Cyborg Status: Confirmed

So uhhh I have my port installed. It was …an adventure. Lemme tell you all about it!

When last we left our intrepid heroine, she had a really nasty reaction to the adhesives on her PICC line dressing. We moved to a different type of dressing that didn’t adhere for shit. The day I was done with the 14 day initial run, my awesome home infusion nurse came by to change the bandaging. She recommended we get the PICC pulled ASAP so I could get the dang adhesives off my skin.

It took a little convincing to get the nurse to refer me for a port; she was concerned that if I had a reaction to the adhesive as it was, having a port would be just as bad. I was concerned about not having tubes sticking out of me at all times; at least the port would give me a 2 week break every month. We eventually secured the referral, and my surgery was scheduled for 12:30 on December 27th, with a separate appointment that morning at 8:30 to get the PICC removed. When I told my home infusion nurse, she arranged to come by that evening (after her company holiday party, even!) to pull the PICC so we could get the wrappings off me and let my skin recover a bit. We love Deena, she’s rad. It was surreal to see her pull the line…it kind just kept coming out, like a magician’s scarf of surgery and horror. It was MUCH longer than I’d thought. And it was friggin’ DELIGHTFUL to have all that out/off. And the shower I was finally able to take was goddamned MAGICAL.

I received a call from St. Vincent’s hospital the next day – the surgeon I’d been scheduled with wasn’t actually going to work that week, could I go to Providence Portland instead? At 9:30am? Sure, no problem. The next day, they called again – sorry, could we make it same time, but back at St. Vincent’s after all? No problem. J worked that day, so I asked my sister-in-law-of awesomeness Jeanette to be my ride.

And then I made a terrible mistake.

OK so I’m a smart person, and with intelligence comes curiosity, and I wanted to know exactly what I was in for with this port. I watched a video of a woman demonstrrating how to access a port; no big deal, kind of persnickety with regards to keeping everything absolutely sterile, but whatevs. And thennnnnnnnnnn I watched a video of a port install surgery. OK, I am not really squicked out by surgery, and I found the whole thing fascinating until the phrase “BLUNT DISSECTION” came up, and the surgeon LITERALLY TORE A HOLE IN THE PERSON BY JAMMING HIS PINKY IN THE INCISION AND WIGGLING IT AROUND. HOLY FUCKBALLS. My brain went NOPE, FUCK THAT, FUCK THIS, AND FUCK YOU FOR SCHEDULING THIS MOTHEFUCKING MEDIEVAL-ASS SHIT. Intellectually I knew it was fine – MORE than fine, because I was gonna be hopped up on Versed and remember NONE of it and get some good drugs afterwards – but the actual crux of the mistake had been watching this video righe before bedtime, so my brain just did donuts in the parking lot for awhile.

The morning of the surgery, I got a call from the hospital – would I mind coming in early? It was 8:30 and I was scheduled to check-in at 9:30, so I was a bit confused, but I told them I’d be there as soon as I could. We parked too far away and I was a sweaty mess when we arrived at check-in, where I was told…I was scheduled at the OTHER hospital, still. I explained the conversations, they luckily sorted it out. I went upstairs, put the goofy little gown on, and waited. The nurses came in, partly to take vitals and start an IV and make sure I wasn’t pregnant, and partly to play peek-a-boo with my adorable niece. The doctor came in to introduce himself and ask if I had questions. he was delighted that I referred to the Huber needle I’d be using to access the port as “the crooked needle”. I was delighted by his use of the phrase “twilight sleep” to describe the conscious sedation I’d be under. They’d give me benadryl in my line along with the sedatives and a dose of antibiotic, he said, hopefully I’d just sleep through the whole thing.

I remember them wheeling me into the surgery, and seeing the GINORMOUS fucking monitor he’d be using to make sure he was placing the port properly, with x-ray. It was weird to see my name there. The several nurses had their A-Game banter going, and we were having a great time explaining names and tattoos to each other. One nurse thought I had an accent, and asked how long I’d been a Yankee. “My whole life?” A male nurse/technician/whathaveya scrubbed me down with a blue solution, making me the world’s least convincing Smurf. I was freaking cold, despite the warmed blanket they’d put over me. The IV nurse told me he was putting the antibiotics in my line now, and I felt the cold sting. he told me he was starting the sedative now, and then I…

I do not remember a thing after that.

