Thursday, February 24, 2011

Meeting the Pediatric Surgeon

Tuesday, February 22, 2011

I'm sorry that it has taken me a few days to get this posted.  I'm trying to keep up with all of the kids' activities and working on my consignments for the One Week Boutique coming up.  Who knew getting ready to sell baby items would be so much work!?!

Tuesday was a typical, interesting day at Shands.  We were out of the house and on the road by 6:40 am.  Both boys slept the entire way there.  It was an uneventful drive, which is always a good thing.  I did see several FHP.  One was driving with a headlight out and another passed me cruising at 82 mph in my trusty dusty van (Sally is running great, just didn't get the carseat and stroller moved the night before).  They need to practice what they preach!  Not that I don't appreciate FHP and their difficult and dangerous jobs, I'm just saying.  So I was thankful for not getting a speeding ticket and happy to get to Gainesville safely.  Jacob was much harder to wake up than Gavin when we arrived at the Medical Plaza at 8:45.  After changing Gavin out of his wet diaper and pajamas I realized I left his shoes at home.  I couldn't believe I had forgotten something that important, but I decided it wasn't that big of a deal and we would just go with it.  After all, I did have his socks he slept in the night before and his fresh socks to match his outfit for the day.  Double layering was going to do just fine.
I was very proud walking into the clinic that we had arrived so early.  It was barely 9:00 am and our appointment wasn't scheduled until 10:30.  When I spoke with the scheduling nurse the week before she had informed me that Dr. David Kays' clinic was completely full, but they were going to work Jacob in because they know Jacob needs his port.  She said even though she put Jacob down for the 10:30 appointment, I could bring him earlier and they would see him at that time.  I thought this was a great idea because I was able to schedule Jacob's eye appointment with Dr. Hazem Samy at 11:30, but life obviously had different plans.  The receptionist checked us in, Jacob was weighed and went through the same ol' song and dance and Gavin and I got settled in the waiting room.  The pediatric floor is very nice with movies playing, things to play with and crayons and coloring pages.  They even have volunteers in the waiting area to interact with the children.  Jacob thought he was going to get to watch Finding Nemo, but a few minutes later the nurse informed us that Dr. Kays was at the hospital performing an emergency surgery and wouldn't be in until after lunch.  I thought, "Ok, things happen and I understand it was out of anyone's control." I put my happy face on and tried to make the best of it.  Jacob wasn't upset and we went on to the eye clinic to see if Jacob could be seen early there so we would be free to return to the surgical clinic that afternoon.  Upon checking in at the eye clinic I was informed that Jacob's appointment was made for the 25th of February, not the 22nd.  Deep breath. At that moment I was so happy that I had listened to not one, but three sermon podcasts from our pastor at KUMC on my way to Gainesville that morning.  I was feeling very Christian-like at the time and I just smiled and said ok.  There must of been some miscommunication during my phone call with the scheduling eye clinic nurse the week before. Dr. Samy wasn't even in clinic for us to be worked in, but I did ask if Jacob could still have some of his testing done.  She checked with Amy, but she was too busy at the time with other doctor's patients.  Jacob, Gavin and I left the Medical Plaza.  After cruising the Archer strip we decided to go to Target.  Gavin got a new pair of shoes and bubbles.  We blew bubbles in the parking lot under a tree and threw acorns.  Jacob stirred up some ants so we relocated to the van for lunch.  I had packed our lunches.  The eye clinic called and said they could do Jacob's Goldman Fielding vision screening at 11:30, but we declined.  It was time to eat and Jacob didn't want to go back right then.  I wasn't terribly concerned because of the extensive testing NIH had just done on Jacob's eyes in January.  Because Shands is so close to our home and NIH is so far away I definitely want them to keep up with Jacob's eyes and vision, but we will just do it on another trip.  I also tried to make Jacob's EEG for this trip, but they weren't able to get him in at all.  His EEG is scheduled for April 6.  There will be a lot of trips to the Swamp coming up in Jacob's near future.  We were back at the pediatric surgical clinic by noon and we waited.  Jacob was thrilled to watch 101 Dalmations, which I didn't know was one of his favorite Disney films.  It was the one that wasn't animated.  Learned something new about my 14 year old!  Finally we went to a room and met with Dr. Kays' ARNP and she was really nice.  I wish I could remember her name.  I liked her very much.  About 30 minutes later, Dr. Kays came in and met with us.  He looked Jacob over and asked a few questions.  He explained how the port would be placed and he asked Jacob if he had a preference to which side he wanted it in.  Jacob said he wanted to be able to go hunting and fire his gun without fear of damaging the port.  Dr. Kays will try and place it in the right side first, but can't guarantee it.  He also asked us about Jacob's MRI and CT scans.  The metal in the port will be titanium so it will not interfere with the MRI.  Jacob will be receiving the smaller port because when he has his CTs he does not receive contrast.  I guess CT contrast is delivered at a very high rate and only a special port can handle that, but it is more visible and sticks out.  We decided on the smaller of the two that will work well for the infusions of chemo, blood draws and MRI contrast. Dr. Kays was pleasent.  He interacted well with Jacob.  I was hoping Dr. Kays would be able to do the surgery this week, but they are slammed with pediatric surgeries everyday.  Another one of those sad moments for me thinking about how many kids need surgeries.  Jacob's surgery will be at Shands Children's Surgical Center in Gainesville, FL on Monday February 28, 2011.  I don't know the time, but they will call us the night before and let us know.  Because Jacob is older he will be a later case because he is more capable of waiting to eat than younger children.  (Obviously they don't know Jacob!) The surgery is considered minor and he will go home that afternoon.  Dr. Smith said he will be sore for a few days.  I am going to have to reschedule his physical with Dr. Kelch for Camp Daniel Boone and contact his school about postponing FCAT Writing next week to later in the week.  According to Dr. Smith, Jacob will start infusions roughly two weeks from Monday.  She likes for the port to heal for about two weeks.
Our drive home was peaceful.  Gavin slept the entire way and Jacob sat quietly and played his iPod.  I missed our normal "on the way home" two hour conversation, but I just thought it best to let him do his thing.  We were home in Tally by 5:00 pm.  Jacob's treat for the night was ice cream at Lofty Pursuits.  I wish he had gotten a sundae, but he kept it simple with ice cream and sprinkles which surprised me because Jacob is always excited about what he is eating.  I thought he would've ordered something really cool.  Have you seen their crazy menu?  And tonight I thought it was adorable that he called his GG and put in a request for her fried chicken, butter beans, mac n cheese and corn bread for when he visits her on Friday. Too cute. 

