HemaBlog™

Origins of Rx

2008-05-05T06:51:00.008-04:00

We finished up the latest issue of PEN, and for the feature I focused on branding and advertising by the pharmaceutical industry. While researching this topic, the importance of company and product names kept coming up. What a company calls itself or its products is extremely important for recognition. The new product "Xyntha" by Wyeth is causing a minor stir due in part to its very unusual and interesting name.

One suffix I encountered and was curious about was "Rx." We all now it means "prescription" but why? In our household we love words and love knowing the origins of words. Rx is used in the company names BioRx, PrecisionRx, and Med Pro Rx... all related to hemophilia services.

According to the book Who Put the Butter in Butterfly? "R" is the symbol of the Roman god Jupiter, the patron of medicines. Rx is also an abbreviation of "recipe," from the Latin recipere, to receive. R appeared on top of all prescriptions, denoting "to take": directions then followed. Even the English word recipe originally referred to medical prescriptions. Over time the word was also used for cooking--not unusual, as many of the same herbs and spices in cooking were being used in prescriptions at the time! I guess flavored medicine has been a round longer than we think!

Great Book I Just Read: Ultramarathon Man, by Dean Karnazes. After meeting with Steve Petty two weeks ago, just before he ran the Boston Marathon (and did very well!), I was inspired to read this marvelous book again. Dean Karnazes tells the story of how he came to be one of the top ultramarathoners, those obsessive runners who think nothing of running 15 miles to a marathon as a warm up and then run the marathon, and then "relax" by going windsurfing all afternoon! His story is amazing: running in 120 degree heat for 100 miles, shoes melting; running 199 miles, without stopping, to raise money for a dying girl's treatment; running a marathon to the South Pole! This is a quick read, and may do for you what it did for me: inspire one to get up and get moving! Dean makes you feel as though nothing is impossible. As much as he has accomplished so much, he tells his tale plainly and humbly. I hear he is a very nice guy. After all, he "let" Steve beat him in a race a few years ago! (Just kidding Steve!) Four stars.

Empowered in Pittsburgh

2008-04-27T11:30:00.018-04:00

I visited Pittsburgh this past weekend to spend time with the families at the Western Pennsylvania Chapter of the NHF. What a fantastic event! Kerry Fatula, executive director, and her team did a marvelous job bringing together families from all over Western Penn for a weekend of education, socialization and fun. One thing that struck me right away was the high involvement of women and girls in this community: from girls with VWD to carriers and siblings. More than almost any other place I see that women have really become empowered in this chapter.

Now, I might be a bit biased, since Kerry is a good friend now and I've been to this event before and really enjoy this part of the country, but WPCNHF works hard to achieve its many goals for its families. The weekend started with a Friday evening dinner and excellent talk by Dr. Margaret Ragni, hematologist from the local HTC. I was impressed with her breadth of knowledge about the future of VWD and hemophilia. She spoke about current trials for longer acting VWD and factor VIII, and our chances of successful gene therapy. She spoke in easy to understand terms, despite the difficult nature of the subjects.

On Saturday we had a mix of speakers: Rob Dash of Baxter spoke about insurance reimbursement issues, on which he is an expert. I gave a talk about raising a child with hemophilia, summing up the various things we must learn and undergo in 20 years. In compiling this talk, I was a bit dazed myself reviewing how much we have learned and how far we have all come. The audience enjoyed hearing my personal stories of Tommy, especially the teenage years stories, and seeing photos of him today (like the one of him skydiving! Is there nothing a child with hemophilia cannot do now?). My friend and colleague Diane Horbacz was here from HHS to give her "Karing for Kids" workshop, an excellent program that actively engages children in hands-on learning about hemophilia in a fun manner. Later on, Laureen Temple of CSL Behring spoke about blood clotting, and Virginia Kraus of Grifols (whom I've known for at least 17 years!) spoke about VWD. I ended the afternoon with my empowerment talk, which can be applied to hemophilia as well as many life circumstances. Many companies set up booths and consumers had the chance to visit with everyone and learn more about services and products. That evening was lively, with a full course meal and a very loud DJ with a selection of music that spanned from AC/DC to hip hop to disco. Something for everyone! And there are a few Bob Segar fans in the audience, and I have the incriminating video of Dawn and Rita to prove it!

I met some truly extraordinary families. Some needed coaching in dealing with tricky medical situations: what should they do, who to turn to? Others simply shared with me what they've been through. I was thrilled to see Guy, a young man with hemophiilia, who I have known through the years and watched grow up. Guy is special to me: his story was in my first edition of Raising a Child With Hemophilia, and many of you might recall his story. He suffered a devestating spontaneous head bleed as a baby. His mom's quotation was the longest in the book, serving as a warning to families who instinctively know something is medically wrong with their baby, but who are lulled into thinking it's only the flu, by families and even doctors! Thankfully, his mom persisted and Guy survived. Today Guy is an accomplished and lovely young man, so outgoing, so active, someone his mom should be very proud of!

Two other families stand out in my mind. One is Chris and Candi Brown, a young couple, parents of Zach, who has severe hemophilia. In 16 months they have been through so much, perhaps more than I have been through with Tommy in my whole life, and they handle it with grace, confidence and mastery. I wonder how such a young couple could be so empowered? They embody what empowerment means. Despite all the medical challenges they have come up against, they always find a way through it and learn from it. Also, I had a lovely three hour chat this morning with Mary and David, who have five children. Conor has type 3 VWD, which is severe. Again, they have learned so much, and never blaming others or cursing their fate; they simply and with dignity accept this life, learn from it and even find a lot of humor in their trials. As I often do when I visit with these families, I fly home feeling so lucky to have met them and to share a bit in their lives. And I hope we all meet again! Congratulations to Kerry and her team for a fabulous weekend!