I woke up with a very large image of my x-ray on the monitor, showing the line running into my artery. I was shivering. They wheeled me back to the recovery room, and I slept a bit. Jeanette had taken my niece Jewel to get some lunch or something. They brought me a turkey sandwich, which I devoured. They had put the dressing on a bit shitty; I couldn’t raise my head all the way, or turn my head much because of the way my skin was taped up. It itched a bit. I was given discharge instructions and no pain killers (“Just take some Tylenol”). My sister came back, I got dressed, she took me home, and I slept some more.

The next morning I was aware something was up. I was naughty and pulled the dressing off a bit to allow myself the ability to raise my fucking head, and where the dressing had been tugging was now an angry red wound. I was very itchy. All over my chest. Angry red hives, all over my chest, up my neck, and across my chin. Tylenol, it turned out, did not fucking suffice, so I took some of the hydrocodone I had leftover from breaking my ankle that the doctor hadn’t wanted to give me either because ibuprofin should be fine. YOU JUST TORE A HOLE IN ME BIG ENOUGH FOR A 1 INCH SQUARE MEDICAL DEVICE AND ALSO CUT MY NECK TO SHOVE WIRES INTO MY VEINS – NEWSFUCKINGFLASH TYLENOL AND IBUPROFIN ARE NOT SUFFICIENT FOR THIS. BLUNT. DISSECTION. BITCHES. I slept some more. I woke up to even angrier hives, annnnnnnnnd my eye was a swollen bag of fluid.


So uh…yeah. I was having an allergic reaction to something. It couldn’t be the dressing itself, since the hives were everywhere. It got worse and itchier over the night. I had a different IV home nurse visit that day, to change the bandage since they’d thoughtfully left a needle in the port for me to start the infusions, but I’d bled a bit so there was gauze under the dressing which needed to go. She was very concerned about the reaction, namely that it might be a reaction to the port itself. My delightful friend Lizzie was coming over that day with delicious food for me, and she kindly picked up some Benadryl on her way in. Luckily I had a doctor’s appointment already scheduled for the next day, for an unrelated issue and holy FUCK will I rant about that in a bit. I took Benadryl, ate delicious home made food, and slept a lot.

My PCP is a cool guy, and didn’t mind dealing with the reaction as well as the initial problem we were scheduled to deal with. He suggested two more antihistamines in addition to the Benadryl, and to contact the hospital if it didn’t clear up by Tuesday (Monday was New Year’s). Luckily it did, mostly. I think the problem was the smurf shit they scrubbed me down with, since the hives pretty much appeared everywhere they used it. I have no explanation for my eye becoming a blister, tho.

We’ve now completed the second round of infusions, the port works beautifully, and we pulled the needle out last night. I am once again dressing-free for two weeks. The scar is already nearly healed up, and the itching has almost entirely subsided now that I’ve got no more adhesives on me. I can feel the port under my skin, and it’s weird.

Now I am become cyborg. beep bloop bleep.

Stuff Keeps Happening

So! Today is the last day of the first run of Radicava. I’m confident at this point that any side effects are tolerable, and I want to continue. The only potential things I’ve noticed is that there MAY be an uptick in the frequency/severity of my headaches, but it’s nothing I can’t tolerate, and there’s been a few times when there’s been a weird panick-attacky feeling, where my heart is beating in my throat, but that always goes away. Labs will be drawn to make sure I’m not experiencing anything serious, of course, but my own internal feeling is that everything’s GO for continuing.

Whether this is even doing any good, I won’t know until March, the next Clinic Day. But it’s not hurting anything, so we’ll keep it going. This also means I’ve asked to go ahead with the port install. It will be a vast relief to no longer have these tubes coming out of my arm that need babying, not least of which is because the adhesives that protect the PICC line itch like a MOFO.

