About a year ago I realized that I probably needed to start paying more attention to songs that I listen to in the car because Will remembered everything and would repeat. This hasn't gotten me in to trouble yet, but I know the day is coming! We were listening to "Roar" by Katy Perry in the car a few months ago, and a couple of days later Will asked me, "Why does that girl dance in the fire?". It took me a few minutes to figure out what he was talking about. I explained to him that it meant she was brave and strong and could get through anything.
JT and I had spent so many hours trying to assure Will that doctors our are friends, shots don't hurt, there is nothing to be scared of, etc. When I watch him at office visits now, it is simply incredible to watch how brave he is. It also makes me very sad that he is so good at these appointments. It isn't fair to him; he should not be this experienced. He can walk a nurse through a vitals check with the greatest of ease. We now get blood drawn every few weeks at the pediatrician's office. He lets out a scream when he sees the needle, and then that is it. No more screaming, no fighting, no tears.
"Roar" has become one of Evie's favorite songs now so we play it a lot. I fight back tears when I hear Will sing along. He often stops when she says "dancing through the fire" and tells me that he is "brave, strong, and I can get through anything". I know that there are so many worse things that we could be dealing with, but this is our fire and it consumes us.
Recent news:
We went to Cincinnati the week of Christmas to get an updated MRI, complete the neurogenic bladder test, and see Dr. Adams and team.
MRI:
The MRI was really tough. Cincinnati does things a little different than Vanderbilt. Instead of taking him from us while he was awake to give him gas to put him to sleep before they insert IV, they suggested that we be in the room with him. Will has never liked the gas mask (he calls it "the circle"), and he fought us as soon as he realized what we were doing. I'm not going to elaborate, but I'll say that it was a terrible experience for all three of us. We told them that we would not be a part of that step again. Now I'm hoping that he can skip the gas in the future and just go straight for an IV. He does well with blood draws, so I don't see any reason to torture him with the gas in the future. The MRI result showed that nothing had changed. The spinal cord is still tethered to part of the malformation. Surgery is really the only option we have to make sure that he doesn't have issues with bowel, bladder, and leg weakness in the future.
Bladder test:
When I was first told that we needed to get this, it wasn't really explained in detail about what actually was involved in the procedure. So I messaged Dr. Thomas at Vanderbilt and asked him to set it up for me. He called me soon after and asked if I knew what this test was. He has had enough experience with Will to know that this wasn't going to be easy or fun. It involved Will being awake, a rectal tube, and a urethral catheter. Did I mention he had to be awake?!?! I called Cincinnati and got some information that the team there did these daily and has excellent child life specialists to help. I decided to schedule it there since we were going for an MRI anyway, and I'm pretty sure Dr. Thomas wasn't sad about that! So we had it done the day after the MRI. The nurses were amazing! Truly wonderful! I had worried and worried about this procedure and the fight Will would put up, but he got through it like a champ! He didn't like it and will be very unhappy when we do it again later this year, but I can honestly say that it went very well. He got to pick a DVD to watch/distract him. He was given 2 squishy stress balls which he somehow knew exactly how to use. He would squeeze them when he was upset and take big deep breaths. Once again JT and I witnessed the strength and bravery of a 4 year old that is well beyond anything we could be ourselves.
Clinic visit:
The visit always starts out with a photographer coming in to take pics of the malformation. It is always the same guy and he was talking to Will about sports. He came back with a really nice basketball net that hangs over the door. Note to self: going to the hospital the week of Christmas has some fun benefits! Dr. Adams and Dr. Dasgupta came in and just did a routine visit. Will ended up in Dr. Dasgupta's lap at one point - she has such a great demeanor with him and he is so comfortable with her. Dr. Dasgupta left the room and Dr. Adams said she needed to tell us something about herself. My heart broke - I knew what was coming. It was the same tone and look that Dr. Snyder had when she told me she was leaving Vanderbilt just 6 months before. I still haven't recovered from that; it was like losing my right hand. So as expected, she said she was moving to Boston. I have officially now lost my left hand too. JT instantly said, "OK, we'll come to Boston". I mean, there is no question...we follow her! She has saved Will in our minds. She is behind the research and she KNOWS how to help him. There are very few people in the world that KNOW how to help him and we will travel to the ends of the earth to continue with them. I fought back my selfish tears (kind of) and kept telling myself that it wasn't about me. It was about her and her need to be near her family. What a great opportunity for her! I did good for a while, and I then I realized it wasn't about me or her, it is about Will. And we NEED her! She assured us that there is no need to come to Boston. She wouldn't be leaving the clinic and her patients if they weren't going to be taken care of. She has taught and trained all of them and they will continue on. We love Dr. Dasgupta and have a lot of faith in Dr. Patel when we need him in the future. We will stay with Cincinnati for now. Dr. Adams will still be communicating with them and we can always reach out to her when we want to.
