Vascular Neurosurgeon and Headache Update

After discussing in depth with my phenomenal neurosurgeon we have decided to go a different route than what I had expected to deal with my headaches! Since the medication to decrease my intracranial pressure (Diamox) has been helping so much we are almost certain that my pressures are high even though my lumbar puncture did not show extremely elevated pressures. We know my headaches are worse at night and the lumbar puncture was done in the morning, so that was one reason that the readings from that test came back borderline and not high. Given all of these factors we now have two options. The traditional treatment would be to place a shunt to drain excess cerebrospinal fluid from my brain/spine into my abdominal cavity where it would be reabsorbed. A shunt is device that will drain this fluid to reduce the pressures in my brain. Prior to this treatment I would need to have more monitoring to ensure that my pressures are as high as we believe they are.

The alternative to this procedure, which is a very new idea that has very little research done on it, is a venous stenting. They are finding that in some EDS patients the veins in our brains are clogged. This clog causes a blood to back up and raise the pressures in your brain. A stent can be placed in these veins to open up the blood flow allowing the pressures to come back down in your brain. This is a  new procedure that there is little research done on it in EDS patients. There is one doctor at the University of Virginia that is doing this procedure on EDS patients and he is having great success. Here is an article about that doctor and his use of stents in patients who have EDS.

http://www.uvaphysicianresource.com/venous-stent-procedure-providing-relief-to-some-ehlers-danlos-patients/

The good news for me is that there is another doctor, a vascular neurosurgeon, who is starting to look into this procedure at Cincinnati Children's Hospital. I am located not even 15 minutes from there, so obviously that is extremely convenient for me! To make things even better this doctor trained with my phenomenal neurosurgeon Dr. Rekate in New York, so he is very familiar with EDS and Chiari. I am not yet sure if I even have venous insufficiency or am a candidate for this procedure. What we need to do first is to get a full brain MRI as well as an MRV. An MRV is a special kind of MRI that looks specifically at the veins. This will show us if there are any clogged veins or not. If there are clogged veins then I will go ahead and set up a consultation with this doctor in order to develop a plan and hopefully schedule a procedure to place the shunts. I will be having the MRI/MRV done tonight and then the results will be sent to the doctor and I should hear back from him in a couple of days. I will also send a copy of the disks up to Dr. Rekate in New York so that he is able to view the scans himself as well. I have had to be off of the Diamox, the medication that lowers my intracranial pressure, for the whole weekend so that we can see what my brain looks like with the high pressures and my headaches have been severe all weekend! This is just one more confirmation that it is my pressures that are high that is causing the headaches. I am looking forward to being able to resume the Diamox tonight after the scan!

The stent procedure is a much less invasive surgery than a shunt surgery and is much less risky. Shunts can become infected and often need to be revised, meaning surgery is done again to replace or fix parts of the shunt. They are not an ideal solution, but if that is the route we have to go then I will pursue that option as well. The stent procedure carries many fewer risks and complications, so please pray that this is the solution we are able to pursue.

I have some other updates coming up as well, but I wanted to update on this as we now have more information. I will update again after I hear from the vascular neurosurgeon following my scan tonight and we are able to begin to develop a plan.

Headaches after Fusion and Decompression

As I mentioned in my last post I have been having terrible headaches the past several months. They are different than the headaches I had before my brain and neck/spine surgeries. They are on the top and feel like the top of my head is going to pop off! I did not have any headaches for about 2.5 years after my decompression revision and fusion, where they made more room for the back of my brain and fused my skull to the top two vertebrae in my neck. I am extremely luck and feel very blessed that I did not have any headaches because this is simply not the experience of many patients! That being said, these headaches kind of blindsided me because I had gone for so long without them that I was caught off guard by them!

