Today I had my second infusion, I had the same nurse who successfully got the IV in last time, but she was unable to this time. Instead the only nurse who didn't stick my last time, got me this time. The first attempt the vein blew for no apparent reason, the nurse kept commenting on how "this isn't normal" and "[she] didn't do anything that should make it blow" also my veins apparently go deeper when you flick them rather than popping to the surface. They had to use the same needles they usually use on premies and are going to order me a box of them to take up to college with me since the medical center up there is an adult hospital. The infusion took 3 hours, but it seems to help so it is worth it.
Unfortunately I appear to be in somewhat of an autoimmune flare as my nausea is getting bad again and I am having some chest pains when I walk around accompanied by an even more rapid pulse than normal (146) and increased blood pressure (I am normally pretty low and am now high which is a huge jump). Hopefully my labs will come back okay and I will be able to titrate up on my methotrexate so we can get my symptoms under control.
Wednesday, December 29, 2010
Sunday, December 26, 2010
Long Update
So I know it has been practically forever since I last posted so I will do my best to preform as accurate an update as possible:
July: third hip surgery, this time a revision of my right hip "scar", this is usually an outpatient surgery but because of my EDS, AI, and autonomic issues they decided to keep me overnight and because of nausea after surgery it turned into a two night stay and probably should have been longer but they got tired of dealing with my meds and sent me home despite the fact that I couldn't keep anything down. On the plus side we added Reglan to my regimen of nausea meds and it has helped quite a lot and my incision is about 80% healed which is way more than it has ever been before.
August: Started school again (last semester)
September: Started to question whether or not I would be well enough to attend college
October: saw the famous Dr. Francomano, confirmed the EDS diagnosis (somewhere between types II and III she said to call it type 2.5) abnormal neuro exam coupled with symptoms suggested Chiari, Tethered Cord, or Cranio Cervical Instability (CCI): to begin wearing cervical collar for 15 min 4x a day and see a neurosurgeon. The cervical collar and cervical traction relieved some symptoms, but can't wear collar all the time
November: MRI comes up negative for everything, I was crushed because it means that my issues are dysautonomia which has no cure or really even treatment beyond what I am already doing (or so we thought). I was started on methotrexate (a mild chemotheraputic) at the end of the month for my Undifferentiated Connective Tissue Disease (autoimmune disorder) had to learn how to give myself injections for this one
December: Graduated from high school! Phone follow up with Dr. Francomano reveals that because of the fragility of EDS tissues, abnormalities do not always show up on MRI, she believes I have occult tethered cord and, because of the helpfulness of the cervial collar, CCI; it is even possible to have an occult Chiari. She also recommends getting one liter of IV saline once a week to help with autonomic symptoms. Rheumatologist wants me to go to the NIH (National institute of health) and Dr. Francomano wants me to see a neurosurgeon in Baltimore.
Fast forward to now:
On Thursday I got my first saline infusion, it took all four nurses on the floor and five sticks to finally get the IV in. They are not used to missing and were about to call in the flight for life team to try and get the IV in before one of the nurses finally got it in my wrist (ouch). The weird thing though was that I could hardly feel them digging around in the veins in my hands. It used to hurt terribly when they did that and I could barely tolerate it, but now it hardly bothered me. Mom was really concerned by this and we are left wondering if it is another neurological change or if my threshold for pain has just increased that much. My geneticist at Children's mis-wrote the order for the amount of time the saline was supposed to go in, but luckily we caught it in time and they believed us rather than the order, plus they had to use such a tiny gauge needle that they couldn't run the infusion very quickly (you aren't supposed to run an infusion in and EDS'er at more than 500mL an hour unless it is an emergency because it can cause the vein to blow or stretch it out significantly). The idea behind the infusion is that is increases blood volume, because EDS veins are so stretchy our bodies tend to believe that they don't have enough blood pressure or volume contributing to autonomic symptoms.
