July 2007

Hemophilia in the Philippines

We enjoyed a visit last Friday by Father Don Kill, a Columban priest who has a mission in the Philippines. He operates a home for teens without families or homes. About five years ago he discovered a boy on the streets who was unable to walk, and whose family was unable to care for him. Father Don took him in and through a variety of testing to find out what was wrong, discovered that “Dodong” had hemophilia; even Dodong didn’t know it. Father Don contacted his home parish in Toledo, Ohio, for help, which then contacted an Ohio NHF chapter, which then contacted us! Dodong soon had a donation of factor.

Since then, Father Don has discovered about 20 children with hemophilia throughout the province he works in. Through our partnership, we are bringing care to all. Father Don registers the boys with the national hemophlia organization, has them tested at the HTC in Manila, and requests factor donations from Project SHARE. As Father Don is a trusted colleague, we also enrolled many of the boys in Save One Life, our child sponsorship program. So in addition to factor, these boys are getting an annual stipend of $240 to ease the burden of poverty and a chronic disorder.

Every year Father Don comes to the US to visit all his sponsors and to fund raise. This includes a visit to us, to brief us on developments, to collect more factor to bring to the Philippines, and to go to lunch with us. This year we made extra contributions to pay for having some of the boys travel to Manila for testing, and also contributed to pay for antibiotics for one young man with inhibitors, who is having a very rough time currently. Father Don is a valued partner in care, and our boys in the Philippines are fortunate to have such an angel on their side. And so are we!

If you’d like to learn more, visit www.SaveOneLife.net

Inhibitor Summit in San Diego

I am in California all this week and just spent the weekend in San Diego at another great Inhibitor Summit, this one with a record number of attendees from across the US. The energy at these events is tremendous, and the parents and patients are actively involved in all sessions. I was glad to see many people returning from last year, which made this very much like a reunion of friends.

Friday began with a wonderful review of inhibitors by Susan Karp, RN (University of California, San Francisco), who is well known and respected in our community. In the evening after dinner, a fun yet insightful icebreaker by Heather Huszti, PhD, a psychologist who works with families with hemophilia, who had quite a few fans in the audience. The activity taught us a lot about teamwork.

The Main Event on Saturday was chaired by Dr. Guy Young, now based in Los Angeles, who also gave us an overview of treatment strategies. And an incredibly inspirational talk was given by 23-year-old Rich Pezzillo of Rhode Island, whom I am honored to call my friend, about his experiences with inhibitors from age 18 to age 23. Rich has suffered tremendously and faced many hardships in the last five years, but it has only strengthened his resolve to live life to the fullest and deepened his faith. His speech really set a positive and uplifting tone for the day.

There were sessions on venous access, diet, surgery, parenting, transition and ITT. The day ended with a great educational talk by Nathan Wilkes of Utah, who has a son with an inhibitor, on what he has learned about advocacy.

See photos of the event here.

Well, the day didn’t actually end there. It ended with an amazingly fun celebration, under a covered tent, with a traveling petting zoo for the kids–complete with monkey, chinchilla and tortoise. And for the grown ups? Karaoke and dancing! Wow, do I have some incriminating photos of some of our community members! Only half kidding, just some memorable shots of dear friends having a great time. I do recall: Matt Compton and Gar Park singing “I Wanna be Sedated”; Chris and Leland Smith rapping “Walk This Way” a la Run DMC/Aerosmith with Ezra Robison; Shuantaye belting out some amazing Beyonce; Eva cutting some amazing dance moves; Eva, Shantaye, Doreen and the ladies looking like “Dreamgirls”; and everyone (me included) doing the electric slide! It was a wonderful time and we all thank Novo Nordisk for sponsoring this fabulous event that not only educated us, but brought us together to make happy memories and new friends.

I have the greatest admiration for families with inhibitors and what they endure, and am impressed with their exceptional medical knowledge. Congratulations to them all for their perseverance, fortitude and achievements!

Next Summit? In Dallas, October 6. Check out www.inhibitorsummits.org to see if you qualify to attend. I hope to see you there!

Anniversary of Tsar’s Death

Today, 89 years ago, Tsar Nicholas II of Russia and his entire family, including Alexis, the prince who had hemophilia, were assassinated in Siberia. This event shocked Russia and the world, and the elimination of the Tsar and the monarchy fueled a bloody civil war in which tens of millions of Russians died. The deaths of the Tsar, Empress Alexandra and their five young children have been the subject of many books and movies. One recent book is “The Kitchen Boy,” by Robert Alexander, which I just happened to read last night, not even aware that I was reading it on the eve of the anniversary.

The book is fiction, but draws on certain facts, and is narrated by the “kitchen boy” who served at Ipatiev House, where the royal family was imprisoned. As a servant, he had access to watch all that happened at the house, including the family’s death. He becomes close to the royals, and details their personalities, flaws, loving manner toward one another, and even their brutal deaths. The “kitchen boy,” now 94, lives in Illinois and wants to share some details he has never revealed before to his granddaughter. He records his memoirs, which becomes the narration of past events in the book. He eventually reveals who killed the Tsar and his family, and what became of the two missing bodies, believed to be Alexis and Anastasia (about whom there are also many movies and books). He reveals that actually Alexis and Maria were missing, and he tells how and why, and how that impacts his life, even now.