Since my fall last Tuesday, I’ve noticed my hip hurting a bit, one spot particularly. I kept expecting a bruise to form, but it never did. Two nights ago, I was pushing on the spot, to figure out exactly where it hurt, and my fingers found a hard lump that rolled around a bit under my skin at the joint. And I remembered wayyyyyyyyyyy back at the beginning of my Godzilla Disease diagnosis attempts, how we initially thought the problem was in my hip, and then I got an MRI, and the MRI showed a small tumor thing in my hip meat. It was deemed medically uninteresting, though, and ruled out as the cause of my woes. I was told that it had probably been there, like, forever, and wouldn’t be a problem, but maybe keep an eye one things and recheck it in a few years.

I guess, yeah, it’s been a few years, so it’s time to get the dang thing rechecked. So THAT will be fun, as I can’t really get up into MRI machines these days. I’ll make an appointment soon. One more damn thing. I’m sure it’s fine, but dang, man.

I’ll let you all know when I get the port installed. 😀

Fall-ow up Post

Tuesday was not a good day.

It was the urology follow up, and the day began with a clear reminder of why the appointment was needed, followed by massively uncomfortable prep, and then the Lyft ride to the hospital. I was half an hour early, thankyewverymuch. Once inside, I don’t even know exactly what happened, only that my walker was moving faster than I was, and I didn’t have the strength to pull it back under me, and suddenly I was kneeling on the ground. Two older men noticed my plight, and asked if I needed help.

“Yes please?” The question mark was because I had serious doubts these two were going to be able to get me off the ground. Doubts well founded. They were not. A third, younger man assisted and we finally got me seated on the walker. They all left with my profuse, if a little breathless thanks. I wasn’t hurt at all. After catching my breath, I stood a little shakily (yay adrenaline!) and made my way to the urologist, who has a very heavy office door. A gentleman inside noticed I was having a hard time pushing the door open, and opened it for me.

…Only I was leaning on the door knob at the time. With my support pulled away from me, I lost my balance and landed on my back in the hallway.

Please ask if someone needs help before ‘accommodating’ them? This is the first time it’s actually caused a fall, but I’ve NEARLY been pushed or pulled off balance many times by someone trying to help. Please offer to help, but wait until I am aware of you and ready before doing the thing. Thanks.

I was surrounded by noisy, concerned people, including the really bitchy admin from last time who was actually quite sweet this time. One of the people who helped me up this time was one of the same older gentlemen from before. “If I’d known you were coming here, I’d have waited with you.” We got me seated again, and I was left to rest in the lobby, blissfully left to my own devices once everyone was sure I was OK.

The appointment itself was…uncomfortable. All sorts of probes and wires were inserted or otherwise attached, then my bladder was filled and i was instructed to bear down. Nothing leaked, which in retrospect was hardly a surprise because it’s only when I get UP that problems happen, but I didn’t have the presence of mind to point that out. The wires were disconnected, the doctor came in and looked at my results, and…instructed me to pee more often.

That’s it.

That’s her whole plan. Just get up every three hours and pee, maybe you won’t leak. Oh and keep doing those Kegels with muscles you no longer possess. She clearly does not speak ALS.

I left angry and frustrated, and took a Lyft home, only to find Amazon had delivered a package while I was gone. Right in front of my door. My case of Monster. My very heavy 24 count case of 20 ounce cans, right in front of the door. Annnnnnnnnnnd in trying to move it enough to get inside the door, I uh..yeah I fell again. Badly this time. Pain shot through my previously damaged knee and my freakin’ ankle that was broken. My mom, alerted by the noise of the fall (but not the swearing, oddly enough, she didn’t hear me yell), came out to try to help, but there wasn’t anything she could really do. Once I caught my breath and got my legs out from under me, I was able to shove the Amazon box out of my way and crawl inside. It was good to have my mom there this time; even though she could in no way help me up, she was able to get the walker out of my way and put our stepstool by my bed so I could use it to haul myself up.

Thank God I had taken an ativan that morning before leaving, or I’d have been a blubbery mess. As it was, I took a well deserved nap and was pretty sadbrained the rest of the day. Yesterday I was pretty dang sore all over and worked from home. Landing on your ass three times in 2 hours takes it out of a girl.

BUT! Yesterday was also the completion of my first week of Radicava! I think it’s going well. It maaaaaaayyyyyyyy be exacerbating my headaches though; I’m keeping an eye on it. It’s been an eventful and stressy week, so hopefully the coming one is kinder and a more useful baseline. I won’t know really if the meds are helping anything until March, the next Clinic day. I’m committed to keeping it going at least until then.

