Saturday, March 26, 2016
Skype Appointment with The Chiari Institute
Although I have had two surgeries at The Chiari Institute in New York I have never had a Skype appointment before! I sent Dr. Rekate my MRI's in January, but due to his own surgery he was unable to meet with me until March. The appointment went very, very well! Dr. Rekate is one of the nicest doctors I have ever met. He cares about not only his patient's health, but there well being overall. He wanted to ask about how both my husband and I are doing and was excited to hear the new things in our lives! He was extremely please with the progress I have made and the symptoms that I no longer have. I turned my head to look at my husband, who was sitting next to me, and he was amazed at the degree of mobility that I have! Most patients lose a significant amount of mobility after a fusion (mine is skull to the second cervical vertebra) and although I have a tiny decrease in my range of motion I do not notice it functionally at all! Overall Dr. Rekate says my MRI looks "perfect"! He does not see any signs of early arthritis, disk degeneration, or spinal cord damage. I was extremely relieved to hear that everything looked ok! Dr. Rekate also asked if he could have his research assistant contact me and discuss exactly what symptoms have gone away and which have persisted. As I have discussed in a previous post Dr. Rekate actually named a syndrome after me called Sara Syndrome. I am so grateful for the research he continues to do on complex patients like me because one day he just might find out the cause of Sara Syndrome which will help us know how to treat it better. I will send Dr. Rekate MRI's again next year as I like to have him take a look once a year to make sure everything looks ok. Until then it is nice to know that neurosurgery is not something I need to think about right now :D
Friday, March 11, 2016
Scheduling Appointments
There are lots of things going on with me medically right now!
First off I did get a Skype appointment with my neurosurgeon, which will be on March 21. I am anxiously waiting to see what he has to say about my latest cervical MRI. I trust him completely and know that I am in very good hands with him, but I am still hoping that he did not find anything that needs another surgery!
The other appointment I was waiting on was from Cleveland Clinic. My insurance company did not cover the smart pill test that the GI doctor ordered. They consider it "experimental", which we expected, but is still frustrating. I will go on April 6th back to Cleveland (a four hour trip each way from my home in Cincinnati). I am going to meet with my GI doctor as well as a general surgeon. We will be discussing if the gastric stimulator is a good fit for me, which my GI doctor has already indicated he believes it is. We will most likely schedule a surgery date during that visit, unless they find something unexpected! I am nervous for this, as I have never had abdominal surgery, but also hopeful because if the stimulator works well I may be able to enjoy eating again!
Those were the two big things I have been waiting to hear back on, so it is nice to have those things scheduled! I still have not heard if insurance will cover my TMJ treatment in network or not, so hopefully we hear back on that soon! We did already have to appeal it once, but it may be a long process before we know the final answer on that one.
I went back once to the TMJ doctor to have him evaluate how my jaw was doing with the splint and unfortunately it did not go very well. My jaw slid forward over the appliance, so it was not in the optimal position. To counter this the doctor raised the height of the splint in the back so that my jaw would not be able to slide forward. I will go back March 31 to see how this new adjustment has been working.
Lots of minor things have been happening as we settle into our new home in Cincinnati. I have been established with a new physical therapist here, which has been amazing! She is incredibly knowledgeable about EDS and has been very helpful! I have been having issues with my left hip and she and I both agree that I have a cartilage tear in my hip. I will be seeing an orthopedic surgeon on March 23 to have him evaluate my hip. He will no doubt order an MRI of the hip. If he does find a tear it will require a minor surgery to clean up the cartilage tear.
My service dog Reilly had a persistent yeast infection in his ear, but I think we have gotten that cleared up! I am relieved that he is feeling better! I depend on him for so much that it just breaks my heart when he is not feeling well. He has had a yeast infection in his ear in the past, but this one did not seem to bother him as much at least!
I got a new IV pump today, which is a huge relief for me! My pump allows me to go anywhere with my fluids. I just throw the IV bag and the pump into a backpack or in a pack my service dog carries and I can do them anywhere. Without the pump I am stuck at home for three hours, so it will be very nice to have my freedom back!
I think that is about all I have for now. LOTS of appointments coming up in the next month or so, which will hopefully help me to start feeling better!
First off I did get a Skype appointment with my neurosurgeon, which will be on March 21. I am anxiously waiting to see what he has to say about my latest cervical MRI. I trust him completely and know that I am in very good hands with him, but I am still hoping that he did not find anything that needs another surgery!
The other appointment I was waiting on was from Cleveland Clinic. My insurance company did not cover the smart pill test that the GI doctor ordered. They consider it "experimental", which we expected, but is still frustrating. I will go on April 6th back to Cleveland (a four hour trip each way from my home in Cincinnati). I am going to meet with my GI doctor as well as a general surgeon. We will be discussing if the gastric stimulator is a good fit for me, which my GI doctor has already indicated he believes it is. We will most likely schedule a surgery date during that visit, unless they find something unexpected! I am nervous for this, as I have never had abdominal surgery, but also hopeful because if the stimulator works well I may be able to enjoy eating again!
Those were the two big things I have been waiting to hear back on, so it is nice to have those things scheduled! I still have not heard if insurance will cover my TMJ treatment in network or not, so hopefully we hear back on that soon! We did already have to appeal it once, but it may be a long process before we know the final answer on that one.
I went back once to the TMJ doctor to have him evaluate how my jaw was doing with the splint and unfortunately it did not go very well. My jaw slid forward over the appliance, so it was not in the optimal position. To counter this the doctor raised the height of the splint in the back so that my jaw would not be able to slide forward. I will go back March 31 to see how this new adjustment has been working.
