It’s the holiday season, and a time to watch all our holiday specials: White Christmas, It’s a Wonderful Life, Rudolph, The Little Drummer Boy, The Grinch and of course, perhaps the best of all, A Christmas Carol.
The other day I was watching Charles Dicken’s A Christmas Carol in the 1970 adaptation with Albert Finney as Scrooge, one of my favorite adaptions. And watching Tiny Tim struggle on his crutch, it got me thinking: just what disorder or disease did Tiny Tim have? He limped, he was in pain, his joint was crippled, and in the vision of the Ghost of Christmas Future, he passed away.
I wondered: could Tiny Tim have had hemophilia?
It’s not so far-fetched. Living in 1859, Tiny Tim inhabited London when Queen Victoria’s son Leopold did (born in 1853), and he had hemophilia. But his condition was never really described in full. We know he could not walk on his own during the story, and needed a crutch. He didn’t appear to have a cough (denoting tuberculosis), or any other pain. One website suggested rickets or cerebral palsy, but the story doesn’t seem too really support that. Everyone overlooks hemophilia, because… well, it’s so rare!
I’m going to imagine he did have it; and thanks to Scrooge’s transformation to a benefactor, Tim got medical care, nutrition, support and grew up. Of course, they did not have commercial factor. But even Leopold lived to his early 30s without it.
To those with hemophilia then and now, in the words of Tiny Tim, “God bless us, every one!” And happy holidays!
If you’ve never read it, read A Christmas Carol soon! It has never been out of print, and is one of the most enduring, powerful stories in the English literature.
So here’s part 2 of our discussion from last week about whether VWD should be von Willebrand disorder or disease. We’ve gotten responses to our social media posts almost exclusively in favor of disorder!
But my former co-author, the late Renée Paper, had a point to make in 2002 when we first considered making a ruckus to change the name legally. In the first edition of our book, A Guide to Living with von Willebrand Disease, she wrote:
“For a number of years, I have heard from various people in the bleeding disorders community that the name von Willebrand disease (VWD) should be changed to von Willebrand disorder. I hear the same arguments that Laurie has made. Disease is a scary word, disorder is not. The word disease has a negative effect on a child’s self-esteem. Insurance companies are more likely to discriminate against someone with a disease rather than a disorder. Hemophilia is called a blood disorder, why isn’t VWD?
“As an emergency room nurse, I agree that parents have influence over their children. I also think that parents influence their children more with their attitudes than with their words. I believe that a child will accept the VWD diagnosis in a manner similar to that of the parent. If the parent accepts the
diagnosis and does not make a big deal over it, so will the child. If the parent has an intense negative reaction, it is likely that the child will react the same way. I believe that insurance companies do not care what it is called. The name of the disease is less likely to affect payment or coverage decisions than the symptoms and cost of care. Whatever it is called, von Willebrand disease, von
Willebrand disorder, von Willebrand syndrome, or von Willebrand’s, the symptoms are exactly the same and so is the cost for treatment.
“If VWD were new on the scene today and I had to name it, I would call it a disorder. But VWD was named about 70 years ago, and since then it has appeared everywhere as disease. Do we really need to draw this fine line now?
“The most important argument against changing the name is that VWD affects millions of people worldwide, yet it is grossly underrecognized and underdiagnosed. We need to devote all our energy and resources to educating others about the disease and its symptoms and to providing appropriate
laboratory evaluation and treatment. A name change would involve a vast amount of money and time. New educational materials would have to be printed, and the FDA would need to approve new labeling on medicines to treat it. It is no simple task to change the name.
“Around the world, medical literature universally calls it von Willebrand disease or VWD. For consistency, we should, too. Let’s not muddy the waters now when we are working so hard to spread the word and increase awareness. Money would be better spent by focusing on the big picture, that is, ending the suffering and isolation felt by the many people worldwide affected by VWD.
Renée Paper, RN
So, what do you all think? The Irish Haemophilia Society has decided to call it a disorder, because its members have requested it. Is it time for us too, to update the name to disorder?
I recently saw on Twitter that the Irish Haemophilia Society has decided to use the term disorder when referring to von Willebrand disease. It will now be called von Willebrand disorder. I want to shout hooray! At long last, one organization has seen the light!