Overall, it was an emotionally exhausting visit. We left with a plan. We wanted to get the tethered cord surgery scheduled as soon as possible, because we wanted it done while Dr. Adams was still there. Not that she is performing the surgery, but it would be comforting to know that she would be checking on him. Unfortunately the timing didn't work out. She is leaving in February and surgery is in March. I am comfortable with the team that remains and know that we are in good hands.
Keep praying for this strong and brave kid! He is about to go through so much, but I have no doubt that with the combination of his strength and the power of prayer, he'll overcome this obstacle and all others put in front of him!
Wednesday, December 30, 2015
Sunday, September 13, 2015
Evie is 2!!!
Our sweet lil' bit is 2! My goodness time has flown by. She is cute, sweet, and funny. She also is wild and demanding. She adds just enough chaos to our family to make my head spin. But when you look into those beautiful blue eyes, all I see is love.
We had a big birthday party with family and some friends. Evie had a wonderful day and loved the attention. She also loved ice cream and presents!
We had a big birthday party with family and some friends. Evie had a wonderful day and loved the attention. She also loved ice cream and presents!
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| 4 generations |
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| Not sure about a bow on her head, so we attached it to her back. New fashion statement? |
Friday, July 10, 2015
Procedures 6/29/15
Will had 2 procedures at the end of June. Luckily Dr. Baron and Dr. Thomas coordinated to do these together to make it easier on Will.
Back to the dreaded 3rd floor at Vandy where JT and I have never been given good news. Makes my stomach turn just thinking about it! As always, everyone was great! Will loved his pre-op nurses and instructed them on what they needed to do - height, weight, give arm a hug (blood pressure), temperature, etc. JT doesn't go to all of the lab appointments with me so I think he was even surprised by how well Will knew the routine. The nurse brought him some washable markers and cars and told him he could draw racetracks on his sheets. Yep, markers on sheets - this is going to go over really well once we get home! He had a blast! He didn't want to go back to the OR and put up a little fight, but of course it all worked out, just involved some sobbing and refusals.
Venogram - This was requested by Cincinnati. They are looking at the vein structure in his right leg and foot. They wanted to see his deep vein system and look for dilations in his large vein. Basically we wanted to see if the large vein was not important so we could eventually do an ablation of it. I do not understand the details of what was found, but I think the large vein probably has to stay. We will get a better explanation when we got to Cincinnati in October.
Cystoscope - On the MRI in May, a spot was found on the bladder wall which was thought to be part of the malformation. Dr. Thomas did a scope through urethra up to the bladder for a better idea. He found lots of inflammation and redness in the urethra. There isn't really anything that can be done about it. Hopefully the Rapamune is keeping it under control. The malformation is present through the bladder wall, but luckily just a small area. We discussed surgery to remove that small area and decided that was probably the best plan. Dr. Snyder had already mentioned this being an option when she originally spoke with Dr. Thomas before the procedure. None of these findings were a surprise to us.
I contacted Cincinnati and Dr. Adams wanted to talk with Dr. Thomas about the surgery. I had a feeling she wasn't comfortable with it, and sure enough the nurse called today to cancel it. We rescheduled another cystoscope in 3 months. I'm not sad about it - I don't want Will to have surgery, but I feel like we are just finding more and more things that we can't do anything about. We are going to be hopeful that the Rapamune is going to continue helping. Who knows, maybe the spot on bladder was much worse before we started the medicine and we are just seeing it now because we looked for the first time.
The saddest part of my day was that it was Dr. Snyder's last day. Will picked out a superhero card for her and even signed his name. We didn't get to see her but dropped it off at her clinic. She was such a great source of knowledge for me and I already miss that I don't have her to explain all of this to me. Now I am struggling with going back and forth between Vanderbilt and Cincinnati and making sure that they are communicating (which they are, but Dr. Snyder made it easier).
