Bleeding Disorder Awareness Month

Tamer’s Story

Tamer in 2022

March is Bleeding Disorder Awareness month, and we are celebrating by sharing the stories of those with hemophilia in developing countries. Tamer Hanna is from Egypt, and I just visited him for the first time in our 16 year history. I asked him to share his story, in his words, of growing up with hemophilia in Egypt.

“Hemophilia!” the two young parents exclaimed.

“Yes, it is a lifelong bleeding disorder,” explained the doctor to the young parents. They were dreadfully puzzled while looking at their first child who had just been circumcised, as the doctor stitched the wound to stop the bleed. The parents’ families had no history of hemophilia and they had never heard about such a rare disorder. They left the clinic with little information and a lot of precautions. The two young parents had no idea what effect a bleeding disorder would have on their family’s life or their child’s future. They felt overwhelmed. They both were sure that their life from that day on would never be as usual as the life of any parents.

In Egypt, children with hemophilia received minimal health care and follow-up during the 1970s. That was to be expected: hemophilia care in Egypt started in 1968 and mainly took place in Cairo, the capital, so patients from other cities had to travel to Cairo for treatment. I still remember my father getting up early every Monday at 3 am so he could catch the first public bus to Cairo to buy me just one vial of 150 IU factor VIII. The national governmental company only sold the factor only on Mondays, and would allow only one vial per patient. My father had to travel as early as possible because “the early bird gets the worm.” Many patients couldn’t even find a vial to buy if they reached the center a little late on Mondays— all the vials were sold. And 150 IU was the only assay size available in the governmental center that sold plasma-derived clotting factor.

Tamer in 1990s

Like most Egyptian hemophilic patients, I spent many years in this dilemma of on-demand treatment strategy. Due to cost and resource constraints of plasma-derived clotting factor, I used cryoprecipitate to manage my bleeding episodes. I also used fresh frozen plasma, although it wasn’t an effective way of treatment. I even used basic first aid treatment like RICE when there was not treatment available on time.

Not getting a proper treatment on time led to a lot of bad side effects. Like most hemophilic patients in developing countries, I am suffering from the most common complications, such as weak muscles, stiffness and limited range of motion in most of my joints.

But the severest complication happened to me when I was 24 years old. A fracture in my left leg and the lack of enough factor to have surgery caused a disaster. In March 2001, I was able to come to the United States, to Tennessee, where an Egyptian hematologist named Dr. Wahid recommended amputation above the knee. I returned home to Egypt a year later with no left leg and a prosthetic. It’s the same prosthetic I have this very day. It was frustrating and devastating for a young man to lose his leg when he was still planning for his future, but the glass-is-half-full part was that I learned a lot and I have met wonderful, helpful, philanthropic people in the US. Also, I learned to use the computer and the internet. I have been taught to cope and adapt to the new circumstances. Knowing how to surf the internet was the step that enabled me to get in touch with the person who has changed my life from misery to happiness for years so far.

Tamer in 2003

I have heard the name of “Laurie Kelley” when I was in the US, so the first thing I did when I returned to Egypt was to google her name. I started to read a lot about her projects. I thought she was an incredible special person to do all these services for people she hardly knew. I wrote a simple email asking for her help with medicine, then I crossed my finger for a reply. Surprisingly, she replied much quicker than I thought. For more than 17 years we have been writing emails. I can’t recall how many times she donated factor to me, but I believe she did it welcomely tens of times. These 17 past years were the best period in my life. It is a blessing to find a vial of factor in your fridge when you badly need it. I didn’t have this privilege before knowing Laurie.

These past 17 years were the period of flourishing and success. I got married and had two children; I accomplished my master degree in psychology; I worked without taking long days off; I have supported my family financially and I was psychologically stable and safe about the future.

Meeting her here in Egypt in February 2022 was culmination for a long friendship and support relation. Years before, she promised to visit me, and she always keeps her promises. Meeting her for the first time after exchanging emails for 17 years was a remarkable day. She promised to come again and I am sure she will do it when she has the time. Before Project SHARE, I spent night after night in pain, and month after month bedridden. Now, I am much better and safer because of this wonderful pioneer humanitarian person who believed in her abilities and started a humanitarian project served the bleeding disorders community worldwide since 1989.

