Graduation and Moving

Hi everyone! I am way behind on updating and have a few new medical things to update you on. I will get to that very shortly hopefully! I just wanted to share a couple of personal developments first!

Most excitingly, I graduated from Northern Illinois University with my Bachelors degree!!!! I graduated in 3 years with a final GPA of 3.891! I had 6 surgeries during my 3 years of college, so this was a HUGE achievement for me. I felt very poorly on the day of my graduation ceremony, but I have AMAZING friends and family who helped me to make the day as easy and stress free as possible. I sat with a few friends during my ceremony which was really nice to get to sit with people I have gone through this experience with. My ceremony was in the evening and I got home and went straight to sleep, but I had also been able to go out to lunch with a group of ladies who have Chiari Malformation (my brain malformation) that morning. It was so nice to be able to go hang out with so many wonderful people who all understand what I am going through! It was also wonderful to be able to speak with a few younger women who will be starting college soon. It is always amazing to be able to share advice and inspiration for others who will be entering the stage of life I just finished!

Here is a picture of my husband and I after my graduation. You can tell I did not feel well, but I am incredibly proud of my accomplishments!

The other thing that has been keeping me so busy right now is that my husband and I just bought our first home! We are moving out of our apartment by my old university and moving about 45 minutes away closer to where my husband was transferred for his work. It is a LOT of work packing up everything and it has been exhausting for me! We are moving this weekend, so we are in the final stages of trying to get all of our belongings into boxes. It will be nice when we get there, but right now it has been a lot of work to get everything squared away for closing and moving. I am sad to say goodbye to my wonderful job as an after school tutor at the local middle school, but hopefully I will find an equally wonderful job in the new town we are moving to!

I will write a blog post on my medical updates, but I just wanted to share my life updates with all of you!

Date Change for NY

We decided to change the date of my tethered cord release to June 24th. We will have my pre-op appointments on June 23rd. This will make it so that we are in NY for a much shorter amount of time. This helps drastically financially and also helps tremendously with scheduling because my husband doesn't have to take as much time off of work.

I just wanted to update everyone on the the date change. There is not much else new with me right now. I have a follow up appointment with my orthopedic surgeon  on Thursday for my wrist. After doing occupational therapy is it feeling back to normal, so I have no concerns for that appointment.

Podiatry Appointment

When I saw the rheumatologist who specializes in EDS a few weeks ago she suggested I see a podiatrist for my hypermobile feet and ankles. The hope was that this would help not only my feet and ankles to have less pain, but also that my calves, knees, and hips would have less pain as well.

I saw a local podiatrist and he was absolutely wonderful! He was amazed at how hypermobile my feet and ankles are, but yet how tight my muscles were. This is very common in EDS and many joints all over my body. The connective tissue is so loose and hypermobile that the muscles tense up to hold the joints in place. The first thought that we had was to put custom inserts into my shoes. The front, middle, and back of your feet all move separately, so with a custom insert he is able to adjust to the different needs of each part of my feet. I have high arches in the middle, but they collapse towards the back of my foot and roll outward. Well the doctor's assistant called my insurance and they would not cover these inserts. They cost upwards of $600, so obviously I was a little unhappy with that option. I then brought up that the rheumatologist had more of an ankle-foot orthotic (AFO) in mind, instead of just the foot orthotic. Thankfully he thought this was the right path to go for me and my insurance DOES cover these. This will be a brace that goes up to the ball of my foot and extend up above the ankle. It will include the foot support that the shoe orthotics would have, but it also holds the ankle in place so that I do not roll it and sprain it so often. The type of AFO that this is will not cause dependence or weakening of my muscles because I am still using my own muscles, this is just supporting me. He recommends that patients start with one foot at a time so that your body can get used to walking with the AFO on. I decided to do my right foot first since that is the one that gives me the most problems. The doctor's assistant took a mold of my right foot using the same material that is used for orthopedic casts (such as if you broke your wrist), but far fewer layers. We just waited for it to dry and he was able to cut it off. This will now get sent to Arizona where they will make an AFO for me. This will take about 3 weeks and then I will be able to pick it up. After I pick it up I will wear that AFO for about 4 weeks and then go back in for a follow up appointment. If I like it and am happy with the results we will then go ahead and go through the same process for the left AFO.

