Hemophilia: The B Sci-Fi Movie

I love sci-fi as a movie genre, and am a big fan of the 1950s classics (War of the Worlds, The Amazing Colossal Man, The Day the Earth Stood Still), and even the silly B-movies (Plan 9 From Outer Space), right up to 2001: A Space OdysseyAlien (all time greatest) and hey, even Prometheus. I never knew hemophilia was ever mentioned in sci-fi but it has been!

Our esteemed writer Richard Atwood is a sleuth at finding obscure references to hemophilia. And he found one in an old sci-fi film–with some prominent cast members.

The film is the 1966 Planet of Blood (a.k.a. Queen of Blood) and is about a space-vampire who is a queen on her planet. But her spaceship is in trouble; so Dr. Farrady (starring Basil Rathbone) at the International Institute of Space Technology sends two rescue teams of American astronauts (played by Dennis Hopper, Judi Meredith, and John Saxon) travel from the Moon to Mars in 1990 to search for unidentified aliens in distress.

The astronauts locate only one mute, green-skinned, female alien (Florence Marly) in a red-bodysuit, with platinum blood hair, picked up from the Martian satellite Phobos. The alien must live on human plasma, which leads to the deaths of two astronauts who were hypnotized first, while the crew return to Earth. She has a secret plot to overrun the Earth by laying as many eggs as possible on the rescue ship that recovers her from her crashed interstellar spaceship.

Disdaining human food, however, the alien queen needs human blood to survive. She hypnotizes male crew members, one by one, and sucks them dry. Ordered not to kill her, the surviving astronauts fear for their lives from the killer in their midst. Spoiler alert!! They accidentally end her life by cutting her skin, and she bleeds to death. Her blood is green.

The crew believe that she is what humans would have become if we had evolved on another planet–hemophiliacs.  This female vampire is labeled a hemophiliac, by the crew, and some sort of royalty. She’s dead, but like a queen bee, she leaves behind her red eggs, with “consequences left to the imagination,” Richard writes.  

Interesting take on interstellar aliens, vampires, and hemophilia!  It’s now a cult movie, and you can order it on Amazon.   

Why You’re Afraid to Switch Products

Many consider the new year to be a great time to make life changes. With new factor products in the marketplace we thought this article on the pros and cons of switching products would be helpful to our readers. Enjoy! 

by Cazandra Campos-MacDonald
originally published in PEN, November 2018 

 A few myths about hemophilia linger in the community: “Only men can have hemophilia.” “A person with hemophilia will bleed faster than someone without hemophilia.” “People with hemophilia can’t play sports.” “You will outgrow hemophilia.” Belief in these myths can spread fear, and can lead to harmful biases or behaviors: for example, prohibiting a child from playing sports, or not believing women who claim to have bleeding issues.

One fear that is still felt by many people with hemophilia, particularly those who’ve had an inhibitor, is the development or recurrence of an inhibitor as a result of switching factor products. Though there is some evidence to support this,1 we also know that the risk of developing an inhibitor as a result of switching products is very small—too small to accurately measure. Yet many parents give this risk more weight than it deserves. Fear clouds their judgment when weighing the benefits of finding a better product and treatment regimen with the risk of contracting an inhibitor.

The fear of switching products became ingrained in my mind when my oldest son, Julian, was diagnosed in 1996 with a low-titer inhibitor at age one. I learned to infuse Julian with a first-generation factor VIII product (from which he developed the inhibitor), and after two and a half years of daily infusions, his inhibitor tolerized. My husband and I kept Julian on his treatment regimen while also gathering information about new factor products entering the marketplace. First-generation recombinant factor products reconstituted into 10 cc volumes, while some second-generation products reconstituted into only 2.5 cc. Yet our fear of triggering an inhibitor by switching products outweighed the promise of quicker infusions.

My second son, Caeleb, developed a high-titer inhibitor at age 11 months. He eventually began immune tolerance therapy (ITT) on a recombinant factor product, and after a couple of years of daily infusions, we moved him to a plasma-derived factor, hoping to lower his inhibitor level. Given the many complications Caeleb endured over the years, I feared that switching to a different factor product would cause his titer to spike. And daily infusions meant accessing his port every morning and pushing 30 cc of product from four vials of factor concentrate. This was not conducive to self-infusing, although he stopped experiencing episodic bleeds. Not having bleeds regularly was a great trade-off.

