LAM usually affects healthy, non-smoking, young women, according to Elizabeth Henske, MD, director of the Center for LAM Research and Clinical Care. Symptoms, such as shortness of breath, often begin in the late 20s, and can worsen with pregnancy. LAM can lead to lung collapse, oxygen dependency, the need for lung transplantation, and even death in young adulthood.

The center will use the funds to conduct a phase I clinical trial to determine the safety of a combination of two drugs to treat patients with LAM. The drugs that will be tested are hydroxychloroquine and sirolimus. Patients will be given different doses of the medications to find out which are deemed safe and efficacious. In addition to determining safety, the researchers will evaluate effects of the drug combo on patients’ lung function, exercise capacity, kidney tumor size and quality of life.

Independently, hydroxychloroquine and sirolimus treat different conditions. Sirolimus is an immunosuppression drug that is given after organ transplantation, while hydroxychloroquine is used to treat rheumatoid arthritis and lupus.

“This will be the first time that this drug combination has been tested in women with LAM,” said Henske. “We will learn whether the combination of [these] two drugs is safe in women with LAM. In our work in the laboratory, we have seen that this combination of drugs is more effective than either drug alone in models of LAM.”

Henske also added, “This DOD Clinical Trial Award would not have been possible without the LAM Foundation’s and Adler Foundation’s support of our laboratory-based autophagy research.  We’re very excited to have this opportunity to make a difference in LAM.  And so grateful for your support.”

