Happy Mother's Day

I hope it was a joyful and restful day for all the Mothers and Grandmothers today. These are the ladies in our lives that keep the rest of us from falling apart. Without them we'd be hungry, dirty, smelly and wrinkled.

So it's appropriate to remind everyone of Project Red Flag, NHF's awareness program for Women's Bleeding Disorders. The most common bleeding disorder for women is von Willebrand Disease (VWD).

Earlier this year the National Heart, Lung and Blood Institute (NHLBI), part of the National Institutes of Health (NIH) and the U.S. Department of Health and Human Services issued the first clinical guidelines in the United States for the diagnosis and management of VWD.

Here's the NHLBI press release.

And here's NHF's press release.

Baseball - Jack legging a triple

Jack had a game last night and I remembered to bring the camera (and actually use it!).

Here's Jack hitting a legitimate double and taking third on the throwing error. But hey it's seven and eight-year-old machine pitch, so that's a triple. Jack has severe hemophilia A but that hasn't stopped him from enjoying his natural athletic ability. Ain't factor great!

With two games left in the season, Jack's team is 8-3-1 and in second place.

Genetics and Hemophilia

Congress Sends Bush Anti-Genetic Discrimination Bill - Fox News

Redefining disease, genes and all - Andrew Pollack, International Herald Tribune


Medical science is constantly progressing, stretching the current limits and breaking new ground about what is known about the human body, its genetic makeup and what causes diseases.

The Pollack article is a fascinating glimpse into how advances in the human genome are changing how diseases are classified.

“The research is already starting to change nosology, as the field of disease classification is known. Seemingly dissimilar diseases are being lumped together. What were thought to be single diseases are being split into separate ailments. Just as they once mapped the human genome, scientists are trying to map the "diseasome," the collection of all diseases and the genes associated with them.”

Hemophilia has always been the classic case study of genetic mutations and has benefited from advances in genetic knowledge.

“Some of the earliest work has until now been with inherited diseases caused by mutations in a single gene. Diseases have been subdivided by the type of mutation. Hemophilia was divided into hemophilia A and B, caused by mutations in different genes for different clotting factors. And what was once considered a mild form of hemophilia was later identified as a variant of a different clotting disorder, von Willebrand disease, caused by mutations in a different gene and requiring a different clotting factor as treatment.”

So it is in this light that the Genetic Information Nondiscrimination Act carries even greater importance.

I’m not usually one for expansion of governmental power or governmental intrusion of this type but this is a good thing. I‘m not alone in this opinion. Separate versions of the bill passed the House 414-1 and the Senate 95-0 (Ron Paul the lone dissenter) and President Bush is expected to sign the final version.

It’s important not only for those with a genetic disorder but for those who’s only knock is they show a higher pre-disposition to more common ailments.

Ensuring genetic non-discrimination goes far to alleviating some insurance coverage and medical care concerns for our kids with bleeding disorders.

New VIIa product set for 2009 human clinical studies.

Catalyst Biosciences, Inc. announced it intends to file an Investigational New Drug application and commence human clinical studies in 2009 for its new factor VIIa variant. Catalyst says CB 813 is, “…an improved, second-generation variant of human coagulation factor VIIa…for the treatment of acute bleeding in hemophilia patients.”

From the company’s press release:

“CB 813 is designed to substantially enhance clot-generating activity at the site of bleeding and therefore achieve clinical efficacy with fewer and lower doses than current therapy. In established hemophilia models of acute bleeding, CB 813 has demonstrated a significant improvement in potency compared with the marketed recombinant factor VIIa product, NovoSeven(R) and a competing, second-generation product, NN1731.”

Eventually one of these potential improvements will be realized and raise the bar for the rest of the industry. Here's hoping it's sooner than later.

Hemophilia fact-checking

I saw this article a couple of days ago. It’s about a laid-off employee of Aloha Airlines with hemophilia and his struggles to maintain health insurance coverage. What caught my eye was a glaring mis-statement about hemophilia.