Whenever Caeleb visited the hemophilia treatment center (HTC) to have labs drawn, I brought along an extra-large plastic bag of his factor and supplies. Recently, Caeleb’s hematologist, Dr. Shirley Abraham, suggested, “I think it’s time to switch Caeleb to Hemlibra.”2 I was stunned. Another switch? I honestly couldn’t understand why Caeleb needed to switch. Even though Hemlibra® is not a factor product, I realized that the fear of switching was alive and well in my mind. Caeleb still had an inhibitor, and not knowing how a new treatment would affect him was nerve-wracking. Dr. Abraham pointed to the extra-large bag and said, “That is why it’s time to switch.” Over the years, I had grown accustomed to carrying all those supplies for an infusion, and to accessing his port daily. With Hemlibra, a month of product and supplies fit into a small container, and Caeleb receives one subcutaneous injection weekly into his thigh—no more infusions into his port. In that moment, speaking to Dr. Abraham, I understood. Caeleb’s quality of life could be even better if I trusted medical advice and didn’t give in to old fears.

Products are chosen—and kept—for many reasons. For example, in families where more than one person has hemophilia, the choice may be based on another family member’s experience. And if a treatment regimen or product is working well, change may not be needed. But what if you can do better? A common response to changing products came from one mother on social media. “Same manufacturer for 18 years because [the product] works. No reason to change.” Claudia Mackaron, a retired HTC nurse coordinator in Albuquerque, New Mexico, says, “Even though a new product could be more beneficial, the old saying ‘if it ain’t broke, don’t fix it’ creeps in. It’s very frustrating as a clinician to fight with patients in switching, knowing [a new product] could help them.” Fear of the unknown may stop a patient from changing products even if the data shows that a specific product could be a better fit. “Psychologically, the fear of the unknown, and a potentially harmful and difficult unknown, can shape bias tremendously,” says Dr. Mike Wang, associate professor of pediatrics at University of Colorado’s School of Medicine. Inhibitors are frightening, and it’s possible someone could develop an inhibitor after switching products. The fear lives on, even with no clinical evidence that the inhibitor resulted from a new product.

Why do some people embrace change without fear? Debbie Porter has an adult son with hemophilia and inhibitors who always “thought the idea of staying with the same product forever was counterproductive to advancing new and better treatments.” Debbie wanted more for her son Matt, who suffered for years from inhibitor complications. Matt infused recombinant products, plasma-derived products, and bypassing agents over the years, so switching wasn’t a fear. The day Hemlibra became available, Debbie immediately requested it for Matt. He has been bleed-free for seven months now, and his veins get the rest they desperately need. And of course, because Hemlibra isn’t factor, it can’t cause an inhibitor to factor VIII. Yet some people may be reluctant to switch, because they experience few complications with their inhibitors and their current treatment works. But for people like Matt and Caeleb, the severity of complications pushes them to anxiously wait for new products that promise better results.

 We are learning more about why inhibitors form, and who is most likely to develop one. Physicians can identify patients who are more susceptible to inhibitor development based on genetics, environmental factors, race, and family history. But people seemingly not at high risk may still develop an inhibitor, and this has continued to feed fears about treatments and products. One HTC provider admitted that for years, he and his colleagues encouraged patients without inhibitors not to switch factor brands because they might get an inhibitor. While there is a risk of inhibitor development when switching products, “current evidence does not suggest that switching products significantly influences inhibitor development,” declared a finding in the Eleventh Zürich Haemophilia Forum.3 Yet fears persist, even in the face of scientific evidence.

Letting go of old beliefs isn’t easy. Once a myth has taken root in your belief system, it takes a lot of effort to remove it. We remain captive to treatments, protocols, and products that may not be the most effective. Staying where we are now—based on assumptions not rooted in the facts—prevents us from embracing the many possibilities that exist today.

Have the courage to overcome fear and seek out what’s in the best interest of your loved ones. Gather the facts, and communicate your treatment needs and concerns to your physician. Trying a different regimen or product may change your life.

 

  1. F. R. Rosendaal, et al., “A Sudden Increase in Factor VIII Inhibitor Development in Multitransfused
Hemophilia A Patients in the Netherlands: Dutch Hemophilia Study Group,” Blood 81, no. 8 (1993): 2180–86, available at www.bloodjournal.org. Thierry Calvez, et al., “Recombinant Factor VIII Products and Inhibitor Development in Previously Untreated Boys with Severe Hemophilia A,” Blood 124, no. 23 (2014): 3398-3408, available at www.bloodjournal.org.
  1. For more on Hemlibra, see Paul Clement, “ACE910: The First Disruptor,” PEN, February 2018, 4.
  2. Elena Santagostino, et. al., “Switching Treatments in Haemophilia: Is There a Risk of Inhibitor Development?” European Journal of Haematology 94 (2014): 284–89.
 

Hemophilia and Astrology

Laurie Kelley’s zodiac sign

What does hemophilia have to do with the stars… as in astrology? Are people with hemophilia born under the Virgo sign more organized and liable to use the sterile technique? Are Aries more apt to be our advocates? Are Leos the performers and speakers at our conventions? Are those apt to be more scientifically-oriented born under Scorpio?