Researchers at Weill Cornell Medical College say they have taken an
important step forward in their quest to “turn on” lung regeneration — an
advance that could effectively treat millions of people suffering from
respiratory disorders.
In the journal Cell, the research team reports that they have uncovered
the biochemical signals in mice that trigger generation of new lung
alveoli, the numerous, tiny, grape-like sacs within the lung where oxygen
exchange takes place. Specifically, the regenerative signals originate
from the specialized endothelial cells that line the interior of blood
vessels in the lung.
While it has long been known that mice can regenerate and expand the
capacity of one lung if the other is missing, this study now identifies
molecular triggers behind this process, and the researchers believe these
findings are relevant to humans.
“Several adult human organs have the potential upon injury to regenerate
to a degree, and while we can readily monitor the pathways involved in the
regeneration of liver and bone marrow, it is much more cumbersome to study
the regeneration of other adult organs, such as the lung and heart,” says
the study’s lead investigator, Dr. Shahin Rafii, who is the Arthur B.
Belfer Professor of Genetic Medicine and co-director of the Ansary Stem
Cell Institute at Weill Cornell Medical College.
“It is speculated, but not proven, that humans have the potential to
regenerate their lung alveoli until they can’t anymore, due to smoking,
cancer or other extensive chronic damage,” says Dr. Rafii, who is also an
investigator at the Howard Hughes Medical Institute. “Our hope is to take
these findings into the clinic and see if we can induce lung regeneration
in patients who need it, such as those with chronic obstructive pulmonary
disease (COPD).”
“There is no effective therapy for patients diagnosed with COPD. Based on
this study, I envision a day when patients with COPD and other chronic
lung diseases may benefit from treatment with factors derived from lung
blood vessels that induce lung regeneration,” states Dr. Ronald G.
Crystal, who is a co-author of this study and professor of pulmonary and
genetic medicine at Weill Cornell.
Dr. Rafii and his researchers had previously uncovered growth factors that
control regeneration in the liver and bone marrow, and in both cases, they
found that endothelial cells produce the key inductive growth factors,
which they defined as “angiocrine factors.” In the current lung study,
they discovered the same phenomenon — that blood vessel cells in the
lungs jump-start regeneration of alveoli. “Blood vessels are not just the
inert plumbing that carries blood. They actively instruct organ
regeneration,” says Dr. Rafii. “This is a critical finding. Each organ
uses different growth factors within its local vascular system to promote
regeneration.”
To conduct this study, Dr. Bi-Sen Ding, a postdoctoral fellow in Dr.
Rafii’s lab and the first author of this paper, removed the left lungs of
mice and studied the biochemical process of subsequent regeneration of the
remaining right lung. Previous pioneering work by Dr. Crystal had shown
that when the left lung of mice is removed, the right lung regenerates by
80 percent, effectively replacing most of the lost alveoli. “This
regeneration process also restores the physiological respiratory function
of the lungs, which is mediated by amplification of various epithelial
progenitor cells and regeneration of the alveolar sacs,” says Dr. Ding.
“This regenerative phenomenon, however, only occurs after a trauma that
abruptly reduces lung mass. Then the specific subsets of blood vessels in
the remaining lung receive a message to start to repopulate alveoli, and
our job was to find that signal,” says Dr. Daniel Nolan, a senior
scientist in this project who developed methods to characterize the lung
blood vessel cells.
The scientists found that removal of the left lung activates receptors on
lung endothelial cells that respond to vascular endothelial growth factor
(VEGF) and basic fibroblast growth factor (FGF-2). Activation of these
receptors promotes the rise of another protein, matrix
metalloproteinase-14 (MMP14). The researchers discovered that MMP14, by
releasing epidermal growth factors (EGF), initiates the generation of new
lung tissue.
When the investigators disabled receptors of VEGF and FGF-2 specifically
in the endothelial cells of the mice, the right lung would not regenerate.
The defect in the lung regeneration was found to be due to the lack of
MMP14 generation from the blood vessels. Remarkably, when these mice
received an endothelial cell transplant from a normal mouse, the
production of MMP14 was restored, triggering the regeneration of
functional alveoli.
“The recovery of lung function and lung mechanics by transplantation of
endothelial cells that stimulate MMP14 production may be valuable for
designing novel therapies for respiratory disorders,” says Dr. Stefan
Worgall, who helped with the functional lung studies in this project.
“This study will also help us understand mechanisms for repair in the
growing lungs of infants and children,” he adds. Dr. Worgall is associate
professor of pediatrics and genetic medicine and distinguished associate
professor of pediatric pulmonology.
Given MMP14′s role, Dr. Rafii classifies it as a crucial “angiocrine”
signal — a lung endothelial specific growth factor responsible for
alveolar regeneration. Dr. Rafii’s team also seeks to reveal the
initiation signals resulting in the activation of lung blood vessels.
“Changes in local blood flow and biomechanical forces in the remaining
lung after removal of the left lung could certainly be one of the
initiation cues that induce endothelial activation,” says Dr. Sina
Rabbany, who is a co-senior author of this study and a professor of
bioengineering at Hofstra University and adjunct associate professor of
genetic medicine and bioengineering in medicine at Weill Cornell.
The researchers will next determine if MMP14 and other as-yet unrecognized
angiocrine factors are responsible for lung regeneration in humans as well
as mice. “We believe the same process goes on in humans, although we have
no direct evidence yet,” says Dr. Ding. The study’s authors theorize that
patients with COPD (a disorder most often caused by chronic smoking) have
so much damage to their lung endothelial cells that they no longer produce
the proper inductive signals. “We know
smoking damages lungs, but lungs may continue to regenerate alveoli,” says
Dr. Koji Shido, a co-author of this study. “But at certain point,
significant injury to the endothelial cells could impair their capacity to
support lung regeneration.”
“Perhaps replacement of angiocrine factors, or transplantation of normal
lung endothelial cells derived from pluripotent stem cells, could restore
lung regeneration” speculates Dr. Zev Rosenwaks, who is the director of
the Ronald O. Perelman and Claudia Cohen Center for Reproductive Medicine
at Weill Cornell, and a co-author of this study. “Currently, we are
generating pluripotent stem cells derived from patients with genetic
pulmonary disorders to identify potential pathways, which may ultimately
enhance our understanding of how lung endothelial cells may improve lung
function in these patients.”

http://www.medicalnewstoday.com/releases/236793.php

December 21, 2011

On behalf of the Asthma and Allergy Foundation of America (AAFA), I commend the United States Environmental Protection Agency (EPA) for its action announced today implementing the Mercury and Air Toxics Standards rule. For the 20 million Americans with asthma – including 6.7 million children for whom breathing without thinking is not so routine and, who are more likely to sleep poorly at night and miss work or school by day – the content of the air they breathe is top of mind. This rule will set the first national safeguard that limits power plant emissions of mercury, arsenic and other toxic substances that burden the health of our children and communities. This new rule will help protect the health of those most at risk: children, teens, seniors, and people with chronic lung diseases like asthma. According to the EPA, the new Mercury and Air Toxics Standards Rule will prevent an estimated 17,000 premature deaths and 11,000 heart attacks, in addition to eliminating 120,000 incidents of asthma symptoms and 11,000 cases of acute bronchitis in children each year.

We recognize that some critics cite adverse economic impacts of tighter standards. However, this new rule will not only avoid lost wages for patients and parents of children with asthma, lost productivity for the companies that employ them, and increased hospital admissions. It will also create 31,000 construction jobs and 9,000 long-term utility jobs while increasing the demand for investments in energy efficiency and clean technology.

