Chiari and Cervical Instability

On Friday I saw my neurosurgeon (NS) for a follow-up consult after having a rotational CT scan, Spectra Cell analysis (blood work), and other testing.

We learned that instead of a 3mm herniation, which was indicated on my MRI report, I have a 5mm herniation. This means that I have Chiari 1 Malformation not Chiari 0. The CT scan also indicated that my C1 and C2 vertebrae in my neck are very unstable. There are risks that come with instability that high in the neck and therefore I am unfortunately stuck wearing a cervical collar. It is VERY fashionable (I hope you sense my sarcasm).

My symptoms are likely a result of both the herniation of the tonsils/Chiari and the cervical instability. The good news is it is something that can be "fixed." There is no cure, but there are effective treatment options that can help with my symptoms. The bad news is it means surgery. Yes, another surgery...and this time it's a bit more complicated. My NS wants to fuse my C1 and C2 vertebrae and perform a decompression surgery. The decompression involves removing a piece of my skull to allow more space/room for the brain. Obviously the fusion is meant to stabilize the cervical spine. There is another procedure that he wants to include but I am hoping that we can avoid that part of the surgery altogether. At this time I have surgery scheduled, but I am still deciding what to do. I have had many people tell me that the surgeries are definitely worth it and their symtpoms have been relieved. I honestly have not heard a bad story for these procedures which is definitely comforting.

I have some more questions that I want answered before I make my final decision.

At the moment I am taking 2 summer classes, Biology- Genetics and an online Math class. It is very difficult because it involves throwing a full semester into 6 weeks. I am only in my first week and I already have a ton of homework and readings. However, in order to graduate in 3 semesters I need to take 2 summer classes both this summer and next. It will feel so great to have these classes out of the way and it will lessen the course load in my remaining full semesters.

I will have 1-2 weeks in between the end of my summer classes and the surgery (if I decide to go ahead with it).

EDS Awareness Month

I know I am a little late in posting this but May is Ehlers Danlos Syndrome awareness month. Instead of recreating the post from last year, I simply copied it into a new post with some minor adjustments and additions. 

Property of EDS Tags & Snags on Facebook

Help increase awareness and education on this virtually unknown disorder. Here is the link to the EDNF.org Awareness Month of Activities List. 

For those of you who are new to my blog or have never heard of Ehlers Danlos Syndrome here is a brief explanation:

EDS is a rare connective tissue disorder affecting about 1 in 20,000. EDS affects connective tissue, which are tissues that support the skin, bones, blood vessels, and other organs. There are 6 subtypes that vary in severity from mild to life threatening. An unusually large range of joint movement (hypermobility) occurs with most forms, particularly the hypermobility type. The loose joints are unstable and prone to dislocations, chronic pain, and early onset arthritis. People with vascular type EDS are at risk of serious complications. Serious complications include tearing (rupture) of major blood vessels and organs. These complications can be fatal. About 1 in 4 people with vascular type EDS develop a serious health problem by age 20, and more than 80 percent develop a life threatening complication by age 40. The median age of death is 48 years. 

There are a number of secondary conditions connected to EDS that can be very disabling as well- Some of the more common disorders are POTS, mitral valve prolapse, orthostatic hypotension, IBS, asthma, dysautonomia, and chiari malformation.

Here are 2 videos I made about EDS to help educate people and raise awareness. The first video is shorter and based more on medical information, while the 2nd video is a little bit longer and provides quotes from people living with EDS and how it affects their life. 

(The link to the mobile version of the above video can be found in the information section on YouTube, directly below the video)

(At this time there is no version of this video compatible for mobile devices including iPhones, iPads, and other cellular devices. I will work to resolve this in the near future but it is a lengthy process)

So You Think You Might Have EDS- very informative article about the symptoms and signs of EDS

*This article should not replace the advice of a medical professional. If you think you might have EDS please consider seeing a genetic specialist. 

How you can help:
1.  Post the following text (or your own version) to your facebook or twitter page:
"May is Ehlers Danlos Syndrome Awareness month. EDS is a rare connective tissue disorder marked by hypermobile and unstable joints, fragile blood vessels and organs, and even heart complications. There is no cure or specialized treatment. Most doctors don't know what EDS is. Help spread awareness and education. Post this if you have or know someone living with EDS. With awareness and research there can be a cure."