Why at long last? Because I’ve been promoting this idea since 2002, when I published the world’s first book on VWD. The excerpt below is from that book, where my co-author Renée Paper and I published our differing opinions on the subject. And not too long ago, a debate erupted on Facebook about what to call VWD. Very heated, very polarized. Tonight I’ll share what I wrote back in 2002. Next week, I’ll publish what Renée, a nurse with VWD, believed.
Excerpted and Adapted from A Guide to Living with to Willebrand Disease, 2002:
For a while, my co-author Renée Paper and I weren’t sure what to title the world’s first book on VWD, published in 2002. We could not agree on what to call VWD—von Willebrand disease, von Willebrand, or break all the rules and call it von Willebrand disorder?
I considered the question as a parent and a person with a degree in child psychology, who had published research on children’s concepts of health and illness in 1979. I questioned using “disease.” Is this what we really want to tell a child? It has long baffled me why the bleeding disorder community refers to hemophilia as a blood disorder but von Willebrand as a disease. Aren’t they both disorders? Can so simple a distinction have an impact on a person’s emotions, thoughts, and even health? In psychology, the meaning that is attached to words matters, especially to a child. I worry for the child who has VWD. To a child, the labels that a parent uses matter a great deal.
To people who work in the medical field, the term disease may not mean anything different than the term disorder. But to a child, disease may carry significant associations that are almost always negative. To a child, disease is usually akin to death—it can mean fatal, degenerative, and contagious.
Think of the impact of telling a child that he or she has von Willebrand disease. How does she view her condition in relation to others? Does he think that he could die of it? Will it get worse? Can others catch it? Calling it a disease, when it could be called a disorder, may provoke unnecessary anxiety, fear, and misunderstanding.
So why don’t we just change the name? I have been told that it’s more important for VWD to remain a disease for now. The bleeding disorder community needs healthcare dollars to educate the public and find those with VWD who remain undiagnosed and suffer in silence. So the community
continues to use a word that generates concern and commands attention. The word disease has impact.
In the end, Renée and I opted to use the conventional term to avoid rocking the political and medical establishments. But as a parent or as a person with VWD, you do not need to follow a political agenda with your child or your family. If you are the parent of a child with VWD, ask your child the difference between disease and disorder and listen what he or she thinks. When you explain diagnosis or treatment to your child, you may want to leave out the word disease and instead say von Willebrand or VWD. Ask your child’s doctors and nurses to do the same. More important than any political agenda and more important than what the medical books say is your child’s self-esteem. What you tell your child about VWD matters a great deal. Be sure it is as positive and empowering as possible.
Tuesday is “Giving Tuesday,” an annual event when Americans will donate up to $550 million to their favorite charities. It’s essential that this Giving Tuesday, on December 1, 2020 be as successful as last year. COVID has wreaked havoc with the economy, and people have much less disposable income. The unemployment rate is at 10%. But the needs are even greater. According to Project Bread, more than 80%, or 4 in 5, food banks are serving more people now than they did a year ago.
And while the pandemic has hit America hard, overseas, developing countries are struggling even more. That’s why I give to Save One Life, the international nonprofit I started in 2001. We support children with bleeding disorders who, even in the best of global health times, suffer cruelly. They often lack access to injections of factor, which would give them a normal life. This medicine is readily available in the US and other developed countries, but we forget about those in need who cannot get this medicine.
Save One Life offers one-to-one sponsorships with a child in need; scholarships for college or vocational school; microenterprise grants to those who wish to start businesses to sustain themselves, and camp funding for children to have some joy in their lives. And of course, medicine—factor to inject into their bloodstream to replace the missing protein that makes their lives painful and short. Look at our stats so far this year!
While we’ve been successful despite the challenges, each day we learn of more children in need. Help us to help them. Consider giving to Save One Life on Giving Tuesday. Visit our website to see if one of the beautiful children there speaks to your heart—they are waiting for a sponsor. I sponsor 17 myself! Or give, any amount, to our mission, so we can continue our wonderful work.
And… just give. To Save One Life, to an animal shelter, to a food bank… find your cause, and act. Many organizations need your support, so they can support those most in need.
A bone to the dog is not charity. Charity is the bone shared with your dog, when you are just as hungry as the dog. —Jack London