Lots of minor things have been happening as we settle into our new home in Cincinnati. I have been established with a new physical therapist here, which has been amazing! She is incredibly knowledgeable about EDS and has been very helpful! I have been having issues with my left hip and she and I both agree that I have a cartilage tear in my hip. I will be seeing an orthopedic surgeon on March 23 to have him evaluate my hip. He will no doubt order an MRI of the hip. If he does find a tear it will require a minor surgery to clean up the cartilage tear.
My service dog Reilly had a persistent yeast infection in his ear, but I think we have gotten that cleared up! I am relieved that he is feeling better! I depend on him for so much that it just breaks my heart when he is not feeling well. He has had a yeast infection in his ear in the past, but this one did not seem to bother him as much at least!
I got a new IV pump today, which is a huge relief for me! My pump allows me to go anywhere with my fluids. I just throw the IV bag and the pump into a backpack or in a pack my service dog carries and I can do them anywhere. Without the pump I am stuck at home for three hours, so it will be very nice to have my freedom back!
I think that is about all I have for now. LOTS of appointments coming up in the next month or so, which will hopefully help me to start feeling better!
Thursday, March 3, 2016
Port and IV FAQs
I get a ton of questions about my port and IVs, so I figured I would do a sort of FAQ on both questions I get asked frequently and questions I had before I got my port and started IV's.
Q: Why do you have a port?
A: I have a port because I have a condition called dysautonomia (or autonomic nervous system dysfunction) which causes me to have low blood pressure and low blood volume. I do IV saline to raise my blood pressure and boost blood volume.
Q: What do you use your port for?
A: I only ever do IV saline through my port at home. I do one liter of normal saline over 3 hours every other day. If I am in the hospital it can be used for medications, blood work, and to sedate me for surgeries. I never have to get a peripheral IV, which is a huge plus because my veins stink!
Q: What are some other reasons a patient may need a port?
A: One of the most common reasons a patient needs a port is for chemotherapy. Chemo is so harsh that it can not be put through smaller veins in your arms and hands, so it is placed directly into the arteries near the heart because they are larger and can withstand the harsh medication. Other patients who often have ports are patients who often need to go on IV antibiotics (such as Cystic Fibrosis patients), patients you need to receive nutrition through their veins because of various digestive diseases (Called TPN or total parenteral nutrition), and patients who are frequently having blood draws, hospital stays, or surgeries.
Q: How does a port work?
A: A port is a small metal device that is connected to a catheter (tube) that is placed into a large artery near your heart. On top of the metal device is a rubber-like pad that a needle can be placed into. The port is completely under the skin and is normally located on your upper chest. You can feel it and normally can see a bump where the port is. A needle is place through the skin into the rubber-like pad and secured in place. Then fluids, medication, TPN, chemo, or any other IV substance is distributed through the circulatory system.
Q: How is a port put in?
A: A port is normally placed through a minor surgery. You are put under twilight sedation, which means you do not have to be intubated and twilight sedation is much safer than general anesthesia. The procedure is short (about 45 minutes to an hour normally).
Q: Did it hurt when your port was placed?
A: It did not hurt exactly, but it was uncomfortable. I was very bruised (see photo below which was taken four days after my port was placed) and it was sore to raise my right arm higher than shoulder height. I couldn't brush or wash my hair for about 4 or 5 days, but after that it was not bad at all.
Q: Where is your port located?
A: My port is located in my right subclavian artery. This means my port is about 4 or 5 inches below my right collarbone and about the same distance away from my right armpit. The port is located under the lateral incision. The red dot is where the needle goes into my port.
Q: How long have you had your port and how long will you have it for?
A: I have had my port for two years (it was put in in November of 2013). I will have my port indefinitely at this point. As long as it continues working and I have no complications I will have my port. We hope that it lasts for many MANY years to come!
Q: Have you had any complications with your port?
A: A few months after I got my port we had some issues with my port tilting. It would turn so that the rubber-like "target" pad was facing my armpit instead of facing straight forward. We are still not quite sure why this happened, but thankfully it resolved itself. That is the only complication we have had thankfully!
Q: What kind of port do you have?
A: I have a single lumen (only one access point, there are some ports that have two access points so two medication can be administered at one time) Bard power port. A power port means that I can have CT contrast injected into my port. Not all ports can handle CT contrast, so make sure you talk to your doctor about what kind of port is right for you.
Q: Can you still have an MRI if you have a metal port?
A: Yep! Ports are made of a kind of metal that does not interact with the magnets in MRIs so it is safe to have an MRI while you have a port.
Q: Will a port set off a metal detector?
A: Most times the small about of metal in a port will not set off a metal detector. I have been asked to allow someone to swab my port at the airport, but I have never set off the metal detectors.
Q: Why are ports not recommended for more dysautonomia patients?
A: The largest risk associated with a port (or any central line) is sepsis. Sepsis is a potentially life threatening infection in the blood stream that can be caused by having a port since a port is put into an artery connecting to the heart. If medication, lifestyle alterations, or other means can be used to adequately manage dysautonomia those are much safer than a central line.
Q: What does access and deaccess mean?
A: Accessing is when a needle is placed through the skin and into the port. Deaccessing is when this needle is taken out. The following picture is what my port looks like when it accessed.
Q: Do you numb your port before you accesses it?
A: I personally do not numb my port. It is an option that many patients like, but I have so much scar tissue at this point that the needle stick is less painful than a shot to me.
