Golf, the good and the funny

Holy Smokes! Did you see Tiger finish his fourth round to force a playoff with Rocco Mediate at the U.S. Open? Unbelievable. Playing as inconsistently as he was on the bum knee. Amazing.

We have our own little golfer around here. In the last couple of weeks Jack has gotten a taste of some real golf and is doing quite well.

Two weeks ago on June 2, the Lone Star Chapter had the Matthew Thomas Memorial Golf Classic. The tournament is one of the chapter's major fundraisers and a fun time (even if it is brutally hot). The last few years the events have included a junior tournament and the past two years Jack has played. This year he came in second overall out of 20-25 kids of all ages (into the teens) and won a junior set of clubs.

Jack has a pretty good swing, what I call an athletic swing. I've taken the boys to the driving range a few times but except for the Matt Thomas tournament Jack hadn't played on a real course. So for him to do so well gave me ample motivation to hit the links with the young guy.

So this weekend we got out twice and played on 'real' courses. First, in keeping with Father's Day weekend, Jack and I went out with my dad on Friday and Jack posted some good scores including a par on a par three. Next, on Saturday, we joined my brother Tim and his son Zach and got in another sweat-drenched 18. Again Jack played well with another par on a par three and a par on a shortened par 4. Allison says we have to get him lessons so I don't screw up his swing with my instructions.

I don't have any pics from the tournament or this weekend but here's some recent video from the driving range. Jack looks good but also pay attention to Carrick in the background. God bless him, it's hilarious.

Been awhile...

Life interferes. It's been a crazy two weeks. School's ended for the boys and summer camps have started, work has hit the hectic/stressful season but biggest of all Allison has started nursing school. She going to the UT School of Nursing at Houston accelerated program. It's two years of nursing crammed into one. Lots of reading, lots of online lectures and lots of studying. Did I mention reading and studying. It occupies the majority of her time. By the time its over the boys may feel like they have a single parent. But in the end it will be more than worth it. For her and us.

Happy Memorial Day

On this day we remember all the men and women who gave their lives in the service of our country. This nation that has expended more treasure and given more blood in the service of the rest of the world. This nation, as Ronald Reagan said, stands like a shining city at the top of a hill. Shining as a beacon to all who desire liberty. Because of the sacrifice of our servicemen and women, this nation has stood and still stands as the greatest nation on the face of the earth.

God bless America.

Bush signs GINA

On Wednesday President Bush signed the Genetic Information Nondiscrimination Act of 2008 into law. The president's critics and detractors are numerous and varied, but here is something where we can stand together on the same side and approve of this landmark legislation.

*UPDATE*

From NHF:

On Wednesday, May 21, 2008, President Bush signed historic legislation protecting Americans from discrimination based on the results of genetic testing. The bill was recently passed by Congress with strong bipartisan support after a 14-year struggle and dedicated efforts by a coalition of healthcare and other advocacy organizations, including the National Hemophilia Foundation.

"This is a tremendous victory for the hundreds of thousands of Americans affected by genetic disorders," said Val Bias, CEO, National Hemophilia Foundation. "It was a long and hard effort to achieve this day".

The new law prevents employers and insurance companies from either requiring genetic test results or from using genetic information as the basis for decisions around employment or insurance coverage. In the bleeding disorders community, the law has particular significance for women who are considering genetic testing to determine whether they are carriers. In the past, some individuals have avoided such tests out of fear of possible discrimination. The law does not affect those who have been diagnosed with specific conditions.

Factor, prophylaxis and…complacency

For parents of boys with hemophilia anti-hemophilic factor products are heaven-sent. Combined with a prophylactic regimen the results are amazing. Although bleeding activity and severity can vary widely we have found that with prophylaxis Jack has few, if any, breakthrough bleeds and the restriction on his activities (within reason) are next to nothing. And this is wonderful. I’m sure there are untold numbers of people who see this active, go-for-it boy who would be shocked to learn he has a genetic condition that in the not too distant past would have likely resulted in debilitating joint damage or, even worse, an increased risk of death.

Jack has been on prophylaxis for nearly five years now, following a year of immune tolerance to defeat inhibitors. In that time we’ve transitioned from a port to peripheral sticks and with each step and a greater awareness (both Jack’s and ours) of recognizing and treating bleeds the results have been liberating.

But there’s a flip side. At times we can be lulled into complacency. A missed infusion here, a missed infusion there and nothing goes wrong. No harm, no foul; right? Busy schedules, few bleeds and we think we can skip a day and get back on schedule tomorrow. But it doesn’t always work out so well.