(Photos: The Browns; Laurie and Guy; Laurie and Kerry; Line dancing Saturday night; tired crew after the event)

Great Book I Read: Devil in the White City, by Erik Larsen. I always thought DisneyWorld was the embodiment of visionary thinking--until I read this book. The Chicago's World Fair of 1893 became the spectacular dream come true at a time when nothing like it existed anywhere in the world. In a passion of patriotism and competition, it arose in the minds of two brilliant architects who wanted to outdo the French Exposition, where the Eiffel Tower was unveiled. With only two years, and hundreds of roadblocks including devestating storms, union strikes, and disease, somehow a magical "white" city arose from the mud of Jackson Field, and dazzled the world. What could compete with the Eiffel Tower? A man named Ferris stepped up with an idea for a huge spinning wheel. While this testiment to the creativity and untapped potential of the human mind unfolds, the story simultaneously follows the depravity of the human mind, in profiling America's first known serial killer. Larsen has a wonderful knack for paralleling stories and then bringing them together in the end as the stories historically collide. This book is simply amazing--learn about America during the industrial phase, the event that captived the world, and a bizzare doctor/killer who stalked the visitors. And DisneyWorld? Well, it isn't a coincidence that one of the carpenters hired to help construct the marvelous buildings was none other than Elias Disney, whose son Walt no doubt heard his father's riveting stories about a Magical Kingdom! Four stars.

Celebrations and Struggles

2008-04-20T19:45:00.008-04:00

April 17 is World Hemophilia Day, which was celebrated worldwide this past week. It's a great chance for hemophilia nonprofits to garner public attention and educate about the disorder. This day was chosen as it is the birthday of the founder, Frank Schnabel, who is commonly refered to as Canadian, because the World Federation of Hemophilia is located in Montreal, but who in fact was American! Before we get all proud of that fact, he moved to Canada to get affordable health care. It's taken his organization, the WFH, decades to move from a family run nonprofit to a more professional, world class nonprofit, but it has done so! This should give hope to the many struggling hemophilia nonprofits in the world.

Speaking of struggling, the Boston Marathon, also world class, takes place Monday, April 21, not too far from me. I had the pleasure of dining Saturday evening with Steve Petty, a Bayer rep from Utah, who shares my passion for adventure sports and the great outdoors, although Steve lives it to an extent I do not. He is in town to run the Marathon! I thought we would have a nice 90 minute dinner, but we enjoyed over three hours chatting about everything from extreme running, to religion, to life's mission and purpose, to children (we each have three, one of whom in each family is a diva), to hemophilia in the developing world. Steve would like to come with us sometime on one of our missions overseas, most likely for one of our camps. Steve is also a volunteer Search and Rescue ranger in Utah (my second favorite state)... pretty amazing! He also volunteers a lot for the hemophilia camps, leading young men with hemophilia to do things they didn't think possible, like rafting, kayaking and rock climbing. The most amazing, jaw-dropping thing I heard all week was that he beat Dean Karnazes, the self-proclaimed "Ultramarathon Man," in a race!! (Check out the book by the same title, which is fascinating!) Good luck, Steve!

We lost one race today: Gary Yanga of the Philippines, a beautiful young man with hemophilia and a severe GI bleed, died yesterday. We had been trying hard to keep him going with donations of factor. It wasn't enough. Life is so harsh in these countries, despite the best intentions and skills of his caring doctors, and volunteers, like Father Don Kill, and our program. We offer our condolences to his family over this loss, and it will spur us to continue to work harder for those who still need us. Factor donations are tight, and there is much less to give. We are gratfeul for any donations, and for those who do pull through, and for those patients whose lives are made a little easier from these donations.

Great Book I Read: Shackleton's Way by Margot Morrell. If you like reading about extreme adventure, like I do, you will love this book! The story of Sir Ernest Shackleton, one of history's greatest polar explorer, is world famous and as riveting today as in 1914, when the story unfolds. His Imperial TransAntarctic journey was to have him be the first to traverse the entire Antarctic on foot. But things didn't work out that way. What happens is a tale of survival and extraordinary leadership under the worst conditions on earth that seems unbelievable: the ship encased in ice and sinking, leaving 27 men stranded; striking out in three life boats for a week; landing on remote, inhospitable Elephant Island; then, the greatest boat journey in history, Shackleton and two other men travel 800 miles in the open, frigid sea to another island, and then stagger miles on foot to finally approach a whaling station, and then organize a rescue for the stranded men on Elephant Island. Morrell tells this tale of Shackleton's remarkable leadership in business terms: how you can apply Shackleton's philosophy and revolutionary leadership to your business. This story can make you a better leader in anything you do. Incredible tale that you will never forget! Four stars.

SpringFest!

2008-04-13T22:44:00.007-04:00

It doesn't feel quite like spring yet in New England, but Saturday was SpringFest, the New England Hemophilia Association's annual family symposium. I was very happy to be home, so I could attend this event, a mere 15 minutes from my house. Our new executive director of NEHA, Kevin Sorge, said it best: "This is like family." And it is. We were reunited again, for some of us 20 years now, our sons grown but our desire to see this community continue to progress still burning bright.

Even some of the pharmaceutical and home care reps have been part of our lives for 12-20 years. How fast it has gone by!

Linda Price of Virginia will be thrilled to know I finally met her son Greg, now living in the Boston area. Last time I saw Greg he was about 10. It was a lively time, because of how long we've all known each other and how rarely we get to see one another.

The speakers and topics were good, and the turnout was excellent, despite the rain. There were lots of children, and teens. My own son couldn't make it, and he missed some of his long time hemophilia friends.

The highlight of the day without doubt was a moment that reduced us all to tears: an award ceremony, with our shining star, Leland Smith, age 15. I first met Leland when he was only a toddler. He has hemophilia, and a devastating inhibitor that at times keeps him wheelchair bound, as he was this day. I know his story well, and I can tell you that not many people has endured suffering like this child. The time he has spent in the hospital, the treatments he has undertaken, the missed school, the excruciating and unrelenting chronic pain; and yet he wants to be a doctor, and is an active lobbyist for NEHA. He has traveled to Washington DC and also to the Massachusetts state house to present himself, to help NEHA get bills passed to protect the rights of those with hemophilia. To know how much this kid has gone through, and to see how at such a young age he is active socially, well... we all crumbled inside in admiration of his character and his strength. Leland reminds me of the words: It is in the deepest valleys that the stars shine brightest; it is pressure that turns coal into diamonds. Congratulations to Leland, a true leader of a new generation of young men with hemophilia. Thanks for making such a memorable day for us all!

Great Book I Just Read: Mawson's Will by Lennard Bickel.
Speaking of pain and suffering, you haven't read anything till you have read this book. Dubbed the greatest polar survival story ever, this tells the true story of Douglas Mawson, a giant in the age of polar exploration in the early 1900s. Mawson, an Australian, was already a veteran polar explorer at age 29 when he traveled to the Antarctic in 1911 to seek the magnetic pole and to chart unknown territories. Splitting into three groups, he and two other men part with his crew for a few months and enter the incredibly hostile terrain. Mawson loses both men on his team and is left utterly alone, without enough food, shelter or dogs to see him back, in 50 degree below zero weather with gale force winds.... for one month. How he survives is a page-turning, gripping and miraculous tale. Mawson is responsible for Australia's claim to 45% of the Antarctic. His tale is legend. This was the fourth time I've read this book and it gets better each time. Four stars!