Q: Does your port hurt or feel uncomfortable?
A: Nope! I can't feel my port at all, even when it is accessed. It causes no discomfort for me. The only time I can feel it is if I try to lay on my stomach, which I can't do because of my neck anyways!
Q: Can you go swimming, take a shower, or take a bath with your port?
A: If your port it deaccessed there are absolutely zero restrictions (after you have healed from the surgery of course!) since the port is completely under the skin. When you are accessed you cannot submerge your port in water, so you cannot take a bath or go swimming. Some people chose not to shower while they have their port accessed, but I choose to cover my entire site with a waterproof dressing while I shower.
Q: Who takes care of your port?
A: I do all of the necessary port care myself. My husband can also do all of my port care, so if I am sick or have had surgery he does it for me. I was taught by a home health care nurse how to access, deaccess, run fluids, use my pump, and flush my line. Not everyone takes care of their own port, so make sure you talk to your doctor if this is something that you are considering doing!
Q: Is it hard to access your own port?
A: There was definitely a learning curve and it took me a few months to be able to do it on the first try. Now it is just part of my routine, but it is hard to do at first!
Q: How often do you use your port?
A: I use my port for fluids every other day. This means I am accessed 5 days a week and then will reaccess it 2 days later.
Q: How do you get your supplies?
A: I have a home health care company that delivers all of my IV supplies directly to my door. We call once a month and tell them what we need more of and they have someone come deliver the next business day.
Q: Does you insurance cover your supplies?
A: I have had multiple different insurance company (Humana, Cigna, United Health Care, Blue Cross Blue Shield of Illinois, and Blue Cross Blue Shield of Minnesota) and they have all covered my supplies. I have never had an issue with any of these companies denying my supplies.
Q: Which doctor ordered your port and your supplies?
A: My port and supplies are all ordered by my autonomic neurologist. He is the one who manages my dysautonomia. He was extremely open to me doing IV fluids since we had exhausted medicine options and my quality of life was still poor.
Q: What type of needle do you use with your port?
A: You have to use a special kind of needle with a port called a huber needle. A huber needle is bent at a right angle and is specially designed to not damage your port. I personally use a 1 inch 20 gage needle. I wish I could use a smaller needle, but the smaller needle offered by my home health company is a smaller gage (width of the opening of the needle) and takes much too long to deliver my fluids.
Q: What type of maintenance do you have to do with a port?
A: The minimum maintenance that must be done with a port is to flush it with saline and/or heparin (a blood thinner) once a month. This helps to ensure that the port does not get a clot in it and stop working. This maintenance MUST be done once a month whether or not you use the port during the rest of the month. If you are using your port the needle and dressing must be changed once every week.
Q: Do you use heparin flushes with your port?
A: I do use heparin flushes after every time I use my port. The type of port I have does not require the use of heparin, but I like to use it anyways because it is one more line of protection against blood clots. If your port clots off and the clot cannot be broken up then the port has to be removed and a new one inserted in a different spot.
Q: Do you use an IV pump or gravity lines?
A: I use both. If I am going to be home I do a gravity line. This is where you set a dial to the speed you would like the IV to go, hang the bag on a pole, and then the IV drips down through a tube you connect to your port. If I am going to not be at home, like when I was working full time, I prefer to use an electronic pump. The pump is powered by a nine-volt battery and is about the size of a CD case, but much thicker. The pump can be placed in a backpack or in a pack that my service dog carries, so it can go with me wherever I go. This is nice because it gives me the freedom to not be stuck at home for three hours while I do an IV.
Q: Why do you have a port?
A: I have a port because I have a condition called dysautonomia (or autonomic nervous system dysfunction) which causes me to have low blood pressure and low blood volume. I do IV saline to raise my blood pressure and boost blood volume.
Q: What do you use your port for?
A: I only ever do IV saline through my port at home. I do one liter of normal saline over 3 hours every other day. If I am in the hospital it can be used for medications, blood work, and to sedate me for surgeries. I never have to get a peripheral IV, which is a huge plus because my veins stink!
Q: What are some other reasons a patient may need a port?
A: One of the most common reasons a patient needs a port is for chemotherapy. Chemo is so harsh that it can not be put through smaller veins in your arms and hands, so it is placed directly into the arteries near the heart because they are larger and can withstand the harsh medication. Other patients who often have ports are patients who often need to go on IV antibiotics (such as Cystic Fibrosis patients), patients you need to receive nutrition through their veins because of various digestive diseases (Called TPN or total parenteral nutrition), and patients who are frequently having blood draws, hospital stays, or surgeries.
Q: How does a port work?
A: A port is a small metal device that is connected to a catheter (tube) that is placed into a large artery near your heart. On top of the metal device is a rubber-like pad that a needle can be placed into. The port is completely under the skin and is normally located on your upper chest. You can feel it and normally can see a bump where the port is. A needle is place through the skin into the rubber-like pad and secured in place. Then fluids, medication, TPN, chemo, or any other IV substance is distributed through the circulatory system.
Q: How is a port put in?
A: A port is normally placed through a minor surgery. You are put under twilight sedation, which means you do not have to be intubated and twilight sedation is much safer than general anesthesia. The procedure is short (about 45 minutes to an hour normally).
Q: Did it hurt when your port was placed?
A: It did not hurt exactly, but it was uncomfortable. I was very bruised (see photo below which was taken four days after my port was placed) and it was sore to raise my right arm higher than shoulder height. I couldn't brush or wash my hair for about 4 or 5 days, but after that it was not bad at all.