Please pray for Jacob and keep him in your thoughts.  Although this surgery will be peanuts compared to him having a craniotomy, Jacob is still scared and anytime your child is put under anesthesia it is concerning.

I will post a post-surgery update as soon as I am able to.

Another mom with a child with NF2 created a web page on Caring Bridge for her daughter and shared it with me and the NF2 crew.  I was inspired to make one for Jacob. I'm not sure if any of you remember, but I tried to do this years ago with Care Pages, but never finished it.  Jacob's Caring Bridge page is active and I will update it as medical things happen.

Visit on the web by clicking the link below.

Jacob's Caring Bridge journal has the previous posts from this blog about his medical information and it will continue to only be about Jacob and will not have any posts about the rest of our bunch.  You can also leave Jacob notes there if you want.  I think Jacob would enjoy reading any words of encouragement or funny stories or just someone saying hi.  Check it out if you have time. 

Below is Port information if you're interested:

In medicine, a port (or portacath) is a small medical appliance that is installed beneath the skin. A catheter connects the port to a vein. Under the skin, the port has a septum through which drugs can be injected and blood samples can be drawn many times, usually with less discomfort for the patient than a more typical "needle stick".

Ports are used mostly to treat hematology and oncology patients, but recently ports have been adapted also for hemodialysis patients.

The port is usually inserted in the upper chest, just below the clavicle or collar bone, leaving the patient's hands free.

A port consists of a reservoir compartment (the portal) that has a silicone bubble for needle insertion (the septum), with an attached plastic tube (the catheter). The device is surgically inserted under the skin in the upper chest or in the arm and appears as a bump under the skin. It requires no special maintenance and is completely internal so swimming and bathing are not a problem. The catheter runs from the portal and is surgically inserted into a vein (usually the jugular vein, subclavian vein, or superior vena cava). Ideally, the catheter terminates in the superior vena cava, just upstream of the right atrium. This position allows infused agents to be spread throughout the body quickly and efficiently.