Back to the dreaded 3rd floor at Vandy where JT and I have never been given good news. Makes my stomach turn just thinking about it! As always, everyone was great! Will loved his pre-op nurses and instructed them on what they needed to do - height, weight, give arm a hug (blood pressure), temperature, etc. JT doesn't go to all of the lab appointments with me so I think he was even surprised by how well Will knew the routine. The nurse brought him some washable markers and cars and told him he could draw racetracks on his sheets. Yep, markers on sheets - this is going to go over really well once we get home! He had a blast! He didn't want to go back to the OR and put up a little fight, but of course it all worked out, just involved some sobbing and refusals.
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| They drew a race track for the cars. |
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| On your mark, get set, go! |
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| Cruising around |
Venogram - This was requested by Cincinnati. They are looking at the vein structure in his right leg and foot. They wanted to see his deep vein system and look for dilations in his large vein. Basically we wanted to see if the large vein was not important so we could eventually do an ablation of it. I do not understand the details of what was found, but I think the large vein probably has to stay. We will get a better explanation when we got to Cincinnati in October.
Cystoscope - On the MRI in May, a spot was found on the bladder wall which was thought to be part of the malformation. Dr. Thomas did a scope through urethra up to the bladder for a better idea. He found lots of inflammation and redness in the urethra. There isn't really anything that can be done about it. Hopefully the Rapamune is keeping it under control. The malformation is present through the bladder wall, but luckily just a small area. We discussed surgery to remove that small area and decided that was probably the best plan. Dr. Snyder had already mentioned this being an option when she originally spoke with Dr. Thomas before the procedure. None of these findings were a surprise to us.
I contacted Cincinnati and Dr. Adams wanted to talk with Dr. Thomas about the surgery. I had a feeling she wasn't comfortable with it, and sure enough the nurse called today to cancel it. We rescheduled another cystoscope in 3 months. I'm not sad about it - I don't want Will to have surgery, but I feel like we are just finding more and more things that we can't do anything about. We are going to be hopeful that the Rapamune is going to continue helping. Who knows, maybe the spot on bladder was much worse before we started the medicine and we are just seeing it now because we looked for the first time.
The saddest part of my day was that it was Dr. Snyder's last day. Will picked out a superhero card for her and even signed his name. We didn't get to see her but dropped it off at her clinic. She was such a great source of knowledge for me and I already miss that I don't have her to explain all of this to me. Now I am struggling with going back and forth between Vanderbilt and Cincinnati and making sure that they are communicating (which they are, but Dr. Snyder made it easier).
Wednesday, June 24, 2015
Evie singing
I have definitely neglected Evie on this post. I'm terrible! She is going to grow up and ask me all about her milestones, and I'm going to admit that I have no clue. Sorry Evie, that is what happens when you are the second child!
She is starting to talk a lot and loves to sing. Here is the first time we have been able to record her. This is Evie's version of "Twinkle, Twinkle, Little Star". And oh my goodness it is hilarious and so adorable!
So I PROMISE I will have an actual Evie update posted soon. I just had to make sure the world gets to enjoy this little show for now.
She is starting to talk a lot and loves to sing. Here is the first time we have been able to record her. This is Evie's version of "Twinkle, Twinkle, Little Star". And oh my goodness it is hilarious and so adorable!
So I PROMISE I will have an actual Evie update posted soon. I just had to make sure the world gets to enjoy this little show for now.
Friday, May 8, 2015
Rapamune update
So Will has been taking the Rapamune for about 6 weeks now. The first week was a nightmare! Karma has given me a picky child meaning that getting this medicine down him is impossible! It has to be given in water or OJ, not anything else (according to the package insert and the drug company). I first attempted in water, because he likes water. I tried it first and it was terrible! I knew there was no way he was going to take it. And of course, he didn't. There was a lot of begging, crying, and arguing, and I decided day 1 was a failure. The next few days didn't go much better. I brought home some OJ, but of course he said he didn't like OJ so he wasn't going to do that. I tried to explain that this was something that he just had to do, but have you ever tried to reason with a 4 year old? A very stubborn, smarter than he should be 4 year old? It is exhausting!
