When you have any sort of severe life-threatening chronic illness, there is a terrible feeling of being at the mercy of the medical system. If your doctor is in a bad mood it could be the end of your life. That is not an exaggeration.
I suffer from a disease called Small Fiber Autonomic Polyneuropathy, which is a very rare condition that most doctors have never even heard of, because of that I often get denied the proper treatment even though I know what the right thing to do is. The doctors don’t like to admit they don’t know something so they just plow forward with their ideas and I’m the one that gets hurt or sicker or almost dies (not an exaggeration, I’ve lost count of how many times I’ve almost died due to doctors who don’t know about my illness deciding they know more than me and going forward with an idea that is potentially lethal and puts me in the ICU.
I had been seeing a nephrologist whose name was Dr. Green to oversee my IV fluids and dysautonomia meds before going into the nursing home I had just been discharged from after six long months. Unfortunately Dr. Green had informed me that she was retiring and that I should look for someone closer to my area anyway.
The day after Jeff, the man at Side by Side that I had secretly fallen in love with, and John, my new friend who knew a lot about computers came over the first time to fix my internet, I had an appointment with my new nephrologist. I had chosen to see a man named Dr. Rose. I knew it was going to be an important appointment because Dr. Rose was going to be the doctor in charge of prescribing my IV fluids. I was starting to get a little anxious because I was halfway through my first week home and only had a few bags of IV fluids left. If Dr. Rose didn’t feel comfortable prescribing them I was going to be completely screwed.
“It’s going to be ok, there’s no reason for him not to continue the treatments that have been working for you, the treatments that Mass General’s top specialists have recommended for you. All you have to do is show him your portal for Mass General, let him read the notes from Dr. Hurrowitz, show him the results of your skin biopsy, you’ll be fine.” Lesley tried to reassure me when she saw how anxious I was that morning when she came in to get me ready.
At that point, I’d had a repeat skin biopsy that had shown my autoimmune-mediated small fiber autonomic polyneuropathy had gone from severe, to the most severe case you can have. I was officially end-stage. Dr. Hurrowitz had repeated a skin biopsy at the end of my last appointment with him and the results had come in a few weeks later and shown up in my portal. Dr. Hurrowitz had then called the next day to explain what they meant.
“What if he’s like Jillian was like at the beginning though?” I asked Lesley. “What if he tries to tell me it’s all in my head or I’m making it up for attention or because I just want to be sick?”
By then I had such bad medical PTSD, that this was a constant fear of mine. Sadly this is an all too common fear in those with invisible, severe, life-threatening chronic illness. We get passed off when our diseases are too complex to be diagnosed with one simple blood test or x-ray. We get told we are too stressed out and are just imagining or creating symptoms, or we are looking for attention, or trying to get something out of being sick, it’s so unfair, but it happens so often.
“You are incredible at explaining your illness,” Lesley told me, “all you have to do is explain it, use your labs, and biopsies, scans, and notes from your doctors at Mass General that you have in your portal on your phone to back you up and you shouldn’t have a problem. All you’re doing by worrying like this is stressing yourself out.”
I knew she was right, but I couldn’t find a way to turn the worry switch off.
After all of the morning routines were done, Lesley brought me over to the main building and wheeled me into the kitchen over to where the ride forms were kept. She leafed through the pile of forms until she found the one with my name on it which she handed to me. I grabbed it in my sweaty little hand, folded it in half, and put it in my lap with my bear Haley. Then I had Lesley wheel me over to the window so I could watch and wait for my ride to come.
Time seemed to stand still as I waited. I watched cars drive by and make gray slush piles fly up and splash a couple of poor pedestrians. I watched a few random people bundled up in winter coats walk by with their dogs talking on their phones or listening to music. I watched a chair van pull up and let a resident come out, and watched the resident push her walker up the ramp and come inside.
Finally, I saw a chair van pull up and a man with a dark brown ponytail and beard/goatee came out of the van with a clipboard in hand. He came up the ramp and into the building and looked around for a moment.
“Is there a Rebecca Pava in here?” he asked.
“That’s me,” I said.
Just then, from out of nowhere Jeff appeared.
“Is that you Muscles Harford?” Jeremy asked him.
“Yeah man,” Jeff smiled.
“I went to high school with you,” Jeremy said. “We used to party together.”
“I remember you,” Jeff smiled. “Those were good times, good times, back before my liver hated me, back before I killed it.”
“I’m sorry to hear you’re so sick,” Jeremy said.
“Hey, you know what, I did it to myself,” Jeff said, “and I had a damn good time doing it. Becca here, didn’t do anything, and she’s got it even worse than me, now that’s not fair.”
“Well I’ll take really good care of Becca,” Jeremy said.
“You better,” Jeff said, “She’s my friend.”
I smiled, but my cheeks were flaming red. I loved how he had told Jeremy to take good care of me and how he had called me his friend. What a total sweetheart. For a few minutes, I hadn’t even been thinking about how worried I was about this appointment with Dr. Rose.
