Tuesday, November 25, 2014

grayness

Dear Emmersyn,

       We are taught as children that things are right or wrong, black or white. It is always wrong to lie, no matter how big or small the lie may seem. Its always wrong to take something that does not belong to you. But one thing you will learn as you grow up, life is rarely black or white. I have always been a planner. So long as I had a plan in my head, I could make it happen. If things seemed stressful, I just needed more organization. When It was overwhelming to work full time and keep up with all you brother's sports, I bought a white board and organized every evening for months. Organization and control have always been a stress relief for me.
     NF is not black or white. NF is gray. Your living in the grayness, the uncertainty. There are over 6300 known gene mutations that cause NF1, and countless physical expressions. NF symptoms range from only a few cafĂ© au lait spots to the development of thousands of tumors to cancer and everything in between. Some NF complications include hypertension, seizures, cancer, learning disabilities, brain tumors, blindness, deafness, renal artery stenosis, pheochromocytoma, orthopedic concerns, etc. There are so many different components to NF there is no way to plan. Your future is a mystery. Of course everyone's future is a mystery. Bad things happen everyday. Healthy people become sick. But for you my dear, your future is likely to be rockier than most. For NF is a ticking time bomb. We will always have to watch for tumors, wait for the explosion, and pray for diffusion before too much damage is done. At just 2.5 Years of age, you have had  three MRIs, one surgery, extensive eye exams, two developmental evaluations, blood draws, Numerous physical therapy appointments, and chronic daily pain with physical limitations. Your life has not been easy. Your life will never be easy. I can not plan the stress away. I can not organize your pain away. Some days the sheer magnitude of your diagnosis, and the uncertainty of your future are too much to take. We have to take the bad days when they come. But we also must celebrate the good days. Currently I am trying to focus on the good things. Focus on your smile, your laugh, your sweet voice, your compassion, your heart, your love. Because all of the bad things that will happen in your life are worth it for just one smile and one kiss. You are loved more than you know, and you love with all of your heart. You are beautiful inside and out. You have purpose in this world. Your pain and difficulty has purpose. We may not know why or how, but God will use this for good. When life seems confusing and the grayness becomes overwhelming, focus on the light no matter how dim it may seem.

Love you always
Mom

Thursday, November 13, 2014

The good, the bad, and the ugly

Dear Emmersyn,
       The past two days have been hectic. In the midst of the first winter snow storm with temperatures of -19 degrees and falling snow, we drove the 4.5 hours to Denver for your MRI. We left 5.5 hours early, but we should have left about 6 hours early. We ended up being a little late for check in for your MRI. It all worked out in the end, and you actually went back for your scan at your scheduled time due in part to the amazing nurses at Children's Hospital Colorado. You did so great on the drive despite not being able to eat or drink anything. You were cooperative with the check in process, and didn't even cry when the nurse took your vitals (which is a great accomplishment for you). You went out easily, and I got to hold you until you were asleep. Then Daddy and I were ushered to the waiting room to wait until your 3 hour scan was over. Its never easy to leave you with complete strangers, and I always joke they should give me your dose of anti-anxiety meds and give me a bed to sleep in next to you until you wake up. You are so much stronger than me! About 2 hours and 45 minutes after kissing your face, the anesthesiologist came out to talk to us. They had planned on avoiding intubation this time and letting you breath on your own while under gentle sedation. Apparently you had different plans. You Coughed while being put under, and you coughed up a huge yellowish green glob of nastiness. Your Oxygen saturations dropped into the 60s and you had to be intubated after they aggressively sucked out your airway. The anesthesiologist was quite worried you may develop an aspiration pneumonia because of the episode, and she warned us to take you to the doctor with any sign of illness no matter how small. We were then taken back to see you in recovery. You were amazing and woke up very well this time (again another big accomplishment for you). We went to the Hotel for the night, and the waiting began. The following morning was the appointment with the Neuro-oncologist to get the results.
     The good news is you have no brain tumors or new tumors in your spine. You do have UBOs or unexplained bright objects in you brain. These do not cause any problems, but rather are just supportive of the NF1 diagnosis. You optic nerves are tortuous and the right optic nerve enhanced slightly with contrast. Dr. Foreman assured us that he did not agree with the radiologist's interpretation and that this did not represent an optic glioma. In fact he explained that all individuals with NF1 have tortuous optic nerves, and this finding on your MRI was only further support of your diagnosis and nothing more. He did however recommend that you have a dilated eye exam every 6 months until you are 16 instead of once a year until 8 as we were originally told.
    The bad news is that your current plexiform tumor continues to grow, although it is not growing fast enough to warrant treatment as all treatment options are strictly experimental right now. He further explained that while your tumor is operable, it would be a very complicated and lengthy surgery and all of the tumor may not be able to be resected. Plexiform tumors are aggressive and when disturbed, they tend to grow faster. By removing your tumor, we would likely cause significant nerve irritation which would lead to worsened pain, not improvement like we'd hoped. So my hopes of having the tumor removed so we could be done with it, were smashed. I knew that sounded too good to be true. The only redeeming part of the plexiform discussion was when he stated that the increased pain from your plexiform tumor is not from malignant transformation of the tumor. Also, your tumor should slow down in growth after you turn three, which is promising.
     Now for the ugly news. There is nothing right now we can do to fix your pain. Essentially, it is something you are going to live with. We discussed several nerve medications, but they all seemed to have side effects that are worse than your pain in the first place. Given you right leg pain, Dr. Foreman thought it was very likely you have a plexiform in your right hip or thigh that may be causing your right leg pain and weakness. You may also have a slight length discrepancy with your right leg being a little longer than your left. Your right leg is also slightly bigger which can be a sign of a tumor stimulating the nerves. He offered a scan to check it out, but stated that he did not recommend it since there was nothing really to do about it right now anyway. So the only thing I was hoping we could solve with this trip was your pain, and it seems like we are back at square one.
     There was so much to be thankful for with these scans, yet I remain disappointed that we could not alleviate your pain. It just proves that we have such a lack of knowledge into NF and plexiform tumors. Someday, through research there will be better treatment options for NF, and maybe even a cure. Until then, I will continue to fight for you and push for more to be done. This week, men landed on a moving comet in space, but we still have no real treatment options for the thousands of Americans suffering with NF. Despite your pain, you will continue to shine. There is nothing you cant do my love, and I will be here for you through it all.

