Daley Ponderings

We had our appointment with the NF1/oncology doctor yesterday. It turns out the neurofibroma is even bigger than we thought. In addition to what we already knew, which is that it goes from her external ear where we can see into her middle ear, it also extends down under her ear into her parotid gland which is a salivary gland in the cheek. This is a plexiform neurofibroma, which is something that has actually been there since birth. And it has grown over time. (There are other neurofibromas that can come later possibly, but they are not the plexiforms.) This plexiform right now is benign, but it could potentially become malignant at some point. An initial screening has indicated that this one is one step towards becoming malignant, but they are still unsure of how many steps the body takes to get there.

Grace will be going on a medicine which is designed to shrink the tumor, and it may prevent some other kind of tumors. This medicine can have some unpleasant and even dangerous side effects, including diarrhea and rashes, vision impairment and heart issues. So Grace will be going in monthly and then every 3 months to check on all those functions. Right now, the plan is for her to take this medicine for 2 years, and then reevaluate, based on new research and her body's response to the drug.

One fascinating thing that I learned yesterday is that the longer hair on her right side that we have noticed since she was very young is related to this neurofibroma. The doctor said that because of extra vascular activity on that side it is not surprising that it would cause faster hair growth. When I heard that, I went back to my list of Grace's symptoms that we made before her NF1 JMML diagnosis while we were trying to figure out what was going on with her. I noted that she also would get redness on her right cheek when she ate sometimes. We thought at the time it was an allergic reaction, but it is likely due to the extra vascular activity. (It still happens sometimes.)

Please pray:

That Grace will be able to swallow the capsule (twice a day!)

That she will not have any serious side effects. We would also appreciate if she didn't have any other side effects. It's been really nice the last few months to not have to deal with diarrhea.

That the medicine will do its job, which is to shrink the tumor. I forgot to ask if her hearing loss is permanent or if it could come back with tumor shrinkage.

Some of you may be wondering ( I hear you Grandma!) why could they not see this in the imaging. The reason stated is that the plexiform neurofibromas are a soft and spongy tissue, and is easy to not notice them, and/or think that they are something else. The ENT even said that it's made of the same kind of tissue that your outer ear is made of anyway, so then it's really hard to distinguish.

I guess that's it for now. Here's a collage of pictures that Grace took of Hope in the grocery store:

One year ago today, our older kids got to see a total solar eclipse, and Grace came home! These annual milestones are flying by this year, and we are thankful. 

Grace's NF1/oncology doctor called Jon with biopsy results on Friday. It is the opposite of what we were hoping. Both biopsies indicate plexiform neurofibroma and it's really one big tumor that connects from the outside where we can see to the inside behind the eardrum. Surgery in this delicate area is not advised. We will have a meeting with the doctors Wednesday to discuss treatment. Some good news is that Jon has talked with other NF1 parents whose kids have done well on the medicine.

That news was a blow, but I am doing better after attending a prayer meeting on Friday and church on Sunday. I am holding onto the hope that God is still working and will heal her one way or another and in his timing.

Both Jon and I are going to the meeting tomorrow, so we can be clear on the options, side effects, pros, cons, etc.

Here is Grace with some presents from ladies at church: 

And helping Daddy with his project: