This looks good..

Seven Days in Utopia

Opening September 2nd.

Happy Independence Day

The words of Calvin Coolidge ring as true today as they did in 1926 on the 150th anniversary of the signing of the Declaration of Independence.

"Ours is a government of the people. It represents their will. Its officers may sometimes go astray, but that is not a reason for criticizing the principles of our institutions. The real heart of the American Government depends upon the heart of the people. It is from that source that we must look for all genuine reform. It is to that cause that we must ascribe all our results.

It was in the contemplation of these truths that the fathers made their declaration and adopted their Constitution. It was to establish a free government, which must not be permitted to degenerate into the unrestrained authority of a mere majority or the unbridled weight of a mere influential few. They undertook the balance these interests against each other and provide the three separate independent branches, the executive, the legislative, and the judicial departments of the Government, with checks against each other in order that neither one might encroach upon the other. These are our guaranties of liberty. As a result of these methods enterprise has been duly protected from confiscation, the people have been free from oppression, and there has been an ever-broadening and deepening of the humanities of life...

No other theory is adequate to explain or comprehend the Declaration of Independence. It is the product of the spiritual insight of the people. We live in an age of science and of abounding accumulation of material things. These did not create our Declaration. Our Declaration created them. The things of the spirit come first. Unless we cling to that, all our material prosperity, overwhelming though it may appear, will turn to a barren sceptre in our grasp. If we are to maintain the great heritage which has been bequeathed to us, we must be like-minded as the fathers who created it. We must not sink into a pagan materialism. We must cultivate the reverence which they had for the things that are holy. We must follow the spiritual and moral leadership which they showed. We must keep replenished, that they may glow with a more compelling flame, the altar fires before which they worshiped."


Read the whole thing here. Its significantly longer and much better than a tweet.

Faster please...longer lasting factor

Not a cure, but we'll take it in the meantime.

...thanks to collaborative work involving the laboratory of Neil Simister,an associate professor of molecular biology at Brandeis, and doctors at Children’s Hospital and Brigham and Women’s Hospital, hemophilia patients may soon have access to long-acting clotting medications that free them from the hassles of frequent infusions and from worries that an internal bleed has gone unnoticed.

Simister’s laboratory, as well as labs at the University of Texas and Ohio State University, independently and simultaneously discovered that the receptor FcRn protects antibodies from breaking down in the body. Building on that basic discovery has enabled the formulation of new hemophilia drugs.

***

...estimates that they are probably less than 12 months away from getting definitive Phase III results for both hemophilia A and B programs. The next step would be filing for FDA approval.

Original article here.

Let your Zinc-Fingers do the walking...or clotting

More reports about the success of the gene therapy treatment in mice using zinc-finger proteins.

http://www.nytimes.com/2011/06/27/us/27therapy.html

http://www.pcworld.com/article/231326/living_mouse_receives_the_first_genome_therapy_cures_hemophilia.html

http://www.hhmi.org/news/high20110626.html

http://www.genengnews.com/gen-news-highlights/zing-finger-based-approach-rectifies-clotting-times-in-mice-with-hemophilia-b/81245353/

Paging Chris Christie...

Proposed Tax on Blood Clotting Factor in New Jersey

ANNAPOLIS, Md., June 15, 2011 /PRNewswire/ -- PPTA opposes New Jersey Assembly Bill 2003 passed by the New Jersey Assembly Appropriations Committee on June 13 that imposes a new tax on therapies used to treat bleeding disorders like hemophilia.

The bill creates The Bleeding Disorders Treatment Fund by taxing the manufacturers of blood clotting factor in the form of a 6 percent rebate. PPTA opposes any policy that would impose a tax on the sale of lifesaving therapies. PPTA calls on the pharmaceutical industry at large to keep a close watch on this bill, which is a slippery slope towards taxing other medicines.

Not your dad's old country club golfers

As Rory McIlroy tear up Congressional this music video of the 'Golf Boys'(Bubba Watson, Hunter Mahon, Rickie Fowler and Ben Crane) is just plain funny.

Hurry!

Bioscience Company shows success in animal trials for Factor IX Gene Therapy Treatment

-Sangamo scientist Michael Holmes presented data from a study in mice which showed encouraging results in a specific type of hemophilia. In this case, the disease comes from a deficiency of a protein know as Factor IX. Scientists gave a single injection of one of Sangamo's zinc-finger compounds and found that mice who got the injection were able to produce Factor IX on their own in high enough levels to achieve normal blood clotting...

Original article from Xconomy.com

The Hunt for Gollum

O.k. I'll break my silence with this. As an unabashed Tolkien fan and a fan of the Peter Jackson adaptations of The Lord of the Rings, finding this movie has me very happy.

Not affiliated with the Tolkien estate or Peter Jackson or New Line Cinema "The Hunt for Gollum" is a not for profit short film made by fans of the author's work. It's set for release on May 3rd.

I only just came across its existence but from the trailers it looks way cool. The story comes from the appendices of The Lord of the Rings. The appendices have so much background material that many more shorts or even features could be made from them. And that's not even getting into the epic Silmarillion (which is rumoured to be in the early stages of a film adaptation).

Here's the original Trailer:

Hunt For Gollum - Trailer One
by HuntForGollum

Make mine a Guiness...

Well, I missed St. Patrick's Day here. Hope yours was good.

One noteable news item with an Ireland tie-in.

Steelers owner is Ireland nominee

6-time Super Bowl Champion Pittsburgh Steelers owner Dan Rooney has been selected to be U.S. ambassador to Ireland by President Barack Obama.







My dad went school with the Rooney boys, North Catholic High School in Pittsburgh and St. Vincent's College in Latrobe. I believe Art Jr. was in dad's class and Dan was a couple years
ahead.

UPDATE:

Well wouldn't you know it. First post after a long layoff and kinda I blow it. The Rooney's went to North Catholic, but only Art Jr. went on to St. Vincent's. Dan went to Duquesne.

Countdown

Countdown to relaunch initiated.

Hiatus (hayh-ey-tuhs)

Defined as a break or interruption in the continuity of a work, series, action, etc.

I fear in my case it's just been plain lazy. A lot has happened recently but I haven't felt compelled to write. I hope to do better in the new year.

Best wishes to all for 2009.

Bone marrow treatment used to combat inhibitors

According to a Children's Hospital of Wisconsin press release researchers there have shown success using patients' bone marrow to treat inhibitors.

The findings were published in the 1 October 2008 issue of Blood Journal.

From the press release:

"...investigators describe how a gene-modified bone marrow transplant can be used to initiate clotting in hemophilia. This type of approach may work in the 30 to 35 percent of hemophilia patients that have developed inhibitory antibodies against the missing clotting protein.

The bone marrow is removed from the patient and stem cells are treated with Factor VIII, a clotting factor, which is placed in the platelets. The marrow is given back to the patient, who then retains the essential clotting mechanisms to stop bleeding that otherwise would lead to complications."

Seven score and 5 years ago...

Today is the 145th anniversary of the Gettysburg address. Two hundred and seventy eight words, only 2 1/2 minutes to deliver yet in such brevity Lincoln relates the breadth and depth of the basic underpinnings of this great nation, this great experiment of representative democracy and federal governance.

In 2 1/2 minutes Lincoln conveyed just how unique, precious and fragile the United States of America was, is and will be. But inherent in its foundation is the principle of Liberty and the notion that "all men are created equal" that make it a concept embodied in a nation that is worth preserving, defending and dying for.

The words ring as true today are they did then.



Four score and seven years ago our fathers brought forth on this continent, a new nation, conceived in Liberty, and dedicated to the proposition that all men are created equal.

Now we are engaged in a great civil war, testing whether that nation, or any nation so conceived and so dedicated, can long endure. We are met on a great battle-field of that war. We have come to dedicate a portion of that field, as a final resting place for those who here gave their lives that that nation might live. It is altogether fitting and proper that we should do this.

