Monday, November 25, 2019

I'm Stronger Than Yesterday (My Bones at Least)!

Well hello there blog readers, welcome to my latest and greatest update!

I now have been on hormone replacement therapy for almost 2 years, and it was about time we checked in to make sure it was doing what it was supposed to be doing... making my bones stronger!

Two years ago we incidentally found that my estrogen levels sucked (to be frank), and that something needed to be done ASAP to help preserve my bone density. Typically, bone density peaks when one is in their mid twenties, and then gradually declines from there. We found out on a bone density scan that my bone density also sucked for my age, and my endocrinologist wanted me placed on estrogen immediately to preserve what bone density I had left and to hopefully build up some more before I started my "natural" decline (because lets face it, I am getting older).

Well folks, today I went and saw my family doctor to get the results I've been anxiously waiting for since I started on this journey two years ago. I was pleasantly surprised when he printed out the report that said my bone density had "exceptionally improved" since my last scan! INSERT HAPPY DANCE HERE! That means that the estrogen is doing its job, and that my bones are literally stronger than they were 2 years ago! HOORAY!

If any of you have been following my journey, you would know that the estrogen I'm on has caused a lot of really unpleasant side effects. Bloating to the point I look pregnant, huge mood swings, headaches, cramping, cystic acne... the list kinda keeps going on and on. We've gradually increased my dose over the last 2 years as my endocrinologist didn't want to "shock" my system with an overload of estrogen. Each time I've increased the amount I'm taking, the symptoms tend to get worse. However, it's such a relief now to see tangible evidence that all this suffering is actually worth it!!!

The initial plan was to bump up my dose one more time (to the max dose one should ever be on), but now I'm hoping he will let me stay on the dose that I am currently taking! Knowing that my bones are actually stronger and I am no longer high risk for the development of early onset osteoporosis makes all the symptoms worth it... seriously!

I will likely see my endocrinologist again in January, I was actually supposed to see him today but he is unexpectedly away for 6 weeks... so once I get in to see him we will continue to talk about dosing and my next steps moving forward. Staying on estrogen for life is likely my best option, as my brain just doesn't communicate well enough with all its parts to send all the hormones to the right places (darn brain tumour), but if there's the slightest chance he'll take me off of it or taper my dose down, I would be over the moon excited!

So there you have it folks, everything is seemingly stable again and I will just keep trucking forward! I've learned over the years that I can complain and whine all I want about what is going on in my life, but ultimately that will not cure me or make me "better". Unfortunately this is something I will deal with for the rest of my life so I might as well make the best of it, right?!

Have a great week friends!

Court
xoxo




Friday, November 1, 2019

Oh My Heart (and Ribs)!

Hellllllo there blogging friends! It's me, here with another health update for you! It's been a while since I've posted anything in regards to my current health status, so here we go!

A few weeks ago I went and had my "annual physical" with my family doctor. I honestly wasn't expecting much to come out of this appointment since I see doctors all year long and they all keep a pretty close eye on my health. However, that wasn't necessarily the case!

When my family doctor listened to my heart, he took a really long listen. I knew I had an existing heart murmur, but nobody has ever seemed remotely concerned about it. Last time it was brought up (with a different physician) it was brushed under the rug and not talked about again. I told him I was aware of the murmur and expected that to be the end of the conversation. He then told me he wanted to look further into it, as he was concerned about the location where he could hear the murmur best as well as where it was in the cardiac cycle. He then actually handed me his stethoscope and let me listen to it (nurse perks!). He told me the murmur could be benign (nothing to worry about) or could be the sign of something a lot more serious that I am "way too young for".

So I got sent for an echocardiogram just to check in on the function of my heart. The scan itself went well, and of course I tried to watch everything that was done but being a labour and delivery nurse I really had no idea what was going on (lol). I also got sent for a chest x-ray because I've been having lots of bone pain in my left rib cage, and we wanted to make sure that the chest wall deformity I have was not getting worse.

After the scan I went on my merry little way and went for lunch with one of my girlfriends. While I was at lunch I got a phone call from the clinic asking me to come in and see my family doctor. Usually they don't call unless something is wrong, so of course I instantly panicked. I called my Mom crying because I was scared and started having flashbacks to when I was 13 and I got the call telling me to come in to see my pediatrician (this is when I was diagnosed with a brain tumour). My Mom being the superhero she is, was able to talk me down and keep me calm enough for a few hours until I was able to get to the clinic to see my doctor.

