NHF Annual Conference in Anaheim!!

Our world here in Ketchikan is small. Unless you want to fly or take a long ferry ride out of here there is really no where to go but Ketchikan. In some ways this is nice. I don't know if I have ever gone to the grocery store without running into a least one person I know. In some ways this is isolating. Add to the mix a bleeding disorder and some times we feel so very alone. I know there are others out there but we don't get to go to any NHF chapter meetings (it is just too darn expensive to get off the island). Other than the blogs I regularly stalk, prior to our trip to Anaheim, we had talked to two people with boys who have hemophilia.

When we got to the NHF meeting it was great to sit around and have an opportunity to talk to other families who were new to the world of bleeding disorders. I enjoyed hearing the stories of how their sons were diagnosed and how they dealt with it. It was great to be able to share and receive advice from others who truly understand what we are going through.

There were a lot of sessions to choose from to attend and at times it could get overwhelming. There are a few sessions that really stand out to me. I loved hearing about how gene therapy trials are going. From that session I learned that they are further along in gene therapy than I thought. I know it still has a long way to go... but the hope that brings is amazing. I also was excited to hear about the genotyping project because it just makes sense to me. I can't wait until they have it set up so that we can get the boys' genotype in there for research purposes.

A not so medical session that I really enjoyed was one about the sibling situation. It is tough being a sibling of someone with a bleeding disorder. So much of  Eve's life is focused around infusions and medical appointments that I feel like she gets robbed of the attention I would like to give her. I was curious to hear what the siblings would say. There were two women that had brothers with bleeding disorders and a boy who had hemophilia and also has a brother with hemophilia. Overall I got the message that there really isn't any resentment of the sibling with a bleeding disorder, they just resent the parents (awesome huh). Other than that I think the best we can do is keep lines of communication open. The boy who spoke just made me cry. The way that he talked about his brother was just amazing. I have felt so guilty having these two boys with hemophilia. Hearing his story makes me feel so much more hopeful. The picture he painted of his relationship with his brother is exactly what I want for my boys. I want them to be able to talk to each other about anything and have such empathy for what the other one is going through.

We ended out the conference with a night at Disneyland. It was awesome to have that opportunity. Disneyland was super crowded but the kids enjoyed it. So I am not going to complain any more than saying it was crowded.

I think we went away from the NHF conference feeling a little sad that we don't really have the opportunity to be involved with any NHF chapter. The reality is we aren't moving from where we live now. We have an amazing support system here, a great job, and an amazing community. We will try our best to do something. I know when our kids are old enough to go to hemophilia camp (starting at age 7) they are going!!!

My dad was able to come to Anaheim to. It was fun for the kids to get to spend time with their Grandpa Lynn. He took us to California Adventure one night and was even suckered into riding a super wet ride with Eve. The kids loved having him to walk around with and it was great having another adult there. I am glad he was able to come. 

This is Eve in Disneyland. You may notice the bruise on her eye. The night before we got on the plane to Anaheim Ezra threw a book at Eve's face. Awesome huh? Evan didn't want her to be alone on being bruised and fell off the bed at the hotel when we was jumping and hit his head on the nightstand.... good stuff.

We got to see the Halloween fireworks show and played until the boys dropped.  Overall, the conference and the trip in general were worth the 4(2 down, 2 back) plane rides with a grumpy Evan.

Infusion baby

On Thursday after I did Ezra's prophy he told me "baby's turn." So we poked a water baby. This all happened while Eve was at school and when we talked about it I could see she was interested. She asked me if the water baby was leaking water (a concern I had but I thought oh well) which it wasn't. I thought that would be the end of that. Then this morning when we started putting the numbing cream on the boys Eve and Ezra both wanted to infuse their babies. Don't worry we did not push real factor into the babies... Ian was thrifty and recycled the syringes from the boy's infusion so we could use them on the babies. The kids were thrilled... and there were Dora the Explorer band-aids to go around.

Yet another ER visit...

