I'm not even sure I would be writing this blog tonight, I would have probably waited a few days to compose myself before relaying this information to all of you, but this blog is the only way I have to communicate with Jacob's father....
On February 25, 2000 Jacob had his first grand mal seizure. He was 3.5 years old. He had gone through several eye surgeries prior to this day, but the day of his first seizure was the day our world changed. Unfortunately I remember it like it was yesterday. It plays over and over in my head like a bad dream I am unable to forget. I remember the moment, the following day after many scans and tests, that Dr. Truman informed us Jacob had brain tumors, brain lesions, abnormalities. I remember how I felt and the fear that overcame me. I have thought about that day a lot this week, as the 11 year anniversary approaches. I have thought about how strong and amazing Jacob has been over these years and how very special he is to us. I never imagined 11 years later this is where we would be, I guess I was too afraid to look to far into the future, but I knew we would fight it all the way.
Today, I felt like I did 11 years ago when they told me that my precious son had brain tumors. Completely devastated and fearful of what tomorrow will bring. Like our life and world as we know it is about to change, again. Today was a lot to take in at once, a lot to process and once again, imagine.
This is what was discussed with Jacob's neurosurgeon, Dr Asthagiri at today's appointment, as best as I can recall and understand. I will have a better grasp on what we talked about when I receive the clinic notes and dictation from today.
Dr. A did not want to talk measurements and sizes with me today. He said it wasn't necessary, that it was useless information for me to worry myself with.
The very first thing Dr. A wanted to discuss with Jacob and me was why he did the CT of Jacob's brain this visit. He wanted to view the changes in the unknown lesion, tumor in Jacob's right frontal lobe. It does have calcifications, which we already knew because we were told that in 2000. Dr. A is concerned as to what the tumor really is and how it may be effecting Jacob. It has changed and grown over the last 10 years. The way he explained it to me was if this tumor was seen on a scan for one of us (in the room) it would be a cause for alarm. He said because it is not clear as to what the tumor is he does not want to just assume because Jacob has NF2 that it is a begin tumor, especially with the changes it is showing. I do not understand what these changes are or think that it was important for me to understand. I did understand that he doesn't want to jump to conclusions, but he doesn't want to assume it couldn't be harmful either. He has requested that Jacob see a neurologist at Shands and have an EEG performed. We discussed episodes of mumbling and eye twitching that Jacob has been experiencing over the last 8 months. We also talked about them last visit. They have not gone away and he believes that their is a high chance that Jacob is having seizures. The EEG should show if there is abnormal activity in that area or any other area of Jacob's brain. I also informed him that many years ago Jacob had a nuclear medicine scan (I can't remember the name) for the tumor in question, in the right frontal lobe looking for activity. The purpose of the test was to help show whether the tumor was cancerous or not. Jacob's doctors have been curious as to what it is all along. Dr. A said there are a few different scans like that they can do for Jacob that will give us that information. We will wait to have those ordered until after we get the results of the EEG. There will be a basic EEG done and the neurologist will then decide if a more detailed EEG needs to be done. I will find out exactly what test Jacob had previously so the results can be compared. The tumor is close to the surface and surgery to remove it would not be without serious risk, but if surgery is necessary it is in a good place (if that is possible).
The next thing we discussed was a schwannoma at the C-7 (nerve 8) in Jacob's cervical spine that they have been watching very closely. It has grown a considerable amount over the last 6 months and is now pressing on Jacob's spinal cord. Dr. A is extremely concerned about this tumor because it is in a place to damage Jacob's left hand and arm, his only hand that really is well working. If the tumor continues to grow it will have to be removed to protect the use of his legs and even the surgery could damage the left hand. Dr. A has requested that we have another MRI of the brain and spine at Shands to monitor the growth of the unknown brain lesion and this schwannoma and the bilateral vestibular schwannomas.They are surprised that Jacob is not having any weakness, problems or pain with the left hand or arm.
Next Dr. A told us that the right vestibular schwannoma is growing and has grown several mm over the last 6 months. He said it is in fact now touching the brain stem and if it doesn't stop growing it will have to be taken out in the very near future. He also said that the VS is at the size now where if surgery was done now Jacob's right facial nerve could be saved, but the surgery would definitely take Jacob's hearing on the right side. If the facial nerve is damaged it effects a persons ability to smile, talk and eat. It is impossible to predict what any of these tumors are going to do, when they will grow or stop growing. The balance nerve is always cut during a removal surgery of a VS. Balance is usually restored a few days after surgery.
