Jacob's 1st trip to NIH Day 4

Jacob's 1st trip to National Institute of Health


Day 4

Wednesday, December 2, 2009

Morning came very quickly. I woke up not feeling very rested at all. Our room was very nice, very quiet, and the beds, for the most part, comfortable. There’s just no place like home.

Jacob’s 1st appointment of the day was at the Eye Clinic, with Ekaterini Tsilou, MD. We checked in on the 11th floor and then were sent down to the 10th floor for an eye exam and vision screening. It was the same song and dance Jacob has done 100 times before, but the results this time left me pleasantly surprised and extremely happy. During the 1st round of tests the tech turned on a lighted box, which looked a lot like an x-ray viewer, except it had rows of letters and numbers on it. It was bright white with bold black letters. He asked Jacob to read the smallest line possible. Jacob looked at it for a minute and started to read. I wish I could have seen my own reaction. Not only was I shocked that Jacob could read any of it at all, but blown away at how small the line was that he did read. Jacob and I exchanged looks. I asked Jacob, “Seriously? Are you guessing?” He was like “What?” He hadn’t even realized his peripheral vision had improved so much. A month after surgery his vision had only improved from 20/400 to 20/200. Now, WOW! What a difference. I had prayed for Jacob to not loose any more vision and for the surgery to at least help him keep what he had left. My mom on the other hand has told me many times she is praying for Jacob’s sight to return to him completely in his left eye. God was meeting us in the middle. Jacob’s vision is now at 20/63. Answered prayers! Thank you Lord! Thank you, Dr. Pincus and Dr. Lewis. Jacob still has to take a minute to look at whatever he is trying to see and the black on white contrast makes a huge difference, but the improvement is clearly there. The meningioma is still on the optic nerve, but obviously Jacob’s optic nerve is truly more “happy,” as Dr. Pincus worded it, and it is sending much better signals. This news made my day!

After the eye exam, the tech took Jacob to do an automatic fielding test and sent us back upstairs. We met with Dr. Tsilou and went over Jacob’s eye medical history. It is the longest and most detailed of all his medical information. Jacob then had his eye numbed, pressure checked, pupil dilated and photos of his optic nerve taken. Jacob’s pressure was good, but his optic nerve is still showing a little paleness. The rest of his eye looks healthy and the drusen was not seen. The doctor also measured how far Jacob’s left eye ball was sticking out from its socket. She said that meningiomas on the optic nerve have been known to cause the eye to protrude. She said they would watch it and Jacob’s measured pretty normal. We were done with the Eye Clinic and it was time to go to the Ear, Nose and Throat Clinic and see Jeff Kim, MD.

Once again we didn’t wait long. Jacob was put right into a room. Dr. Kim came in and asked us the same questions we had been answering the past few days. He looked at Jacob, his skin, his tongue, Jacob’s nose and his ears and with Jacob’s permission took pictures of Jacob’s visible fibromas and café au lait spots. He informed us that the once thought cyst on Jacob’s tongue is not a cyst, but a fibroma. Dr. Kim went over Jacob’s scan and confirmed that Jacob’s vestibular schwannomas (VS) or as I usually call them, acoustic neuromas (ANs, or simply the ear nerve tumors) are growing. The right side is around 9 mm and the left around 7 mm. Dr. Kim believes that there is another tumor, along with the ones already seen, on the right side inside the inner ear. This was not good news. He went on to explain to me that the balance nerve, the facial nerve and the hearing nerve are all in the same area. They believe the VS start on the balance nerve and then take over the other nerves when they start growing. He discussed why they do surgery and some of the different results. Most patients lose hearing after a VS removal surgery is done on that side. If the hearing nerve can be saved during a VS removal then a cochlear implant (CI) can be placed. A CI is a surgically implanted electronic device that provides a sense of sound to a person who is profoundly deaf or severely hard of hearing. The cochlear implant is often referred to as a bionic ear. We also talked about an Auditory Brainstem Implant (ABI). The ABI, like the CI, replaces part of the hearing mechanism that is not working properly. Whereas the CI is implanted in the cochlea and replaces the function of defective cells, the ABI is implanted in the brainstem, and replaces the function of a defective auditory nerve. It is used most often in patients who become deaf due to NF2, because NF2 causes tumors on the cranial and spinal nerves. Removing those tumors often requires severing the auditory nerve, which destroys the hearing in the affected ear. Bilateral surgeries can destroy the hearing in both ears. Traditional CIs are ineffective in these cases, because they rely on the auditory nerve to transmit signals from the cochlea to the brain. But the ABI bypasses the auditory nerve to inject acoustic information directly into the brain.
Jacob’s case is complicated because of the schwannoma growing at the bottom of Jacob’s brainstem/top of his spinal cord and because of his visual loss. They are not recommending any surgery at this time, but will be watching them closely. The tumor on the right side in Jacob’s inner ear can not be removed or debulked with out taking his hearing. More bad news.

Later, Jacob had vestibular balance testing – which was really cool. We also had a very nice dinner at the Double Tree Hotel this night. I will write about both of these later. I have to run for now, but I didn’t want to wait any longer to post the good news about Jacob’s vision.

Day 4….to be continued!