2019

Update: 06/21/2019

Yesterday we met with my urologist again.

We met with him on February 13th at 8:00AM. During that appointment they did an ultrasound on my bladder that should have showed an empty bladder because I experience the least trouble emptying my bladder first thing in the morning when everything is relaxed. but it showed that my bladder was holding on to urine… this is important for later.

At that appointment he asked me to keep a journal of my intake and output and because I needed to do that for the Food Sensitivity and Allergy Clinic at RUSH, which was very convenient… killing two birds with one stone.

He was hoping that maybe he would be able to see a pattern. Maybe they would be able to see that when I eat X medication or X food, I pee less often. Or maybe my liquid intake isn’t really what we thought it was…

Unfortunately, the journal showed that I drink between 80 and 12o ounces of water a day and urinate 1-3 times a day. BUT when urinate, I urinate between 800mL and 1200mL… which means that my bladder is holding more than 800-1200 because my bladder is still holding on to urine on top of whatever urine it is releasing. The “normal” human’s bladder holds holds between 300mL-400mL.

So that’s awesome…

He gave me three options:

  1. Pelvic Floor Therapy
  2. Medication
  3. Urodynamics testing

He said that due to the fact that due to the complexity of my situation and the fact that I only have one or two bowel movements a week, he would really prefer not to put me on any of the medications they would typically use for this situation, but if I was adamant about wanting to go that route, he would assist me.

I told him that I would really prefer not to add another pill to my regimen so we are on the same page.

I asked if we could pursue pelvic floor therapy before and see how that goes and if it doesn’t help then we will proceed from there.

He agreed that we could try pelvic floor therapy for the next six months, but if pelvic floor therapy doesn’t help, we will need to proceed with urodynamics testing.

I was thankful to put off the urodynamic’s testing because I was fully expecting to walk out of his office with an order for urodynamic’s testing because two days before this (on the 18th), I saw my physical therapist (who is also a pelvic floor therapist) and we were talking about my upcoming urologist appointment and she told me to expect them to order urodynamics testing and to expect them to order self catheterization to help me urinate more often than I do so that my bladder isn’t holding as much as it is… needless to say, I am really excited to at least not have to do that for now. Hopefully pelvic floor therapy helps.

~Trinket

Update: 05/26/2019

The last couple of days have been a complete shit show…

We drove up to Chicago for a consultation with a physical therapist who specializes in EDS as well as an appointment at the Food Sensitivity and Allergy Clinic at RUSH.

I worked on Thursday (May 23rd) until noon, when my dad picked me up and we jumped on the highway.

We live a 2 and a half hour or three hour drive from Chicago normally… But the Chicago traffic can sometimes make that drive much longer.

… And to make matters worse, about an hour into the drive, my stomach became really upset and we had to pull off the road so I could spend 15 or so minutes in the bathroom and once we got back on the road, we hit traffic that had us stop nearly dead in our tracks on multiple occasions…

Despite all of that, we still made it to the address we had been given 20 minutes early. Except that the address we were given was the wrong address.

We were given:

1775 Dempster St. Evanston, IL

We should have been given:

1775 Dempster St. Park Ridge, IL

When we arrived @ 1775 Dempster St. in Evanston, we knew something wasn’t right. We were in the middle of a shopping center…

I called the physical therapy center repeatedly, but kept getting their voicemail that told me that someone would get back to me within 24 hours…

On my last attempt to get ahold of someone, anyone… I listened to all of their menu options and heard that there was an option to hear their hours of operation, their address, and contact information. It was at that point that we realized that we were still 20 minutes from where we should needed to be and our appointment was in 5 minutes.

Once we arrived at the hospital, dad dropped me off at the front door and I ran inside to the floor and room I had been given while he parked the car. On my way up to the office, I noticed that I was in a children’s hospital, but I didn’t think much of it because my geneticist works at a children’s hospital, so it didn’t really seem odd to me that he may refer me to another doctor or medical professional in another pediatrics hospital… But when I made it up to the doctors office, they told me that I had also be given the wrong floor and office number. My appointment was actually in the basement on the complete opposite side of the hospital… on the adult side of the hospital.

At this point I felt completely defeated, but the ladies at the desk had me take a seat while they called the adult physical therapy office and waited to hear back to see if they could squeeze me in.

Thankfully they were able to squeeze me in. We took the elevator down to the main floor and walked across the hospital… at which point we got checked in and registered. We then waited and after having my appointment pushed back twice, they were able to squeeze me.