So I made a post on the Cincinnati Facebook page looking for guidance. Water, OJ, and some even said that gave it straight out of the bottle with no dilution. (The medicine is about 3% ethanol, so undiluted is horrible.) I finally resorted to putting it in his chocolate milk. The drug company is cringing as I type that! That told me over and over again that water and OJ were the only ways, anything else was off-label. Well guess what, the reason we are using it is off-label! So I'm going to do my own thing to get it in him. I even explored the options of tablets, but I don't think he can understand the concept of swallowing a pill. (We've talked about it and shown him, but he isn't willing.) Dr. Snyder said consistency is all that matters, so we do it the same way each day. It is working. He knows it is in there, but he also knows that it is helping, so he can't argue too much.
So I just said it was helping - that's right, it is helping so much! And just in a matter of weeks! The hard bulge on his calf is soft, he doesn't limp, no waking up crying that his leg hurts, and less bleeding in the bathroom. I want to shout from the rooftops how amazing this has been! We didn't think the results would be so fast or so obvious, so we are very pleased!
We haven't found the perfect dose for him yet, so he goes every 2 weeks to have blood levels drawn. He is so brave! He cries when they draw blood (but I want to cry when I have blood drawn too), and then he hops up, grabs a sticker, and continues playing with his nurse friends. His nurses are amazing! They all remember him (and Whiskers) and are just truly special people.
I'm typing this as I am waiting for Will to get out of his MRI. With the kidney ultrasound done a few weeks ago, they found a spot on his bladder. Fingers crossed that it is just the malformation pressing on the bladder, but it had to be checked out. I won't know the results until next week.
So I made a post on the Cincinnati Facebook page looking for guidance. Water, OJ, and some even said that gave it straight out of the bottle with no dilution. (The medicine is about 3% ethanol, so undiluted is horrible.) I finally resorted to putting it in his chocolate milk. The drug company is cringing as I type that! That told me over and over again that water and OJ were the only ways, anything else was off-label. Well guess what, the reason we are using it is off-label! So I'm going to do my own thing to get it in him. I even explored the options of tablets, but I don't think he can understand the concept of swallowing a pill. (We've talked about it and shown him, but he isn't willing.) Dr. Snyder said consistency is all that matters, so we do it the same way each day. It is working. He knows it is in there, but he also knows that it is helping, so he can't argue too much.
So I just said it was helping - that's right, it is helping so much! And just in a matter of weeks! The hard bulge on his calf is soft, he doesn't limp, no waking up crying that his leg hurts, and less bleeding in the bathroom. I want to shout from the rooftops how amazing this has been! We didn't think the results would be so fast or so obvious, so we are very pleased!
We haven't found the perfect dose for him yet, so he goes every 2 weeks to have blood levels drawn. He is so brave! He cries when they draw blood (but I want to cry when I have blood drawn too), and then he hops up, grabs a sticker, and continues playing with his nurse friends. His nurses are amazing! They all remember him (and Whiskers) and are just truly special people.
I'm typing this as I am waiting for Will to get out of his MRI. With the kidney ultrasound done a few weeks ago, they found a spot on his bladder. Fingers crossed that it is just the malformation pressing on the bladder, but it had to be checked out. I won't know the results until next week.
Thursday, April 2, 2015
Cruisin'
It has been some beautiful weather here lately! So 3 years after getting the coolest birthday present ever, Will is finally driving his Escalade! And Evie loves being the passenger and controlling the radio.
Thursday, March 19, 2015
Cincinnati Children's Hospital - first visit
We finally had the appointment that we have been waiting for on 3/10, and it lived up to all that we had hoped for! We left with a plan for Will and a higher level of confidence than we have had so far.
It was an exhausting 2 days, but we tried to make it fun for Will. We turned it in to a mini-vacation for him. After dropping Maggie off at Paw Pals and Evie off at Gram's house, we took off to Louisville to stay Monday night. The hotel had all of the requirements to make Will happy - an elevator, a pool, and an escalator. Happy boy! Will and JT swam and played for a while before we went to bed. Tuesday morning we had breakfast and took our time getting ready. We headed off to Cincinnati. We made it to the hospital just in time to grab lunch and head to our appointment.
