We had a great Easter. I didn't even get out the camera to take pictures but it was lots of fun... believe me. Eve was so excited about hunting for Easter eggs!! Then on Monday Grandma Kim bought a swing set!! Eve loves it... she plays on it and runs around screaming "this is the best day ever!!!" It was so sad to see Kim go. It was nice having someone around entertaining Eve, giving me a break, someone who could actually stay on top of dishes. There is a reason that woman has survived 4 kids.... she has it together! I am so happy she was able to come down at a moments notice and spoil Eve rotten while we were out of town. Eve did not want us to come home... she was worried that would mean Kim would be leaving right away. Now Eve is sad she is gone.... she is begging us to snuggle with her at night and neither of us has the guts to deflate our air mattress... we know that is going to cause a meltdown.

We did end up getting the factor for Ezra. That makes me so relieved. Now I don't feel like I have to helicopter parent him... if/when something happens we have something to treat him with. It was pretty stressful knowing that if something happened we didn't have the factor.

So far he is doing pretty well. He is bruised up from nothing but otherwise doing great and happy as a clam. I am amazed at how happy he is. He is acting like he is teething... which should be a wild ride. Every time I get him out after nap I brace myself for the bloody mess that they told me to expect with teething... but he has yet to cut any teeth. He is just gnawing on the chair legs and other things we wish he wouldn't gnaw on and I keep trying to force teething toys on him... and at some point one of us will give up the fight... I am hoping he gives in first.

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.

Hooray!

Who ever thought I would be so excited about a doctor's appointment? Not me! Our scheduler called today and we have an appointment on Wednesday!!! I can't wait to hear from someone who can answer some of our MANY questions.

I keep bleeding . . . I keep, keep, bleeding...

So now we know what is wrong with Ezra. It doesn't make it any easier... He has factor 9 deficiency, otherwise known as Hemophilia B. We really won the genetic lottery here. There are only 3,300 people (males) with it in the United States. Aren't we lucky? I have so many fears for the future. I worry that he will hate us for trying so hard to protect him, that I won't be able to protect him, that he won't be able to give us grandchildren. It is so overwhelming. We are still waiting for Children's Hospital in Seattle to call us and set up an appointment. I am hoping after going to Seattle I can feel a little more hopeful about his future and ours. But right now I am just going to lock myself in the bathroom and cry for a while.

Ouch!

For this post I will not post pictures because I know how much my mom hates pictures of my kids hurting. Since Ezra started crawling he has been getting crazy bruises. We called the doctor about it and over the phone they decided that he was just a kid getting bruises and that we shouldn't be too concerned about it. Then on Friday I took him in because he has had a tough time getting over this nasty cold that has been going around. While there the doctor notices his bruise and asks what happened... and then I explain this is what I am talking about... horrible bruises with no event that I can think of that would cause them. So then one of my worst days as a parent happened... they had me take him in for blood work... all I can say is horrible. I guess I should also mention Eve was there with me because I thought at most we would be getting something for the cold. We were in there for 45 minutes while they poked and poked and poked Ezra trying to get one of his tiny little veins. Of course they had me holding him down and I was crying and Eve was yelling that Ezra didn't like it and just wanted to go home and take a nap... it was traumatic for all involved. All we know now is that Ezra has a blood clotting disorder and are waiting for more test results before we know where to go next. I dread it... I hate the blood drawing and I will tell you, he is bruised from the middle of his upper arm to the middle of his lower arm on both sides... it isn't pretty!! So now I am just waiting for answers and hoping my baby will be okay.