Camp Yes I Can… and I Will!

Five years sure went by fast. That was the last time I attended the Dominican Republic hemophilia camp “Yo Sí Puedo!” (Yes, I Can!). I don’t understand how the time slipped by, especially when I have such a deep attachment to the DR, which is only a 4-hour plane ride away. The DR was the first developing country I ever worked with, back in 1998, when there was no camp, and no factor. While Project SHARE supplied factor when we could, together with the fledgling Fundación Apoyo al Hemoílico (FAHEM) we created the DR’s first hemophilia camp. We were all new at this, so the first camp was a bit wild… but magical. While the camp grounds were trashed by Hurricane Georges, no one much minded when Red Sox pitcher Pedro Martinez, Houston Astros Moises Alou and Toronto Blue Jay Tony Fernandez showed up to autograph baseballs and play ball with the kids!

Those children I first met at camp are grown now, and many are serving as camp counselors. In fact, the one who was the quietest at that first camp, Brahian Valdez, is now the most extroverted, and has become Camp Director!

It was a joyous reunion in the parking lot of the Robert Reid Cabral Hospital in Santo Domingo. The teens were men, the little boys were teens, and a new generation of youngsters with hemophilia were present, wondering who this “gringa” was.

And then it felt like no time had passed. This was my ninth camp, so I knew everyone pretty well, except for the smallest ones and of course the newcomers. One of the joys of camp is that you get to see children grow and hopefully flourish through the years. While we have lost some to bleeds through the years, the majority are here, and have become best friends.

The theme was “Super Heroes,” perfect for this group. I could easily see during the four days of camp in the Lomas Lindas area outside of Santo Domingo that the older guys hobbled about on stiffened ankles, arthritic knees, and misaligned hips. The younger ones had no joint problems and dashed about madly, enjoying the excitement of camp and the freedom from their parents. After working with developing countries for 20 years now, this amazed me. Children without joint problems. Here, in the Dominican Republic.

Camp is now a highly structured program that mixes plenty of activities with quiet time and lectures for the 60 campers with hemophilia. Each day starts around 7; by 7:30 everyone is on the grass doing exercises, led by Cuchito, the coach. Blowing his whistle, Cuchito has the children stretch, do light exercises and then march in ever tightening circles, which ends in a crush and everyone cheering. Breakfast follows, which is always delicious. The food in the DR is superb! Then activities: it might be swimming in the pool, arts and crafts, or a game of ball that serves as teambuilding.

The pool is the favorite unstructured activity. Though they live on an island in the Caribbean, most of the children never get to spend time in water. The surf near the beaches in the capital is too rough. And the children come from very humble homes, where there is no access to a pool. Camp gives them a chance to enjoy water, which almost every child loves.

Camp founder Haydée de Garcia, who has an adult son with hemophilia, realized this and arranged for top-notch, young swim instructors to visit, and they gave each child swim lessons which mostly consisted of overcoming a fear of putting your head under water. Once that fear was conquered, the children enjoyed practicing floating and strokes.

The favorite activity, judging by the cheers and participation level, is wiffle ball. You must know that baseball is almost a religion in the DR! The DR sends more ballplayers to the US than any their country, and about 25% of all ball players in the leagues are from the DR. While we don’t use baseballs or softballs for safety reasons at camp, the kids and adults attack the wiffle ball as if it were! The games are exciting, loud, fun, funny and tiring!

This year we even had a bonfire just outside the camp facility, where we roasted marshmallows and everyone participated in some singalongs that ranged from traditional Spanish songs to rap!

A great presentation was made by Fendi, who was at the original camp in 1999. Wearing a Spider-Man mask, he talked about how the boys may have developed hemophilia: inherited, like WonderWoman; a mutation, like Wolverine; or acquired, like Storm. Very clever! And he stressed the need for community, like the X-Men, and proposed a program called Hermanos X (Brothers X), to keep this community strong.

The beauty of camp is abundant factor. Prior to about 2016 camp might be touch and go; you cannot have a camp if there is no factor, and the DR has struggled to get factor. SHARE always tries to assist and we stockpile factor starting in January for camp. A medical ward is set up before camp ever begins where everyone gets factor. This year? Everyone got factor prophylactically. How does that happen?