Our contributing writer Richard Atwood found a reference to hemophilia and astrology. A medical dictionary for astrology, to be precise.  He writes, “According to the author, this medical astrology dictionary is “geared to the layperson who has limited knowledge of medicine.”  The dictionary uses “the most commonly agreed-upon medical astrology definitions.” The author also acknowledges that there is a lack of agreement among astrologers “as to what rules what in the body.” 

Hemophilia is defined as “malefics in second-eighth axis, Mars Saturn stress aspect, Jupiter afflictions, afflictions to Sun, Moon or Ascendant.” 

Definitions are also provided for possibly related terms, such as bleeding, blood, blood clots, blood disease, inherited blood disease, blood loss, blood plasma, blood serum, blood transfusion, arterial blood, venous blood, cerebral hemorrhage, coagulate, fibrin, hemorrhaging, hereditary disease, joint inflammation, limping, nose bleeds, plasma, plasma balance, red blood cells, white blood cells, styptic, and wounds.

In the section on Uranian Astrology, the definition for blood disorders is “Neptune = Sun/Hades; Sun + Hades – Mars = Neptune.”  A bit hard core for my tastes!

Richard notes that the dictionary has 89 pages for the medical astrology definitions, plus 5 pages for Uranian Astrology definitions. The book includes a 3-page bibliography.  The only bleeding disorder to be listed is hemophilia; all other bleeding disorders are omitted. As for the planets, Mars rules blood ailments, Jupiter rules arterial blood circulation, Saturn rules chronic disease, andVenus rules venous circulation. The author, who has the highest certification of the National Council of Geocosmic Research with a CA NCGR Level IV, specializes in medical astrology and nutrition in New York City.

Well, I am a true-blue Scorpio, and Mars rules my sign, so that works for me. How do your zodiac sign characteristics fit your bleeding disorder? 

 

Diane L. Cramer, 2003, Dictionary of Medical Astrology: A Compilation of Astrological Terms (Physical, Emotional and Mental) and Disease Significators Used in Traditional Astrology. Tempe, AZ: American Federation of Astrologers. 98 pages.

 

 

Bombardier Blood: Incredible Journey with a Cast of Characters

Coincidences are God’s way of staying anonymous. Albert Einstein 

Yesterday I was in Denver, Colorado, to attend the hometown premiere of Bombardier Blood, the new documentary that chronicles the life and achievement of Chris Bombardier, a person with hemophilia B this year who became the first person with hemophilia to complete the Seven Summits. The movie was debuted at NHF’s annual meeting in Orlando, in October, to an audience of over 500.  Yesterday, the Colorado Hemophilia Society rented an IMAX theater, and invited the Colorado bleeding disorder community to attend.

Chris and Jess Bombardier with Laurie Kelley and Amy Board Photo: Rob Bradford

What a proud day for Colorado! Chris and his wife Jess attended, flying in as I did from Boston, which recently became their new home. Chris’s whole family came: parents Alan and Cathy, Aunt Bev and Uncle Jay Labe and cousin Nicole, and of course, “Crazy” Uncle Dave. And while Chris’s whole family is incredibly warm, down-to-earth and personable, I really wanted to meet this character Uncle Dave! When you see the movie—and you will someday—you will know what I mean!

Chris’s poster is right next to Bumblebee’s and Aquaman’s!

We congratulated the astounding film-making and editing team of Rob Bradford and Steven Sander, who were both present, and thanked expedition and film sponsor Octapharma. The theater filled with community members—moms, dads, small children, and many of Chris’s peers with hemophilia—holding popcorn, soda and high expectations. We settled into our seats before the mammoth screen, hushed our voices, silenced our cell phones, and the theater darkened. The darkness faded into a scene of Everest, prayer flags, snow and ice, and Chris, trying to skewer a vein in the icy air. The film continued from this moment at Everest, into a 90-minute tale of what it took to get him there. It is a fascinating story of a boy with hemophilia who dreamed of being a baseball player, who was thwarted in his dreams, who suffered depression, anger, who was disconnected from his own local community… until key events unfolded in sequence, “like divine intervention,” one audience member confided. The events included the right people appearing in his life at the right time, until his destiny seemed all but spelled out. This young man would conquer Everest.

Chris’s proud parents, Cathy and Alan

I took part in Chris’s journey at some point, and have seen this movie three times now. It never fails to inspire. You want to jump out of your seat and get moving, to find your own dream, and then make it happen. If Chris could climb the Seven Summits (a feat only about 450 humans ever in history have done!), and did it with hemophilia, then what can the rest of us accomplish? We are only limited, it seems, by our own imagination, and our belief in ourselves.