Q: Where is your port located?
A: My port is located in my right subclavian artery. This means my port is about 4 or 5 inches below my right collarbone and about the same distance away from my right armpit. The port is located under the lateral incision. The red dot is where the needle goes into my port.
Q: How long have you had your port and how long will you have it for?
A: I have had my port for two years (it was put in in November of 2013). I will have my port indefinitely at this point. As long as it continues working and I have no complications I will have my port. We hope that it lasts for many MANY years to come!
Q: Have you had any complications with your port?
A: A few months after I got my port we had some issues with my port tilting. It would turn so that the rubber-like "target" pad was facing my armpit instead of facing straight forward. We are still not quite sure why this happened, but thankfully it resolved itself. That is the only complication we have had thankfully!
Q: What kind of port do you have?
A: I have a single lumen (only one access point, there are some ports that have two access points so two medication can be administered at one time) Bard power port. A power port means that I can have CT contrast injected into my port. Not all ports can handle CT contrast, so make sure you talk to your doctor about what kind of port is right for you.
Q: Can you still have an MRI if you have a metal port?
A: Yep! Ports are made of a kind of metal that does not interact with the magnets in MRIs so it is safe to have an MRI while you have a port.
Q: Will a port set off a metal detector?
A: Most times the small about of metal in a port will not set off a metal detector. I have been asked to allow someone to swab my port at the airport, but I have never set off the metal detectors.
Q: Why are ports not recommended for more dysautonomia patients?
A: The largest risk associated with a port (or any central line) is sepsis. Sepsis is a potentially life threatening infection in the blood stream that can be caused by having a port since a port is put into an artery connecting to the heart. If medication, lifestyle alterations, or other means can be used to adequately manage dysautonomia those are much safer than a central line.
Q: What does access and deaccess mean?
A: Accessing is when a needle is placed through the skin and into the port. Deaccessing is when this needle is taken out. The following picture is what my port looks like when it accessed.
Q: Do you numb your port before you accesses it?
A: I personally do not numb my port. It is an option that many patients like, but I have so much scar tissue at this point that the needle stick is less painful than a shot to me.
Q: Does your port hurt or feel uncomfortable?
A: Nope! I can't feel my port at all, even when it is accessed. It causes no discomfort for me. The only time I can feel it is if I try to lay on my stomach, which I can't do because of my neck anyways!
Q: Can you go swimming, take a shower, or take a bath with your port?
A: If your port it deaccessed there are absolutely zero restrictions (after you have healed from the surgery of course!) since the port is completely under the skin. When you are accessed you cannot submerge your port in water, so you cannot take a bath or go swimming. Some people chose not to shower while they have their port accessed, but I choose to cover my entire site with a waterproof dressing while I shower.
Q: Who takes care of your port?
A: I do all of the necessary port care myself. My husband can also do all of my port care, so if I am sick or have had surgery he does it for me. I was taught by a home health care nurse how to access, deaccess, run fluids, use my pump, and flush my line. Not everyone takes care of their own port, so make sure you talk to your doctor if this is something that you are considering doing!
Q: Is it hard to access your own port?
A: There was definitely a learning curve and it took me a few months to be able to do it on the first try. Now it is just part of my routine, but it is hard to do at first!
Q: How often do you use your port?
A: I use my port for fluids every other day. This means I am accessed 5 days a week and then will reaccess it 2 days later.
Q: How do you get your supplies?
A: I have a home health care company that delivers all of my IV supplies directly to my door. We call once a month and tell them what we need more of and they have someone come deliver the next business day.
Q: Does you insurance cover your supplies?
A: I have had multiple different insurance company (Humana, Cigna, United Health Care, Blue Cross Blue Shield of Illinois, and Blue Cross Blue Shield of Minnesota) and they have all covered my supplies. I have never had an issue with any of these companies denying my supplies.
Q: Which doctor ordered your port and your supplies?
A: My port and supplies are all ordered by my autonomic neurologist. He is the one who manages my dysautonomia. He was extremely open to me doing IV fluids since we had exhausted medicine options and my quality of life was still poor.
Q: What type of needle do you use with your port?
A: You have to use a special kind of needle with a port called a huber needle. A huber needle is bent at a right angle and is specially designed to not damage your port. I personally use a 1 inch 20 gage needle. I wish I could use a smaller needle, but the smaller needle offered by my home health company is a smaller gage (width of the opening of the needle) and takes much too long to deliver my fluids.
Q: What type of maintenance do you have to do with a port?
A: The minimum maintenance that must be done with a port is to flush it with saline and/or heparin (a blood thinner) once a month. This helps to ensure that the port does not get a clot in it and stop working. This maintenance MUST be done once a month whether or not you use the port during the rest of the month. If you are using your port the needle and dressing must be changed once every week.
Q: Do you use heparin flushes with your port?
A: I do use heparin flushes after every time I use my port. The type of port I have does not require the use of heparin, but I like to use it anyways because it is one more line of protection against blood clots. If your port clots off and the clot cannot be broken up then the port has to be removed and a new one inserted in a different spot.
Q: Do you use an IV pump or gravity lines?
A: I use both. If I am going to be home I do a gravity line. This is where you set a dial to the speed you would like the IV to go, hang the bag on a pole, and then the IV drips down through a tube you connect to your port. If I am going to not be at home, like when I was working full time, I prefer to use an electronic pump. The pump is powered by a nine-volt battery and is about the size of a CD case, but much thicker. The pump can be placed in a backpack or in a pack that my service dog carries, so it can go with me wherever I go. This is nice because it gives me the freedom to not be stuck at home for three hours while I do an IV.
