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.