Sclerotherapy Procedure #1

Today is a day that JT and I have been anxiously anticipating. We have been ready to get this process started so that we would have an idea of how it would help Will and how quickly we could move forward. I'll get to the long story of all of it for the rest of this post, but for anyone that wants a quick answer...it didn't work. We are pretty upset, but we know that this just marks a possible treatment off of the list and we will continue looking for alternatives.

We rolled Will out of bed this morning and left the house at 5:30 to be at Vandy by 6:00. He kept asking if he was going to get a shot, and I was happy to tell him that he was not (not that he would be awake for anyway). He wasn't too excited, but he got in the car with Woof-Woof and Whiskers without too much of a fight. We got there a little early and went to see the trains - Will's favorite! Then we headed up to the dreaded 3rd floor. (JT and I say this our least favorite place at Vandy. We don't ever seem to get great news here.) He was in a great mood through check-in and pre-op. The nurses and surgery team were are wonderful as always and Will seemed comfortable with them. Dr. Barron came and met with us and explained the procedure again. He was going into Will's lower back to hopefully find some veins that could clot off. His plan was to go slow and just see what he could do. He didn't want to do too much at once since the goal is to help Will, not hurt him. He also didn't want to be too close to the spinal cord. It would take about an hour he said. Will was very brave and drove himself and Whiskers to his operating room (no tears!).

Checking out his car that the nurse brought him.

Off they go to the OR! Whiskers had the perfect spot to ride with him. 

JT and I went to get some breakfast and then headed back. We stopped by the gift shop so JT could get Will "baby Woof-Woof" that he has been wanting. Right as we were getting in the elevator, they called to say Dr. Barron was ready to meet with us. He came in and said that Will was doing great, but he wasn't able to do anything to help him. I think it is safe to say that we both felt punched in the stomach right then. So the explanation was that the vessels on his back/buttock are lymphatic, not venous. You can't clot lymphatic vessels. He went to several different sites, and the result was always the same. These different types of vessels apparently couldn't be differentiated on the MRI.

Baby Woof-Woof

We got to see Will about 30 minutes later. I almost passed him by because he wasn't crying hysterically as with all of his other post-ops. He was sipping on his Sprite asking to take his IV out. We gave him Baby Woof-Woof and he was excited about that. We got him dressed and spoke with Dr. Barron again. We asked if we could go ahead and plan a procedure for the leg since it is obviously a vein, but he didn't seem like he was ready to do that. Will is "complicated" in his mind, so I think he probably isn't ready to attempt to take on Will again until we talk to Cincinnati.

I think that I am so frustrated because I have put Will though a lot since the end of December. We have been to Vandy almost every week for an appointment, lab work, consultations, etc. (I have a post about all of this that I will try to get on here in the next day or so.) I am just trying to look at it as information that we already have to continue moving forward with a new plan.

Thank you for the thoughts and prayers! As always, Will is fine. He was playing normal as soon as we got back home.

"Am I going to get a shot?" -Will

Poor Will, he asks this question every time we go to the doctor now. By shot, he means blood draws. A shot would be much less traumatic!

The info that I am about to list is more for my knowledge and everyone may want to skip over. I need all of this documented so that I can always remember what was happening as we move along through this process.

At the end of December, Will had lab work to check his hemoglobin and hematocrit to make sure that he isn't anemic from blood loss. He has urethral bleeding so we often see blood in his underwear and he has had rectal bleeding at times. The doctor went ahead and did a full lab work up on him just to have some baseline info. This showed that he was not anemic, but his D-dimer was high along with C-Reactive Protein. The D-dimer means that he is at a higher propensity to clot, and it may also be elevated in the presence of a clot. C-Reactive Protein is a marker of inflammation. When Dr. Snyder and I discussed these labs, we thought these might be high because Will may have had an active clot going on - we had thought this was happening in his scrotum or rectal area since he was in such severe pain over Christmas. With a high D-dimer, Will was going to have to use Lovenox injections (blood thinner) before and after sclerotherapy procedures.

