Olivia - NF1 Update

Last week Olivia had an MRI to check the tumors on her optic nerves and the new tumor near her cerebral aqueduct and last night I spoke with her genetics specialist about the MRI report and a few other concerns from our April visit. 

First concern: Tumors- 

Optic Pathway Tumors - remain unchanged and stable, we will continue to monitor them in future MRIs. 

Cerebral Aqueduct Turmor - appears slightly larger, however the image is a different cut (me trying to explain what that means: in an MRI, images are taken in layers every 1/2 cm or so, and its hard to match up exact layers from previous MRIs).  They aren't certain if the volume of the tumor has increased or if it appears larger in this different cut.  Dr. Viskochil plans on discussing with the radiology team and we will be doing another MRI in 3 months to check on the tumors again. 

Next concern: Growth Velocity-

From the notes of our last visit with Dr. Viskochil: "a height of 114.3cm (>97th centile). Her height reflects a growth velocity of 1.35cm/month or 16.2cm/year, which is highter that we typically see in NF1 . . . It is notable that she has tall stature, which is unusal for NF1.  More important is her growth velocity that I only noted after the visit while plotting her growth parameters.  It is possible that the hypothalmic-pituitary axis is stimulated by the gliomas, and I recommend ongoing close attention to her growth and pubertal status."

As this says, this is something he noticed after we had left and we didn't learn about it until we had received the letter with the appointment notes. It came as a bit of a surprise and caused a bit of worry (mostly for me).  In some cases of NF1, gliomas in the brain can put pressure on the hypothalmus and pituitary gland and possibly cause early puberty (precocious puberty). 

Fortunately, a couple weeks later Sophie had a well-child visit and I took Olivia with me so we could discuss the letter with our family doctor.  Dr. Daynes measured Olivia on both of the office's wall rulers) and measured her at 110.5cm each time.  He wrote a letter and faxed it, as well as her growth charts from her last visits with him, to Dr. Viskochil. 

Last night when we discussed this the first thing he asked was "Did we see you at the Riverton center?" which is where I scheduled the appointment because its only a 10 minute drive instead of an hour.  Well, the nurses there aren't familiar with how important growth measurements are to these type of conditions so its likely there was a mistake made when they measured her.  In addition to this, Olivia has always been in the 95th+ percentile for height and she comes from a tall family. 

Because of the tumors we will continue to monitor their possible affect on the pituitary and hypothalmus, but the latest MRI shows they look good , so early puberty is NOT a current concern.  YES, I love it when we don't need to be worried about something.

Last concern: Speech-

Following our April visit, we scheduled a speech assessment for Olivia, which we did in May, as well as an audio assessment.  She does need speech therapy.  She is on a waiting list to begin this hopefully soon with Primary Children's.  Unfortunately, our health insurance has denied our claims because they feel it is a development issue and not related to injury or illness.  I know what your thinking - WHAT?! According to Dr. Viskochil, studies show that ~90% of kids with NF have speech delays.  Anyways, Dr. Viskochil will be sending them a letter as well as several medical studies and published journal articles that will hopefully help them see the error of their ways :)  Well, we'll see what happens.