But, in a larger sense, we can not dedicate -- we can not consecrate -- we can not hallow -- this ground. The brave men, living and dead, who struggled here, have consecrated it, far above our poor power to add or detract. The world will little note, nor long remember what we say here, but it can never forget what they did here. It is for us the living, rather, to be dedicated here to the unfinished work which they who fought here have thus far so nobly advanced. It is rather for us to be here dedicated to the great task remaining before us -- that from these honored dead we take increased devotion to that cause for which they gave the last full measure of devotion -- that we here highly resolve that these dead shall not have died in vain -- that this nation, under God, shall have a new birth of freedom -- and that government of the people, by the people, for the people, shall not perish from the earth.

Happy Birthday USMC

More Israel

The remainder of the PEP Train The Trainers program went beautifully. I expect wonderful things out of this remarkably talented, highly motivated, deeply insightful group of people. It was an honor and a privilege to have been a part of the team to bring PEP to Israel.

Since I was there I took extra time (a week) to travel a bit and saw some amazing sites. I am so glad to have made the trip but truth be told I don't ever want to be away from Allison and the boys for that long again.

Here's some more pics from Israel:


Jaffa Gate - Jerusalem Old City




Church of the Holy Sepulchre - site of the Crucifixion




Church of the Holy Sepulchre - Edicule - Christ's tomb




The Western Wall

Greetings from Israel

I'm here with the PEP Training team doing a Train the Trainers for Israel. I'm so excited and grateful to be here. We had some doubts and apprehensions, but 1 1/2 days into the training we are so impressed with the participants. They have been remarkable.

Here's some Israel pics.


Tel Aviv



Site of the Nativity in the Church of the Nativity



Dig site in Jericho

I'm announcing a name change

for the next 3 weeks I want to be known as 'Joe the Plumber'

Injured student’s suit against Tecumseh schools dismissed

That's the headline from a Michigan newspaper website. The story's lead:

A lawsuit against the Tecumseh school district and an elementary teacher over bullying injuries a first-grade student with hemophilia suffered in 2005 was tossed out of Lenawee County Circuit Court on Monday.

Man! First of all bullying injuries in first grade! Hemophilia or not what the ...?
From the report:

Michael suffered severe injuries when his arm was twisted behind his back...

Now we can't know all the details from just a news article but what's going on. I'm not sure these incidents called for a lawsuit but certainly they raise some questions about the supervision or discipline at the school, child with hemophilia or not.

As parents of a child with hemophilia we strive to emphasize that Jack is not to be treated differently or excluded from school activities. He doesn't need special attention save in the event
that an injury does happen and then he needs specific medical treatment.

So with that in mind these comments (for the purpose of a lawsuit!) stung:

“They knew he had severe hemophilia. They knew they had to protect him,” argued Hanson’s attorney, James Cmejrek. Although the teacher was not in the lunch room where the second injury occurred, Cmejrek said she failed to instruct lunch room monitors to give Michael special protection because of his condition... (italics mine)

Kogenate FS approved for Daily prophylaxis

The FDA has approved a "new use" for Kogenate FS.

FDA quote:

"Administering Kogenate FS to children with hemophilia A on a daily basis before a bleeding event occurs will reduce bleeding into joints and help prevent joint damage, a major cause of disability in hemophiliacs," said Dr. Jesse Goodman, director of the agency's Center for Biologics Evaluation and Research. (italics mine)

Nice bit of marketing juice for Bayer's Kogenate FS. But perhaps an underlying benefit is to signal an acknowledgement for insurers that a robust prophylactic regime should be fully embraced and properly covered.

New Orleans PEP Train the Trainers

We had a great weekend! New Orleans lives! At least the parts I saw (French Quarter, Downtown/Financial District and the Garden District by Tulane and Loyola). We didn't get to the hardest hit areas that I know are still decimated and showing slow progress.

As far as the TTT - what a fantastic group we had! To a person they were talented, passionate and motivated. I expect this group will create many PEPs back in their own communities.