So now you’re up to date, and I’m gonna go play on the internet. <3

A Rad Update

Step 1. Gather the required materials.

One box of Radicava, which contains two 100ml bags. Two saline flush syringes. One package of IV tubing. At least two alcohol prep pads. One IV pole.

If we were taking labs, or redressing the PICC line, there would be so many more components. This, though, is the minimum. There will come a time when this is routine and second nature, but for now, everything is still double and triple checked. We lay things out like we’re preparing for surgery. It’s not far from the truth.

Step 2. Wash your hands.

There’s hand sanitizer, which also gets liberal use, but there’s no substitute for scrubbing your hands with soap and hot water. I find it almost impossible to wash my hands well these days, and drying is a nightmare, so I typically opt for the sanitizer only. If your hands work, though, go wash them.

Step 3. Open all the packaging.

The box of Radicava contains two trays of 100 ml bags of medicine, each with a silica bag and a little pink oxygen indicator. We peel back the lids on the bags, which each have a plastic seal over the input valve. We leave those intact until the last minute. We unwrap the two saline syringes, but leave the caps on. We open the first alcohol prep pad and set it aside. We unwrap the IV line and hang it over the IV pole. OK. I think we’re ready.

I’m appalled every day at how much waste this generates.

Step 4. Prep the line and bag.

We close the IV line, either through the clamp or dial, depending on the tubing style. Removing the bag’s seal and uncapping the IV line’s spike, we push the spike into the gray seal on the bag as straight as possible so as not to split the bag. It takes a surprising amount of force to do this. Because the line is clamped, nothing happens yet. We squeeze the tube’s chamber to fill it about half full of medicine. Carefully, slowly, we unclamp the line and allow the medicine to flow through the tube, stopping it just before it spills out of the end. We clamp it off and set it aside, dangling harmlessly from the IV pole for now.

Step 5. Sanitize the input valve on the PICC line.

The alcohol pad is scrubbed over the PICC’s blue input valve for 15 seconds. It doesn’t have a cap, so it’s out in the open all the time, well, tucked up under the sock against my skin, and must be cleaned carefully. We let it air dry.

Step 6. Saline Flush to clear the line.

Uncapping the saline syringe, the plunger is pushed juuuuuuust a little bit to push the air out. Or, you know, accidentally create a beautiful arc of saline in the air if you push too hard. The syringe is then coupled to the blue port on my PICC, twisted in place to secure it, and the saline injected a push at a time, to the rhythm of a heartbeat. Bublump. Bublump. On the second push, I feel the cold liquid in my vein, and a moment later, I smell and taste the saline in the back of my throat. It’s a hospital smell. The rest of the syringe is injected, decoupled, and disposed of in the biohazard bin.

Step 7. Insert tubing into PICC and begin infusion.

The IV tube is pushed into the blue PICC port, the little plastic collar screwed tight to secure the connection. The tube is unclamped or undialed, and the Radicava begins to flow into my vein. It feels like nothing. It’s not cold, like the saline, it has no taste, no burning like some antiseptics and anisthetics. There is no sensation at all, and the only reason I know I’m getting medicine is to watch the IV chamber steadily drip drip drip drip…

Step 8. Swap bags when the first one is empty.

The tricky part to this is allowing all of the medicine to drain out of the bag without letting the chamber empty, which will allow air down the line. Air in the line is bad. I have remedy available, if that should happen; it’s easy enough to use a saline syringe at the bottom of the line to force liquid back up into the chamber and clear air out. But it’s best to not let that happen. We swap the bags and sit back to wait some more. This is supposed to be a 1 hour infusion, but it’s taking closer to 2. A lot of the reason for this is an extension of the PICC line we added so that I can reach the ports myself, which bottlenecks the flow AND adds extra distance for the medicine to have to travel.

I can do a lot of this myself, except the coupling of the IV line to the PICC. Both ends are very floppy and you can’t touch the ends without having to start over and resterilize. Doing all these things, though, cause my hands to cramp up a lot and then be completely useless for the rest of the day, so I happily leave it to others when I can. J’s been doing it the last couple days, which is marvelously helpful.