2. Download and/or post the following EDS posters to your facebook or twitter page. Ask your doctor, physical therapist and/or hospital if you can post them in their lobbies or waiting rooms. Check with other local businesses. Ask permission before posting. *If you are interested in larger poster size versions for distribution please contact EDS Network Cares (click here)

(Property of EDSNC)

(Property of EDSNC)

(Property of EDSNC)

(Property of EDSNC)

3. Fundraising! Donate to EDSNC (Ehlers Danlos Syndrome Network C.A.R.E.S). There are several research projects currently underway--any money donated to the cause is extremely helpful (click here for an EDSNC template for donations). If you are interested in purchasing awareness bracelets through EDSNC click here! Donations are also accepted through EDSNF (Ehlers Danlos Syndrome National Foundation)! 

4. Be creative! 

-Create an "Ask me about EDS" shirt or badge! 

-Hold a bake sale with the proceeds benefiting EDS research

-Contact local radio stations

...There are many different ways to spread awareness. 

**Not only do most people not know what EDS is, but most physicians have never heard of it either. Here is a .pdf file outlining EDS (Facts of EDS). These templates are great to help educate your doctors about EDS- provided by EDNF (.pdf files)

-Pain Management guide

-Ophthalmology

-Vascular EDS Guide

-EDS in Practice- Stages of Life

-Dentistry and EDS

-Guide for EDS in Children (for parents)

-Emergency booklet for Surgery (CD is no longer available)

EDS downloadable wallet card

EDS poster for coaches and trainers--(click here)

For pages I may have missed, here is a comprehensive page including other specialty areas and EDS documents--(click here)

*Many people who are beginning their journey with EDS don't know where to begin. When people ask me what to do first, I usually say they should see a genetic specialist to confirm their suspicions. If they have already been diagnosed, then the next step varies and depends on the individual case. There are many specialists involved in an EDS patient's case. These include genetic specialists, rheumatologists, surgeons, dentists, ophthalmologists, physical therapists, psychiatrists, pain management specialists, and many many more! Here is a Physician's Directory from EDNF.org and may be helpful if you don't know who to see. 

There are many great Facebook support groups available and can be found by simply searching Ehlers Danlos Syndrome on Facebook. If interested in purchasing EDS zebra ribbon car magnets for awareness, I believe more were recently ordered and can be purchased via this Facebook group--EDS Awareness Magnets!!

I also created a few EDS awareness shirts on Zazzle a few months ago-- 

Version 1: click here

Version 2: click here

Other EDS products can be found on cafepress.com and zazzle.com--simply search Ehlers Danlos Syndrome

Other useful websites can be found in the EDS tab/page at the top of my blog!

With awareness and research there can be a cure! Help make a difference!

Florida

So it's been awhile. As always I don't have a lot to update on.

I finished the Spring semester. I still have no idea how I managed to start in January, 4 weeks post-op from reconstructive knee and ankle surgery, and finish 3 months later. I started on crutches, in a cast, and with a post-op knee brace, and ended on a cane and with a knee and ankle brace. I am very proud of this accomplishment and it feels great to know that I am now 2 semesters closer to graduation, which is expected to be in the Fall 2013. I am taking 2 classes this summer in order to stay on schedule.

Speaking of graduations, my mom and I just returned home from sunny Florida and Nicole's college graduation weekend. I had a fantastic time despite not being able to do everything (I could not walk on the beach). The trip itself was exhausting but totally worth it. I have not been able to truly enjoy a vacation in almost 3 years. That is definitely not the case anymore. Family (immediate and extended) and friends were all in Florida together to celebrate this milestone in her life. We stayed at the most perfect hotel that was on the beach and just about every window overlooked the water. We all went to dinner together and spent the days at the pool and beach (I did not go to the beach). At the graduation we were definitely the loudest group of people.