During my appointment they tested hyper mobility in a majority of my joints. The therapist was surprised by my hyper mobility and how far I could hyperextend my spine. She said she didn’t think she had ever seen anyone bend like that before…

I also left her with a binder full of all of my local physical therapists progress notes and such. She was really excited to have all of that. She said it would be really helpful for her to have for reference since we would only be seeing each other every 6 months.

Over all, the appointment went really well.

After finally seeing the physical therapist, we checked in to our hotel. I was disappointed to find that despite paying more for a hotel with a pool, their pool was under construction. I have not been able to swim in 3 years because I have been on a fentanyl patch that can’t get wet. I am able to shower if I keep the area directly out of the stream of the water, but I cannot take a bath or go swimming… but I recently found Tegaderm on Amazon. We put a piece of gauze over the patch so when we remove the Tegaderm, it doesn’t remove the fentanyl patch with it, and then we put the Tegaderm over the gauze to help create a waterproof barrier.

The next morning we got up and ready and headed to my 9:30AM appointment at the Food Sensitivity and Allergy Clinic. We arrived 15 minutes early and started the check in process.

During the check in process they had to call registration and… register me. The lady in registration asked me if I had experienced previous issues with my insurance having my birthday incorrect. This confused me because it was the first time that I had ever heard of there being any issue with my birthday and I have been sick since August of 2015… The lady in registration said that she would call my insurance company and get things sorted out and she would call us back.

After over an hour of waiting, we still had not heard back from registration… so the woman at the front desk called registration to check back… getting a different woman this time, she was able to register me immediately with no hiccups or mention of there being any issues with insurance and my birthdate.

At which point I am informed that my appointment was never scheduled for 9:30 like the e-mail welcome packet stated… It’s actually at 11:00. So we could have slept an additional hour and a half…

But the chaos was worth it because the team of doctors I saw was nothing short of incredible.

During the Food Sensitivity and Allergy Clinic you see three doctors: An Allergist named Dr. Mary Ann Tobin, a Gastroenterologist named De. Demeo, and a Nutritionist.

You may have heard me mention Dr. Mary Tobin before because she is the same allergist and immunologist I worked with last fall who ordered testing and then diagnosed me with MCAS (Mast Cell Activation Syndrome).

Dr. Tobin and Dr. DeMeo have noticed significant patterns and correlations between MCAS and GI issues so they created this clinic that they hold once or twice a month on Fridays in an attempt to evaluate patients who are diagnosed with EDS and MCAS and are struggling with GI symptoms.

To help assist with that, they have you keep a dietary journal. But this dietary journal isn’t just about tracking the food that you eat and when you eat it… You also track what you drink and when, what medications you take and when, how much you pee and when, how often you poop, etc. as well as tracking any symptoms you experience. So that they can try to see if there appears to be a pattern… if any specific food, drink, or medication appears to be causing any of your pain.

Ultimately after a thorough exam, reviewing my medical history, and a hilarious (because of his sense of humor) but lengthy conversation… We have finally come to the conclusion that I am not only struggling with motility issues, but with pelvic floor disfunction. He is concerned that I am struggling with pelvic floor dysfunction because I only urinate 2-3 times a day and only poop 1-2 times a week. And even when I do use the bathroom (urinate or poop) I am not clearing everything. All things that would be impacted by the pelvic floor muscles.

He feels that the puborectalis muscle is not relaxing all the way… which is making it impossible for the solid matter to pass even if I use a “squatty potty”. So when I do go, I am only able to pass loose matter or diarrhea and gas. So I am more or less constantly impacted.

This was confirmed by an x-ray performed that afternoon. Having had to pull off the interstate for diarrhea the day before… I shouldn’t have shown impaction… But here we are.

He asked for time to review the portions of my medical files he couldn’t review while I was there for my visit and will get back to me as to whether or not he wants to order an anal rectorial manometry.

After reviewing my medical records, he decided that we needed to schedule a Hydrogen Breath Test and an Anal Rectorial Manometry. So now we are trying to schedule these test so that we can do them in one trip.

I also saw Dr. Tobin who ran some more allergy testing. That allergy testing has determined that I am not only allergic to eggs, but also tomatoes, bananas, crab, and lobster.

Insurance has approved the Xolair. We are waiting for a call from the special pharmacy to get all of that seat up. I should be starting that soon.

I think that’s all for now.

~Trinket

Update: 05/07/2019

This week has been rough to say the least… 

Tuesday morning I woke up feeling my “normal” self… (what I would consider normal pain levels, what I would consider normal nausea levels, etc… no alarm bells were sounding.) I took my morning steroids and sleepily snuggled my kitty while they kicked in. I dozed off while he purred on my chest. 

