medical facilities, relevant logistical support and also a host of things non-South African. We as South Africans can relate to each other more meaningful in a country where we all share the same experiences in terms of medical care, -facilities, -policies, -aid/schemes and a multitude of other issues relating to our common disease, i.e., pulmonary fibrosis. People from other countries should however feel free to share their unique experiences with us. After all, we share a common disease which is the same for all IPF sufferers wherever they are in the world.

South Africans suffering from other forms of lung disease are warmly invited to join this forum to participate and interact with fellow members.

Quote:
Without faith, nothing is possible. With it, nothing is impossible

The web-forum has no association with any official body. The possibility of this happening in future can not be excluded, and will depend on its future growth. The vision for this forum include that it may eventually become a mouthpiece for all sufferers of PF and a platform to bring our problems to the attention of the relevant powers in this country of ours.

Contact Details

Website Administrator: Leon Harmse

E-mail Address: admin@ipfsg.org.za or info@ipfsg.org.za

Fax Number:

Kennisgewing

Die belangstelling in die IPF Ondersteuningsgroep is oorweldigend en ek is diep dankbaar daarvoor.

Ek merk ook op dat daar talle Afrikaanssprekendes as lede registreer of slegs net die webblad besoek.

Vertel asseblief jou storie in Afrikaans, dit is tog die taal waarin jy jouself die beste kan uitdruk.

Daar is baie oorsese belangstelling in die webblad en ter wille van hierdie persone sowel as ons eie landgenote wie nie Afrikaans magtig is nie sal die administrateurs die webblad se kommunikasie in Engels voer.

Komaan,laat die lyne gons in Afrikaans!!

Researchers find target for pulmonary fibrosis

A diagnosis of Idiopathic Pulmonary Fibrosis is not much better than a death sentence: there is no treatment and the survival rate is less than three years.

But researchers at the University of Michigan have discovered that targeting of a novel gene utilising genetic and pharmacologic strategies was successful in treating pulmonary fibrosis in mice and will be developed for future testing in humans.
The treatments attack an oxidant-generating enzyme, NOX4, that researchers discovered is involved in the fibrotic process - which involves scar-like tissue formation in an organ such as the lung. The researchers' findings will be published in the September issue of the journal Nature Medicine.

'We've identified the target. We know the enemy now,' said Subramaniam Pennathur, M.D., assistant professor of internal medicine/nephrology. 'This is the first study that shows pulmonary fibrosis is driven by this NOX4 enzyme.
'But what's really significant is this discovery may have relevance to fibrosis in other organ systems, not just the lung.'

So those suffering from common cardiac or kidney diseases, which often involve fibrosis, also may benefit from treatments stemming from this research, Pennathur said.

Pennathur said continued support from the National Institutes of Health will eventually allow researchers to take the treatment to human studies. The University of Michigan also has filed for patent protection and is currently looking for a licensing partner to help bring the technology to market.
The discovery was made in the University of Michigan lab of Victor J. Thannickal, M.D. He was assisted by Louise Hecker, Ph.D., a post-doctoral research fellow.
Thannickal said the study points to a very viable treatment strategy for idiopathic pulmonary fibrosis, and researchers saw success both in mouse models of lung fibrosis and in fibrogenic cells isolated from lungs of patients with Idiopathic Pulmonary Fibrosis.

'It remains to be seen if fibrosis is reversible,' he said. 'But therapeutic targeting of this pathway this may allow us to halt the progression of fibrosis and preserve lung function.'

The lung disease often affects older people, Thannickal said, and its cause is generally unknown. It is possible that cumulative injuries like exposure to environmental toxins and pollutants in genetically susceptible individuals could contribute to causing fibrosis.

There is a gradual scarring of the lung, thickening and contracting the organ until it loses its ability to exchange oxygen with blood, Hecker said. Patients experience extreme fatigue, rapid weight loss, chronic cough and shortness of breath.
There are five million people worldwide that are affected by this disease, according to the Pulmonary Fibrosis Foundation. In the United States there are over 100,000 patients with Pulmonary Fibrosis.

When U-M researchers induced the fibrotic process in the mice, they discovered that the NOX4 enzyme was elevated. By knocking down that enzyme at the genetic level or inhibiting its activity, the fibrosis was stopped, Hecker said.
'So we may be able to halt lung scarring even after the injury has occurred and fibrosis is set in motion,' she said. 'This research provides proof of concept that we can target this pathway for therapeutic benefit, which could potentially be used in humans.'

Both Hecker and Thannickal left U-M this summer for the University of Alabama at Birmingham, but they plan to continue to work with Pennathur and other U-M researchers on anti-fibrotic therapies based on these studies. The patent will stay with U-M.