The left VS has also grown some, but is not as big as the right. He also said the left facial nerve tumor has grown. He said that the facial nerve tumor is at a good size to be removed and some doctors may recommend having the facial nerve tumor removed to save the facial nerve and may tell us the chance of hearing loss is only 20 or 30 percent, but he does not agree with this and feels like a surgery like this for Jacob would leave Jacob with a 50 or 60 percent chance of losing his hearing if they went in to remove the facial nerve. He does not recommend anything be done at this time for the left VS other than just watch it.
We went on to talk about the 2 ependymomas inside Jacob's upper cervical spine, inside the spinal cord, one of them at the base of the brain stem. The good news is that the eppy at the base of the brain stem that doubled in size over the last year and a half that we have been terrified over has not grown over the last 6 months and seems to be stable. The bad news is that the one under it that showed up last MRI in June of 2010 has doubled in size over the last 6 months. That one also will have to be removed if it doesn't stop growing. Dr. A did say the spinal cord still has room on the sides for the fluid to flow through. These will also be imaged in 3 months on the MRI at Shands. We hope it will stop growing after this spurt like the other one has stopped.
Dr. Asthagari then went into details about how the surgeries would be preformed and the recovery time. We talked about the risks and the many ifs, maybes, and possibilities. We also talked about chemotherapy. He gave me information about different places doing protocols for NF2. He said there is no drug that has proven to work on any of the types of tumors Jacob has. He is not against Jacob having chemotherapy, but he made it very clear not to wait until something starts to go wrong to get Jacob into it. It will be too late then and he will have to have surgery either way. We talked more about the different drugs, but until I do more research I won't ramble on about that.
This is all the information I can share at this time. I am exhausted, overwhelmed and my emotions are drained.
Please continue to pray for our precious Jacob. He needs Gods hands on him to heal him more than ever.
First thing this morning we saw Chris the Audiologist and he gave me Jacob's report from December 2009 and the CD he promised me that contains information about the FM system we talked about Tuesday. There are other pamphlets in the envelope, but I have not read over them yet.
Then we met with Dr. Kim, Jacob's Ear, Nose and Throat (ENT) doctor. He reviewed Jacob's scans and audiology testing from last few days. He did an exam and asked Jacob the typical questions. The information he gave us is as follows:
Jacob's Vestibular Schwannomas (VS) have both grown and he used the word "growing". He did not give me measurements for each side. He said his method of measuring differs from the typical way and we can get those numbers from Dr. A tomorrow.
Dr. Kim said because Jacob is not suffering any hearing loss, pain, or balance problems and the tumors are not pressing on the brain yet that he does not recommend any surgery at this time. He also feels like Jacob is not a candidate for radiation for the VSs. He did recommend that Jacob try chemotherapy, calling it a "chemo protocol" to attempt to keep the tumors from getting any larger. He believes there is a trial offered at NIH that Jacob could be part of, but we will have to check with Dr. A.
I realized that I like it better when they refer to chemo as a drug trial or drug therapy. Connotation is an amazing thing. I asked once again what size a VS usually is when it needs to be removed, before it starts to press on the brain. Dr. Kim's answer was that everyone is different, but 3 cm is the average size. Jacob's are still under 2 cm, but the right side was 19 mm six months ago. Getting too close for my comfort and the surgery to remove the VSs will take Jacob's hearing.
He also said Jacob's facial nerve tumor on the left side has grown, but he said that it will take a long time for it to effect the facial muscles and facial paralysis takes years to occur.
Dr. Kim said the results of the audiology testing shows the left side nerve seems more effected than right, which is odd due to the right side tumor has been larger. He tried to explain to me that the left side tumor is fatter now, but I will wait to talk to Dr. A tomorrow to make more sense of it.
I am very interested to hear what Dr. A says about chemo for Jacob. He is not a believer that chemo works for NF2 patients, but he is not against Jacob trying it either. I have asked his opinion in the past and I have wondered how we will know when it is time to start the chemo. It is a huge decision. I prayed we would have more time. Jacob also has an appointment coming up with Dr. Amy Smith at UF Shands to review Jacob's scans from this week. This past July she and I discussed types of chemo she can do at Shands to try and control the growth of not only Jacob's ear nerve tumors, but also the spine. I'm a nervous wreck worrying about what Dr. A will tell me about Jacob's other brain and spinal tumors tomorrow.