Step 9. Bleed the line carefully.

Once the bag is empty, we play a game of chicken with the medicine, allowing it to drain down the line until the air is allllllllllllllmost to the PICC line. We don’t want air in my line, obviously, but we also want every last drop of the insanely expensive medicine in my veins and not in the trash. Once the line’s drained as far as we dare, the line is clamped, decoupled, and the entire bag and IV line is discarded. More trash.

Step 10. Saline flush.

Hands are washed and sanitized again. After another scrub of the port, the second saline syringe is rid of air pockets, attached to the blue port, and screwed in. More heartbeat push, more cold, more hospital smell and taste. Yummy. The syringe goes in the biohazard bin, even though it never really touched my fluids. Quite the opposite.

Step 11. Redress and cleanup.

We’re done! Everything is thrown out, the IV pole is collapsed and stowed, and the PICC line is coiled up and tucked under the itchy fishnet sock around my arm keeping everything covered.


Today will be day 5. Fortunately, it is not time sensitive in application like an antibiotic might be, so as long as I do it sometime on that day, it’s fine, it doesn’t need strict scheduling. Last night’s dose began around 8; tonight will be closer to 10 or 11, my first ever dose was 9 AM. For the second round, I have to do 10 infusions over 14 days, and I can also pick and choose dates and times. If I want to do Monday through Friday and skip weekends, I can. If I want to do all 10 days up front and get it done, I can. It’s up to me.

The main problem I’ve had so far has been dealing with the PICC, in that it’s inconvenient as hell (I miss showering – I can only shower if I take these huge complicated precautions to wrap it all up watertight and I just can’t pull it off on my own) and itchy AF. The bandage tape is itchy, the sock is itchy, the lines are tickly and like to snake their way out of the sock and say hi to the world from under my sleeve. It’s convenient as hell for not having to have an IV poked in every single time, but everything else about it sucks. I’ve already decided to get the port-a-cath installed once I’m done with this round.

I’m tolerating the drug just fine. I *think* it’s exacerbating my headaches, but nothing unbearable, and it may be causing night sweats, but I have to research that to find out if it’s even a thing that might happen. Otherwise I’m doing good. We’ll find out in March if it’s affected my decline in the least bit.

So that’s everything so far about radicava, darlings. It’s going well. I’m still terribly excited about all of this.

The Good Kind of Progress

I met yesterday with my PT specialists and a sales rep to pick out my new wheels. I’m getting one of these!

new wheels!

I opted for the midwheel drive, it’s got a smaller footprint and pivots around corners rather than steering like a car, so it’s easier to drive. I’m still totally gonna run into things. Count on it. I got the USB charging port (instant popularity!) and a cup holder and..yah! I also opted for the seat elevation feature that will let me look people in the face when sitting in the chair, even though insurance is proooooobably not going to cover it. But I think being able to see over tall counters is important.

Tomorrow I start Radicava infusions. The nurse is coming over around 11 to teach me how to do it, and then Friday s/he’ll come back to watch me do it and make sure I learned properly. They’ll also change out the dressing tomorrow, for which I am grateful. It’s itchy.

This has been your baby update.


The latest word on the street in ALS Land is Radicava.

It’s a new, FDA approved treatment, and we’re all pretty dang excited about it because there’s been nothing new AT ALL in 30 years. All we have is riluzole (brand name Rilutek) which you take twice a day and maaaaaaaaaybe gives you another six months or so? Or maybe destroys your liver? Other than that, the only treatment is really an attempt to mitigate some of the symptoms, like the muscle twitches (‘fasciculations’) and cramps, your muscles locking in place (spasticity), the complete fatigue because everything is a lot harder when half the muscles in your legs are gone, depression and anxiety – because it’s not really the five stages of grief so much as the five constantly cycling whirlwind moods of grief. Sometimes I hit them all on the same day.

Radicava (generic name edaravone) isn’t a miracle drug, by any means. It’s completely ineffective for a lot of people, and at best gives just a 33% reduction in the rate of decline. It has a lot of issues, of course, it can cause problems in people with asthma, and’s expensive as all hell. I’ll get to that in a moment. For now, here’s the basics, if you want to read some more, and here are some of the naysayings about it.

