Fight Song 2015

At the end of every year I look back on the year and choose a song that was my anthem. This year although cliche Fight Song by Rachel Platten is the most fitting.

I look at the last year and realize even though I encountered some health obstacles from lung issues following a cold to a joint flare that hit me off guard, overall my health was pretty stable and I had a lot less ER trips and hospital admissions which must mean something.

Some milestones for this year including my first pulmonary function test, trying and failing a biologic, and having my port placed. Some non medical mile stones include my first trip to the shore with the family I nanny for, surviving two more semesters of college, signing my first lease, getting in to an accelerated master's program, and going on two school paid for trips to Florida including my first professional conference and a college football bowl game.

I have fought a lot to get to where I am now but I am only holding on by a thread. I am going in to the new year with a lot of uncertainties hanging over my head in terms of my health but "I don't care if no one else believes cause still got a lot of fight left in me."


Bringing Out the Big Guns (or Shots Anyways)



As I mentioned in a previous update, a couple month ago my joints started flaring badly. This is definitively on of the worse flares I have ever been in if not the worst. After a burst of prednisone did not stop it (well it helped till I weaned off of it) I went to see my rheumatologist.

At the appointment after describing my symptoms she mention Enbrel but wanted to do a physical exam first. If there was obvious signs of inflammation on the exam she would prescribe the medication but if not she was going to send me for an MRI to look for inflammation first. Well by the end of the exam there was no question my joints were not in good shape, Enbrel was prescribed.

We wrapped up the appointment by adding back in some lower dose Prednisone as well. Then the nurse came in to teach me how to do the Enbrel injections, super fun. Overall doing the injections is actually relatively simple.
Compared to an Infusion the Enbrel Supplies are Pretty Simple 

Now to get down to the nitty gritty of what exactly Enbrel is. The drug is part of a relatively new class of medications call Biologics. Enbrel was actually the first FDA approved Biologic. Biologics are used in the treatment of quite a few autoimmune disease and there are a few types. Enbrel in an TNF inhibitor. That means it suppresses TNF proteins, which are part of the immune system. In someone with autoimmune arthritis there is too much TNF from their over active immune system. Taking some of it away help bring down inflammation. However that also means Enbrel suppresses the immune system making it harder to fight off infections.
Enbrel and TNF in knee joint
Taken from the Enbrel Website
As far as side effects the most concerning is the suppressed immune system. I spend a lot of time with kids both in schools and as a nanny so I always have to be super careful and I go through lots of hand sanitizer. I also got headaches after my first couple injections but they seem be be getting better the longer I am on Enbrel. This week I had a site reaction for the first time but it did not show up till two days latter. This week I am going to try taking Benadryl prior to the injection to hopefully prevent any reactions.

The Freedom of my Port

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 me nearly enough fluids but besides for that it wasn't a hard surgery. Someone had told me it took them 3 weeks to get back to normal activities which freaked me our since I had plan to go to classes 3 days later since I was having surgery the first Friday of the semester. I knew that I would probably be fine and the doctor placing the port said to me after reading through me medical record this was no big deal compared to a lot of things I had been through. And he was right, not only was I fine to go to classes but I even went to a staff event for my job a couple days later.
When your surgical glue matches your shirt you know your outfit is on fleek

The port has been amazing. I am now getting fluids 3x a week. Every Monday a nurse comes and access the port, then I run the fluids myself and on Fridays I take the needle out. I can also do extra infusions as needed, which has saved me quite a few ER trips. I even can do them on the go. I have done infusions while in class and took all my supplied on trips with me. I really feel like I have gained a lot of freedom and control of my life with my port.

Quick Up Date

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 week. It work by suppressing certain parts of the immune system. Hopefully my insurance will approve it soon and I can start it next week.

^What I get to stab myself with weekly.


Right now I am trying to focus on myself and not constantly over doing it like I have a tendency to do.

Let's Talk About God

I do not usually talk about God here or a lot of places outside my Hebrew School classroom. I have never prayed to God to be healed, that's not really my style. To be honest some times I get mad and ask why me? But it is silly to dwell in these thoughts for long.

