CIDP AND TURTLES

My eleven-year-old tells me Facebook is for old people. I just laugh. It’s true, I don’t use Instagram, TikTok or Snapchat. And Facebook is a lot of unrealistic posts of “perfect” pictures, but it is also a good way to connect with others. The first thing I did, before I even left Rush, was search for a group about CIDP. After I requested and was accepted, I hungrily read every post on there. I learned a few things.

1)     CIDP presents as a wide range of symptoms

2)     I was one of the lucky ones

3)     A turtle is the mascot

The turtle represents slow and steady. A variety of members in the group talked of being in wheelchairs, using walkers, falling, or stumbling frequently, and being on disability. Some people have been on infusions for years. You could have home health come do them at your home, you can do Sub-Q, which you do yourself. A lot of them constantly fought with insurance for their treatment and were even denied. A few had no support from family and friends who were ignorant or intolerant.

The majority of people have no idea what CIDP stands for or what it is. Guillén-Barre syndrome is the more common little sister of CIDP. What I have is the chronic form of that. One or two people in 100,000 are affected by it. It was certainly never on my radar before I got it.

After reading the posts in the FB group, I realized I was one of the lucky ones. I would find out in the coming months that I could live a relatively normal life. I showed very few symptoms between infusions and though my blood pressure would rise when I had to deal with insurance, I still managed to get all my treatments covered. My neurology team seemed to know what they were doing and had a plan for me. I definitely had the support of my family, friends, and employer. Despite the horrible disease I was diagnosed with, I still felt blessed.

 

 

ON WITH MY LIFE

So now I’m home.  Now what?  The doctor at Rush had decided that in order to keep me maintained with no relapse, I should receive IVig infusions every three weeks, for two days in a row.  I was relieved to discover I could do this at my local hospital and not have to drive a long distance.  It was 10 minutes away.

 

The three weeks passed, and I was scheduled for a Thursday and Friday in Outpatient Infusions on the 2^nd floor of the hospital.  We showed up, were taken to a room and waited. We were there maybe 5 minutes before someone from the pharmacy came in an informed us my infusions were not cleared by my insurance yet.  I was very upset.  This was all very new and scary and I had no idea what would happen if I could not receive my treatment.  They sent us home with the hope that it would be cleared up that day and I could come back the next. I have mentioned previously my love/hate relationship with insurance companies.  This would not be the only time it came down to the wire to get approval for my IVig.  Only so many are approved at one time and each time they had to be renewed there seemed to be hiccups. I was set up to be under the care of an RN at Illinois Neurological Institute since my doctor at Rush was not covered under my insurance. I even sent an impassioned letter to my insurance requesting my doctor at Rush to be approved, but their response was there plenty of good neurologists in network and just NO.  But INI  had no privileges at my local hospital so anything the RN ordered, my infusions, bloodwork, etc., needed to go through my PCP and approved. Lots of room for miscommunication and ball dropping.  Luckily everything got okayed and I went back the next day.

 

Care to hear exactly what happens at my infusions? It started out quite rocky. I was like some exotic animal they had never seen before. The infusion process for IVig is a little complicated until you get used to it. It uses special tubing, it gets infused at differing rates, and my vitals are taken every 30 minutes to make sure I am tolerating it.  I really have nothing but good things to say about all the nurses who have had to deal with me. There was a learning curve for sure.  And I am not a good stick. As in, my veins like to roll and hide. Makes starting an IV a bit of a challenge sometimes.  It usually took a couple tries. I can’t watch when they do it and if they take too many tries, I get woozy. Not the best patient.  Since the infusion were over two days, I would wear the IV home.  Which sounds horrible. And it was. Try sleeping peacefully with that on your arm, trying not to roll onto it.  But thinking about going back and looking for a good vein on the second day sounded worse to me. 