At the beginning of January, Will and I went back to Dr. Snyder's office for Lovenox injection training. I give shots almost daily at work, but the thought of giving it to my child makes me panic! These would have to be done every day for 7 days before procedure and 7 days after procedure. Dr. Snyder wasn't really sure she wanted to do this since he bleeds a little anyway, so we decided to do the lab work again. The D-dimer this time was still elevated but improved. We decided not to do the shots for this first round of sclerotherapy since he was getting closer to normal and risk of bleeding vs. benefit couldn't really be justified. Yay for me (and Will)!

With that round of lab work, Dr. Snyder noticed that his aPTT has been elevated at all of the lab draws so far. This led to further investigation. Her explanation was that he clots slowly in the test tube and she needs to know if he actually does that in the body. Back to the lab for us and this time they and to stick him 2 times because they had trouble getting his blood. He wasn't happy, but he left with 4 band-aids, and even Whiskers got 1! They mixed his blood with some "normal" blood and re-ran the test. The results that time were normal so this told us that Will has an issue with a clotting factor. But they didn't have enough blood to run the tests to find out which clotting factor.

Back to the office for more blood work - this time he actually refused to go. I bribed him with everything that I could think of and finally after lots of "no's" and tears (from both of us), I finally convinced him with a Disney app that he has wanted on the iPad. The results were that he is deficient in Factor XI. If you want to be deficient in a factor, this one is a good choice - easily fixed during procedures with some plasma if needed.

The nurses in the lab know him now. They do an amazing job! They are so kind and so comforting. Will's favorite person is Katie. She is a Child Life Specialist in the Cancer Center and she certainly has a gift! (Dr. Snyder is an oncologist, so that is why we go to the Cancer Center - no concerns for Will on that part!) She tags along with us and she and Will play games on her iPad. He screams and cries when the needle sticks, but then he just cries while playing whatever game they are doing. And the tears are dried up by the time he is picking out his band-aid colors. He is such a trooper!

Update

2 things:

What I think is important:
Will's first procedure was rescheduled to Feb. 2nd. This gave us time for more blood work to be done. He seems to clot slowly (in the test tube), so there were more tests done to see if it is a clotting factor deficiency or no big deal. I am hoping to hear back in the next day or so.

What Will thinks is important:
Whiskers the cat has been renamed Figaro, depending on the day. (Minnie Mouse has a cat that looks just like his named "Figaro".) So if you actually get to meet the cat that makes him brave, don't be surprised if Will changes its name in the middle of your conversation.

Whiskers

For those of you that don't know about Woof-Woof, this was given to Will when he was in the hospital by JT's boss. It is Will's best friend and companion. Woof-Woof has a major limitation though, he is as big as Will!

Therefore, I have never allowed Woof-Woof to be taken in anywhere (he can ride in the car, but must stay there). When Will is hurt, sad, happy, playing, sleeping, etc Woof-Woof is always by his side. With everything that Will is about to go through, I thought he needed his best buddy to tag along with him to keep him comforted. Woof-Woof is too big though, so I had a plan. I let Will pick out a "brave toy" to be a substitute for when Woof-Woof can't be there.

So everybody, meet Whiskers the kitty-cat.

I let him pick it out at the hospital gift shop at one of his last appointments. We go in there every time we have an appointment to say hi to Woof-Woof's family (3 sizes of them) - a "Woof-Woof", and "small Woof-Woof" and a "baby Woof" as Will calls them all. He always pets them and hugs on them. So my plan was to let him pick out something small that he could carry to all future appointments and procedures, because Woof-Woof is obviously way too big to be taken anywhere! We discussed why we were picking something out, and he immediately was set on "baby Woof". Fine by me, it is his choice on what he thinks will make him brave. Unfortunately they were out of stock, but Will found this cat and was instantly obsessed! He named it Whiskers and we took it with us to our blood work appointment that day. I can't say that it made him brave (since I had to hold him down along with another nurse), but it sure did make him happy telling anyone who would listen about his new friend. I wasn't sure how Whiskers would fit into our daily life once we got back to the car to Woof-Woof, but Will happily exclaimed that "Woof-Woof always wanted a kitty-cat", so it was love at first sight. Thank goodness! Whiskers, Woof-Woof, and Will are now a trio around the house - they do everything together!