Chun has hemophilia; a bleeding disorder that means his blood does not clot. So he could bleed to death from even the smallest cut. (emphasis mine)

No, he couldn’t bleed to death from the smallest cut.

Part of any advocacy work is providing education about the particular issue of interest. It’s frustrating that loads and loads of work that goes into educating the public about hemophilia can be nullified by one sentence in one article where the research and fact-checking wasn’t done.

Because the bleeding disorders community is (thankfully) small getting free publicity in the form of news articles is extremely valuable. But misinformation like this does nothing to serve our greater goals.

McCain's Health-Care Vision

Republican presidential hopeful, Sen. John McCain had an opinion piece Thursday at National Review Online outlining his vision for the future of the American Health-Care System.

“The problem is not that Americans don’t have fine doctors, medical technology, and treatments. American medicine is the envy of the world. The problem is not that most Americans lack adequate health insurance. The vast majority of Americans have private insurance, and our government spends many billions each year to provide even more. The biggest problem with the American health-care system is one of cost and access, and as a result tens of millions of individuals have no insurance.”

From my standpoint it hit the right notes:

Control of health care choices in the hands of the individuals.

“American families know quality when they see it, so their dollars should be in their hands. When families are informed about medical choices, they are more capable of making their own decisions, less likely to choose the most expensive and often unnecessary options, and are more satisfied with their choices.”

More choices in finding an insurance provider.

“Americans also need new choices beyond those offered in employment-based coverage. They want a reformed system so that wherever you go and wherever you work, your health plan goes with you.

… another option would be available: Every year, they would receive a tax credit directly, with the same cash value of the credits for employees in big companies, in a small business, or self-employed. You simply choose the insurance provider that suits you best.

Increased competition in the marketplace for your insurance business.

“Millions of Americans would be making their own health-care choices again. Insurance companies could no longer take your business for granted, offering narrow plans with escalating costs. It would help change the whole dynamic of the current system, putting individuals and families back in charge, and forcing companies to respond with better service at lower cost."

Competition through reform in the maddening array of states health-insurance rules.

“Right now, there is a different health-insurance market for every state. Each one has its own rules and restrictions, and often guarantees inadequate competition among insurance companies. Often these circumstances prevent the best companies, with the best plans and lowest prices, from making their product available to any American who wants it. We need to break down these barriers to competition, innovation and excellence, with the goal of establishing a national market to make the best practices and lowest prices available to every person in every state.”

Extremely important for the bleeding disorders community provisions to insure preexisting conditions within the marketplace framework.

“But we also need to ensure that those without prior group coverage and with preexisting conditions, who have the most difficulty in the individual market, have access to the high-quality coverage they need.”

“I will consult with the governors to solicit their ideas about a best practice model that states can follow — a Guaranteed Access Plan or GAP — and work with Congress, the governors, and industry to make sure that it is funded adequately and has the right incentives to reduce costs such as disease management, individual case management, and health and wellness programs.”

I don’t agree with Sen. McCain on all issues. But this is a sensible, workable approach to addressing the nation’s health-care needs without the leviathan of government run universal coverage. Universal coverage is well intentioned and admirable in principle but in practical terms would be a disaster for this country. The cost of health-care is already outpacing the ability of Americans to pay it. Involve the federal government (more than it already is) and can we seriously envision those costs going down. On a list of bloated, inefficient government programs health-care would be the biggest colossus of bloat and inefficiency. Rather than sending huge premium checks to insurers the American public would instead send their health-care dollars to the federal government in the form of vastly higher taxes.

One major aspect missing from the senator's proposals is some strong steps toward transparency of medical costs. How can the American public be savvy medical consumers if they can't compare costs because those costs are hidden? How hard is it to get the price of a unit of hemophilic factor out of a specialty pharmacy (negotiated contract price with your insurance carrier be damned).

Senator McCain’s proposal may need work and revisions but it is a sensible, responsible and comprehensive start.