Well, we had our own superhero at camp this year. Bioverativ CEO John Cox visited the camp with his wife Ivette (who is Dominican) and his four children, to experience hemophilia camp in a developing country, and to see the results of his visionary idea (along with other visionaries; read about it here) to donate one billion IU of factor to the World Federation of Hemophilia (WFH) over 10 years. With this strategy, knowing there will be a steady flow of factor that doctors can count on, hundreds of children around the world in developing counties are now on prophy for the first time ever. These include “my” boys, here in the DR, here in camp. As I watched the older boys still hobble about, I watched the younger ones race and jump and climb, without fear, without arthropathy. It seems like a miracle, but it is in reality this vision come true—that with proper treatment, children with hemophilia can live full and active lives, in our countries and theirs.

The World Federation of Hemophilia, at Bioverativ’s request, donated all the factor to camp this year. No child had to worry about running out, which allowed them to run about!

And for the first time, WFH attended camp. Miquel Izquierdo, WFH regional director, arrived from Mexico to enjoy the camp—he himself has hemophilia! He fit right in and was beloved by all. He witnessed the great results of the factor donation, and also of Save One Life as about 16 enrolled beneficiaries attended camp, all looking great and doing well!

See photo gallery here.

We all loved having outside visitors, and next year, for our 20th anniversary, I am hoping to entice more volunteers from the hemophilia community to attend, to see the needs, the progress, to make connections. Despite the overwhelming success of camp, we can do more. Yes, we can… and we will!

From John Cox:

“I want to thank you and Presidenta Haydée Benoit de Garcia for including my family in hemophilia camp in the Dominican Republic.  We were so impressed with the camp and honored to be there. The Dominican boys were learning to deal with hemophilia in a poor country, and they were having fun too (the lively whiffle ball game was our favorite activity).  It was clear that this camp was something these boys looked forward to all year.  What struck me most was that Haydée and her counselors were teaching the boys to live a values-driven life no matter their circumstances.  Haydee’s leadership and her love for these boys — as well as yours, Laurie — is clearly impacting generations of Dominicans living with hemophilia. My children walked away believing in the camp theme “Yes You Can!” (¡Yo Sí Puedo!). Thank you for all you do, Laurie, and for the role of Save One Life in impacting lives in the Dominican Republic and around the world.”

Thanks to John Cox and family for spending family and vacation time with us, to the WFH and Bioverativ for the factor donation, to uniQure for gifts for the children, to Haydée and team, for another great camp!

See more photos here.

Amusement Parks and Summer Safety

This article was originally published in PEN, August 2002, Vol. 12, issue 3

Summer is in full force and with it, trips to theme and amusement parks. I’m a huge lover of them, including the thrill rides, like roller coasters. In 2000-2002 there were many stories and studies in the news about the potential for brain bleeds, and these were about people without bleeding disorders. Last year came the first reported case of symptomatic bilateral subdural hematoma associated with riding a centrifugal motion simulator ride. 

What does this mean for our kids (and big kids–adults!) who love to ride roller coasters and simulator rides? Have you asked your hematologist for his or her opinion? I decided to rerun one of my favorite articles from PEN 2002 about risks for kids with hemophilia at theme parks, written by Dr. Richard Lipton.

Summer is here, and millions of families will seek adventure and thrills at theme parks like Six Flags, Disney World or Universal Studios. What a wonderful day a family can have at a theme park! Kids and parents love the rides, water slides, entertainment and general excitement. Are there any special safety concerns or precautions for families with a child with hemophilia? Yes!

Think of a theme park as a very big playground, but with an atmosphere favoring less parental control—a setting that can lead to impulsive behavior by children.

Imagine yourself at a typical theme park. You’ll have general health concerns. Parks can be crowded, hot and sunny—so apply sunblock and drink plenty of water.

Theme parks have paved surfaces, harder than public playgrounds, and filled with children running. Your child needs to wear appropriate footwear. Flip-flops or Tevas might be suitable for water activities, but sneakers are safer for walking and running.

You’ll also have concerns specific to the theme park you visit. “Mind Eraser,” “Shockwave,” and “Nitro”—what about these special high-end rollercoaster rides? Riders are frequently subjected to changing speeds that result in “G-forces” similar to those experienced by trained, appropriately suited and restrained combat fighter pilots. Your child becomes “Top Gun” in shorts and a T-shirt! Parents should remember that any person can experience head trauma on these specialized rides.