Source: University of Michigan Health System

Genetic Breakthrough Spells Hope for Lung Fibrosis Patients

WEDNESDAY, July 8 (HealthDay News) -- Genes that can help doctors predict when patients with idiopathic pulmonary fibrosis are becoming seriously ill have been identified by U.S. researchers, who said the findings might help keep patients alive until they can get a lung transplant.
Idiopathic pulmonary fibrosis (IPF) is a lung-scarring disease that progresses slowly and causes a gradual decline in lung function. There is no cure or effective treatment for IPF, and median survival is about three years. However, some patients experience a more rapid deterioration.
"Approximately 10 percent of patients develop an acute phase that in most cases is lethal," senior study author Dr. Naftali Kaminski said in a news release. She is director of the interstitial lung diseases center at the University of Pittsburgh School of Medicine and the University of Pittsburgh Medical Center.
In a study designed to learn more about the molecular mechanisms of accelerated IPF, the researchers compared the gene activity profile of the lungs of eight IPF patients who were experiencing dramatic worsening of their disease when they died, 23 stable IPF patients and 15 people with healthy lungs.
Differences in the expression of nearly 600 genes were noted between IPF patients with accelerated disease and those with stable disease. The researchers found no evidence that infection or inflammation was the cause of accelerated IPF. They did find signs that the cells of the alveolar epithelium, the tissue that covers the surface of the lung's air sacs, were rapidly dying.
"That could mean that drugs that are used to protect the epithelium in other illnesses, such as cancer, might help IPF patients survive an exacerbation. If we can keep them alive, there's a chance they could get a lifesaving lung transplant," study co-author Dr. Kevin Gibson, an associate professor in the pulmonary, allergy and critical care medicine division at the University of Pittsburgh School of Medicine and medical director of the interstitial lung diseases center, said in the release from the school.
The study appears in the July 15 issue of the American Journal of Respiratory and Critical Care Medicine.

Copyright © 2009 ScoutNews, LLC. All rights reserved.

Pitt team first to profile genes in acutely ill idiopathic pulmonary fibrosis patient

PITTSBURGH, July 7 – The first findings from a one-of-a-kind, patient-driven effort to provide lung tissue for research might help doctors predict when patients with idiopathic pulmonary fibrosis (IPF) are becoming dangerously ill and also could point the way to interventions that could sustain them until life-saving transplants can be performed.

According to senior author Naftali Kaminski, M.D., associate professor of medicine, computational biology and pathology, and director of the Dorothy P. and Richard P. Simmons Center for Interstitial Lung Diseases at the University of Pittsburgh School of Medicine and UPMC, the research published today in the American Journal of Respiratory and Critical Care Medicine addresses a dilemma in IPF care that currently is unsolved.

"Approximately 10 percent of patients develop an acute phase that in most cases is lethal," Dr. Kaminski explained. "There has been very little understanding of the molecular basis of this syndrome, but because of the dedication of our patients and their families, we are getting closer to some answers."

For most patients, the lung-scarring disease progresses gradually and lung function slowly deteriorates. But there is neither a cure nor effective treatment, so median survival is about three years. For unknown reasons, some IPF patients experience rapid declines that cause diffuse damage of the lung alveoli, the tiny sacs where the exchange of oxygen and carbon dioxide occurs.

To better understand the molecular mechanisms of disease exacerbation or, as Dr. Kaminski puts it, acceleration, the researchers compared the gene activity profile of the lungs of eight IPF patients whose disease was dramatically worsening when they died with those of 23 stable IPF patients and 15 people with healthy lungs.

In the first analysis of its kind, "gene activity patterns were found to be more similar among all IPF patients compared to healthy people," said lead author Kazuhisa Konishi, M.D., a visiting postdoctoral fellow in Dr. Kaminski's lab who performed the gene profiling. "But nearly 600 genes were differentially expressed between IPF patients who had accelerated disease and those who were stable."

There was no evidence that infection or inflammation was the cause of disease acceleration, he noted. Instead, there were indications that the cells of the alveolar epithelium, which is the tissue that covers the surface of the air sacs, were rapidly dying.

"That could mean that drugs that are used to protect the epithelium in other illnesses, such as cancer, might help IPF patients survive an exacerbation," said study co-author Kevin Gibson, M.D., associate professor in the Division of Pulmonary, Allergy and Critical Care Medicine at Pitt School of Medicine and medical director of the Simmons Center. "If we can keep them alive, there's a chance they could get a life-saving lung transplant."

