Canadian Association of PNH Patients raises the volume on plea for access to life-saving treatment

With so few people affected by rare diseases, how does a group of less than 90 patients across Canada make their voices heardon a life-and-death issue such as access to treatment? Barry Katsof, President of the Canadian Association of PNH Patients, says his group is turning to social media to ask others to take a stand with them in the fight for their lives.

“Today, patients living with Paroxysmal Nocturnal Haemoglobinuria (PNH) and their loved ones will mark Rare Disease Day in Canada by turning up the volume on an issue that is threatening our lives – our immediate need for publicly-funded access to treatment with Soliris,” says Katsof. ”We had the government’s attention for a while, but they have stopped listening. Through this campaign, we hope to rallywide support for access to Soliris, the first and only treatment proven effective in treating our ultra-rare and life-threatening disease.”

In January, 2011, Lucas Maciesza’s front-page battle with PNH spurred a change in a government policy which granted him only temporary access to Soliris, a treatment he requires for the rest of his life. This impromptu move by the Ontario government came at the eleventh hour for Maciesza who, after being pulled back from the brink of death by hospital funding for his life-saving infusions of Soliris, found himself again not knowing where funding for his treatment would come from.

“No one should have to beg for their life in that situation,” says Lucas, 26, in a new video released today on YouTube by the Canadian Association of PNH Patients. ”I barely had any energy to beg for my life in that situation.” Read the rest of this entry »


Conversely, never giving birth significantly lowers the risk of such cancer, study finds

Full-term pregnancy has long been associated with a reduced risk of breast cancer, but a new study finds that the more times a woman gives birth, the higher her risk of “triple-negative” breast cancer, a relatively uncommon but particularly aggressive subtype of the disease. Conversely, women who never give birth have a 40 percent lower risk of such breast cancer, which has a poorer prognosis than other types of breast cancer and doesn’t respond to hormone-blocking therapies such as tamoxifen.

These findings, from a study led by Amanda Phipps, Ph.D., a postdoctoral research associate in the Public Health Sciences Division of Fred Hutchinson Cancer Research Center, are published online ahead of the March 16 issue of the Journal of the National Cancer Institute.

“Unlike most breast cancers, triple-negative tumors don’t depend on hormonal exposures to grow and spread, so our assumption going into the study was that reproductive factors would not be associated with a woman’s risk of this cancer subtype,” Phipps said. “We were surprised by these findings because researchers have known for quite some time that women who have children, especially those who have them at an early age and have multiple full-term pregnancies, have a lower risk of breast cancer overall.” Read the rest of this entry »


Protein Could Be New Target to Reduce Damage After Heart Attack

  • Author: Health Informer
  • Filed under: Health News
  • Date: Feb 25,2011

Scientists have identified a protein that plays a key role in debilitating changes that occur in the heart after a heart attack, according to research reported in Circulation Research: Journal of the American Heart Association.

These changes, or “remodeling” of the heart, often lead to fatal heart failure, which kills nearly 60,000 Americans each year. The findings suggest a possible future therapy for preventing or reducing heart muscle damage after a heart attack.

Researchers compared the effect of heart attacks in two groups of mice. One group was genetically engineered to lack fibronectin-EDA (FN-EDA), a protein that exists in the space surrounding cells and is important for processes such as cell migration and wound healing. The other mice were genetically normal.

After inducing a heart attack in the left coronary artery of each mouse, the team found that  the hearts of mice lacking FN-EDA had less enlargement in the left ventricle, better pumping ability and less thickening of the heart muscle compared to the control mice.

At the tissue level, the genetically engineered mice also had less inflammation; diminished activity of the enzymes metalloproteinase 2 and 9, which are involved in heart remodeling; and reduced myofibroblast transdifferentiation (a process in which cells near an injury site transform into myofibroblasts, which are cells that help heal injured tissue).

Bone marrow transplantation experiments revealed that the FN-EDA involved in the remodeling process came from the heart and not from cells circulating in the bloodstream.


In a finding that may potentially improve survival from war injuries and disasters, laboratory researchers report that refrigerated whole blood may have a shelf life well beyond the current standard of 24 to 48 hours.

“We have found that whole blood retains its clotting properties at least 11 days under standard refrigeration,” said the study leader, David Jobes, M.D., a cardiothoracic anesthesiologist in the Cardiac Center at The Children’s Hospital of Philadelphia. “If this lab discovery can be confirmed in human subjects, it may lead to a change in clinical practice, and possibly to improved survival for massively transfused patients.”

The majority of patients receiving blood transfusions only require specific components of whole blood, such as red blood cells, plasma and platelets. However, whole blood may be preferable in specific situations such as infant heart surgery and combat casualties.

The definition of freshness of whole blood with respect to its clotting properties has not been systematically studied. The current practice at The Children’s Hospital of Philadelphia assumes a fresh whole blood shelf life of 48 hours when refrigerated. After that point, the red blood cells may be recovered from the whole blood, but the other components, such as plasma and platelets must be discarded. “In any case, postponed surgeries currently waste resources,” said Jobes. Read the rest of this entry »


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