How is the head affected by a ride like the Mind Eraser? Recall that your brain is surrounded by fluid; it is floating inside your skull. This arrangement cushions the brain, and reduces movement, protecting your brain from direct trauma and sudden shifts in skull position. It works quite well in our daily activities, and in automobiles (as long as we’re wearing a seatbeat). Now imagine speeding over the crest in a roller coaster. All of a sudden you’re weight- less, like an astronaut—this is called a Negative G- force. (You’ll have no trouble recognizing this moment, because every- body screams!) Then, after the coaster speeds down and resumes its climb, you feel your backside being pushed into the seat. It feels like the force of gravity has suddenly increased. This is a Positive G- force. Although your body is restrained, high G-forces could exceed the protective cushioning of fluid surrounding the brain, and could cause injury.

Interestingly, this year New Jersey became the first state to seek legal restrictions on the maximum allowable G-forces on amusement park rides. The regulations result from concerns raised by physicians about the association between neurologic damage and high G-forces on these rides. Certainly, such injuries occur very infrequently, but serve as a cautionary note to all riders of high-end roller coasters—with hemophilia, or without.

My advice? Take some precautions. Level the playing field by giving your child a prophylactic infusion of factor the morning of your visit to a theme park. Yesterday’s dose is not sufficient! Don’t wait until your child reports the symptoms of a bleed—it may be too late. Besides, your child is not going to report the flop he took running to the haunted house until you’re stuck in traffic on the long, long ride home.

Infuse first, then have a great time!

Dr. Richard Lipton was the physician in charge of the hemophilia treatment center at the Long Island Jewish Medical Center. As a United States Air Force Physician (1966–1968), Dr. Lipton knew several fighter pilots, who took him on “joy rides” (with lots of G-forces) that more than fulfilled his childhood fantasies. He is now retired.

Welcome to Massiechusetts!

The US hemophilia community is renowned for being politically active: we’re educated, smart, proactive and dedicated to protecting our need for access to affordable and safe factor concentrate, and medical help from HTCs. No doubt our political savvy was forged in the hellfire of the hemophilia holocaust of the 1980s and 90s, where we fought from the outside, securing justice from government to ensure this never happens again.

But it’s rare that the hemophilia community makes change from inside the government, as members of government.

Currently we have two people that I know and know of, running for offices. 

One is long-time colleague Randi Clites, mother of a child with hemophilia, who is running to be a representative in the Ohio House of Representatives. Randi is currently associate director at the Northern Ohio Hemophilia Foundation, and someone I’ve met at many hemophilia meetings. She is passionate about health care, dedicated, and a true advocate. On June 15 she posted: “It’s official. I am grateful to have earned the Democratic nomination to run in November to become the next State Representative for the 75th House District.” She has a beautiful website that covers her campaign and asks for donations.

I love this post of hers on Facebook from two days ago: “Yesterday I was out door knocking and I stopped at Kent Dairy Queen for a break while it rained. A young man there asked why he should vote for Randi, because I was wearing my campaign shirt. I introduced myself and he had some of the best questions I’ve been asked while canvassing. I am so inspired by our young people. They are paying attention and engaging. My parents didn’t talk to me about politics. I think we need to. Please take a few moments today and talk with your youth and young adult children about their thoughts on politics.”

Canvassing is hard work, as is campaigning. I’ve been trying to help the second person in hemophilia I know, Bob Massie, in his bid to become Massachusetts’ next governor. You may know Bob; he is quite a legend in our circles, though he no longer has hemophilia. Bob was profiled in the movie “Bad Blood.” Bob was also the subject of the 1973 book Journey, written by his parents, Pulitzer Prize winner Robert K. Massie and then wife Suzanne. The book is a gripping true story of Bobby’s life, which chronicled his immense suffering as a child, and showed the origins of his political activism. His parents were politically oriented, especially his mother, who, with her knowledge of Russia, became President Ronald Reagan’s advisor on Russia! His parents also helped pioneer home treatment of hemophilia. His parents are good friends of Al Gore (and the Clintons), and Bob himself has adopted climate issues as a personal and political cause, as well as health care and racial justice. Bob has had long and rewarding careers in many fields, as an executive leader, Episcopal priest, and author. He knows what it is like to suffer, and to worry about paying for health care costs. He has a vision for how our state, and world, can be better.