The audience loves it! Photo: Rob Bradford

But some new footage was added to this version of the film—the role played by the vivacious and energetic Amy Board, executive director of the Colorado Hemophilia Society. Amy was the person who drew Chris out from the crowd. She encouraged him to come to hemophilia camp, where Chris met someone else with hemophilia for the first time. He saw how the children were active, happy, connected. And Chris became connected. He volunteered at camp, became a mentor to the children. Due to Amy, Chris became part of the hemophilia community. It was the first major step on the long hike to better mental health, a career, a new vision… and ultimately Everest.

Laurie Kelley meets Crazy Uncle Dave!

Chris did not just scale the Seven Summits or make a movie about himself; he has promoted hemophilia worldwide with his life-risking achievement, and he has made a call for action for others, to help those in developing countries through Save One Life. Without Amy, without camp, he might not have joined the community. Without working at the Indiana HTC as a lab tech, he might never have gone to Africa, where his eyes were opened to the suffering of his blood brothers overseas. Without Uncle Dave? Who knows. Bombardier Blood boasts a cast of supporting characters who embody this mystical truth: We never know what ripples we create when we reach out, take a risk, and care about another human being.

Chris did it. He made history. First person with hemophilia to scale Everest, and to bag the Seven Summits. And I suspect that Chris’s story is far from over, but is really just beginning.

Chris with photographer Rob Bradford
Laurie with Octapharma reps Paul Wilk and Elizabeth Pulley
Laurie Kelley with photographer Rob Bradford and film editor Steven Sander

         

 

 

 

 

 

China: Aggressive Growth, but Hemophilia Lags

There’s no describing China adequately: it’s massive, it’s spectacular, it’s impressive, it’s ancient. I visited on holiday from November 1 to November 17 as part of a Viking Cruise, part of which took us all down the Yangtze River, to view the Three Gorges and the incredible Three Gorges Dam, the world’s largest power station. And everywhere we looked there is construction. Gigantic tower cranes dominate the skyline in all the cities we visited, like mechanical birds dipping down into a field of high-rise apartments that jut up and on forever like a field of metal grass. I’ve seen some things in my travels to over 3o developing countries, but nothing like this. As we all gazed at the endless rows of enormous apartments, stacked like a line of dominos, I felt like we were in a Blade Runner sequel. Each city stunned us more than the previous one.

Laurie Kelley at the Great Wall

China recently surpassed Japan as the world’s second largest economy. You can see economic activity everywhere; it is a country on the move. And yet, I found myself wondering about medical care for those with hemophilia. How does China care for its patients?

Because it was a vacation, after all, I didn’t research anything about hemophilia. But I did get a chance to visit a school, supported by Viking Cruises. It is beautiful and the children treated us to a lovely performance of acrobats and dancing with fans. When I asked our guide if there were any children with disabilities at the school, he quickly said no. Might there be some children allowed sometime in the near future? Not likely.

City of Xian

And despite all the construction, there are no handicapped facilities. People with handicaps are seen much as a burden, in other words, like in any developing country. I do know that due to the one child rule (recently changed to allow two children per family), parents who give birth to a child with hemophilia may be tempted to drop the child off at an orphanage. We’ve seen in the last 20 years many children coming from China with hemophilia, now being adopted into American homes. 

While I was in Shanghai, for the final days of the vacation, I contacted an old friend and colleague, Delin Kong. Delin contacted me back in the 1990s as he wanted to start China’s first patient organization. At the time, my company provided leadership training and supported patients like him in developing countries. We assisted, gave China its first funding for a hemophilia patient organization, and Delin and his team did the rest. We provided factor donations up until recently.

It turned out Delin lives only a few blocks from the Westin, so he visited on our final day. How good it was to see him after so many years! However, I noted his severely crippled joints. He had great difficulty walking. Luckily, he gets government assistance, and used to be a financial trader and did quite well for himself! But he is in pain.

Laurie Kelley with children at Viking-supported school

I asked about hemophilia and was surprised to know that patients in China still do not have home therapy. For every bleed, they must go to the hospital, according to Delin. This is always a major problem in developing countries. No matter how much factor gets donated, patients will suffer long term if they cannot infuse as needed at home. 

And a knee replacement? Delin is on a waiting list for 6 months or more. He has asked repeatedly, but is put off by the medical team. From not enough factor to not enough medical help, Delin, despite China’s economic   standing in the world, still suffers like others in worst off countries. My hope is that combined with patient advocacy, the national or provincial government may at long last meet the needs of its people with hemophilia. 

Visiting China opened my eyes to its beauty, history and aggressive growth, but medical care still lags, at least for those with hemophilia.