We met with several specialists all at once. They had the entire afternoon devoted to Will. It was so great! We were there for almost 3 hours and never once felt rushed. Will was a rockstar! I still can't believe how good he did. He let them examine him multiple times and he played with a nurse practitioner student while we were talking with the team. (I doubt that was supposed to be her role for the day, but Will immediately became friends with her. She has a son Will's age, so they had some serious discussions about Paw Patrol and other important things in Will's daily life!)
So we met with:
Dr. Adams - (medical director of this team) I believe she is a hematologist/oncologist. She had a special interest in this small population of kids with vascular malformations and started this team. The research she has done and knowledge about these kids is just unbelievable. She is truly a blessing!
Dr. Dasgupta - She is a pediatric general surgeon. We really loved her! She just had a way of explaining things that made us feel so at ease.
Dr. Patel - He is the interventional radiologist. Super knowledgable! He trained Dr. Baron (Vanderbilt) and I think we'll have a great line of communication between these two.
Dr. McCarthy - He is the orthopedic surgeon. We only met with him for a few minutes. His role really won't be needed until Will is about 10-12 years old. At that point we'll be deciding if Will's leg lengths differ enough to do something about it.
Nurse - I don't know her name, but she explained stuff to us at the end of the appointment that pretty much pieced it all together. She was great! (I have got to figure out her name!)
The Diagnosis: CLOVES syndrome (www.clovessyndrome.org)
Congenital - formed in utero
Lipomatous - fatty mass in his lower back/bottom
Overgrowth - I think this goes along with the fatty mass, but may also be his bigger right side in general.
Vascular Malformations - vascular and lymphatic
Epidermal - red/brown spot on his back
Skeletal/Scoliosis - Will doesn't have anything in this part that we know of.
The Plan:
Start Rapamycin - This is a medication used in patients that have had kidney transplants. It has been found to improve the symptoms and maybe even the severity of vascular malformations by slowing the progression of tumor cells. (Will doesn't have a tumor, but the malformations act like a tumor.) This is a liquid medication that Will is going to take twice daily. We won't see results right away, but I think it is a great start to at least feel like we are doing something. It does have several side effects, but I am most concerned about the immunosuppression (Will's immune system won't be as great as it is now) and increases in cholesterol. He will take Bactrim (antibiotic) on Saturdays and Sundays while on this medication as prophylaxis. Lab work will monitor cholesterol. Really though, he is going to be on a much lower dose of this medicine than is given to transplant patients, so hopefully the side effects will be minimal anyway. Dr. Snyder at Vanderbilt is taking care of this step, and we plan on beginning 3/25.
Compression Stocking - Will has already been measured for this and we're waiting for it to come in. Because Will is at a higher risk for a blood clot, he will wear this during the day. Think of it as a thigh-high sock. (We'll be calling it a "superhero sock" or "makes you run faster sock" or whatever we can to make it fun for him.) They are also making him some compression boxer briefs hoping that it will make him more comfortable sitting. I'm really wishing we had gotten this sock this during the winter because it is going to take some getting used to, especially when it gets hot outside.
Tethered Spinal Cord - We've known since Will's first MRI when he was about 18 months old that he has a tethered spinal cord. The bottom of your spinal cord should hang freely and move within the spinal canal. Will's is attached ("tethered") to the fatty mass/malformation. Luckily in his case, he has never had any neurologic symptoms because of this. The Vanderbilt neurosurgeon would like to untether the cord, but he isn't familiar with how to do it while avoiding the malformation. Cincinnati said that they have seen this and can do the surgery if needed. The neurosurgeon (which we did not see) is reviewing Will's past MRIs to decide if we need to move forward with this procedure. If so, we will do this before anything else. We will go back to Cincinnati to have this done.
Scope - Will needs to have a urethral and rectal scope done at some point to see what type of lesions are in those areas causing him to bleed. The urethral bleeding is a constant issue for him. This will be done at Vanderbilt. We don't have a GI doctor at either place yet, so we are working on that with Vanderbilt. For now he is taking Miralax daily to prevent constipation. I mix it in his morning milk so he has no idea - I wish all medicines were this easy!
Venogram - This is an x-ray that involves injecting contrast material (dye) into a vein to show how blood flows through the vein and what type of condition the vein is in. We need to know that Will's right leg has a deep vein system (which we are sure that is does) and that the big vein isn't vital for his leg. I believe this will be done at Vanderbilt by Dr. Baron. If everything looks as we hope, we will move on to the ablation procedure.
