January 2008

There Will Be Blood

When I decided to write a book about inhibitors (which I am currently doing), I thought how cool it would be to actually visit with a family and see what their regular life is like. It’s hard to write about something when you don’t see it in action or live it. Now, I wouldn’t say that the Fatulas are a typical family with inhibitors: four boys, and three have hemophilia and inhibitors! And lucky me, I was able to visit with them in Western Pennsylvania this past weekend.

Some of you may know Kerry Fatula. She’s the executive director of the Western Penn Chapter (her aunt, Louise, is executive director of the Alaska chapter). She and I served on the Novo Nordisk Consumer Council for the past two years, where we became fast friends. Okay, we became friends after I made her watch a really terrible movie after one council meeting, and we either would be friends for life or never speak to one another again. So friends it was. Turns out we have lots more surprising things in common. Inhibitors, however, is not one of them.

I hung out with Kerry and her husband Chuck at NHF in November, and proposed the idea of a visit. They were so accommodating and welcomed me like family. I arrived Friday evening, Kerry picked me up at the airport, and we promptly went and ordered what seemed like 16 chili dogs at a drive through for the family. It was great seeing everyone again. Paul, 18, headed out that evening to a sleepover so we didn’t get to chat much. Nathan, age 16, is a delightful conversationalist and budding guitarist. Stephen, 5, slept through the whole evening on the couch even though we were pretty loud. And Colin, age 8, just stole my heart (he’s the hemophilia-lite one).

On Saturday I must have interviewed Kerry for 5 straight hours. We covered everything you would want to know about inhibitors: how each boy was diagnosed with inhibitors; early problems; ITT; products used; relationship with the medical community; searching for info; having more children. Kerry is superwoman: all these children, trying to infuse each one daily for their ITT and get them to school. She painted a hysterical picture of herself sitting on each boy, every morning, poking them with a needle to get their factor, like an assembly line! Kerry confirmed what I am learning: each family with inhibitors is almost completely different from every other one. It’s hard to draw generalities. What I learned most is what amazing parents Kerry and Chuck are. Four lovely, healthy young men who are just regular boys and who will be wonderful men some day.

Back at their house Saturday afternoon, Nate let me interview him for a while. He missed so much public school due to bleeds, that he uses Cyber School now. Of the three boys with hemophilia, only Nate has not been tolerized. Kind of like saying he’s never been domesticated! His blood runs wild. He has a great attitude, and wants to make a career of music. 

After the interviews, the youngest boys, Stephen and Colin, decorated me and Kerry with tattoos: a butterfly for Kerry; a psychedelic heart for me, right on my hand for all to see. Stephen chose a mushroom for his hand, and Colin chose a guitar for the back of his neck. Then Kerry and I stole away to see “There Will Be Blood,” the new movie that is getting rave reviews and Oscar nods for Best Picture. It also stars Daniel Day-Lewis. Kerry and I learned we were both huge DDL fans. Appropriate for two hemo moms to see a movie with such a title. And when you have inhibitors, there will be blood. It’s a whole different ball game than just having hemophilia. The movie was great, and my weekend even better. What a wonderful treat to spend a weekend with such a great family. We parted with many good byes and the nicest hug from little Stephen.

Great Book I Just Read: The Kokopelli Theory, by Kevin Correa. This medical thriller is a first-time novel by a local Georgetown resident. The author actually stopped by to give me a copy, but it’s the topic that had me read it: a medical student slowly uncovers a shocking theory about the spread of hepatitis C. From a stolen serum in California in the 1970s, to the Serengeti plains, to the highest reaches of the World Health Organization, the story keeps you guessing and doubting. Good read for a first time author! I think this would make for a great movie, too; it has all the right components for a great summer flick.

The “Hematoma”

And now for something very light but worthy of a good head-scratch: Adidas is advertising a mountain bike shoe called the “Hematoma.” It’s not black and blue, but black and white.

I mean, is this good marketing? Would someone buy a biking shoe basically called “The Bruise”? Are people going to know what a hematoma is? I ran a google image on “The Hematoma” and got some pretty gruesome images. Apparently dogs get a lot of hematomas in their ears. Other running/biking shoe names by Adidas include “The Blockbuster,” “Hammer,” “Cyclone” and “Marathon.” I kind of get those, but Hematoma? Does it give you hematomas? Protect you from them?

My colleague and contributor to PEN Richard Atwood first brought this to my attention. He tells me the Hematoma comes with the price tag of $129.99. Ouch. “At that price,” Richard suggests, “your wallet will be severely bruised.”

