JT is not my Uncle Eric

We have been living in triage mode for the past week since being scared by the huge blotch of swelling on my MRI image at NIH. Now that I have been put on medication to control the situation and am able to look forward more then a week in my life, I have to deal with the rest of the news we received at NIH.

As self-centered as I have been over the weekend for personal preservation of my sanity, JT did in fact go to NIH for exams as well. Pretty much everyone in my NF2 family has started with spine tumors around the age of 9. I think all of us close to the situation had begun to mentally prepare for JT to have a spine tumor either this trip or in the next couple of years. Spine tumors are no walk in the park, and the idea of my baby's beautiful smooth brown back being cut into, the muscle being cut through, and his tiny baby spine being touched, makes me nauseous to the very pit of my stomach.

So on Thursday when JT saw the ENT at NIH he sat looking at his brain MRIs a bit too closely. I didn't like the look on his face, I didn't like how he kept switching from series to series throughout the scans, zooming in and out and rotating. Finally, he turned to us and told us that JT has an Acoustic Neuroma*. The ENT was very conservative with his diagnosis, stating that it could be a swollen blood vessel. Paul held my hand and held me back, because we have been together long enough that he knows what havoc "possible tumors" have wreaked throughout my life.

So I held it in, and I looked at JT and took a breath. I waited until we saw Dr. A the next day for our big final appointment, and I asked his opinion. Apparently, JT's hearing nerve is already swollen. That breaks my heart. Sometimes, these tumors grow on a nerve, other times like my own, they weave themselves in, making it virtually impossible to simply remove them. All of JT's hearing and balance tests came back great, and the images are so small, there is really nothing to do right now but wait for the next MRI to begin to gauge how aggressive this tumor is and what our response will be. Just waiting, and looking at my baby everyday wondering how long I will have him and how much he will suffer.

Every NF2 patient is affected completely differently, each genetic defect, each tumor, each treatment is completely independent from all prior history. That is what the doctors tell us. Those of us with familial NF2 have seen different. I have pretty much followed the same pattern as my Mom, and any regular Fabulous Running Mommy readers know that a recurring theme of mine is to remind myself that I am not my Mother. My Mother also had a brother, my Uncle Eric. I have fond memories of my Uncle Eric sprinkled with uncomfortable images that scared me as a child. My Great-Grandmother Leticia, who I called Titi, spent a large part of her life caring for my Uncle Eric and Grandma Norma as they lay in hospital beds in her home wasting away from NF2. She was

an amazing woman and dedicated her life to their comfort. I would visit, and I remember walking in as she situated my Uncle, and seeing his long pale limbs as she adjusted his blankets. He was so skinny, and completely hunched over from the spine tumors. Tumors that started at a very young age. He had eye tumors, and although imaging was not what it is now, he had Acoustic Neuromas at a young age as well. His eyes bulged from the tumors and he wore a patch over one, and I distinctly remember him being so tiny that when he sat in a wheelchair he didn't even fill the seat. He was as small as a child in that big wheelchair, with a grown man's mind trapped inside of him. He was fiercely intelligent, and in

his last years he found God through becoming a Jehovah's Witness. It filled his heart and although I have been told he was an angry person at times, I only remember him as loving and curious. When I was in 2nd grade the teacher called my name to go to the front office. For no apparent reason I thought my parents were pulling me out to take me to Disneyland. I was a ridiculous child, seriously. I was so excited, being called out of class was really special at that time, my parents both worked and I was happy to see them. I believe it was only my Dad who was there, which was a bit disconcerting, and he was way too solemn for a trip to Disneyland. Still, I was my usual chatty self, and talked his ear

off the entire way home, throwing question after question at him. When we got home I remember sitting on our white sofa across from he and my Mom, who was very quiet, and being told that my Uncle Eric had passed away. I don't remember any reaction, my memory goes blank for years after that. I knew what death was. My Grandma Norma had passed away a couple of years prior. I just remember being told he was gone, and knowing how young he was. In later years some of the blanks were filled in. I was told that at the service I was too young to attend all of his fellow Jehovah's Witness members from his hall filled the church, it was standing room only. His battle and faith were an inspiration to them all. To this day, knowing that about him, fills me with a pride for my Uncle who lived such a short and tragic life. He was only 27 when he passed, but he didn't curse his fate, he found God and filled himself with what he could. He never had a wife or children, o

r many friends until he joined the church. His childhood photos are slightly off, his eyes not quite staring straight ahead because of the tumors. Just like JT's.

