Monday, January 26, 2015

Optic Gliomas

Optic gliomas are another complication that is commonly seen in people living with NF, so here is some information for you all!

There are multiple different kinds of tissue that make up the brain, and an example of a type of tissue that can commonly cause tumors is glial tissue. Glial tissue makes 3 different type of cells which can cause different types of tumors:
  • Astrocyte cells, which can cause astrocytomas 
  • Oligodendrocyte cells, which can cause oligodendrogliomas 
  • Ependymal cells, which can cause ependymomas 
Now, there are also multiple different types of gliomas, but this blog is going to focus on optic nerve gliomas to keep it simple!

An optic nerve glioma is a type of tumor that can grow in various parts of the brain (not just on the optic nerves). In the general population, optic nerve gliomas make up approximately 5% of all childhood tumors and in the NF population, approximately 14-40% of people have this type of tumor. Generally, optic nerve gliomas are discovered before the age of 20, and luckily they tend to be slow growing and noncancerous. In fact, a large number of people are asymptomatic and therefore it is extremely important that people under the age of 20 see an ophthalmologist yearly.  

Optic nerve gliomas can affect one or both eyes, and only cause blindness in 5% of cases. Due to the location of where some of these tumors grow, they can also cause hormonal imbalances and issues. 

Typical signs and symptoms include but are not limited to:

1. Involuntary eye movement
2. Outward bulging of one or both eyes
3. Squinting 
4. Problems with balance
5. Headaches 
6. Nausea and Vomiting 
7. Hormonal Imbalances or early puberty 

In order to be diagnosed with an optic glioma, yours or your child's physician may order some of the following tests:
  • Cerebral angiogram (a test that uses a special dye and x-rays to see how blood flows through the brain)
  • Biopsy of a tumor that is removed during surgery 
  • CT scan or MRI scan of the brain 
  • Visual field tests (kind of like the ones you get at the eye doctors)
Treatment depends on the size of the tumor and the location of the tumor. Sometimes the tumor can be removed by surgery, but sometimes methods such as chemotherapy and radiation are necessary to treat optic nerve gliomas that are particularly aggressive or inoperable due to their location in the brain.

I was diagnosed with an astrocytoma when I was 13, which at the time was deemed inoperable due to its location. My tumor is right near the hypothalamus and pituitary gland, which is essentially right in the middle of my brain. At first we were unsure how long the tumor had been there, if it was growing.... so on and so forth so we had to play the "wait and see game". Luckily however, my tumor over time as shrunk to almost half it's normal size. This apparently, is very common for optic gliomas (for them to shrink and even disappear).

Thanks for reading!


Resources Used:

Monday, January 19, 2015

Medical Botox

Alrighty.... if you aren't already following "Manitoba Neurofibromatosis Support Group" on Facebook I highly recommend you join the group to meet some new friends! For those of you who haven't already liked that group, I asked people on there to guess "What are some off-label/non-cosmetic uses for Botox?" before they read this article. So buckle down to read something interesting, the answer(s) to the question will be at the end of the article :)

Headaches are seen in 22% of people living with Neurofibromatosis, myself included. Unfortunately in my case, my headaches seem to be treatment resistant. One of the many suggestions I received from doctors was for me to try medicinal botox to see if that would reduce the number of headaches I was having in a month. So here is the lowdown on how Botox works to treat headaches!

What is Botox? 
In high doses, Botox is actually a toxin that causes paralysis of muscles. The FDA approved the usage of Botox as a treatment for chronic headaches/migraines in 2010.

Why Botox for Headaches?
As with many things in the medical field, there are conflicting beliefs on how Botox works to treat headaches. One theory is that Botox helps to stop the release of a special chemical in the brain called a nociceptor. By stopping these chemicals from being released, a person is not able to feel pain, or the pain they are already experiencing should decrease. Another theory is that Botox temporarily relaxes some of the muscles in the head and neck which is thought to help decrease headache pain.

