I had an appointment last week with my primary care physician (PCP). I had a wonderful PCP when we lived in the most recent town we were in in Illinois, so I was extremely sad to leave him! Thankfully my geneticist used to live in Cincinnati (where we recently moved to) and still has connections here! He was able to give me a recommendation for a PCP who is knowledgeable about Ehler's-Danlos Syndrome (my connective tissue disorder). This doctor was not taking new patients, but since my geneticist referred me to him he was willing to take me on!
I had a few questions for him, so the appointment was timed perfectly. Firstly, I have been taking a muscle relaxer for about 8 months now for the extreme tightness in my neck down into my shoulders. The muscles that run down from my neck into the top of my shoulders get as hard as rocks (literally, not an exaggeration). This can be caused by many of my conditions (chiari, EDS, and instability) and is extremely uncomfortable for me. When I saw my genetesict last fall he recommended that I start a muscle relaxer called Flexiril to attempt to alleviate some of this tension. This helped a little bit, but every time I saw a new doctor or therapist they still were amazed at the amount of muscle tension that I held in my neck and shoulders. I asked my PCP if I could increase the dosage of the Flexeril, but he said he was not comfortable increasing my dosage. Flexeril can cause electrical changes in your heart and that risk increases as the dosage increases. Instead he recommended that I change to a different muscle relaxer called Baclofen. He started me out on 10 mg once a day and I will increase that dosage to twice a day and then finally three times a day over the course of three weeks. I am currently on 10 mg twice a day and it has helped so much!!! My muscles are not constantly tight and do not feel like rocks! It is so nice to have some of that tension reduced! One note of caution is that my doctor did say that if I had not had my cranial cervical fusion (screws from my skull down to my second cervical vertebrae) that he would absolutely not recommend a muscle relaxer since I am an EDSer. This is because the tightened muscles are actually helping to hold your cranial cervical joint stable and when you relax those muscles the instability that many of us with EDS have gets worse.
I also asked him to check my thyroid and my electrolytes. My thyroid is considered to be borderline hypothyroid, so I like to monitor my levels and make sure they do not tip into the range where I would need to be medicated. My levels were still borderline, so for right now we are good to go! I get my electrolytes checked because a medication I take (florinef) depletes my potassium and thus I need to take prescription potassium. Low or high potassium can cause serious issues with your heart, so it is essential to monitor my levels to make sure we are not over or under supplementing my potassium. Thankfully my electrolyte levels all look good! I was able to take my orders for the blood work down to the cancer center and have the labs drawn through my port. It is incredibly convenient that the cancer center is located in the same office as my PCP because then I do not have to drive to a separate office just to get my labs down.
As I mentioned in my last post that I also was able to get a recommendation for for a doctor who will do the botox injection into a muscle in my stomach (see this blog post for more info on that), so I was incredibly thankful for that! I will see that doctor next week, but I am excited to try the botox inject and see if I get a symptom relief from my gastroparesis. I also got a prescription from the PCP for a brace that my physical therapist recommended that will hopefully help support my spine and improve my neck pain and discomfort. I will write a blog post more on that when I receive the brace and have a chance to try it out!
I believe that those are all of the major this that we discussed at my primary care appointment. I will go back and see my PCP in 3 months. In the mean time I have a few appointments coming up in the next week or so, so I will update on those as they happen. I also had an appointment for my hip so I will write an update on that ASAP. Thanks as always for following along on my journey!
Thursday, April 21, 2016
Thursday, April 14, 2016
Cleveland Clinic Gastroparesis Appointment
Last week we went back to the Cleveland Clinic to meet with their gastroparesis specialists for the second time. We had been waiting on insurance to cover a test that they had ordered (a smart pill which measures time, pH, and pressure of your entire GI tract), but insurance would not cover this test and deemed it experimental. Since they wouldn't cover this test we had to go back up to Cleveland to come up with a new care plan without those results.
It is a four hour drive each way to Cleveland, which makes it quite a long day!!! My service dog Reilly DESPISES the car, so he was left at home with my sister-in-law. We live next door to my brother-in-law and sister-in-law right now, so to Reilly they are family and he is very comfortable with them. It is still hard on the both of us to be separated since we are together at ALL other times. I feel like I am without my shadow when I don't have Reilly because I do not go anywhere without him (even the bathroom normally!!!).