Growing up I was always taught God has a plan, we just might not understand it. Human cannot see and know everything the way God can. Then when it was my turn to teach I had the opportunity to teach my students about partnering with God. Both these ideas seem to play a major role in my own relationship with God.

I really do believe God has a plan for me and always has. When I was in elementary school I wanted to be a pediatrician, around middle school I had no idea what I wanted to do with my life, and by high school I discovered I wanted to be a special education teacher. However I still sometimes wonder if I should go into health care. Since I started spending so much time in a Children's Hospital I actually discovered there are teachers who work there teaching kids who are there long term or in and out of the hospital a lot. I feel like this may be the path God wanted to put me on. In high school I received little to no support to help me with all the school I missed so I feel very passionately about providing better support to students with chronic illnesses who are constantly in and out of school. My own experiences have showed me a way of partnering with God to make the world a better place for others.

Each person has there own way of being spiritual and/or connecting with God. For me I feel like I am connecting with God when I am helping others, or as I teach my students, when I am being God's partner. And everyday I try to practice what I preach. Right now I can't teach at a Children's hospital yet as I am still in school but it does not mean I am not trying to actively help. I am currently a committee member for HootaThon, which is my college's dance marathon that benefits CHOP. What's really special about my position in the organization is I am in charge of the actual hands on volunteering we do at the hospital.

Maybe I have answered my own question of why me? Being sick has put me on the path in life I am currently on, the path that allows me to give back to others. God has a plan for me.

Here is a link to my HootaThon page to learn more about it: http://events.dancemarathon.com/index.cfm?fuseaction=donorDrive.participant&participantID=56389

The Hospital Checklist



As I prepare for an upcoming hospital stay which will hopefully go as planned and only be two days I asked a few of my chronically ill friends what are their hospital essentials.

Clothes

Underwear- I always bring a bunch of extra pairs and I go for pretty but with plenty of coverage because at some point during the stay you will be on a gown. (Although as I go into below still bring some hospital appropriate outfits)

Sports Bra- (This one is only for those with boobs obviously) Nurses and doctors will be in your room at all hours so you will probably want to sleep in a bra. You also might have a test of procedure where you need to remove all metal so steer clear of the under wire.

Comfy Pants- I go for either yoga pants or pajama pants (bonus points for fuzzy PJ pants). Even if your hospital does make you wear a gown many times you can get away with your own pants. My doctor even lets my wear my own yoga pants for endoscopies.

T-Shirts- Personally I like soft closer fitting (although not tight) tees. This come down to the fact if I am wearing loose pants I want to wear a slightly more fitted top to look somewhat acceptable even in a hospital. Many people will disagree with me and opt for a way too big tee, it is really a matter of personal preference. The short sleeve part is important for IV access (unless you have a central line).

Comfy Sweatshirt- It' no secret hospitals are cold! I prefer an over-sized full zip hoodie, very accommodating to various medical equipment and tubing.

Fuzzy Socks- Even when you have to wear hospital socks you can wear comfy ones under them.

Slip On Shoes or Slippers- Even in non slip socks I like to wear some type of shoe when our of bed, L.L. Bean moccasins are my shoe of choice.



Toiletries

Shampoo/Conditioner/Body-wash-  If you are lucky enough to shower (thankfully I have always been able to shower almost daily while inpatient) you will want your own hair and body products.

Deodorant- No explanation need hopefully!

Hairbrush- See above

Toothpaste/Toothbrush- If you do forget these items the hospital probably has some but I still like my own.

Dry Shampoo- For days you don't get to shower, or shower at an odd time (I find that when you do get to shower its usually not when you normally would). This work best if you put it in your hair at night right before you go to sleep.

Body Mist/Spray- I love the Bath & Body Works ones. They make me feel fresh in the morning when I don't get a shower till later.
Hair Ties- An absolute must if you have long hair. I am also a big fan of headbands.

Chap-stick- Hospital air is dry!

Comfort/Other

Phone- Contact to the outside world is important for the sake of your sanity, (Don't forget your changer!)