Wednesday, February 17, 2016
EDS Documentary
Here is a short EDS documentary centered on a young woman (around my age) who has EDS. Her name is also Sarah (she has an H though) and she has a similar story to mine. She is in Canada, so some of the aspects of the healthcare system are different for her than me. The documentary is only about 12 minutes long, but it is a wonderful video that portrays many of the realities and struggles of living with EDS. You can watch the documentary, entitled "Still Talking" over at https://vimeo.com/125161284. Sarah also has a Facebook page that chronicles her journey with EDS and other conditions, as well as raises awareness for these conditions, called "Sarah's Shout: Fighting and Raising Awareness for EDS and More". If you would like to follow her for more updates on her journey her page can be found here: https://www.facebook.com/sarahsshout/?fref=ts
Thanks as always for helping to raise awareness and understanding for EDS and related conditions!
Thanks as always for helping to raise awareness and understanding for EDS and related conditions!
Hurry up and.... wait
I feel like that is the story of my life right now! We are waiting (impatiently on my end) for so many things right now! As I mentioned in my TMJ post we are waiting to hear if insurance will cover my TMJ treatment or not. In theory they should, but I have feeling that it will take a fight to get it covered as in network. We should hear back about that soon hopefully!
We are also waiting to hear back for out insurance company on whether or not they will cover the Smart Pill motility monitoring test that my GI doctor at the Cleveland Clinic wants done. This is the test that will determine whether or not I am eligible for the gastric stimulator. The insurance lady at the doctors office told us that Blue Cross Blue Shield will not cover this test, but we cannot appeal their decision until we get the denial letter in the mail. We should hear about this next week, but I am not optimistic. We will try to fight it, but from the sounds of it the insurance company has deemed this test "experimental", so they will not cover it. The frustrating part of this is that they will pay $30,000 for surgery to put in the gastric stimulator and then will pay nearly that amount again to take it out if it does not work, but they refuse to pay less than $2,000 to do a test to confirm that there is a good chance (obviously there is never a guarantee) this MAJOR abdominal surgery will help me. We are not sure what will happen if the insurance company refuses to pay for this test, so please keep us in your prayers that we, along with my doctors, can make the best decisions possible.
On the topic of my GI status right now, the new medication (Remeron) that Dr. Cline at the the Cleveland Clinic prescribed helps a little bit, but is not nearly as effective as I would like. It is the best option right now, but I am still praying that we will be able to pursue the gastric stimulator sooner rather than later because I am not eating very much right now and am having nausea and abdominal pain.
We made the decision with my autonomic neurologist to go back on a medication called fludrocortisone which helps raise my blood pressure. I had come off of it last fall because it is a medication that depletes my potassium and is considered a hormone altering medication (corticosteroid), so if possible we wanted to see if I could go off of it. I did ok without it, but I was having black out episodes more and more frequently without it. This is when my vision goes black and if I do not sit down I will faint. I have gotten really good about knowing when I need to sit and haven't fainted in a few years, but blackouts are not a fun feeling! We decided to go back on the medication because it is not worth risking fainting and the fludrocortisone was clearly helping more than we thought! It will take another week or two to see if this helps bring my blood pressure up, but it should since it has worked in the past.
Last week I saw a primary care doctor that my geneticist recommended to me. My geneticist, Dr. Tinkle, used to be located in Cincinnati for many years, so he has numerous contacts here. This primary care doctor is not taking new patients, but since Dr. Tinkle recommended him he was willing to take me on! I had a "get to know you" appointment last week and it went so well! He is extremely knowledgeable on EDS and the related conditions I have, which is so nice! I didn't have to explain anything to him! That just simply never happens! It is so nice to have a doctor that is able to manage all of my health conditions and symptoms who is only ten minutes from my house! I will go back to see him in two months since we only had a 1/2 hour initial appointment.
I sent an MRI I had done of my cervical spine to my neurosurgeon last month just as a follow up. It has been two years since my cranialcervical fusion, so we just want to do an MRI to ensure that all of my hardware (screws and rods) is still in good shape and that everything is looking good. Unfortunately my neurosurgeon had to have surgery on his ankle, so he was not able to get back to me until last week. He wants me to have a Skype appointment with him (he is located in New York and I am in Cincinnati so it is not easy to have an in-person appointment). I have not yet heard back form his assistant to schedule this appointment. Please pray that he did not find anything major and that we will not have to go back to New York (for the third time) for another neurosurgery! I hope that we hear back from his office soon!!!!
On a more positive note I will see a new physical therapist next week. She is another profession who was recommended to me by Dr. Tinkle, my geneticist. She is very knowledgeable about EDS and I am excited to get back into PT. Many EDSers benefit from PT because by strengthening our muscles it can help to support our joints. It is VERY important, however, to find a PT who is knowledgeable about EDS because a PT who does not understand EDS can do more harm than good. This PT is located very close to my house, which is nice since when we were in Chicagoland I had to drive about 45 minutes to get to the PT office! I will start off seeing her twice a week and then we can decide from there what frequency will work best.
As you can see we are waiting on a number of different things right now and I am trying to be patient, but I am not a very patient person by nature ;) I will update as we start to hear back from the people we are waiting on, but don't expect news anytime soon. Insurance companies work at one speed: slow. I think that is all I have to update on right now. As allows thank you for all of your thoughts and prayers they mean so much to me!