How did we go over a year with Ezra having a port before we had to take him in because of a fever? I think it is probably because I was pregnant and had a new baby and we never went anywhere to pick up germs. Yesterday we went to gymnastics.... the breeding ground of all good germs in Ketchikan. Ezra sure loves it there... my nerves don't love it there so much. One time he fell and magically cut his head open while at the very padded gym. If I remember right this was before Evan was born. Can you imagine watching two little boys going in different directions while other kids run wildly around them... this is what I decide to do for fun on a Wednesday? Anyways...

Evan woke up from his nap yesterday afternoon feeling a little warm. I thought I better keep track of this fever. Easier said than done. I spent an hour cleaning the house looking for the darn thermometer that actually works. The one that doesn't really work said his temp at 99... not much to worry about. Then I had to 

torture my one year hold by making him hold still enough for the thermometer to read. Is it just me or do they take forever? Well it didn't finish reading but it got up to 101.7 before he decided he just wasn't having it.

 I thought the earlier we got to the emergency room to have his blood drawn out of his port the better. But I was also in the middle of making dinner and making our bread for the week. So I called my mom to come watch the kids and my bread while we went to the ER and we set down to eat dinner. Then we called the hemophilia nurse to give her a heads up about what was going on.

At the ER the doctor was not on top of his game... I think that is a nice way to put it. Doesn't anyone read a chart? So he said Evan had an ear infection and was ready to send us on our way with antibiotics... I said so  don't we need to have blood drawn out of his port to check for infection. He was like oh he is the one with the hemophilia.... A real gem of a doc. I know he was probably tired. It was the end of his shift but come on! Then the lab came to draw blood out of Evan's vein. Ian said what about his port... they had to check to see if that was okay. It better be okay because that is where it needs to come from. So they had us access his port and they drew the blood out. Then we flushed his port and they sent us home with antibiotics.

So today I had to go follow up with our pediatrician. Evan doesn't have an ear infection. Just as I suspected. He does have a cold... just as I thought. We have to keep an eye on him and wait to see what happens while they grow the cultures (takes 5 days). I wish these things would happen when our clinic was open. Our doctor is really amazing... can't she just be there 24/7? At least we have a hemo nurse on call at all times.

Double Prophy day...

My least favorite day of the week is the one day a week when we have to infuse both boys. Ezra's prophy schedule is every other day... Evan's is twice a week. So depending on the week their prophy schedules align on a Tuesday or a Friday. For some reason in my mind it just seemed like that would be easy; line them up and get it done. I think maybe it wouldn't be so bad if we could do it at our own pace... but there is a rush to at least get Evan infused before Ian goes out the door to work... and usually we try to get set up for both boys while we are at it. Try is the important word... looking at my records Evan usually happens at 8... so Ian is late for work and Ezra has to wait until after Eve goes to school and Evan goes down for a nap. That just makes me feel like I spend half of the day dealing with prophy.... but that is way better than spending half the day in the emergency room... or even the doctor's office.

It's the Protocol

It's been a long night.

I know it is a terrible picture but we are waiting to be released from the hospital and he was sleeping on my arm..  Since Ezra had his port placed we have not been able to give Tylenol for fevers.  Fever is a sign of a possible infection in his port.  So, when he has a fever we wait to see if it will go over 101.  Sunday was the first time Ezra had a fever that broke that barrier, 103.3.  A quick call to a hemo nurse and we were off to the ER.  I walk in and tell the receptionist Roskam, Ezra like we were there for an appointment.  I get a blank stare and the doctor behind the counter says "we've been expecting you" and we get walked right to a room.  Now that is what I call service.  The whole reason for this visit is to determine what is causing his fever.  One blood culture from his port, one from an arm, 4 additional vials from his arm for other tests, some unexpected chest xrays, and a fight for a urine sample and we were both exhausted.  Then we had to wait almost an hour for the prophylactic IV antibiotics. Tylenol kept his fever down for the night and he didn't need it the next day.  Now he is nearly back to normal.  Tomorrow we should find out what the cause was but it is pretty clear it was bacterial.  The real question is where the infection was.  During the follow-up appointment Ashley asked what we should do if Evan developed the same fever.  The answer?  The same thing, it is the protocol.  Feels strange to say this but I am looking forward to when the boys infuse IV.  Of course that has it's own set of problems but at least a fever doesn't mean a trip to the ER and blood tests.