Ffffsssttttt…splash

That was the sound of the air going out of Barack Obama’s sails last week following his Pennsylvania primary defeat and then the sound of Rev. Jeremiah Wright being thrown under the keel today.

The presidential hopeful ship of Barack Obama sailed into Pennsylvania waters last week amid what could have been a political perfect storm. For Sen. Hillary Clinton that is. The Keystone state primary was set against the backdrop of the Rev. Wright controversy, renewed interest in the Senator’s ties to former Weather Underground member, William Ayers and Obama’s association with political fundraiser and wire fraud, bribery and extortion defendant Tony Rezko. Not to mention his own disparaging San Francisco comments about middle-class Midwesterners.

All of this didn’t so much fill Clinton’s sails as take the wind out of Obama’s. Pennsylvania’s more blue-collar electorate set up nicely for the former First Lady in the first place and add to it Obama’s recent woes and victory was all but ensured for Clinton.

In the end it may not matter. By throwing the Chicago pastor overboard today Obama more than likely will have righted his ship. Especially in the eyes and opinion of the fawning adoration of the nation’s media.

But let it be a cautionary tale. Let it serve as a reminder of how much we don’t know about the junior Senator from Illinois. People want him to be something different. People want to feel good about him. He is charismatic and witty and smart and people want him to succeed for so many reasons. But just wanting someone to be what you hope for doesn’t make it so.

Hope and change are universal ambitions. But with nothing behind them they are just words.

And remember he just disowned Rev. Wright a mere six weeks after saying he could no more disown his former pastor than he could his own grandmother. Look out grandma and keep your mouth shut.

Mirror - mirror

I know this just has to have been done before but are Steve Nash (on the right,or left) of the Phoenix Suns and British musician James Blunt (on the left, or right) the same person or at least long lost twins. Eerie.

While we've been away...

O.k. I took off last week from work and thought man, I'm gonna blog every day. This is great!

What did I do? Nothing. Absolutely nothing (in the way of blogging).

Nothing happened though, right? The Pope's visit, primary election in Pennsylvania, nothing.

I'll get back in the swing here shortly.

World Hemophilia Day!

Today is world Hemophilia Day 2008. This year's theme is Count Me In.

The World Federation of Hemophilia describes it better than I could:

This year the theme of World Hemophilia Day (April 17) focuses on registering and identifying patients: Count Me In.

It has often been said that there is strength in numbers. This is definitely true in the bleeding disorders community. As a rare disease group, in order to be heard by governments and others, getting the numbers to back up your case for support makes a huge difference.

Having accurate statistics on the number of people with bleeding disorders identifies how many people are affected and helps ensure that they get the necessary treatment. It also helps to validate claims with ministries of health and build a case for support.

The WFH’s vision of Treatment for All is that all people with bleeding disorders need access to adequate care and treatment. The first step to achieving this is to identify those patients. For 10 years now, the WFH has been collecting data from national hemophilia organizations around the world to quantify the number of people affected with bleeding disorders. Our most recent survey identifies 205,472 people with bleeding disorders in 100 countries.

On April 17 we are launching a new publication on outreach to identify patients. The WFH has also developed a number of tools to help patient organizations, hemophilia treatment centres, and others raise awareness about the need to be counted and help identify patients. These include a poster, news release, factsheets on outreach and national patient registries, as well as educational materials for patients on hemophilia, von Willebrand disease, and rare bleeding disorders.

World Hemophilia Day was started by the WFH in 1989. April 17 was chosen as the official date in honour of WFH founder Frank Schnabel, who was born on that day.

On the day, hemophilia organizations and treatment centres around the world organize gatherings, sporting events, exhibitions, and other activities to raise awareness of the issues affecting people with hemophilia.

We encourage all patient organizations, treatment centres, and corporate partners to celebrate World Hemophilia Day this year.

We want to count you in!