After a visit with my primary care physician (PCP) we decided to order the CINE MRI, as I discussed previously, which is different than a traditional MRI because it looks at the flow of the fluid that surrounds your brain and spinal cord (cerebral spinal fluid-csf). My PCP thought that it appeared that my CSF flow was blocked by what appeared to be scar tissue from my previous surgeries. We sent the disk off to my neurosurgeon in New York so that he could take a look at it. He does not think that scar tissue build up is the issue and feels that there is something else going on.

In order to find out what more is going on he wanted to do a lumbar puncture. A lumbar puncture is exactly what it sounds like: a needle is used to puncture your lumbar (lower back) spine. They are able to do lots of labs with  test, but for me they wanted to see what the pressures of my CSF were. Your CSF pressure is the same throughout your brain and spinal cord, so they can measure the pressures through this lumbar puncture. My pressure was 16, which is borderline high. It not super elevated, but it is higher than you would expect to see.

Since my pressures were elevated somewhat we decided to start a medication to reduce the intracranial pressure. This medication is called Diamox and must be started very slowly. After building up to the full dosage of this medication (250 mg 3x a day) it has been helping significantly with my headaches! This is great news.... but there is a side effect that has come with this medication (of course it couldn't be that easy!).  I have been having horrible pins and needles in my hands and feet that have been occurring since I have gotten on this medication.  This is an extremely painful sensation and I say it feels like little elves are shoving tiny needles into my hands and feet. The good news is that we do not think this symptom will continue after I discontinue the medication, but the bad news is that we think this side effect will continue for as long as I take the Diamox. Of course we must now decide what to do about this since this side effect is becoming intolerable.

It is looking like I am going to have to discontinue the medication, which of course is not ideal because it is helping my headaches so much! I have a Skype appointment with my neurosurgeon in New York tomorrow afternoon so hopefully we will get more information from him then! Please pray for us to get guidance and peace for whatever the next step may be.

Primary Care Appointment and Other Updates

I had a primary care visit a couple weeks ago (yes I know I am behind on updates again!)  and had many things to discuss with him!

First off, when I had a chest x-ray before one of my surgeries they noted a “sudden change in course of the port tubing possibly as it enters the right internal jugular vein, raising the possibility of kinking". Now my port is still working just fine, but since the port leads directly into my heart any irregularities or concerns need to be treated with urgency. We don't think anything is wrong and it probably just a benign cyst, but we will still check it out. I met with a new general surgeon here in Cincinnati since the doctor that put it in is back in Illinois where we used to live. She again indicated that since the port was working well that she did not see any reason to replace the port, which would be the only course of action to remedy the kinking.  If it stops working because the kinking gets worse only then would we need to replace the port. She did take some x-rays because she wanted a different view than the ones that were done before my surgery, but unfortunately my collar bone was in the way of the cyst and kinking in the tube   that she wanted to see, so we were not able to get the view that she wanted to see. For now we are just going to leave everything be and hope and pray that my port continues to keep working!  

Next, I had had a biopsy of my stomach taken when I had a Botox injection done at the beginning of May. This biopsy was to check for something called mast cells. Mast cells are the cells in your body that respond to allergens. Newer research is showing that mast cells play a role in wound healing and regulating the immune system, but these functions are still not well understood. There is a disorder that is a common comorbid condition with EDS called Mast Cell Activation Syndrome (MCAS). The mast cells in people who have this disorder are not too numerous, rather they are overactive. They respond to things they shouldn’t and respond too severely to things that they should respond too.  It is a very complicated disorder that is not well understood. MCAS can cause GI symptoms, flushing, headaches, rashes, unexplained anaphylaxis (severe, life threatening allergic reaction), and breathing related issues, among other symptoms.  I also had a blood test and did a 24-hour urine connection to check for markers of MCAS. The blood test almost always comes back normal unless a person is having an MCAS reaction at that exact moment the blood is drawn, so we were not surprised that this came back normal for me as well! The 24-hour urine collection also came back as normal, which was a surprise! The biopsies came back with mild-moderate mast cell reaction in my stomach and small intestine. At this point we are not treating the mast cells in any way and they think I have a very mild case of MCAS. The reactions could worsen and I get older and I have had some clinical signs of MCAS in the past such as unexplained anaphylaxis and unexplained rashes/reactions, but for now I am just happy that I do not have one more thing to add to my list!