Things To Do- There is lots of waiting and down time in the hospital and you are basically a prisoner. Some of the things I bring:

  • Laptop
  • Knitting (or the craft of your choosing)
  • Books
  • Magazines
  • Coloring Books (Yay for new adult ones!)
  • School Work (calling all high school/college students)
  • Lesson Plans that need writing (I am probably the only person that does this)
Blanket- It is nice to have your own, I am not a big fan of hospital ones.


Stuffed Animals- Who says just cause I am 19 means I can't have a stuffed animal or 10?



Candid Conversations

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 my peds GI had diagnosed me with and rerun all the test (including the one testing for the Celiac gene). He came to all the same conclusions and then wanted me to do a gluten challenge and another endoscopy to see if I really did have Celiac but could not tell me how it would be different from my last endoscopy I had while still eating gluten.

My PCP at this point said I was the second person he saw this week to have an experience like this at the GI department. He then turned to me and said he was going to say something very candid. I was a teenage girl with a lot of random diagnoses and no overarching or connecting diagnosis. Many medical problems yet most of what I have does not show up in blood work (although it shows up is another test like biopsies and gastric emptying studies). Many doctors look at my case and think it has been over medicalized and is really just psychosomatic issues such as a social disorder or and anxiety disorder. However patients who fit into that category tend to still live with their parents and are not in school or socialize. I do not fit into that category. I am full time student with two jobs and involved in lots of extra curriculars. My PCP said when he saw my chart he was not expecting me to be as put together and have as good of a handle on everything as I do. I am not sure how to take this. I am glad my PCP was honest with me. It confirms what I already knew about doctors treating teenaged girls differently. I just find it ironic that doctors have suggested to me I take on too much and to consider taking time off from school. Yet if I wasn't in school they wouldn't treat me the same? And think my problems weren't real?

To lighten the mood here are some other quotes from today:

While getting my blood drawn:
"Wow your veins are shot"
"Which one do you use for your infusions? I don't want to blow it because you don't have a lot of options"
"The great thing about a butterfly needle is you can really dig around"
"Don't leave the tape on too long, I can see your past reaction" (as she puts tape over the past reaction)
The reaction fro all the tape since this is the only place anyone seems to get vascular access on my viens anymore..

Then at PT after I explain how low my BP was during my PCP appointment my PT said it was okay because for the next half hour at least there was an ICU nurse there (a fellow patient)... awesome.

Stumbling Blocks...

As I previously state PT has been a struggle. It's been almost 2 months since I started and I haven't gotten very but at my PT pointed out today was only my 5 visit and at least 2 of them where washes due to other health issues.

I did have to call my Rheumy's office since I was supposed to call a month in...oops. She decided I need to have joint ultrasounds, which I got done today. If they don't show anything I am being sent to MRI, but I am not sure for which joints because having 4 MRIs doesn't sound right. Either way I do not want MRIs, my joints have been through a lot so there is a good chance they might show something but will it show the actual source of the issues? Definitely going to be pushing spending more time in PT.

Today in PT I was informed that my left glute is not firing. I shouldn't be surprised, this happened to my quad after my knee surgery, but that was only 2 weeks later, not almost 2 years. So basically most of my current joint problems will probably probably improve with activating my glute. I know from past experience this will take work but it is really best case scenario. PT I can handle, I have handled.

Although part of me is upset. After the disastrous recovery I had with my knee I was pleasantly surprised will how well my hips handled surgery and had few complications, I guess it was too good to be true.

An Open Letter To My High School Gym Teacher

Dear Senior Year Gym Teacher,

We only met briefly on the first day of the semester, then for the next third of it the seniors went to health class during the gym period. Our next encounter was at a meeting with my parents and all my other teachers as well as an administrator about my upcoming absences due to a hip surgery. You were annoyed to be there clearly. Well I was annoyed to be missing part of my senior year due to surgery but if your attitude stopped there I would have been okay.

After the seniors came back from health you asked anyone who had a doctor's note to come speak to you. I was very proud to walk over without the assistance of crutches for the first time in five weeks, a whole week sooner than predicted. The nurse had already emailed you informing you of my inability to participate in class. You asked me if I exercised outside of class, apparently you were unimpressed with my new found ability to walk. "Yes," I responded with a smile, "I go to physical therapy 3 times a week." After all what else were you expecting me to say?

