Ezra and hemophilia

Our trip was a lot of information... and that made me realize when I say that Ezra has hemophilia B a lot of people have no idea what that is. Hemophilia is when the blood does not clot properly. In order for the blood to clot properly there are a number of clotting factors that need to be present. Someone with hemophilia is missing or has reduced amounts of one of those factors. The most common factor to be missing is factor 8. Ezra has a normal amount of factor 8, he has less than one percent of factor 9, which means he has severe hemophilia B (also known as Christmas disease, or just Factor 9 deficiency). This does not mean that he bleeds faster than a normal person it just means that he bleeds longer than a normal person because his poor little body is missing what it needs to for a good clot.
Hemophilia is a genetic disorder so it is something that he has had since birth; we just found out about it now. Hemophilia is something that is an x-linked trait. If the genetic mutation did not start with him it came from me. Right now we are doing genetic testing to see if I am a carrier and if I am the next step is to see if my mom is a carrier too. The doctor in Seattle said that hemophilia usually breeds true. So since Ezra is severe that means that if it manifest in another male in the family we should expect it to be severe as well. It would be almost impossible for someone with severe hemophilia to go undiagnosed for years and years... it is pretty obvious that something is wrong. Just looking at Ezra's little bruised up body from crawling around lets you know something isn't right. So it probably is not undiagnosed in any adult males in my family.
The big dangers with hemophilia are not actually the visible cuts that most people think of, those are obvious, you know there is bleeding and if needed you can treat with an infusion of factor. The scary thing is the bleeding that we can't see. There are many types of bleeds. The one that we are hoping to delay as long as possible is a joint bleed. Once Ezra gets a joint bleed he will go on prophylaxis. Prophylaxis is treating with factor usually about twice a week to reduce the chance of him bleeding. I am all for this! I don't want his joints to get destroyed from bleeding! I want him to have as normal of a life as possible.
Seattle Children's was not sure how we are going to get our factor. They are in the process of figuring it out. They are not sure they can send it up to Alaska since they aren't authorized to prescribe in Alaska. So we will see... something will be figured out. It was hilarious because they have no idea about Alaska. I mean Ketchikan is pretty darn close to Seattle I wouldn't expect them to have those wild ideas about Alaska that a lot of people from the lower 48 have. They honestly thought we might be living in the boonies with no electricity and no emergency room anywhere close to us.
It was an informative visit. We met with a great doctor, an experienced mom, and an amazing hemo nurse. Of course we are still in the honeymoon phase. We know what he has but we haven't had anything happen that would need treatment. I am sure the reality of it won't sink in until we are sent to the emergency room after calling the hemo hotline and Ezra has to have an IV to administer factor.