Tuesday morning, I woke up early. Usually, I tried to sleep as late as possible because there wasn’t much to do in Hell-Crest Commons, the nursing home that I’d been living in for months now. The only times I’d get up early was if they for some reason scheduled PT or OT early. This time I had an appointment that afternoon with the Nerve Injury Clinic. The Nerve Injury Clinic was at Mass General Hospital in Boston. They were the ones that had done my skin punch biopsy while I’d been at Mass General for 5 months in 2014. Dr. Hurrowitz who was my primary neurologist there was the one following me. He had been the one that had originally come into my room at Mass General to tell me that he knew what was wrong with me and had proceeded to explain my new diagnosis to me.
This appointment was the follow-up that the case manager at Berkshire Medical Center has set up for me.
The chair van wasn’t picking me up until 10 AM but I was too nervous to go back to sleep once I woke up around 6:00 AM. The morning aides didn’t come in until around 7 AM, but by that point, I was able to transfer myself in and out of bed independently and walk around the room a little bit with just the walker to the wheelchair. All of my hard work in PT and OT had paid off.
I used the bed controls to sit myself up, then I used my arms to slide my legs over the edge of the bed and scoot my bottom to the edge of the bed. My walker was waiting for me right at the edge, where I had left it the night before.
Once I was in the wheelchair I pulled the IV pole in front of me so that it would move with me when I moved the wheelchair, and then I headed over to my closet to pick out the perfect outfit to wear to Mass General. I knew I was just going in a chair van and then a hospital, but I didn’t go out much and wanted to look good.
My mom was going to go to my appointment with me at Mass General as well. She was going to drive down separately and meet me there. I had been calling her the three times a week that I was allowed, but they were very stilted conversations and I had a feeling that our relationship might have been forever damaged. Still, she came up to visit me the once a week she was allowed by the rules she and Jillian, and Tanya the social worker, had worked out, and when we weren’t fighting we had nice visits and I was looking forward to having time with her at this appointment.
At my last appointment with Dr. Hurrowitz at the Nerve Injury Clinic they had done a second Skin Punch Biopsy for comparison. I knew at this appointment we would be reviewing those results and I was very curious as to what he would say about that. In the patient portal, it had the results and said that they were grossly abnormal but that was all they said, I didn’t know what to make of that and had a bunch of questions.
I was hoping my mom had a lot of questions as well and that he went into detail explaining what exactly was wrong with me, how it was so much more severe than garden variety SFN, and what the recommended treatments were so that my mom could be refreshed on all the info that she had somehow completely blocked out with a hefty dose of my dad’s denial she had guzzled down after Jillian shared her ideas that I could be totally cured of a condition, she knew nothing about, with a little tough love.
The woman who came to pick me up for my ride down to Mass General at 10 AM was super nice. She had a very happy smile and asked me about my build a bear. That day I had Haley with me.
“Ready for a long road trip?” she asked me.
“All set!” I told her, as she pushed me in the wheelchair down to the elevator and then out to where she was parked in the front of the nursing home.
The driver, whose name was Jamie, pushed me onto the wheelchair lift and then locked my chair. I got a little scared as the wheelchair lift, with me on it, slowly went up to bring me into the wheelchair van. At first, it felt a little like I was going to tip backward, and then I was terrified that I was going to slide off.
It only took a few seconds for Jamie to raise me to the level of the floor of the van, but it felt like time stood still the entire time. Once the chair was level with the van, Jamie loaded me on and used special clamps to lock my wheelchair to the floor of the van. Then she put a seatbelt on me and got in front.
Jamie and I chatted the whole way down to Boston, and although it was a bumpy ride, and took about 3 hours, it went by fast. My mom met us at the front entrance so Jamie let down the lift and unloaded me right there.
Getting off the lift was even more terrifying than getting on. Jamie opened the side doors of the van and pressed a button that made a metal platform unfold through the doors. The platform had a little gate at the end of it that stood up to block a wheelchair from going over the edge, but it was only about two inches tall at the most.
She pushed me onto the metal platform until I was only about an inch in front of the little end gate. Then she locked my wheels, held onto me, and lowered the lift to the ground. I swear the planets stopped their rotations for a few seconds as the lift lowered me to the ground. Yes, it was that scary.
Nowadays, I drive myself on and off the lifts in my powerchair with no help, no problem, I don’t think about a thing, but back then, six years ago when I was new at the whole wheelchair-bound thing, boy was it terrifying.
The first thing my mom said to me, after Jamie passed me off to her, was,
“Where’s your walker?”
“I didn’t bring my walker,” I told her.
“Why didn’t you bring your walker?” she asked me.
“Because I haven’t been able to walk around Mass General with just a walker for years,” I explained confused.
I had been using wheelchairs at Mass General since 2011, how in the world did she expect me to suddenly be able to use a walker here.
“Haven’t you been using your walker at Hillcrest Commons?” my mom asked me.
“Yes, but only to walk a few steps,” I explained. “The farthest I’ve walked with a walker is across a very small room.”
“You need to keep pushing yourself,” my mom said.