Massachusetts (or “Massiechusetts” as Bob’s campaign cheekily calls it) is one of our nation’s smallest states, but a leader among states. It is home to the birthplace of our country. It is home to some of the world’s best universities and colleges. It is a medical Mecca for research and hospitals; center of the biotech universe; and has a rich history and culture. It is a multicultural state as we attract so many immigrants, especially from the Caribbean.

But like all of America, we face tough times ahead and need strong leadership. 

It’s heartening to know that people like Bob and Randi are sacrificing so much personally to try to represent us in government. On Thursday night I held a fundraising get-together at my home, where members of our community and my neighborhood were able to meet Bob personally, and hear his platform on health care, transportation, and economic reform. Bob had just been in Springfield the night before, a two-hour commute, and had actually just met my mom!

I’ve known Bob for 30 years, which is a story for another time. (Meet Bob here) But that night, someone asked him where did he get the energy, at age 60, with all that he has been through, to keep up this pace, to sacrifice so much, to meet and greet the thousands of people has this past year. “I think it’s because I missed so much as a child, and was in bed so much, that I just love people and getting out to meet them!” he replied. I’ve heard other guys his age say much the same thing.

We are all so grateful to people like Randi and Bob, for their dedication and sacrifices, to hopefully bring some compassion and leadership back into government, and to especially keep in mind those with chronic disorders like hemophilia, who need insiders in government, more than ever. Good luck to both!

To donate to Bob, visit:    https://secure.actblue.com/donate/bobmassieforgovernor2018
To donate to Randi, visit: https://secure.actblue.com/donate/randi

Why Plasma Matters

I attended the Plasma Protein Therapeutic Association (PPTA) Forum in Washington DC this past week, which is always a stimulating gathering of bright people in the plasma industry. The theme of the meeting was the launch of the campaign, “How is Your Day?” Every session started with this friendly question, which had the effect of the audience responding back, “Great!” 

The meeting was a call for global access and availability of treatment, and the need for policies to improve patient care. Jan Bult, president and chair of the PPTA, reminded us that “plasma derived products can only be made if we have donors.”

While much of the US uses recombinant factor products, plasma products still play a significant role for many people with hemophilia, particularly those undergoing treatment for inhibitors, older patients who grew up on plasma-products and are reluctant to switch, many patients with von Willebrand disease, and above all, those in developing countries, where recombinant products are too expensive.

Jan reminded us that the goal and need is high quality source plasma from healthy, compensated donors.

HowIsYourDay.org

David Bell, executive vice-president of Grifols and chair of the PPTA global board of directors. commented in his opening remarks that derogatory comments have been made about the plasma industry. Yet there has been no viral transmission for more than two decades. PPTA seems constructive dialog with patient organization and policy makers.  He praised Jan Bult for his long-term efforts leading the PPTA and always trying to push for improvements in treatment.

The keynote speaker was someone who left me amazed and inspired. Anthony J. Castaldo is president of the US Hereditary Angioedema Association. Hereditary angioedema (HAE) causes unexpected horrific swelling and excruciating pain. Tony is a patient himself.  It is more rare than hemophilia, and affects one in 30,000. Untreated, the mortality rate is a stunning 30%! It’s a pediatric disease. Tony made it clear that this is not allergic angioedema—swelling in the throat, for example, due to a food or drug allergy. But HAE is often misdiagnosed in ER, where the patient will be given antihistamines— but these don’t work. 

I had never heard of HAE, and clearly there were many parallels to hemophilia as a chronic disorder and the need for advocacy. It was interesting to see that factor XII is involved in the disorder. Tony showed photos of patients, before and after an attack, which were devastating! Previous to current plasma-derived therapy, patients were given toxic anabolic steroids. Tony showed a video sharing patients’ stories, and I felt the way many people unfamiliar with hemophilia must feel when they see our videos. You want to cry for the people who suffer so much!

