The BIG Birthday!!!

2/14/2010 Sunday

Happy Valentine’s Day to you all! I am so excited to say that we survived a wonderful year with our incredible son. William Henry turned 1 yesterday and we had a BIG party! Of course though everything was done William style, which lately seems to occur with no less than a handful of drama. ;)

We have been planning William’s party for months actually…gathering ideas and items if I saw them along the way. (I have to admit my dark secret that I actually bought a party kit on clearance during Black Friday shopping when I was still PREGNANT with him…but hey it was on clearance and I had a 40% off coupon on top of it, so I’ve had the monkey theme picked out since then). We’ve been looking forward to a big celebration since many of the most recent holidays have been a little crazy with all of the medical issues popping up at the most wonderful times.

This week was pretty hectic for us as Paul’s bowling schedule kicked back in, he started a new session of community ed pottery classes, and I had both parent/teacher conferences Thursday night, and an Autism conference to attend all day Friday which meant sub plans for one of the craziest days for first graders: Valentine’s Party Day!!! Dun dun dun!

Wednesday afternoon I was nearing the end of my school day when I got a phone call from our Daycare lady Jan. She called to let me know that Will had vomited while napping in his bed and was laying in it. This was a surprise as she said he had been doing fine all day. After cleaning him up she said he sat and played happily until I came to pick him up. He also popped his first tooth on top (which makes for 3 teeth now) so it made us feel pretty unsure if he was sick or if perhaps it was the tooth coming through that made him feel ill.

Wednesday had been our scheduled day to run a few errands, including a trip to buy Will’s birthday presents. Paul and I decided to meet up in Woodbury after Will’s finger poke. We got his gift and then went to dinner at Noodles and Co. where they were doing a fundraising night for my school. Will ate the mild baby food I had picked up very hungrily, but a few minutes later vomited it all up at our table. We packed up our meals and everything else so we could take him home. In the mean time he puked all over Paul’s black jacket and through his two shirts. We got home and put him to bed.

Paul stayed home with him Thursday and they had a very typical day, playing, napping, and carefully eating. He did fine until he vomited around 4:30 pm. Paul put him to bed that night while I was at conferences and just like the night before he slept soundly and never had any issues.

Friday I dropped him off at my Dad’s house pretty early. The plan was for Mimi (my mom) to leave work early and spend the day with Will and Yaya (my dad) getting the last things from the store for the big birthday party. Unfortunately Will vomited up his whole breakfast and spent a lot of the day being sick. I was at my wits end by the time I made it to my parent’s house after my conference in River Falls. We continued to get everything ready for the party, but I was worried sick that Will wouldn’t recover and we’d have to cancel his 1st Birthday party.

We brought William home and tried to give him his antibiotics (from last weeks ER trip for the fingertip infection) right before bed. He was coughing and gagged a bit on the medicine and then proceeded to puke up everything, all over himself, the white carpet in his room, and Mommy and Daddy. During the night he slept well again and nursed well in the morning. We had planned to let Paul and William stay home in the morning to nap normally while I went to my parent’s house to decorate and set up everything left for the party. I guess the morning at home went ok, and his breakfast stayed down. When they arrived at my parent’s he was very clingy and attached to me. I again felt extremely nervous that he was going to be sick for his party. (I can’t discount the fact that there were almost 40 people there, but generally he is friendly and happy to be around most anyone). We fed him carefully but soon enough he vomited all of the baby rice cereal back up!

We were leery about giving him cake, but he dug right in to his carrot birthday cake and even got his face right in there to suck off a lot of the frosting! He did his job as a one year old and it felt good to have that special moment. Amazingly he seemed to feel a little better after that. He got on the floor and played with the kids at his party. He smiled and was held by people. Then he survived the storm of presents that everyone spoiled him with! (THANK YOU EVERYONE!!!) Not long after that he fell asleep on my grandma’s lap and stayed on the couch with she and my grandpa for maybe 45 mins.