Enough with the bad news and onto the good!
Next we went to the Eye clinic. Our papers said we needed to go to the 11th floor clinic, but we needed to be on the 10th floor. The doctor Jacob saw last time is no longer with the clinic. I was so excited walking into the 11th floor clinic. It had a big space to play, with lots of toys. I was completely deflated walking into 10th floor clinic. It was full of people, chairs and nothing for kids. I also encountered the first rude person of any of our visits to NIH. I would of thought he was just having a bad moment or maybe even a bad morning, but he was rude to me later in the day too, so I decided he is the only person (so far) that I don't like at any of the clinics we have visited. I realized after checking in that everyone in the 10th floor eye clinic looked miserable and it was only 10 am .
Jacob did a Goldman's Field test, got numbing drops so they could test his eye's pressure and for dryness, and he had photos of his iris taken. He did a vision screening and then had his eye dilated.
The new opthamologist/retina specialist, Dr. Bishop, did an exam of his retina, optic nerve and then sent him for more photos. He had pictures taken of his optic nerve, iris (again) and retina. They also did a laser nerve scan, which was pretty cool.
Jacob's vision has not changed in the last year!! His optic nerve meningioma appears to be stable and unchanged. Finally good news!! Jacob still has 20/63 vision with some obstructions from the tumor.
Four hours later Jacob was done!
We went to Records to request Jacob's images to take to Dr. Smith and went to the cafeteria for lunch. To my surprise they had a new vendor who had great sushi. Brie and I were happy girls eating delicious sushi. Jacob and Gavin were happy boys having pizza, again! After we were all stuffed with yummy food we headed back down the hill to the Inn. Walking out, Jacob was blinded by the sun and had to hold onto me as a guide. Half way home Jacob says to me that he thinks they should give patients and families golf carts to drive around campus. I got so tickled thinking about Jacob blindly terrorizing the NIH campus in a golf cart. Jacob and I laughed out loud. The fresh air and a good laugh were both well past due for the day.
Gavin passed out during our walk back and took a great almost 3 hour nap. I thought I was going to love the quiet time, the alone time (the kids were doing crafts and playing games), but I didn't. Reflecting on the past few days, I sat and sobbed, my heart breaking for Jacob. Jacob came in later to check on us and sat next to me. I asked Jacob if all of this ever gets to him, if he was bothered by his appointments and all the doctors. He said to me, "No, I just zone it all out." I smiled and said back to him, "Maybe that's what I need to do." I laughed when he said he didn't think that would be a very good idea.
Wow! My son, my Jacob, is so amazing. He takes it all in stride and just lets it roll off. I want to be more like him! I love him more than any words can ever express.
All I have left to say tonight is...
My Lord, God,
Please, please, please hear my prayers!
Note: I know I always slaughter the spelling of VS. Here is the correct spelling and an explaination of what they are.
What is a vestibular schwannoma (acoustic neuroma)?
A vestibular schwannoma (also known as acoustic neuroma, acoustic neurinoma, or acoustic neurilemoma) is a benign, usually slow-growing tumor that develops from the balance and hearing nerves supplying the inner ear. The tumor comes from an overproduction of Schwann cells--the cells that normally wrap around nerve fibers like onion skin to help support and insulate nerves. As the vestibular schwannoma grows, it presses against the hearing and balance nerves, usually causing unilateral (one-sided) or asymmetric hearing loss, tinnitus (ringing in the ear), and dizziness/loss of balance. As the tumor grows, it can interfere with the face sensation nerve (the trigeminal nerve), causing facial numbness. Vestibular schwannomas can also press on the facial nerve (for the muscles of the face) causing facial weakness or paralysis on the side of the tumor. If the tumor becomes large, it will eventually press against nearby brain structures (such as the brainstem and the cerebellum), becoming life-threatening.
Day 3: Pediatric Oncology and Second Day @ Audiology
Jacob's first appointment this morning was with Pediatric Oncology. Jacob was interviewed and examined by Dr. Kathy Warren and a very nice fellow. All the fellows at NIH have already completed their medical training, have been doctors and decided to return do specific research. I really like this clinic. They have a great play room with lots of toys and games for the big and little kids to enjoy. There is always an intern in the play area to interact with the patients and children.