I don't know how long I will be able to do the infusions either because of my veins. I currently have one vein that can support a peripheral IV and if it is anything like the others, I have used up my one shot with it. The nurses asked about a PICC line, but those only last 6 weeks max and I will probably need the infusions for the rest of my life. Because of the way I heal and my allergy to silicon AND the methotrexate a central line is out of the question and a port could cause me to end up with yet another non healing wound. I know the EDNF recommends ports and central lines to people getting more than one IV and/or blood draw a month, but I heal so poorly that no surgeon is going to touch me.
We were the last ones out of the infusion center and the nurses were all wonderful, they wanted this to work for me as much as I do. I am feeling better and haven't nearly passed out since I got the infusion. My nausea is also much improved (not sure if that is from the methotrexate or the infusion) to the point where I am down to every 8 hours from every 6 on my zofran. I am scheduled to go back next Thursday, but after that I am up at Greeley and we have no idea what we will do then as I cannot drive and we have yet to find someone who can drive me to appointments and just generally check on me and be an emergency contact for when I fall and can't get up or dislocate something and need help pulling it back in place.
I was supposed to have an appointment with a new neurologist on Monday, but they called and said that they didn't want to see me and that I should go to University Hospital instead. They also faxed us back a copy of my summary sheet highlighting psycosomatic and opana (a narcotic) on it. They apparently didn't read on to see that my knee pain was not psycosomatic but a plica pulling my fat pad into the joint, squishing it to the point where most of my fat pad had to be removed. The nurse claimed that I was too complicated, but the fax we received contradicts that a bit. I hate that they sent that fax because now I am really anxious that I am about to go down that "it's all in your head" path again and that I will loose the allies I have gained and will be stopped from getting my infusions and the methotrexate.
This is horrible timing too because I am supposed to start college in two weeks and as things stand right now I am not sure I am going to make it. The university won't let any employees of the school help me get my joints back, the RA can't even come help me get off of the floor if I fall, all they can do is call 911 which is totally unnecessary for a simple dislocation or subluxation. I can't advertise that I am looking for someone to help me with laundry and call when I am in trouble either. I am going up with no support system in place or even a way to find one. I have no idea what to do! And if I start and have to drop out, that looks bad for when I want to start again because there is no medical withdraw.
July: third hip surgery, this time a revision of my right hip "scar", this is usually an outpatient surgery but because of my EDS, AI, and autonomic issues they decided to keep me overnight and because of nausea after surgery it turned into a two night stay and probably should have been longer but they got tired of dealing with my meds and sent me home despite the fact that I couldn't keep anything down. On the plus side we added Reglan to my regimen of nausea meds and it has helped quite a lot and my incision is about 80% healed which is way more than it has ever been before.
August: Started school again (last semester)
September: Started to question whether or not I would be well enough to attend college
October: saw the famous Dr. Francomano, confirmed the EDS diagnosis (somewhere between types II and III she said to call it type 2.5) abnormal neuro exam coupled with symptoms suggested Chiari, Tethered Cord, or Cranio Cervical Instability (CCI): to begin wearing cervical collar for 15 min 4x a day and see a neurosurgeon. The cervical collar and cervical traction relieved some symptoms, but can't wear collar all the time
November: MRI comes up negative for everything, I was crushed because it means that my issues are dysautonomia which has no cure or really even treatment beyond what I am already doing (or so we thought). I was started on methotrexate (a mild chemotheraputic) at the end of the month for my Undifferentiated Connective Tissue Disease (autoimmune disorder) had to learn how to give myself injections for this one
December: Graduated from high school! Phone follow up with Dr. Francomano reveals that because of the fragility of EDS tissues, abnormalities do not always show up on MRI, she believes I have occult tethered cord and, because of the helpfulness of the cervial collar, CCI; it is even possible to have an occult Chiari. She also recommends getting one liter of IV saline once a week to help with autonomic symptoms. Rheumatologist wants me to go to the NIH (National institute of health) and Dr. Francomano wants me to see a neurosurgeon in Baltimore.