It became clear you disliked me, maybe you thought I was only trying to get out of gym. However if you had taken a moment to know me or talk to my previous gym teacher you may have looked at me differently. Did you notice my knee braces? Did you know I had knee surgery the year before? Or I was headed to hip surgery number 2 just a couple days after graduation to avoid missing any more school? If you had just asked you may have found out I was fighting an incurable autoimmune disease that had taken years for doctors to diagnose, leaving me with multiple joint surgeries. I know you probably thought I was just a teenage girl who didn't want get sweaty in between her other classes. However you did not realize how badly I wanted to be normal and fit in. It was bad enough I stood out with my crutches and joint brace through so much of high school, I did not need a gym teacher to make me stand out even more.

I ask that in the future you do not judge your students who cannot participate in Gym because of  medical problem, even the ones who look okay, maybe even especially the ones who look okay. Treat your students with respect and kindness. Please remember that if a student is unable to participate in gym for a whole semester (or two years) they are probably facing a challenge no teenager should be.

Sincerely,
A girl who was trying her best

When I Feel Like I am Banging My Head Against the Wall



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 appointment however did not go as well. POTS was acting up, all the sudden I realized this was not going to be easy. For as much time in my life as I have spent in PT, with POTS it is a whole different ball game. From then on out things seemed to get worse at each appointment. I was only going once a week because of school plus work plus other medical stuff. My hip made a little progress but not my knees. And once my lungs started having issues all bets where off. Apparently being able to breathe really helps with PT.

So here I am, doing home exercises for the next couple weeks until my next appointment. Instead of increasing my appointments at the end of the semester we are decreasing them  because I am not physically handling PT well. I am trying so hard to push through like I have in the past but this time I am not pushing through pain, I am gasping for air and fighting with my cardio-vascular system, pushing through leads to passing out. Yet I need to find a way to keep going.

Today I had a reminder of what I was up against. I may have pushed myself too hard. I had an awards ceremony followed by volunteering for the College of Education graduation today. Which was fun but my job required me to go outside for a while, which I actually did okay during. However towards the end I was talking to my friend and the woman who was in charge of all the volunteers. I started laughing at something and before I knew it I was gasping for air, having a pre-syncope episode, and on the ground. I am pretty sure I never lost consciousness but I felt bad for the two people I was with. I think they were more freaked out than me. There I sat on the floor as I took some Midodrine for my blood pressure and albuterol to help me breath with a bag of chips. If I couldn't function in daily life how I am I supposed to conquer PT?

I know I will figure it out. I have no intentions of giving up. If I don't keep up with PT I can say goodbye to joint function so I am going to persevere. I just also need to be okay with myself if I struggle a little.

Surviving the Semester

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 health problems that have come up were not new, this go around was different though. 

Being in a kindergarten placement not so shockingly there are a lot of germs going around. In fact I am suprised with all my regular interactions with small children I have stayed pretty germ free this year. Well about two months ago all my peers in my placement got a cold, me included. Within a week or two everyone recovered without issue, except me. I continued to have a cough but it would improve throughout the day, so I didn't pay much attention. A little under a month ago it started getting worse and I was struggling to get my usual liter+ a day of fluids in, so I emailed my doctor. We decided if it did not start improving in a few days I should come in. I went in and was prescribed albuterol. All that did was make my heart rate skyrocket, the next day I was a million times worse, gasping for air. It was scary, really scary. 

Writing papers and creating unit plans in between coughing fits leaving me gasping for air was probably one of the roughest things I have been through during college yet. What was worse was during every class and final for the last two weeks all my classmates (I had every class with the same students) and professors asked if I was okay. Honestly I wasn't okay, I was struggling to breath and had multiple pre-syncope episodes, probably from the lack of oxygen. When it came down to it there was nothing they could do to help me so I did not want to worry anyone. I went to a doctor, in fact I went to see doctor's at my GP's office three times in a little over a week.
Finally in time for my last final the Flovent (steroid inhaler) started to kick in after maxing out the dose.Next week I have a follow up and will probably have to go on a lower dose but I am trying not to worry about that. I can not get in to see a pulmonologist till June(and if I waited for an attending it would have been July. So until then I am seeing an ENT and going for Pulmonary Function Test. Never a dull moment. 