We are also waiting to hear back for out insurance company on whether or not they will cover the Smart Pill motility monitoring test that my GI doctor at the Cleveland Clinic wants done. This is the test that will determine whether or not I am eligible for the gastric stimulator. The insurance lady at the doctors office told us that Blue Cross Blue Shield will not cover this test, but we cannot appeal their decision until we get the denial letter in the mail. We should hear about this next week, but I am not optimistic. We will try to fight it, but from the sounds of it the insurance company has deemed this test "experimental", so they will not cover it. The frustrating part of this is that they will pay $30,000 for surgery to put in the gastric stimulator and then will pay nearly that amount again to take it out if it does not work, but they refuse to pay less than $2,000 to do a test to confirm that there is a good chance (obviously there is never a guarantee) this MAJOR abdominal surgery will help me. We are not sure what will happen if the insurance company refuses to pay for this test, so please keep us in your prayers that we, along with my doctors, can make the best decisions possible.
On the topic of my GI status right now, the new medication (Remeron) that Dr. Cline at the the Cleveland Clinic prescribed helps a little bit, but is not nearly as effective as I would like. It is the best option right now, but I am still praying that we will be able to pursue the gastric stimulator sooner rather than later because I am not eating very much right now and am having nausea and abdominal pain.
We made the decision with my autonomic neurologist to go back on a medication called fludrocortisone which helps raise my blood pressure. I had come off of it last fall because it is a medication that depletes my potassium and is considered a hormone altering medication (corticosteroid), so if possible we wanted to see if I could go off of it. I did ok without it, but I was having black out episodes more and more frequently without it. This is when my vision goes black and if I do not sit down I will faint. I have gotten really good about knowing when I need to sit and haven't fainted in a few years, but blackouts are not a fun feeling! We decided to go back on the medication because it is not worth risking fainting and the fludrocortisone was clearly helping more than we thought! It will take another week or two to see if this helps bring my blood pressure up, but it should since it has worked in the past.
Last week I saw a primary care doctor that my geneticist recommended to me. My geneticist, Dr. Tinkle, used to be located in Cincinnati for many years, so he has numerous contacts here. This primary care doctor is not taking new patients, but since Dr. Tinkle recommended him he was willing to take me on! I had a "get to know you" appointment last week and it went so well! He is extremely knowledgeable on EDS and the related conditions I have, which is so nice! I didn't have to explain anything to him! That just simply never happens! It is so nice to have a doctor that is able to manage all of my health conditions and symptoms who is only ten minutes from my house! I will go back to see him in two months since we only had a 1/2 hour initial appointment.
I sent an MRI I had done of my cervical spine to my neurosurgeon last month just as a follow up. It has been two years since my cranialcervical fusion, so we just want to do an MRI to ensure that all of my hardware (screws and rods) is still in good shape and that everything is looking good. Unfortunately my neurosurgeon had to have surgery on his ankle, so he was not able to get back to me until last week. He wants me to have a Skype appointment with him (he is located in New York and I am in Cincinnati so it is not easy to have an in-person appointment). I have not yet heard back form his assistant to schedule this appointment. Please pray that he did not find anything major and that we will not have to go back to New York (for the third time) for another neurosurgery! I hope that we hear back from his office soon!!!!
On a more positive note I will see a new physical therapist next week. She is another profession who was recommended to me by Dr. Tinkle, my geneticist. She is very knowledgeable about EDS and I am excited to get back into PT. Many EDSers benefit from PT because by strengthening our muscles it can help to support our joints. It is VERY important, however, to find a PT who is knowledgeable about EDS because a PT who does not understand EDS can do more harm than good. This PT is located very close to my house, which is nice since when we were in Chicagoland I had to drive about 45 minutes to get to the PT office! I will start off seeing her twice a week and then we can decide from there what frequency will work best.
As you can see we are waiting on a number of different things right now and I am trying to be patient, but I am not a very patient person by nature ;) I will update as we start to hear back from the people we are waiting on, but don't expect news anytime soon. Insurance companies work at one speed: slow. I think that is all I have to update on right now. As allows thank you for all of your thoughts and prayers they mean so much to me!
TMJ Specialist
A couple of weeks ago I was able to get in to see a TMJ (temporomandibular joint) expert who also specializes in EDS. He is only about an hour away from my home and was well worth the trip! It is so nice to see doctors who are not only knowledgeable about their speciality, but also know, work with, and research EDS. EDS causes complications in nearly every part of the body since it is a generalized connective tissue disorder (affects ALL of the connective tissue in my entire body). The issues us EDSers have tend to be unique to us and many doctors have no idea what to do with us. This was not the case with Dr. Mitakides! He says I am a difficult, puzzling case (what's new there!), but thankfully because he is so familiar with EDS it will be no problem for him! In contrast the last "expert" I saw for TMJ had no clue what complications EDS causes and wanted to treat me just as she would any other patient.
At our first appointment with Dr. Mitakides he did a physical examination of my neck, jaw, head, and face to determine what affects my wacky jaw position was having on the other structures of my face, neck, and head. He determined that my jaw is in a much worse position on the left than the right, which we knew because during my last surgery in June of 2014 (can you believe I have gone over 1.5 years without surgery?!?!) my jaw was dislocated. He said this is not an uncommon complication for EDSers, particularly those of us who have neck fusions because they cannot hyperextend our necks so they pry our jaws open too far. The next time I have surgery he recommended to have the anesthesiologist use a pediatric tube for intubation because the chance of jaw dislocation are lower with a smaller tube.