When I got to the clinic I was immediately ushered into his exam room, which again was odd because there is ALWAYS a wait. My level of panic continued to rise as I thought of every horrible thing that could be wrong with me (this is the inner nurse coming out in me). My Mom and I didn't actually wait long until he came breezing in and sat down in the chair across from me.

He must have been able to read my face really well because the first thing he asked me was,

"The desk told you this wasn't an urgent matter, didn't they?!"

I honestly almost cried with relief. I haven't been that afraid of test results in a LONG time. He continued to tell me that he just wanted me to know the results of my scans before he left on vacation in a few days....

So what was the verdict of my scans? I have a "benign systolic ejection murmur", which basically means I have a bit of an abnormal heart beat that starts after the first heart sound and ends before or during the second heart sound. I also seemingly have some regurgitation in my aortic, pulmonic and mitral valves that they are NOT worried about. My chest x-ray also came back showing that the deformity was unchanged from my last exam... whoop whoop!

There we go folks! Another "clean" bill of health! I think my heart rate and blood pressure is still trying to come down from the few hours of intense stress, but I am SO relieved that yet again things seem to be just groovy!

Have a great weekend!

- Court

Monday, October 7, 2019

You Don't Need to Be a Jerk...

Well it's been a hot moment since I've updated this blog... I hope everyone had a great summer, I know I sure did!

So, I had a run in last week with an imaging center in the town I live in. 18 months ago, I was sent for a bone density scan because my estrogen levels were in the toilet (thanks Herman). Estrogen plays a really important role in bone health, so my specialist wanted to ensure that I had optimal bone health to prevent early onset osteoporosis... seems like a pretty valid reason to give me a bone density scan, no? Long story short, the results were not optimal at all. Basically, I was given the option to start hormone replacement therapy or be fully osteoporotic by the time I was 40... seemed like a pretty easy choice right?

Well now that I've been on the hormones for 18 months, my specialist wants to check in on my bone health again to ensure that the medication is actually working. In order to do this, he wants to send me for another bone density scan and then compare it to the initial scan I had a year and a half ago.

Piece of cake, right? Yeah no.

So to preface what I'm about to say, I anticipated having issues booking a bone density scan because I was given the complete run around last time. However, this time I was floored with how I was treated. Lemme set the scene for you guys:

I take my requisition from my specialist into the imaging center and give it to the lady at the booking counter. She looks at the form, and then at me and sneers as she says,

"You're aware you're not over 50, right?"

I really wanted to remark something back like

"No, actually I didn't realize that at all! Thanks for letting me know!"

Instead I smiled and replied back,

"I know I'm only 25, but this requisition is coming from my specialist in Edmonton"

She continues to hit me with 10,000 questions. Who is this doctor, what is is specialty, does he not realize I can't have a bone density scan because I'm not over the age of 50... and on, and on, and on...

Finally I'm frustrated, and tell her that I had this test done 18 months ago and there should be no problem with me getting it done again. I was informed it had to be "approved" before they would even consider booking me in to get the scan.

I. Was. Furious.

My entire medical history was laid out on that requisition for her. There should not  have been questions about it, but most importantly I should not have been made to feel bad about needing the scan. Had my diagnosis been something more well known, I can guarantee I would not have been given the third degree. I can't even count the number of times my symptoms or complications from my NF have been discounted because I don't look sick.

I understand there are policies that need to be respected, because I follow them everyday at my job. Policies and guidelines rule my life at work. However, there is a difference in following policies and being nice about it, and just being a jerk. There is seriously no need to be so rude.

I was almost treated like I was just seeking out this test. Like I wanted it. I didn't just decide one night,

"Hey, Courtney wants a bone density scan. Let's go in and try and trick the imaging center into giving me one."

No. It's thanks to Herman that I need to worry about my bone density at the age of 25. I need to worry about my bones not maturing properly. I need to worry about developing osteoporosis years before any person should. I need to worry about these medications working because THIS IS MY LIFE.

