Life with a Flare

A little over a week after Sjogren's was confirmed I had an appointment with my rheumatologist. My head was filled with questions and possibly a little too much online reading (however I have graduated from google and now use my college's online database to read medical textbooks). I had a list of questions on my phone I wanted to ask and did get to most of them. However the ones I missed was due to me already feeling overwhelmed and wanting to focus on specific things, not because the doctor rushed me which is a nice change. The most important part of the appointment was figuring out a treatment plan. My rheumy asked what the most pressing symptom is and obviously it is the autonomic nervous system issues (autonomic neuropathy). He thought the best treatment option was a medication call azathioprine (Imuran), which is an immunosuppressant drug. However before I could start I need a blood test to see if I had the enzyme needed to properly metabolize the medication (10% of the...

I just realized that 3 months have pasted since I last posted on here. They have been a crazy 3 months at that. In my last post I had mentioned uncertainties and hanging on by a thread as I was not tolerating my feeds. I ended up spending a good portion of January inpatient due to ever worsening GI motility. I had some pretty extensive testing that did not give us any answers, which was frustrating. I was finally discharged not tolerating any better but forcing myself to push on with feeds because I wanted to get out of the hospital. I also had to increase my IV fluid to 1-2 liters a day. I now run fluids and/or feeds 20 hours a day, but thankfully I am back up to a healthy weight. I was at such a slow rate at one point in the hospital they used a med pump instead of a feeding one. A few theories were brought up by a GI motility specialist I am now seeing in addition to my normal GI, one being there is an autoimmune issue and the others are all genetic issues which can be contrib...

At the end of August I had a Bard Power Port implanted in my chest. After 8 months of weekly schedule infusions and about a year of have almost weekly IVs scheduled or not my veins were becoming less and less usable. I was exhausted too. I was exhausted from my weekly trips to the infusion center, in addition to my normal medical appointments. I was exhausted from still crashing only a few days later from POTS. And I was exhausted from the ER trips that happened when I crashed particularly hard. My cardio did not want me to have a central line but when I came to my appointment mid-August the PA saw my arms that were so beaten up from the failed IV attempts and blown veins coupled with exhaustion from my most recent ER trip and a port was ordered. The port surgery was at the Hospital of the University of Pennsylvania (HUP), since that is where my cardio is. Unlike CHOP they were not as familiar with POTS patients (ironic since that is where I am treated for POTS), so they did not give...

Remember that time when I used to blog? Well I am going to attempt to do that again. Lots has happened since my last post and I am not sure how to fit it all in but I will do my best to try. On the POTS/IV fluid front I finally got a port! This was very exciting since it was taking multiple tries to get IVs in me more often than not. I am also able to get IV fluids 3 times a week at home now and if I need an extra one I can do it myself with no ER needed. The port probably deserves it's own post, but in short I love it. My Port is a Pretty Purple! Unfortunately after over a year of pretty calm joints a cold triggered a major flare. My joints are currently worse than they have been since high school. My rheumy was hoping a burst of Prednisone would calm everything down, and it did till I stopped taking it. So her office managed to fit me in for yesterday and she put me back on Prednisone and prescribed Enbrel. Enbrel in a biologic medicine that is taken by injection once a...

Every 6 months I go to my PCP for an appointment (unless a problem pops up prior to that), I call it my "I am still not dead" appointment, as normal people only have to see their PCPs every two years for a physical. Today's appointment led to some great conversations. Let me start out by saying one of the things I really like about my PCP is his honesty. We started out the appointment by him saying "Your vitals aren't horrible but..." apparently my blood pressure was pretty low. This isn't uncommon for POTS patients but as far as POTS patients go I tend to run on the high side so the reading (which I don't quite remember but was 90something/60something) was low for me especially since it was the day after an infusion. Later on during the appointment we talked about what specialties are being transitioned from peds to adults. Of course my adult GI disaster was talked about. Long story short the adult GI I saw wanted to un-diagnose me with everything m...

In the past couple months of my life I have felt like I have been trying so to get my health in order but the harder I try the worse it goes. At my last rheumatologist appointment about 2 months ago between my knee pain and lost ROM in my left hip we decided maybe it was time to try PT again. I was ready to hit these problems head on. Prepared to make PT work. It HAD to work. The other options weren't pretty. If it didn't I would probably be sent for a knee ultrasound (that isn't the bad part, and then either Prednisone (a.k.a. Satin's Tic-Tacs) or even worse sent back to ortho. Last time I saw my ortho he told me to come back when I was ready for knee surgery. I went to my PT evaluation hopeful. I soon found out my PT also had a hip scope for FAI. Everything seemed to be going well. I made a lovely sticker chart to keep track of ding my home exercises everyday. It had glitter! And owl stickers to go on it! What more do I need to keep me on track? My second...

My Lungs Got a Close Up and I Got to Choose the Gown Color Every semester I seem to be muddling through at the end. In fact this trend pre-dates my college career. December of senior year I was diagnosed with Uveitis. Two days after graduation I had hip surgery, meaning the two weeks before that I was medication less, not pretty. Then in college I was diagnosed with Celiac about 5 days before my first set of finals. Spring of last year the week before finals was when erythromycin stopped working and my downward spiral towards the feeding tube began. This past fall semester my POTS was so bad during finals I thought I was going to pass out during my math final, I lasted until 2 hours after it. In the ER I found out I at least got a 90% on the final and the doctor high fived me.  So in true Joan fashion something had to go wrong in the past two weeks of my life and sure enough something did. Unlike in the past I was much more blindsided by it. With past finals weeks the healt...