Each day I would show up they would have to access my vein before they would call the pharmacy to start mixing my meds. They would not start until then in case something when wrong and I didn’t end up being able to be infused. The medicine is no good to keep around.  And very expensive to waste. I eventually saw a bill for my infusion appointments.  For the two-day period I was there, the hospital billed my insurance around $34,000.  (I will revisit that little fact in a later post).  Bottom line, they weren’t mixing anything until I was ready to go.  Once accessed I received pre-meds that consisted of 650mg of Tylenol, Benadryl and Solumedrol, which is a steroid.  I also needed a bolus of saline to clear my veins before they started the big guns.  I would sometimes sit quite a while waiting for the pharmacy. Apparently mixing up this stuff is a major process. Slowly swirling it until it dissolves in the liquid. I think someone just stands there and slowly swirls it.  Once we got going, the drip starts quite slowly, increased every 30 minutes if my temp does not rise and my BP stays within range.  From start to finish, my day at the hospital ranged anywhere from 4-5 hours.  I will add here that as time has passed, the norm nowadays is mostly 4 hours, sometimes even sooner. 

 

I started back to work, and life continued.  I felt pretty much back to normal. It was miraculous.  The three weeks between infusions I stayed stable.  I felt very lucky. 

NOT MY IDEA OF A GOOD TIME IN CHICAGO

 NOT MY IDEA OF A GOOD TIME IN CHICAGO

I was grateful I had a room to myself. It was quite nice. A great view out the window. The food was not bad. Huge bathroom with a shower. I think it was Saturday that the neurologist came in and after putting me through the paces, declared she felt more strength when I pushed against her hands. She was very excited; therefore, I was too. I managed an actual shower and managed barely to wash my hair. This was something new! I looked bedraggled but I didn’t feel disgusting anymore. It was a long day, and I was anxious for Sunday to come so I could go home. 

I had started my period due to lack of my birth control pills and was given what felt like a twin mattress to put in my underwear. Apparently, they have not heard of maxi thins. I had a physical therapist come in and we walked the hall a bit with a belt tied to me in case I fell, which I did not. I was given something to help me poop. If they could fix THAT problem, I would consider them miracle workers. Never been regular a day in my entire life. I had annoying nausea going on, which I think was a side effect of the spinal tap. Headache too. That more than likely from the infusion liquid. Which they referred to as liquid gold. The bottles they were giving me each day were upwards of $17,000. And I was getting 3 days of it. Liquid gold indeed. The bill I would be getting from Rush was starting to concern me. What would my insurance cover? Not really things I wanted to be worrying about while I was trying to recover. I put it to the back of my mind and decided it would be what it would be, and we’d cross that bridge when we came to it. 

Sunday arrived and it was up in the air if I could go home. Another day of IVig might be beneficial. I hoped my strength was returning enough for me to leave. Family got there around 11am and it was decided shortly after I could go home! I was still dealing with horrible nausea. I never had morning sickness with my daughter, so this was something I had never experienced before. I can’t remember what time we left the hospital, but we had several hours to kill before the train left at 6 back to Macomb. We Uber-ed to Navy Pier and found a place for lunch. I was not hungry at all. At one point I made my way to the bathroom because I really thought I was gonna vomit. I was miserable. My husband wanted to go back to Rush to see what they could do but I refused. I called my doctor back in Macomb and was told I might need a blood patch to help my symptoms but for the time being, something called Nauzene at a drugstore might help. Luckily there is a Walgreens on every corner, and we managed the walk to one to purchase some. Can I just put this out there to the makers of Nauzene? Something you are taking to help you with feeling pukey should not taste disgusting. Chalky cherry was what I had to deal with. I barely got them down. They worked marginally but at least I managed to get down some cheese pizza. I had no food at all before then. We sat on benches and watched pigeons and people. I felt somewhat better lying down, so I imagine I looked like a homeless person trying to catch a nap. My arms looked like a druggies from so many IV pokes and placements. FINALLY, it came time to go to the train station and home. It was not a pleasant train ride. I was able to sprawl across 2 seats and spent most of the three-and-a-half-hour ride bent awkwardly at the waist attempting to lie still. Longest train ride of my life. 