As I mentioned the doctor was alarmed at how tight the muscles in my calf are. There are two groups of muscles in the calf: one, called the gastrocnemius, is higher up towards your knee and one, called soleus is down lower towards your ankle and runs underneath the gastrocnemius. The gastrocnemius is the muscle that is causing me tension and pain. To remedy this the doctor is having me place a non-stretchable item, such as a towel, under the bottom of my foot while I am laying on my back and pull it upwards so that my foot is flexed towards my head. This is then held for 15 seconds and then repeated four times for each foot. I have to do this twice a day: in the morning and at night. This will help gently stretch the calf muscles out so that they are not so tight. In addition to these stretches the doctor sent me home with a night splint for my foot. This is what it looks like:

 

It looks really big, but it is not that bad. It is not tight enough that it holds my foot firmly in place (I can still move my foot around). It is simply meant to keep my ankle at a 90 degree angle. When you are relaxed your feet naturally drop down and for most people this is fine, but since my muscles are over compensating we need to keep my foot at a 90 degree angle to stretch out those muscles. I wear the splint on one foot one night and then switch it to the other the following night. This gives my muscles a chance to relax every other night so that they are not stretched to the point of injury. I wore the splint on my left foot last night and it is definitely a little bit sore, but nothing unbearable. 

Back to New York

So like I said in my last blog post I've got some big news regarding my neurosurgical care. We are headed back to The Chiari Institute in New York for a tethered spinal cord release surgery. This is why I had to see a urologist and get those tests done.

The urologist did ultrasounds of my bladder and kidneys and a urine test. All of those came back completely normal, which is great! He then did a urodynamics test which studies how well your bladder fills and empties. He claimed this test was normal, but my neurosurgeon and I do not agree. They fill up  your bladder and the amount he put in my bladder was normal, but the output was nearly twice what went in. Obviously this is going to screw with the results of tests, but he didn't want to discuss that... We decided that even though these results are normal on paper that we are going to proceed with the surgery.

A tethered spinal cord is a condition in which the spinal cord is attached to the spinal column with a piece of fatty connective tissue at the bottom of the spine. The spinal cord is supposed to be free floating and able to move up and down freely. This piece of fatty tissue is stretched out over time to the point that it cannot be seen on an MRI. For this reason, it is called occult (or hidden) tethered spinal cord. This causes a progression of symptoms such as low back pain, pain in the legs, feet, and pelvis, decreased sensation in those areas, and urinary and/or bowel issues. Since these symptoms are progressive and irreversible it is highly recommended that surgery be done sooner rather than later.

This surgery will be slightly less lengthy than my previous neurosurgeries. It will take about 3-4 hours hopefully. They will open up my spinal column and cut any filums that are attached to the spinal cord. It is really that straightforward and unless they run into any concerns then it is a relatively low risk surgery. The recovery is also fairly quick. I will be in the hospital for 3-4 days. I have to lay completely flat because the biggest concern is that I will develop a spinal leak.

The surgery is scheduled right now for June 3rd and my pre-op appointment is May 29th. This might change though due to scheduling reasons, so that we do not need to stay in NY for so long. I am hoping that we can do something so that they pre-op appointment and the surgery itself are closer together.

General upate

Hi everyone! There are lots of little updates on how I am doing, so I figured I would make a general update post and then another post for a specific topic I will need to go into more detail about.