New Factor IX Products in Development

What’s going on with hemophilia B? A lot! I’ve read recently about four new studies about new products for hemophilia B patients. In a nutshell:

A. Inspiration Biopharmaceuticals (www.inspirationbio.com) has signed an agreement with Cook Pharmacia to develop an injectable recombinant factor IX. So, what’s new about that? I mean, we already have that. Well, Inspiration is focusing on revolutionizing hemophilia treatment in two ways: 1) developing lower-cost versions of existing intravenous recombinant therapies; and 2) developing non-invasive administration of therapies. Like inhaling? (Hence the name, Inspiration? Just a wild guess)

B. Baxter International (www.hemophiliagalaxy.com) has begun preclinical work on a genetically engineered product for bleeding in patients with hemophilia B. Again, we already have that, so what’s new? With its partner Nektar Therapeutics of San Carlos, Calif., Baxter wants a more long-lasting treatment for hemophilia B. Imagine infusions lasting all week instead of a day, or maybe two weeks!

C. Nastech Pharmaceutical (www.nastech.com) has begun a feasibility study with an undisclosed global leader in the production of plasma products. The study will examine the possibility of developing an alternative delivery method of factor IX without an injection. Not much said about this one: who’s the company? What’s the method? More to come, hopefully!

D. GTC Biotherapeutics, Inc., of Framingham, Massachusetts, has purchased a license to develop a new recombinant factor IX product with ProGenetics LLC of Blacksburg, Virginia.

Currently, the only existing recombinant factor IX product is BeneFIX, made by Wyeth Pharmaceuticals of King of Prussia, Pennsylvania. But there sure is a lot of commercial interest in this hemophilia population, which is a small. Of the approximately 17,000 people with hemophilia in the US, only about 15% are estimated to have factor IX deficiency.

If this is all interesting to you, then see about attending The Coalition for Hemophilia B one-day consumer symposium on Saturday, March 8, 2008, from 9:30 am–5 pm, at the Millennium Broadway Hotel in New York City. For information, call Kim at 212-520-8272 or email her hemob@ix.netcom.com

Great Book I am Reading: Dreams from My Father: A Story of Race and Inheritance by Barack Obama. I thought, oh great, a book ghostwritten for a politician seeking election. But I was wrong. He wrote this in 1995, when he was a senator-elect. I am captivated already: it is honest, intimate, frank and eloquent. This is a guaranteed great read (and not a political endorsement), and I always appreciate a well-written book. I am only on Chapter 3 but it is insightful and informative. Obama has led an amazing life, and in this book, he traces his roots, from his grandparents to his childhood, and the unusual path his genealogy has taken and why. Eventually he returns to Kenya to learn more about the father he barely recalls. This is a look at race relations in America, one man’s quest to make a difference for his race, and a quest for the meaning of family for a man who was raised in several diverse cultures. (For those of you fearing I am making a “statement,” I also have it on my list to read John McCain’s book, Faith of Our Fathers, also about the impact his grandfather and father had on his life. ). Three stars out of four.

Sharpen Your Brain and Relax

Reading the book Mozart’s Brain and the Fighter Pilot got me thinking about hemophilia and the caretaker. I mentioned last week I was reading this book. I found it very enriching and motivating: three stars out of four. Its premise is simple: the functioning of the brain is dependent on coordination between its many components (e.g. memory, problem solving, speed, perception) and each component can be improved if you work at it. In other words, you can make yourself smarter. Author Richard Restak, a neurologist and neuropsychiatrist, presents fun and challenging ways to stimulate your brain while teaching you that it’s never too late to exercise your brain to make it stronger and better. And when you do that, you will enjoy life more.

It’s a great message for parents caring for children with chronic disorders, like hemophilia. I know from experience long ago that we tend to get over focused on the daily care of hemophilia. When we do this, we experience burn out, mental fatigue and sometimes despair. Restak has a solution: we need to exercise the parts of the brain not involved in the things we do routinely–like infusions and hemophilia care. He presents the geography of the brain–locations on the brain where various mental and physical activities are controlled, and how hormones affect the brain. He then shows how we can neglect certain areas of the brain, leading to a deterioration overall in its functioning, and subsequently, in our mood and thoughts.

The brain functions best when it networks with itself. So if one part is overused, and another is underused, the whole brain suffers. If we dwell on certain thoughts as well, especially negative ones, we also risk deterioration in sharpness, memory, and flexibility.