Nicole and I have been friends since the day she was born...I was born first :). My mom and her mom, Ellen, lived together in college and have been best friends since. Nicole and I grew up together. She is about as close as you can possibly get to being a sister, without actually being related. I am so proud of her and wish her nothing but happiness and success in the future. My mom and Ellen are a perfect example of how it doesn't have to end after college. Sure the partying, roommates, and classes may end (to some degree), but the friendships can always live on and continue to grow! I am fortunate that my mom and Ellen have remained best friends because had they not I would not have known Nicole and Michelle (her real sister).

Congratulations Nicole! I love you.

Now for health related information...

My hips have been killing me lately. Cam says it is because I walk by only using my hips, which is incorrect, and therefore it should get better when I walk correctly (we are working on this). Both knees still hurt. Going up stairs or standing up from a sitting position is still very painful for my left knee, which was operated on last year. My right knee, the newly reconstructed knee, still gets lightning bolt pains through the incision at random times. Quad strength is progressing and I am farther along in this recovery than last time, but the strength is no where near functional and I am still in a brace. My ankle still doesn't flex very much but Cam did clear me to start weaning from the brace. I still have to wear it when going out for longer periods of time or where there would be a lot of people, but I no longer have to wear it in the house. This should hopefully get some more natural flexion back.

Originally we had planned on having my right shoulder reconstructed this July, however, after talking to Cam we both agree that I really need to give my body a chance to recover. My right shoulder is definitely in very bad shape and I usually wake up with it dislocated, but this is just something that I need to hold off on right now. I will have it reconstructed at some point in the future (likely NEXT summer), it just will not be happening this year. Dr. Kramer wants me to wear a shoulder brace/sleeve. Hopefully that provides a little more stability than I already have.

My headaches haven't changed at all. I go back to the specialist this week for a follow-up to review the testing I had done.

I start one of my classes on Monday. It is an online class so it will involve a lot of work, but it'll feel so nice when I am completely done for the summer at the end of June!

Headaches, Homework and Hockey

I haven't posted in awhile mainly because I do not have a lot to update on. I did, however, see the neurosurgeon for Chiari and EDS. While I do have low lying tonsils, he does not believe it is significant. That is good news. However, he does think that I have cervical instability of C1 and C2 and that this is the main cause of my symptoms. I had a rotational CT scan to check for this and we will discuss the results at my appointment in May. Aside from the CT scan, I need to get blood work to check for vitamin and mineral deficiencies. Significant deficiencies can add to the severity of my symptoms and are known to be a problem for EDSers. I will also go for Urodynamic testing--one of the steps to determining whether or not I have a tethered cord. This will be done on the same day as my follow-up appointment. My headaches and other symptoms have not changed at all. I still have headaches everyday. I am always tired.

After the neurosurgeon appointment we went to Boston for post-op appointments (knee and ankle), hip x-ray follow-up, and to meet my new geneticist at Children's. My knee and ankle are progressing nicely. I have switched from the post-op brace to an ACL brace. I had no work done to my ACL, however the brace gives me the support I need and is a lot smaller. It also prevents hyperextension and that is an added bonus. I am in my old CaligLoc ankle brace. I still have limited flexion and ROM and this is very frustrating--especially in the morning. I will continue in PT with Cam. During this appointment I also had hip x-rays as a standard follow-up. At my last appointment with Dr. Kim in the summer, he was concerned with joint space narrowing. These x-rays will help indicate whether or not the surgery has been successful. I did not see Dr. Kim or Dr. Yen, but will talk to Dr. Yen about the x-rays via e-mail or when I see him this summer.

As I mentioned, I saw the EDS specialist at Children's. She is so nice and I really liked her. She was thorough yet very laid back. She will see me every year and is available if/when needed. I am going to try to see the Gastro specialist at Children's during my next visit. Stomach issues are a problem for a lot of EDSers.

At the end of my trip to Boston my dad, brother and I went to the Bruins last regular season game. It was my birthday present and was AMAZING. The Bruins won in a shootout and after the game we went to the Fours Bar for dinner and drinks. The Fours Bar is where I met Brad Marchand in November after the Thanksgiving game.

Shane and I in front of "The Goal" statue outside of TD Garden!

After the Bruins game we went to Connecticut for Easter with the Murphy family. Love spending time with family!