We got home close to 10pm and our wonderful neighbors greeted us with a full-blown meal. My husband and daughter were delighted and dug right in. I was so grateful for the caring and support, even if I was not up for eating anything yet. 

I was SO happy to be home. But my journey with this horrible auto immune disease was far from over. 

I'VE GOT WHAT?!?!

I’VE GOT WHAT????

July 7, 2021 was one hell of a day. Once we were notified, I had an appointment with a neurologist at Rush University Center in Chicago, a flurry of activities began. Book train tickets, ask mom to come stay with our daughter, try to gather any medical records I could find about what brought me to where I was. It was a tough decision about leaving Anna at home, but we figured it would be a very boring day for her, not to mention having to be up so early to catch the 7am Amtrak.

So my mom came at a moments notice to stay the night so we could get up and out to catch the train. I remember crying at some point on the train, head up against the window like some bad chick flick. I was scared. And hopeful. And so very tired of it all. Arriving at 10:30am left us a few hours before our appointment. We hung out around Michigan Avenue near the art museum. Hotter than hell. Too unsteady to really walk anywhere. Crappy street vendor food.

The Uber to Rush came and we headed out. I have never been to a Chicago medical center and wasn’t sure what to expect. It was busy. And crowded. But the Neurology floor was quiet and serene. The waiting room was almost empty. They verified I had prepaid my mandatory $250 for the visit. Ouch.

Most of that day was a blur to be honest. I was taken in for the EMG where a personable funny guy administered one part of it.  I just laid still on a bed, and they stuck little patches all over me. It required nothing on my part. He sent mild electrical stimulation to test the nerve’s ability to carry electrical impulses. I had no clue how I was doing, and he offered nothing. The neurologist came in for the second part and conducted it herself. This part of the test involved a tiny needle she inserted in various places on my body. The needle acted like a tiny microphone. It was attached to a computer and fed back audio and visual. It almost sounded like when they do a sonogram. I still had no idea what my results were telling her.  The whole procedure was pretty painless. I know some people find it uncomfortable or are sore afterwards, but I have a pretty high threshold for pain.

So afterwards we sat in the waiting room and waited for her findings. It was starting to get close to time to go to the train station to make the train headed home so we were both quite antsy. After what seemed like forever, the doctor came out and told me most likely I had Chronic Inflammatory Demyelinating Polyneuropathy. This meant absolutely nothing to either of us. She nut-shelled it by saying basically my body was attacking the myelin sheath covering my nerves, thus slowing, or stopping the response. Which was why I was weak and couldn’t lift my arms very far or take big steps when I walked. I was not entirely sure what all this meant but it was the next sentence she said that rocked my world.

She HIGHLY suggested I be admitted to the hospital right away. The hospital 4 hours from home, 4 hours from my daughter who I had not spent one night away from since she was born. The hospital that most definitely was NOT covered under our insurance.

So, after checking that they did have a bed available for me, we determined going through the emergency room was the only way insurance would cover my stay. Time was ticking on the train departure too. I have no idea why, but I kept insisting my husband go home to be with our daughter and they would both come back the next day. I was put in a wheelchair, and we travelled the maze to get to the emergency department. A Chicago ER at 5pm on a Wednesday. Not a pretty place. Scary really. Finding a place to put me was a challenge. Easily 100 people milling around. My husband reluctantly took off and was gone maybe 15 minutes and was back. Train ticket or not, he couldn’t make himself leave me there. I was never so relieved in my life. My bluster was all gone. I didn’t want to be there alone.

So began our wait. We booked him a hotel close by so as soon as I was in a room, he could grab some shuteye and get the train home in the morning. Hours passed. Fourteen to be exact. The hotel never got used. The morning train was now out. We found a better place to sit ourselves, in a large atrium just off the emergency room. We watched it slowly get lighter as my husband would go check periodically to see where we were on the list. Real trauma emergencies kept bumping us back. Around 7am I was finally called. In a tiny room we waited some more.  Just because we were there didn’t mean I was actually being seen. They had me pee in a cup. My vitals were taken. I was finally told they were waiting for the attending ER doctor to be available. Once I was assessed and they were aware the neurologist wanted me admitted, suddenly there was no bed available. I wanted to cry. Would I have to do this all over again?? I made a phone call to the neurologist’s office, which by this time was open, and spoke to someone who told in fact there WAS a bed open, and my name was on it. Just as I hung up the ER people came in and said I was good to go. So mini heart attack for no reason.