To test whether the changes in the lungs could be revealed in the blood, the Pitt investigators contacted Dr. Kaminski's longtime collaborator, Dong Soon Kim, M.D., a renowned IPF researcher at Asan Medical Center and the University of Ulsan in Seoul, South Korea, who has been at the forefront of studying the acceleration syndrome. With her help, they found that levels of a protein called alpha-defensin were particularly high in the blood of patients experiencing an exacerbation. If the findings are verified with more research, which is underway, the proteins could be the first biomarker blood tests that doctors could track to identify patients at risk for sudden deterioration of lung function.

"This work opens an important window into the mystery of why patients with lung fibrosis suddenly decompensate and how to identify these patients for more aggressive therapies," said Mark T. Gladwin, M.D., chief of Pitt's Division of Pulmonary, Allergy and Critical Care Medicine. "Our current research efforts in the division focus on the development of novel therapeutics that will target the molecular pathways identified by our basic science laboratories."

If not for the altruism of IPF patients, the research and its promising results would not have been possible, Dr. Kaminski noted. The IPF tissue samples used for the study were collected through the Simmons Center's warm autopsy program, which is the only one for lungs in the world.

Several years ago, an IPF patient told Kathleen Lindell, Ph.D, R.N., the Simmons Center nurse, a quality of life researcher and a study co-author, that he wanted to aid research efforts by donating his lungs to science after death. In response, she developed the warm autopsy program.

"The tissue has to be collected within six hours of death, so it demands a great deal of flexibility and commitment on the part of caregivers and family," Dr. Lindell said. "The gene components of the lung cells degrade very quickly, so without the warm autopsy protocol, we couldn't have done the activity profiling that was the foundation of this research."

###

Other study authors include Thomas J. Richards, Ph.D., Yingze Zhang, Ph.D., Simmons Center; Rajiv Dhir, M.D., Michelle Bisceglia, and Samuel A. Yousem, M.D., Department of Pathology; Sebastien Gilbert, M.D., Department of Thoracic Surgery, all from the Pitt School of Medicine; and Jing Woo Song, Asan Medical Center, University of Ulsan, Seoul, South Korea.

The research was funded by the National Institutes of Health and by the Dorothy P. and Richard P. Simmons Endowed Chair for Interstitial Lung Diseases.

Air Travel safe for people with Lung Disease

Last Updated: 2009-05-26 14:01:45 -0400 (Reuters Health)

NEW YORK (Reuters Health) - People with lung disease can travel safely on commercial flights, but should be tested first to determine if they will need extra oxygen on the trip, new research in the journal Respirology confirms.

There's significantly less oxygen in the air people breathe on an airplane compared to the air on the ground. Most studies looking at how people with lung disease respond to these conditions have focused on obstructive lung disease, such as emphysema or bronchitis, Dr. Paul T. Kelly of Christchurch Hospital in New Zealand and colleagues note. It's not clear, they add, whether people with non-obstructive lung disease, in which their airflow is not physically obstructed, but their ability to diffuse oxygen into the body is impaired, might respond differently.

To investigate, Kelly and his team followed 14 people with non-obstructive lung disease on flights ranging from 1 to 10 hours long. They tested the study participants' oxygen saturation, a relative measure of the amount of oxygen in their blood, and other measures of respiratory function before the flight, and also checked in-flight oxygen saturation. After the flight, all were given a hypoxia challenge test, which measures how well the body is able to respond to low oxygen conditions.

All of the study participants experienced a drop in their oxygen saturation during the flight, which was greater for people who walked around while on the plane. These individuals, however, only had "minimal" symptoms of respiratory problems. The strongest predictor of a person's in-flight oxygen saturation was his or her oxygen saturation before flying, the researchers found.

The British Thoracic Society recommends that patients with respiratory disease have an oxygen saturation test before flying, Kelly and his team note. According to the Society, people with oxygen saturation levels between 92 percent and 95 percent should have a hypoxia challenge test to determine if they need supplemental oxygen, while those with oxygen saturation below 92 percent will definitely need oxygen.

Only two of the patients in the current study would have benefited from in-flight oxygen, Kelly and his colleagues note; both had pre-flight oxygen saturation below 92 percent. Relying strictly on the British Thoracic Society guidelines would have resulted in a number of "false positives," according to the researchers. Nevertheless, they add, the guidelines are "practical and uncomplicated."

Another study in the same issue of the journal looked at the policies of 54 different airlines on in-flight oxygen for people with lung disease. Lead investigator Dr. Jacqueline Walker of the Christchurch School of Medicine and her colleagues found the 43 of the airlines "were able to support passengers requiring in-flight oxygen."

Six airlines offered the oxygen for free, but three airlines charged passengers for an extra seat. Fifteen of the airlines charged passengers per cylinder of oxygen used, while 14 charged a flat rate.