“I know,” I agreed. “I am. I’m trying as hard as I possibly can. Believe me, it’s not for lack of trying. You can even ask Lynn my PT, I work my butt off in therapy 5 days a week in PT and then 5 days a week in OT too.”
My mom looked like she was going to say something, then looked at me again, and then was quiet. She quietly pushed me to the elevators and we headed to the eighth floor of the Wang Building to see the neurologist.
Dr. Hurrowitz did a whole bunch of tests. He shined a light in my eye and explained to us that my pupils are very dilated and don’t react normally to light. He said that I don’t have much sensation from my knees down on either leg which is why I’m having so much trouble moving my legs. He also said that I have diminished strength all over my body and that it’s only getting worse since the last time he saw me and that my balance seems worse too. Then he showed us the test results. He explained that my skin punch biopsy results came back showing a density of neurites of skin surface area at less than the 1st percentile, and values that are at or below the 5th percentile mean you have a severe form of small fiber neuropathy.
“This indicates that you have the worst possible case of Autonomic SFN that you can have and that it is a degenerative form.” Dr. Hurrowitz explained to me. “This is a very bad sign. I’m so sorry. But we are going to do everything in our power to help you. Unfortunately, though, there is no cure for this disease. You seem to be declining rather rapidly which I’m sure you’re aware of. All we can do is try to find measures to slow its progress and keep you as comfortable as we can. At some point in the future, not at this appointment, it’s too much for this appointment, we need to talk about overall wishes for treatment and treatment goals. We need to decide at what point is it too much treatment, at what point do we pull back and just focus on comfort care, or do we want to just keep going and keep on fighting with every last tool. All of that is up to you Becca. All of that is your decision and your decision only. We do need to have that discussion though. I know it’s a really tough discussion but it’s something that we need everyone on the same page on.”
I felt my mom’s hand reach out for mine and grab it, so I looked over at her and saw an expression of fear and some other emotion I couldn’t place, smeared all over her face. My mom’s hand felt smaller than I remembered. I squeezed her hand, then she squeezed my hand back. I’m not sure if either of us knew who was comforting who. Somehow in the last minute, the whole dynamic of our relationship had just shifted again.
“What’s the plan then?” my mom asked, attempting to gather herself back together.
“Well, IVIG is still definitely the best option, but I’m getting all of these records of changing IV fluids, ER trips, and hospitalizations about electrolyte imbalances and V-tach due to low potassium sent over by the nursing home. I need to know what’s going on with that. We can’t do IVIG until I can see that we’ve had a solid six months on a stable amount of IV fluids with stable electrolyte levels and no crazy arrhythmias due to electrolyte imbalances.” Explained Dr. Hurrowitz.
“That was Jillian,” I explained. Jumping in before my mom could shoot at any good guys. “She decided that people with SFN don’t need IV fluids and that I, in particular, didn’t need IV fluids and that I could get all of my fluids via J tube and tried to cut down my IV fluid and replace it with J tube water boluses. That caused major electrolyte imbalances and caused me to go into V-tach.”
“With the autonomic type of SFN you need the IV fluid to survive and the J tube water boluses are obviously, as you guys learned, not going to work. Is this Jillian still your provider?” Dr. Hurrowitz asked me.
“Yes,” I said.
“I will have a word with her then,” he assured me.
“What do we do in the meantime while we’re waiting for the IVIG to become an option?” my mom asked.
“We focus on keeping her stable. We monitor vital signs, manage pain with her liquid Dilaudid through the J tube, Baclofen for muscle spasms, gabapentin for nerve pain, plenty of IV fluids with electrolytes, add in extra IV fluids if her blood pressure drops too low, fludrocortisone twice a day, midodrine three times a day,, Clonidine if her blood pressure goes too high, add in extra Metoprolol if her heart rate spikes too high, frequent EKGs, at least weekly bloodwork, monitor weight, adjust tube feed as needed, use compression clothing, physical therapy, occupational therapy, keep nausea under control with antiemetics and if the under the tongue dissolvable anti-emetics or rectal anti-emetics aren’t working we switch to IV anti-emetics, manage constipation with enemas, suppositories, and laxatives, manage diarrhea with Immodium, monitor for frequent infections, treat infections with IV antibiotics, support breathing with supplemental oxygen, and just keep putting out all the little fires. I want you guys to make sure you follow up with all of the specialists as well, and whatever you do, don’t let Jillian stop those IV fluids again, those are the biggest key factor in her treatment..
Dr. Hurrowitz had us make a six-month follow-up appointment with him and then we decided to go to the coffee shop. I wanted an Earl Gray tea and my mom wanted a latte. We sat together in the lobby as the whirlwind of the hospital spun around us. We didn’t talk much, but there had been a shift in the underpinnings of our relationship again. I had been right about the IV fluids being necessary and about me not faking my illness or it all being in my head but didn’t say anything about it, it just kind of hung in the air, unspoken. My mom reached out her arm and rested it on my arm.
“I love you,” she told me.
“I love you too,” I told her back.