A year and a half ago our busy (happy) lives changed forever. Our healthy "seemingly perfect" 4 your old son (first born of 3) went to a routine eye doctor appointment and just like that we began our medical journey (or more accurately medical nightmare) with neurofibramatosis type 2 - nf-2 for short (a mouth full right).
So you can google nf2 (I spent almost 9 months on the internet night and day- LITTERALLY) I missed so much doing that and what I learned was NOTHING of relevance to Raphael. I watched and cheered my 6 month old daughters first time sitting up but in reality I wasn't there and I will always regret that. Good thing I'm a photographer so I have the memories I mentally missed in those moments but even that sometimes that makes my guilt worse.
Every nf case is different. There is no way to predict the future and every thing that comes up will have a different route of options. I have learned the names of so many doctors. Neurologists, neuro
ophthalmologists, oncologists, ENT's, speech specialists, swallow therapists, pulmonary specialists... the list is literally endless. And when I say list I mean specific names of doctors and hospitals of practice in multiple states and countries. NF has become my life, my obsession my indescribable guttural pain that I wake and sleep with.
NF is weird and scary and wildly misunderstood. NF is a medical mystery. There is no cure for NF. There is no right answer for how to move forward with treatment options because there is no data. There is no doctor that has the answers. Every decision we make could be the wrong choice. NF makes every ounce of my being flooded with guilt that this is my sons reality. There are very few NF specialists in the world and they all have conflicting opinions. NF is my nightmare. Making it more of a nightmare is only the fact that I wish I had it instead of my baby.
My good friend who I met thru the small nf community has a gorgeous brilliant college aged daughter with nf-2 and I read something she wrote once that brought me to my tears. She said that she was partaking in a clinical trial in an effort save her 1 and last hearing ear from her bilateral vestibular schwanoma (another term I've become to familiar with , I can name tons of tumors) which was growing with no known reason or way to stop. She was handed 2 pills by a nurse fully garbed in a suit, mask and 2 sets of gloves who handed her the (?) pills . I think of this often when I feel fat or I hate my shirt or have jealousy over my friends apartments and vacations. This grounds me and she and her mother are my heroes. I can not imagine the fright that this entailed for their whole family and am also grateful for their trust in their doctors and willingness to help nf get a little closer to a cure. Will Raphael be be the next guinea pig? Sadly, that is our hope. That makes me want to cry too though if you know me it doesn't take much for the flood gates to open.
In the last year and a half we have put Raphael under anestesia over 20 times tracking multiple lesions on his brain. He is completely blind in his left eye (blackness) due to a tumor that has rotted his optic nerve. That tumor is growing in multiple directions (back towards his brain, to the right towards his seeing eye and forward towards his blind eye threatening to literally push it out of his face (gross but reality). If we do nothing we should go to jail. Every scenario is horrific. We have to stop the tumor and soon. He also has tumors corroding both of his hearing nerves but those are small and we are told not to act on them yet - hoping science catches up to the growth. 95% of nf2 patients to date end up stone cold deaf. Can you even imagine reading this data on google pertaining to your perfect, sweet, charming athletic little boy? Sorry for the pity party. In all honesty he's also a pain in the ass like all children but having 3, the tantrums and general 6 year old behavior just make me joyful that he is living his life with no boundaries.
We were given 2 options. Brain surgery - craniotomy to be specific (use your imagination) or a new type of radiation called proton therapy. NF specialists worldwide have maintained that radiating nf tumors might lead to malignancies later in life (in the brain). Proton therapy is site specific - the beams start and stop at the site of the tumor. Data out of the states has not shown the above (future malignancies) to be the case. Brain surgery comes with the risk of among multitudes of issues, losing a eye, personality changes and worse, not being able to get the whole tumor plus possible infection and the need for multiple brain surgeries and eventually radiating anyway. I cried and sobbed when our loved and trusted neurologist agreed that there was no right answer. Eventually we decided to go the radiation route after speaking with a highly respected nf neurosurgeon. 1 thing I've learned thru this journey is surgeons love performing surgery. Well when our dream neuro surgeon wasn't convinced surgery would be successful, our minds were made up.
So we are about to embark on Proton therapy. Our oncologist who we found serendipitously (a story for a later blog) has worked with nf patients for upwards of 20 years and has never had a problem with future malignancies with his nf patients. What proton therapy entails is withdrawing Raphael from kindergarten, driving him 2 hours every morning at the crack, putting him to sleep and then zapping beams thru his eye to his skull and driving 2 hours back home to pick up his brother and sister and try to continue a normal routine (whatever that means) 5 days a week for 6 weeks. He will go to sleep in a special room in my arms every day and wake up the same way. He will never see the scary machine which literally made my arms and legs weak. He will be treated with kindness and get used the the routine. He will have a graduation ceremony with medals and bells. I am at peace with my decision.
Now, what I really want to share is that we are no different than anyone else. My daily challenges remain the same though sometimes derailed by doctors. I have 3 children all delightful and challenging in their own right. Charlie (a girl) just turned 2, Gus is 4 and Raphael a week past 6. Our lives are fun, hectic and hard in the same ways most families are. Tonight, getting used to special treatment, when I wouldn't buy Raphael another pokemon card set he told me he didn't love me and would rather be in a orphanage like Annie. At the same time Charlie was clinging to me begging for m'ms (she learned about them last week) and Gus was crying because he sensed I was sad and was upset that raphael's birthday was last week and his "not forever or ever or ever" (it's in november). Thru their sobs, I realized life is stressful no matter what and at the very least how lucky am I to have a husband I am deeply in love with and kids I think about every second of my life. It could be worse. We go to so many hospitals and see children that I look at and know in my gut they are not going home. It brings me huge perspective. I get to take Raphael home every day and he is happy and crazy as all the kids in his class.
Our journey is gonna suck but we will live to the fullest and love one another and feel grateful for our family.
End note, Raphael eventually crawled on my lap and told me he was lying and I'm the only mommy he ever wants. He then asked for a tight squeeze. I must say, I feel pretty lucky.
I will continue to update here for those interested and am scared as well as grateful for the platform.
If you wish to make a donation on Raphael's behalf here is a link to do so. new link to follow
http://ctf.kintera.org/faf/donorReg/donorPledge.asp?ievent=1128644&supid=411039010
xxx Lara