JT is not my Uncle Eric. The fact that his tumors are starting to grow while he is so young does hint at a severe case, and that terrifies me to my core to look at my JT, my angel, and think of him going through the hell that is a severe case of NF2 before he even starts high school. Will he be deaf? Will he lose the rest of his vision in his good eye from the small tumor he has in that one as well? Will he have a paralyzed face and give me soft kisses that simply press upon my cheek without a pucker as my Mom's did? Will he struggle to communicate and make friends, and question the commitment of time involved in earning a college education when compared to the life span he may not be promised? Will he simply s

nap as I did and be lost, would he be lucky enough to find his way back as I did? Will he be able to work, and support a family, and have a wife who can handle having a husband she must care for? Will he have children and will they have NF2? Will he live long enough to see those possibilities?

And so it seems although JT and I live different paths, we end up on the same one for now. We simply can't worry about the future, we have to live for today, and love each other as fiercely as possible for as long as we can. Neither of us will be held back by long gone fears that really have no true hold over our current situation. We can learn from those before us, and be brave and filled with promise today.

*An Acoustic Neuroma is the hallmark tumor of NF2. These tumors grow bilaterally on the hearing, balance and facial nerves. They are why I am deaf and have horrible balance. They are why my Grandma, Uncle and Mom, as well as many of my best friends are Deaf. They are why so many of my loved ones and friends have facial paralysis and cannot smile. Treatment options include surgery, radiation, and hopefully medication if we all keep supporting research for a cure!

One Day at a Time

The next time I have potential Gamma Knife damage on a huge area of my brain, remind me to see my Gamma Knife doctor before freaking out. This morning my Dad took me down to Newport Beach with my scary MRI CD in hand to see Dr. Duma. Over the weekend I had a few episodes where my vision on the left side of my left eye got a bit... wobbly. I saw a black spot at one point. My headaches have been much better on the steroids, but I am still just tired and nauseous all the time. Of course, its hard to be tired on steroids, so I have been exhausted while filled with pent up energy that has fueled my anxiety. I was ready to get information from Dr. Duma today about the huge area of swelling around my right ventricle, and expecting the worst after Dr. A scared the bejeesus out of me last week in DC.

Dr. Duma came in, looked at my scans, asked questions, and let his resident do a neurological exam. He then told me that when I had Gamma Knife in February he treated both ventricle tumors as well as the tumor running down the center of my brain, and that unfortunately a healthy area of my brain had gotten a bit too much radiation as well. I asked how this happens, he said it just does, it's a known risk. I asked if it was radionecrosis, and he said he does not like that term because it means brain cells are dying, and they are not. (sigh of relief) He said the entire white area is edema (swelling) and that I should continue the steroids for about another week and the swelling "should" absolve itself within the month.

Excuse me? Did I misunderstand? Last week I was told this was a really dangerous thing on my brain and I could be facing surgery. That my vision fibers were involved. I had already heard from Dr. A Monday morning clarifying that the fibers involved were directly related to the left side of my vision, which was scary because of the weird shimmering I have been having to my left.

Dr. Duma believes the swelling in the ventricle is causing small visual seizures. He also said it is time for me to get on anti-seizure meds because I just have a lot of tumors, and any of them could cause a seizure, but specifically for my current situation it is the best course of action. He said the modern anti-seizure meds are not a problem to take and I should do fine on them. I have to take steroids for one more week, and got some Xanax to calm the heck down. While Dr. A wanted me to MRI again in 3 weeks, Dr. Duma said if my headaches don't come back then 6 months is fine. I asked if for my own mental health and well-being we can just MRI in 1 month to be sure this issue is under control and he agreed although he really didn't think it was a big deal! I reminded him he wasn't walking around with this in his brain but he pretended not to hear me, HAHA, I am teasing, I love Dr. Duma. I don't blame him or Gamma Knife for this ordeal. I blame the fact that I have NF2 and countless brain tumors, and treatment is never going to be perfect. I will still have Gamma Knife in the future when possible and will continue to avoid surgery.