Fast forward to now:
On Thursday I got my first saline infusion, it took all four nurses on the floor and five sticks to finally get the IV in. They are not used to missing and were about to call in the flight for life team to try and get the IV in before one of the nurses finally got it in my wrist (ouch). The weird thing though was that I could hardly feel them digging around in the veins in my hands. It used to hurt terribly when they did that and I could barely tolerate it, but now it hardly bothered me. Mom was really concerned by this and we are left wondering if it is another neurological change or if my threshold for pain has just increased that much. My geneticist at Children's mis-wrote the order for the amount of time the saline was supposed to go in, but luckily we caught it in time and they believed us rather than the order, plus they had to use such a tiny gauge needle that they couldn't run the infusion very quickly (you aren't supposed to run an infusion in and EDS'er at more than 500mL an hour unless it is an emergency because it can cause the vein to blow or stretch it out significantly). The idea behind the infusion is that is increases blood volume, because EDS veins are so stretchy our bodies tend to believe that they don't have enough blood pressure or volume contributing to autonomic symptoms.
I don't know how long I will be able to do the infusions either because of my veins. I currently have one vein that can support a peripheral IV and if it is anything like the others, I have used up my one shot with it. The nurses asked about a PICC line, but those only last 6 weeks max and I will probably need the infusions for the rest of my life. Because of the way I heal and my allergy to silicon AND the methotrexate a central line is out of the question and a port could cause me to end up with yet another non healing wound. I know the EDNF recommends ports and central lines to people getting more than one IV and/or blood draw a month, but I heal so poorly that no surgeon is going to touch me.
We were the last ones out of the infusion center and the nurses were all wonderful, they wanted this to work for me as much as I do. I am feeling better and haven't nearly passed out since I got the infusion. My nausea is also much improved (not sure if that is from the methotrexate or the infusion) to the point where I am down to every 8 hours from every 6 on my zofran. I am scheduled to go back next Thursday, but after that I am up at Greeley and we have no idea what we will do then as I cannot drive and we have yet to find someone who can drive me to appointments and just generally check on me and be an emergency contact for when I fall and can't get up or dislocate something and need help pulling it back in place.
I was supposed to have an appointment with a new neurologist on Monday, but they called and said that they didn't want to see me and that I should go to University Hospital instead. They also faxed us back a copy of my summary sheet highlighting psycosomatic and opana (a narcotic) on it. They apparently didn't read on to see that my knee pain was not psycosomatic but a plica pulling my fat pad into the joint, squishing it to the point where most of my fat pad had to be removed. The nurse claimed that I was too complicated, but the fax we received contradicts that a bit. I hate that they sent that fax because now I am really anxious that I am about to go down that "it's all in your head" path again and that I will loose the allies I have gained and will be stopped from getting my infusions and the methotrexate.
This is horrible timing too because I am supposed to start college in two weeks and as things stand right now I am not sure I am going to make it. The university won't let any employees of the school help me get my joints back, the RA can't even come help me get off of the floor if I fall, all they can do is call 911 which is totally unnecessary for a simple dislocation or subluxation. I can't advertise that I am looking for someone to help me with laundry and call when I am in trouble either. I am going up with no support system in place or even a way to find one. I have no idea what to do! And if I start and have to drop out, that looks bad for when I want to start again because there is no medical withdraw.
Tuesday, April 27, 2010
Infections galore
This has been the month of infections, I just finished my 3rd round this month and still have 9 days to go on my antibiotic ear drops. My second ear infection of the month is finally clearing up, and it was a disgusting oozing mess-though not as bad as the first one of the month. I managed to get an emergency ENT appointment yesterday and was told that I don't have any real hearing loss as of now except for some at a fairly high pitch. He said it may indicate future hearing loss and is something to watch. There were some issues with my left eardrum not moving and looking abnormal, but it is probably just due to some residual pus from the infections and should clear up soon.
Additionally it would seem that my hip incision also was infected. It was draining green smelly pus when I saw the derm last week-he didn't think it looked infected though. When I was started on the antibiotics for my ears it cleared up as well. The derm thinks that my hip probably isn't going to heal without some sort of intervention and is sending me to plastics to get a consult for a scar revision.
Additionally it would seem that my hip incision also was infected. It was draining green smelly pus when I saw the derm last week-he didn't think it looked infected though. When I was started on the antibiotics for my ears it cleared up as well. The derm thinks that my hip probably isn't going to heal without some sort of intervention and is sending me to plastics to get a consult for a scar revision.