The cure is to exercise various parts of the brain routinely. You won’t know what to exercise if you don’t know where the functions originate in the brain. For example, balance and coordination reside in the cerebellum, at the base of the brain. Restak puts big emphasis on this part of the brain, since as we get older we tend to not use balance and gross motor much. He makes a great case for getting up and getting active! It can actually help you get smarter. I think people who run, jog, dance, play sports well into their middle age know this already, but for those of you who have given it up in favor of caretaking or due to exhaustion, think again. Restak says force yourself to exercise the brain though gross motor movement: you will feel better both physical and mentally.

Restak also advocates many other activities: chess, art, walking, touring. All of these will positively affect very different parts of the brain, stimulating in turn all the brain. Painting or piano playing stimulates parts of the brain that control the hands, where a heavy concentration of nerves lie. Developing manual skills leads to creation of new nerve circuitry, too. Keep a journal of observations; read the same book several times (over the course of a few years, even). I am glad to hear that as I always read books a few times each (good ones, that is).

I only object to his emphasis on Mozart (one of my least favorite classical composers). Music is fabulous, to both listen to and to play, and any music will do. But classical music does have a depth to it that is lacking in contemporary music. So if you have not listened to classical music, pick up some Bach or Rachmaninoff for a real emotional and mental experience. You may find it both relaxing and stimulating, and I think that is part of Restak’s message. When you stimulate new areas of the brain through new and/or varied activities, you actually will feel more relaxed, because your brain will function better as a whole. A wise message for those caretakers with daily stress.

I do love Restak’s upbeat and can-do attitude. You will read this book feeling that you must and will want to get more active, and try new things, perhaps the things you once loved to do as a child. Tree climbing, beach walking, piano playing, crossword puzzle solving, reading, acting, literally smelling the roses… these are not just things that make us happy, they actually alter our brains and get us thinking better. And I am so glad he advocates reading the same books several times, because for the life of me, I don’t recall reading anywhere anything about a fighter pilot in this book. I think I need to work on my memory a bit more!

Try This for the New Year

My last blog for this year comes with a wish for our community: for good health, good parenting and hope. And above all, turn off your television, take charge of your precious time and read!

As an author, reading is essential for my line of work, but I absolutely enjoy it. Reading educates, entertains, motivates and inspires me. From the jungles of Africa to the mesas of unexplored America, to the polar caps, I love to read about explorers; I also love to read about the history of science and disease, how physicians uncover clues about its etiology and treatment. I love to read about the psychology of communication, and child development. And I love history. Nothing can take the place of reading in my life.

Sadly, most Americans miss this kind of joy. Newsweek reported this year that 25% of adults have not read anything this year. And the average American reads only 4 books annually.

I also fell into the trap of not reading much at one time. But since 2000 I have tracked every book I have read, each month, just to study my own habits of reading. The more I tracked, the more it became a competition to see how many I could finish. I’ve been shooting for 25 a year, but I think I can do better in 2008.

It’s incredibly stimulating to meet up with someone who has read the same book you have read. You feel immediately like you’ve both stumbled onto a secret! When you read a great book, you feel like you want to share your discovery with the world.

I am going to try to do that here. As much as I focus on hemophilia-related subjects, I am also going to include a blurb at the bottom of each blog letting you know what I am reading. This puts the pressure on me to read each week for sure! And to read well. I’ve been delighted that people have emailed me to tell me that they read Crime and Punishment for the first time because of my recommendation, or Mayflower, both excellent books.

So give it a try! First, determine how many you read a year on average. How many do you think you could read if you made a conscious effort? Set a goal in 2008 of how many books you will read, and check in monthly to see how you are doing. Do better than the average four, and let me know what you are reading, too.

Just today I received this email from friend and colleague Richard Atwood, who also writes a book review on hemophilia-related literature for my newsletter PEN: “Now is the time of year to pause and to reflect, and it is not too late to make the resolution to read more hemophilia literature in the upcoming year. By reading hemophilia literature, you can smugly realize that you are stimulating parts of your brain that are not stimulated by other activities, and that you are bucking the disgraceful national trend towards less reading by adults. So by all means, read more creative literature to be a better person. And where else other than a romance novel would find a character of Irish descent named Lori Kelly?”

You know, I kind of like that character’s name!

Happy new year, everyone, and make reading a priority in 2008!

And, here’s the current

Great Book I am Reading: Mozart’s Brain and the Fighter Pilot. I picked this up because a) it’s about how our brain works and how to sharpen it to make it work better, and 2) I love the catchy title! I’ve only read the first two chapters, but this looks to be a great book about the physiology of the brain and how to make it work better for better results in life. I’ll let you know next week how I liked it.

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