A half hour later when my second alarm went off signaling me that it was time for me to get up and get ready for work, i got up, got dressed, and went to the bathroom. After, I came back to my room to brush my hair. When I lifted my arm to brush my hair, unimaginable pain shot threw my chest, arm, and neck… I lowered my arm hoping the pain would dissipate but it didn’t. I rolled my shoulder in a circle and flexed and stretched my arm hoping maybe i had some how subluxed my shoulder or something when I went to brush my hair, but again, no luck relieving the pain… It hurt to breath, it hurt to bear any weight on that side, it hurt to move. 

Around lunch time the pain had moved up into my jaw. 

Around 3:30 the pain moved over my shoulder and down my back and was progressing down my arm and up the back of my neck into my head. 

I already had a physical therapy appointment scheduled for 4:30 that afternoon so I suffered through work taking the day one hour at a time… sometimes even just one minute at a time. I kept telling myself that I just needed to make it to my PT appointment and she would be able to work her magic and find whatever was wrong and fix it… finally providing relief. 

…unfortunately that wasn’t quite the case. After spending an hour massaging various muscles and trying to see if something was out of place, we didn’t have any luck. This left her worried that I was having a heart attack.

I’ll be honest, at first I didn’t think she was serious because I had been dealing with this pain for 9 hours at this point. My brain was logically telling me that if I was having a heart attack, I would have died by that point. But turns out that she was very serious.

When my dad arrived to pick me up, I let him know about her concerns and ultimately we decided we would rather be safe than sorry, so we headed to the ER instead of heading home to the comfort to my own bed… Where I wanted to desperately to be.

When we got to the ER, they confirmed that the symptoms that I was experiencing were the same symptoms that a woman having a heart attack would experience. They were also able to quickly rule out a heart attack by performing an EKG in the triage room before taking me back. They then continued to run tests to rule out any other cardiac events. And because EDS puts me at risk for an aneurism, they also ran tests to rule out an aneurism.

After blood work, an x-ray, a cat scan of my head, and a CT with contrast of my chest, we ruled out all cardiac events and aneurisms.

But 7 hours in the ER was not all for nothing!

The CT with contrast of my chest showed that my 1st right rib is not shaped properly. The doctor said that it is likely that the “deformity” is likely something that I was born with.

And it likely didn’t cause any issues until now because EDS has effects more of my my body the older I get… I have just been very fortunate to never sublux or dislocate that rib before.

After we figured out what was causing the issue (almost 7 hours after we arrived), they discharged me to go home… Requesting that I schedule a follow up with my primary care physician to discuss treatment options.

It’s been two days since I was discharged and there has been little to no reprieve from the pain. I am still having difficulty taking deep, full breath, bearing weight on that side, etc. Hopefully taking it easy and some good rest over the weekend will help. 

~Trinket

Update: 03/30/2019

It has been a long while since I have updated because while there have been two trips to the ER and several doctors appointments, there wasn’t any progress at any of those doctors appointments.

So far this year has not been off to a great start and I have been trying really hard to not focus on the negativity and stay positive… and rehashing the negative things that have happened makes it really hard to do that…

I started the year off with a trip to the ER on January 2nd during our vacation in Florida. I have never seen a waiting room more crowded than this one was and it was late morning on a Wednesday when we arrived. We spent 3 and a half hours in the waiting room before we were called back.

Once we were called back they gave me 100 micrograms of fentanyl and nausea meds via IV for the kidney pain I was experiencing. They also gave me a liter of fluids.

Once the blood and urine results came back showing that I did not have a UTI like we had originally suspected and my kidney markers were within range, they did not want to do any further testing or imaging but came in every hour on the hour to ask if I wanted more fentanyl.

We were frustrated because we had a 18 hours (and then some depending on traffic) car ride home in three days and fentanyl on Wednesday isn’t going to help with the pain on Saturday… We asked if they could at least do an ultrasound of my kidneys to try to find the source of the pain and they explained that because my doctors up north are more familiar with my case and they don’t know what imagery has already been done, they didn’t feel comfortable issuing more testing but they were willing to help me get my pain under control so that I could manage it until we managed to get home.

I left in slightly less pain thanks to the fentanyl they were willing to hand out like candy but with as few answers as we came in with and having wasted 8 hours that we could have spent on the beach. And within a few hours, when the fentanyl was out of my system, I was back to where I started which put a damper on the rest of our vacation.