The economic burden of asthma and other respiratory diseases, cancers and cardiovascular diseases are borne by taxpayers via Medicare and Medicaid, and are being borne by corporations who employ these Americans, pay the costs of health insurance for them and their children, and lose productivity when they are sick or caring for their chronically ill loved ones.

AAFA believes that this EPA action is the right step to help keep Americans with asthma safe and healthy.

(AAFA is an independent, not-for-profit voluntary health agency dedicated to improving the quality of life for people with asthma and allergies. AAFA was founded in 1953 by the two leading professional medical organizations in the United States devoted to the allergy/immunology specialty.)

Hanania NA, Celli BR, Donohue JF, Martin UJ

Section of Pulmonary and Critical Care Medicine, Asthma Clinical Research
Center (Dr Hanania), Baylor College of Medicine, Houston, TX; Division of
Pulmonary and Critical Care Medicine (Dr Celli), Brigham and Women¹s
Hospital, Harvard University, Boston, MA; Division of Pulmonary Disease
and Critical Care Medicine (Dr Donohue), University of North Carolina at
Chapel Hill, Chapel Hill, NC; and Clinical Research, Respiratory and
Inflammation Therapeutic Area (Dr Martin), AstraZeneca LP, Wilmington, DE.

Abstract

COPD is a preventable and treatable disease characterized by airflow
limitation that is not fully reversible. The diagnosis of COPD is based on
spirometric evidence of airways obstruction following bronchodilator
administration. Although it used to be commonly believed that patients
with COPD have largely irreversible airflow obstruction, evidence now
suggests that a considerable proportion of patients exhibit clinically
significant bronchodilator reversibility. The complexity and inherent
variability of a patient’s acute response to a bronchodilator and the lack
of a standardized procedure for assessing bronchodilator reversibility
have led to significant confusion surrounding this concept. Although
bronchodilator reversibility commonly is defined based on thresholds for
improvement in FEV(1), lung volume-based measures of pulmonary function
may be of particular importance in patients with severe COPD. The
usefulness of acute reversibility to short-acting bronchodilators in
predicting a patient’s long-term response to bronchodilator maintenance
therapy is also unclear, although most studies suggest that a lack of
acute response to short-acting bronchodilators does not preclude a
beneficial long-term response to maintenance bronchodilator treatment.
This review outlines recent findings about the prevalence and usefulness
of bronchodilator reversibility in patients with COPD based on the
available literature and proposes areas of future research.

PMID: 21972384

http://www.ncbi.nlm.nih.gov/pubmed/21972384

The combination of COPD and anesthesia can be risky business. In fact, the long-term survival rate of COPD patients with severe airway disease who have any type of major surgery is poor. There is also a significant risk of postoperative complications, especially within the lungs. But does this mean that COPD patients should never have surgery?

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“2011 marked the 100^th anniversary of immunotherapy and the release of the ACAAI’s third update of Practice Parameters for Immunotherapy,” said Dana V. Wallace, MD, ACAAI president, article co-author and associate clinical professor of medicine, Nova Southeastern University, Fort Lauderdale, Fla. “These milestones provided an excellent opportunity to review the parameter highlights and advances in treatment and create new credible resources for allergists, primary care physicians and other health care professionals.”

The perspectives article, podcasts and proceedings are based on the panel’s discussion at a recent Roundtable Forum on Immunotherapy.  Topics include:

• The latest standards of care in immunotherapy, including an overview of recently updated practice parameters;

• The status of sublingual immunotherapy (SLIT) in the U.S. and how it compares with traditional subcutaneous immunotherapy (SCIT);
• Other delivery methods now available or on the horizon, including rush and cluster immunotherapy, intranasal, epicutaneous and intralymphatic immunotherapy, and needed research and unanswered questions; and
• How advances in allergen immunotherapy may change and challenge the practices of allergists and other physicians.

“These materials give practicing physicians a succinct overview of the latest research and developments in the field of immunotherapy, a convenient way to identify and access specific topics of interest and ‘listen in’ on the opinions and insights of top leaders in the field,” said Linda S. Cox, MD, article co-author, associate clinical professor of medicine, Nova  Southeastern University, Fort Lauderdale, Fla., and immediate past chair, ACAAI Immunotherapy and Diagnostics Committee.