Now both of those things were very positive updates, but unfortunate I also have a few not so good updates. I have been doing physical therapy for my hip after my hip surgery back in May. My physical therapist is quite pleased with the strength of my muscles and the range of motion of the joint, but unfortunately I am still having pain within the joint. I have pain walking and doing stairs. The physical therapist has tested some different exercises and movements to see if she can pinpoint why the hip is hurting and based on those results she really believes that it is coming from deep within the joint. The surgery I had was very minor and was to repair the torn cartilage in the joint. We did not address the anteversion (turning inward of the hip) because that is a much more invasive surgery and we were not sure if it was necessary, so I am really hoping that is not the problem now :( I was not set to see the orthopedic surgeon until August 24^th, but my physical therapist wanted me to try to get in with him sooner. I called today and was able to get an appointment with him next Wednesday, August 3^rd. I hope he is able to give some insight into why my hip is still hurting and help us to figure out what we can do about it!

The other issue I have been having is severe headaches for a few months now.  I have not had any headaches since my craniocervical fusion 2.5 years ago. The headaches from my Chiari and craniocervical instability were always in the back of my head and down into my neck, but these headaches are on the top of my head. It feels like a ton of pressure and my head is just going to explode! I brought this up to my primary care doctor who also happens to be a headaches specialist. He was concerned that my intracranial pressures are high. Since these headaches are a new occurrence he thinks that my increased pressures are due to scar tissue build up. I have quite a bit of scar tissue in that area from two spinal/brain surgeries that went through the same incision. He ordered a MRI that looks specifically at the CSF (Cerebrospinal fluid), which is the fluid that flows constantly around your brain and spinal cord. When its flow is blocked, for any reason, it can raise the pressure within your skull called the intracranial pressure. The MRI was called a CINE MRI. The results came back and it shows that my CSF flow is blocked in both the front and back of my craniocervical junction (where the head and neck meet). I sent the disk to my neurosurgeon in New York and he is having some technical issues trying to view the disk, but hopefully he will be able to get back to me soon with his thoughts on the images and how he thinks we should proceed.

I think that is all for now! Please pray for my hip and my headaches and that the doctors know how best to help me! Sorry for the long delay between updates! I am almost done with my summer classes and I get about 2 weeks off, so I am looking forward to a much needed break!!!

Gastroparesis Surgery- Pyloromyotomy

I had a surgery done on my stomach last week (6/22) to hopefully help my gastroparesis. Gastroparesis is a condition that causes the stomach to not empty fast enough. For me my stomach empties at 50% the speed of someone with "normal" digestion. Now it is important to note that some have better or worse gastric emptying scans and those numbers do not necessarily coordinate with symptoms.

I had had a botox injection into the pylorus muscle, which is the muscle that forms the sphincter between your stomach and intestines, back at the beginning of may. This test allowed us to see if I had symptom reduction by relaxing the pylorus. The botox injection helped me greatly, but it is only a temporary solution (normally 8-12 weeks, but can last up to 6 months). I had a greater appetite, decreased pain and bloating, less nausea, and could eat larger meals without as many symptoms after the injection.

Since the botox injection went so well I contacted my team up at Cleveland Clinic. The two doctors I see there, a GI specialist and a general surgeon, recommended that I undergo a surgery called a pyloromyotomy. This surgery permanently opened the pyloric sphincter. It is essentially a permanent version of the botox injection. Cleveland Clinic is one of the only places in the world to do this surgery endoscopically. This means that I do not have any incisions on the outside of my abdomen because they did the entire surgery through a tube and cameras down my throat into my stomach.

