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.

Background
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.