I was finally taken to a room. My husband managed to find a train that would take him as far as Galesburg and my mom and daughter would pick him up there. After a tearful goodbye and a promise of returning the next day, he left.

So here I was miles from home when I had expected to be home the night before. Which meant I had no extra clothes; no personal care items and no birth control pills. I was in a hospital gown, but I had been awake 28 hours by this time. But could I sleep? No.

They decided I needed a spinal tap to doubly make sure I had CIDP so they could start appropriate treatment. I had never had a spinal tap. Had my daughter completely drug free. They came right to my room to do it since that was quicker then booking something somewhere else in the hospital. There were at least 4 people in there doing the procedure, a couple of them just observing. It was horrible. It felt unnatural and invasive and going off of NO sleep, I admit I cried. It was just the culmination of all I had been through.

It was decided yes, I did have CIDP and it would require 3 days of infusion of an immune globulin. Also known at IgG. So, what my treatment is called is IVig. There are only a few types available and the first one they tried that evening spiked a fever in me, so they had to slow the drip. It ran on into the night, vitals every 30 minutes. I didn’t really sleep well, amazingly enough. I recall looking at the clock at 2am and wondering why I just didn’t pass out. The automatic blood pressure cuff puffing every 30 minutes did not help. It was decided the next day they would try a different brand.

Friday my hubby returned with my daughter, and I was never happier to see clean underwear! And the two of them of course. I still hadn’t showered and felt gross. The neurologist and a team of others were in a lot, constantly testing my strength and a load of other things. I just have to say though, everyone I encountered at that hospital was amazing. The nurses, the interns, the cafeteria staff, the janitors, everyone was wonderful. I felt well cared for. What a change from what I had experienced so far. I knew what I had! I knew they were treating it! I felt relief.

After the fam left on the evening train Friday they would not be back till Sunday when I hopefully would be discharged.

HOW IT ALL STARTED

 HOW IT ALL STARTED

It was a Saturday in March. I was done with my evening shower. After combing out my hair, I reached down and ran my fingers over the rug in front of the sink to gather up all the loose hairs that land from combing and styling my hair every day. That night it felt different. My fingertips were numb. It was a weird sensation. The weirdness continued for several more days. It wasn’t super noticeable unless I was doing the hairy rug thing, but as someone who has seen a chiropractor since grade school, I decided maybe a trip there might fix things. 

My chiropractor had a couple theories on what might cause numb fingers, so two or three visits followed over the next few weeks. Unfortunately, not only did it not fix the numbness, I was also noticing some new strangeness. 

You know that back and forth motion you do after you shower and are drying off your back? That shimmy with the towel that starts up by your shoulders and ends down by your butt? I was struggling to make it all the way down. My arms refused to make the shimmy motion by the time I hit my mid back. It didn’t really happen all at once. It seemed to be getting difficult and worked up to just plain not being able to do it all.

Now what the hell was this about? To be honest, I didn’t even really think much of it. Not for awhile anyway. In kind of stayed in the back of my mind as “well that’s kinda weird but whatever”. It didn’t really affect my life in a huge way, other than my backside not being totally dry, lol. 

By the time the end of April came around though, I was starting to notice more serious things. It was harder to open jars. Plates seemed heavier. I was noticing stairs seemed to be a little harder to climb. I was starting to freak slightly. So I made an appointment with my PCP who I had only seen once due to my doctors kept leaving. I recall going to the appointment hoping the doctor would have an idea about what was going on. She did some strength tests and said she did notice some weakness in my arms and said she’d like to order an MRI but should probably start with just x-rays so insurance would cover the eventual MRI order. So began my hate-hate relationship with insurance and the healthcare system in general. 