After he did the physical examination I had x-rays taken of my jaw to see what my jaw looked like both open and closed. He expected to see abnormalities on the left, but the x-rays showed that the right side of my jaw did not line up correctly either. Your jaw is supposed to sit on a pad of cartilage when it is closed, but my jaw slides past that pad of cartilage on both sides, so it sits bone on bone. In addition to this, the jaw is supposed to rotate and then when it reaches its full degree of rotation it pivots forward. My jaw rotates normally, but then does not pivot forward the way it is supposed to. Because of this my jaw does not open as far as it should. I should be able to open it wider than most people because of the hyper mobility that EDS causes in all of my joints, but my jaw is on the very low end of "normal" for someone who is not hyper mobile. This is why it is difficult for me to eat an apple whole or open my mouth widely at the dentist.
After discussing it my husband and I decided that we would like to pursue treatment with Dr. Mitakides. The treatment that he uses is a splint that will help reposition the jaw. It is a piece of hard plastic that is molded to my top teeth. I have to wear this splint all of the time, except for when I am eating. This splint helps the jaw to go back into the correct position. I will go back between 3 and 6 times for him to adjust the splint as my jaw keeps shifting so that he can assure that my jaw ends up in the correct position. There is also the possibility that I might need to pursue orthodontics to get my bite aligned, so that my jaw will be able to get to the position we need. We will not know this for several months, however. After he determines that my jaw is in a good place and my symptoms have subsided I will go back to see him about every 6 months to ensure that everything looks good. I will also only have to wear my splint at night once my jaw is where he wants it to be.
The goal for me specifically is to get my jaw to shift to the right because it is deviated to the left and slide forward so that it sits on the pads of cartilage that it is meant to rest onto of. I can already tell that my bite has changed drastically and it has only been just over a week since I got my splint! My jaw is still in some pain, but my symptoms have improved dramatically! I will go back in two weeks to get the splint adjusted and he mentioned that he may do a botox injection in my chin because the muscles there are constantly spasming. With these muscles constantly tight they are part of the reason my jaw is being pulled out of proper alignment. I am so grateful that we found someone (finally!) who can help me! Jaw pain and teeth grinding was the first problem that I was diagnosed with when I was a young teenager, so proper treatment has been a long time coming!
We are still waiting to hear if my health insurance company will pay for the splint and office visits or not, so please keep your fingers crossed that they will!
At our first appointment with Dr. Mitakides he did a physical examination of my neck, jaw, head, and face to determine what affects my wacky jaw position was having on the other structures of my face, neck, and head. He determined that my jaw is in a much worse position on the left than the right, which we knew because during my last surgery in June of 2014 (can you believe I have gone over 1.5 years without surgery?!?!) my jaw was dislocated. He said this is not an uncommon complication for EDSers, particularly those of us who have neck fusions because they cannot hyperextend our necks so they pry our jaws open too far. The next time I have surgery he recommended to have the anesthesiologist use a pediatric tube for intubation because the chance of jaw dislocation are lower with a smaller tube.
After he did the physical examination I had x-rays taken of my jaw to see what my jaw looked like both open and closed. He expected to see abnormalities on the left, but the x-rays showed that the right side of my jaw did not line up correctly either. Your jaw is supposed to sit on a pad of cartilage when it is closed, but my jaw slides past that pad of cartilage on both sides, so it sits bone on bone. In addition to this, the jaw is supposed to rotate and then when it reaches its full degree of rotation it pivots forward. My jaw rotates normally, but then does not pivot forward the way it is supposed to. Because of this my jaw does not open as far as it should. I should be able to open it wider than most people because of the hyper mobility that EDS causes in all of my joints, but my jaw is on the very low end of "normal" for someone who is not hyper mobile. This is why it is difficult for me to eat an apple whole or open my mouth widely at the dentist.
After discussing it my husband and I decided that we would like to pursue treatment with Dr. Mitakides. The treatment that he uses is a splint that will help reposition the jaw. It is a piece of hard plastic that is molded to my top teeth. I have to wear this splint all of the time, except for when I am eating. This splint helps the jaw to go back into the correct position. I will go back between 3 and 6 times for him to adjust the splint as my jaw keeps shifting so that he can assure that my jaw ends up in the correct position. There is also the possibility that I might need to pursue orthodontics to get my bite aligned, so that my jaw will be able to get to the position we need. We will not know this for several months, however. After he determines that my jaw is in a good place and my symptoms have subsided I will go back to see him about every 6 months to ensure that everything looks good. I will also only have to wear my splint at night once my jaw is where he wants it to be.
The goal for me specifically is to get my jaw to shift to the right because it is deviated to the left and slide forward so that it sits on the pads of cartilage that it is meant to rest onto of. I can already tell that my bite has changed drastically and it has only been just over a week since I got my splint! My jaw is still in some pain, but my symptoms have improved dramatically! I will go back in two weeks to get the splint adjusted and he mentioned that he may do a botox injection in my chin because the muscles there are constantly spasming. With these muscles constantly tight they are part of the reason my jaw is being pulled out of proper alignment. I am so grateful that we found someone (finally!) who can help me! Jaw pain and teeth grinding was the first problem that I was diagnosed with when I was a young teenager, so proper treatment has been a long time coming!
We are still waiting to hear if my health insurance company will pay for the splint and office visits or not, so please keep your fingers crossed that they will!
