I’m in Egypt on vacation, and to visit Tamer Hanna, a very special hemophilia patient, and one couple in the group of tourists I am with mentioned they have visited the Äland Islands in Finland. My ears picked up; that’s where VWD was discovered! It was a great opportunity to educate them about the most common inherited bleeding disorder.
March is Bleeding Disorder Awareness Month, and I think it’s a great time to remember those with the most common inherited bleeding disorder in the world.
When did this bleeding disorder get identified?
Dr. Erik von Willebrand
Von Willebrand Disease was named after the doctor who first identified it: Dr. Erik Adolf von Willebrand, a Finnish physician who first described VWD in 1926. He had been studying an unusual bleeding disorder in the local communities, called Älandic hemorrhagic disease.
In 1925, while in Helsinki, Dr. von Willebrand evaluated a five-year-old girl named Hjördis. She lived on the remote island in the Baltic Sea, between Sweden and Finland.
Hjördis was bleeding excessively from her lip following an injury. She had a history of nosebleeds and bleeding following tooth extraction. She was one of 12 children, 10 of whom showed bleeding symptoms. Sadly, four of her siblings died of uncontrollable bleeding between the ages of two and four. Hjördis herself later bled to death during her fourth menstrual period.
When Dr. von Willebrand discovered that 23 of 66 family members (16 females and 7 males) also had bleeding problems. The history of the original Finnish family has now been traced back to 1750! Over 1,000 members, with 125 known to be affected, and 12 who died from bleeding.
Dr. von Willebrand concluded that this was a previously unknown type of hemophilia, characterized by prolonged bleeding time and mucocutaneous bleeding. The disorder affected both males and females. Dr. von Willebrand called this new disorder “pseudohemophilia.” But because he believed platelets were involved, he later renamed the disorder “constitutional thrombopathy.” In 1928, four American doctors described a similar disorder, but it wasn’t until decades later that the actual blood-clotting protein involved was identified. The protein—von Willebrand factor (VWF)—and the disorder were named after the doctor who first described the unusual symptoms.
It’s estimated that VWD affects up to 1% of the world’s population, which dropped the jaws of my fellow tourists. Overlooked for too long in favor of focusing on hemophilia, I think VWD is now getting the recognition, and help, it deserves. But women in countries like Egypt still suffer and need education and medication. We can all help with that!
I’m in Egypt, first to visit a patient with hemophilia (read this story in last week’s blog!) and second to vacation and learn all I can about ancient Egypt. It’s an incredible tour, complete with lectures and a visit from the famous Dr. Zahi Hawass!
As I am learning about the ancient gods and pharaohs, one symbol keeps appearing on the carvings on the temples and tombs. A bull with the sun sign between his horns. Where have I seen that before? It’s the logo for the pharmaceutical company Novo Nordisk, which is a Danish company.
Who was this god? He is Apis, one of the sacred animals of ancient Egypt. He represents strength and fertility. His mother is sometimes listed as Hathor, an extremely important goddess, whose temples are everywhere. I visited some and carvings everywhere show the cow and bull.
Our guide, Mohammed, tells me Hathor is the goddess of health and motherhood, and was also the mother of the god Horus, one of the most important gods of ancient Egypt.
I just found this all so interesting. The word “logo” is defined as “meaning,” so it’s always important to choose a logo (image, color, size and intensity) that has specific meaning to the company. I think Novo Nordisk chose well!
Tamer Hanna is blessed: intelligent, handsome, fluent in English, beautiful wife and two wonderful children. At age 45, he has a steady income and is self-sufficient. He also has a problem: he has hemophilia. And he lives in Egypt, which does not receive enough donated factor so that he can have some at home, or even when he bleeds.
I met Tamer via email about 17 years ago, long enough that I can’t recall exactly when he contacted me, or how we became friends. It was about needing factor, and he knew that I donated factor. His story grabbed my attention and heart: he had had his left leg amputated above the knee due to uncontrolled bleeding. While this sounds contradictory, it’s true: bleeding into the leg can cause the leg to swell, cutting off various blood vessels. The trapped blood can become infected, gangrenous, threatening the life of the patient. In March 2001, Tamer was able to come to the US, to Tennessee, where an Egyptian hematologist named Dr. Wahid recommended amputation. While this is a story for next week, Tamer eventually returned home to Egypt with no leg and a prosthetic. The same prosthetic he has this very day.
I started sending him factor around 2006, and have continued also to this day. In fact on this trip, my first to Egypt, where I am right now, I had about $200,000 with of factor to give him. Egypt has become so difficult to ship factor to. This was the easiest way.
I had promised to meet with him one day, the latest promise was in 2018. He had given up asking when I was coming to see him. I only told him about this trip about three weeks ago, and sent him the flight itinerary so he knew I was serious this time!
I left last Thursday, and in 24 hours I landed in Cairo, and sailed through customs.
On Saturday morning, February 26, my driver Ahmed picked me up and after a couple of stock phrases from each of us (he spoke almost no English and I almost no Arabic), we were on our way. The streets of Cairo were clogged with auto-rickshaws, cars, trucks, buses, “microbuses,” and tour buses. Ahmed was a deft driver, weaving in and out of traffic on the unlaned highway. After a few heart-stopping moments, we would give each other the thumbs-up sign. Traffic was heavy and a 90-minute trip took almost three hours. I took in the towns and farms we passed in this ancient land.