A Plan for Will

JT and I had decided before Thanksgiving that we needed a second opinion on Will's vascular malformation. He wasn't really having any problems except that we could just tell that the growth was more and more obvious. Online research led us to Children's Hospitals of Cincinnati and Boston.  I called the pediatrician and he immediately started our referral to Cincinnati. Just a few hours later, we noticed blood in Will's bowel movements. That night, the right side of his scrotum looked red and inflamed and it was hurting him. Then he started limping on his right leg, especially after sitting for a while or getting up in the morning. The limp would get better throughout the day as he seemed to work through it. He doesn't sit well on his bottom ( and really he never has). This was something that I ignored because how can you tell that isn't just a 2-3 year old that doesn't want to sit still? His teachers and Hope have brought it up again - he usually ends up sitting on his knees if he is in a hard chair. This led to some frantic calls to Vanderbilt specialists that we have been seeing for some appointments.

The urologist thinks maybe he has hemangiomas in his genital and rectal area that get aggravated. This causes blood when he pees. As far as the pain and inflammation of scrotum, he probably was having come clotting in there. It got better within just a few days.

The general surgeon (Dr. Neblett) that we have been keeping up with was extremely thorough and got more people involved. We did blood work to make sure that he wasn't losing too much blood (everything was fine). I told him that we had contacted Cincinnati and were looking to go there. He understood and said it was definitely a place that he would recommend. I guess I was afraid to offend anyone by asking for a second opinion, but at the end of the day my first priority is Will, and everyone is on the same page and just wants to help Will. He set aside the rest of his morning to bring in a team of specialists to review all of Will's files to see if he could get us a plan at Vanderbilt so that we don't have to travel.

I finally got a call from Cincinnati to get the process started. The girl that I have been in contact with has been so helpful. After a few conversations, she asked me if I knew that Vanderbilt now had a hemangioma and vascular malformations team like they did. No, obviously I did not. She said that it was very new and probably connected to the regular hospital (instead of Children's Hospital), so many of the doctors probably weren't even aware of it. She gave me a name of Dr. Snyder that was part of the Vanderbilt team. Dr. Snyder had been at Cincinnati and remains in close contact with the doctors that we would be seeing there. I called Dr. Neblett and asked him about it, but he didn't really know much about this vascular team. He immediately got in touch with Dr. Snyder and had a consultation appointment set up for us 3 days later. Dr. Neblett spent time with JT and I explaining what he, Dr. Snyder, and Dr. Baron (radiologist) had discussed and a possible plan. He is letting Dr. Snyder take over here as far as planning procedures.

We met with Dr. Snyder, and it was like a breath of fresh air! I can't even begin to explain the relief of finding someone that has actually seen this before! See is a hematologist/oncologist, but will kind of be heading up all of the procedures and watching Will's blood work. An interventional radiologist will actually be performing the procedures. He also trained at Cincinnati.

The procedures are called sclerotherapy. (I'll post a link that you can get more information from.) These will be outpatient procedures, but Will is going to be under anesthesia. What I understand about this procedure in the simplest form is that a catheter is inserted in the vein, something is injected to see how the blood flows, and if the vein isn't crucial, another solution will be injected to clot off and shrink the vein. This will probably be painful for Will for a few days after while that clot is forming. He will be given pain medication if needed. This is not going to be taken care of all in 1 procedure. There will be multiple procedures, so JT and I think this is going to take at least a year. Our goal is to be done in time for him to start kindergarten.

These 2 websites get you to the main pages of vascular malformations. You can read about them and look at treatment tabs for more info. Will has a combined malformation of venous and lymphatic.

http://www.cincinnatichildrens.org/service/h/hemangioma/default/

http://www.childrenshospital.org/health-topics/conditions/v/vascular-malformations-tumors-and-hemangiomas

I am meeting with Dr. Snyder tomorrow to get more information. The first procedure is scheduled for Jan. 26th.

We are going to meet with the Cincinnati team March 10th. They are really good about making it possible to see all of the specialists at one time so we don't have to make multiple trips. That however puts us to March before we can do that. We decided to go ahead and get started with Vanderbilt because we don't want to be 6 months behind since this is going to be a lengthy process already.

Please keep Will in your prayers. He isn't in pain the majority of the time and hardly ever complains. He is a typical crazy 3 year old that runs and jumps around, plays soccer, plays with his baby sister and dog, annoys his baby sister and dog, and makes us smile all of the time! I'm going to keep this blog updated as much as possible so everyone can stay informed that wants to be.