Does this actually work?
1384 patients were used in a study to see if Botox could help decrease the number of headaches they were having in a month. Each of the participants were experiencing approximately 20 headaches or so a month, 18 of which were "severe". Patients were given Botox every 12 weeks for a year, and the results were very surprising! After the course of treatment, 70% of the patients were having 50% FEWER headaches a month than at the beginning of the trial... meaning 70% of the patients were having less than 10 headaches a month! Results seem to have stayed the same, as most sources say Botox is an effective treatment approximately 75% of the time.
** Unfortunately in my case the Botox did not work to alleviate my headaches**

Where is the Botox given?
The Botox is injected by a very small needle into your head and neck, and causes minimal pain.

Who can get this treatment? 
Not everyone who has headaches is eligible to receive Botox treatments. Only patients with chronic migraines are able to receive this treatment. Chronic migraine is defined as "...headaches occuring 15 or more days each month, at least half of which have migranious features (ex: sensitivity to light, nausea and vomiting, pain behind your eyes, etc)".

What is the cost of this treatment? Is it covered by health care?
The cost of treatment really depends, but it usually ranges from $300-600, and is not covered by most health care plans in Canada.

What are the side effects? 
The most common side effects from this medication are: neck pain, muscular weakness, and drooping of the eyelid.

So there you have it folks, I hope you all learned something new today! Thanks for reading everyone!


P.S. Obviously one of the answers to my question was "to treat headaches", but some other off-label uses for Botox are: drooling, hair loss, overactive bladder, ulcers, bladder problems, excessive sweating, and hand tremors a just a few of the many off-label uses for Botox!

Resources Used: 

Monday, January 12, 2015

Different Types of Fibromas

Hey there everyone! Thank you to everyone for your kind words after I posted my last blog past last week on eating disorders and body image, it truly means a lot to me! This week I am going to share information on different types of fibromas commonly seen in patients with NF. There are 3 types of fibromas:

1. Cutaneous Neurofibromas
2. Plexiform Neurofibromas
3. Spinal Neurofibromas

Alright then, lets start with cutaneous fibromas (which is pictured below)

Cutaneous neurofibromas occur when the covering of a nerve below the skin begins to form a slow-growing tumor. This occurs, because people with Neurofibromatosis are unable to make a protein called neurofibromin, which is responsible for "monitoring" the body for tumor growth... kind of like how cops monitor for crimes... However, people with Neurofibromatosis do not make this specific protein, which is why tumors can rapidly grow in the body.

Cutaneous neurofibromas begin to develop right around puberty, it is very uncommon that these types of tumors to develop in infancy or early childhood. Typically, as an individual gets older there is an increase in the number of cutaneous fibromas...this is especially true of women who are pregnant (there is speculation that tumor growth in NF is aggravated by hormones and hormonal changes). These types of tumors hardly ever cause problems, and are extremely unlikely to ever become cancerous. The most commonly reported "problems" of cutaneous fibromas are: an intense itching sensation, pain when touched or bumped and cosmetic deformities.

In terms of treatment for cutaneous neurofibromas, the size and location of the tumor depends on which course of treatment a doctor will take, Most commonly the tumors will be removed during surgery, but radiation and chemotherapy are also methods to remove tumors if they can't be accessed by surgery. Also, sometimes doctors can prescribe creams that will help to stop the itching!

Next we will discuss plexiform neurofibromas!

Plexiform neurofibromas are present in about 30% of individuals living with NF, and unlike cutnaneous fibromas these tumors are often found in early infancy and sometimes even at birth. Plexiform fibromas grow similarly to that of a cutaneous fibromas, except it involves tumors growing on multiple nerves instead of a tumor growing on just one nerve. Usually these types of tumors grow around the eye socket, face, arm, leg, back, chest or abdomen. Typically these tumors grow at a very slow rate, and are unlikely to become cancerous. When these tumors do become cancerous however (in 5-10% of plexiform neurofibromas) they are called "malignant peripheral nerve sheath tumors"

The main complications that plexiform neurofibromas cause are: pain, loss of function and cosmetic deformity. Another symptom that commonly occurs in these types of tumors is called "Hyperpigmentation Overlying a Plexiform Neurofibroma".... sounds super complicated eh? All it really means is that it is common for a tumor to become an orange-like colour, grow more hair than normal and develop an uneven surface (like the picture above!)