Same old stuff

Well last night Evan split his frenulum AGAIN. Of course his big brother helped him out pushing his face right into a kitchen chair. Then not even a half hour later his big brother helped his head into the corner of a dresser. These kids.... We thought the biggest culprit of splitting his frenulum was the pacifier... so we have only let him have it in bed. If the real culprit is Ezra I don't know how to restrict that. Yes he got a time out...  but he is two, and for the most part doesn't think he is trying to hurt him... he thinks he is playing. So we try to be patient and just correct play that is a little too rough. Obviously we fail. Maybe I should just give in and get Evan a big ol' plastic bubble.

Frenulum

So we had a bleed free Thanksgiving... but Evan doesn't like us to get too complacent. He decided to crawl off of a tub of Legos and dive face first into the floor. This kid doesn't even put up his hands while he going towards the floor just takes the entire impact on his face. Of course I pick him up and yes there is blood coming from his mouth. Since he still doesn't have teeth there wasn't much question about where the blood was coming from. AHHHH! 

I think we should be able to order these kids without the frenulum... but I guess if I was picking options for kids I wouldn't necessarily choose hemophilia either. It would be nice if the kid at least put up his hands so his face didn't bang right into the floor when he was doing normal baby things. I mean I am not going to stop him from exploring things... I guess I would rather deal with the split frenulum than keep him from doing the things that a normal kid wants to do.

Seriously?

So this morning when Evan woke up I went to change his diaper... and saw a spot of dried blood on his onesie. Once his clothes came off it looked like this.

It shouldn't be bloody at all what was his body thinking bleeding like that? Probably that he's a hemophiliac or something crazy like that. So we had to call to find out exactly what the plan was... big problem. I guess our pediatricians office hadn't been in contact with our hematologist about Evan's suspected infection and the hematologist wasn't too happy about it. So I spent the morning on the phone with the hemophilia nurse and our pediatrician's office. 

Then I spent the afternoon at the pediatrician's office and the hospital. They drew blood out of his port to check to see what exactly was going on in there with the infection. Then they infused some antibiotics into his port, then they pulled out his huber needle and sent us home. Yay home!! I immediately went to wash off the hospital grime. There is a message on the answering machine reminding me of Evan's follow-up appointment tomorrow. This kid is trouble. Anyone want him dropped off on their doorstep?

Lots of stuff

Today the Fedex lady got quite the workout! She had to carry all of these boxes up our stairs. Factor and supplies for two boys with ports really adds up. The kids think it is like Christmas. They play with bubble wrap, make forts out of the boxes... mom on the other hand doesn't want to think about where to put it all. At least that is the biggest worry about factor for the boys... we know that if we need it we can have it and insurance will cover it. That is a luxury that I am SO thankful for especially since according to Ian (I don't do math) the cost of just the factor for this two month supply for both of the boys is somewhere around 40,000 dollars. Ouch! Our insurance has to cringe every time they see a claim going through with our name on it.
I have to say I know we are so blessed to live where we live and when we do. We never have to question whether or not we can afford to give the boys the factor that they need. This makes it so much easier to treat them as normal as possible.

Keeping me on my toes

Today we went in to pull out the huber needle that was in Evan's port and put in a new one. Pretty routine stuff. When they pulled out the needle I looked down and knew it wasn't looking right. It had pus and was leaking fluid. I said a few swear words in my head... it didn't make me feel any better. So now Evan is on a super strong antibiotic and we will see what that does. I think I will go outside and scream as loud as I can for as long as I can... maybe that will get rid of my frustration. We just can't win... these boys like to keep us on our toes. It seems just when we feel we can relax into some type of normal they like to shake it up for us.

We have a port!!

 

Port surgery doesn't take very long... but it felt like FOREVER!! I know we had done it for Ezra but it didn't make it any easier. Once we got him back I did a quick count on the number of IV attempts I could see. First off I have to tell you the anesthesiologist had given me this talk prior to surgery then looked at Evan's hands and said this is going to be no problem. Yeah right.  They gave Evan gas before they attempted his IV so they didn't have to fight him, so I thought maybe they would really be successful right away. There were 4 pokes in one hand, two in the other... and the IV ended up being in his foot. Good thing we were there to get a port huh?