We were able to meet with both the gastroenterologist and a general surgeon who works with the GI doctor. The gastroenterologist raised the dosage of a medication (Remeron) that is supposed to help my stomach empty. It is not a "traditional" medication used to treat gastroparesis, but he uses it with many of his patients who have gastroparesis in conjunction with autonomic nervous system dysfunction. The idea is that this medication will help the nervous system to effectively tell the stomach to empty. We doubled the dose and so far I have not noticed much of a difference in my symptoms since increasing the dose, but it has only been a few days so I will give it some more time!
After having a conversation with the GI doctor we decided that the gastric neurostimulator is not the route for me right now. This device is what we had discussed as the next step previously, but we decided at this appointment that this is actually not a good next step right now. One main issue with the gastric neurostimulator is that it is not MRI comparable. This means that I would never be able to have an MRI. Having EDS and needing MRI on joints and my brain and spine on a fairly regular basis this is not ideal. The alternative to an MRI is a CAT scan. Now CAT scans themselves are not a bad thing, but they do expose patients to radiation. Having a few CAT scans is no big deal, but if I had to have multiple CAT scans a year the radiation would definitely add up over the years and this is a huge concern.
Another issue we have with the gastric neurostimulator is that the success rate is only 50-60%. That is not very good odds in my book! The surgeon also said that since I have chronic constipation he thinks my intestines may be slowed down as well as my stomach so the success rate drops even further. It is a major abdominal operation to place the device and then an even more complicated operation to remove the device if it does not work. The surgery to take it out includes the surgeon removing a piece of your stomach because the nodes on the stimulator become embedded in your stomach. Obviously this is not an ideal situation!
Now all this aside, this does not mean that someday this device will be a good next step for me. Right now I am actually gaining weight and eating much more than I have in the past. I still have symptoms and discomfort, but in general my GI status has greatly improved. For all of those reasons the benefits do not outweigh the risks at this time.
Since we decided we are not pursuing the gastric neurostimulator at the moment we discussed alternative options with the surgeon. After discussing a few different things we decided that the next thing we will try is injecting botox into the pyloric sphincter. The pyloric sphincter is the valve that allows food to pass from your stomach into your intestines. By injecting botox into the muscle that forms this sphincter the muscle relaxes. Once the muscle relaxes the hope is that food will pass easier from the stomach into the intestines since it does not have to work to get past the sphincter. If the botox does work I may notice results immediately or it might take a few days before I notice any results. Even if the botox does work, however, the results are only temporary. The general surgeon says that most of his patients only see results for about 6-8 weeks at the maximum. For this reason the botox injection is not considered a long term solution, rather it is used as a diagnostic tool. If I respond well to the botox injection then that tells the surgeon that I would be a good candidate for a surgery called a pyloroplasty (more on that in a moment). The botox injection is done via a scope, so there are no incision in the abdomen. There are very few risks of the botox injection itself, besides the risks of the anesthesia of course! For this reason we decided that there is no harm in trying the injection and seeing if it helps.
Since Cleveland Clinic is so far away for us to drive to the doctors and I decided that I would try to find a doctor to do the botox injection in Cincinnati. I had an appointment with my primary care doctor at the beginning of this week, so I asked him if he knew of anyone. He sent me the name of a doctor that will do it, so I set up an appointment with this GI doctor for Monday the 25. We will schedule the procedure to do the botox at this appointment.
If the botox injection provides relief from symptoms then we will most likely preceded with a surgery called a pyloroplasty. A pyloroplasty essentially does the same thing as the botox injection, but it is permanent. During the pyloroplasty the pyloric sphincter is cut so that the valve is widened. This provides a permanently opened pyloric sphincter. The Cleveland Clinic is one of the only places in the world that can do this surgery endoscopically. This means the surgery can be done through a scope down the throat rather than through incision made on the abdomen. The recovery is faster, the risks are lower, and there is no scarring if the surgery is done this way. For this reason we will travel up to Cleveland if we decide that this surgery is the best next step for me. The risks are very low and there are no long term complications with this surgery. We will not know whether or not I am a good candidate for this surgery until after we do the botox injection.
That was a very long update I know, but it was an extremely informative appointment! I will update as to when the date for the botox injection will be after the appointment I have schedule on the 25th.