We were caught missing an infusion last week. At a time when we should have been hyper-vigilant we let our guard down. Our normal prophylaxis is M-W-F but sometimes we will purposefully alter the schedule to match up with games, events or activities to ensure that Jack has factor on board.

Now, Jack is finishing baseball and starting swim team practices. So last week he had swim practice on Wednesday, a baseball game Thursday, swim again Friday and baseball again (playoffs) Saturday. He had his normal infusion Wednesday morning but we missed his Friday infusion. On top of all this activity Jack planned to spend the night Friday at a friend’s house. So of course we got a call around 10:00 pm from Jack saying his leg was hurting and it was painful to walk. After all his activities it's no wonder. I collected him within 5 minuets, came home and Allison infused and Jack was off to bed. We suspect it was a soft-tissue bleed about mid-thigh. The next morning Jack couldn’t walk from the bedroom to the couch. He had a 9:00 am game so we infused again about 8:00 am. I dreaded the phone call to his coach.

I told Jack that if he couldn’t play he would still put on his uniform and go cheer his teammates. He said he wanted to give it a go, so we wrapped his thigh in an ace bandage and went to the ball field. I’m not sure the ace bandage really did anything save boost his confidence, but by the time we got to the field he could walk fine and after warming up he was jogging and running without pain.

He played and played well helping his team win. I’m proud of him for calling us when his leg was hurting and getting out and trying when he was unsure if his leg would hurt. He handled the situation like a pro. It was his parents who dropped the ball, letting distractions get in the way of the proper infusion schedule.

Of course we use this as a ‘teachable moment,’ using our mistake to show how important it is to take care of yourself and follow the proper schedule so as to avoid injuries like this.

Undoubtedly this won’t be the last missed infusion (try as we might, it’ll happen again) but it goes to illustrate that all the factor products in the world do you no good if not used effectively and how easily we can be lulled into a false sense of security when what is really required is constant vigilance.

Hooray for the Home Team

A group of researchers at The University of Texas Health Science Center at Houston have developed a modified Factor VIII protein that will help treat inhibitor patients.

From the press release:

With a shortage of the blood-clotting protein Factor VIII (FVIII), people with Hemophilia A typically receive injections of FVIII derived from plasma or produced synthetically to control potentially life-threatening episodes of bleeding. Unfortunately as many as 1 in 3 people with Hemophilia A produce inhibitor antibodies, which attack the externally-administered FVIII and negate its blood-clotting benefits.

To combat this problem, scientists in the laboratory of Sudhir Paul, Ph.D., at The University of Texas Medical School at Houston, developed a chemically modified version of FVIII which during laboratory tests neutralized these inhibitor antibodies, thus paving the way for the correction of the blood-coagulating process. The modification is called electrophilic FVIII analog (E-FVIII).

“It’s a two-step process,’’ said Paul, the senior author. “The E-FVIII permanently inactivates the antibodies that inhibit blood clotting in 20 to 30 percent of patients receiving Factor VIII replacement therapy. Once the antibodies are cleared, additional FVIII can be injected.” The study involved blood donated by eight people with FVIII-resistant Hemophilia A.

Miguel Escobar, M.D., of the department of pediatrics and internal medicine at the UT Medical School at Houston and the Gulf States Hemophilia and Thrombophilia Center, led the clinical aspects of the work.(emphasis mine)

This is great news from just around the corner. Congratulations to Dr. Escobar and the rest of our HTC team. We are so very fortunate that Houston has one of the premier Medical Centers in the nation, if not the world. We are doubly blessed for Jack to be under the care of Dr. Keith Hoots and the whole Gulf States HTC staff (the finest in the nation, and yes we’re biased).

Project DOCC

I helped out with Project DOCC on Friday. What’s Project DOCC? Well, from the Houston Project DOCC website:

Project DOCC, a groundbreaking national program for pediatric physicians-in-training, has a simple goal:

To improve each patient's quality of life by teaching physicians-in training the effect of chronic illness on patients and their families.

Created in 1994 by three parents, Project DOCC's philosophy is that, by understanding how families deal with illness and or disabilities out side the hospital setting, pediatric physicians-in-training become better and more compassionate physicians.

Project DOCC's teacher - parents and guardians of children with special medical needs - provide physicians with a unique perspective on culturally effective, family-centered care.Project DOCC Houston is a non-profit organization that is a collaboration of Houston-area families of children with special health care needs, Baylor College of Medicine's Department of Pediatrics and Texas Children's Hospital. Project DOCC Houston is mandatory for first year pediatric physicians-in-training as one of the teaching modules of Pediatrics 101.