Our car ride home was the most difficult trip yet. Approximately 3 hours from home the pain became unbearable and we had to pull off the road immediately so that I could get to the bathroom. I barely made it to the bathroom before it started coming out of both ends. I was trapped in this gas station bathroom with a line forming outside for more than 45 before I was finally able to pull myself together enough to get back in the car.

Six weeks later I saw PA Doug Doll who said that it is likely that thanks to hEDS allowing my bladder to hold more urine than it should, urine is backing up into my kidneys and causing the kidney pain but without imaging when the pain is occurring… there is no way to say for sure.

On January 25th I saw my Electrophysiologist, Dr. Arnoldo. We have been working to try to get my Inappropriate Sinus Tachycardia under control. We have tried several medications and for one reason or another, they’ve not worked.

My geneticist, Dr. Tinkle, originally put me on 20mgs of Propranolol at night to help with the tachycardia that was keeping me up at night. Dr. Arnoldo decided against adding Propranolol during the day because it had a sedative affect and I already struggle with fatigue and a sedative will make that worse. Instead we added 20mgs of Atenolol during the day. At 25mgs Atenolol had no effect. They increased the dose to 50mgs caused my blood pressure to tank and increased my dizziness and syncope.

After Atenolol we tried 20mgs of Propranolol at night with Ivabridine. Unfortunately Ivabridine caused a signficant bone and body pain and severe fatigue… a mast cell reaction.

After Ivabridine Dr. Arnoldo told me our two remaining options are stopping the Propranolol at night and starting extended release Propranolol instead or to take the immediate release Propranolol as needed… which is basically constantly.

We chose to try the extended release Propranolol and while it isn’t working… I don’t have the heart to give up on it (no pun intended) completely knowing that the immediate release is a sedative and I experience tachycardia for hours on end, multiple times a day which would mean that I would inevitably be dealing with the sedative affect while I am at work.

I am still waiting to hear back from Dr. Blair Grubb’s office to schedule an appointment. I went on the 9 month to year long waitlist in September of 2018. Once I receive the call back to schedule the appointment, I am not sure how far out the appointment will be scheduled. Fingers crossed it won’t be another year.

On February 13th I went to see Doug Doll, an Urology PA because I only have the urge to pee 2-4 times a day despite drinking between 80 and 120 ounces of water and when I do pee I pee between 800mL and 12oomL all at once.

At the first appointment they did an ultrasound that showed that despite having just emptied my bladder (because I experience the least trouble urinating first thing in the morning) there was still urine in my bladder. And despite there being urine in my bladder… I was not able to produce a urine sample.

PA Doll went over our options. We ruled out medications because it is likely that medications could make some of my other issues worse. We decided that I would keep record of my food and fluid consumption, medications (he reviewed my medications but he wants to see if there is a correlation between the time the medications are taken and the times that I am urinating.) and times taken, symptoms, bowel movements (because i am only pooping 1-2 times a week so he wants to see if I urinate more often or easier after having a bowel movement), urine output, etc. for several weeks in hopes that we can figure out if there is a trigger. It is likely there isn’t, but it never hurts to check.

If there isn’t a trigger, we will have to do a bladder study done. More or less they catheter you and empty your bladder and then they slowly fill your bladder again to determine exactly how much your bladder can hold before your bladder tells you that you need to pee and then they continue to fill your bladder to see exactly how full they can fill your bladder until you are REALLY uncomfortable.

On February 14th I had a follow up with Dr Wo, my Gastroenterologist. At my last appointment he said he wanted to reach out to my geneticist before moving forward with any testing. Unfortunately he has not made any progress in reaching out to my geneticist or my mast cell doctor.

This week my nausea has been unmanaged. On Monday afternoon while I was at work, I became severely nauseous. I tried to make it the last 45 minutes of the day but was bent over the trash can at my desk. My dad came to pick me up from work and I barely made it in the house before throwing up my lunch (eaten 4.5 hours earlier) and my half digested afternoon meds (taken 4 hours earlier). This incident and unrelenting nausea prompted me followed up with an e-mail but I have not heard back. It has been 6 weeks since I saw Dr. Wo on the 14th. I understand that I am not his only patient, but I don’t understand why it is taking so long for him to reach out to my other specialists.

On February 18th I had a follow up with Dr. Tinkle, my Geneticist. It was more or less an in and out appointment.