Tax Day

I tend to be a procrastinator. But I had no intention of waiting until the last minute to file our ‘lack of’ income taxes. We’re getting money back for goodness sakes! But still I put it off and put it off. I finally got them done on Sunday only to get an e-mail today from TurboTax telling me the eFile was rejected. I messed up on the 2006 AGI figure the IRS uses as verification for the ‘electronic signature’. So here I am on tax day scrambling to correct the eFile and resend.

Fair Tax anyone? A federal consumption (sales) tax doing away with all income taxes? Doesn’t sound like such a bad idea right now. I know there’s plenty of well-reasoned criticism of the Fair Tax, plan but at least it’s a discussion moving in the right direction. Year after year we hear about simplifying the tax code yet it never happens. Instead it gets more complicated with additional and revised deductions and credits and qualifications pushed by lobbyists and passed by well-meaning but ill-informed legislators.

It’s time for a change. Change we can believe in. Hope we can believe in. I didn’t just say that.

Heparin Problem has not gone away

I posted about the Heparin problem back in January here. Apparently the situation has gotten worse. A recent New York Times article details the latest developments.

Here's the opening graphs:

The number of suspicious deaths in the United States linked to the blood thinner heparin has risen to 62 from 19, with most of them reported this past December, January and February, according to the first detailed analyses of heparin fatalities by the Food and Drug Administration.

The F.D.A. is still investigating whether those deaths and hundreds of allergic reactions were caused by a heparinlike contaminant made in China that was added to the drug somewhere during the manufacturing process.

The drug agency defined suspicious deaths as those involving one or more allergic reactions or a drop in blood pressure. There have been no reports of deaths since the end of February, after Baxter International recalled heparin made with ingredients from a Chinese supplier.

Before we jump to conclusions about Baxter:

In addition to Baxter, companies that have recalled heparin products made with Chinese ingredients include Covidien, formerly Tyco Healthcare, and B. Braun. Both Covidien and Braun said that they had received no reports of adverse reactions from the heparin and that the recalls had been undertaken merely as a precaution.

The common thread between this product and some seemingly unrelated consumer goods is China. I posted about China's exports last year here. Coincidence for the 2008 Olympic Summer Games host? Time will tell.

Cool Perfomance Art

I'm not usually much of a YouTube guy but this is pretty cool. No protest, no motive, just let's see if we can do it. Two hundred people freezing in place for five minutes in Grand Central Station.

The Masters

It’s time. I know you can hear the first smooth strands of the piano music as the images of azaleas, dogwoods, brilliant green fairways, Rae’s Creek, the Hogan Bridge and Magnolia Lane roll across the screen. You are welcomed by the mellifluous voice of Pat Summerall and now Jim Nance. It’s Masters Week at Augusta National.

The only one of the major championships played on the same course every year The Masters holds a special appeal for both players and fans alike.

Both the club and the championship were the vision of golfing legend Bobby Jones and Clifford Roberts. The list of Masters champions reads like a roll call of golf’s greats; Gene Sarazen, Byron Nelson, Sam Snead, Ben Hogan, Arnold Palmer, Gary Player, Jack Nicklaus, Tom Watson, Phil Mickelson and Tiger Woods.

It’s Masters week at Augusta and I plan to enjoy.

Returning a favor - BLOOD4

A while back I got some nice words about my blog from a fellow blogger in Brazil. So I want to return the favor. Frederica Cassis is a psychologist at a hemophilia hospital clinic in Sao Paulo, Brazil. She believes in the power of images and uses what she calls ‘blood symbolism’in her hemophilia work. People like Frederica; our doctors, nurses and social workers are so precious to our (relatively) small bleeding disorders community.

Frederica helps the children in her care educate themselves about hemophilia and cope with living with a bleeding disorder. She has created a flashcards game to teach kids about blood clotting and hemophilia called HEMOACTION. Examples of some of the HEMOACTION cards are below. For more about HEMOACTION go here.

I can barely put two coherent thoughts together at one time and Frederica blogs in French, Spanish and English. Remarkable!