Ablation of Vein in Right Leg - If we get a good report that Will's deep vein system is good and the big vein is not needed, Dr. Patel in Cincinnati will do an ablation procedure. It was explained to us that he goes in with a laser and basically burns (cauterizes) his way down the entire vein to close it and make it no longer viable. This is very similar to a procedure for a varicose vein, except this one is much larger and Will is going to obviously need anesthesia. This seemed to be a new thing that is being done for vascular malformations. Dr. Patel explained that just doing sclerotherapy would never have really helped that leg vein since it is so large.
Other follow-ups -
Will is going to have a kidney ultrasound every 3 months until he is 8 years old to check for WILMS tumor. This is a cancer of the kidney that is sometimes found because of the "overgrowth syndrome". Very unlikely, but at least we are checking.
Orthopedics will begin following, but no surgery would be done until age 10-12. We will only have to do something if the right leg ends up being longer than the left leg.
So, that's the plan. JT and I are comfortable, relieved, and ready to get this going. Sweet Will left the appointment in Cincinnati saying "but my foot still hurts". I had been preparing him for the trip telling him that we were going to meet the doctors that could help him, so he was expecting some immediate remedy. Oh how we wish that were possible! But, we are just so happy to be moving forward.
As always, thanks for the prayers, love, and support!
If you want more information on anything in the plan, you can google most of it. Although if you are one of my patients, you know that I NEVER recommend looking up health info on the internet to patients! Hahaha!
It was an exhausting 2 days, but we tried to make it fun for Will. We turned it in to a mini-vacation for him. After dropping Maggie off at Paw Pals and Evie off at Gram's house, we took off to Louisville to stay Monday night. The hotel had all of the requirements to make Will happy - an elevator, a pool, and an escalator. Happy boy! Will and JT swam and played for a while before we went to bed. Tuesday morning we had breakfast and took our time getting ready. We headed off to Cincinnati. We made it to the hospital just in time to grab lunch and head to our appointment.
 |
| Pool time with Daddy |
We met with several specialists all at once. They had the entire afternoon devoted to Will. It was so great! We were there for almost 3 hours and never once felt rushed. Will was a rockstar! I still can't believe how good he did. He let them examine him multiple times and he played with a nurse practitioner student while we were talking with the team. (I doubt that was supposed to be her role for the day, but Will immediately became friends with her. She has a son Will's age, so they had some serious discussions about Paw Patrol and other important things in Will's daily life!)
 |
| Will didn't want to take a picture, but he did show off his brave friends Whiskers and Baby Woof. Yes Big Woof-Woof made the trip too, but he had to stay in the car. |
Dr. Adams - (medical director of this team) I believe she is a hematologist/oncologist. She had a special interest in this small population of kids with vascular malformations and started this team. The research she has done and knowledge about these kids is just unbelievable. She is truly a blessing!
Dr. Dasgupta - She is a pediatric general surgeon. We really loved her! She just had a way of explaining things that made us feel so at ease.
Dr. Patel - He is the interventional radiologist. Super knowledgable! He trained Dr. Baron (Vanderbilt) and I think we'll have a great line of communication between these two.
Dr. McCarthy - He is the orthopedic surgeon. We only met with him for a few minutes. His role really won't be needed until Will is about 10-12 years old. At that point we'll be deciding if Will's leg lengths differ enough to do something about it.
Nurse - I don't know her name, but she explained stuff to us at the end of the appointment that pretty much pieced it all together. She was great! (I have got to figure out her name!)
The Diagnosis: CLOVES syndrome (www.clovessyndrome.org)
Congenital - formed in utero
Lipomatous - fatty mass in his lower back/bottom
Overgrowth - I think this goes along with the fatty mass, but may also be his bigger right side in general.
Vascular Malformations - vascular and lymphatic
Epidermal - red/brown spot on his back
Skeletal/Scoliosis - Will doesn't have anything in this part that we know of.
