Update #7 (Jeeze, 7 already!)

As the doctor put it at our last visit, "be ready to spend the whole day at the hospital"...turns out we did. Our appointment was at 11:00, and after getting checked in we got settled into our exam room. At about 11:45 one of the nurses, Tia, came in and put numbing (aka Magic) cream on the tops of his hands and also on the inside of his elbows (aka the antecubital, or so my brainiac wife tells me). Shortly after, the doctor stopped in to see how Jack was doing, and to check if we had gotten our tests done. Nat had, but the labs had lost my slip, so we didn't have the results yet. The doctor said he would take a look at Nat's bloodwork, but stated that his "money is still on Dad being the one to pass down the vWB"...Thanks Doc.

A half a hour later or so they came in to draw some blood to run his baseline lab values before the actual medication challenge. But before they got started, a "child health specialist" came in to "coach" us all about how to minimize the trauma of what Jack was going to go through today. We decided to give her suggestions a try, mainly to distract him by turning on movies and making sure he doesn't pay attention to what the nurses were doing. She was adamant that I hold him against me and keep him from looking at the procedure. At one point he turned to look and she literally put her hands over his eyes.....needless to say that was the worst of the draws he went through today. Not only was there this crazy lady thinking that if she turned up the "big bird movie" loud enough, Jack wouldn't notice the needles stabbing him, but apparently the nurses couldn't get the needle positioned well in his hand and he ended up bleeding all over the nurses, on the floor, on me. After about 10 minutes of keeping Jack smooshed into me to stay still while "gushing" they finally called it quits. They had gotten all but one of the vials of blood that they needed, but said that they would come back later for the last one when they were putting in the iv for the DDAVP challenge. We got Jack calmed down and he quickly went to sleep in Nat's arms. We took turns holding him, both feeling bad for what he had just gone through, but of course upon waking up he was back to himself and was jamming to some ipod music when it was time to start the medication.

This time we kept the distractions to a minimum and he did much better, and he seemed to calm down after being able to see what was going on. After placing the iv line, they started him on a 30 minute DDAVP drip. This is the medication that activates the von Willibrand proteins that are stored in the lining of the blood vessels, ideally bringing his levels to normal for a short period of time. They were looking for his levels to triple after giving the med, and if this occurred then they mentioned that we would be able to keep a supply of DDAVP at home, as a nasal spay, in case of injury.

Nat took a break at this point to run down and get us all some lunch, just as she was getting back, Jack's iv pump started ringing. They checked all the lines, and nothing was wrong with the iv itself, but was more likely due to the pump machine. They replaced it and Jack was able to finish the med dose. After disconnecting the iv lines the nurse decided to leave the catheter and a short tube in Jack's hand in case they needed to draw blood from it later on.

Then it was time to play. We had to wait an hour for the med to take effect so the nurse showed us to a play room, and got Jack a tricycle to ride up and down the hallway that Nat or I could stand behind and push. I'm pretty sure that as soon as he saw "ME BIKE!" as Jack puts it, he completely forgot about all that had happened earlier in the day. He rode that bike all over that place, then we "raced," some people up and down the hall, then we rode up and down the elevators some more, with a pit stop to get a shiny "Choo Choo" and some "EEMMMeeeesss" (M&M's). Nat and I took turns going with him and before we knew it the hour was up.

The team came back in and this time I just sat him on my lap and he watched the whole thing as they drew his final, post med labs. Jack shuttered a little when they placed the needle but other than that barely made a sound, and just watched the nurses do their work...what a good boy.

Then it was back to waiting, we thought for the results, apparently the labs need a few weeks, but they did want to check his potassium levels before they could discharge him, so we had about another hour to kill.

We went down to the playroom again and played some more, and soon Jack heard a real life "Choo choo" out the window and we got to watch is roll by outside, and I'm pretty sure that made his day. Nat had gone back to our room just before the nurse Tia came in. She checked on us and then decided to take the IV needle out of his hand. It came out with ease and she quickly bandaged it up, and then went to wash off his fingers. We both looked down just in time to see more "gushing" of blood out of the iv site that she had just put a band aid on. Tia quickly got it cleaned up and then with an anxious laugh said "What, does this kid have a clotting problem or something?" The next bandage came with a big lump of gauze underneath to keep more pressure on the wound, and it did fine. Another trip on the elevators and then we were given the "Okay" to leave. It was nearly 5:00 at this point and we were all relieved to be done for the day.