On the brightside, I did not pass out during any of my finals. I handed in all my papers and unit plans. I even submitted my application to the accelerated master's program for special education today. I am done with school for the next few months, I have survived yet another semester.

What One Text Can Mean

Being chronically ill in college comes with many obstacles. One of the hardest ones is avoiding normal people illness. Being an education major makes this even harder. I spend lots of time in elementary schools and so do most of my friends do as well. It is safe to say there is always something going around our classes. On top of that no one ever wants to miss a class especially since we are on a Tuesday/Thursday schedule to accommodate our field placements (which I totally get because I hate having to catch up what I miss). This leads to my classmates coming in to class sick all the time. Once one of our professor even came in and told us she had a low grade fever.

For me this is really scary. Let me give you a little history of what has happened to me when I have gotten normal people sich since being chronically ill. In high school one I had a small cold or bug that was only bad for a couple days but then triggered a had joint flare, forcing me to miss multiple days of school (no morning classes for a week), led to needing a completely new med regiment, and back tracked my hip surgery recovery by weeks. A year ago when I had a simple viral infection gastroparesis was triggered and now I have a feeding tube. Yet without knowing this history most of my peers think I am crazy for never leaving my apartment without hand sanitizer and carrying around a travel sized lysol spray and wipes.

Most other college kids do not think twice about coming to class sick and do not realize how it may affect those around them. So last night when I received a text from my friend starting with her saying she had a low grade fever, me being half asleep and not bothering to read the text, assumed she probably was asking me to take notes for her or come over after class to help her catched up. When I woke up this morning and read the whole text I found out she was letting me know because while she could not miss class she wanted me to know to stay away from her (we usually sit next to each other in one class) so I do not get sick.

I do not think she realized how much I appreciated that text. Not only did I avoid a bug but I also saw that she really understood my challenges today.

A Letter to The Battles I Have Fought

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, foreshadowing my future. You taught me to work hard even when the goal seemed unachievable. I learned about persistence the hard way thanks to you. The lesson, slow and steady wins the race became my motto to defeat you.

Celiac- Your timing was just about as bad as it could have been. The week before finals my first semester of college. You taught me how to make my voice heard, how to stand up for myself. You made me outspoken and to let people know what I need. You taught me to be creative and how to make a gourmet meal in a microwave,

Gastroparesis- I do not even know what to say to you. You have taken away one of the most basic joys in my life. I have had to adjust myself to you and your needs. Controlling does not even begin to describe you . Yet you have taught me to enjoy the little things in life. I no longer take being able to enjoy a small piece of chocolate for granted.

POTS/Dysautonomia- We are still getting to know each other. At least you had the decency to first show your affects on me in front of every single doctor on the GI wing, a double edge sword. You are a tricky one, most doctors do not know what to make of you. You have taught me how integral planning is, how the smallest mistake can take a week to fix. On the bright side you have given me an hour of relaxation once a week during my infusions.

As a group you guys have tested me, put me through the ringer. Yet the struggle has made me stronger. I am now equipped with confidence and integrity. When I look back on all I have faced and the battles I have fought I know I can push forward. For that I thank you.

Sincerely,
Joan

After a Battle Well Fought


The Adult GI and Other Adventures of the Week

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 do an experiment surgery with only a 50% success rate. He seem to use the same course of treatment for all his patients and I didn't like the success rate so I passed. Once it was clear he was only going to offer me surgical options I was done, plus there were a couple issues with his office and Temple Hospital.

