In this issue ...

Update from Milena

Von Willebrand Disease: What makes it different from Hemophilia?

1. VWF is a very large protein that circulates in the blood and helps to stop bleeding by acting as the glue that sticks platelets together so that they can plug the hole in a damaged blood vessel. Clotting factors VIII and IX, which are low in hemophilia A and B, come along later in the process to form a mesh over the plug and keep the blood clot stable until the damage can heal [figure downloaded from VCAhospitals.com and modified 18-Jun-2021].
2. People with hemophilia are more likely to have deep bleeding in muscles and joints.  People with VWD are more likely to have bruises, nosebleeds, and heavy menstrual bleeding. BUT, this is not an absolute: anyone with a bleeding disorder can bleed from any site, given the right circumstances.
3. Making a diagnosis of hemophilia takes one blood test. Making a diagnosis of VWD takes several tests: one to measure the level of VWF, one to measure whether it is working properly, and one to measure its size. These tests are not simple. That is why people are sometimes asked to come back for a second or even a third round of testing, to sort things out.
4. VWF levels can be affected by many things including age, hormones, pregnancy and medications. So, someone’s VWF can vary over time depending on what else is happening to them. This is much less of an issue for clotting factors VIII and IX, in hemophilia.

1. Getting a diagnosis is helpful. Once you can name your bleeding disorder it is easier to determine how it is best managed.
2. If your bleeding disorder is severe, being connected with a Bleeding Disorders Program can assist you in managing  it.
3. If you think that you may have a bleeding disorder, talk to your health provider about whether you should be tested. For women and girls with heavy menstrual bleeding, a set of questions about your bleeding [called a bleeding assessment tool (BAT)] is a good place to start to figure this out. You can try the BAT at Let’s Talk Period: letstalkperiod.ca

This 2½ day event will centre around the theme of the lifecycle of comprehensive care for WGBDs. There will be plenaries, educational sessions, and several opportunities for exchange through “hot topics” sessions, meet-the-experts sessions, and workshops where participants will hear about best practices and learn practical skills. You will hear from women, youth, and girls about their realities, and what is being done around the world to improve care and treatment for these patients. Register

Supply of DDAVP injectable discontinued

Ferring says supply of DDAVP injectable to return in “one to two months”

Montreal, June 23, 2021 – Ferring Canada, in discussions with the Canadian Hemophilia Society, has stated that supply of DDAVP, the 15 mcg/mL formulation of desmopressin, injected subcutaneously, for the treatment of mild hemophilia A, Type 1 von Willebrand disease and platelet disorders, will resume in “one or two months.” The shortage was apparently caused by shipping issues. Ferring had earlier cited a global manufacturing issue to explain the stoppage in supply to Canadian pharmacies.

The worldwide production of Octostim Spray, inhaled through the nose, remains stopped and is not expected to resume until 2023.

Patients who use either of the formulations should contact their treatment centre for advice on alternatives.

The Hero of our Family's Love Story   

My name is Milijana, I am 34 and I have been living in Winnipeg for the past 2.5 years. I am an IT Business Analyst, but my most important role is that I am a mother of two beautiful girls and a wife to my husband. His name is Dragan, and he is a mild type A Hemophiliac.
 
Before I met my husband, I didn’t know anybody with any kind of bleeding disorder. To be honest, I was so clueless about it, that when he told me that he has Hemophilia, I had absolutely no knowledge about it, besides the name itself. Surely, after that, I made every effort to learn about Hemophilia as much as possible, especially about the type Dragan has, and the ways it affected his life. 
 
My first real contact with any chronic illness happened before I even met Dragan - it was on the day that my father found out he has Diabetes type 2, and shortly after that he was diagnosed with chronic Heart disease. His life, as well as the life of our whole family changed significantly after that, but one thing connects the most important two men in my life: neither of them ever used his condition as an excuse or saw it as an obstacle for anything in life, for which I admire them tremendously.
 
Dragan told me that has Hemophilia as soon as we met. Interesting fact is that we met playing recreational sports - volleyball to be exact. We are both passionate about sports in general - especially volleyball. After that, I started to learn and understand many details and risks about life with Hemophilia, and especially how they increase being involved in sports, being that Dragan is one of those people that love sport so much, and sees in it an opportunity to live a normal life, to be active and improve his health, instead of always looking for and seeing only risks. Thanks to his positive attitude towards Hemophilia and life in general, I started to see it as something that is quite possible to lead a normal life with, although during our relationship and marriage, events that require medical care and interventions different than for people without bleeding disorder occurred from time to time. Surely I’ve tried to understand and learn as much as possible about Hemophilia even more, when we started planning out our family. Today we have two girls and although both are carriers of Hemophilia gene, I want to believe that they will teach their kids to live a life full of possibilities instead of limitations, regardless of whether their potential sons tomorrow will have it or not.
 
Due to the fact that my husband has only mild Hemophilia A, our day to day life is not severely affected - except for a couple of bleeding episodes after dental procedures or sports injuries that required treatment with factor 8. Being that my husband is trained to self infuse the medications he can do it at home, with me being his helper. Sometimes, when his condition would require it, I would go to the hospital with him and take care of the administrative part of his hospitalization, because in our country (Serbia), even today bureaucracy will give you a big headache.
 
What I wished to be different for people with Hemophilia is, in a way, coming true for us here in Winnipeg: every aspect of the health system has accessibility and knowledge for persons with Hemophilia, as well as non existing limitations within the system which are common in countries like ours, where people with Hemophilia have 0% chance of serving in the army or becoming professional athletes or pilots for example. I see it as completely unfair, and as something that could have substantial consequences on the younger population when choosing their life and professional path.
 
What I see as very important for anyone starting a relationship with a person with some kind of bleeding disorder is to try and deeply understand what it means, to learn about treatment aspects, potential conditions and limitations. To put it shortly: the more you know about these issues, the better able you will be to deal with them.
 
Driven solely by my partner’s way of thinking and his approach to his type of bleeding disorder and life in general, my recommendation to anyone that suffers from this or any other type of bleeding disorder is that you should always include your partner in every aspect of it: information, worries, fears, treatments… and never hesitate to ask for help because that doesn’t make you weak - it makes you strong!
 
At last, the most important thing: love is never limited by your condition - on the contrary - your honest and positive attitude makes you a hero of your love/family story.