What’s Autonomic SFN?

Autonomic Small Fiber Neuropathy is also known for short as Autonomic SFN. It’s a shutdown of the unconscious nervous system combined with body-wide neuropathy. Eventually, your whole organ system shuts down. I’ve been struggling with autonomic SFN since I was eight years old. At age 26 my tests started coming back showing that I had the most severe form of it possible. This meant I was in the end stages of it.

In the last few days, my body had taken a beating

First, there had been all the trauma of feeling so sick and almost dying. Then there was the further drama of being suddenly kicked out of Berkshire Medical Center. After that, there had been the hour-long drive to Baystate Medical Center. During the drive, I had to force myself to keep talking to Melody. I was being pulled toward the sleepy abyss of Autonomic SFN. My fear was that if I let go and spun into it, I would never return.

Arriving at Baystate Medical Center

Once we got to Baystate Medical Center I was moving in slow motion and unable to transfer into my wheelchair. A whole bunch of people came running out of the ER and put me on a stretcher. Then they rushed me into the trauma room where they assured me they would take care of me. When I felt reassured I’d be cared for, I finally let go and allowed the darkness to pull me under.

When I woke up, I was no longer in the emergency room, I was in a room in the ICU. The double-lumen PICC line was still in my right arm. My port was still in my chest. Now though, I also had another central line in my neck. This one was a triple-lumen central line. There were a million IV bags hanging on a pole next to me. I had a special line stitched into the inside of my wrist.

“That’s an arterial line,” explained the nurse. She had come into the room as soon as she saw that I had woken up.  “It’s in an artery in your wrist. We use it to get the most accurate readings of your blood pressure. It’s been so low you could trip on it. It was also helping us know if we had you on the right ventilator settings. When we had the tube in that was breathing for you.”

So that explained the gravelly throat and the taste of lubricant in my mouth. They’d had me intubated and on life support.

Autonomic SFN Stole My Time Again

“How long have I been here?” I asked the nurse, almost afraid to hear the answer. The Autonomic SFN dropped my blood pressure so low that it made me pass out. Sometimes for days at a time., That was one of the most frustrating things that happened to me. It also caused other severe complications like bowel perforations due to the gastroparesis it caused. I would end up passing out and needing emergency surgery. Or it would cause septic shock because it shut down my immune system. This would make me black out and lose days or weeks as well. All the complications caused lost time due to being unconscious on life support.

It felt like such a waste to lose all that time. Even more so lately, since I’d been getting sicker. The question of how much time I had left kept popping up in everyone’s heads

‘Three days,” she told me.

Making Sure People Know I’m Alive

“Does anyone know that I’m here?” I asked.

“We called your mom because she’s your healthcare proxy. I’m sure she told everyone who needs to know where you are and what’s going on.” The nurse assured me.

‘Where’s my phone?” I asked her.

The nurse picked it up off my bedside table.

“It was going off quite a bit, but then I think it died. Do you have a charger with you?” she asked.

“Yeah, in my purse,” I told he

She went over to a closet, pulled out my Hello Kitty purse, and handed it to me. I ruffled through it and pulled out the charger.. As soon as I found it I handed it to the nurse so that she could plug it in. I needed to reach Jeff.  Hopefully, my mom had gotten a hold of him and had been giving him updates, but I wasn’t sure.  He was probably going out of his mind with worry.

The nurse listened to my lungs, heart, and stomach. She adjusted some of the multiple IV bags. Then she gave me some pain medication,

“I’ll leave you alone now to make your phone calls,” she told me. “He must be one special guy,”

“Oh, he is,” I told her. I showed her the home screen on my phone which was a picture of the two of us.

‘I can tell the two of you are completely in love with each other.” She said.

“We were made for each other,” I told her.  “God meant for us to be together, I am 100% sure of that.”

“I can see it in your eyes and posture and the way you hold each other,” the nurse agreed.  “Let me get my little butt out of here so you can let your honey know you’re okay.”