I was helped once I asked for her help, but there are thousands of Egyptian hemophilic patients who need help too. After 40 years, there are 20 hemophilia treatment centers in Egypt and the Egyptian Society of Hemophilia (ESH) was founded to help with increasing awareness and improving care and empowering people with inherited bleeding disorders. Even still, there is much to be done and we need the help of everyone on this earth to save more lives. It is heartbreaking to read about the death of a human being because the lack of proper amount of factor to stop his bleed. There is a great gap between the number of patients and the available amount of medicine in many developing countries. The government is trying to improve health care; the budget allocated to care and treatment for inherited bleeding disorders is progressively increasing but we are still on the first step of a long road. Thank you, Laurie, and thanks to everyone who has donated factor to Project SHARE. With everyone’s help and Laurie’s zeal, the dream to save thousands of lives is achievable one day.

Hemophilia in Tanzania: Meet Hepson

March is Bleeding Disorders Awareness Month, when we highlight the struggles and triumphs of those with bleeding disorders like hemophilia and von Willebrand disease. Many of the stories you’ll read are based in the US. But this month, I’d like to highlight stories from developing countries. While we all struggle with bleeding disorders, those in developing countries have special challenges.

Tanzania is the most populous country in East Africa, with an estimated 62 million people, the 24th most populous country on Earth. Its population is 77% literate, with a low unemployment rate. Infectious disease is a high concern here. The average monthly wage is $157. The national anthem? “Mungu ibariki Afrika”—God Bless Africa.

Meet Hepson A. Minja, from Tanzania

I was born on August 28, 1962, the last in a family of four children. I was born in Tanzania, in the Kilimanjaro region of rural Moshi. My parents were small scale farmer’s, farming the land and selling our produce.

Hepson in 2004

I started having problems with my blood when I was three years old, but nobody knew what the problem really was. When I was seven years old, my legs and other body parts began to hurt and my parents still had no idea what the problem was.  My pain and swelling made me start my primary education very late compared to all the other kids in my village. My family struggled to find out what was really happening to me, so they took me to a witch doctor. But still, no one knew what the problem was .

In 1973 my parents took me to Nairobi, Kenya , to Kenyatta Hospital, where I was diagnosed with hemophilia. At that time there was no medication for hemophilia there,  and due to poor record keeping, my medical records, diagnosis and visits were all forgotten.

In 1980, when I was 18, I was circumcised. I lost a lot of blood which required me to be hospitalized for two months for regular blood transfusions. The doctors in Tanzania didn’t know what the problem was but I eventually got better.

Dr. Mwanda, Laurie Kelley and Hepson Minja

In 2005, at age 43, I fell and broke my right leg. The internal bleeding caused my leg to swell; to avoid infection and gangrene, the doctors suggested that my leg be amputated. The amputated it and failed to understand why my blood did not stop coming out! I had to stay in the hospital for four months for regular blood transfusions.

Things changed in 2006. My brother Richard, who was educated, got in touch with Laurie Kelley on the internet and they started communicating on the best possible way to help me. Laurie suggested I go to Nairobi to visit Professor Mwanda, who headed the hematology department at Kenyatta Hospital. He diagnosed me with hemophilia A, and prescribed factor FVIII. I returned back home and continued with my regular job as a TV and radio repair man with only one leg.

In late 2007, I fell down again and broke my second leg. I was at the regional hospital for a month and that was when the first factor from Laurie arrived. But the local doctors at that time had no idea how to infuse factor VIII, so I was discharged , and sent to Nairobi to visit Professor Mwanda again.  I was at the hospital again for four months, where Laurie Kelley came to visit me, as well as Dr. Assad Hafar of the World Federation Federation of Hemophilia.

Laurie Kelley with Hepson and his family

I was the first person in Tanzania to be successfully diagnosed with hemophilia but there was no factor VIII at the time. With Laurie’s help, my brother Richard and our doctors in Dar es-Salaam, the capital, founded the Tanzania Hemophilia Society, to help everyone in the country with hemophilia.  Richard and his team facilitated trainings for medical personnel, and received an outreach grant from the Novo Nordisk Hemophilia Foundation, to try to identify more people with hemophilia in Tanzania. Based on our population, we could have up to 3,000 patients! But I was the first identified.

I personally would like to thank Laurie for her never-ending support to me and to people with my condition. She saved my life in more ways than one. Sometimes it is hard for me to believe that I am 60 years old now. I’ve spent a lot of time in hospital beds and I could never set aside a retirement plan for me and my family up until now. I still have two kids that depend on me financially. But I can work, and will work hard for their future.

For anyone who would like to help Hepson and his family, contact Laurie Kelley or write directly to Hepson.  

Don’t Forget VWD This Month!

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!

Visit the World Federation of Hemophilia and Save One Life to learn more.

Excerpted from A Guide to Living with von Willebrand Disease by Laureen A. Kelley and Paul Clement.

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