I went through a full day of testing and appointment at Cleveland Clinic the day before my surgery. I had blood work, EKGs, a chest x-ray, and appointments with anesthesia and my surgeon's nurse.  This was an all day project, but we were able to get it all done and were ready for surgery the next day!

They were very delayed on the day of my surgery, which was not much fun so I did not go back into surgery until about 4:30 in the afternoon. The procedure itself was only about 90 minutes, which is pretty short! They were able to intubate me without dislocating my jaw, so I was super happy about that! I did have some issues waking up from the anesthesia though, which I unfortunately remember! I was shaking uncontrollably; Not small shivers, but violent shaking. They were able to give me some medicine in my IV to control the shaking, but it took a short while to kick and that felt like an eternity. I also was coughing fiercely when I came out of anesthesia. This was probably because they had a breathing tube down my throat as well as the endoscopic equipment to do the surgery, so my throat was irritated form having all of the tubes in it. I was coughing so hard that they were concerned that I was going to throw up. Obviously they did not want me throwing up right after stomach surgery! They were able to get that calmed down after a while as well.

I got to my room that evening and was sadly placed on an NPO diet (stands for Nil per os in latin). which means that I could not have nothing by mouth. I was feeling pretty crummy because I was not even allowed to take my medications. The next morning I was taken down for something called an upper GI series. During this test I had to drink two different kinds of contrast and then they took x-rays in a number of different positions. This test was to ensure that there were no leaks in my stomach or intestines from where he made the incision to cut the pylorus muscle. It was not a painful test, but it was uncomfortable because I was not allowed to take my blood pressure and heart rate medications, so I was dizzy and feeling weak.  We made it through the test and I got to go back up to my room and rest for a little while.

Finally the nurse came in and said that my upper GI series looked good, so I was able to start on a clear liquid diet. A clear liquid diet is exactly what it sounds like: a diet that consists of only liquids you can see through. Jello, juice, frozen ices, broth, water, and popsicles are the staples of a clear liquid diet. It may not sound exciting, but after over 36 hours with NOTHING to eat or drink I was quite excited for a glass of water and popsicle!

I was discharged later that afternoon to go home since I was doing so well. I did not have any pain, but did have a fair amount nausea. When I was discharged I was able to start on a full liquid diet. This diet includes any liquids and any solid that is liquid at room temperature. I can have: pudding,  ice-cream (with no chunks or pieces), yogurt with no fruit in it, sherbet, creamy soups blended to have absolutely no chunks in them, and everything I could have on the clear liquid diet as well. I have since added in applesauce and smoothies with no chunks in them to help me have a little bit more variety. I have been using a lot of protein powder in my smoothies and milkshakes to help get some more protein into me. Needless to say after my popsicle and jell-o lunch the first thing we did when I was discharged was go get a milk shake! I will have to be on the full liquid diet for two weeks, but honestly I am so nauseated right now that I have no desire for solid food.

The nausea has been really intense for me. I have a nausea medication called phenegran, which has been taking the edge off a little bit. It is not working that well and the other typical medication used to treat nausea, zofran, does not help me at all. I spoke with my surgeon's nurse today and she is going to call in a prescription for something called a scopolamine patch. This is a small patch that is put behind your ear to help with nausea and vomiting.  I use this patch when I have surgery to help prevent the nausea and vomiting I have after anesthesia, so we know they work well for me. I am waiting on a call from the pharmacy to tell me they are ready, so I am hoping I can get those on and start getting some relief from the nausea. The nurse also recommended I do an IV infusion of fluids every day instead of the every other day that I typically do. Due to the nausea and not eating and drinking enough they are very concerned I will become dehydrated. We are doing our best to prevent that and I am extremely grateful that I have my port to help me stay hydrated!

I will go back in about 6 weeks to see both my gastroenterologist and my general surgeon to check up on how things are doing. We will not know if the surgery worked until I can begin eating solid food again. I would appreciate prayers for this nausea to stop soon!

