Olivia & NF1 Update

 Well, so. . . . here it is: 

These are the results from the National Cancer Institute (NCI) on the volumetric measurements of the plexiform neurofibroma growing in Olivia's left cheek/neck/ear area- pictures.

The vertical axis is the volume of the plexiform in milliliters The horizontal axis is time in months. The first dot represents the first MRI the NCI measured way back in February 2013 at 19.2 mL and the last dot is the MRI from December 2015 at 38.0 mL. So basically it doubled in size over 2 years and 10 months. 

What about more recents data? Well, we can't use the MRI Olivia did in July 2016 for a more recent update, because {for whatever lame reason} the radiology department didn't do a complete scan of Olivia's *only* plexiform currently going through a growth phase & being monitored- cutting it off right in the middle, even though the orders were identical to the orders of the last year. {deep calming breaths} So, Olivia will do another MRI in October- between 2 family trips & Kurt's lasik surgery. 

And then, we hope, it gets sent off to the NCI in a timely manner. 

And then, we hope, we get results in a timely manner. {Right} 

And then, if it still seems like it's growing at a consistent pace we'll look at treatment possibilities, likely medical chemotherapy trials of some sort, depending on what's available. 

So, other stuff: 

The latest MRI also found another low-grade glioma in her brain, but no need to worry about it unless it starts causing problems. {Stuff like that comes out of my mouth and it seems so surreal. Am I actually saying- and really feeling calm- that we don't need to worry about a brain tumor???}

Also, the other plexiform on her right eyelid is stable, as are the ones on her collar bone. {But, how can we know they are actually stable? The radiology department has been saying the growing plexiform was stable for the last 3.5+ years when the NCI's more precise measurements show it's actually growing consistently over time and doubled in size??? And I can look at her face and see it's been growing! Argh}

Optic nerve gliomas are stable {oh, that word again}, however she is experiencing loss of color vision in her left eye. As in, last time she did the test she was able to identify about half the numbers in the color vision test with her left eye, and just last week, she couldn't identify any of them. But the good eye doctor says he's ready to push out her visits to once a year, since the optic gliomas are stable {oh boy} and optic atrophy in the left optic nerve is stable {kill me now}. 

Olivia still has light sensitivity & frequent migraines- can you tell in the picture above? I feel a headache coming on just looking at the painful look on her face. We incorporate a lot of brain health guidelines like getting good sleep, eating well, exercising, vitamins, learning new things, etc., and hope that it all makes some kind of difference. 

In the last update, I talked about how Olivia had made some great strides in physical health & strength, but then she spent January through March sick with every Tom, Dick, & Harry illness that came through. Ever since then, she has been extra fatigued and has really struggled to regain her strength. She's been taking naps, which is way weird. We took a break from all outpatient therapies this summer and it's time to get back to PT. 

The fatigue issue may be related to sleep troubles. There is some concern that Olivia's tonsils may now be blocking her airway- last year when she had surgery the doctor left her tonsils in because they were supporting the palate and now they are maybe bigger and blocking the airway. Olivia did a sleep study last week and meets with the ENT next week. So more to come on that....

The fatigue issue may also be related to food/taste troubles. Olivia has lost some weight and has had changes to her food-tastes, which may be back to that growing plexiform in her left cheek/neck/ear area which has infiltrated all her glands and possibly made food taste differently to her. This plexiform isn't interfering with her airway at all, but may be causing other kinds of problems. {Or maybe she's just a 9 year old girl who has decided she doesn't like any of the foods she's eaten since the beginning of time- both options seem reasonable.} 

So that's mostly everything with Olivia & NF1 right now. 

Thanks for all your love & prayers & support!  

Olivia can do her school work sitting like this:

Or she can do it like this:

Guess which one is better for her back?

I'm being eaten by a grizzly bear!

Cupid's Undie Run. Lots of fun with great friends. 

I am wearing underwear. 

And my awkward adult onesie pajamas. 

More Appointments

Olivia Update - Jan 2016

Left Ear/Neck Plexiform Neurofibroma

Nothing from the National Cancer Institute (NCI) yet - they are doing volumetric measurements of of this tumor, which is still going through an active growth phase. Hopefully results will come soon. Finally.

The bigger red circles are around the plexiform in Olivia's left ear/neck- the two pictures are different frames from her last MRI. The dark mouse-like shape in the left picture is Olivia's throat & the picture on the right is closer to her spine. 