The x-rays didn’t show anything that would explain my symptoms. Nor would the MRI they did at the end of May on my upper spine. 

Things were getting weirder. And scarier. I needed the handrail to pull myself up stairs. My drawer at the bank I work at was getting too heavy for me to put back into the vault slot because it was higher than my shoulders. I work with mostly 20-somethings and nothing makes you feel older than asking a youngun to lift a simple cash drawer for you. I didn’t dare bend my knees to get something low because there was no coming up. I am a co-leader of a Girl Scout troop and we took the girls to a local lake to canoe. I didn’t even consider going in the water. If I fell in I couldn’t even dog paddle to save myself. I wasn’t thinking and squatted to help push a girl off in the canoe and ended up sitting there like a duck until my co-leader paddled back to shore to help me stand up. I sat in a lawn chair and waited till they came back, considering the fact that if I even tried to stand I was close enough to the edge that if I stumbled and fell in, I would drown. 

June was a bad month. We scheduled a family vacation to Galena for a few days to get away and I just wanted to get through it with nothing terrible happening. Every week there seemed to be something new I couldn’t do. We got through it okay and even had fun. Did some hiking with goats and survived. I might have needed an arm for support, but we did pretty much everything we wanted to. 

After we got back it was late June by then. I had another MRI scheduled for my lower back this time but I knew it wouldn’t show anything helpful, and I was right. At this point there is a neurologist involved looking things over. There is a decision made I need an EMG. This stands for electromyography and assesses the health of muscles and the nerve cells that control them. I would need to go to a Peoria to do this and they scheduled one for me. The only problem was that it wasn’t until mid August. No amount of pleading could get me in any sooner. It was one of the tests they put a stop to during Covid so now that they were offering them again, the list was a mile long of people needing one. 

I was at the end of my rope. At this point I had taken a leave of absence at work. I no longer felt safe there. I couldn’t sit down without making sure there was something near to pull myself up with so the bathroom at work was out. Couldn’t get coin from my bottom drawer. Felt unsteady walking. At home I needed assistance standing up from the couch. I needed help getting dressed. I couldn’t even wash my own hair. My arms would not raise any further than my shoulders. My family was THERE for me. My 10 year old helped me put dishes away. She took over some of the laundry. I remember breaking down one night, miss drama queen, which I rarely am. Ran, well no, running was not an option anymore, more like shuffled to my room and slammed the door. My daughter followed me and in a parenting moment I am not proud of, I proceeded to lay it all out about how scared I was, how I had no idea what was going on or how much worse it was going to get. She patted me on the back and with such certainty, told me it would be fine. We would find out what was wrong and would fix it. My heart! And my husband took on more roles around the house. You don’t expect having to be helped getting dressed at my age. And having your hair washed for you? He didn’t even blink an eye. Did my daughter put away dishes and do laundry like I would? Did my hubby wash, dry, comb and mousse my hair like I would? No and no. It was frustrating but I was grateful. 

July 2nd I met with a nurse practitioner in my PCP’s office and ended up in tears, trying to convince her I needed this EMG now, not 6 weeks from now. I was convinced if I had to wait until August, I would need to be carried there because I was sure I was not far from being unable to walk anymore. She was no help. An EMG is not considered an emergency test and there is no cutting in line. 

Our neighbors are wonderful people we are very blessed to live next door to, and one evening at our mutual friend’s house they were shocked to see I needed pulled up from the chair and started a personal quest to help me. Their daughter called me and having had to deal with her own medical issues over the years, told me my best bet was to go plant myself in a big city emergency room and insist to be seen. 

My BIL has a friend who works in pediatric neurology at Rush University Medical Center and had her call me one night. She listened to my symptoms and all my problems and said she would talk to someone in neurology there and see if they could get me in. It wasn’t a hospital covered under our insurance but by then we didn’t even care. We just wanted answers. True to her word, I got a phone call a few days later and had appointment at Rush July 7th. It would be a day that would change my life.