Evan has done pretty well since the surgery... he is a little fussy (who wouldn't be) and his pain meds make him sleepy (I am not complaining about my baby sleeping). We actually flew home yesterday... the day after his surgery. We flew in on the late flight so mom has the other kids still... I can't wait to see them!

Round 2 FIGHT!

It hasn't even been a week and we were back in the ER.  Evan was munching on a Barbie table and toppled forward breaking his frenulum open again.  This time the bleeding would not stop.  I called the blood center knowing what the answer would be. So, we packed up the family and headed to grandma Mil's to drop off Eve and Ezra before heading to the ER to get the infusion of Benefix that Evan needs.  We had a doctor that was telling us what the options were for a split frenulum and it was obvious that he had not been informed of Evan's special lack of clotting skills.  We pulled out the factor and pretty much told him this is what he needs then kindly asked what other things we could do to help that little tab of skin heal.  After he recovered from his surprise he was very helpful.  Ultimately, he said he was going to sign off on the infusion and get the nurses in to administer factor.  Our favorite nurse from the night shift happened to be on duty this morning too.  One of the other nurses gave Evan a poke then let Bethany have a stab at it.  Success in his foot again.  So now Bethany is 3 for 5, yes we keep score.  It took nearly 30 minutes after his infusion before the bleeding stopped.  Now, that is what we expected when he broke it in the first place.  Only 12 more days till Evan gets his port.

We're back!

My mother used to tell me there is nothing good that happens after midnight.  Well, for Ashley and I it seems like nothing good happens after 6.  While we were waiting for Eve to finish her swimming lessons Evan split his frenulum.  Well it is a little unfair to say that he did it all on his own.  He had a lot of help when his brother pushed his face to the floor.  The paci pushed his lip up and ripped that darn frenulum.  We have been warned that those things bleed profusely.  René the hemo nurse said when she split her frenulum as a kid you could have followed her blood trail for two blocks.  We must have lucked out.  Not more that two drops of blood came out of that boy.  But, we knew that a split frenulum meant a visit to the ER for a dose of factor.  You see that's the great thing about hemophilia and humans being carnivores.  The weak clot that Evan makes is dissolved by the enzymes in his saliva that break down meat and other proteins.  So, the frenulum keeps bleeding.  After a quick stop to pick up uncle Andrew to watch the older two we ran home to get factor and headed back to town.  The ER was crazy.  The lack of close parking should have been a giveaway.  Every room was full with a couple requiring security to stay posted.  We got our room and started the wait.  An hour later Dr. Meloche took a quick peek and said one of the most wonderful things I have heard a doctor say.  "In my years I have learned that when a hemophiliac or parents of a hemophiliac come in they know more than I do about the disease.  I am going to let Bethany give him the medicine he needs."  Then he was gone.  Ten minutes later Bethany poked Evan and got the IV in on the first poke.  AMAZING!  That was the best ER visit we have ever had.  I never thought I would use the word wonderful to describe an ER visit but that's what it was.

It's scheduled!!

So the 25th of this month is the day... can you believe it? It is crazy how quick it all happens. I know it was the same with Ezra but it still amazes me. We go from the talk to two hours later the scheduler calling with a surgery date. It is scary to think about my little baby having surgery but I will be so relieved to have two boys with ports. I will feel like we have more control of our lives, not that at any minute we will have to put our lives on hold and hang out at the hospital for however long it takes to get Evan his factor. Luckily we are already trained to access a port so once Evan is released from the hospital they are fine with us flying home. So we will only be away from home for around 5 days.

Darn joints...