Mission: Dominican Republic

2008-04-09T18:49:00.008-04:00

The last day of our trip to the Dominican Republic was reserved to visit the homes of four families with hemophilia. We started out bright and early, the tropical heat slowly rising with the sun, and headed for Bonao, a pretty town located on the roaring Yuca River. It took about an hour to get there, to meet with the Carlos Manuel and Jose Luis Ortiz. With me were Jeannine Cardoza, executive director of Save One Life (a child sponsprship program), Haydee de Garcia, president of FAHEM, Maria Espinal, nurse at the Robert Reid Cabral Hospital, and Zoraida Rosado, general manager of LA Kelley Communications. Zoraida sponsors Jose Luis, a young man with hemophilia, and our visit would check on how he and his brother were.

Meeting with them was pure joy. The Ortiz brothers are natural poets, and every word and sentence is chosen to express kindness, civility and warmth. No matter that they have hemophilia, that their family struggles economically, that they both have severe joint damage that leaves them with unbending knees and hobbling gaits. When you are with them, you are the most important thing in the world. They exude a kind of hospitality very rarely found. Marisa, their mother, had not seen me in about four years and we pounced on each other with hugs. She laid out a fantastic meal. We then walked down to the river, to see the beauty of Bonao. It was a lovely visit. We were sorry to leave.

An hour later we were searching for the home of the Gimenz family. They live on the fringe of Santo Domingo in a place I am sure very few if any Americans have seen. Dirt roads, towering palms, rows of tin roofs with chickens scuttling everywhere, this village or settlement is remote, not easily accessible and devestating for two children with hemophilia. Angel is only six and almost died in December from a head bleed. Thankfully, his mother, also named Zoraida, was educated by FAHEM about symptoms and knew what to do. Angel was hit in the head and began exhibiting symptoms of a head bleed; he was brought to the hospital that night. A miracle considering Zoraida does not own a car nor have much money, and transportation is questionable and unreliable. Angel's older brother Andres watched and listened as we heard this story, ocaasionally smiling and dropping his head shyly when we looked at him. He had just attended camp with us. An extremely handsome young man of 15, he has a killer smile and a friendly manner. He gave me a brief tour of their tidy wooden home, ony three rooms, shared with four dogs, cats and a hen and rooster in the back. Both boys need sponors, so if you are interested in helping this family, please go to www.SaveOneLifeInc.org and let us know!

Next stop, the Vasquez family, who live closer to the capital and down a small "tienda" or shop selling candy and things. The shop is really just a window from which they display what little they have to offer. Gabriel is the young boy also looking for sponsorship. Last stop was the home of Misael and Jayro Medina. Misael attended camp as a counselor and did a great job. His brother Jayro, deeply religious, has not walked in years. So many bleeds left him bedridden, during which time his muscles atrophied. But he does not whine or complain but shows again that warm hospitality. Though these are men, we will still look for sponsors to help ease their burden in life. Their father only earns about $25 a month, and life is expensive in the city.

Seeing the conditions of the poor, the crippled joints of hemophilia, might leave a person feeling overwhelmed, stunned, depressed. But not us. We feel honored to have met these families, impressed at how confidently they face life's harsh challenges, and motivated to help them financially through our program. We saw the kids at camp who are already benefiting from having a sponsor--they use their sponsorship funds to travel to the hospital, to buy medicine, to stay in school. The Ortiz brother attend college and will one day get jobs in the tourist industry, where they can charm visitors to their land, thanks to the support from Save One Life, for funds, and Project SHARE, for factor. They are one success story among many; and you can help us have more. Adios for now!

Camp: Dancing, Swimming, Learning, Bonding

2008-04-06T18:35:00.014-04:00

Yesterday was so busy at camp I didn't have a second to blog. The day started with some wake-up exericses on the lawn, in this case a dodge ball game. All the campers and staff are wearing T-shirts with the camp name on it--"Yo si pudeo!" After breakfast, the campers listened to a lecture by a lively orthopedic surgeon who used Bob the Puppet (renamed "Pepe") to demonstrate joint damage in a kid-friendly way. Then off to the pool! Wow, did the children come alive! All campers are divided into groups, who give themselves team names (like the Stars, or Alpha-Omega), chaperoned by teens with hemophilia, who used to be campers themselves. And they were wonderful! Like big brothers to their little "hermanos" with hemophilia.

After lunch came another lecture, then arts and crafts, with board games for those who opted out of arts and crafts. Then practice of the big event--a talent show! And what a time it was. What imagination! Each team presented a skit, something to do with hemophilia and empowerment. No one told them what to do. They just instinctively wanted to do skits that showed how they were empowered and who anyone could be empowered. Most of the skits were hysterical, showing comical situations and exaggertaed characters. But one took a serious turn when it showed a father coming home drunk and striking his child with hemophilia, the mother helpless. Domestic abuse is a huge problem in Latin America and the boys deftly integrated this social problem with hemophilia. Prizes were awarded to everyone, and then the real fun began. Dominicans are expert dancers and the DJ (Horatio, former camper) pumped out some wild meregue and salsa for everyone to dance to. Not even hemophilia and arthropathy can keep these boys from dancing. We had a conga line, contests, and everyone--from patient to staff to hematologist and nurse--got in on the fun.

Today was a bit more subdued. At least no bleeds as a result of the dancing, thankfully! The camp celebrated a Catholic Mass in the pavillion after breakfast, followed by heart warming testimonials from the older boys about the role faith plays in their life with hemophilia. They spoke directly to the younger boys, instructing them to pray and keep faith even in the darkness of a bad bleed. Later, everyone went for another swim, and then had lunch. The weather was beautiful, sunny and hot.

Though camp was only two and a half days, close bonds were formed, even for the new boys. We enrolled more boys into Save One Life, to help them financially. Tomorrow we will visit some of the boys in their humble homes.