Even without the NCI's measurements, we are certain the plexiform is still growing. It's become more obvious when looking at Olivia's {beautiful} face. However, we won't be doing any treatment at this time. Surgery is not an option- tumors grow on nerves which would cause facial paralysis if damaged. It's not interfering with her mouth or throat. It causes pain, but not constant or extreme. It is causing her ear canal to narrow and should it close, then we would do a debulking surgery to open it up. 

After reviewing different chemo options with Olivia's doctor we feel like they aren't an option at this time- results of past studies aren't promising enough & scary side-effects. There is a MEK-inhibitor trial going on right now where some kids are seeing excellent results- reduction of tumor size which hasn't really happened before. However, Olivia's doctor feels it's better to see what happens with puberty over the next 3-5 years and the medical community can get a better look at how the MEK-inhibitors work and in that time there may be other drugs in the same MEK-family that come up & work better. 

Soooooo......... wait & watch, watch & wait....... and fret & worry. 

{{{puberty causes hormone surges and this causes tumors to grow - not a good time for NF kids}}}

Other plexiforms:

The smaller red circle in the left picture is another plexiform growing on a nerve in the collarbone area- there is some concern this is causing a slight weakness in Olivia's left arm & hand- we've been watching it along with everything else.

Olivia's left eyelid plexiform does seem like it is growing as well- the eyelid looks a little fuller, but it's not pushing the eyelid over her pupil when she looks straight ahead & she's not tipping her head back to see better. We check in with her facial surgeon this week, but it's unlikely we'll do anything about it. 

Light-sensitivity & Migraines 

Olivia eyes are extremely sensitive to light causing headaches, and she gets headaches more frequently anyways, and nasty migraines more frequently. We went to a neuro-opthamologist and essentially Olivia has an NF brain and a migraine brain- a double whammy of bad headaches & migraines. They were willing to prescribe a stronger medication, but so far ibuprofen works well when needed. 

Otherwise we do what we can to help her have a healthy brain- eat well, sleep well, exercise daily, and so on. We also keep sunglasses & ibuprofen with us all the time- just in case.

 

Physical therapy & orthopedics 

The x-ray shows Olivia's scoliosis. 13 degrees is minimal- they don't look at bracing until it's 20-30 degrees. With NF, there is a bad kind of scoliosis and a less-bad kind. Because Olivia's vertebrates are looking good (edges aren't crumbling) and she's older than when the bad kind typically shows up, the doctors believe she has the less-bad kind. This is another thing that could be hit hard with puberty & all that bone growing that goes on. 

Olivia recently finished up a round of PT. In addition to weekly swim lessons, more swimming, and gymnastics, this has helped target some of her weaker areas. Over the Christmas break Olivia did a complete cartwheel- hand, hand, feet - and stayed on her feet at the end, and she also recently passed the green level of swim school. She has been working on both of these for over a year- amazing accomplishments!!!

ENT & Speech

I don't remember if I posted about Olivia's surgery follow-up with the ENT, but it happened a couple months ago. Everything was healed and looked great. We feel like her speech has improved and the numbers on her testing were way better. Now that she's on a PT break, she'll start speech again. There will be some pronunciation work, as well as language work. 

Learning

Olivia does have learning challenges. She did follow-up neuro-psych testing this past fall- we do them every 3 years. The results help me have an idea of her weaker areas, as well as give me learning strategies to help her learn best. But she is always making progress and that's the most important thing!

I think that's all the nitty-gritty mumbo-jumbo

What else? Olivia is fabulous! 

She runs & plays & swims & laughs & cries & fights like a typical 9 year old. She loves putting together outfits. She's always begging someone to play a card game with her. She likes to get her list of daily things done early so she has the rest of the day to do what she wants. She watches hockey with Kurt and nature shows with me. Reads with Austin and draws with Nyah and has the most imaginative games with Jolie & Sophie. She remembers all the details about where we're going and what we need to take and when we have to leave. 

We love our Miss Olivia!!

Salt Lake NF Walk 2015

We ended our staycation with the Salt Lake NF Walk. It was a beautiful day!