Thanks, Frederica.

Visit her at BLOOD4

Great new Product - Brūz Wear

I remember hearing about this idea a while back and though it was great. These pants for your little one have padding sewn into the pants over the knees. When Jack was starting to crawl we worried about bruises and must have gone through 4-5 sets of knee pads to help ward off the black and blue knees. He never seemed to be bothered much by the bruises but they did look nasty, which sometimes got us nasty looks.

From the 4/6/2008 Press release:

"Brūz Wear’s pants are the creation of Colorado mom, Kathryn Ames, who was determined to find a way to encourage her son, Nicholas, to crawl, climb, and stumble even though he has a severe bleeding disorder named hemophilia.

“I wanted Nicholas to feel like a normal, active little dude that could keep up with the other kids,” Kathryn said of her designs. “Other kids notice his ‘cool’ skater pants but I’m thankful his knees are protected from hard surfaces!” It was not hard to share the pants with all active children. “Many Moms on playgrounds ask where they can find Brūz Wear pants for their own kids. Hemophiliac or not – the pants are in demand!”

Good Luck to Kathryn and Brūz Wear.

MLK...40 years on

...April 4
Shot rings out in the Memphis sky
Free at last, they took your life
They could not take your pride

In the name of love
What more in the name of love?
In the name of love!
What more in the name of love?

Pride(In the Name of Love) --U2

Martin Luther King, Jr.
He was a transcendent person to whom none should be compared.
He believed in what was right. He believed in the concepts and ideals upon which this nation was founded. He believed in America. It came through in his words and it came through in his life.



I have a Dream

"I have a dream that one day this nation will rise up and live out the true meaning of its creed: "We hold these truths to be self-evident: that all men are created equal."

I have a dream that one day on the red hills of Georgia the sons of former slaves and the sons of former slave owners will be able to sit down together at a table of brotherhood.

I have a dream that one day even the state of Mississippi, a desert state, sweltering with the heat of injustice and oppression, will be transformed into an oasis of freedom and justice.

I have a dream that my four children will one day live in a nation where they will not be judged by the color of their skin but by the content of their character.

I have a dream today."

"When we let freedom ring, when we let it ring from every village and every hamlet, from every state and every city, we will be able to speed up that day when all of God's children, black men and white men, Jews and Gentiles, Protestants and Catholics, will be able to join hands and sing in the words of the old Negro spiritual, "Free at last! free at last! thank God Almighty, we are free at last!"



I See The Promised Land

He was tragically prescient. His "I See The Promised Land" speech was given one week before he was cut down by an assassin's bullet.

"Well, I don't know what will happen now. We've got some difficult days ahead. But it doesn't matter with me now. Because I've been to the mountaintop. And I don't mind. Like anybody, I would like to live a long life. Longevity has its place. But I'm not concerned about that now. I just want to do God's will. And He's allowed me to go up to the mountain. And I've looked over. And I've seen the promised land. I may not get there with you. But I want you to know tonight, that we, as a people will get to the promised land. And I'm happy, tonight. I'm not worried about anything. I'm not fearing any man. Mine eyes have seen the glory of the coming of the Lord."

Parents Sue After Child Inherits Hemophilia

http://www.news.com.au/heraldsun/story/0,21985,23416072-24331,00.html

http://www.foxnews.com/printer_friendly_story/0,3566,340737,00.html


This story is a few days old and it's taken a while for me to react because the emotions keep changing. Disbelief, anger, sorrow. The immediate response was here is another example of the notion so pervasive today that life should be painless and free of sorrow and disappointment and if life is otherwise then it’s someone else’s fault and they should pay.

Life is. It’s up and down, in and out, good, bad, sad, boring, exciting and everything else. It is both joyful and painful. It never goes in a straight line and no matter how well you plan and prepare the unexpected will happen. For good or otherwise. There are no guarantees.