The bus arrived and everyone departed back to Santo Domingo, a quick jaunt, to congregate at the Robert Reid Cabral Hospital and to await some anxious parents. Happiness shown on the faces of both parents and child as they were reunited. The boys were armed with toys donated from the New England Hemophilia Association, and infused with factor donated from Project SHARE. Does it get any better then that? It was a great camp; it seemed so effortless, but tremendous planning and coordination goes into a project like this. We will miss all the boys dearly as another year passes. Hasta el proximo ano !

Hola! From Camp in the DR

2008-04-04T20:00:00.010-04:00

Tropical, warm, friendly... these words always come to mind when I am in the Dominican Republic, a country that I have been fortunate to have worked with for 10 years now. Today was our first day of camp, the 9th annual "Yo Si Puedo" (Yes, I Can!) bringing together 42 boys with hemophilia for three days of fun, socialization, education and medical treatment. With me are Jeannine Cardoza, Save One Life executive director, and Zoraida Rosado, my co-worker and our company's general manager. Zoraida is fluent in Spanish and serves also as translator for the only two people at camp (me and Jeannine) who are woefully not fluent!

We arrived last night and were met by Mecho Benoit, good friend and executive member of the Fundacion del Apoyo Hemopfilico (FAHEM), the national hemophilia organization. We drove to her sister Haydee de Garcia's home. Haydee is the president of FAHEM. Any baseball fans reading this? Her husband is Damaso Garcia, former second baseman of the Toronoto Bluejays. They have a wonderful son with hemophilia, now 23.

We brought with us lots of factor, toys and supplies for the 42 campers. After a wonderful meal (food is delicious and fresh in the DR) we repacked a bit and retired, worried a little about the weather as it might rain.

We had overccast skies today but no rain. We met up with the boys and their moms at the Robert Reid Cabral Hospital in the capital, Santo Domingo. After some joyful reunions, we toured the new hemophilia wing of the hospital. Most impressive! Funded by the Bill Clinton Foundation, it was amazing to think that just 10 years ago, there was barely any care at all for patients with hemophilia: no factor, little training, certainly no place to call their own. But one thing the DR did have was dedicated people, like Haydee and Mecho, and also doctors Joanne Travers and Rosa Nieves.

Camp was just 20 minutes away at a lush and beautiful location in San Cristobal. Today's agenda was settling in, some athletic and team building games on the basketball court by Raphael, a volunteer phys. ed teacher, arts and crafts, lunch and a lecture by a dentist, which was actually informative and a lot of fun.

We also took the opportunity to interview some of the campers to update their profiles for Save One Life. I won't lie to you: not everything is happy and rosy in the DR. Too many children, far too many, are crippled, espcially the teens, but even some of the younger ones. There are many reasons for that, which I'll try to share tomorrow (if I can blog).

This is a special camp for us. Save One Life rasied enough funds last year to provide 50% of camp funding. We have provided almost all the factor. But most of all, I witness that all those boy I met when they were eight years old, are now young men, returning as counselors, ready to help the next generation. I know I am getting old when I gasp at their height, their maturity, their potential now being realized. Where did those little boys go? Now we marvel at the younger boys, just learning about leaving home, socializing with other with hemophilia and experiencing camp for the first time. Whatever the language or culture, one thing for certain about hemophilia, camp is a winning program for all.

Switching Time

2008-03-29T12:59:00.014-04:00

Insurance challenges was the topic of my talk in Pleasanton, California at a Herndon Pharmacy sponsored event Saturday evening. We had a great turn out with about 50+ patients and parents attending. We all enjoyed a delicious dinner at the Pleasanton Hilton, followed by my presentation on "The Current Storm." The hour-long presentation covers what's happening with hemophilia reimbursement, why it's happening, how it all got started, and what families shoudl do to protect their insurance and choice. California is facing two major challenges: sudden switching of homecare companies by insurance companies, and a 10% reduction across the board on reimbursement by MediCal. Both measures hurt factor providers (340Bs and homecare) and parents are not very happy with the home care switching. For many parents, they don't even know what questions to ask. Insurance is complex, ever changing... and necessary.

It was really great to meet some new parents like Christina and Colleen, patients like Art and Stanley, and families I've known for a long time, like Vicky and Burt, and Betty. All the attendees really enjoyed meeting each other: everyone knew somebody and it was like a big reunion. You could see the comraderie and joy everyone was feeling. Before everyone left we all sang happy birthday to Mark Helm, president of Herndon, who just had his 50th birthday.

Thanks to Zuiho Taniguchi of Herndon Pharmacy for inviting me and handling all the logistics, and to Mark Helm, for sponsoring this enjoyable event. And please see www.herndonpharmacy.com for more information on Herndon Pharmacy, a contributor of factor to Project SHARE for patients in the developing world, and provider of hemophilia services in the US.

(Photos: 1) Colleen, Laurie, Christina 2) Laurie, Beth, Aban, Leslie 3) With Zuiho and Mark, of Herndon Pharmacy)

Book I Just Read: Enough About You, Let's Talk About Me: How to Recognize and Manage the Narcissists in Your Life
Les Carter

We all know people who love to talk about themselves. And we all know bores. But these are not necessarily people with bona fide narcissistic personality disorder (NPD). Carter explains very simply and easily what sets these people apart from people who just love talking about themselves. People with NPD can make your life miserable especially if you are married to them, have one as a sibling or parent, or as a boss. They lack the ability to be empathic and are notoriously difficult if not impossible to get into therapy or even get to see your side of a situation. Being with them is positively exhausting and narcissists are great at making you feel guilty and responsible for all that is wrong in a relationship. Carter offers excellent scenarios and great tips for dealing with those with NPD. Carter has a fundamentalist Christian background, and I did not like or agree with his view of children, despite that he is a popular author and therapist, and you may not agree with his linking original sin to this disorder. I also found the book very light, mostly anecdotal, with dialogues from his sessions with patients. This is a good book if you are new to psychology or narcissism. Two out of four stars.

Parents Sue Over Birth of Child With Hemophilia

2008-03-25T18:35:00.007-04:00

The newswires are alive with this story: An Australian couple are suing their doctor because their baby was born with hemophilia. The mother knew she was a carrier. She knew in-vitro fertilization could not guarantee a daughter... and the result is a gorgeous baby named Jess who apparently has caused such emotional damage that the parents want financial retribution. Now age three, Jess is at the heart of what could be the first of a kind lawsuit.