Letting Jeff Know I’m Alive

After the nurse left, I immediately called Jeff.

“You’re awake and talking,” he said with the purest joy in his voice.

“Yeah, thank God I took your advice and went to Baystate. If I waited any longer, I probably wouldn’t be here right now.  You saved my life again,” I told him. “Dumb Autonomic SFN.”

“Well, that’s me, Jeff the LifeSaver.  My sisters always told me I was a Sour Patch Kid growing up. I knew I was really a Spearmint LifeSaver.”

I giggled.  Jeff was too funny sometimes.

A Three Day Nap On Life Support

“So did you have a good three-day nap?” he asked me.                  

“Yeah, I shouldn’t need another nap for a little while. It seems like there’s so much I could have done in three days. Now, I missed out on all of it because I was lying in an ICU bed. I had a machine breathing for me, a tube peeing for me, and a tube eating for me.”

“At least now you have your whole life in front of you again,” Jeff said.

“Good point, you always help me refocus the right way, I love that about you,” I told Jefff. Secretly I was trying to ignore the lingering voice in the back of my head. It was asking, but how long is the rest of my whole life going to be?

I tried to push the dark cloud that was Autonomic SFN away. But clouds don’t push away, your hand goes straight through them,

“Yeah well, now that you had such a long nap there are no excuses for falling asleep during movies and TV shows, I am going to wake your ass up,” Jeff told me.  “We have to think about the important things in life, like your movie education.”

Trying to Stabilize Worsening Autonomic SFN

After I hung up with Jeff, I called my mom to let her know that I was finally awake.  She promised to come down to visit me later that evening.

I stayed in the ICU for another few days before being transferred to a regular floor. I stayed on the regular floor for two days, different doctors came by to talk to me.

“We think this was caused by a flare-up of your Autonomic SFN. The Autonomic SFN itself and the complications it causes seem to be getting worse fast. We have put some new protocols in place to try and slow them down. Your blood pressure runs dangerously low and your heart rate dangerously high.”

“So what do we do?” I asked. In my head, i was praying that they had an answer. I prayed that we hadn’t yet reached the stage where they would be saying “there’s really not much more we can do…”

New Treatments to Put in Place

“We have increased your Fludrocortisone dose and added a fourth dose of Midodrine.  In addition, we increased the rate of your hydration fluids. We will also be ordering you 500 ml bags of normal saline. Those are to keep on hand in case your blood pressure starts to drop under 85 systolic. Or if your heart rate goes over 120. Do you feel ready to go home?” he asked me.

“Yeah, I think so,” I told him.  “My heart rate has come back down to the 120s which is my normal. My blood pressure has been low 100s over the mid-60s which is also my normal.  The only issue is my breathing. When I take off my oxygen or turn it down to 2 L I feel like I’m suffocating.

Worsening Lung Function

“Pulmonology wrote that they want you on 3 Liters continuously 24/7. Your testing shows that you have something called atelectasis in your left lung. There are little air sacs in your lungs called alveoli. They are responsible for exchanging air so that we can breathe. Your alveoli in your left lung are collapsed and can’t exchange air. Your left diaphragm is also higher than your right because of this. This gives your left lung less room to hold air.  Both your lungs hold less air than a normal person’s lungs. This is because your autonomic SFN is also causing diaphragmatic weakness . You need the oxygen to constantly force air into your lungs. Your lungs aren’t strong enough to breathe for you. You need an oxygen machine forcing air into them.,” the doctor explained to me. “These are all typical findings of end-stage Autonomic SFN.”

“Thanks for explaining,” I said to the doctor

Fighting the Autonomic SFN Tooth and Nail

Then the nursies’ aide began to help me start packing up my belongings. That way I would be ready when the ambulance got there to discharge me home. I had at least bought myself a little more time with Jeff. Autonomic SFN wasn’t going to take me down that easily.