Love you always
Mommy
   

Monday, November 10, 2014

Fake It 'til You Make It

Dear Emmersyn,

     Going through medical training, there was a statement our professors always told us; "fake it 'til you make it". What they were trying to instill in our class of nervous PA students was confidence and self assurance. Nobody wants to see a healthcare provider that is apprehensive, anxious, and lacking confidence. If you can't conquer confidence just yet, you put on a brave face and "fake" it. Eventually, what you will learn is that you never really had to "fake" it in the first place. The knowledge and the skill were there all along. You only needed to calm your fears and recognize the truth. It was never truly lack of self assurance nor lack of knowledge that tripped you up in the first place. It was simply an every growing fear. Fear of failure. Fear of making a mistake. Fear of feeling inadequate. By "faking" confidence, you take a little power away from that fear, until eventually fear has no more power.
    After five years of practice, I am now back to the "fake it 'til you make it" mentality. No longer with work of course, but with you and your medical care. Every time someone asks how I am doing, I answer "I am fine". They cannot see my inner turmoil. They cannot feel the knot in the pit of my stomach. They cannot hear the 'what ifs' running through my head. They cannot imagine the burden of my worry. They remark at how calm and together I seem, because they themselves would be freaking out right now if this was happening to their child. I just nod and smile, faking the calm with every forced smile and warm hug. Eventually, I will no longer have to "fake" the calm. Eventually this perceived calm will come somewhat naturally to me. Every time I put on a brave face and participate in life instead of letting the negative overcome me, I take away a little power from the fear. Every time I let myself enjoy your beautiful smile and contagious laugh instead of perseverating on your growing tumor, pain, or motor deficits, I take power from the fear.
    Last week, I referred to this feeling as a numbness, because I lacked a better understanding. I also did not know how it was becoming so easy for me to "fake" the calm leading up to your MRI in two days. Don't get me wrong, I have had you hospital bag and snacks packed for days. I have had  arrangement made for your brothers for weeks. I have toys packed and schedules set. I still have sleepless nights and moments of weakness. But the only way out of this never ending storm, is to focus on the calm and fake it 'til I make it. Keeping my faith bigger than my fears, and focusing on the calm leading into this week. Whatever this study reveals, whatever decisions are made, I know we are strong enough to make it through it.

Love you always
Mommy

Monday, November 3, 2014

In the midst of the storm

Dear Emmersyn,

       Today I woke up to see the snow falling peacefully to the ground. Now growing up in Wyoming, you will learn that there are very few times the wind is not blowing, so it is rare for snow to fall peacefully without getting blown around. As I stood there at the window watching the snow fall, it dawned on me how beautiful and peaceful the storm was in this moment. The traffic on the slick streets on the way to work this morning was definitely not peaceful, and the dreary day and overcast sky was not beautiful. But in that moment, I was reminded that in every storm there is still peace and beauty. You just have to look at it differently. I took a moment to just stand there in front of the window and watch the fluffy white flakes float softly to the ground. (And it may have made us all a little late this morning).
     With the MRI of your brain and spine and visit with NeuroOncology a little over a week away, I needed this gentle reminder. Storm clouds have been brewing, and I have been letting the winds of worry tear me apart. But with one morning snow storm, God reminded me to "Be still and know that I am God" Psalm 46:10. Today, we are enjoying the stillness of the storm and respite from the chaos.
      Never forget to see the beauty in life. Through every storm there is beauty, and in all chaos there is peace. If you cannot find it, try a different view, but keep looking. If nothing else, cultivate your own inner peace to be so strong, that no degree of chaos can ever disrupt it.

Love you Always
Mommy