After he woke up he acted very hungry again (throughout all this he has had the DESIRE to continue eating and I think he has been truly hungry for days). We decided to give him a bottle of breast milk, which he drank right up. Unfortunately he sat up after that and vomited it all over me! This sort of started an off and on cycle for the rest of the evening. We’d feed him broth or other clear liquids, and as soon as he would cough, up it all came! After everyone left the party, his sweet daycare lady Jan, and her husband Mark stopped by to bring him his gift. He did ok while they visited but threw up a lot after they left. We decided that this could not continue. He was not keeping anything down and we were worried that he was getting very dehydrated. Paul called the on-call Hematologist and then we decided it was best for us to just take him to Children’s ER. In fact I was on my 3rd outfit for the day by the time we made it to the ER!

So yes, poor baby got to spend his memorable first birthday visiting the ER… ☹

The Dr. there decided that since he was coughing, sneezing, and also had diarrhea it was probably not just the antibiotics irritating his stomach. He felt that it must be some sort of virus. They said that they would give him a medicine called Zofran which is an anti nausea medication which would hopefully get him to stop the gag reflex which occurred every time he coughed. Since William was dehydrated their plan was to give him an IV, and at the same time take a little blood to check his hydration levels.

The nurses used something on him that we had not seen before. They called it a “J tip” which I guess is a pressurized tube which shoots air into his skin, pokes a hole with the air pressure, and injects Lidocaine to numb he skin instantly. They said that he wouldn’t feel any of the pokes after that. He still cried very hard, but the nurse explained that kids often get really scared of the sound the “J tip” makes. After 2 attempts, the 2 nurses could not get a vein in his arm. I think it was due to being dehydrated. They were able to get enough blood to “check his chemistry”, meaning that they would see how dehydrated he had become. The Dr. said that level 20 was pretty normal, 12 was admission to the hospital for dehydration, and Will was about a 15 at that time.

We decided against another poke attempt to get an IV started and instead gave him an oral dose of the Zofran. Will then nursed very well and we got to try and give him his first popsicle. They were pleased to see that he nursed so well and kept the milk down. They sent us home with instructions on how to try and re-hydrate him and with a little bit more of the oral Zofran.

Today (Sunday) he has gagged and burped off and on but has not vomited any of the liquids we’ve been giving him. Now we just have to hope that he is on the upswing!

Thank you to EVERYONE for the different ways that you helped to celebrate William’s birthday! Despite the drama, he had a wonderful day with lots of smiles here and there. We are SO lucky to know so many amazing people. We are proud of ourselves for surviving the first year, but so immensely proud of what a tough little boy we have! He is incredible…

Love,
Katie, Paul, and our Birthday Boy

It's been a while...

2/8/2010 Monday

We’re FINALLY updating the blog! A little and a lot has happened since our last update…I decided to wait until today as we just came from an appointment with the hematologist.

Backing up a bit, things have been going very well in the last 2 weeks. William was healthy and as always has been handling his 3X per week finger pokes like a champ! No tears as usual. We got up early on Saturday and had Sarah our wedding photographer over to take some 1 year photos of him. We had some cute ideas and had a great visit. (She is a teacher in Paul’s district as well). We did have to work a bit to get William to smile though and he tired easily.

We noticed after William’s morning nap that his fingertip (the one that was poked on Wednesday) was red, swollen, and the poke site was turning white. Basically bad news, he had an infection! We called the on-call hematologist and he recommended putting some antibiotic ointment on it and waiting a while to see if that helped. He said to wait maybe 6 hours, then go to the ER if it had not improved.

I was lucky enough to get to go to a movie with my friend Elizabeth that afternoon and I was really excited. After I got back from my movie we checked his finger again. Of course it had not improved, we felt that it looked worse. (The only good thing about that is: William has low neutrophils, the white cells that fight bacteria and make pus…in this case it was a good thing that his body was able to make this reaction happen). We packed up a diaper bag and headed off yet again for Children’s ER.

We were anticipating I.V. antibiotics and a possible dose of GCSF (the drug to raise his neutrophils). We were pleasantly surprised to be sent home quickly with 2 oral antibiotics and instructions to let him play in warm water to soak his finger. Yay! (They also knew we’d be going in for our appointment in 2 days so they were satisfied by that.

The plan for today’s appointment was to find out about the genetic testing results. (We have been waiting on these for about 8 weeks). It turns out that the results are STILL not officially written up and reported to our hematologist, but she was able to call and talk to someone who told her that all of them came back negative.

His means 2 things on the surface… 1. We still don’t know why William has neutropenia and 2. There were no genetic mutations that showed up on the tests, which is a GOOD thing!

Digging deeper, there is a lot to explain. Think of the issues he could have in two categories: Production and Circulation of the Neutrophils. Production meaning: how his bone marrow makes the neutrophils. Circulation meaning: how his body takes the produced neutrophils and sends them out into his blood stream.

On the production side, the genetic testing could not find issues with ELA2 and Hax1 genes. Often when they find a mutation on one of these two genes they see that the person has something called Kostmann’s disorder, more commonly referred to as Congenital Neutropenia. This is permanent and the patient has to be treated with high doses of GCSF to keep them healthy. The uncertainty remains though, as some people will still have this form of neutropenia and will not show the genetic mutations.

The other production issue could be Cyclic Neturopenia, where your neutrophil leves cycle from low to nearly normal and back again over a few weeks time. So far Will’s fingersticks 3x a week have not shown any signs of cycling, but at this time we need to keep watching just to make sure.

On the circulation side, the potential issue would still be Autoimmune Neutropenia, sometimes called Chronic Benign Neutropenia of Childhood. (I blogged about this form of Neutropenia explaining it more on 12/18/09). This is something that most children grow out of by age 2 and would be our ultimate diagnosis. There are things that point towards this, but other things that point away from it.

First William has an antibody present in his bloodstream that seems to be killing off the produced neutrophils as they enter. (Autoimmune neutropenia has/does this, which points us towards it). William’s neutropiena has presented itself a lot sooner than the average child who has this though, which points us away from it. Also William has had a few infections through all this…most kids with autoimmune neutropenia do not have many health issues other than low neutrophils, another thing that points away. The interesting thing is that if he does get a diagnosis of autoimmune neutropenia, it CAN be caused by a virus…ahem H1N1 anyone??? (Dr. Schultz is not discounting this theory either).

Dr. Schultz has decided to stay on our course right now. William is staying generally healthy and she does not see any reason at this moment to put him through more tests to narrow these options. Some of the tests could involve another bone marrow biopsy, and/or putting him on something called IVIG Intravenous immune globulin (IVIG). IVIG is a mixture of plasma parts taken from thousands of people’s blood antibodies. Putting these into William’s bloodstream would cause what I would call a windstorm. His body would kick into high gear to fight off all of the antibodies introduced and would then hopefully “forget” to keep producing the antibody he makes that kills off his neutrophils. There is some risk involved as there is potential to cause temporary allergic reactions and to infect him with viruses that could mistakenly be present in the blood plasma. (I took that as he could get infected the same way anyone who gets a blood transfusion could). The IVIG could confirm for us though if William really has Autoimmune neutropenia or not.

To sum it up, we still don’t know why he is neutropenic and it seems like we may not know any time soon. Maybe another year will pass and this will all be over. Who knows! In the meantime we have to finish his 6 week course of finger sticks 3x a week and continue to watch him for infections and take care of them. We will meet with the hematologist once a month to stay on top of everything. Dr. Schultz did send out a blood test today to see if the neutrophil killing antibody is still present and I think we’ll hear back on that one sometime soon.

Paul and I think we can handle all this for now. We’re so lucky for the bits of good news and thankful that things are not worse. The only thing that saddens me is that throughout all of this drama we feel like William has not been his normal perky, smiley, self. He tires more easily too. I asked Dr. Schultz if there is a correlation between low neutrophil counts and lower energy levels. She said, “Absolutely!” I just feel like we’re missing out on a little piece of the boy inside. It makes remember how precious every smile, every giggle, and every moment he acts like crazy man are!

We will celebrate like mad this weekend as our sweet little boy turns 1!

Love,
The Moeller’s and our Little Monkey