It is a four hour drive each way to Cleveland, which makes it quite a long day!!! My service dog Reilly DESPISES the car, so he was left at home with my sister-in-law. We live next door to my brother-in-law and sister-in-law right now, so to Reilly they are family and he is very comfortable with them. It is still hard on the both of us to be separated since we are together at ALL other times. I feel like I am without my shadow when I don't have Reilly because I do not go anywhere without him (even the bathroom normally!!!).
We were able to meet with both the gastroenterologist and a general surgeon who works with the GI doctor. The gastroenterologist raised the dosage of a medication (Remeron) that is supposed to help my stomach empty. It is not a "traditional" medication used to treat gastroparesis, but he uses it with many of his patients who have gastroparesis in conjunction with autonomic nervous system dysfunction. The idea is that this medication will help the nervous system to effectively tell the stomach to empty. We doubled the dose and so far I have not noticed much of a difference in my symptoms since increasing the dose, but it has only been a few days so I will give it some more time!
After having a conversation with the GI doctor we decided that the gastric neurostimulator is not the route for me right now. This device is what we had discussed as the next step previously, but we decided at this appointment that this is actually not a good next step right now. One main issue with the gastric neurostimulator is that it is not MRI comparable. This means that I would never be able to have an MRI. Having EDS and needing MRI on joints and my brain and spine on a fairly regular basis this is not ideal. The alternative to an MRI is a CAT scan. Now CAT scans themselves are not a bad thing, but they do expose patients to radiation. Having a few CAT scans is no big deal, but if I had to have multiple CAT scans a year the radiation would definitely add up over the years and this is a huge concern.
Another issue we have with the gastric neurostimulator is that the success rate is only 50-60%. That is not very good odds in my book! The surgeon also said that since I have chronic constipation he thinks my intestines may be slowed down as well as my stomach so the success rate drops even further. It is a major abdominal operation to place the device and then an even more complicated operation to remove the device if it does not work. The surgery to take it out includes the surgeon removing a piece of your stomach because the nodes on the stimulator become embedded in your stomach. Obviously this is not an ideal situation!
Now all this aside, this does not mean that someday this device will be a good next step for me. Right now I am actually gaining weight and eating much more than I have in the past. I still have symptoms and discomfort, but in general my GI status has greatly improved. For all of those reasons the benefits do not outweigh the risks at this time.
Since we decided we are not pursuing the gastric neurostimulator at the moment we discussed alternative options with the surgeon. After discussing a few different things we decided that the next thing we will try is injecting botox into the pyloric sphincter. The pyloric sphincter is the valve that allows food to pass from your stomach into your intestines. By injecting botox into the muscle that forms this sphincter the muscle relaxes. Once the muscle relaxes the hope is that food will pass easier from the stomach into the intestines since it does not have to work to get past the sphincter. If the botox does work I may notice results immediately or it might take a few days before I notice any results. Even if the botox does work, however, the results are only temporary. The general surgeon says that most of his patients only see results for about 6-8 weeks at the maximum. For this reason the botox injection is not considered a long term solution, rather it is used as a diagnostic tool. If I respond well to the botox injection then that tells the surgeon that I would be a good candidate for a surgery called a pyloroplasty (more on that in a moment). The botox injection is done via a scope, so there are no incision in the abdomen. There are very few risks of the botox injection itself, besides the risks of the anesthesia of course! For this reason we decided that there is no harm in trying the injection and seeing if it helps.
Since Cleveland Clinic is so far away for us to drive to the doctors and I decided that I would try to find a doctor to do the botox injection in Cincinnati. I had an appointment with my primary care doctor at the beginning of this week, so I asked him if he knew of anyone. He sent me the name of a doctor that will do it, so I set up an appointment with this GI doctor for Monday the 25. We will schedule the procedure to do the botox at this appointment.
If the botox injection provides relief from symptoms then we will most likely preceded with a surgery called a pyloroplasty. A pyloroplasty essentially does the same thing as the botox injection, but it is permanent. During the pyloroplasty the pyloric sphincter is cut so that the valve is widened. This provides a permanently opened pyloric sphincter. The Cleveland Clinic is one of the only places in the world that can do this surgery endoscopically. This means the surgery can be done through a scope down the throat rather than through incision made on the abdomen. The recovery is faster, the risks are lower, and there is no scarring if the surgery is done this way. For this reason we will travel up to Cleveland if we decide that this surgery is the best next step for me. The risks are very low and there are no long term complications with this surgery. We will not know whether or not I am a good candidate for this surgery until after we do the botox injection.