Wednesday, February 17, 2010
Here it goes again
Oh joy, I have yet another UTI. This one we caught early though and I got on the right abx the first time. Luckily I also have a urology appointment tomorrow and I hope to talk to him either about referring me to someone who has done the botox procedure more than twice and has a better than 50% success rate that or we could get him to talk to Hopkins so when I go there I might see a urologist that has more ideas. His only other suggestion with any merit last appointment was a mitronoff procedure, where they move the appendix to make a tunnel between the bladder and the skin-usually through the belly button.
I started OT again 3 weeks ago with someone that Kevin recommended at the main campus. She is decidedly not my favorite person and I really miss Kevin, but she has seen a lot more EDS and is unafraid to make suggestions and chart things that she sees that are unusual. She says that she has never seen muscles or facia as tight as mine and in several places she can't distinguish between muscle and bone. She has never seen this much tightness with EDS but strongly believes that I have some sort of connective tissue disorder going on but has no idea what it might be. It is strange to have so much tightness in muscles and facia and be so loose in my tendons and other connective tissue. The muscles in my shoulders are so tight that they are subluxing my shoulders when I reach up and the muscles inbetween my ribs are so tight that she cannot distinguish individual ribs and my rib cage cannot expand when I breathe.
My pain doctor is pleased with how much I have been able to lower my pain meds-one good thing at least. We finally heard back from John Hopkins and the geneticist thinks that he needs to start over completely with me and that I really need a good genetic and internal medicine workup and that I will probably be able to get in with him in the next few weeks!! He is unsure if the EDS diagnosis is correct but feels that there are multiple things going on and that they really need to be addressed and soon! I hope we will hear back by the end of the week about when we will go, it is going to be hard because I will have to use my manual chair and forearm crutches to get around as wheelchair van rentals are super expensive and generally not available at the airport. I am so excited that we seem to be moving in the right direction and that there may finally be an answer at the end of this 18 year long tunnel!
I started OT again 3 weeks ago with someone that Kevin recommended at the main campus. She is decidedly not my favorite person and I really miss Kevin, but she has seen a lot more EDS and is unafraid to make suggestions and chart things that she sees that are unusual. She says that she has never seen muscles or facia as tight as mine and in several places she can't distinguish between muscle and bone. She has never seen this much tightness with EDS but strongly believes that I have some sort of connective tissue disorder going on but has no idea what it might be. It is strange to have so much tightness in muscles and facia and be so loose in my tendons and other connective tissue. The muscles in my shoulders are so tight that they are subluxing my shoulders when I reach up and the muscles inbetween my ribs are so tight that she cannot distinguish individual ribs and my rib cage cannot expand when I breathe.
My pain doctor is pleased with how much I have been able to lower my pain meds-one good thing at least. We finally heard back from John Hopkins and the geneticist thinks that he needs to start over completely with me and that I really need a good genetic and internal medicine workup and that I will probably be able to get in with him in the next few weeks!! He is unsure if the EDS diagnosis is correct but feels that there are multiple things going on and that they really need to be addressed and soon! I hope we will hear back by the end of the week about when we will go, it is going to be hard because I will have to use my manual chair and forearm crutches to get around as wheelchair van rentals are super expensive and generally not available at the airport. I am so excited that we seem to be moving in the right direction and that there may finally be an answer at the end of this 18 year long tunnel!
Thursday, January 21, 2010
And the Suck continues
We are still waiting to hear from Hopkins, they have gotten my records and are reviewing them now. The person Mom talked to said that the internists are booking 4 months out so I may not be going as soon as we hoped. Last week I had a physical with my PCP and she was at a loss about what to do. She asked if I thought I could keep going on like this and I told her yes it's not like I have a choice. I don't think I can keep going though. It has been almost a week since my stomach has been able to digest anything more solid than a banana. My nausea is better than earlier in the week with my doubled steroids. But they give me a horrible headache and stomach ache no matter how much I try to eat with it. My hip injection is wearing off and I have had a really bad week joint wise. I'm too tired, all I can do is drag myself out of bed for a few classes and crawl back in.