The Roundtable and resource materials were supported by a grant from Merck.  Dr. Wallace and Dr. Cox co-chaired the Roundtable with the following participants: David I. Bernstein, MD, professor of internal medicine and co-director of the allergy training program, University of Cincinnati College of Medicine and member, ACAAI Board of Regents; Michael S. Blaiss, MD, clinical professor of pediatrics and medicine, University of Tennessee Health Science Center, Memphis, Tenn.; Ira Finegold, MD, chief of allergy and director of the Cook Institute of Allergy, St. Luke’s Roosevelt Hospital and clinical professor of medicine, Columbia University, New York City and chairman, ACAAI Immunotherapy and Diagnostics Committee; Bobby Q. Lanier, MD, clinical professor of immunology, University of North Texas Health Science Center, Fort Worth, Texas, and executive medical director, ACAAI; and Harold S. Nelson, MD, professor of medicine, National Jewish Health and the University of Colorado School of Medicine, Denver.

 Read more about changes in immunotherapy

About ACAAI

The ACAAI is a professional medical organization headquartered in Arlington Heights, Ill., that promotes excellence in the practice of the subspecialty of allergy and immunology. The College, comprising more than 5,000 allergists-immunologists and related health care professionals, fosters a culture of collaboration and congeniality in which its members work together and with others toward the common goals of patient care, education, advocacy and research.  Learn more at www.acaai.org and www.allergyandasthmarelief.org.

Access to healthcare and equipment, choice, quality homecare and a positive relationship with your oxygen provider are important to many Americans who rely on medically necessary oxygen. A new change in Medicare policy threatens those rights for seniors and people with disabilities who rely on Medicare for their durable medical equipment.

The Center for Medicare and Medicaid Services (CMS) implemented a bidding system for medical equipment providers. It requires providers to bid the lowest possible price they are willing to accept for individual pieces of equipment such as power wheelchairs, CPAP machines, oxygen supplies, mail order diabetic supplies and hospital beds. The lowest bidder won the right to contract with CMS at those prices, while the other equipment providers were eliminated from the system. In the Kansas City metro area, 420 original providers available to Medicare beneficiaries were narrowed to 20.

The program known as the DMEPOS Competitive Bidding Program was approved by Congress as a cost-saving measure and is currently being implemented in nine areas of the country. Ninty-one areas are to be added by 2013 and the rest of the country to follow during the next three years. Many Medicare beneficiaries are unaware of the change until new supplies or updates are required, which is creating dire consequences.

Provider shortages create long waits for service and longer hospital stays as caseworkers struggle to find the equipment necessary to discharge patients. Medicare beneficiaries are forced to use lower-quality, cheaper supplies rendering their old equipment of other brands such as oxygen concentrators to be useless. Contract winning providers are located many states away from beneficiaries and may not be experienced in selling and servicing the equipment they won a contract for.

Doug Kaploe from Kansas waited three months to have his scooter repaired because none of the winning providers in the area were capable of servicing his brand of scooter. Lowell Click of Texas was ordered to dispose of his old, better quality diabetic testing machine because the new strips sent to him would not match. They sent him a new machine as well. Oxygen user Earl from Texas, was appalled when the list of oxygen providers available to him hailed from Florida, Ohio and a Texas town located two hours away.

The frustrations of medical equipment providers and their customers have not gone unheard. On March 11, 2011, Congressman Glenn Thompson (R-Pa.) and Congressman Jason Altmire (D-Pa.) officially introduced the bipartisan House bill to repeal “competitive bidding, H.R. 1041, which currently has 145 co-sponsors. Representatives who have not yet signed on and senators who have not moved to create a companion bill are asking for more stories from beneficiaries who are feeling the impacts of provider shortages.

People for Quality Care (PFQC), based out of Waterloo, Iowa, seeks to educate individuals and health advocacy organizations about the change and encourage them to speak out to legislators in opposition of the program by asking them to support H.R. 1041. The organization interviews beneficiaries to gather the stories of delays and frustrations caused by the program.

If you have experienced a delay in receiving medical equipment or service, call People for Quality Care at (888)-544-7913 to help tell the story of how Medicare’s competitive bidding program inhibited your access to quality care. For more information about the cities currently involved, a full list of medical equipment included in the bidding process and the stories of beneficiaries who are speaking out, visit www.peopleforqualitycare.org.

BY Kelly Turner

Use in humans is still years away, but for the 200 million lung disease sufferers worldwide, the device is a major step toward creating an easily portable and implantable artificial lung, said Joe Potkay, a research assistant professor in electrical engineering and computer science at Case Western Reserve University.

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