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.
 

Our Christmas Carol 2018

Jose Pepito of the Philippines

Merry Christmas! Happy Hanukkah! Happy Kwanzaa and to all, Happy Holidays!

In holidays past, we used to send out Christmas cards to everyone. We loved doing that and we love receiving them. As we have grown, and have expanded our humanitarian programs more internationally, we are seeing so many desperate needs. We decided instead to send a holiday e-card, with a story of someone in need we have helped. Instead of the usual $1,000 on cards, we are channeling this money and more into urgent needs at the holidays. We hope you understand and approve. We’re calling it our Christmas Carol! As you know, in Dickens’ A Christmas Carol, Scrooge by the end was doling out his money to help those in great need, especially Tiny Tim, who had a chronic disorder. And he discovered love and joy in the process.

 

Our “Christmas Carol” this year was helping Jose Pepito of the Philippines. Jose Pepito is 48, has hemophilia and inhibitors, five young children, and is single; his wife abandoned the family five years ago. His heartbreaking story is right from a Dickens novel.

He was orphaned early; his mother died while giving birth to him. His father died when he was nine.  After his siblings abused him, he 

left home at 16 and lived on the streets. He learned to drive a tricycle (called a tuktuk) at age 19, and used it to transport people. His ankles and knees took a beating and he endured many bleeds. He also survived an appendectomy, gallstone operation and a head injury! 

He now lives in a slum, as a squatter. It’s known as a drug haven, making it difficult to visit him. But Andrea Trinidad-Echavez, a woman with VWD and founder of Hemophilia Advocates-Philippines (HAP), dared to visit him, to photograph his conditions, and ask for help from us.

Jose Pepito suffered a psoas bleed—horribly painful—and pseudotumors. In November, the family’s tricycle – their main source of income – was taken by lenders after their father failed to pay monthly amortizations. Most importantly he needed an operation, which required the expensive and rare inhibitor drugs. HAP reached out to us, and we provided over $200,000 worth of medicine for his operation, thanks to inhibitor medicine donations from you all!

Andrea asked Jose Pepito what would be a good livelihood, since he is unable to use a tricycle now? He said a small store, as he lives in a colony. He can have a decent store with $1,000. “We will be providing his family with weekly grocery in the meantime,” Andrea said. “Right now, the kids are begging from their neighbors, just for them to eat!”

 We forwarded the $1,000 for food and necessities. And we will get him a grant from Save One Life for a store. Merry Christmas, happy holidays and God bless us all!

You can help someone like Jose Pepito too!     www.saveonelife.net 

 

“And how did little Tim behave?” asked Mrs. Cratchit, when she had rallied Bob on his credulity and Bob had hugged his daughter to his heart’s content.

“As good as gold,” said Bob, “and better. Somehow he gets thoughtful, sitting by himself so much, and thinks the strangest things you ever heard. He told me, coming home, that he hoped the people saw him in the church, because he was a cripple, and it might be pleasant to them to remember upon Christmas Day, who made lame beggars walk, and blind men see.” 
― Charles Dickens, A Christmas Carol

 

 

 

Out of Horror, Hope

A parent’s worst nightmare—their child is murdered. You might think that life itself ends, and a child becomes nothing more than brief memories. What difference can a child make in 5 years, except to his immediate family?

Imagine my shock when I opened the Boston Globe on a hot Monday morning in 1994, August 29th to be exact, and saw the blaring headlines that a little boy with hemophilia in Easthampton, Massachusetts was dead. I knew of this little boy. His family was on my mailing list; his grandmother Jenny had faithfully been sending me Kodak snapshots of him as he grew through his brief life. I pasted them in my hemophilia scrapbook.

According to police, Eric Dostie was in the care of his step-mother that Saturday, his biological father at work, when intruders broke in, tied her up, left their infant Emily in her crib unharmed, and smothered the boy with a pillow. There was no theft and no reason to kill a child. Police found no footprints, no forced sign of entry; neighbors saw or heard nothing. She was immediately a suspect, and a week later was arrested for murder. The case was strong when a neighbor came forward and said that the step-mother had once mentioned that she wanted more children, but Eric’s healthcare bills were too high. If only she could get rid of him.

Eric died the same year my youngest daughter was born, and the story shook me terribly. Not long after, I had lunch with a new business associate, Patrick M. Schmidt, president of FFF Enterprises of California. It was a meet and greet in Boston, having “chowda” at a seafood place. Patrick was just entering the world of hemophilia as a distributor of product, and wanted to know what he could do to give back to the community. His own daughter was the same age as Eric.

I thought of a lasting gift to the community, to keep Eric with us always. I proposed a scholarship in his name. When Patrick heard the story, it hit him hard that a little boy was murdered due to the cost of his healthcare—and at the hands of a mentally-ill step-mother. Patrick is an expert in healthcare and associated costs.

Almost 25 years later, the Eric Dostie Memorial Scholarship is still going strong, having helped hundreds of young people pay for college. We thought of a scholarship, because Eric once told his grandmother he wanted to be a scientist someday, and invent a cure, maybe a “chocolate pill,” that would avoid shots or cure hemophilia. He loved donuts, ducks and his family. He never got to meet his two half-brothers, both born after he died, but who both share the middle name Eric.

I was amazed to know that his half-brother Aaron, who I met several times as a child, is now on the committee to vet applications for the scholarship that bear’s his brother’s name. We recently became friends on Facebook.

It appears that a 5-year-old can have a lasting impact. Through the horror, there was hope. Thanks to Patrick M. Schmidt and NuFactor for sponsoring this scholarship, which turns 25 in 2019, and giving young people, and Eric’s parents, hope and permanence.

Applications are now being accepted for the 2019 Eric Dostie Memorial Scholarship.