So needless to say, I walked away from my experience furious. I was told I would be called later to see if my scan had been "approved" (despite the fact my specialist already approved the study months ago). And I will likely have to go through this exact same ordeal again when I actually go in for the scan... fun times.

Fingers crossed friends, not only for the staff actually being nice people, but for the estrogen doing it's job! We don't really have a back up plan if it doesn't...

- Court

P.S. I did JUST get a phone call from the imaging center saying "We would be more than happy to book your bone density"... stay tuned for the results!

Saturday, June 8, 2019

MRI Time

One more MRI, and I seriously might just stick to the fridge! I've actually lost count of how many scans I've had over the years, but the number is most certainly in the double digits now!

So yesterday I had an MRI to check in on my pesky friend Herman. For those of you who don't know, Herman is the pet name I gave to my not so friendly brain tumour back in 2008 when I was first diagnosed. Apparently giving a ridiculous name to a mass of tissue in my brain made it easier for 13 year old me to cope with it....

Rest assured friends, this MRI was just my routine scan. I haven't had any weird or new symptoms that warranted a trip to the scanner. Back when I left pediatric neurosurgery my neurosurgeon suggested I have scans every two years to monitor Herman and make sure he hasn't decided to have a little growth spurt, and to make sure he didn't bring any new friends along to play.

Brain tumours are common for people who live with Neurofibromatosis, and despite all the times I have been in the scanner the nerves never seem to go away. I always get myself worked up that they're going to find something new... I don't know if that the pessimistic side coming out in me, the nurse side coming out (prepare for the worst, and hope for the best) or just the realistic side knowing that I live with a chronic and progressive condition.

Herman was discovered completely by fluke, we weren't even looking for anything when I had my first MRI. Right before my first surgery to remove a mass of tumours from my back, the pediatrician decided it would be a good idea to have a "baseline" scan of my brain, just to make sure everything was fine. I'm sure you all can guess what happened... Herman made his first unwanted appearance!! How rude of him to join the party uninvited **insert eye roll here**.

So that's why I'm always nervous and anxious when I get a scan... if I wasn't having any symptoms the first time a tumour was found, what's saying that I don't have another 2, 3, 4 tumours staking their claim in my brain matter? Herman set up shop no problem, what's preventing Bitsy or Juan from doing the same thing?!

All jokes aside, I am very grateful to have access to technology such as an MRI scanner. Although it brings great anxiety, I have always walk away feeling comforted by the fact Herman hasn't grown or anything over the last 10 years. My fingers, toes and everything else are crossed that all is well!!! It's also got to be a good omen that they were on time and got my IV on the first try... huzzah!

Thanks for tuning in friends, I will have the results of my scan back in my hot little hands in a few weeks (could have been earlier if I didn't have to work so much...) and I will make sure to post a little update.

- Court


Friday, May 17, 2019

31 Neurofibromatosis Awareness Facts

Hey friends! May is Neurofibromatosis awareness month, and if you've been following my blog since the beginning, you'll know that every year I post 31 facts about this condition that I live with on a daily basis! 

Feel free to ask questions in the comment section below... happy learning!

1. Neurofibromatosis is one of the most common genetic disorders caused by a mutation of a single gene.... more than 2 million people worldwide are affected by this condition! That's similar to the population of the country Latvia!

2. Neurofibromatosis is more common than Cystic Fibrosis, Huntington's, and Muscular Dystrophy... COMBINED!

3. There are 3 different classifications of Neurofibromatosis: Neurofibromatosis Type 1, Neurofibromatosis Type 2 and Schwannomatosis, all of which are caused by mutations of different genes

4. Neurofibromatosis is an autosomal dominant disorder, meaning people who have the condition have a 50% chance of passing it onto their children. However, more than 50% of NF cases are due to "spontaneous mutation" of a chromosome (extra fun fact: My genes spontaneously mutated in utero, which is how I ended up with NF-1)



5. Neurofibromatosis Type 1 has a prevalence rate of 1 in 3,000 people and makes up 90% of all NF cases

6. Neurofibromatosis Type 2 has a prevalence rate of 1 in 25,000 people

7. Schwannomatosis is the most "rare" of the Neurofibromatoses, as it only occurs in 1 in 40,000 people

8. Neurofibromatosis Type 1 is caused by a mutation of chromosome 17, which is responsible for making a protein called "neurofibromin". Neurofibromin plays a role in tumor growth inhibition, which is why people with NF have tumors grow all throughout their bodies