Friday, January 15, 2016
Cleveland Clinic GI Visit
As I mentioned before the past few months have been very busy for me and my family! We have finally completed our move to Ohio and I began graduate school this week for speech-language pathology! My service dog Reilly and I are still adjusting to not working full time and we desperately miss all of our coworkers and students back in Illinois, but I am excited for the new prospects that graduate school brings!
An exciting update medically for me is that I was able to get an appointment on 12/30 at the Cleveland Clinic in their gastroparesis clinic. I saw one of their specialists who is considered one of the top doctors in the country for gastroparesis. He will help us figure out what the best next step is to manage my gastroparesis and allow me to get more adequate nutrition. I mentioned in my last GI update that I had a couple of medications to try, but unfortunately the one we decided to try, erythromycin, did not work. I tried it for two weeks and it did absolutely nothing :( My husband and I both agreed that we are not comfortable trying a medication that can cause permanent neurological side effects (Reglan), so unfortunately those are the three "typical" medications used for gastroparesis and we must now work outside the box.
Dr. Clein from the Cleveland Clinic was absolutely phenomenal! It was completely worth the four hour drive each way to see him! He is incredibly kind and knowledgeable about gastroparesis. He agreed that he does not like to use Reglan because of the side effects and that we needed to find something else to try. He wants to further rule out various issues that could be causing my gastroparisis. They drew 10 vials of blood during that appointment (once again I am incredibly thankful for my port because it means I can have blood draws done without having to get an IV and since it is placed into a major artery blood is always flowing well, so they can take as much blood as they need easily!). All of that blood work was done in order to rule out autoimmune diseases. I have been tested for autoimmune diseases many times previously, but it is always good to be rechecked! Most of those results have come back and they seem to indicate that I do not have an autoimmune disease causing my gastroparesis.
Dr. Clein wants me to get a Smart Pill test done. This is a non-invasive test during which a small mechanical device in the shape of a pill is swallowed and passes through the digestive tract. It collects information on the time it takes to go through your GI tract, the PH of your GI tract, and the pressures in GI tract. All of this information is transmitted to a small monitor worn on your hip or around your neck. This test will confirm that the paresis (slow movement) is isolated to my stomach and not generalized throughout my intestines. As of now I am scheduled to have this test done on April 11, but I am hoping to get a sooner date that is closer to my home.
If this test comes back stating that the issue is isolated to my stomach then we will pursue a gastric stimulator. This device stimulates the stomach to contract and thus empty itself at a faster pace. This device works well for many patients, but not all and there is not always a known reason why. We are hoping that I will be a good candidate and have good success with the stimulator, but we will have to wait and see! In the mean time we are trying a couple of different medications to try to help my GI symptoms, but so far we have not had much luck.
I am still working on getting everything set up down here in Ohio, so hopefully I will be able to restart physical therapy soon and get in with a primary care physician to establish care. I do have an appointment on February 1 with a TMJ (temporomandibular joint dysfunction) expert who also specializes in EDS. I hope that he will have the knowledge and ability to help my jaw pain!
An exciting update medically for me is that I was able to get an appointment on 12/30 at the Cleveland Clinic in their gastroparesis clinic. I saw one of their specialists who is considered one of the top doctors in the country for gastroparesis. He will help us figure out what the best next step is to manage my gastroparesis and allow me to get more adequate nutrition. I mentioned in my last GI update that I had a couple of medications to try, but unfortunately the one we decided to try, erythromycin, did not work. I tried it for two weeks and it did absolutely nothing :( My husband and I both agreed that we are not comfortable trying a medication that can cause permanent neurological side effects (Reglan), so unfortunately those are the three "typical" medications used for gastroparesis and we must now work outside the box.
Dr. Clein from the Cleveland Clinic was absolutely phenomenal! It was completely worth the four hour drive each way to see him! He is incredibly kind and knowledgeable about gastroparesis. He agreed that he does not like to use Reglan because of the side effects and that we needed to find something else to try. He wants to further rule out various issues that could be causing my gastroparisis. They drew 10 vials of blood during that appointment (once again I am incredibly thankful for my port because it means I can have blood draws done without having to get an IV and since it is placed into a major artery blood is always flowing well, so they can take as much blood as they need easily!). All of that blood work was done in order to rule out autoimmune diseases. I have been tested for autoimmune diseases many times previously, but it is always good to be rechecked! Most of those results have come back and they seem to indicate that I do not have an autoimmune disease causing my gastroparesis.
Dr. Clein wants me to get a Smart Pill test done. This is a non-invasive test during which a small mechanical device in the shape of a pill is swallowed and passes through the digestive tract. It collects information on the time it takes to go through your GI tract, the PH of your GI tract, and the pressures in GI tract. All of this information is transmitted to a small monitor worn on your hip or around your neck. This test will confirm that the paresis (slow movement) is isolated to my stomach and not generalized throughout my intestines. As of now I am scheduled to have this test done on April 11, but I am hoping to get a sooner date that is closer to my home.
If this test comes back stating that the issue is isolated to my stomach then we will pursue a gastric stimulator. This device stimulates the stomach to contract and thus empty itself at a faster pace. This device works well for many patients, but not all and there is not always a known reason why. We are hoping that I will be a good candidate and have good success with the stimulator, but we will have to wait and see! In the mean time we are trying a couple of different medications to try to help my GI symptoms, but so far we have not had much luck.
I am still working on getting everything set up down here in Ohio, so hopefully I will be able to restart physical therapy soon and get in with a primary care physician to establish care. I do have an appointment on February 1 with a TMJ (temporomandibular joint dysfunction) expert who also specializes in EDS. I hope that he will have the knowledge and ability to help my jaw pain!
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