Me, though, I wanted to try it from the get-go. I’ve been abundantly clear (or so I thought) that I am willing to try any new treatment, just to be doing something, to provide a little more data for future research, if nothing else. Every Clinic Day, we asked about the latest updates, the most recent news, when is it available. And so I was frankly…crushed, and a bit angry when I found out at last Clinic that people have already been trying it and I wasn’t contacted. That anger increased when they seemed surprised that I was interested in trying it. could I have been more clear?

Anger turned to rage when I was told that insurance would not pay for it. Because..once again..I have had ALS for too long. Because statistically, I should be dying any minute now. The average life expectancy is three to five years, and I hit three years in April. Never mind in actuality I’m still VERY MUCH alive and not going anywhere soon, thenkyouverymuch. And it wasn’t the pharmaceutical company denying me, it was the insurance company. I wasn’t worth the cost. If I’m statistically almost dead already, it’s not cost effective for them to pay for this. You’ll recall I said it was expensive – let me show you how expensive. Radicava is administered by infusion. Each infusion lasts for about one hour and administers a 60mg dose. The cost for each infusion is as follows:

The medication itself: $1238.80
The medication to flush the IV/PICC/port line into your vein: $180
If you do in-home infusion, the nurse’s time costs $251.20 for the first 2 hrs, $86.10 each hour after if needed
If you go to an infusion clinic, the cost for nursing is $107.10 for the first 2 hours, $50 after.

Now, since it’s not easy to get anywhere and do anything with ALS, we’ll go with in-home infusions, so that is $1,670. Per dose. How many doses do you need? Well, you start treatment with 14 consecutive days of infusions. That’s $23,380. Then you take 14 days off. Then you do 10 consecutive doses over the next 14 days. Then 2 weeks off. Then 10 over 14. For the rest of your life. It’s over twice what I make a year, to use this drug. As much as I hate it, I get why insurance would consider this a dumb investment. I hate that that is even a consideration in whether or not I have access to this.

Knowing that I was going to get denied, I asked to try anyway. I filled out a form giving the infusion clinic access to my medical records, on the off chance they could use this to leverage my insurance company to pony up some dollars to help a dying girl out. Monday I got a call from Searchlight, the company coordinating the treatment, and they said that typically my insurance covered this treatment after a 5% copay. Luckily, she said, once I hit my out of pocket max, it’s all covered. Did I have any questions?

“Yes,” I said. “…Does this mean that I actually am going to be covered for this?”

“Well, we’ll be sending your information along to your doctor provider, but it looks like we’re good to go.”

I dared to let myself hope. Just for a moment. I went out with J and had celebratory sushi, but said nothing to anyone else (except my mom, who was here when I got the call) just in case it was a misunderstanding.

It wasn’t.

Wednesday I got a call from the nurse to discuss the actual cost, how much I was responsible for, and to start making appointments. She gave me the above price list, and we discussed would I like a PICC line or a port? Since this was pretty much going to be a regular thing, infusions by regular IV aren’t an option – imagine having to get your vein stabbed every day for an hour long IV drip. Folks on dialysis, infusions, or chemotherapy usually opt for either a PICC line or an implantable port. A PICC (peripherally inserted central catheter) line goes in your upper arm, and then into a central vein in your chest. An implantable port, or port-a-cath, gets implanted under the skin in your chest and also snakes into a central vein. PICC lines are technically good for a month, though if you treat it carefully, it can last much longer. A port-a-cath is considered a permanent implant. PICC lines are much simpler to install, it’s just a complicated IV insert with a chest x-ray to make sure it’s done right, but a port requires at least conscious sedation. A PICC line needs upkeep once installed; it’s basically two little ‘pigtails’ as she called it, sticking out of your skin and held in place by tape. You can’t get it wet, and have to wear some kind of sleeve to keep it from getting caught on things. A port is just a little bump under your skin, no maintenance required. PICCs are easy to remove if you change your mind. You’re kind of stuck with a port for the rest of your days.

So yesterday I had a PICC line installed.

If I tolerate the meds, we’ll install a port.

This is happening. Even if it does me no good at all, even if it actually makes things worse, we have tried. I am a data point. I did something. I did everything I could. I should start infusions next week.

I can’t wait to keep you guys posted.