The Project DOCC program is made up of three components; the home visit, the parent interview and the Grand Rounds panel presentation.

Both Allison and I have participated in the home visit portion (check out here for more on the parent interview and Grand Rounds). During the home visit the resident(s) come to the home of a special needs child to get a feel for a day-in-the-life of that family. We go over medications and dietary issues and storage issues for supplies. We get into insurance and Medicaid, home modifications for handicap access, marital and financial stresses and so much more. There’s also a ‘visiting parent’ who interjects their own experiences as well as providing a more global perspective of the challenges of caring for a special-needs child.

I take special satisfaction from being able to tell our story and educate another doctor about hemophilia and challenges that come with it.

So on Friday I was the ‘visiting parent’ and we had a very interested and observant resident. I’ve done home visits where we’ve had two or three residents and, honestly, having the undivided attention of just one is so much better. With three residents or even just two one can shrink back and not engage, letting another resident take the lead.

All in all it was a good visit and hopefully enlightening and productive for the resident.

Check out to see if Project DOCC is in your area and how you can get involved.

FDA approves new NovoSeven product

On May, 13 the Food and Drug Administration (FDA) approved Novo Nordisk's new formulation of NovoSeven(R)RT recombinant factor VIIa product. What makes the new Novo so novel? The RT is for room temperature. RT can be stored at room temperature. According to the press release the new NovoSeven will be available in higer concentrations for smaller infusion volumes.

From the Press Release:

NovoSeven(R) RT has been manufactured to be room temperature stable (from 36 degrees F to 77 degrees F outside a refrigerator). The new formulation does not require refrigeration and can be moved in and out of the refrigerator. In addition, NovoSeven(R) RT has a higher concentration for lower infusion volumes and quick administration. The needed infusion amount is almost half of that needed with the original NovoSeven(R).

Unassisted triple play

No, not Jack in little league,but the Tribe's Asdrubal Cabrera. On Monday night the Cleveland Indian second baseman turned only the 14th such feat in Major League history. To put it into perspective there have been 15 perfect games which makes the unassisted triple play the rarest of baseball achievements. What's really cool about the play-by-play call is that even before Cabrera caught the ball the announcers were calling 'triple play, triple play.' The runners were moving on the pitch and the broken bat liner was so close to second base Cabrera couldn't help but turn the rare three-out play.

Asdrubal Cabrera Turns Unassisted Triple Play
Uploaded by Machochip

'Good night, neighbor'

Veteran Houston newsman, Ron Stone, passed away Tuesday after a bout with cancer. Ron Stone was on the air on Channel 2, KPRC when my family moved to Houston in the late 70's. The team of Stone, weatherman Doug Johnson and sportscaster Ron Franklin were the best, most relaxed, funniest and down to earth news team I've ever seen.

Stone and company were the genuine article. He signed off each night with, "Good night, neighbors." And one felt that he truly meant it and I know he did. He came into our homes each night and gave us the news as a neighbor. When he presented the news it was like you could go outside, walk over to the fence and continue the conversation with him.

For so many years he was a part our Houston life, first on Channel 2 then picking up for Ray Miller on 'The Eyes of Texas' and later with his production company, Stonefilms of Texas.

I also remember staying up as late as I could manage and watching Ron and the rest on the local broadcast of the Jerry Lewis MDA telethon.

I haven't thought about Ron Stone in a while, things change and you move on. But now that he has passed it's hard to forget him. Thanks for years and good night, neighbor.

KPRC report.

Houston Chronicle article.

Channel 11, KHOU report

Channel 13m KTRK report

'Impossible is nothing'

This is a great story about an amazing young woman from the Wichita Eagle. Ngoc Trang Nguyen is the valedictorian at East High School in Wichita, Kansas. That's nice, but so what, you say. Four short years ago, having emigrated from Vietnam, she spoke no English. By junior year, she was doubling up on math and science courses and getting straight A's. Trang plans to attend Creighton University in Omaha and major in biochemistry. But that's not the best part.

"I imagine myself in a lab coat," she said. "One of my wishes is to figure out the cure for hemophilia, which is a blood disorder."

In Vietnam, one of Trang's best friends had hemophilia. "But his family was so poor, they could not afford medication, and he suffered tremendously from that," she said. "This is what inspires me."

This is a story that warms the heart on so many levels. It's fantastic that someone so bright wishes to work on a cure for hemophilia. It also shows that in the meritocracy of this great nation of ours opportunities exist for anyone. With encouragement and dedication, hard work is rewarded regardless of ones background. It's there for the taking. As the Nike slogan says "Just Do It."

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.