Adios, Edouard. We hardly knew you.

Which is just fine. All in all, not bad. Some needed rain, a day off from work. One of those rainy days where you just don't do much.

I took the boys to the movies and saw Journey to the Center of the Earth. I almost choked on the ticket prices. Not realizing the movie was 3D, $29.00 for one adult and two kids tickets was quite a surprise. The plastic throwaway 3D glasses must be expensive. The movie was o.k. The boys liked the 3D effects although we should have sat closer to the screen so it would occupy the whole frame of vision. The story was blah but it had enough action and effects to keep a 5 and a 8 year old boy entertained.

¿Que pasa, Edouard?

Tropical Storm Edouard seems to be heading straight toward Galveston and Houston beyond. Will it really be much of anything? Who knows?

Three years ago, just weeks after Hurricane Katrina, Hurricane Rita bore down on the Texas coast. Hundreds of thousands tried to evacuate the area only to run out of gas on the congested highways of the Houston area. Luckily for us (we stayed put) Rita veered eastward and around Houston we had ten's if not hundred's of downed leaves. Points east did not get off so lucky.

So what will happen this time? Only time will tell. But we're good. Factor, food, water, beer; all the essentials.

Self-Infusion Success !!

Jack did it! Yesterday before his baseball game Jack tried self-infusion again and got the vein. Under Allison's tutelage, Jack has been working on self-infusion since he got home from camp a week ago and yesterday was THE DAY.

We are so proud of him. This is a huge step toward full self-sufficiency and ownership of his hemophilia care.

Congratulations Jack!!

Keep it up!

Lifetime Insurance Caps

Here's a good article on lifetime insurance caps.

As medical costs have risen these cap limits have remained stagnant and they need to change.

But just as important we, as consumers of medical care, need to demand transparency in medical costs. Everything; doctors visits, procedures, tests, examination gloves, alcohol swabs, everything. There are countless instances of a provider having two prices; one charged to an insurance company, and one to the guy paying out of pocket. This cycle of provider charging insurer, charging employer, charging employee with higher premiums and higher deductibles has got to stop. It's a rigged system where costs are hidden and choices are narrowed. Like the three gas stations down the street at the corner that compete for my fuel money it's time for the medical industry to join the rest of the market system and compete my health care dollars. Let the consumer be the judge of what's best for them considering services, prices, location and everything else.

I know, I know. Universal health care. I don't buy it. Costs will continue to rise, choices will diminish as will the number of docs and other health care professionals leading to a reduction in quality of care.

D. C. Highlights

The boys and I had a great time in D. C. earlier this month. We were there for just under a week and I tried to pack in as much as the boys could handle. Being there over the 4th of July weekend made for some large crowds and some long lines so a few of our destinations had to be scratched.

We did get our fill though; The National Portrait Gallery, The Smithsonian Museum of Natural History, The Marine Corps Museum at Quantico, Mt. Vernon, a Tour of the Pentagon, of course the Sunset Parade at the Marine Corps Memorial at Arlington National Cemetery, the Smithsonian Air and Space Museum near Dulles Airport and the Bull Run (Manassas) Battlefield.

Here's a few snapshots.

Still not an excuse to spend the day playing Wii

Aerobic capacity lower in boys with hemophilia

From Reuters.

NEW YORK (Reuters Health) - Boys with hemophilia have a lower aerobic capacity than their healthy peers, according to study findings published the Journal of Pediatrics. However, the overall muscle strength of these children is comparable to that seen in the normal population.

None of the participants experienced joint or muscle bleeds or other adverse events because of participation in the study and all of them were able to perform at maximal or near-maximal levels on exercise tests.

Compared with their healthy counterparts though, the peak oxygen capacity, heart rate, and work capacity were significantly lower among the boys with hemophilia.

However, total muscle strength was normal in the patients compared with the healthy controls, and almost no joint impairment or decrease in functional ability was found.

Thanks to prophylaxis our boys can do most of the activities other boys do and it's good to see there were no joint or muscle bleeds.

The study shows that boys with hemophilia have lower aerobic capacity but doesn't explain why. Is there still a predisposition to a more sedentary lifestyle? Do they 'get away' with being less active because of hemophilia? Or is a lower aerobic capacity a by-product of hemophilia?

Off to Kamp Krusty

Well, maybe not Kamp Krusty, but Camp Ailihpomeh (hemophilia backwards). Jack is at hemophilia camp this week, he left Sunday morning. Here's hoping for some positive peer pressure and successful self-infusion.

Camp is again at the great Camp John Mark near Meridian, Texas. I'm sure it's hot as all get out but the boys will, as always, have a fantastic time. Pics from camp are updated daily at the Lone Star Chapter Website, here. Below is the first one of Jack (third from right) and his cabin-mates.

In D.C.

The boys and I are in the D.C. area for the next few days. We're here to attend a Marine Corps Sunset Parade. My brother Mike is the hosting officer on July 8th. On the 4th we opted to see fireworks at Quantico rather than deal with the crowds at the Mall in D.C.

Yesterday we did some sight seeing in D.C. Went to the National Portrait Gallery and the National Museum of Natural History. Pics (so far) below.

Happy Independence Day

It's been crazy around here. So we're due for a break. I'm taking the boys to D.C. for a short vacation (hopefully updates and details to follow) which will give Allison some breathing room to catch up and maybe get ahead of her nursing studies.

In keeping with the patriotic flavor of the holiday here's a few pics from our front yard and the sunflowers Carrick planted in our new front planting bed. These things are not just sunflowers or giant sunflowers but were labeled 'mammoth sunflowers.' I think they live up to their name.

The second pic is of the proud gardener and the third should give a little scale.

Happy 4th everyone!

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."