Olivia Update

For those who are new to Olivia's NF1 journey-
Learn about NF
Facts & Statistics
Diagnosis of NF1
&
Olivia's Story

Recovering after surgery

In the beginning of June, Olivia had a sphincter pharyngoplasty surgery- hopefully to fix her palate which didn't completely close. This was most noticeable when Olivia talked- she lost a lot of air out her nose, sounded very nasally, and was difficult to understand. But it really was a problem when she did anything where she needed to breath- running, playing, eating- so, you know, pretty much everything. She couldn't even build up enough pressure to blow a raspberry! 

The week after the surgery she had a nasty sore throat- not strep, but a red, inflamed, painful throat all the same. And then a week after that is when she broke her teeth dancing in the shower. So not a great month, but she is feeling much better. As for whether or not the surgery is successful, that still remains to be seen. She has a post-op appointment this week, but it's mostly to check on her throat's healing. They won't retest her speaking or scope her throat for another 3 months, so she gets a nice long break from speech appointments & daily practice. 

Other areas of concerns- 

Olivia was horrified to find a fox skin- her favorite animals!

For the last year Olivia has been super sensitive to light. It makes her eyes hurt, and she often wears sunglasses indoors or covers her eyes. Its not just bright light, but everyday, normal light- although bright lights are more painful. It seems worse when she's congested due to allergies or a cold, but it is a constant problem. So far her opthamalogist and other eye specialists don't have specific answers, so we are investigating with her geneticist & possibly a neurologist.

We don't know the status of Olivia's plexiform neurofibroma in her left neck/ear area. It was growing at about a 16% growth rate as of last year. She had an MRI in December 2014, but the woman responsible for forwarding the scans to the National Cancer Institute had moved on to a new job, so it wasn't sent and we didn't realize it wasn't sent until about a month ago. So, hopefully, now its been sent off and we'll hear back on that soonish. Note to self: call this week to find out. Olivia is scheduled for her next MRI this week. In addition to the plexiform in her neck & optic pathway gliomas, they are also checking on her vocal chords. In the pre-surgery exams, the ENT also noticed her vocal chords and throat were more sluggish on the right side, which may mean there is something growing on those nerves as well. 

Hiking near home

Physically, Olivia still gets pretty worn out by daily life. My goal for her this summer was to build her strength so she could have better breath support and not be worn out by basic day-to-day activities. For example, one goal was for her to ride her bike to the top of the hill on our street. It's not a big hill, but she's only able to go about halfway up so we started riding bikes every day. Obviously my goals for the summer were put on hold when we decided to go forward with the surgery, but now that she's mostly recovered we are back to focusing on her physical strength. She needs 2 30-60 minute exercise sessions a day of really focused exercise, not just playing around. We try to do a variety of things so it's fun- physical therapy exercises together, swimming, riding her bike, hiking, exercise classes or swim lessons at the rec center. It takes a lot of time, but it makes such a difference to how she feels and what she's able to do!

Olivia's Appointments

Eye appointment - Olivia's vision is great (20/20), but she's still super-sensitive to lights. They did a couple extra tests, but there's no good indication of why she's having such a hard time with bright lights. 

Speech is going well overall.

Having fun at physical therapy.

Still no word from the National Cancer Institute on her last MRI. 

Olivia's NF1 Update

What is going on with Olivia these days? 

For the most part, Olivia continues to be her fabulous, amazing, wonderful 7-almost-8 year old self. She spends her days playing with her sisters, coloring and drawing, snuggling George, and always looking for ways to help out. 

Unfortunately, there are a number of physical health concerns going on right now. She is getting headaches quite a bit more frequently (3-4 times a week), she is more severely worn out by activities that were once normal like taking walks or riding her bike, her speech continues to be difficult to understand, and there is some weakness in her left arm/hand the doctor is concerned about.

Her MRI in July showed stable optic gliomas and tectal glioma. The plexiform in her left ear/jaw area continues to grow, although not yet at a rate where we feel treatment is necessary. She is getting another MRI this month to continue to check the growth of her left ear/jaw plexiform, but it has also been extended to include her brachial plexus. When we saw her genetics doctor in October and he felt she had some weakness in her left hand, but was not able to feel anything abnormal in her arm, so the MRI will check the nerves to see if there is anything to be concerned about.

We were also concerned by what appeared to be an unevenness in her legs, so Olivia had some x-rays. She has a slight 1 cm pelvic tilt and an 11 degree curvature between T7 and L3 vertebrae (scoliosis). There is no degeneration of the vertebrae and the doctor doesn't usually become concerned about the curvature until is is 20 degrees or more.

