Thursday, January 30, 2014

Trust Your Gut

The world should stop but it doesn't. It doesn't seem fair that bills keep coming, food empties from the fridge and people demand things from you during a tragedy. Life should stop. You should have time to lick your wounds and spend time reorganizing what is left of your life. But alas, there is no rest for the weary. Anyone who has dealt with severe illness or a death in the family knows what I'm talking about. So we march onward.

Today we met with a genetics specialist who has a background in NF. She said we were correct not to jump into chemo. Just like I read in my own research she said chemo only pauses the tumor growth in 50% of patients. The typical NF tumor will stabilize and shrink on its own. Chemo has a laundry list of horrible side effects, some are permanent. So it is not to be taken lightly.

She wants us to get a VEP done every 3 months and another MRI in March so we can compare results over a period of time. While we wait she is researching other treatment options. For the first time I really feel like we found someone who is educated in NF, level headed and empathetic. I'm happy God sent her to us!

So the plan is more tests, compare results, research treatment options and pray Emma keeps her
Let's go Mama! 
eyesight. For now chemo is off the table and thank God for that!

A huge thank you to our amazing church, St. Peter's, who has been supporting us through prayer and aid. We are forever grateful for all of you! I also want to send out a huge thank you to all the wonderful mothers who have been there for us, you make day to day life possible!

Next stop is a VEP test. And the world spins madly on...

Wednesday, January 15, 2014

Treatment: A Fork in the Road

Treatment: A Fork in the Road

Its a nightmare, a very literal "damed if you do, damned if you don't" situation. Not cut and dry like stage 4 cancer, then you know chemo is your best or only course of action. No this is a terrible choice and my hope is Emma reads this one day so she knows we struggled with what to do.

Emma has a stage 1 optic nerve glioma, it is a noncancerous tumor which grew because of Emma's NF1 disorder. Typically these tumors receive zero treatment and are only monitored with a MRI every 3 months. Rarely these tumors undergo radiation or chemotherapy treatments or surgery. Surgery is not an option in our case, it is too invasive and risky.

Option 1 - chemotherapy
The Oncologist wants to use chemo drugs Carboplatin and Vincristine. She would receive treatment every week for a year. Because of her age and frequency of treatment she would need a Venous Access Device placed under skin that can feed a vein directly. Placing that device is a surgery. This type of chemo is not guaranteed to shrink, stop the tumor from growing or preserve her eye sight. If fact, a study I read done by John Hopkins University took 9 kids under 6 with NF1 optic nerve tumors and only 1 out of the 9 improved from chemo. Odds are if we do chemo Emma will still lose her eye sight in her right eye because of the pressure already placed by the tumor up to this point. Common side effects are constipation, stomach pain, hair loss, numbness in fingers and toes, muscle weakness, blurred vision, jaw pain, seizures, low blood count, nausea / vomiting, liver damage, kidney damage, diarrhea, rash / itchy skin, hearing loss (permanent) and low levels of nutrients in the blood like calcium and sodium. We also run a huge risk of having her undergo chemo for nothing.

Our Othulmologist today said that this type of cell in her tumor is a supporting cell and not a cancer cell so chemo may not be the best choice. His first choice is surgery but our Pediatric Neurologist
Emma making a few calls at our appt.
already said that surgery is too risky and invasive.

Option 2 - Wait and Monitor
Emma has to get a MRI every 3 months so it is possible to wait until March so we have two MRI scans over a period of time to compare and say yes it is growing or it is stable. She would need to get a VEP or Visual Evoked Potential exam to determine for sure that she has eyesight in that eye as of now. Her eyesight would be a ticking time bomb.

The Reality
No matter what we decide we are facing a future of one blind eye or possibly two since the nerve pathways cross in the back of her brain and could be taken over by the tumor. We have no guarantees, cures or answers. Only questions, appointments and tears.

The Decision
We have not decided which way we will go yet but we have decided to get a second opinion on chemotherapy and have Emma evaluated by a second Pediatric Neurologist who has more experience with NF1 patients.

What we Need
- if anyone has any helpful information or referrals specifically for NF1 optic nerve tumors please contact Anya.
- please do not bring up Emma going blind to us, we are too heartbroken to discuss it right now.
- please pray for our appointment with the Genetics team on the 30th
- please continue to distract us with play dates, phone calls and love.
- please excuse our "mess" both literal and emotional, we are all a little messy right now.

Thursday, January 9, 2014

When You Hit A Wall, Go Around.

Funny update today.