Unfortunately, they didn't get Jack's labs back that same day, and still needed the results of tests on Nat and I, so we'll be going back to see the doctor again in three weeks. Hopefully that will put this chapter of our lives to rest.

Before we could go home though, we had one more stop. I (Larry) had to stop at Labcorp to get my vWB test done. Luckily this was pretty quick, and 18 vials of blood later we were on the road home.

Here is the before picture, we were waiting forever and Jack was playing nicely under the exam table with his trains.

Here Jackson is trying to show us his Emla or "Magic" Cream as they called it.

Zonked out after the first lab draw experience.

Waiting for the IV med to go in and watching Thomas.

Riding the halls!

This was him being silly while we waited to be released. He was cracking us up as usual.

Update #6

We were hoping that this would be our last update on the subject of von Willebrand but it seems that we have a few more labs and tests to do. Our appointment today was with Dr. Torkelson, the hematologist, who confirmed the Type 1 vWB diagnosis. He said that he has had two other cases where the Type 1 diagnosis was made because the child had ITP (a low platelet count of unknown origin) too, which led to vWB factor to be measured. He still is not sure that Jack had/has ITP so he will do a CBC next week to check Jackson's platelet level again. If his platelet count is back to normal, then we'll know he had ITP but if it is still low we will have to keep looking to figure out why.

The doctor then talked about the treatments for von Willebrand, and said that Jackson will not be restricted in any way physically. It is recommended that we not give him Motrin, which is a bummer since it works so well for him, but hopefully since he has the ear tubes we will not have such trouble with high fevers. The only treatments that Jackson will have to worry about are if he has to have surgery or gets into a major accident. There are two options for him at this point, he either can get something called Factor 8 which is a blood product that will help him with clotting. This has it's own risks, however, since it is harvested from donor blood. So, instead, the doctors like to use a synthetic hormone called DDAVP. This medication will actually help pull von Willebrand factor out of the cell lining of his blood vessels and increase his blood volume of von Willebrand, normalizing his ability to clot for 24-48 hours.

On Tuesday the 16th, Dr. Torkelson has ordered for Jack to have an IV dose of the DDAVP, with labs drawn before and after to make sure that he responds to the medication. (They will also be getting his CBC at this time). If he responds, then we know that he can have this med before surgery and after a major trauma if he were to ever need it (hopefully not).

The other labs that will be drawn are on Larry and I, which I have to have my O.B. order since Dr. Torkelson said that insurance companies don't like to pay out when pediatric doctors order labs on adults. Hopefully I can get my other lab work that has been ordered for me at the same time for the baby. I'm sure these labs will take another 2 1/2 weeks or so to get these results so I hope to get them done soon. To give some indication of how mild this vWB thing is, it is likely that Larry or I also have this issue, but neither of us have had any problems with it. The other possibility is that a genetic mutation occurred when Jack was developing in utero that caused this issue to develop...if this is the case, please refrain from calling him 'the mutant,' or 'mutant baby' or 'the little mutant boy,' etc. ;-)

Update #5

I went to the lab today and got the results for the final blood test. It took about a week longer to get than the lab had first estimated it would take but the news is finally in that Jackson has Type 1 von Willebrand disease. We don't see the hematologist until Monday because of the lab delay but we hope to find out more at that appointment and then probably be sent off to the lab ourselves to get tested. We are so happy that he has the mildest form and that we now have an answer. I'm also grateful that we didn't find out after a major surgery or trauma. We now know that he may need to get lab work and/or medicine to boost his levels before he has surgery. We also know that we need to tell people that we don't abuse our little man and that he is an easy bruiser...please don't be calling CPS on us. The boy has never been spanked, beaten, pinched, etc...that we know of. But let me know if you hear of anyone doing that to him and I will take that person down!!