In an effort to figure out why Olivia is worn out by activities that were once normal, she did a sleep study and had some blood drawn for testing. The sleep study showed normal sleep patterns and there was nothing amiss with her labs. Her exhaustion is mostly likely general NF1 related, but what does that mean we do? About 4 years ago when Olivia had a similar problem (too inactive due to the winter weather and was worn out by daily activities), we stepped up her level of physical activity and this strengthened her body to the point she was able to resume a normal activity level. So, we're making daily physical activity a higher priority to see if that will help - this means daily visits to our local rec center for swimming and alternating with a kids' fitness class, but fortunately, this is something all of us will benefit from doing!

Olivia will also be getting a physical therapy assessment to see if focusing on her core strength and breathing will improve her speech. In the past, the ENT has not found any physical defects to be responsible for her speech struggles, and we'll be doing another visit with him this month, but don't expect to find anything has changed. Speech therapy continues to help with her articulation problems, but is not helping her overall intelligibility. This month Olivia will also be getting a physical therapy assessment to see if she could use some help there by focusing on her core strength and breathing to improve her speech. 

So lots of concerns right now, lots of things we're watching, and not really much within our radius of control. Please keep Olivia in your prayers this month- lots of appointments & her MRI. 

Olivia MRI

Austin & Olivia were playing tic-tac-toe while we were waiting. Austin beat her two games, and then I guess he wasn't paying close enough attention and she beat him. It was so cute when he realized what had happened. He blushed and got this huge grin on his face like he just couldn't believe it. Olivia was thrilled. 

No results yet. It was quite a bit longer than past scans (1h45m vs. 45m-1h), as they were looking more closely at her optic gliomas and the plexiform. It is a little concerning that it took so much longer, but no need to borrow trouble. 

NF Women's Day

I attended our yearly NF Women's Day - a support meeting for women who have NF or who have someone in their family with NF. It was wonderful to see old friends and make new ones.

Update: Olivia & NF1

A problem is not a problem unless its a problem. 

That's my new mantra these days and it's especially applicable to Olivia & NF1. 

Olivia has had a busy month. 

Every time we meet with one of her specialists, we learn something new. They tend to say things like "And, of course, she has all these deep tissue neurofibromas, but I'm sure you knew about those." 

Um, no, not really. No, we didn't. 

"Well, we don't need to worry about them, because they aren't doing anything."

And they are right, a problem is not a problem unless its a problem. 

We met with Olivia's genetics doctor to go over the National Cancer Institute's (NCI) results:

Understanding the results: 

These images are of the plexiform neurofibroma in Olivia's left ear/neck area from the 3 MRIs she did in 2013. The plexiform is the brighter grayish area on the right side of the images.

(1) There was a 16% growth rate from 2/2013 to 7/2013. 

(2) There was a 32% growth rate from 2/2013 to 12/2013, or another way of looking at it, a 16% growth rate from 7/2013 to 12/2013. 

So it's growing and continuing to grow. But it's growing under 20%, which is sort of the cut-off rate of needing to do treatment, and it's not interfering with her airways, arteries, eating abilities, hearing, etc. 

But it is growing, continuing to grow, so another MRI in June.

And it seemed like she was having trouble with some tongue movements, so it's back to her ENT. The other ENT. Do people have 2 ENTs? She also has 2 opthamologists. So hard to keep up. 

Then we met with the oncologist to discuss possible treatment options. It was agreed that treatment is not needed at this time, but it's good to be knowledgeable of possibilities, even if they will probably change in the next year, 6 months, weeks, days.

Then we met with an opthamologist/facial surgeon to discuss a different plexiform above Olivia's right eye that also appears to be going through an active growth phase- again knowing that surgery is not necessary at this time, but it's good for the doctor and us to have an idea of where the baseline is at. 

But then I hear new things, like the one above.

Like this- "She has a slight turning in of her right eye, which may be caused by the optic glioma on her optic pathway tumor."

"But I'll let so-and-so and so-and-so (other specialists we didn't have any plans of seeing anytime soon) take care of addressing that."

And "she does seem to have a slight loss of vision in her left eye." 

"But again, I'll let so-and-so and so-and-so take care of addressing that."

 And so we have more appointments this summer with so-and-so and so-and-so.

And I just tell myself, "A problem is not a problem unless its a problem."

And I know, without a shadow of doubt, that Olivia is in God's hands. That he loves her and is watching over her, from little things to big things.