Why JT Will Never Be a Stay-At-Home-Dad...

There are lots of reasons why JT won't be a Stay-At-Home-Dad, but today ensured that his sanity couldn't handle it. For one of the few times in Nashville history, the city was mostly shut down. The roads were so bad, that it was recommended that no one get out on them because of the ice. Guess who wasn't closed...Publix! I will admit that my boss was kind of enough to let me work at the store closest to my house, so I didn't have to travel far. Hope obviously couldn't get to us by 9:00, so JT decided to stay home with the kids until the roads cleared and then he would go to work. The roads never really cleared, and his business ended up closing for the day, so he got an entire 13 hour day with Will, Evie, and Maggie. Oh boy! (He does keep the kids every other weekend when I work with NeeNee and Gram's help, but I had not worked any weekends in January and February, so this was a first for him.)

By 9:15 A.M., Hope and I both received this picture...

Signed by "Will, EB, and Maggie's paw print"

I checked in on them a few times throughout the day, and of course everyone was doing fine. Then around 5:00 P.M., I got this. Here is what I noticed...

1. They have changed clothes (just to more pj's).
2. Will is wearing JT's visor.
3. Evie is wearing JT's hat and looks like a rapper/gangsta.
4. Evie is wearing Will's "Planes" t-shirt. I'm surprised he didn't knock her out of that bumbo when he noticed!
5. Will is having one of his favorite snacks.

I think they had a great day together! It is tough trying to keep up with these two, especially when you are trapped in the house all day! At least I try to go somewhere with them for a little change of scenery. Speaking of scenery, there was NO snow on the ground. Ice only. Will and JT didn't get to even enjoy their "snow day" outside playing.

The next morning when Hope pulled up, JT announced very excitedly, "The angel has arrived!" He couldn't wait to go back to work to his easy job! I'm not going to lie, I feel the same way most days. There is no job harder than being a Stay-At-Home-Parent!'

Good job JT!

Stitches for Will

First official ER visit for Will ended with 4 stitches above his left eye.

Will was going through his normal bedtime routine of reading 2 books when he started running around his room in circles (normal for him). He fell and hit his forehead above his left eye on the end of his bed. I was cleaning up the playroom and heard the panic in JT's voice. Will was bleeding a lot and I was trying to get him cleaned up so I could see how deep it was. It was really hard to tell though, and Will didn't want us to touch it any more than we had too. He calmed down pretty quickly and JT tested his eye movements, so we weren't really concerned about a concussion. JT called Dr. Scott and he said he would go to the ER since it might not heal well on its own.

Evie was asleep, but we decided to take her with us. We both wanted to be with Will so we loaded up the car for a Saturday night adventure at the ER. I talked to Will the entire car ride to keep him awake and make sure he wasn't acting different. He was being funny and smart as usual.

We got there and got in right away. Will didn't like getting his temperature taken, but he did like the scale. Will (plus 2 airplane toys) weighed 35 pounds. Next we went back to a room where a few nurses took turns talking to Will and keeping him happy. Besides a bleeding head, you'd never have known there was anything wrong. They put some numbing gel on his head. We had to wait 30 minutes for it to be effective, so Will and I laid in the bed and played with the airplanes, counted numbers on the clock, and played with the bandage tape.

"Lil' Maggie" was injured too. 

Once he was numb, JT helped hold him down so they could clean it out and get a good look at it. 4 stitches were put in and covered with an Elmo band-aid. They offered him a popsicle (which he refused) and gave him a teddy bear.

Will tells everyone that he got "4 stitches", and he usually holds up the correct number of fingers. 

Later in the week, we were able to remove the stitches. Will's pediatrician said that I could do it at home (he apparently has a lot of faith my surgical ability), but Will wouldn't let me (he doesn't trust me). I loaded up Will and Evie and back to the ER we went. They remove the stitches for free, so I decided it would probably be best to leave it to the professionals. He was a complete angel for them! He just laid there and let them take them out like it was no big deal. I think really he was just impressed with the magnifying glasses that the nurse was wearing. This time when they offered him the popsicle, I gladly accepted it for myself!

He climbed right up on the bed like he knew what he was doing. 

So we have had our first trip to the ER. I highly doubt it will be the last.