Surgery Day

So with the crazy schedule I have had recently I have been behind on updating! We are actually up at Cleveland Clinic right now and I am waiting to go back for surgery! I am having a procedure called a pyloroplasty on my stomach. This procedure permanently opens the valve at the bottom of your stomach to help allow food to pass through easier. I will post a more full update later, but I wanted to let everyone know I am headed back into surgery. This is number 8 under general anesthesia and I always do remarkably well, so we are not concerned. My two requests are that they control nausea and vomiting and that they do not dislocate my jaw! As long as they do those two things I will be happy!  I will be in the hospital for at least one night and we are hoping to go home tomorrow.

I will update as soon as I can! As always prayers are appreciated!

Hip Surgery

 Wow I am way overdue on an update on my hip! It has been a crazy month, that is for sure! My husband and I moved into our new fixer upper house here in Cincinnati. It is not our first home we have owned, but it is our first fixer upper! It has been so much fun to work on the house, but also extremely time consuming! Then my brother moved in with us! I am so excited to have him with us and get to spend so much time together! We haven’t lived together, or even in the same city, for about 8 years so I am beyond thrilled he is here! I also started my summer semester for grad school. I am taking a 5-week intensive neuroscience class, so that has been tons of work on top of my other two classes! All that to say that I have been incredibly busy since my hip surgery!

The surgery itself went extremely well! It was done at a Children’s hospital and they were just exceptional! It was a relatively quick procedure, about 2.5 hours, and they did not find anything that they did not expect to find. They were so accommodating for my service dog Reilly as well!

The surgeon did not end up needing to remove any bone from my femur or pelvis bone, which is wonderful. He did not expect to need to, but we were not 100% sure until he opened me up. If he would have had to take of bone it would have been a longer and harder recovery. He did have to anchor down the cartilage, but we expected that. The cartilage was torn away from the bone, so he basically pinned it down. In addition to anchoring the cartilage he trimmed off the dead pieces of the cartilage. When a tear happens the edges of the cartilage get ragged and began to die. Overall it was a very straightforward surgery and the doctor was very pleased with how it went!

I woke up in recovery and got to my room with no issues. The pain was very minimal and I was feeling pretty good! My hip was more sore and stiff than painful. I did not take any of the strong pain medications that they offered me. I was instructed to take muscle relaxers for 5 days after the surgery and Tylenol for a week. I did this as instructed, but I did not take anything stronger than that! It was a huge relief to not be in a significant amount of pain.

I, unfortunately, have to be on crutches. Even though there is no pain the surgeon wants to give the joint capsule a chance to heal. He had to open the join capsule, which is a sack that contains all of the cartilage and fluids that make up the hip joint, so there is always a chance that this capsule could become damaged if you do not let it heal for an adequate amount of time. I thought it would be two weeks that I would have to be on crutches, but it turns out it will be almost 6 weeks. There was a slight miscommunication with the surgeon’s office regarding the amount of time I would be on crutches, so I was disappointed by that. I still have another 1.5 weeks left on crutches and I am so ready to be done with them!!!! I am looking forward to the day that I can be rid of them for good! I was on them for about 3 months about 6 years ago, so it was very much like riding a bike in the sense that I got back in the swing of using them very quickly.

I go back to the orthopedic surgeon next Friday, so he will do an evaluation then and hopefully clear me to wean off the crutches and start physical therapy back up. I will let you all know how that appointment goes and will be updating about another surgery I have coming up as well!

I leave you with an adorable photo of my service dog Reilly and I in recovery after the surgery

 

Mobility Service Dog Harness

As most of you know I have a mobility service dog name Reilly. He is a 3-year-old golden retriever that I have had since December of 2014. Service dogs are not required to wear any kind of gear stating that they are service dogs, but many handlers choose to mark their service dogs as such in some way because it causes less questions, fewer accesses issues, and makes life easier. I have always chosen to have Reilly marked in some way for these reasons.