The Plan:
Start Rapamycin - This is a medication used in patients that have had kidney transplants. It has been found to improve the symptoms and maybe even the severity of vascular malformations by slowing the progression of tumor cells. (Will doesn't have a tumor, but the malformations act like a tumor.) This is a liquid medication that Will is going to take twice daily. We won't see results right away, but I think it is a great start to at least feel like we are doing something. It does have several side effects, but I am most concerned about the immunosuppression (Will's immune system won't be as great as it is now) and increases in cholesterol. He will take Bactrim (antibiotic) on Saturdays and Sundays while on this medication as prophylaxis. Lab work will monitor cholesterol. Really though, he is going to be on a much lower dose of this medicine than is given to transplant patients, so hopefully the side effects will be minimal anyway. Dr. Snyder at Vanderbilt is taking care of this step, and we plan on beginning 3/25.
Compression Stocking - Will has already been measured for this and we're waiting for it to come in. Because Will is at a higher risk for a blood clot, he will wear this during the day. Think of it as a thigh-high sock. (We'll be calling it a "superhero sock" or "makes you run faster sock" or whatever we can to make it fun for him.) They are also making him some compression boxer briefs hoping that it will make him more comfortable sitting. I'm really wishing we had gotten this sock this during the winter because it is going to take some getting used to, especially when it gets hot outside.
Tethered Spinal Cord - We've known since Will's first MRI when he was about 18 months old that he has a tethered spinal cord. The bottom of your spinal cord should hang freely and move within the spinal canal. Will's is attached ("tethered") to the fatty mass/malformation. Luckily in his case, he has never had any neurologic symptoms because of this. The Vanderbilt neurosurgeon would like to untether the cord, but he isn't familiar with how to do it while avoiding the malformation. Cincinnati said that they have seen this and can do the surgery if needed. The neurosurgeon (which we did not see) is reviewing Will's past MRIs to decide if we need to move forward with this procedure. If so, we will do this before anything else. We will go back to Cincinnati to have this done.
Scope - Will needs to have a urethral and rectal scope done at some point to see what type of lesions are in those areas causing him to bleed. The urethral bleeding is a constant issue for him. This will be done at Vanderbilt. We don't have a GI doctor at either place yet, so we are working on that with Vanderbilt. For now he is taking Miralax daily to prevent constipation. I mix it in his morning milk so he has no idea - I wish all medicines were this easy!
Venogram - This is an x-ray that involves injecting contrast material (dye) into a vein to show how blood flows through the vein and what type of condition the vein is in. We need to know that Will's right leg has a deep vein system (which we are sure that is does) and that the big vein isn't vital for his leg. I believe this will be done at Vanderbilt by Dr. Baron. If everything looks as we hope, we will move on to the ablation procedure.
Ablation of Vein in Right Leg - If we get a good report that Will's deep vein system is good and the big vein is not needed, Dr. Patel in Cincinnati will do an ablation procedure. It was explained to us that he goes in with a laser and basically burns (cauterizes) his way down the entire vein to close it and make it no longer viable. This is very similar to a procedure for a varicose vein, except this one is much larger and Will is going to obviously need anesthesia. This seemed to be a new thing that is being done for vascular malformations. Dr. Patel explained that just doing sclerotherapy would never have really helped that leg vein since it is so large.
Other follow-ups -
Will is going to have a kidney ultrasound every 3 months until he is 8 years old to check for WILMS tumor. This is a cancer of the kidney that is sometimes found because of the "overgrowth syndrome". Very unlikely, but at least we are checking.
Orthopedics will begin following, but no surgery would be done until age 10-12. We will only have to do something if the right leg ends up being longer than the left leg.
So, that's the plan. JT and I are comfortable, relieved, and ready to get this going. Sweet Will left the appointment in Cincinnati saying "but my foot still hurts". I had been preparing him for the trip telling him that we were going to meet the doctors that could help him, so he was expecting some immediate remedy. Oh how we wish that were possible! But, we are just so happy to be moving forward.
As always, thanks for the prayers, love, and support!
If you want more information on anything in the plan, you can google most of it. Although if you are one of my patients, you know that I NEVER recommend looking up health info on the internet to patients! Hahaha!
Monday, February 2, 2015
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!).
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.
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.
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 |
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.
Sunday, February 1, 2015
"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!
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!
Tuesday, January 27, 2015
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.
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.
Wednesday, January 7, 2015
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!
So everybody, meet Whiskers the kitty-cat.
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.
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.
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