Jeremy loaded my wheelchair onto the lift (I’d gotten much braver on the wheelchair lift by now, I was no longer scared out of my mind when the lift went up to raise me to the level of the chair van entrance) and then locked my wheelchair in place on the van and we set off for the Medical Arts Complex, which was a big building next to the hospital. It was full of doctors’ offices. Jeremy turned out to be a really nice guy and he calmed me down a little bit by telling me stories about knowing Jeff in high school. This was a common theme with Jeff. Everywhere we went in Berkshire County, people knew Jeff as Muscles Harford and had quite fond memories of partying with him in high school. Apparently, he had always been the life of the party. In my eyes, he always will be the life of my party.
Dr. Rose was on the sixth floor. Jeremy personally pushed me up there himself and made sure I got all checked in before leaving his phone number with both the receptionist at the front desk and me. Then I just waited in the waiting room filling out paperwork and feeling my heart hammering away inside my chest at a rapid-fire pace.
By the time I finally got called in, I almost felt like I was going to puke, and my fingernail beds were turning blue, but I hadn’t brought my oxygen with me. It’s amazing how badly stress can aggravate an already sick person.
“Your blood pressure is only 82/38,” Dr. Rose told me when he came in the room. “And your heart rate is 146, that is quite unsafe. Are those numbers normal for you?”
“Yeah, they’re part of my dysautonomia,” I explained. “They’re a little worse than normal, but I was also stressed out about this appointment.”
“If it was stress-induced your blood pressure would have gone up,” Dr. Rose explained. “This is dysautonomia. I understand that you are on IV hydration?”
“Yeah, I have a port-a-cath,” I gestured to my chest,” and I am accessed 24/7. I have fluids running approximately 12 hours a day and I used 5 percent dextrose and 40 milliequivalents of potassium. I run 2 liters a day.”
“We may need to increase that, or play around with that somehow.” Dr. Rose told me. “I don’t like your vital signs. Do you take in anything by mouth?”
“I do sips of clear liquids, but they immediately drain out into my G tube drainage bag.”
“What would happen if you didn’t let them drain?” Dr. Rose asked me.
“If I didn’t throw them up right away I would dry heave for a while and then develop a bezoar and a bowel obstruction and end up needing emergency surgery for the bowel perforation that would occur.”
“Well, let’s make sure you let those clear liquids drain then, huh?” Dr. Rose smiled at me. We went on to discuss more about my autoimmune-mediated small fiber autonomic polyneuropathy, my brain tumor, my diabetes insipidus, my hypothyroid, and all of my other diagnoses.
“I am blown away by how much you know about yourself.” Dr. Rose told me.
“No one else is going to be able to advocate for me as well as I’m able to advocate for me, so I listen to everything the specialists and experts tell me and do my own research and pay attention. In the past, I’ve saved my own life like that.” I explained.
“I commend you for that,” Dr. Rose said. “You would not believe how many patients I get in here that I ask, ‘what medications do you take?’ and all they can tell me is ‘I take a circular blue pill in the morning I think, or maybe that’s in the afternoon, and then two white ones, but I don’t know what they’re for’.”
I just shook my head and smiled. I would never take a pill unless I knew exactly what it was called, what it was for, what some of the side effects to watch out for were, and how I would know if it was working or not. I had been through too much in my short life to be any other way.
“So, we need to order you some IV fluids and get you some home nursing orders and supplies.” Dr. Rose said.
I gave Dr. Rose the phone number for Baystate Home Infusion, the company I had been using when I lived in Springfield MA before moving to the Berkshires. I had already checked with them and they said that they delivered out to Pittsfield and that as long as I had a prescribing doctor they would be happy to work with me again.
Dr. Rose took down all of the information and then explained to me that he would like the nurses to draw weekly bloodwork and visit me twice a week and that he would like to see me in his office once a month at first. He also explained that he would probably want to increase my doses of Fludrocortisone and Midodrine to see if it would bring up my blood pressure a bit.
My relief at the fact that I was being taken seriously and getting appropriate treatment was palpable, it was like I had been breathing through a pinched off hose, and someone had just let the hose go and now fresh oxygen was rushing into me.
After the appointment was over I wheeled myself back out to the receptionist, scheduled an appointment for the next month, and then called the number that Jeremy had given me.
“I’m all set to be picked up from my appointment at the Medical Arts Complex,” I told the woman that picked up the phone at CRT the chair van company.
“It will be about half an hour or 45 minutes,” she told me.
“You guys can’t get me sooner?” I asked.
“Nope,” she told me.
I sighed.
“Ok, I’m on the sixth floor in the waiting room,” I told her.
“Okay,” she told me and hung up.
I hated that they thought just because we were disabled we had all of the time in the world to just sit around in a waiting room waiting for a ride and doing nothing. I had a book I was writing, I had college classes for a degree I was finishing up, I had sketches of my bears that I was working on. My time was just as valuable as anyone else’s.
I sighed again, and settled in for the wait, trying to soothe myself with thoughts of how soon I could invite Jeff back over again to finish helping me fix the internet.