Then since my GI was going to be out on maternity leave she thought I should try to transition then. Of course I couldn't get an appointment with the GI recommended to me until the day before my next appointment with her after her maternity leave was over. Well both those appointments were this week. The adult GI wants scrap everything and start from scratch (I mean everything to the point he is redoing genetic test because DNA clearly changes *eye roll*). I am not super thrilled about it to say the least. I also did not like that he did not seem to want to listen to everything I had to say. He would not even let me wait until the next day when I was getting an IV infusion to get my labs drawn. I did like a few things, unlike the doctor at Temple this one looked at the whole picture. He is concerned because my ANA results were high (ANA is a marker of autoimmunity). This plus my past autoimmune issues plus all my other issues he thinks points to a bigger autoimmune connective tissue disease. He also is pretty sure I have raynaud's which has been brought up before but not in a while.

The next day I went to my peds GI and reported what the adult GI had to say. She also agreed it was unnecessary to reorder some of the test he ordered. We agreed I should keep seeing him for a second opinion because he had some new ideas but said I could continue to see her in peds until I graduated college, giving me a little over 2 more years. She agreed I should make a rheumatologist appointment (there goes my year between visits). She also wants me to see a Dysautonomia specialist in NYC for an actual diagnosis since it does not look like I am getting one from the cardio I am currently seeing. This is an important issue because during my hospitalization last week the messed up my POTS medication (florinef, a steroid by the way) and my fluids resulting in me feeling really horrible and fainting twice this week. With an actual diagnosis my fluids would have been taken more seriously with the residents hopefully.

So now I wait for the blood test the adult GI ordered to see if there is more proof of a bigger autoimmune issue and I see my rheumatologist is a month (cause that is the soonest appointment I could get but at least my bloodwork will be back by then for sure).

Feeding Tube Awareness Day 3 and 4

What Joan couldn't manage to post everyday for a week? Shocker right? 3 for 4 isn't bad though. So the topic I missed was feeding tube myths! Boy have I heard a lot of those!

Myth 1: You need to be in the hospital if you have a feeding tube. (Thanks Red Band Society for keeping this one alive and well)

Truth: Actually feeding tubes keep people out of the hospital. Feeding tubes allow people who are unable to eat enough food orally to stay out of the hospital. If you are like me and need constant fluids as well as the calories then if the feeding tube is unusable even for a few hours you end up in the ER but as long as the tube works you are good to go!

Myth 2: Only babies, the elderly and coma patients have feeding tubes.

Truth: There are actually over 300 reasons a person may need a feeding tube. Many of these can affect people of any age and they do not always look sick.

Myth 3: You look too healthy to need a feeding tube.

Truth: Many people with feeding tubes look healthy because they are healthy thanks to their feeding tube.

Myth 4: People with feeding tubes do not eat at all.

Truth: Many people use feeding tubes as a supplement to their oral diet. My ability to eat or not fluctuates depending on my med cycles. A feeding tube does not stop a person from being able to eat.

NYC Adventures Brought to You by a Feeding Tube

Feeding Tube Awareness Week Day 2 The Conversation

Certain conversations will forever be ingrained in my memory, one of them is the conversation that I had with my GI on July 9th.

After erythromycin had stopped working we were scrambling to get my symptoms under controlled. Reglan another gastroparesis med (that had many scary side effects) failed to work for me and we had maxed out my dose of Periactin. I was only getting in about 300 calories a day and feeling it. To add to the mess I started not tolerating ensure. And trust me after throwing up ensure you will never want to touch it again.

After increasing my dose of Periactin we gave it a few days to kick in but by July 9th I had to call and after explaining where I was at with the nurse I received a call back from my GI within an hour or two. Whenever a doctor calls you back that fast it is not a good sign (although in all fairness my GI does usually call my back in 24 hours).

She asked how I was and I as always replied "okay."

"Well it does not you are doing okay based on what I was told, eating 300 calories a day is not okay" she gave me a much needed dose of reality. And she was right I was not doing okay at all, I was barely hanging on. She mentioned that maybe erythromycin would work after I hadn't been on it for a few weeks or I could be admitted, which would probably end up happening. I didn't have the energy to try erythromycin again on the chance it worked and I told her that. She brought up IV fluids and a feeding tube and I knew it was time.