I asked what daily activities could potentially trigger seizures or problems. He then dropped the bomb on the Fabulous Running Mommy, he said hard exercise is not recommended for patients in my type of situation. He basically said, don't get red in the face, but that I can listen to my body and find my balance. No more marathons? No more speed miles? No more weight lifting? I'd rather be slightly chubby and be able to see my children grow up then hurt myself trying to attain a level of fitness slightly out of my body's reach, but the idea of giving up such a large part of my active lifestyle is a bit disheartening.

So, I was sent home with some meds and a smile, and told not to worry. I trust Dr. Duma's opinion because he is a Gamma Knife doctor and sees this all the time, and he said it generally resolves. I've been so afraid all weekend, I am having a hard time believing it could be that easy. My plan is to take my meds and rest until the MRI, and then take baby steps from there. One day at a time is all any of us can do, but a lot can be accomplished in a day.

This is your brain on Gamma Knife

My life is as stagnated as this blog. In 6 weeks I have gone from getting up early every day to run my house, care for my kids, bang out routines at the gym, hang with friends and cook fresh healthy dinners from natural ingredients... to laying on my couch with a neck pillow, getting my chores done in increments between dizzy episodes. My life was simple, but it was mine, and I want it back.

Last week I flew out to NIH for my biannual MRI's. I assumed these headaches would be explained away by a sports injury. I would continue to nurse my neck and take Excedrin, focus on yoga and jogging, and then slowly ease my way back into my routine, back into my life.

Instead I was told that one of the brain tumors I had treated with Gamma Knife has doubled in size in the last 6 months and introduced to this image...

The bottom images are 6 months ago, and the top images are now. The left images show the tumors themselves, and the fact that they look black inside is good, tumor necrosis has set in. However, the images on the right show the swelling and fluid around the tumors.

Yes, that top right image is my brain now... and no I am not on drugs. Doesn't it remind you of those old anti-drug campaigns? "This is your brain... this is your brain on... radiation spillage?"

Yes, somehow, radiation spilled into my brain and a huge area of it is crazy swollen. I have no further information until my GK doctor gets me in this week so I can show him the MRI's.

The doctor I see at NIH for the NF2 Clinical History Study said "I am afraid for you." Honestly, that is a bit disconcerting coming from a man who sees worst-case-scenario patients on a daily basis. Apparently the swollen area has a bunch of nerves that control... vision. Yes, my vision is now at high risk.

I can deal with a lot, and I will continue to do so. I cannot even fathom being blind and deaf but the idea terrifies me. I think of how hysterical I was when the power went out and I couldn't find Paul for a few minutes. Everything was pitch black and no matter how loud I called out I had no idea if anyone was coming for me. I sat down and curled into a ball until I felt Paul's hands touch me, and then I just hid in his arms until he lit a candle and shadows lit up the walls. How would it feel to never escape that darkness?

I have to deal with this as I always do, I compartmentalize, and put things away, and just don't deal with them. Dwelling on a hypothetical situation is a waste of time, and I have half-convinced myself that I am being dramatic, and that any day now I will get my life back. But as day turns to night and another day of baby stepping through my chores passes, I can't help but glance at the fear edging my mind as I go about my business.

So, I am on steroids to hopefully bring down the swelling. I am to see Dr. Duma ASAP and have another MRI in 3 weeks. If the swelling or radiation has spread, then I am proverbially f*cked. At that point we would discuss "options" and in the meantime I have been sent home to let my radiation... marinate? I am a total hermit as of now, I am afraid to drive in case I have a dizzy spell or just suddenly go blind. I am bored and feel guilty for trapping the kids when they should be out enjoying their summer vacation. I am consciously forcing myself not to lay all of this on Paul, and trying to do nice things for him whenever I can because I don't want our family to revolve around me, and also because he is an amazing person and pleasing him makes me happy. So today I rest, and come Monday I don't care how I feel I am going to get a little piece of my life back. One day at a time, I will get it back.