Jacob and I met with Dr. Warren and her fellow for about an hour. They usually see the patient individually, but since Jacob had a limited amount of time, he saw them both at the same time. They asked Jacob a series of questions, like how has he been feeling, how is school going for him, is he suffering from any pain, has anything changed since last visit, etc etc. They looked at Jacob's skin, eyes, nose, mouth, ears. They did strength and balance testing. After looking in Jacob's ears the fellow said it is very apparent that Jacob's right inner ear is "not normal". This comment has been replaying in my head all day. This is the first time anyone has said that anything is "obvious" after viewing the ear from the outside. Jacob's circulation in his right hand was also discussed. There is a very noticeable difference in temperature when touching Jacob's right hand compared to the left. His right hand always feels cooler and this winter he has complained of pain when being outside. We have a hard time warming his hand up and he suffers from tingling as the cold wears off. Neither doctor had any solutions to this problem. I was wondering if they made circulation arm socks or something, lol. Can you picture that? I'm convinced we see Dr. Warren more for her (or really her clinic's) information than for us, but overall this is our main purpose for bringing Jacob - so his special case of Neurofibromatosis Type 2 (NF2) can be researched and compared to normal genetics and others with NF2, hopefully finding a cure one day. Dr. Warren said that they will look at Jacob's scans Thursday afternoon with the rest of Jacob's doctors, including Dr A. It was a painless visit for Jacob and Jacob liked the fellow. He was from Atlanta, GA and he loved Jacob's Georgia boots. Suppose it reminded him of his roots.
Jacob was late for his ABR and Audiogram (2 hours). It made our day a little longer than I originally expected, but not by too much. The ABR and Audiogram took two hours. Jacob returned well rested (he can sleep during the ABR testing) and picking at the sticky glue they use to place electrodes all over his forehead, ears and scalp.
Note: ABR stands for Auditory Brainstem Response. It tests the ear's reactions to sound, traveling through the nerve into the brain. An Audiogram tests only the hearing.
The Audiologist who performed Jacob's hearing tests (as well as the balance vestibular testing yesterday) was overflowing with information. He said Jacob's hearing is still within normal ranges, but the signals are slower getting to the brain. Six months ago it was only on the right side, now it is on both sides. He also said that Jacob suffers from Auditory Processing disorder due to the Vestibular Schwanomas (acoustic ear tumors). Speech and sounds are getting to his ears fine. His ears are in perfect working order, but the nerves are being effected by the tumors and not processing info to brain right. Speech and sounds get screwed up in transmission.
He recommended an "FM" broadcasting system for Jacob, but there aren't many states or schools using them. A teacher wears a mic, the child has a receiver on their hip (belt or pocket) and wears ear buds. This system blocks out any outside/background noise or interruptions. Proven to also control learning problems for children with ADHD and ADD. Auditory Processing disorder is often misdiagnosed as ADHD and ADD.
After our conversation with the Audiologist we had lunch and took Jacob and Gavin to get their ID badges. We also had to have Jacob's badge bar coded, because he is the patient and they are now using a bar code system for blood work and prescriptions. I got more coffee and it was back up to 5th floor clinic to finish up with Audiology.
The last testing Jacob had done today was explained to me as an extension of the ABR and Audiogram combined to obtain more detailed information.
The results were the same as the tests from before lunch. The only thing the Audiologist added was to create an extremely quiet studying area for Jacob and for us to do our best to explain things and repeat things in situations when speech and sounds are contaminated. Like loud speakers and intercoms.
We left the clinical center around 3:00pm. We enjoyed relaxing for the next few hours before going to the Capitals Hockey Game at the DC Verizon Center!! At the beginning of last week I did a little research online about things to do in DC and I found contact information for the Capitals' Director of Community Relations. I thought a hockey game would be something different and fun for the kids to do. Just this morning I received a response from the director that she received my email and left three tickets for the kids and me at the Verizon Center's will call. How wonderful! We were so excited. We were going to see the Washington, DC Capitals play against the Tampa Bay, FL Lightening. Little did I know they were tied for first place in their hockey division and this was a huge game for them! At 5:00 pm we boarded the Metro subway and went to Jacob, Brie and Gavin's first hockey game. We had so much fun. Even though we are from Florida and Tampa Bay is Thomas' hometown the kids got Caps gear and looked great sporting the Red! The seats were fantastic, the music was awesome, the fights were nuts and the players and fans were extremely entertaining. We laughed, danced, yelled, and ate stadium food. It was a great way to get rid of some heavy stress. The kids and I loved it so much. Thank you so much, Capitals!! Sorry you didn't win.