Dear Illnesses, Enthesitis- You are my oldest constant companion. For years no doctor could find you and yet I was well aware of your presence. At age 10 you started to attack me, stripping me of my active lifestyle. For all you took away from me you have given me just as much if not more. You taught me how to fight and that doctors do not always know best. You taught me perseverance from a young age. Without you I do not think I would be able to handle my other health issues. Thank you for preparing me for the world. I wish you waited a little longer to show up but I am glad you came. Uveitis- You took me by surprise, possibly the only condition to accomplish that. I will never forget the day you were found in my left eye. I was scared and not sure what lied ahead. I still have a fear of eye drops and all things near my eye thanks to you. You taught me sometimes I have to do things I hate but thats just the way it is. AMPS/RSD- You were the first to affect my nervous system, for...

Remember that time I tried to blog for a week straight and failed miserably? It wasn't totally my fault, I ended up finishing out feeding tube awareness week at CHOP due to an out of place tube, how ironic. On the brightside Valentines day in the hospital is now something I can check off my bucket list along with Halloween and my birthday. I haven't talked much about my attempted transition to adult care. So far I have cardio and a GP and I am not getting very far with the rest. My peds GI had mentioned to me that usually they transition patients at the end of their first year of college but I had been really sick and was still pretty sick so I did not have to transition quite yet. She did want me to see an adult GI at Temple Hospital because he was the top doctor in the country for gastroparesis and might have options not available in pediatrics, and possibly at some point I could transition. He had one non FDA approved medication I tried and failed and then he wanted me to ...

Today is the beginning of feeding tube awareness week! A year ago if you had told me I would be feeding tube dependent in less than 6 months I would be feeding tube dependent I would say that's crazy. Anyways here I am and its not super scary like one might think. Day 1 : Talk about the reasons you, your child, or your loved one is tube fed. Raise awareness of the medical conditions that made tube feeding necessary. I am tube fed because I have gastroparesis and dysautonomia. Gastroparesis: Delayed stomach emptying, which means I can not tolerate normal amounts of food because it doesn't leave my stomach as fast as it should. The symptoms include feeling full, nausea, and vomiting (there are many others, these are just the ones I deal with the most). Gastroparesis is caused by nerves not firing and telling the stomach to empty. Due to gastroparesis I can only consume a few hundred calories a day so I receive most of them through my tube that goes into my small intestin...

Many people have asked me how and why my cardio decided to do IV fluid therapy. I will preface this with I have a very specific set of circumstances so what works and doesn't work for me will not be the same as for others. We have known for as long as I have had Dysautonomia symptoms that IV fluids made a huge difference. When I first fainted during my hospital admission in July the doctors immediately upped my fluid rate from 100ml/hour to 150ml/hour during the day and I had no more issues. Furthermore any time I have been put on IV fluids my heart rate stabilizes and doesn't jump when I stand. During my hospital admission in November I was actually kept an extra night for fluids because my body just couldn't seem to wean off them. In the recent months I have been in the ER about every other week for fluids. So while I never did a trial run like some people have ask, we knew IV fluids would help me. Fluids was not the first treatment option I tried and is not the only ...

Once upon a time this was a blog I updated on my health multiple times a week. I now realize since the whole NJ tube thing I have not given an actual update on my health. A lot has happened in the last few months to say the least. I think the best way to go about this would be to go by specialist: Rheumatology- As I mentioned a few post ago at my last appointment with my rheumatologist my joints were doing amazingly and I don't have to go back for a year. As of now I am only on celebrex for my joints, which I was really hoping to get off of but since sulfasalazine clogged my tube that went instead. The last week or two my left SI joint has been a bit stiff and sore but I am hoping tylenol will be enough for it. GI- In September I saw a GI at Temple Hospital who is supposed to be the top doc for gastroparesis. He put me on domperidone, which is not FDA approved and did not help. The only thing he had left for me was an experimental surgery with a 50-60% success rate so I am taki...

Two years ago I was doing my research before my first rheumatology appointment and I discovered most people with autoimmune arthritis are on self injectable medication. 90% of kids with Juvenile Arthritis take a medication call Methotrexate, which can be taken orally but is mostly done by injection. Although I was also pretty positive we were not going to find anything at my rheumy appointment so I didn't think much about it. Once we discovered Uveitis in my eye though I thought my fate was sealed. Just under two years ago I was trying to prepare myself for the idea of sticking a needle in my leg. Within a few months of my then rheumy not doing anything I realized as long as I was under her care, for better or worse, I was not in danger of having to self inject. Under the care of my current rheumy the thought of possible self injections was on a very far back burner in my mind for a while but since my joints have done so well in the recent months it has totally disappeared. So to...

Remember last year when I started posting really sporadically because I was too busy living life? Well I wish that was the reason for why I haven't been posting lately but unfortunately I have been busy being sick. I have spent too much time at CHOP. Typing it all out would take a while so I am going to do a by the numbers post for the semester so far. 1 Hospital Admission 1 Gastric Emptying Study 1 DEXA Bone Scan 2 NJ Tube Changes 2 EKGs 3 Different NJ Tubes 3 ER Trips 3 Doctor's Appointment 4 Abdominal X-Rays 5 IVs 5 Trips to Interventional Radiology Countless Episodes of Tachycardia From My 4th Trip to IR At Least the X-Ray Rooms Look Like This ER Selfie! Yes I Spent Two Days in a Row in IR Just Getting Some IV Fluids! Sitting(top) vs Standing(bottom)...Eeeek! Two say I am excited for my cardio appointment is an understatement! 11 more days!!!!