We finally arrived at a modest concrete building, which housed apartments. Luckily there is an elevator. With only one leg, and always on crutches, it’s hard for him to get around. He works as a teacher of English as a second language at a nearby school. On his days off, he mostly stays home, in a small but comfortable apartment. The small balcony overlooks a busy side-street, directly across from the Coptic Christian church, of which he is a dedicated member.
Our meeting was surreal! Tamer asked ahead of time if he could hug me; something not common in conservative Egypt. But of course he could, and we did. We rode up to his floor, and seated ourselves in the living room. His wife Susan had gone to great lengths to prepare a feast for me: two kinds of soup (including the delicious molokhia), chicken, beef, vegetable rolls and my favorite, a baclava with ground beef in a fluffy pastry. It was delicious, especially after such a long ride.
His children, Dede, age 12 and David, age 10, politely stayed throughout the entire visit, and were well behaved and respectful. I had brought gifts for them, and they gave me a lovely teacup with Nefertiri on it!
We chatted for three and a half hours, about what life is like in Egypt when you are disabled. There are no disabled facilities or special treatment for those with chronic problems. Wheelchair accessible? Forget it. Access to factor for a random bleed? Not going to happen. Even getting married was a problem. Tamer had had prospective wives (“The girlfriend/boyfriend concept does not exist here,” he once told me), but all had backed out when they learned about hemophilia. Oh, it wasn’t his hemophilia that was the problem; what if they had a daughter and the disorder was passed on to a grandchild? He found love in Susan, who happens to be a first cousin. She is kind, loving, very funny, devoted to making a happy home for them all.
His children are learning English, and Dede wants to be a doctor someday!
I promised Tamer I would always find factor for him. He’s been a faithful friend all these years, soon to be decades, and I have no doubt our friends in America will always donate enough to help him.
Tamer works hard and yet earns in a month about what one nice meal would cost for 3-4 people at a nice restaurant. He doesn’t take vacations or go shopping or attend sports or concerts. He doesn’t exercise for fear of causing a bleed. Think about this: he messaged me this once, “Many times I imagine what my life would be without this factor that you guys would throw away. My family wouldn’t find a loaf of bread to eat because the father, who is the only working person, would be bedridden. There would be no hope for giving my children education. As long as I am on my feet working, these three people in my small family will be safe and secure. Laurie, you are doing more than you imagine.”
But I can only do this with everyone’s help. So thank you, everyone, who has donated recently, because your factor went to help tamer.
Tamer and I share the same faith, which says, “Anyone who has two shirts should share with the one who has none, and anyone who has food should do the same.” (Luke 3:10-11)
Substitute factor for a shirt and food and this is what we are doing for Tamer. Thank you, Tamer, for a great visit, and thank you hemophilia community in America for sharing “with the one.”
Spring is coming, and it feels like it, as more and more, the country is opening up. It’s wonderful to see that some of our community events are back in person! Read below to see which ones you might attend. Remember, we are not yet through the pandemic. Please be sure you are vaccinated, and be careful. I’m going to wait a bit more before attending, but remember, you can always attend virtually. Check it out!
Factor IX Families Welcome!
Register for the Coalition for Hemophilia B’s annual meeting in Orlando! May 19-22; travel grants available! Attend also virtually. Register here.
NHF’s 74th Annual Meeting
NHF is thrilled to announce that its 74th Annual Bleeding Disorders Conference (BDC) will take place from August 25 to 27, 2022, in Houston, Texas. After two years of uncertainty, NHF is excited to gather our community in person! You can also attend virtually. Read more here.
Saddle Up!
Texas is the place to be this year! HFA will hold its annual meeting in person in San Antonio, the first time since 2019. Register here!
“All you need is love. But a little chocolate now and then doesn’t hurt.” – Charles M. Schulz, Cartoonist
It’s Valentine’s Day (almost)! And since HemaBlog is about anything to do with blood and hemophilia, why not include the heart? The heart is associated with our emotions–probably because we feel it beat fast when we are afraid… or in love. I confess when I met actor Paul Newman, my heart rate shot up to about 200 bpm! And I told him so!
Here are some fascinating facts about this important organ*:
The heart beats about 115,000 times and pumps about 2,000 gallons of blood every day.
An electrical system (a cardiac conduction system) controls the rhythm of your heart.
The heart can continue beating even when it’s disconnected from the body.
The first open-heart surgery occurred in 1893. It was performed by Daniel Hale Williams, who was one of the few black cardiologists in the United States at the time.
The earliest known case of heart disease was identified in the remains of a 3,500-year-old Egyptian mummy.
The fairy fly, a kind of wasp, has the smallest heart of any living creature.
The American pygmy shrew is the smallest mammal, but has the fastest heartbeat at 1,200 beats per minute (which actually felt like mine when I met Paul Newman).
The giraffe has a lopsided heart, with their left ventricle being thicker than the right. This is because the left side has to get blood up the giraffe’s long neck to reach their brain.
Most heart attacks happen on Mondays.
The beating sound of your heart is caused by the valves of the heart opening and closing.
Broken heart syndrome has similar symptoms as a heart attack. But a heart attack is from heart disease and broken heart syndrome is caused by a rush of stress hormones from an emotional or physical stress event.
Heart cells stop dividing, which means heart cancer is extremely rare.
And where did the Valentine shape come from, that represents the heart? It’s believed to come from the siplhium’s seedpod. It looks like the modern Valentine’s heart. The seedpod’s role with love and sex may have been what first helped associate the symbol.
Happy Valentine’s Day!
*Source: www.healthline.com
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