When we first saw the pediatric Othulmologist he referred us to UC Davis for treatment. Our back up choice was The Children's Hospital. He put the referral in and we waited a couple days to let it go through. An amazing office worker at our Othulmologist, Norma, and I began calling several times a day to make sure they received the referral and were working on scheduling an appointment. Finally after four days of this back and forth with UC Davis and being on hold for an hour an office worker told me in a rude, non understanding way, that the referral was on the doctors desk and even though it was marked "urgent" we will be contacted soon for an appointment but he was taking ten days off for the holidays.

Those who know me understand that all that was to me was a challenge to get my baby the care she needed, when she needed it even if it meant going some where else. So Norma and I sent the referral to Children's, they called me the next day on a Friday and we were in for an MRI that Tuesday.

Well guess who called today? Yes, UC Davis. She wanted to finally make that appointment for Emma. I told her in a nice voice, "this was an urgent matter involving a two year old child. I was not willing to wait for you so I transferred her to Children's. She has a brain tumor and we are glad we caught it. We will be staying there." Honestly, I was half expecting an apology for the delay since it has been an entire month since the referral was sent in but instead she said in a rather rude voice, "fine, what ever suits you." And then she hung up on me.

Why do I share this today? For two reasons, first, medical facilities are rarely in a hurry although often they should be. If I had waited Emma would have gone more then a month undiagnosed and untreated. It is important to be sweet and kind to everyone but unrelenting and when you run up against a wall then go around it! Second, I thought people should know that UC Davis may not be the best place to pursue medical care, enough said.

In other news, the essential oils I mentioned have visibly reduced the damage on Emma's eye. Her neurologist is pleased, gives us her blessing to continue the oils and she is hopeful that they are making a positive impact. I guess we will know for sure if the tumor has reduced in size at our next MRI.

I also registered Emma with The Children's Tumor Foundation which is working for a cure for Neorofibromatosis. I highly encourage anyone who reads this who suffers from NF to register, the more information they have and subjects to monitor will aid toward a cure for us all.

Monday, January 6, 2014

Small Step Forward

A quick update for all our loved friends and family praying for Emma,

- we had a consultation with our pediatrician on ways to support Emma. His suggestions were great but it is hard to find doctors who know about the methods mentioned so we will be doing some research to see if any of them are possible. One is particularly intriguing, it's called Insulin Potentiated Therapy. The basic idea is you intentionally drop the blood sugar to make the tumor cells venerable then do chemo. It is supposed to help you get "more bang for your buck" with each dose of chemotherapy. If anyone has any leads on this please contact us.

- all our phone calls to the hospital paid off, we got Emma into the Pediatric Neurooncologist on the 14th. That is a full two weeks sooner! Hopefully that appointment will answer some of our questions.

That's all for now.

Friday, January 3, 2014

Appointments and Red Tape

Take normal business hours, take away two of the five business days for a doctors schedule, coordinate two different offices so you go on the same day as per their instructions then add in a family of five and what do you have? A huge mess. It took a week and 10 phone calls to get our next appointment, the earliest day they can see Emma is the 30th. We are on call for an earlier day if someone cancels but now its a waiting game. Today I left a message for Emma's neurologist to make sure waiting that long is alright because I hate waiting. Hopefully we get in sooner.

At our next appointment we will meet with the Geneticist to discover which type of Neurofibromatosis she has in her little body. After that appointment we will walk the results down to the Neuropediatric Oncologist for a consult and to create a treatment plan.

There is so much red tape to deal with, everything from drama with the health insurance to doctor 1 will not do a consult until doctor 2 sees her but doctor 2 is booked until the 30th. In a flash I went from not even owning a cell phone to having one glued to me at all times.

This month the waiting will be really hard for Mom and Dad. We could really use some distraction. Please be understanding if we are randomly in a bad mood, we could have been on the phone all day with Blue Cross. Or if we cancel on you last minute, sometimes it is just too hard to get everyone dressed and out the door. Or if I forget to send you a thank you note, each day I have a list if things to do and I'm trying to properly thank everyone for his or her help but its hard to get everything done in a day. Please know we are thankful for all of you, the help you offer, the prayers you say and the hugs you give.

For the next couple weeks we could use help and prayer for the following,
- play dates: please keep us distracted. The shock is wearing off and we are beginning to face the reality of our situation. It is going to be difficult.
- help on or around the 30th: it takes a day to prep for the hospital so anyone who can help me the day before would be awesome and we need a Gracie play date on the 30th. If anyone is free on the 31st we may need friends to keep us in good spirits.
- prayer: please pray for a smooth month, a productive appointment and for peace and strength.

For now that's all I know, if we get in sooner I will update Emma's blog.