The septum is made of a special self-sealing silicone rubber; it can be punctured hundreds of times before it weakens significantly. To administer treatment or to withdraw blood, a health professional will first locate the port and disinfect the area. Then he or she will access the port by puncturing the overlying skin with a 90° Huber point needle although a winged needle may also be used. (Due to its design, there is a very low infection risk, as the breach of skin integrity is never larger than the caliber of the needle. This gives it an advantage over indwelling lines such as the Hickman line.) Negative pressure is created to withdraw blood into the vacuumized needle, to check for blood return and see if the port is functioning normally. Next, the port will be flushed with a saline solution. Then, treatment will begin. After each use, a heparin lock is made by injecting a small amount of heparinized saline (an anticoagulant) into the device. This prevents development of clots within the port or catheter. In some catheter designs where there is a self-sealing valve at the far end, the system is locked with just saline. The port can be left accessed for as long as required. The port is covered in a dressing to protect the site from infection and to secure the needle in position.

The surgery itself is considered minor, and is typically performed under both local anaesthesia and conscious sedation. Patients sometimes have a little discomfort after the procedure, and can be managed with a narcotic, such as demerol for 24–48 hours.

A port is most commonly inserted as a day surgery procedure in a hospital or clinic by a surgeon or an interventional radiologist, under conscious sedation. When no longer needed, the port can be removed in the operating room.

Thursday, February 10, 2011

Feburary 9, 2011 Trip to Shands at UF

Trip to Shands Healthcare at the University of Florida


Waking up at 5:00 am is never pleasant for me. Especially after only sleeping two and a half hours due to a precious little one who hates to go to bed and a twenty year old cat who cries to come in and out of the house all night.

Jacob had a hard time waking up, as usual. He went to bed with another headache Tuesday night and woke up with it still this morning. He also woke up complaining of neck and arm pain and again had the shakes. I was thinking this was not a good way to start our morning. I get so worried every time Jacob says anything hurts or tells me he doesn't feel good. It is impossible for me to just think it's a typical headache, just a virus, muscle fatigue or something simple and normal. I am always on edge, waiting for new symptoms of one of Jacob's many tumors to show out.

We made it out of the house on time at 6:10 am. I forgot my sunglasses and I was dreading the sun rising and blinding me before we made it to I-75 South. To my surprise we were at the end of our drive on I-10 East when the sun started to come up reveling the most beautiful sunrise I have seen in years. The stunning shades of reds, pinks and yellows changed my mood and I felt like God was showing me a "light at the end of the tunnel," or the light of a new beginning. I felt like today was going to bring us hope. I had prayed for peace today for many different reasons and I felt like this was God's sign to me that he was going to carry me through the upcoming hours.

Without any doctors notes to look at, I will do my best to recall the things Jacob's doctors went over with us today.

Our first appointment was with David Pincus, MD, Jacob's neurosurgeon. We arrived on time for Jacob's appointment at 8:30 am. Jacob was happy to see his dad and stepmom, down from Georgia to support him for the day. The nurse called us right back and she weighed Jacob, measured his height and took his blood pressure. Jacob weighed 109 lbs and is now 5'7"!! Wow. Tall and Skinny! Dr. Pincus came in around 9:15 and he went over Jacob's recent scans from NIH with us and he discussed with us the areas of concern. I was relieved to hear that Dr. Pincus met with their head radiologist and they are not concerned about the “unknown” right frontal lobe tumor. Dr. Pincus even made the comment that out of all of Jacob's tumors and lesions, the one in the right frontal lobe remains the most stable. They are sticking to their past belief that it is a meningioangiomatosis. Dr. Pincus definitely does not believe it needs to be removed just because we aren't positive what type of tumor it is. The right side Vestibular Schwannoma (VS) was discussed. Since Jacob isn't having any hearing loss Dr. Pincus thinks it is best to wait to have it removed. He also said radio surgery (gamma knife) is an option to treat the right VS. Even though I have read about many other people with NF2 having their VSs treated with gamma knife, no one has ever recommended this for Jacob. I will have to read and research this new idea.

In 2009 we took Jacob to explore the benefits & risks of radiation and radio surgery for his left optic nerve meningioma. We decided at the time radiation was not the best option for that situation. Radiation is scary.

We talked about the VSs or the VSs surgical removal causing damage on Jacob's facial nerves, but right now we are most worried about preserving Jacob's hearing and will worry about the facial nerve being damaged later.