Why do we need them? They are pesky little suckers! We thought Ezra was early for a joint bleed at a year old... I think there must have been a friendly competition between brothers for the earliest... well Evan wins. Yesterday we were eating dinner at mom's house after a busy day and I was holding Evan in my lap and when I moved him a little he started to scream so bad!! I thought weird... and that was the end of that. Then Ian had him and it was obvious something was wrong he was screaming in pain. So we did the joint check and when we attempted to straighten his right knee he screamed and screamed and screamed. It kinda explained this crazy bear walk he had been doing all afternoon. I had commented on how silly of a crawl it was not even thinking he was having a joint bleed.
Luckily we were at mom's so instead of just one of us going in or having to take time out to figure out what to do with our other kidlets mom just took care of them.
Let me tell you I don't know what the difference was this time with the emergency room but it was so much better. I think part of it is that it seems so much more serious when your kid won't stop screaming in pain. He would stop and then if he even moved that knee a little bit he would start right back up. The staff acted so much more informed about hemophilia this time and man were they serious about holding down our little guy while they poked him. It took 3 pokes... not too shabby. The nurse said she felt so sorry about that but we were geared up to be there all night so 3 pokes felt like such a relief.
So where now? I don't know. We will see what the plan is from here. We know that once the joint bleeds start we go straight to prophy. I anticipate a call from my favorite hemophilia nurse tomorrow talking about the possibility of a port; but we will see what everyone decides is best for Evan.

Hating the ER

Yesterday morning Evan crawled right off of the bed. He didn't have a big bump on his head but he cried for 5-10 minutes before he calmed down. So we called our trusty hemophilia nurse and after she consulted with the hematologist she recommended that we bring him into the emergency room and administer factor just to be safe. Well... easier said than done is all I can say. There was a real emergency that came in just after we arrived so we waited for a few hours... then they attempted to poke him 5 times with no luck. After that the ER doctor told me to just go on home. WHAT?? I said I would leave if the hematologist gave me the clear but big warning lights were flashing in my head because the hemophilia nurse has drilled in my head that once they start poking they can't stop until they are successful. Well Evan ended up with a CT scan. Luckily it was clear. Now we are just icing his poor little spots where they attempted to access his veins hoping that they don't swell... worrying that they might and then we might need factor again.
Sometimes it is just so discouraging. What is the point of taking him in when no one is going to help us? They did more damage than good. I hope that the continual icing works... the hemophilia nurse talked to me about compartment syndrome. Scary stuff that we just shouldn't have to deal with. I am not a medical professional. I am just a mom and I want someone to take care of my kid. I don't have a problem holding him down for... lets see Ezra was 12 attempts the worst time but I really hate this attitude that there is nothing they can do for my child. So hopefully we don't have to take him in for a long while... and if we do... well I think I would rather hop on the next plane to Seattle than go into our emergency room again.

Bruising

I took Evan to the pediatrician's office because he had nasty sores in his mouth... poor boy! There was a new medical assistant there and you should have seen her face after I took off his clothes so that we could weigh him. It totally cracked me up!! The whole time she was in the room she was just staring at all his bruises; I don't think she could believe I was bringing in a baby so bruised up. Sometimes I forget... this is just normal for our crawling little boy.

Evan's first bruise

So it is nothing big and doesn't seem to bother him but Evan did get his first bruise. Actually I noticed one on his ankle last week but it didn't feel all lumpy like this little hemophiliac bruise that showed up on his elbow the other day. He also got one on his hand as well. And so it begins. *sigh*
He is so determined... look at him up on his hands and knees trying to crawl!!

And here is Mr. Smiley!! Yes he has a little scratch on his face. I turned my back for one second and came back to a scratch on his face... I am not sure if he did it to himself with his fingernails... or if Ezra did it but he didn't even cry so however it happened it didn't hurt him.

Good news, everyone!!

The results are in on Eve's carrier testing!! Her factor activity level is 77% and she is not a carrier! Praise the Lord!

Here I was thinking my mutant genes were trying to make an entire mutant family... epic fail on their part, but us mutants still have the advantage in our household.

It's World Hemophilia Day

Which means we all are wearing red, or as close to red as we could find in our closets. How else are we celebrating? Two infusions in one day (pray that this finally stops this dang muscle bleed), a trip to the doctor's office for Ez (all because of stupid hemophilia), and then the good stuff... ICE CREAM for dessert tonight!