That was a very long update I know, but it was an extremely informative appointment! I will update as to when the date for the botox injection will be after the appointment I have schedule on the 25th.
Thursday, April 7, 2016
Hip Appointment and Arthrogram
Since working with a new physical therapist in Cincinnati her and I both realized that there is something wrong with my left hip. We are not sure when or how I injured it, but with EDS that is quite common. My joints all pop out of place regularly and sometimes they do not go back into place correctly and can catch and tear. Activity really seems to aggravate the hip (walking, stairs, etc.) and the pain is deep in the hip joint. We started trying to treat the pain conservatively and tried doing different exercises to see if it would improve, but it has not improved. After a few weeks of this treatment the PT recommended that I see an orthopedic surgeon in order to get my hip assessed.
I was able to get an appointment with an orthopedic surgeon at Cincinnati Children's hospital (they accept patients into their thirties, which is fantastic for me!). At that appointment he took some x-rays and did a full assessment of both hips. The x-rays looked great, which we expected them too, but based off of the amount of time I have had pain in the hip, the fact that the pain has not gotten any better, and the specific positions I have pain in the hip he recommended that we do further testing to see what is going on.
The test that he wanted done is called an arthrogram. An arthrogram is done when contrast is injected directly into the joint and then an MRI is done. I have never had one of these, so it was new experience! I was taken into an operating room (a sterile environment), my hip was numbed, and a needle was injected directly into the hip using x-ray guidance. This allowed them to put IV contrast directly into my joint so the soft tissue structures of the hip would show up much clearer on the MRI than they would if IV contrast was used.. While he had the needle in my hip they also injected steroids into the hip. The steroids can be used as a treatment for joint pain because they reduce the inflammation in the joint and can decrease pain. Sadly the steroid injection did not seem to help much at all.
The arthrogram itself was not painful necessarily because of all the local anesthesia that they used, but it was definitely uncomfortable! I could feel pressure and tugging as the needle was in the joint and when the procedure was completed my hip was VERY stiff. The doctor inserted so much fluid into the hip that it was extremely stiff and sore. I was on low activity for 48 hours, so we just used my wheelchair since we had family in town and were going out to a museum. Part of my connective tissue disorder means that I bruise severely and easily, so the point that the needle was inserted into the hip is definitely bruised and still sore a week later.
I will go back to the orthopedic surgeon next Wednesday (4/13) to get the results from the arthrogram. If it is a tear in the cartilage like we think it is we will then schedule surgery to repair the tear, but we will not know that until after I get the results from the arthrogram.
I was able to get an appointment with an orthopedic surgeon at Cincinnati Children's hospital (they accept patients into their thirties, which is fantastic for me!). At that appointment he took some x-rays and did a full assessment of both hips. The x-rays looked great, which we expected them too, but based off of the amount of time I have had pain in the hip, the fact that the pain has not gotten any better, and the specific positions I have pain in the hip he recommended that we do further testing to see what is going on.
The test that he wanted done is called an arthrogram. An arthrogram is done when contrast is injected directly into the joint and then an MRI is done. I have never had one of these, so it was new experience! I was taken into an operating room (a sterile environment), my hip was numbed, and a needle was injected directly into the hip using x-ray guidance. This allowed them to put IV contrast directly into my joint so the soft tissue structures of the hip would show up much clearer on the MRI than they would if IV contrast was used.. While he had the needle in my hip they also injected steroids into the hip. The steroids can be used as a treatment for joint pain because they reduce the inflammation in the joint and can decrease pain. Sadly the steroid injection did not seem to help much at all.
The arthrogram itself was not painful necessarily because of all the local anesthesia that they used, but it was definitely uncomfortable! I could feel pressure and tugging as the needle was in the joint and when the procedure was completed my hip was VERY stiff. The doctor inserted so much fluid into the hip that it was extremely stiff and sore. I was on low activity for 48 hours, so we just used my wheelchair since we had family in town and were going out to a museum. Part of my connective tissue disorder means that I bruise severely and easily, so the point that the needle was inserted into the hip is definitely bruised and still sore a week later.
I will go back to the orthopedic surgeon next Wednesday (4/13) to get the results from the arthrogram. If it is a tear in the cartilage like we think it is we will then schedule surgery to repair the tear, but we will not know that until after I get the results from the arthrogram.
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!
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