I have always used a vest for Reilly in order to identify him as a service dog, but I chose to get a mobility harness for him so that he can provide more support for me. I got his harness from DD Photo and Design who sells on Facebook. The woman who runs the site and makes the harnesses is named Amanda and she has fantastic customer service! She offers many different options and colors, so there is something to fit everyone’s needs!

I am going to take a quick moment to put a disclaimer out there! These numbers are not concrete and ultimately you and your vet should decide if you dog is large enough, healthy enough, and old enough to do mobility work, but these numbers are a rough estimate to go by. A dog who is expected to do light mobility work, such as counter balance and momentum pulling, needs to be at MINIMUM 30% of you weight and 30% of your height (at the shoulders). For heavy mobility work, such as bracing, your dog should be at MINIMUM 50% of your weight and 40% of your height. This would mean if you weigh 150 pounds and are 5 foot 6 inches tall your dog should be at least 75 pounds and be 26.5 inches tall to the the shoulder. That being said I would not use a small dog for mobility work even if you do weigh only 100 pounds! Additionally, your dog needs to be cleared by a vet to do mobility work and is not normally physically ready for mobility work until around their second birthday.

That being said Reilly is tall enough, old enough, and weighs enough to do heavy mobility work for me. This harness will allow him to do more momentum pulling and be more comfortable while doing his job. 

Here is what Reilly's new harness looks like on him. 

 

And here are a couple of photos of the harness off of him.

 

There are numerous features that you can choose from on these harnesses. First, his harness has a Y-front which provides more stability and greater comfort for him as it distributes the weight better than a straight front harness does. I also chose to get a detachable handle. The handle is fed through a couple of rings and clips onto the harness itself. This allows me to remove the handle if he needs to fit  into a tight space, if he will be laying down for an extended period of time, or if I am using my wheelchair and won't needing the handle for a while. It is just convent to have the option to remove the handle if I want to.  I also chose to get a padded handle. The handle has handle, and all of the harness actually, is lined with teal fleece. This just makes the handle all the more comfortable! The next upgrade I made was to have a double buckle put on. This means that I don't have to try to have him step into one side and pull on the other. It has buckles on either side, which means that I just put the front piece between his legs and buckle it on either side of his belly. The harness does have velcro on these straps as well to adjust them. 

The final upgrade I decided to make was cosmetic. I chose to have SERVICE DOG embroidered on either side of the harness itself. This is just one more way to make sure that people know he is a service dog and hopefully prevent people from coming up and trying to pet him! I also chose the teal color, but Amanda has a bunch of colors to choose from! I thought the teal looked nice on him though!

I chose to get a cape that snaps onto the back of his harness. The cape has zippered pockets which is incredibly useful to store things in! The red patches on either side of the cape say "Mobility Service Dog" and above that it says "DO NOT PET". On the top of the cape There is a large black patch that says "Not all disabilities are visible" along with a small "DO NOT PET" patch. I really wanted the not all disabilities are visible patch because I am constantly questioned as to why I have a service dog since I do not visibly have a disability and people always think that I am training him because, again, I don't look disabled. On the handle of the harness itself I have two patches Velcroed together that have stop signs on them and say "Service Dog Do Not Pet". I wanted to have something on the harness itself that said do not pet so that if I chose not to put the cape on him that message was still clearly displayed. All of his patches came from an Etsy shop called DannyLuannEmbroidery and the woman who made the harness sewed the patches onto the cape for me!

This harness was definitely an investment, but will last Reilly the rest of his working career and I may be able to use it on my next service dog if he/she is of a similar size to Reilly and since it is adjustable there is a degree of flexibility to the sizing of the harness. The harness itself was $90 with all of the upgrades, the cape was $30, and all of the patches were $50. Like I said it was an investment, but one that will be well worth it! If you are looking for a mobility harness I would definitely recommend you check out DD Photo and Design on Facebook!