"We love our little boy, but we are very sorry he has to go through so much in his life," the couple told The Sunday Telegraph (Australia)."We tried everything to avoid this situation, and now our boy has to go through all the pain and treatment in order to survive. We now face the fact that Jess will require treatment for the rest of his life."

Part of me wants to shriek get real, grow up, toughen up, face the real world. Suffer? After reading about what inhibitor patients endure, it's unlikely Jess will suffer much. After visiting so many developing countries where NO treatment exists, Jess is living a dream come true. Good family, great health care system, plentiful medicine, no threat of viruses... from where I stand they are very lucky people... yet still they are seeking retribution.

http://www.foxnews.com/story/0,2933,340737,00.html

"Paul and Fiona (who do not want to reveal their surname) are claiming damages for the shock and nervous anxiety caused by the unexpected nature of the birth." Get in line... thousands of families have dealt with it--with no family history, no warning whatsoever. Their claim seems to negate all the emotional suffering the rest of us go through--and eventually get over.

I was speaking to another mom today about this case, which has everyone buzzing. She thinks the parents are just not informed about hemophilia today (the mother's brother had hemophilia and was crippled). But I wondered, are they not aware or too aware? Are the parents profiting from something we all learned to accept, and they under took this risk willingly? They claim no one told them Fiona was carrying a boy, despite ultrasounds (and she didn't ask?); had she known she would have aborted. Are they truly in agony, three years later? Are they just not aware of how good life can be with hemophilia in developed countries? Or are they looking to make a killing from the physician's insurance, which also drives up health care costs for everyone?

But wait. In speaking with this mom tonight, I realized we are only skimming the surface. Snap judgments based on media reports are dangerous: what is the media not telling us? And despite what they print and even what the parents tell them, perhaps there is a bigger, more important plan here. Perhaps the situation wasn't handled well medically. After all, they were assured at some point that what was selected and implanted was a female embryo. They must have paid good money for this procedure. Throughout her pregnancy, if she is correct, no one told the mother she was carrying a boy (hard to imagine no one thought to ask!! and where were the ultrasound pics?). And if the baby was a boy and the doctor knew, could the doctor have put the baby at risk of a head bleed by having a natural birth (the article did not say if the baby was born naturally or by C-Section)? If all this is true, maybe there was true medical negligence and what is being sought is not so much emotional damage (obviously they are not so damaged they cannot be good parents to Jess) but a punitive measure against the medical facility that failed them in so many ways. In this manner, the lawyer has to follow whatever strategy works to get that justice.

Tough case to judge. I hope the parents at heart are grateful for Jess. Knowing how fiercely almost all parents of children with hemophilia I have met love and protect their children, it is painful to read about the burdens they say this child is causing them --even if it is only said for the benefit of the courts to seek justice for all. It will be interesting to hear more of the facts come out and hear from the hemophilia families in Australia comment on this case. Thoughts, anyone?

Book I Just Read
The Alchemist by Paulo Coelho. I generally like Coelho who has a lovely, simple style of writing but usually manages to capture fundamental themes on life and the human character. Here, Santiago, a shepherd, leaves his life behind to follow his destiny after a dream about searching for a treasure at the Egyptian pyramids. He meets assorted people along the way who help him rethink his journey and ultimately his destiny. This book is a quick read, enjoyable, easy to understand. I don't think it is Coelhos's best by a long shot. It's a bit too simple, though I enjoyed it because it was about traveling, far way places and above all following your destiny. I thought the ending was a disappointment--too much like a neat Hollywood ending. The book has met with mixed reviews. If you are spiritual, seeking or just like light tales with thought-provoking questions, you may enjoy this. Two out of four stars.

Happy Easter to All!

2008-03-23T21:36:00.003-04:00

Please visit us on Tuesday for our regular blog.

(Photo: Catholic Communications)

The Most Famous Celebrity with Hemophilia

2008-03-16T21:10:00.005-04:00

I have my friend and colleague Richard Atwood of North Carolina to thank for this entry, and my editor Sara Evangelos for editing this. I enjoyed it so much I had to use it tonight. This is taken from Richard's review of two books, listed below, and it will also appear in PEN in May. I wonder how many in our community knew of this?

The lives of Elizabeth Taylor and Richard Burton intersected with volatile chemistry, and a small part of that mixture involved hemophilia. Yet the role of hemophilia in their celebrity relationship is difficult to uncover, and was possibly meant to remain hidden from their public presence.

Elizabeth Rosemond Taylor was born in 1932 in London, England, and Richard Burton (Richard Walker Jenkins) in 1925 in Pontrhydyfen, Wales. They both began acting careers, and their personal relationship developed while filming the movie Cleopatra in 1962; the love affair between Antony and Cleopatra seemed to transpose into the torrid affair and two stormy, highly public marriages of Richard and Elizabeth.

In Elizabeth, it is revealed that in n May 1964, Richard was upset after being booed as the lead in Hamlet on Broadway. He began drinking heavily and then argued with Elizabeth, who was watching a Peter Sellers movie on television. During their argument, Richard kicked the television screen with his bare foot and cut a toe to the bone. The blood flow wouldn't stop for an hour. At the hospital, Richard received a dozen stitches. There, Elizabeth discovered that Richard suffered from mild hemophilia, a condition he had known about since childhood, as four of the Jenkins brothers had "the disease of kings."

As a precaution, Richard began using an electric razor to avoid nicks while shaving, and Elizabeth ensured that a supply of vitamin K was available for his hemophilia. When Elizabeth learned that there were "more than a hundred thousand sufferers in the United States alone," she and Richard contacted the National Hemophilia Foundation for ways to raise funds for public awareness. In June 1964 the couple established the Richard Burton Haemophilia Fund, with Elizabeth as chair. For raising hundreds of thousands of dollars, the fund was recognized in the June 17, 1964, issue of the United States House of Representatives Congressional Record, and in the June 27, 1964, issue of the British Medical Journal.1

Richard Burton: A Life is a an in-depth biography, and yet hemophilia is mentioned only once and the Richard Burton Haemophilia Fund is not mentioned at all. Elizabeth contains a short section titled "Richard's Hemophilia." The index includes two listings for hemophilia, under "Charities and Causes" and Richard Burton; but there is no listing for the Richard Burton Haemophilia Fund, although NHF is listed.