The airlines varied widely in their ability to provide service and information to patients who needed oxygen, Walker and her team found. They rated Quantas the most "user friendly," while Olympic Airlines was among the worst; staff were "unsure about costs and unwilling to provide any information without a flight reservation," according to the researchers.

"The findings highlight inconsistencies in airline policies and substantial cost differences for supplemental in-flight oxygen," they conclude. "We advocate an industry standardization of policy and cost of in-flight oxygen."

SOURCE: Respirology, April 5, 2009

New Prognostic Indicator for Patients With Idiopathic Pulmonary Fibrosis

NEW YORK -- February 20, 2009 -- There may be a new way to predict mortality in patients with idiopathic pulmonary fibrosis (IPF), according to a study published in the March issue of the American Journal of Respiratory and Critical Care Medicine.

The study found that maximal oxygen uptake during exercise, or VO2max, can predict mortality in patients with IPF.

"There is no effective treatment [for IPF] and many patients, if eligible, are referred for lung transplantation," said lead author Charlene Fell, MD, Division of Respiratory Medicine, University of Calgary, University of Calgary, Alberta. "Identification of surrogate short-term measures of mortality is critical to the management and study of patients with IPF."

Dr. Fell and colleagues performed a retrospective analysis of 117 patients in the Pathobiology of Fibrotic Lung Disease database from the University of Michigan, Ann Arbor, Michigan, and analysed their oxygen uptake during exercise with their subsequent mortality.

"We found that VO2max examined as a continuous variable does not predict mortality in IPF. However, baseline threshold VO2max of 8.3 mL/kg/min predicts mortality in these patients," said Dr. Fell.

Those patients with a VO2max of less than 8.3 mL/kg/min had a risk of death more than 3 times that of patients whose VO2max was above the threshold. VO2max was compared with 2 other predictors of survival in IPF, oxygen desaturation during a 6-minute walk test and resting arterial oxygen partial pressure, and in both cases, VO2max was found to be a more robust predictor.

"Furthermore, VO2max is easier to use in the clinical setting than other predictors which require cumbersome calculations," Dr. Fell added. "One caveat to the VO2max predictor is that only a small number of patients had a VO2max below the 8.3 mL/kg/min threshold in our study, [so] further prospective studies are needed to validate these findings."

"If the value of this predictor is proven in prospective studies, it may help clinicians prioritise patients for lung transplantation or identify patients for clinical trials."

SOURCE: American Thoracic Society

Protein found to be more common in patients with lung fibrosis

Idiopathic pulmonary fibrosis (IPF) is a scarring disease without a cure, irreparably damaging the ability of the tissue to transfer oxygen into the bloodstream. This disease affects more than five million people worldwide, killing forty thousand of these every year. In a paper in the forthcoming issue of the open-access journal PLoS Medicine, researchers identified a protein called osteopontin that may play a key role in the lethal disease. Identification of this protein could lead to the development of new treatments for IPF.

Annie Pardo and colleagues, based in University Medical Centres in Pittsburgh and Mexico City, took samples from a number of people with IPF in order to analyse the pattern of gene expression in the lungs. They discovered that osteopontin was more prevalent in the lungs of people with the disease than those without it. Examining the role of this protein further, the researchers found that it increased the proliferation and movement of cells involved in lung fibrosis. These findings reinforce previous research that suggested mice are protected from a similar lung disease if they do not have the gene for osteopontin.

The results of the study are important because there is currently no effective medical treatment for this disease. The results suggest that treatment may be possible through drugs specifically directed against osteopontin. By highlighting the role of this protein, the study could also aid the early detection of a disease that is frequently misdiagnosed and little understood.

Citation: Pardo A, Gibson K, Cisneros J, Richards TJ, Yang Y, et al. (2005) Up-Regulation and profibrotic role of osteopontin in human idiopathic pulmonary fibrosis. PLoS Med 2(9): e251.

SOURCE: http://www.plosmedicine.org

A new drug brings hope to IPF sufferers

A California biotechnology company said Tuesday that it would apply for approval of what could be the first drug to treat a fatal lung disease that affects about 100,000 Americans and kills most of them within five years.

The company, InterMune, made its announcement after two late-stage clinical trials produced mixed results in slowing the course of the disease, which is called idiopathic pulmonary fibrosis.

One study showed that patients who took the drug had a significantly smaller loss of lung function after 72 weeks of treatment than those who received a placebo. The other study, with a nearly identical design, did not. But InterMune said that even in the unsuccessful trial there were signs that the drug, called pirfenidone, was helping patients. So the company said it would make the case to the USA Food and Drug Administration and to European regulators that the drug should be approved, given that there are no other approved treatments.