The book is easy to read–I read it in one sitting–and the language and style is a bit too easy. It reminded me of the kind of book we had to read for junior high over the summer. Interesting but light. You can almost believe the conversations and events took place, and I think that’s the best part of the book–the way Alexander makes the royal family come to life. But the book loses credibility towards the end. It tries to read like a da Vinci code a la Dan Brown, but it’s much too simplistic a plot for that. The plot takes a sudden radical twist at the end, which kind of left me head-scratching. I just didn’t get it.

Part of the ending included reference to someone with hemophilia (no spoilers) who had mild hemophilia, apparently had no manifestations of hemophilia his whole life, then got into a car accident, hit his head on the steering wheel and died at the scene. I am not a doctor, but this just didn’t sound accurate to me. There were plenty of accurate references to Alexis’s hemophilia, and how the poor boy suffered.

All in all, I would recommend this as a read, but don’t expect a great historical book or a great mystery. It’s light, not perfect, but a great summer beach book that can be read fast, and you may come away with a new appreciation of the Russian royal family. Hemophilia is a pervasive theme throughout the book, and plays a part in solving the mystery of what happened to the royals, and the two missing bodies. (Two stars out of four)

River Wild!

I am writing from Maine, a great get-away state, full of natural wonders and adventure. Every year we try to go white water rafting as a family. It is an incredible adventure! It appeals to everyone: and it is impossible to feel sad, angry, distant when you are battling pounding waves and skirting huge rocks!

Tomorrow we are tackling the Kennebec River, and it’s not an ordinary day: there is a scheduled dam release, which means eight thousand cubic square feet of water will flood the river, causing enormous waves. Adrenaline injected, heart thumping excitement!

Rafting is a great team-building exercise in addition to just being fun. For families, work colleagues and friends, rafting requires excellent communication skills and solid teamwork. Our guide is clearly the leader, and we are his team. He shouts commands while steering the raft, and we paddle, rest and give feedback as he needs it. The constant action as we battle wave after wave, and huge rapids, means that we have no time to argue or question his leadership. As we have children with us, we are constantly watching out for each other, to ensure safety. It’s fantastic to watch your child’s confidence grow as he or she faces his or her fears, overcomes them, and takes pride in completing a very challenging and often frightening course. I always learn a lot about teamwork and about myself when I raft.

We infuse before heading out for the day. I know a lot of kids with hemophilia do white water rafting, and it’s a great physical activity for kids with hemophilia.

Afterward we’ll have dinner with the entire rafting group and laugh about our fears and our adventures. What could be better than this? Well… skydiving! August 18! Stay tuned!

Book I am reading: Crime and Punishment by Fyodor Dosteovsky. Considered one of the greatest novels of all time, it is a tough read at 540 pages. The dialog is detailed and intricate, requiring great concentration (one paragraph of one character’s speech is three pages long!). It takes dedication but is well worth it. A fascinating study on the actions we choose and their consequences. Four stars.

“Slicko” Movie on Health Care

You might feel like moving to Canada, UK, France or even Cuba after you watch “Sicko,” Michael Moore’s new entertainment documentary on the US health care system. With his characteristic folksy manner, light humor and up close personal profiles, Moore tackles the convoluted managed healthcare system, and manages to shock, shame and scare the audience…and that includes even those of us who know how this system works and who advocate better coverage.

I wondered if he would touch on pharmaceutical companies, PBMs and chronic disorders like hemophilia. Some things we already knew: Fifty million Americans uninsured (more like 44 million); the US spends a greater percentage of its GDP on health care than any other country (about 15%); the US ranks a dismal 37 in overall healthcare compared to other countries, while France and Canada rate in the top ten. Not a pretty picture. America’s for-profit health care sector is driven by the bottom-line, where medical directors of insurance companies receive annual bonuses for how much money they save the company–through denying claims of ill people. Contrast that to England, where doctors receive bonuses for the number of patients they convince to stop smoking.

Moore’s excursion to Cuba is a highlight of the movie, where he begs for medical care (via a megaphone from a boat) for suffering 9/11 rescue workers from the US naval base at Guantanamo Bay, where al-Qaeda prisoners get state-of-the-art, free care. He is scared off, and instead enters Havana Hospital where his entourage gets free care, treatment and new teeth. Clever and convincing.

This was a good movie, well done, but not his best. It’s myopic and selective. It’s very sad when you learn that 18-month-old Mychelle dies, but then that’s the end of the story. While Moore flits on to the next healthcare system victim, you are left wondering if the mother did not have a multimillion-dollar lawsuit to pursue, but the movie does not provide any details on what happens to those who were denied care with catastrophic consequences. And that makes you realize the movie does not discuss at all the role of litigation and malpractice suits. It also does not discuss the pharmaceutical industry and the role of biologics–which have caused our costs to rise, but are also what the consumer demands.

I found it baffling that Moore focused only on private insurance and the uninsured, yet makes no mention of Medicare, Medicaid, and other state and federal programs. Still, even without mentioning these, he has a strong case for riddling the US healthcare system with holes. Managed care appears a disaster and no one in the movie comes out looking worse than Kaiser Permanente.

Moore’s timing is perfect for the hemophilia community, which has battled managed care and insurance companies for years. The community has been fighting especially hard these last two years to keep factor in the formulary and to ensure choice of product and treatment regime. Moore could have a movie with our community alone.

In trying to take on the entire US healthcare system, Moore is able only to focus on a few cases, limited perspective and few solutions, other than universal coverage, like Canada or England. But the movie is worthwhile, thought provoking, and is “must see” for our community and anyone involved with the healthcare community. Thumbs up!

HemaBlog Archives