Richard Burton had more pressing medical problems than his hemophilia. Drinking and smoking adversely affected his liver and lungs, and he also suffered from epilepsy and acne. He blamed his arthritis on neck and back injuries from playing rugby as a youth, and on the "weak Jenkins bones." Richard's hemophilia didn't prevent him from fighting, and was only mentioned in the book because of the 1964 bleeding incident. For some reason, perhaps the for fear of being stigmatized, the role of hemophilia in Richard Burton's life never reached center stage.

Source:
Richard Burton: A Life
Melvyn Bragg, 1988. New York, NY: Warner Books. 643 pages.

Elizabeth
Randy Taraborrelli, 2006. New York, NY: Warner Books. 671 pages.

Brainstorming in Naples

2008-03-10T07:25:00.013-04:00

I spent this weekend in beautiful Naples, Florida, attending a Bayer Multidisciplinary Board meeting. This is a fascinating chance to brainstorm on concerns within the community with some talented and diverse people, to discuss current insurance trends, and hear about new ideas on treatment coming from a manufacturer.

One the most interesting segments was listening to Dr. Glenn Pierce, VP Clinical Studies at Bayer, and former NHF president, share progress on Bayer's longer lasting formulation for Kogenate FS. While we wait for a cure, which could take years, longer lasting factor could be the next great thing for our treatment. Imagine prohylaxis that only requires one shot a week, but is as effective as three shots a week.

As always, I am humbled by how fortunate we are to have in our community so many dedicated to our welfare, and who joined us this weekend, such as Dr. Craig Kessler, currently chair of MASAC, Dr. Prasad Mathew, Regina Butler, RN, and Mike Rosenthal, formerly of the Hemophilia Association (Arizona). And as consumers we are grateful to be included in these round table, informal gatherings. This weekend I was able to see my buddy Rich Pezzillo, friend Kyle Callahan (former president of HHS), and fellow mother Shari Bender, whose husband Steve sits on the NHF board.

Whiel I cannot share the topics we discussed, I can say that these opportuities are golden to learn and to give feedback. If anyone is offered the chance to serve on any manufacturer, HTC or homecare advisory board, I strongly encourage them to participate. Thanks to Bayer for providing us all this wonderful opportunity to serve! (Photos: Rich Pezzillo and MIke Rosenthal; Craig Kessler with Shari Bender and Laurie Kelley)

Great Book I Just Read: The Truth About the Drug Companies by Dr. Marcia Angell. You may think this is a strange juxtaposition, after I just attended a drug company sponsored meeting, but I am researching an article on marketing by the pharmaceutical companies. Angell, former editor of the prestigious New England Journal of Medicine, has an axe to grind for sure, but reports on the behind the scenes maneuvers by "big pharma" as it attempts to weild influence over both consumers and law makers. While this is mostly about pharmaceuticals (pills) and not biologics (typically injectibles, like factor) it nonetheless will open your eyes and make you more critical when you order or purchase any pharmaceutical product-- and critical when you meet with your physician. Angell describes practices such as how the drug companies influence physicians to prescribe their products through gifts, junkets and marketing programs. Most interesting is her claim that the cost of marketing is much greater than the cost of R&D. It's well known that the drug industry is America's most profitable, and she discusses some of the reasons why this is so, and perhaps why it should not be so. Not totally applicable to the factor industry, but it is excellent food for thought. Three/four stars.

Those Very Special Inhibitor Families

2008-03-02T23:34:00.006-05:00

Inhibitor families are a special lot and my admiration for them just continues to grow. For the past six months I've been interviewing parents and patients for my new book on inhibitors and I have learned what amazing hardships they face, and with such courage. Though I helped facilitate the Novo Nordisk Consumer Council for the past two years, I still didn't have a full appreciation of their lives. This past week in New York City we inaugurated a new group of parents and patients for the Consumer Council, and I feel better able to represent their needs by knowing more about the medical care, parenting concerns and social issues they face.

We had a wonderful time on Friday. Meeting at the Westin Hotel Times Square for a full day, the marketing team at Novo Nordisk and I presented questions and listened to nine consumers share their experiences, thoughts, suggestions and concerns. None of them had ever met one another, as inhibitor patients are pretty rare and in a country as big as the US, it is hard for them to meet. The Novo Nordisk Inhibitor Summits brought inhibitor patients together for the first time two years ago, and yes--for all who are reading this--there are going to be two more this year.

We had breakout groups, exercises and ice breakers. One ice breaker--meant to help us get to know one another--asked each participant to identify themselves with an animal. Everyone chose different animals, from a kangaroo to a dog to a lion. But Schlander chose an ant--unusual because almost no one in these types of exercises ever chooses an insect. Why an ant? Because though small, they are strong in groups and can accomplish something that seems impossible, given their size. Given that this group will be together for two years, it was a perfect animal to choose to highlight what a small team of dedicated people might and will accomplish.

Great Book I Just Read: Blood, by Douglas Starr. Four stars! This book took me a while but it was well worth it. Fantastic overview of the history of blood. It starts with the story of a madman running naked through the streets of Paris... reads like a novel but is packed with information about the meaning of blood in society, medicine and business. Learn about its incredible importance during World War II, and how much we advanced our knowledge of blood because of the war. Fully half of the book is devoted to the hemophilia holocaust, and I read with sadness and pride about our community, and its fight to bring safer measures of blood treatment and justice to the victims. It was startling and impressive to read about the leadership and courage of people like Bruce Evatt of the CDC, and Corey Dubin and Dana Kuhn of COTT, true heroes in our midst even today. I had read the history of the HIV infection before, and even watched the HBO movie about it, and still see Corey and Dana at events. But.. time goes on, and being human, we all tend to forget the past. This book reminded me of how privileged we are to have these warriors; how lucky my son and anyone born after 1985 are because they benefited from their perseverance to get a settlement from the government and drug companies, and have safer measures. And they still persevere in protecting our blood supply even today. Blood is required reading for anyone involved in the hemophilia community on any level.

Best Film on Hemophilia

2008-02-24T19:11:00.003-05:00

Tonight are the Academy Awards, and I am bound to watch the end to see if my favorite actor, Daniel Day-Lewis, wins Best Actor for his portrayal of a wildcatter in "There Will Be Blood." The movie's name could have aptly suited its rival, "No Country For Old Men" (which I think is the better film overall and should win Best Picture) with the amount of blood shed in it. But blood is something Hollywood and its fans feast on these days.