9. It's hard to predict how mild or how severe any NF case will be. Approximately 60% of people will have very mild symptoms (ex: cafe-au-lait spots), while the remaining 40% will have moderate to severe symptoms/complications that may be corrected by surgery/medication or that may be persistent and life long (ex: scoliosis)

10. Neurofibromatosis is a systemic condition, meaning that it impacts all systems of the body.


11. Cafe-au-lait spots are the most common sign of Neurofibromatosis. Cafe-au-lait spots are caused by an excessive amount of melanin in the skin. Melanin is a protein that gives skin its pigment. One of the diagnostic criteria for Neurofibromatosis is to have six or more cafe-au-lait spots that are at least 0.25 inches in diameter. It is rare for tumors to grow where there are cafe-au-lait spots (BONUS FACT: 10% of people in the general population have cafe-au-lait spots)

12. People can also have hypopigmented patches of skin called nevus anemicus, which like cafe-au-lait spots are completely harmless! 

13. The one distinguishing diagnostic criteria that separates NF-2 from the other Neurofibromatoses is the development of tumors on the 8th cranial nerve in both ears (this nerve is important for hearing). In some cases these tumors can cause loss of hearing, and in some cases deafness. These tumors are almost always seen in NF-2 ONLY

14. Lisch nodules are the most common ocular complication seen in Neurofibromatosis, as approximately 95% of people have these by the age of 6. Lisch nodules are hyperpigmented patches in the eye that do not impede vision. Sometimes they can be seen with the naked eye



15. It is estimated that 15-40% of people with NF have optic nerve gliomas. Most often, these tumors develop in childhood and are very unlikely to develop in adulthood. Typically these tumors are very slow growing and have very low malignancy rates. They can however cause vision loss in some people

16. More than 60% of people living with Neurofibromatosis also have a learning disability. Children with NF are 5 times more likely to suffer from ADD and ADHD

17. People with NF have a slightly higher chance of developing cancer in their lifetime than those in the general population. Approximately 25% of people will have cancer in their lifetimes, and 30% of people with NF will develop cancer

18. Treatments for NF tumors include surgery, chemotherapy, radiation and medications to control various other symptoms/complications. Sometimes chemo and radiation are used for tumors that are not cancerous... in these cases these tumors may not be operable or surgery may not be an option! As of right now there is no cure for NF, but researchers are working hard to find a cure!

19. Skeletal abnormalities include scoliosis/kyphosis, tibial dysplasia, osteoperosis, short stature, macrocephaly (large head) and chest wall deformities are frequently seen in people with Neurofibromatosis



20. Neurofibromatosis occurs equally in all races and all genders

21. No two cases of Neurofibromatosis are alike... every person is going to be impacted differently

22. People who have NF-1 tend to show symptoms by the time they are 10 years old

23. Less than 1% of people with NF-1 will have the following complications: early or late onset puberty, being too tall/too short, excessively itchy skin, hormonal imbalances or having brain tumors other than optic nerve gliomas

24. People with NF-2 tend to show symptoms by the time they are in their late teens to early 20's

25. People with Schwannomatosis can be symptom free until they are 40 years of age

26. The most common problem that people with Schwannomatosis face are issues with chronic pain. This is usually the first symptom that appears before a person is diagnosed.

27. Schwannomatosis does not share the same genetic patterns than NF-1 and NF-2 do. It tends to skip generations... so your grandma could have it. then your mom may not have it, and then you could have it.

28. It is not entirely known if there are things that trigger tumor growth. It seems that there are periods of tumor growth during puberty and pregnancy.

29. 1 in 3 people with Neurofibromatosis have a plexiform neurofibroma



30. NF is NOT the Elephant Man Disease. It is actually hypothesized that the "Elephant Man" had a disorder called Proteus Syndrome, which causes there to be an overgrowth of tissues

31. Neurofibromatosis is not contagious. People with this condition are no different than those from the general population. Help me raise awareness by sharing these facts, and commenting below with your own NF Awareness Facts... this is just a very small amount of information on NF!!!


I hope you've all learned something new today, now go spread some NF awareness :) 


- Court