Now I am listening to all three of my beautiful children snore... missing my Thomas... and missing my Grissom... trying to unwind and mentally prepare for what tomorrow will bring.
We started our day in Tallahassee at 7:30 am. After riding in a car, on 2 planes, the DC Metro subway and in a police car, Jacob, Brie, Gavin and I finally made it to the NIH Children's Inn at 9:00 pm.
Let me just say I am writing an email to US Airways expressing my distaste in our travel day with them on Sunday.
Monday, January 3, 2011
Day 2: MRIs, CTs, and Balance Testing
Today has left me with the knowledge of a whole new definition of tired.
Starting the day I knew we would spend the entire day at the Clinical Center with very little time in between Jacob's appointments.
After arriving at the Clinical Center, Jacob was sent directly to have an IV placed. The IV specialist assured Jacob he had great veins and she would get it in on the first try. She lied. It took many sticks and a lot of digging in Jacob's arm to get the IV in. She said Jacob has bad scarring in his right arm, in his inner elbow veins. I'm not surprised. Jacob wore the IV all day because we didn't know if his CT at 4:00 pm would require contrast. At 10:00 am Jacob was taken back for his brain and spine MRI. I wish I had taken a photo of Jacob in his paper PJ pants and Georgia boots. Very cute.
While Jacob was having his pictures taken Brie and I got coffee and took Gavin to the big playroom. The only drama we had in the next three hours was a lost sippy cup and another little girl having physical therapy in the playroom that Gavin was extremely worried about because she had a walker and was crying. Gavin met a super sweet guinea pig named Harmony.
Right before going back to Radiology, Brie and I went to Security to get our extended visitor badges. (Mine and Jacob's from December of 2009 have expired). I wasn't expecting them to tell me Gavin must have one also. Just something else to remind me how big he is getting, and how fast!
Jacob's MRI was done at 12:30.
We inhaled our lunch and took Jacob to the 5th Floor Audiology Clinic.
All of the clinics and appointments tend to be very much on time and very little nonsense before seeing their patients. Jacob went back promptly at 1:00 pm. I was informed Jacob would be doing 3 hours of balance testing today. The usual is only for 2 hours, but because of Jacob's results last time they wanted to do more extensive testing. I'm not sure why. I spoke with one of the specialist and he said he will have more results for me tomorrow when Jacob returns for his ABR and Audiogram. Jacob's input is more difficult to measure due to only having one eye. He did say Jacob's overall balance is better this trip, but this could be due to Jacob being familiar with the testing. He is worried that the signals are not traveling to the brain as fast as before. Jacob's reaction time is slower. All of the balance testing gives us information on how the bilateral vestibular schwanomas (acoustic tumors) are effecting Jacob's balance and brain functions. He is the same "PhD" who took care of Jacob last year when he had the same testing. I also like that about NIH. The same group of techs, doctors, specialists always see us.
Gavin and Brie waited patiently for another 3 hours while Jacob did his tests. They even took a good long nap together. I was very jealous. Thank goodness for iPods, iTouches, Umizoomi, Toy Story and a notebook and pen. When they weren't sleeping, they were still occupied.
Jacob was done with the vestibular testing at 3:55 pm. Just in time for us to haul butt downstairs for his 4:00 pm CT scan. Jacob was very happy to know he didn't have to have contrast and he got his IV out.
The CT scan was an ueventful "piece of cake" according to Jacob, as was the 2nd brain MRI.
Finally Jacob was all done and we got to head back down the hill to the cozy Children's Inn. We ate dinner, played in the two different playrooms, drew and colored in the art room while Brie made bead bracelets. The big kids played xbox and chatted on facebook. I washed clothes and had a tubby with Gavin.
I think I could sleep standing up right now......I am signing off.
Thank you all for your continued prayers. It means so much to our family.
Gavin is on the couch to the left, Brie on the right
Jacob is in the room in the picture below, in the dark, in a space ship chair, with an alien headset on, being asked questions and monitored. I didn't get any photos of all of the other tests they did, but ask him about them. He says they are super cool!!