I'll start with a quick one first. I had an appointment with my ENT (ear, nose, and throat specialist) today for my 1 year allergy shot check up. I have to see him once a year just to make sure that everything looks good with my sinuses, ears, and throat. Everything looks great and he has no concerns for me! He wants me to try to space my allergy shots out to every 3 weeks instead of the every 2 weeks that I am currently doing. This will help my body to build up it's own immunity to my allergens (which is the goal of allergy shots), so hopefully in another few years I can get off of the shots. I have only been on them for 1.5 years and he said we will evaluate if we can discontinue the shots after 4 years. The only question I had for him was about how one of medications interacted with the allergy shot because when I had done some research I read that this class of medications (beta-blocker) can be contraindicated in allergy shot users. He said that this used to be a concern, but the research has proven that they are not an issue and that we don't need to worry about it at all! I also got a new prescription for epi-pens, since mine are expired (whoops!). Anyone who uses allergy shots is required to have epi-pens in their home just in case they have a severe allergic reaction to the shots, which can happen since the shots are a concoction of all of the things that you are allergic to.

My wrist is doing really well! The pain at rest has completely cleared up and the shooting pain when I lift something or push on something is greatly decreased. My exercises are going really well at home and I have been able to discontinue some of the more basic exercises that we were using at the beginning. We have decreased therapy to once a week instead of twice a week. I have an appointment with my surgeon in 2 weeks so hopefully we will get the all clear from him! I got a new brace as well that my therapist recommended. It is to provide compression and support only, not immobilize like my other ones do. I need to wear it when I am doing a lot of activity or heavy lifting. It is very comfortable and not as restrictive as my other braces, so that is nice!

I started on the vitamin C supplement that my rheumatologist suggested I try. So far I have not had any negative reaction to it, which I was nervous about since my mom reacts negatively to large doses of vitamin C. I haven't noticed a huge difference yet, but most patients say it is a gradual difference that occurs over a longer period of time, so hopefully it will help in the long run. I forgot to mention in my post about the rheumatology appointment that she recommended that I order a book called "Joint Hypermobility Handbook- A Guide for the Issues & Management of Ehlers-Danlos Syndrome Hypermobility Type and the Hypermobility Syndrome". It is written by the geneticist that I am on the wait list for, Dr. Brad Tinkle, and is supposed to be excellent. I am going to order it and will hopefully get some good insight from it! I have an appointment with a podiatrist on Monday to discuss getting fit for ankle foot orthotics to stabilize my hypermobile ankles. I am looking forward to this appointment and hope it will really help me with ankle, knee, and especially hip pain. Maybe it will even help with my lower back pain!

I got a call with the results from my thyroid ultrasound back today as well and that came back clear. This is great because we were concerned that the endocrinologist thought that there may have been a nodule on the right side. Overall, I am doing really well on the new thyroid medication. I am sleeping better and waking up easier. I have more energy in general, which is really nice! I go back May 21st and we will retest my blood levels to see if we need to increase the dosage at all or if I am in normal levels on this dose.


Like I said I have a few appointments coming up, so I will keep you all up to date with how those go. I also have a blog I need to write on the next step in my treatment neurologically, so keep an eye out for that!

EDS Rheumatology Visit

I met with an Ehler's-Danlos Syndrome (EDS) specialist for the first time on Monday. She has only been working with patients with EDS for a couple of year, and admittedly still has a lot to learn, but she is doing research and learning as much as she can! It was a good visit and I got a lot of good information. We talked about how EDS affects me and what my main concerns were regarding EDS. The main thing we discussed at this appointment was pain management. Thankfully I do not have severe joint and muscle pain as a result of the EDS, at least not yet I don't as pain in EDSers tends to get worse as you age, but it is still enough that at times I am quite uncomfortable. She refilled my prescription of an NSAID prescription pain reliever called Celebrex. This medication is similar to Aleve, but is stronger, longer lasting, and easier on the stomach. She also mentioned some options we could try for localized pain like a pain relieving gel or a lidocaine patch, but we are not going to start those yet.

She wants me to see a podiatrist to get ankle foot orthotics made that will stabilize my hypermobile ankles.  My ankles tend to roll easily even when I am walking on flat surfaces and we are hoping that if we stabilize my ankles this will help my knees and hips as well. My hips have been causing me increasingly more problems, so hopefully this will help! I haven't made an appointment yet, but it is on my to do list!