Dr. Pincus explained how the two eppys could be removed at C-2 and the brainstem base. He still believes that it is best to watch Jacob for pain or other signs that those tumors are causing problems before going into surgery. Lastly we talked about the C-7 tumor that is compressing the spinal cord. Dr. Pincus did an exam and did find some mild reflex abnormalities, which could suggest spinal compression, but isn't enough reason to do surgery and it isn't positive which tumor could be causing it. The rest of Jacob's exam was normal. His walk and balance were both normal also. Dr. Pincus will look at Jacob's scans as he has them, but he wants to see Jacob back in his clinic in six months to re-evaluate him physically. Dr. Pincus and Leigh Ann, his nurse practitioner, are always a pleasure to see and to talk to, and they explain things well in "English."

Jacob's next appointment was with Amy Smith, MD, Jacob's Pediatric Oncologist. Oncology is always very busy, maxed out with kids and their families. It is very sad if you think about it. We waited for several hours, but Dr. Smith is always worth the wait. It was nice to see Mrs. Hillary, Dr. Smith’s nurse practitioner, and chat with her for a bit. After being greeted with a much needed hug, Dr. Smith asked Jacob how he was feeling, how his hearing is and the typical questions Jacob has grown accustomed to. We talked about Jacob's headaches and tremors. We are wondering if Jacob's blood sugar is getting low. She suggested we try an afternoon snack and she told Jacob that his oreos weren't going to cut it. Dried fruits and nuts, whole-grain crackers, bread with peanut butter, yogurt and fruit are all good foods to help increase blood sugar. We will start paying better attention to this for Jacob and encourage him to remember to eat before he starts to feel bad. If eating more frequently doesn't help, we will let Dr. Smith know and we will try to see what else could be going on. Finally we talked about drug therapies. Dr. Smith thinks that Avastin could help stop the growth of Jacob's tumors and possibly shrink them some. She also said she may prescribe Rapamycin with the Avastin later after she does more research to see how it will do with Jacob's seizure medication. Rapamycin is now being used to treat some patients with Tubersous Sclerosis, which is what Jacob was first diagnosed with in 2000. Dr. Smith talked to Jacob about having a port placed, how the surgery is done and how the chemo is administered after the port heals. Jacob was happy to hear he would still be able to play basketball with the port in his chest. No crazy rough-housing allowed though. So now we wait to have the treatment approved by our insurance and the outpatient surgery to be scheduled for the port. After two weeks of healing Jacob will be able to receive his first infusion of Avastin. It takes about an hour and a half to receive and has to be done at Shands every other week. We talked about side effects and she said the tumors will be monitored by MRI. Jacob will also have blood drawn at each infusion appointment to have his white blood count checks and they will do a UA to check for protein in his urine. It is an extremely odd and almost guilty feeling to be excited that your child is going to receive chemo, but in Jacob's lifetime we have never been given anything to fight against his NF2 other than terrifying surgeries. I feel like we are going to do something and not just sit on our hands waiting for his NF2 to get worse. I pray that this drug therapy will buy Jacob more time with his hearing in both ears and spare him from having to have any surgeries this year. Avastin isn't a cure, but we are hoping it will be a huge bandaid that will help for a long time. Deep breath, exhale. Jacob was excited for lunch and really happy he didn't have to have a complete physical at this appointment, lol!

Our last appointment was with Pediatric Neurology. I won't go into great detail about this appointment because it was with a new doctor and we mostly went over Jacob's medical history. I really like the new neurologist, Dr. Edgard Andrade. He was very attentive and spent a lot of time with us. The main reason we were there was to have an EEG scheduled to see if Jacob could possibly be having some seizure activity. Dr. Andrade talked about the different types of EEGs and said we will start with the 45 minute one with Jacob. We are going to try and schedule that the day we take Jacob to have the port placed. We talked about increasing Jacob's seizure medicine and what the average dose was for a person his size. Jacob's dose is very, very low.

That appointment was over around 3:15 pm and finally we could go home. Jacob was dying for Dairy Queen. I googled their locations and we hit one on the way out of Gainesville. Jacob said it was the best chocolate milkshake he had ever had. It rained on our way home, but Jacob and I had a nice drive and talked and laughed and everything felt normal for a few hours.We drove straight to pick up Brie from art class and headed home. I was thrilled to be home, see my beautiful Brie, my little G-man and get a big, much needed hug from my darling Thomas. Thank you Babe for taking such good care of G while we were gone today. I didn't worry once about him and today was the longest time I have been away from him in his life. (Other than the 2 times I was in the hospital, but those days don't count.) I am so thankful for our family and my friends. I would never get through any of this without your prayers and support. Keep them coming!

I love you all so very much.