Monday, December 28, 2009
Eventful Day
Today I had my BXS (bone density scan) at invision and it was uneventful apart from running 45 minutes late and almost getting run over by a short lady on her cell phone determined to beat us out of the elevator. We went to CPK for lunch with plans to hit Barnes and Nobles and get Mom's blood drawn after lunch, this was foiled by a car that wouldn't start...at all...just made a clicking noise in the glovebox region. So an hour and a phone call to AAA later a tow truck showed up driven by a very happy dirty long haired man with slightly slurred speach and a rather stoned expression on his face. He also only had one seat belt in the cab besides his own (we were supposed to ride back to town with him) not to mention that to get in one practically had to climb a ladder with the rungs impossibly far appart. We decided to hoof it to the mall and hit the Borders there and wait for Dad to get off work and come get us. By the time we got to the door of the mall I was ready to fall over, and by Borders I was struggling to put one foot in front of the other. Dad got off 2 hours early so he could get us thankfully and we escaped in one peice (mostly) with new books!
Sunday, December 27, 2009
Looking back on 2009
January began the GI bleeding issues to which we still have no answers
February a new round of MRI's and other GI tests
March and ER trip and pneumonia
April is when Dr. Soden (GI) came into our lives and brought Dr. Ellias(GI), I had a skin biopsy and more abdominal CT's and ultrasounds
May I had my first colonoscopy and second skin biopsy (because the first wouldn't grow out which they had only seen one other time in 14 years), the surgeon freaked out and ended up not really doing it because she was afraid of tearing something
June I had my final period and started the process of getting my power chair, we also scheduled a date to get the screws removed from my hip
July the screws were removed from my hip and what was supposed to be a simple outpatient procedure turned into a week in patient for pain control; while I was inpatient I also had a capsule endoscopy which never left my stomach and finally started moving the day before it was scheduled to be surgically removed. During this month I began having worse nausea and began having to take nausea and pain meds around the clock and had a bad UTI.
August brought breast milk and more nausea and no period
September a colonoscopy and endoscopy with a capsule endoscopy placed, another UTI my power chair came in and made my life
October was filled with general feeling like crap, the questions of seizures, and a diagnosis of adrenal insufficiency
November was more ugh with a negative EEG and a switch to hydrocortisone from predinisone I was finally able to decrease my opana from every 4 hours to every 6
December was/is super crappy with doctors shrugging and not really caring or knowing what to do, I tried to decrease my zofran from every 6 hours to every 8 (still keeping phenergan every 6) and was retching constantly went back to every 6 (at the same time as the phenergan every 6) still wasn't enough, I had to shift it back to in betweeen predinisone doeses.
February a new round of MRI's and other GI tests
March and ER trip and pneumonia
April is when Dr. Soden (GI) came into our lives and brought Dr. Ellias(GI), I had a skin biopsy and more abdominal CT's and ultrasounds
May I had my first colonoscopy and second skin biopsy (because the first wouldn't grow out which they had only seen one other time in 14 years), the surgeon freaked out and ended up not really doing it because she was afraid of tearing something
June I had my final period and started the process of getting my power chair, we also scheduled a date to get the screws removed from my hip
July the screws were removed from my hip and what was supposed to be a simple outpatient procedure turned into a week in patient for pain control; while I was inpatient I also had a capsule endoscopy which never left my stomach and finally started moving the day before it was scheduled to be surgically removed. During this month I began having worse nausea and began having to take nausea and pain meds around the clock and had a bad UTI.
August brought breast milk and more nausea and no period
September a colonoscopy and endoscopy with a capsule endoscopy placed, another UTI my power chair came in and made my life
October was filled with general feeling like crap, the questions of seizures, and a diagnosis of adrenal insufficiency
November was more ugh with a negative EEG and a switch to hydrocortisone from predinisone I was finally able to decrease my opana from every 4 hours to every 6
December was/is super crappy with doctors shrugging and not really caring or knowing what to do, I tried to decrease my zofran from every 6 hours to every 8 (still keeping phenergan every 6) and was retching constantly went back to every 6 (at the same time as the phenergan every 6) still wasn't enough, I had to shift it back to in betweeen predinisone doeses.
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