Some individuals with EDS can have a decreased bone density, which can cause major problems later in life. My rheumatologist recommended that I get a bone density screening just so we know if this is something we need to be cautious of as I age. I show no signs of low bone density, but it is important to know if low bone density is present. I was able to do the bone density scan that day and it only took about 8 minutes. It is almost like an open MRI machine that scans your hips and spine and then analyzes how dense the bones are. I got the results a few days later and thankfully my bone density is slightly above average for my age. I will continue to take the calcium supplement I am on (since I do not drink milk or eat a significant amount of dairy) and the vitamin D supplement I am on (since I am vitamin D deficient) and hopefully this will be enough to maintain my bone density as I age.

The final thing that this doctor recommended is that I start taking 2000 mg (or 2 g) of vitamin C everyday. Vitamin C will do a few different things for me. First, as most people know vitamin C boosts the immune system which is crucial for me because if I get sick it is very difficult on my body and I take a lot longer to get over an illness than most people. Second, vitamin C helps to heal bruises quicker and reduce inflammation. I bruise extremely easily because of EDS and my bruises tend to last for a long time. Finally, vitamin C has recently been found to aid in the production of collagen, which is what is defective in EDS. It is not known how taking vitamin C affects EDSers, but there has been some preliminary research and various case studies that suggest that it may help to reduce some of the symptoms of EDS in some patients. I haven't gotten to the store yet, but I am going to at least give this a try! My mom did warn me though that when she was younger she was recommended to take a high dose of vitamin C for bruising and she broke out into a rash. I will need to watch for this when I do start the supplement.

This rheumatologist confirmed that I need to see the geneticist that I am on the wait list for.  I am not sure when I will be able to get in with him as it is a very long list and he is one of the top two EDS specialists in the country (with the other being in Maryland). I got put on the list in December, but it looks like it could be next March before I am able to get in with him. She had me make an appointment for 6 months which is in October just to check back in and see how I am doing. I really liked her and it is nice to now have a specialist addressing my EDS issues!

Endocrionology Results and Hashimoto's Thyroiditis

I finished all of my blood work (which they did through my port super easily! They even had a separate room and nurse just for people with ports!) and ultrasounds yesterday. My endocrinologist called me back yesterday with the results. My adrenal glands were perfectly fine and my B12 actually came back slightly elevated. This will not hurt me and is better than being low, so we are going to leave it alone. My thyroid levels came back in the very high end of normal. Since they were elevated in December, people with my conditions are prone to thyroid issues, I am of child bearing age, and I show symptoms of low thyroid he decided to start me on a very low dose of thyroid medication. I now take 25 mcg (that is 0.000025 grams) of a synthetic thyroid hormone called Synthroid every morning. This is the starting dose and we will adjust it from there.

He diagnosed me with a condition called Hashimoto's Thyroiditis, sometimes called Hashi's for short. This is the most common condition that causes hypothyroidism (under active thyroid). Hashi's is  an autoimmune condition in which your body produces an antibody that attacks your thyroid gland. The disease tends to be progressive; more damage is done the longer the condition goes untreated, which is why it is good that we caught this condition while I am still young. Some symptoms of this disease are: fatigue and sluggishness, increased sensitivity to cold, constipation, unexplained weight gain, muscle aches, joint pain or stiffness, and excessive or prolonged menstrual bleeding (menorrhagia). As you can imagine, many of these symptoms overlap with my other conditions so it is very difficult to tell what disorder causes which symptoms.

One of the main reasons that my doctor decided to start treatment for hypothyroidism is because I am of child bearing age. Although my husband and I do not have immediate plans to have children, it is something that we would like to do at some point in within the next few years. An underactive thyroid can make it difficult to get pregnant, increase the risk of miscarriage, and increase the risk of intellectual and developmental disorders as well as birth defects of the face, nervous system, and thyroid of the baby. We want to start getting my thyroid levels stabilized now so that down the road when we do decide to have children we decrease the risk of running into these issues. 

I do not know what the results of my thyroid ultrasound were, so I will have to ask at my followup appointment in 6 weeks. I go back to see my endocrinologist on May 21st and we will see how I am doing then. He will most likely also order blood work to see how my blood thyroid levels are and if we need to adjust the dosage of the Synthroid.