One woman in the video commented on the excruciating pain, of almost dying… a laryngeal attack that closed her throat was one of worst. She said, you’ll never know when you’ll have an attack, how far it will go, and if next attack is your last one. She suffered high anxiety in between attacks. In a poll, 100% of patients felt it prevented them from attending school and work. Each year they had between 20-100 days of incapacitation.

Tony’s own 5-year-old daughter started having attacks, including weekly abdominal attacks, which are torture. His daughter grew emaciated, depressed. When she then got the C1-inhibitor plasma-derived medicine, he watched in two weeks as his daughter transformed. Tony spoke with passion and commitment, reminding me of so many in our own community!

He closed by saying, “Plasma products are life-saving and life giving. We appreciate what industry does, what donors do— we get it; we love it! We as a patient community want to protect the gains we’ve gotten.”

So do we in the hemophilia community. Plasma-derived products still have their place in our community. If you can, donate blood. It’s the basis for plasma-derived products… and all of life.

Are you enrolled in the Patient Notification System? If not, you should be. PNS is an alert system to notify you when or if your factor product has been withdrawn or recalled from the market. This is an essential program, developed by manufacturers and operated by the Plasma Protein Therapeutic Association (PPTA).

A Facebook Group that Works!

Milena started her talk by reading a post by one mother, about how devastated she felt at the diagnosis of hemophilia, and how much Hemophilia Mother had helped her–more on that letter in a bit.

Milena started Hemophilia Mother in 2011, when her son was 20. Her son with hemophilia–there’s the connection! Of the 6,328 members, 4,526 are “active,”  members.

Mileana added: 3,158 members are from the US.  531 are from India. 69.5% of members are women, and 30.5% men! These include fathers of children with hemophilia and men with hemophilia.

Top cities where members live are: Dhaka, Bangladesh 62; New York 50; LA 42; London 42; Chicago 39; Karachi 29.

More interesting than all the stats is Milena herself. Obviously not from Canada, with a foreign accent. She is a doctor, from Sarajevo, capital of Bosnia and Herzegovina. She survived the siege in 1992, which she recounts online:
“Some 10,000 civilians, including 1,500 children, were killed in Sarajevo during the 44-month-long siege, mostly by snipers and mortars fired from mountains surrounding the city. UNICEF reported that of the estimated 65,000 to 80,000 children in the city, at least 40 per cent had been directly shot at by snipers; 51 per cent had seen someone killed; 39 per cent had seen one or more family members killed; 19 per cent had witnessed a massacre; 48 per cent had their home occupied by someone else; 73 per cent had their home attacked or shelled; and 89 per cent had lived in underground shelters. We lived in a besieged city for 31 months with a toddler, without food, electricity and under constant shelling! And we were somehow spared? Survivor guilt mixed with hemophilia mom guilt stayed tangled in my brain for many years to haunt me more than I would ever want to admit.”

Milena emigrated to Canada and settled in Manitoba. Her son was diagnosed with hemophilia at age 6. She was very much alone, with trauma embedded in her memories. It was a difficult time. 

“All our struggles to survive the Bosnian war during the siege in Sarajevo, immigration and my recent diagnosis of a chronic condition were nothing compared to the feeling of loss after hearing our son’s diagnosis. Was it because we were both doctors unaware that he had an underlying condition, or the fact that I didn’t know I was a carrier, or that we were alone in that suburban apartment far away from our family and friends that day? I don’t know and I will never know but it hit us harder and deeper than my understanding goes. I went to my son’s room, I hugged him, I squished him tighter and longer than he wanted. He looked at me with his innocent smiling eyes. He had just turned six.”

Hemophilia Mother was born. And here she was today, sharing her story and her Group with a rapt audience. She closed her talk by finishing the letter from the mom; the mom at one point was seriously considering to take her own life, so severe was her depression. But the support she received from others in the group made her want to live again; the community members gave her hope.

Coming home, reading my notes from that day, I had jotted down what the CEO of Sobi, Guido Gelkers, said when speaking about the 1 billion IU factor donation made by Biovertiv/Sobi to the WFH, referring to those who live in developing countries: “When you sit in your office, you forget what others’ lives are like.”

Hemophilia Mother knows what the lives are like of anyone in any country struggling with hemophilia. Members live it every day. Join it: give advice, get advice.     And be supported and inspired. Thank you, Milena, for your service and your inspiration!