He gave me his direct e-mail address to give to Dr. Wo because Dr. Wo still had not been in touch. Dr. Tinkle said that Dr. Wo is not well versed in EDS so he thinks that Dr. Wo is overwhelmed but trying to understand. Dr. Tinkle also said that he has referred several EDS patients to Dr. Wo recently so hopefully Dr. Wo will get a better grasp on EDS and become more comfortable with it…

I have been working with a physical therapist he referred me to locally. That has been going well. He would like me to continue that. He says that even though my hip is shot and it won’t reverse the damage done to my hips, it looks good to insurance company because I am trying other options before surgery as well as building up strength before surgery. PT is also really helping in other areas too. She gives me an abdominal massage every time I see her and I think it helps a lot. And we are working to strengthen the joints I haven’t destroyed to prevent further damage.

But she does not specialize in EDS so he is sending me to a PT who specializes EDS in Chicago who while more or less get to know my body and the provide insight on my case. If something comes up that my local PT doesn’t quite know how to handle, my local PT can call the specialist. Kind of like a phone a friend.

On February 28th I had a follow up appointment with Dr. Tobin, my Allergist/Immunologist. The appointment went well. Because I am still struggling to breath when around people who are wearing strong scented lotion, perfume/cologne, smoke of any kind, chemical products (including cleaning supplies), etc. and having daily hives despite benign several histamine blockers we are looking into starting Xolair.

We discussed Cromolyn Sodium and Ketotifen but she has concerns about the way both for my body. She is concerned that because I am already only pooping 1-2 times a week (though when I go, it is diarrhea) that Cromolyn may make this worse. And because of my history of migraines and fatigue that both Cromolyn and Ketotifen may exacerbate those issues and therefore may not be a good fit.

We have submitted the prior authorization for Xolair to insurance. We’ll see how things go.

We also discussed me meeting with their Food Sensitivity Clinic because my current Gastroenterologist is not actually doing anything… Let’s be real. So far he has charged my insurance for two appointments and not actually done anything. He has just said he *wants* to do something but not until after he talks to my other doctors and continually puts off doing that.

So, we scheduled an appointment with the Food Sensitivity Clinic for the 24th of May.

The Food Sensitivity Clinic is a team of doctors. A Gastroenterologist, the medical students that report to him and my Allergist/Immunologist and the medical students that report to her. They ask that you keep a log of your input and output (similar to what the Urologist requested above which is convenient) for 4 weeks prior to your arrival which they will review upon your arrival to see if they can find any possible triggers and if they cannot they will proceed with testing like scoping with biopsies to test for mast cells and such.

Unfortunately there has been a lot going on at work that has caused mental and physical stress… And because my adrenal glands no longer create cortisol on their own, my body doesn’t handle stress very well.

On Tuesday, March 5th I woke up at 3 in the morning in severe pain despite my fentanyl patch. I would later puke twice and have several rounds of diarrhea. We waited a few hours hoping the nausea would subside and I would be able to keep my steroids down but it never did. On top of that my urine out put dropped from 2-3times what it should be to 100mL. I was becoming dehydrated.

Around 10:30 in the morning we made the decision to go to the ER. Upon arrival the took me straight back. While there I received 2 liters of fluids, steroids, nausea meds and a protonic.

It was a good thing that we went in when we did because tests showed that I was dehydrated and my potassium was low as well. I was headed into an adrenal crisis.

On March 19th I met with Dr. Waddell, my Endocrinologist, for a follow up appointment.

We had spoken in late January/early February via MyChart when I began experiencing low cortisol symptoms more regularly again. He ordered blood work (a renin test) that showed that my steroids were once again not being absorbed properly and increased my Prednisone dose to 10mgs spread out through out the day (6AM: 6mgs, 2:30PM: 3mgs, 10PM: 1mg).

At this appointment he reviewed my meds and we discussed how I didn’t feel like the med changed made a significant change in the symptoms I am experiencing. That I am concerned that my meds aren’t being properly absorbed like they should. That I am absorbing some and it’s barely getting me buy but when I am experiencing significant stress or my period comes or I become ill my body isn’t getting what it needs. And because my Gastroenterologist is moving at a snails pace… we aren’t making any real progress in absorption issues department.

He ordered some tests to check my thyroid levels to make sure my Levothyroxine is at the right dose and to check my steroid dose again. He was hoping that I could get those tests done at his lab before we left but his lab closed at 4 and his office didn’t finish releasing us until 10 after. Now I am working to try to find a local lab that I can have the tests done at.

I think that is all I’ve got for now. I will try to be better about staying up to date.

~Trinket