The next day I was admitted to CHOP for an 8 day hospital stay. During that stay I became a tubie. Even though I was far from strong when I was discharged I was about a million times stronger than when first admitted. In the 7 months since that conversation I have been nothing but thankful for it. Thankful to have been admitted. Thankful for my feeding tube. Thankful we did not wait a day longer.
The NG

It has not been easy to say the least. I have been through an NG tube, 5 NJ tubes, and one GJ tube (but I am on my 4th J part). I can say it has been worth it. Worth all the ER trips, hospital admissions, and enough trips to IR to be besties with all the nurses. Its been worth it because its given me a chance at my life. A life I would not have without a feeding tube.

Feeding Tube Awareness Week Day 1

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 intestines instead of my stomach.
A Nice Graph of a Not so Nice Condition

Dysautonomia: This is the name from a group of conditions causing autonomic nervous system dysfunction. The autonomic nervous system controls everything your body does without you having to think about it. This includes breathing, food digestion, blood pressure and heart rate control, temperature regulation and many more. There are many types of Dysautonomia, each with slightly different symptoms although each patient will exhibit their own set of symptoms. I most likely have POTS or Postural Orthostatic Tachycardia Syndrome. This affects my cardiovascular system, mostly my blood pressure and heart rate. When I stand up my nerves do not tell my blood vessels to constrict causing blood to pool in my legs. This causes my BP to drop and my heart to work extra hard to try and stabilize it making my HR sky rocket and sometimes causing my to faint. One of the most important treatments for this condition in fluids. Orally I can only drink about 40-50 oz a day but then I get almost 3 liters through my feeding tube. By adding in a liter a pedialyte through the tube during the day my HR jumps went from over 100bpm when I stood up to only around 50bpm (for reference I would start around 105 usually and anything over 100 is considered tachycardia and the diagnosis criteria for POTS is only a 30bpm jump). Dysautonomia can also cause gastroparesis.




IV Fluids: How Did We End Up Here?

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 treatment I am currently receiving. I start with midodrine, which clogged my feeding tube twice. After the second time it clogged my tube we stopped it. We have since added it as needed when I feel good enough to take it orally. This medication also just helped a bit, not enough. Next we tried Florinef, which has also helped but even after I had seemed to max out of benefits I was still fainting. Finally my cardio wanted to try epogen, which has a fantastic success rate but my insurance wouldn't cover.

I also have my feeding tube running 24/7 with pedialyte or formula. That plus my oral intake equals almost 4 liters of fluid a day!

IV fluids are not ideal. I am quickly losing veins and my cardiologist is totally against ports so I am not just what we will do next which is scary. It also means going to an infusion center once a week and not leaving Philly for more than a week at a time. IV fluids definitely should not be used as the first line of treatment.
At my 1st infusion! Quite the upgrade from the ER!

This blog post will be part of a series on my experiences with IV fluid therapy since I have been asked about it so much. If you have a question or topic you would like me to discuss please comment!

On an unrelated note, today marks 2 years since my right hip surgery! Since it is doing so well I did not think I needed to make a separate post to update on my hip.

An Actual Health Update

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 taking a pass. I was largely unimpressed with him and his office so I am not seeing him any more. I also had a more permanent feeding tube put into my stomach called a GJ tube (yeah I had surgery and forgot to put that on the blog). In February I am seeing both and adult GI at UPenn and my GI at CHOP, although I do not think much will change.
There is now a hole in my stomach!


Cardio- As my fainting episodes have continued to get worse we keep playing around with medications. I am currently on a steroid called  florinef, although thankfully it does not have all the same side effects as prednisone. However it is not enough to get my BP up and my heart rate down. My cardiologist wanted me to start epogen injections but my insurance wouldn't improve it so we are trying to get up weekly IV fluid treatment. I also have the official diagnosis of Dysautonomia.

Cardiology appointments require button down shirts.
GP- Okay, so this isn't a specialist but whatever. I just wanted to mention I really like my new GP in Philadelphia. He is at UPenn which is where my cardio is too so that makes life easier. He wants to see me every 6 months because I am medically complex. I like that he gets that and is very reachable by email.