We who have children with bleeding disorders know this all too well. Our dreams, hopes and aspirations for our perfect children were shaken to the core with the diagnosis of hemophilia or von Willebrand’s. For most of us; time, education and the support of others in the bleeding disorders community was all that was needed for those previously crumbled dreams to be rebuilt. Rebuilt and perhaps standing stronger having been tempered by the lesson that fate is fickle and dreams need not be discarded but rather reshaped and refocused.

It’s a lesson that this couple in Australia has obviously not learned or are unwilling to learn. Opting rather to wallow in victimhood and self-pity (or even more disgraceful – playing the victim card for purely monetary motives).

Laurie Kelley is right to point out that our only knowledge of the case, its facts and the litigants comes from these stories. The Melbourne Sunday Herald Sun had, I believe, the full original story. And from it I’m willing to make some inferences.

I’m no lawyer but this couple doesn't appear to be suing for ‘breach of contract’. Meaning: they were not given a 100% guarantee of having a girl. This is supported in the Herald Sun story:

"They [Lawyers for Melbourne IVF] claim the couple was given and signed an IVF consent form and a embryo biopsy consent form which stated: "If a pregnancy is achieved from biopsied embryos, we understand that further diagnostic tests are recommended to confirm the early embryo diagnosis."

"The plaintiffs knew . . . that undergoing IVF and PGD did not guarantee a female fetus if a pregnancy were to occur and could result in the sort of injury, loss and damage about which the plaintiffs complain," court documents say.

"The plaintiffs agreed to undergo IVF and PGD with the full appreciation of the nature and extent of the risks involved."

And is it possible that tests and ultrasounds were inconclusive as to the gender of the baby? Sure. How many stories have we heard of people expecting a baby of one sex because of an ultrasound only to be blessed with a child of the opposite sex? Also, given the nature of the IVF selection in this case the baby’s sex was perhaps taken for granted, by everyone.

It’s a sad case on so many levels.

“And they are claiming money to cover medical expenses, because Jess will need treatment and care for life.”

Did they count on psychiatric help after Jess starts wondering why “Mum and Dad didn’t want a boy like me. So much they considered killing me before I was born.”

“The couple say that, had they known [the baby was a boy], they would have considered terminating the pregnancy.”

Of course I find the above quote entirely repugnant. It recalls an excerpt I read from Practical Ethics a book by Pete Singer, a so-called bioethicist from Princeton University.

“When the death of a disabled infant will lead to the birth of another infant with better prospects of a happy life, the total account of happiness will be greater if the disabled infant is killed. The loss of happy life for the first infant is outweighed by the gain of a happier life for the second. Therefore if killing the haemophiliac infant has no adverse impact on others, it would…be right to kill him.”

Infuriating and sad. Mostly sad.

It’s not the fact that this couple went to extraordinary lengths to have a hemophilia-free child, Allison and I had similar discussions before choosing to have another child and leave it up to God. But by pursuing this litigation this couple is demonstrating a clear resentment and disdain for the life of their son Jess. A life and condition shared by my son.

Like all children our boys with hemophilia are gifts. Gifts to be cherished and nurtured and loved. Each one with unique talents and abilities, needs and wants. They are undeniable gifts and constant sources of joy.

Jess is three now. And I hope his parents know, and he knows, that he is a gift and a joy.

Baseball...closer to home

This is Jack, my 8-year-old. Jack has severe hemophilia A but thanks to modern factor concentrates and prophylaxis he gets to do things like play baseball and basketball and soccer and swimming. And you know what (he says proudly), he's quite good.

Play Ball!

The line are chalked, the grass is trimmed and the red, white and blue bunting is hung with precision.

Baseball. America's pastime.

It's Opening Day of the 2008 Major League season.

Who's it going to be in October? Red Sox again? Yankees? Tigers?

Can the Braves rebound? Will it be the Mets? Can the Rocks surprise once more?

Or will it finally be the year for an Astros - Indians Matchup? I know. I won't hold my breath.