Blood is our domain, too. The Awards got me thinking of movies about hemophilia. And the first that came to mind is the spectacular "Nicholas and Alexandria," the story of the last Tsar of Russia, whose son Alexis had hemophilia. Actor Michale Payton uncannily resembles the Tsar. The movie is based on the Pulitzer Prize winning book of the same name, whose author Robert Massie, has a son with hemophilia (who lived, oddly enough, about 2 miles from my house when Tommy was born. I happily met him and confided his father was one of my favorite authors). The book is of course much better than the movie, but the movie does a tremendous job of bringing to the screen one of the most endearing love stories, most tragic leadership failures, and most climatic political outcomes of the 20th century. No spoilers here: everyone knows how the story ends. The royal family is gunned down and Lenin assumes control of the country, ushering in the Communist regime. The movie invites you into the intimacy of the royal family, reveals the sinister designs of the monk Rasputin, and relives the horrors of a world at war. As painful as watching little Alexis suffer is watching his father sign a document, renouncing the throne--300 years of Romanov rule ended--then turn and cry like a child.

It's interesting that the movie opens with the birth of Alexis, the long awaited heir to the Romanov throne. But immediately the family, who knows hemophilia is a risk, sees signs of the disorder. I strongly encourage you to rent this movie through NetFlix, or purchase it on Amazon to get an appreciation of historical hemophilia. Never before or since has Hollywood so carefully and beautifully made hemophilia the center of world events, or the center of such an epic film.

The Power of Alternative Medicine

2008-02-17T21:52:00.007-05:00

On Saturday I drove down to Rhode Island to visit my friend Rich Pezzillo. Rich is a 24-year-old with hemophilia and inhibitors. One of the toughest cases I have ever seen. He's had quite a year this past one. I wrote about him in my blog December 2006, when I visited him in the hospital. He was in excruciating pain then. It was very hard to be with him for three hours, because he was in constant pain, deep pain, apparently from his sciatic nerve. He is only a bit older than my son, so it was hard as a mother to see someone so sweet hurting and not be able to help. And Rich really is sweet, a great guy.

And so is his dad, Richard. I spent four hours interviewing Rich Jr. for my book on inhibitors and came away for a whole new appreciation of parents. Richard is a mechanic and small business owner, and father of three wonderful sons. Two have hemophilia, Anthony and Rich. But only Rich has inhibitors. What they've been through could fill a book. But I marveled at the father, and how devoted he is to his sons. Rich told me of the time when he was hospitalized in Vermont while at college, and his dad drove up from Rhode Island every other day to visit him. That's a long ride, and a huge commitment when you own a business.

And when the doctors in Rhode Island informed Richard that his son would never walk, he refused to accept it. He was told the inhibitor bleeds had done too much damage to Rich's nerves. Rich would always be wheelchair-bound. Through sheer desperation, Richard found a way, oddly enough, through karate. Anthony, despite having hemophilia, is a karate master. When he injured his shoulder, he was told by his fellow karate teammates to try a renowned acupuncturist in Haverhill, Massachusetts, about 15 minutes from where I live. When the visit cured Anthony's injured shoulder, Richard convinced Rich, who put up a fight, to see him. They have gone weekly over the past year.

Now, I've seen Rich many times, at NHF meetings and at advisory board meetings we are both on: he is always in pain, and almost always in a wheelchair. On Saturday I had a shock. When I saw him, he was standing completely erect, relaxed, with no wheelchair in sight! I first thought "He's so tall! When did he grow?" I never saw him at his full height! I was delighted. We chatted for four hours, with no interruptions of pain, as in the past. Richard is convinced it was the acupuncturist, who told him that all the wheelchair time had compressed a nerve. The nerve, overstimulated, didn't know how to stop sending pain signals. With treatment, the nerve stopped, and Rich is much better.

It's an amazing testiment to the power of alternative medicines. I think I am going to check out this guy for my right shouder. Too much scratch-pad movements while writing this book. And look for Rich at more hemophilia events. If he had high attendance while in dire pain, you can imagine how much he'll get around now! Indeed, he said aloud how he couldn't wait to go to Istanbul for the WFH meeting in May, after which we heard a shriek from his mother in the kitchen. Apparently he hadn't told her!

Don't Quit Your Day Job

2008-02-10T20:47:00.000-05:00

I had a nice visit this week from a young man with hemophilia named Tom. Not my son, another nice young man named Tom. I have determined that this Tom, age 26, is the person with hemophilia who now lives closest to me. Even my own son doesn't live with me anymore, so he can be a substitute.

Anyway, the way we met was strange. Last September I decided to rip up the carpet in my basement, which was in dire need of extermination, after years of pets and children had taken their toll. I hired a local flooring company to place lovely tiles that are immune to regurgitated dog food and ground up Doritos. One of the three young men tiling my floor asked me, "Does your son have hemophilia? Because I do." He had seen photos on my basement walls of me with Paul Newman at Camp Hole in the Wall (oh yeah, can't miss that photo), and put two and two together. Here he was, on his knees all day, using really sharp cutting tools to tear up the carpet! We talked for a long time, and I sent him away with all kinds of books and newsletters, and told him to contact me if he ever needed anything. He only lives about three miles away.

So this week Tom did stop by. He quit his job. Tom, Tom! What were you thinking, I wailed, as if he were my own son. Now he has no insurance and no way to pay for factor. No unemployment checks, nothing. Tom also didn't know what to do. He had never really been connected to the community, even though, as I learned, he and my son Tommy had the same nurse as children.

My first thought was, don't get any more factor sent you from your home care company. If his rep decides to send a quick shipment and does not check in first... Tom could be stuck with a bill he could never afford. Did he contact his home care company immediately to stop any more shipments? No. I made him promise to call that afternoon, so he will not accidentally get stuck with a big shipment that would eventually ruin his credit rating.

Did he tell his HTC? No. That would be his next call. Does he know what product he uses? Yes. And thankfully, his home care company enrolled him in a coupon program, so he has lots of coupons. Cash them in! He should get some free factor.

After talking, Tom saw his error. A person with hemophilia if at all possible should never just quit their job. So much preparation needs to be done first. At least we have lots of help in this community. From ACCESS to PSI to the Hemophilia Hotline, Tom has many resources to contact for advise and help. And as he walked out the door (I couldn't tell if he was happy or sorry he came), I handed him this issue of PEN--"What To Do When You Lose Your Insurance." The forlorn young man on the front could have been him.

Guys with hemophilia--don't quit your day job. Tough it out for the sake of your health. Or I am coming to lecture you, too.