I was sick this week with the beginning stages of bronchitis and cancelled PT as a result. I needed the break. I have been exhausted and overwhelmed lately with work and it was nice to sleep in on my days off and just focus on schoolwork (which with an on and off fever and sore throat is not all that easy). Looks like the antibiotics are working because I have not had a fever since Friday and I feel a little better.

I only have 2 more weeks of the semester left and of course it's going to be insane. Professors seem to realize at this time that their time management and lesson plans did not go according to plan and they are scrambling to get everything in. This unfortunately sucks for the students. Once I am done with the Spring semester I am going to Florida. My friend is graduating and I am excited to be able to go to the graduation and spend time with friends. This summer I will be taking 2 courses (Anatomy/campus and Math/online). I have also mapped out my 3 remaining semesters (including this summer and next). I am set to graduate after the Fall 2013 semester. The end is finally in sight.

This weekend was the MNOC reunion. It's always great to see my family. I had a lot of fun and enjoyed a long day of watching hockey with everyone. We also celebrated all of the April birthdays in the family (there are a lot). My birthday cake was Bruins themed and it looked AWESOME. It also tasted great (although it was so hard to cut into it).

Me with my cake! We matched :)

The CAKE!

The Bruins are playing the Capitals in the first round of the NHL Stanley Cup playoffs. The series is currently tied 1-1. LET'S GO BRUINS! There is nothing better than playoff hockey.

International Chiari Association

Please visit the International Chiari Association website (click here--you can also find the link in the Chiari tab at the top of the blog). The ICA is the only Chiari nonprofit organization and provides updated news, videos, medical centers, and doctor lists (to name a few). What a great organization!

Chiari Struggles

My spring break is over. I basically spent the entire break doing insane amounts of homework. I also had physical therapy. My next break is in 2 weeks. I have 6 days off due to Easter and my class schedule. I am excited for this break because I am going to Boston for an appointment and a Bruins game and we are spending Easter with the Murphy family. We have not been to Easter with the Murphy's for 5 or 6 years.

In 2 weeks I have an appointment with Dr. Henderson for Chiari and EDS. I am hoping he has some answers for my neurological symptoms. The craniocervical manipulations (treatments) that I have been getting only seem to make my headaches and symptoms worse...and by worse I mean I get sick after the sessions because of how bad the headaches are. My symptom list is pretty long...

My headaches are getting much worse. I feel nauseous most days because of my head. I am hopeful that Dr. Henderson is the answer. I have heard nothing but wonderful things about him.

I will be having a lumbar spine MRI in the next week. This will be a scan that is evaluated at my appointment...along with my upright and supine brain MRIs. 

In terms of my leg recovery (ankle and knee), I am progressing a lot faster and smoother than normally. I am hoping to get out of the walking boot in the next couple of days. I will wear my CaligaLoc ankle brace that I wore before surgery. (For EDSers with lateral ankle instability, I highly recommend the CaligaLoc brace--if interested click here). My ankle is very stiff..especially in the morning. Cam said that when I get out of the boot and move my ankle more it will start to move better. I am not ready to get out of the post-op knee brace. My quad strength is not really close to what it needs to be to support me. I am however, close to moving to a cane. Technically I am ready now but Cam does not trust my balance enough to let me ditch the crutches at school or when going out somewhere. We are working hard on getting me stable enough to move to the cane within the next week because my right shoulder dislocates constantly. 

I redecorated my cane so it is ready whenever I am...no surprise on the theme I chose..BOSTON BRUINS! The NHL Stanley Cup playoffs begin on my birthday :)

I am now taking Lyrica for my CRPS/RSD. It has not started working yet...hopefully soon! I still have major memory problems so it is very possible that Chiari is the reason..not Neurontin. However, it was important to get off of Neurontin...2700mg a day is just too much!

I am looking forward to this semester being over! I have no idea how I made it this far but I am proud that I have pushed through despite having headaches 24/7, other neurological symptoms, and of course I am recovering from 2 reconstructive leg surgeries. Hopefully I manage to accomplish my GPA goals by the end of this semester!

New EDS Awareness Video

I made a new EDS video. It is very similar to my first video, however this time I included quotes on how EDS affects different people.

Thank you to those who contributed quotes and/or pictures for this video!