“We believe we have a very strong argument on behalf of patients,” Dan Welch, the chief executive, said in a conference call with securities analysts.

The disease, the cause of which is unknown, is a progressive scarring of the lungs that interferes with the transfer of oxygen into the blood. There are about 30,000 new cases a year in the United States, and only 20 percent of patients live longer than five years from diagnosis, Mr. Welch said.

Dr. Paul W. Noble, a professor at Duke and co-chairman of the committee that designed InterMune’s trials, said in the company’s conference call Tuesday that he was encouraged. “I think you can make a strong case that this is the first step — hopefully, there will be many steps — for this horrible disease,” said Dr. Noble, who is a consultant to InterMune.

Other drugs now being tested as treatments for pulmonary fibrosis include Tracleer from Actelion, Letairis from Gilead Sciences, and Pfizer’s sildenafil, the ingredient in the impotence pill Viagra. Pirfenidone was approved in Japan last year, where it is sold by Shionogi. InterMune said that results from Shionogi’s trial would also be used to help it gain approval in the United States and Europe.

InterMune said that when the results of its own two trials were pooled, pirfenidone reduced the risk of disease progression by about 25 percent. About 7.8 percent of those who got the drug died, less than the 9.8 percent who got the placebo, but that difference was not statistically significant. The main side effects were nausea, a rash, dizziness, fatigue, indigestion and diarrhea.

Adapted from the New York Times article by Andrew Pollack published on 3 February2009

Oxygen- The newest airline fee racket!

by Scott Carmichael Jan 24th 2009

Not content with charging you for water and checked bags, some airlines have discovered the lucrative business of charging for oxygen.

Not to worry, most passengers won't run out of oxygen mid-flight when they don't pay, the fees are being charged for those passengers that require oxygen for medical reasons.

One passenger had already booked $3,500 in tickets with Emirates when the airline notified her that she'd have to pay an additional $4,000 for a single oxygen canister for her husband who suffers from idiopathic pulmonary fibrosis.

Once she created some bad PR for the airline, they gave in, and offered to provide the oxygen for a "mere" $200, but by then she had already rebooked on a different airline.

All is not lost though, several airlines, including British Airways, Virgin and Cathay Pacific provide free oxygen canisters to any passengers that require it for medical reasons. Emirates recently joined that lineup when they announced they too would cease charging for air.

That still leaves plenty of other airlines who feel they can get away with charging between $75 and $350 for medical oxygen. In addition to these unfair charges, most airlines also ban passengers from carrying their own oxygen supplies, citing safety concerns.

One UK charity has already spent just under $70,000 in grants to provide its members with oxygen for their vacation flights.

Source: Gadling.com

Australian scientists develop potential "blockbuster" drug

SYDNEY: Monday Aug 18, 2:10 PM (AFP) - Australian researchers Monday said they had developed a drug which could potentially spell an end to a life-threatening condition caused by diabetes, heart disease and fibrotic illnesses.

Scientists from the University of Melbourne and the city's St Vincent's Hospital said the drug had been shown in animal trials to prevent fibrosis, the build-up of irreversible scarring on internal organs.

There are currently no treatments on the market for fibrosis and the new drug, called FT-11, could be as important a discovery as blood pressure drugs if effective, said Professor Darren Kelly of the University of Melbourne.

"It would be an enormous blockbuster drug with an initial market of around 2.0 billion dollars," he said.

Kelly said while the drug would not prevent diabetes -- a chronic illness in which the body fails to produce enough of the hormone insulin to process sugar -- it could prevent complications such as kidney or heart disease.

"We are hoping to delay or prevent those complications which would basically keep those patients off dialysis -- which would have a huge benefit for their lifestyle," Kelly told AFP.

The drug, expected to be tested in clinical trials within 12 months, could be used to prevent diabetic kidney disease, heart disease and potentially other health problems such as liver and lung fibrosis, he said.

Speaking to the Australian Broadcasting Corporation, Kelly said about 45 percent of diseases in the developed world could be associated with some sort of pathological fibrosis.

"We know at the moment in rat studies that our compound inhibited the development of fibrosis, and the interesting thing in the future would be to see whether we can actually reverse fibrosis," he said.

Agence France Presse.

Not a member yet?
Click here to register.

Forgotten your password?
Request a new one here.

Member Poll

Shoutbox

You must login to post a message.

gerhard van der sandt

01/09/2009 18:32

Ons God is wonderlik

Annelize

24/01/2009 14:05

Dankie Ouboet.. Ek begin so stadig meer en meer leer.