Treatment options for plexiform neurofibromas is very similar to the treatment of cutaneous neurofibromas (surgery, radiation or chemo). With this type of tumor, it is very common that parts of the tumor will grow back after the procedure if they entire nerve is not taken out... so surgery does not pose a 100% cure!

Lastly, we are going to discuss spinal neurofibromas! The  yellow arrow in the MRI is pointing to the spinal cord, and the red arrow is pointing to the spinal neurofibroma. Signs and symptoms of spinal neurofibromas depend on where the tumor is located. Sometimes these tumors produce no symptoms, and sometimes they can cause complications such as weakness in the arms and legs, back pain and numbness. Typically these tumors grow slowly, and rarely become cancerous. Treatment usually includes surgical excision.

Thanks for reading, I hope you all learned something!


Resources used:  

Monday, January 5, 2015

The Fight Against Body Image

Hey everyone! So I thought I might take a step away from writing about Neurofibromatosis this week, and focus on an issue that is wide spread in today's society, and something that has hugely impacted my own life. I was scrolling through Twitter the other day and came across this picture. Take a good look at it, as well as the caption underneath: 

So I can pretty much guarantee that at some point in your life, you have envied a mannequin (seriously, don't laugh!). Ever tried an outfit on that looked good on the mannequin, but looked awful on you? Ever thought "Man, mannequins look good in everything!"? Ever wished you were as thin as a mannequin so you could fit into those skinny jeans? I know I have.

I've been struggling with body image issues since I was about 14 years old. At one point in my life I strove to look like that stick thin, sickly looking mannequin. How horrible is that? Looking back on pictures of me from 6 years ago, I finally realize that my 84 pound self looked horrible. I was awfully thin, my face looked sunken and you could see my collarbone from a mile away. Since when did that become "beauty"? And since when did these sickly looking legs on the mannequin become acceptable? 

So many girls (and some boys) strive to look like the people they see on TV or in the magazines, and if you ask me, this mannequin gives yet another reason for people to feel inadequate. Many people with eating disorders compare themselves to others, and I know that because I used to do it all the time. I used to say things to myself such as,  "Oh, well she's thinner than me so I need to be skinnier." Why are we facilitating self destruction and allowing girls to hate their bodies, and teaching them that beauty is based on the number you see on the scale? Why are we allowing society to teach girls to despise their bodies, which results in them starving themselves to simply look skinny?

I'm sure you all have jammed out to Meghan Trainor's "All About that Bass", and for a while I felt enlightened that someone in the music industry was breaking down the walls and trying to implement the idea that girls who aren't a size 00 are beautiful too. However, I was appalled when Meghan Trainor said in an interview that she "... tried to go anorexic for a good three hours. I ate ice and celery, but that's not even anorexic. And I quit. I was like, "Ma, can you make me a sandwich?""... I have a lot to say about this but some of it probably isn't appropriate for the internet but I will say this: 

You don't "decide" one day that you want to be anorexic. You don't decide that you are going to stop eating the things you love and essentially starve yourself. You don't decide to turn your life upside down because you fear becoming fat, ugly or unwanted. You don't wake up one morning and say "This is the day I become anorexic". It doesn't happen like that. Eating disorders slowly manifest overtime, it's almost like you become blind to what you are doing to yourself. You don't see what's happening. Before you know it you have something on a plate in front of you that you used to love eating, but now it sits there and mocks want to eat it but can't find the courage to do so. You're afraid of what food might do to you, and you're afraid of the person you might become if you give into the "temptations" of food. Meghan Trainor said that it takes "strength" to have an eating disorder. I'm sorry, but you don't need strength to develop anorexia or bulimia, it takes strength to face your daemons and overcome your eating disorder. Strength is acknowledging that something is wrong and deciding to make changes in your life to guide you to recovery and changing the way you think about food and your body. If we can change the way society portrays beauty, then that's a stepping stone to eradicating eating disorders and self-destructive behaviours.  

Thanks for reading everyone. I've had this post sitting here for a while and I've been unsure about posting it, but I feel like this is an issue that needs to be brought to the table to be discussed openly. So please feel free to comment below!