Today Manny and I visited Aflac Cancer Center and Blood Disorders Service of Children’s Healthcare of Atlanta
and saw Dr. Dolly Aguilera. I'll try my best to reiterate what she said. Manny screamed most of the way to Atlanta, and whined, yelled, cried and tried to escape the room throughout most of the visit. I would have very much have liked to ask more questions, but as it stood, I had to make the best use of time by asking the poor woman to repeat herself several times so that I could hear what she was saying.
Dr. A asked a lot of questions, the usual ones. She went over the significance of the spots on Manny's brain as well as the tumor on his spine. The spots on his brain, aside from the largest one, she referred to as "bright spots" and said that these types of spots will often be brighter or even fade in subsequent MRIs. She is most concerned with the tumor on his thalamus, the part of the brain that controls a person's personality as well as being a gateway for other parts of the brain to communicate with the rest of the body. Should this tumor begin to grow then treatment would be necessary. Should this happen, Dr. Reisner, the neurosurgeon, would be consulted to see if the tumor could be excised, or surgically removed. If not, then other treatments would be explored.
Children with NF1 have a higher risk of cancer and leukemia. Tumors over the size of 5 cm have an even greater chance of becoming cancerous. Manny's spinal tumor is 1.8 centimeters. If a child with NF1 gets cancer, then treatment options are limited because they can not be exposed to radiation, and are especially sensitive to many chemotherapy drugs. Ironic, eh?
Dr. A wants Manny to have MRI's every THREE OR FOUR MONTHS. What?! That did come as a blow to me. Dr. Reisner had previously mentioned getting an MRI in nine months, then yearly. Thats a lot of sedation, contrast dye and stress to pump into one little guy. Dr. A has recently joined the Aflac Cancer Center's team and came from a group in Chicago that has one of the premier NF1 clinics in the nation. She seems to have a TON of knowledge about NF1 and her interest and knowledge of the disease definitely goes beyond the blood, tumor and cancer boundaries. I am glad to have her as one of Manny's doctors, but this type of thoroughness is well.. a lot to take in.
Dr. A wants to keep an eye on the tumor on Manny's spine (the largest) for fast growth or changes that might mean cancer. Luckily, this tumor is not putting pressure on the spine or any of the soft tissue surrounding it. If it does begin to cause pain or pressure, it too would have to be removed or treated.
Dr. A also told me that getting Manny's eye exams every 6 months is extremely important. Tumors of the optical nerve, liche nodules and other things that could threaten Manny's ability to see may sometimes be quicker diagnosed by an ophthalmologist than by an MRI, or vice versa, thus saving a patent's sight.
We discussed my concerns with Manny's hearing. Although I KNOW that he can hear some things, and even responds to commands when his back is turned to me, he seems to not hear everything. His limited speech sounds much like a deaf person.. nasally. His word for "milk" being a very throaty "nnnguh", etc. Granny told me today that she and Pops were "testing" his hearing by making loud noises behind him, and, being the parents of a child born profoundly deaf, she is concerned about his hearing as well. Although the function of Manny's eardrums has been tested and no abnormalities found, Dr. A's response to what I told her was, "Yes, that is extremely concerning." Luckily, during Manny's next MRI, they will be able to do a very in-depth test of his hearing that will actually test how his brain is receiving signals. This is great news to me, to be able to know if he is hearing well, or if his lack of response is more of a social delay, like Jake exhibited near the same age.
Dr. A also wants to do a blood panel to check Manny's organ functions and for other abnormalities. This will also be done during the next MRI, which will be scheduled for next month. The risks involved in so many MRIs are, of course the risk of sedation, and the risk of kidney damage due to the contrast dye. We will be doing the next MRI next month, but I would very much like to discuss the frequency of these treatments during the next visit. Three or four MRIs per year is just a lot.
This is the part where I usually sum up how I feel about this particular visit. It was definitely more than I expected. The doctor made it clear that there is a spectrum of outcomes with this disease, but I was kind of made to feel like at least some of these things will become a reality in the near future. I guess I've sort of let myself believe, so far, that Manny's case will be less severe, and that maybe if we ignore these tumors.. they'll go away, and one day we'll laugh about that time they told us Manny has a "brain tumor" as he beats all odds and begins to develop normally. I really have trouble with this "wait and see" attitude that I'm supposed to have. It makes me crazy. I know that the cost of this care will put a greater strain on our family, but what are we supposed to do?
As I was leaving, a man, probably in his late 40's came to use the elevator at the same time as me. He had a badge and was some sort of employee of the hospital. We entered the elevator together. The first thing I noticed were his forearms, which were covered in small tumors that looked exactly like tiny beads, peas, and even regular sized marbles had been pushed just underneath the skin. Maybe 50 of these were visible on each forearm and his hands. Probably 30 or 40 more visible on his face and neck. He was otherwise a normal looking man. His speech was short and curt as I asked him what floor he would go to, and he looked at the ground. I tried not to stare but, of course, my eyes searched for cafe-au-lait spots. He had several on his neck and the top of his head. This man had NF1. It just so happened that he was parked near me so I followed him down the sidewalk and stairwell. He was shorter than me, maybe 5'3" and showed some difficulty walking down the stairs.. just a slight limp. Otherwise he went about finding his keys in his pocket, his parking ticket and going to his car like anyone else. The scene made an impression on me, thought I'm not sure I can describe it yet.
I've been grumpy and tired the rest of the day. I really just wanted to write down the facts of the day... I will remember more, I'm sure.
I viewed this video the evening that Dr. Michaels first pointed out Manny's cafe-au-lait spots and familiarized me with the term Neurofibromatosis. It horrified me, and now it inspires me.