The "Bloody" Ring Tone

2008-02-03T20:03:00.001-05:00

I've rediscovered ring tones. It happened last weekend at Kerry Fatula's. While chatting in the kitchen we heard a ring tone and everyone went silent, wondering whose cell phone it was. It was the dinosaur in "Jurassic Park III" (on TV), you know, the one who swallowed the cell phone and then... well, if you know the movie you will know the outcome. We all acknowledged that has to be the most famous ring tone in history.

Kerry Fatula and I thought it would be funny if she and I had our own ring tone (kind of an inside joke, right, Kerry?) I've been using the odd little pings and rings provided by Apple, which are kind of lame. When I use them, I can hear a distinctive noise come on and know immediately who I do not want to answer. (Kerry is not one of those.)

So I though it would be fun if I selected ring tones for each member of my family. This gets to be very fun and creative, and addictive. I am still trying to find one for Kevin. Hmm, a long, long time ago (21 years to be exact) we each suggested songs for our wedding. I picked "Better Be Good to Me" while he chose "Under My Thumb." Now, I would choose this for Kevin, but the Rolling Stones don't allow their songs as ring tones, at least not through iTunes.

Tommy: what to use for the young man in my life? Kevin, as a joke, suggested looking for something with bleed in it. I logged on to iTunes and typed in "bleed." Popular name for songs, apparently.

"Bleed"--Anna Nalick, Col, Puddle of Mud, Action Action, Angel Dust, Blitz, Collective Soul, Dear Leader, The Fade, Fat Jon and
Styrofoam, Gary Numan, Floatsam and Jetsam, Further Seems Forever, Godhead, Ill Repute, Intuition, Joel Rush, Justin Manning, Matter, Michael Lord, NLX, No Man, Neil Zaza, Oxymoron, Shiloh, Sinch, Seize the Day, Scar'd Sanity, Sentenced, Smoke of Oldum, Soulfly, Vixen, Wumpscut. I am not making up these band names. I couldn't be that creative.

A good ring tone for hemophilia, with the widest assortment of artists and genres. Something for everyone, which is good because many of the songs are heavy metal and the lyrics aren't so nice--apparently when they say "bleed" they are not referring to their own blood. But if these don't strike your fancy, try these:

"Bleed it Out" --by Linkin Park
This is what happens when you don't infuse fast enough.

"Bleed Alone"--Haste the Day
A good ring tone for all the single guys with hemophilia.

"Bleed and Blister"--Moneen
A ring tone for when you wear shoes that are too big.

"Bleed Black"--AFI
A ring tone for when you have an upper GI bleed?

"Bleed Forever"--Super Furry Animals
A ring tone for while we wait for a cure.

"Bleed from Within"-- The Music
A ring tone for those prone to joint bleeds.

"Bleed Me an Ocean"-- Acid Bath
A ring tone for when you forget your factor.

"Bleed Together"-- Lovedrug
A ring tone for hemophilia camp!

"Bleed, Everyone's Doing It"-- Spill Canvas
Could be the ring tone theme song for all with hemophilia?

"Born to Bleed"-- Dirty Sweet
A ring tone for those who inherited their hemophilia.

"Brother Bleed Brother"-- Finch
A ring tone for your sibling.

"Let it Bleed"-- The Rolling Stones
Not good advice, but you can't get it in a ring tone anyway.

"Let it Bleed Again"-- God or Julie
Still not good advice, which we don't want to be reminded of in a ring tone.

"As I Bleed"-- Bipolar
A ring tone for an infusion.

"Please Bleed"-- Ben Harper and the Innocents
A ring tone for when you want to stay home from school.

"Punch Me I Bleed"-- Children of Bodom
A ring tone for those who don't have lifetime caps.

"Bloody Nose"-- Earlimart
A ring tone for when you say the above to someone.

"Bleeding"-- Ignite, Prom Kings, Todd Rundgren, Sixpence None the Richer, Five Finger Death Punch, Delerium, Sprung Monkey, Tiger Lillies [very weird], One King Down, Jacksom Rohm, Venom, Raindancer, Cord, Raunchy
A ring tone for when you first get diagnosed.

"Done Bleeding"-- Sebastian Bach
A ring tone for those on prophy?

"Bloody Reunion"-- Molly Hatchet
At last, a ring tone theme song for NHF meetings

"Through His Blood"-- Bloody Sunday
A ring tone for grandchildren of those with hemophilia

"Only Women Bleed"-- Alice Cooper
A ring tone for those, of course, who have VWD

"Bleeding"-- F5
A ring tone for those with Owren's disease (factor V deficiency)?

And my favorite... "You're Bleeding" by NOFX, as in no factor X?

And what about Tommy? What ring tone did I finally choose? Well, he's a musician, loves heavy metal rock and is picky. He chose Slipknot. Yes, Slipknot. Those weird, carnival-faced, mutant KISS-like boys who like to scream. The song?

"Wait and Bleed." Yikes.

Great Book I am Reading: Blood: An Epic History of Medicine and Commerce, by Douglas Starr. This book should be mandatory reading for anyone involved in the hemophilia community. I don't know why I have waited this long to read it. Starr reveals the history of blood, as a source of fear and magical beliefs, and eventually blood as a commodity. In the latter half, the book focuses intensely on our community and the contamination of 10,000 of our own. At 400+ pages I will be reading this for a while, but it is already four stars out of four.

There Will Be Blood

2008-01-27T19:41:00.001-05:00

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 like my son, wants to make a career of music. Look for him at future hemophilia events; I have a feeling he is going to be one of our youth leaders someday.

After the interviews, the youngest boys, Stephen and Colin, decorated me and Kerry with tattoos: a butterfly for Kerry; a psychdelic 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 reveiws 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"

2008-01-14T12:13:00.001-05:00

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

2008-01-13T22:19:00.000-05:00

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. Kevin bought this for me for Christmas. 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. Father's Day gift for Kevin?). Three stars out of four.

Sharpen Your Brain and Relax

2008-01-06T21:57:00.000-05:00

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

2007-12-31T19:37:00.000-05:00

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.

Merry Christmas!

2007-12-24T08:36:00.000-05:00

We at LA Kelley Communications--Julia Long, Zoraida Rosado, and I--thank all of you for reading this blog all year long, and for subscribing to our services. We look forward to serving you in the new year.

Wishing you all a Merry Christmas on this Christmas eve... and may you all have a successful, healthy and happy new year!

Laurie Kelley