Round 2, the IVIG works, but things are snowballing

The second dose of the second round of IVIG was done yesterday. I don’t sleep on the nights after an infusion so I’m exhausted today. I get tachycardia all night and flu like symptoms for the next day or two after. I do two doses separated by a day in one week, followed by another two the next week, then two weeks off. Each infusion takes about 5 hours.

The IVIG works. The burning pain that started out very mild back in June and July, that slowly built up to a severe 7 or 8 out of 10 on the pain scale during October and November, has almost entirely gone away. It’s incredible. I barely notice it now. And it feels different, maybe that’s the nerves healing instead of being damaged? It’s so incredible how quickly you forget what horrible pain feels like. In my mind I know it was horrible, I remember laying at home all day curled up in a ball sobbing sometimes because it just wouldn’t stop, and no drug stops that type of neurological pain. I remember crying in the ER pleading for them to do something. But that’s basically gone, and now I just have these oddly rational memories of it being really-really bad, but can’t feel it.

My orthostatic measurements are improving too. Back in November before the first round of IVIG my standing heart rate had worsened to the point where it was ranging between 130 bpm to at times an incredible 160 bpm. It’s now ranging anywhere from 110 to 130. My standing diastolic blood pressure prior to the first round was typically over 100 mmHg, usually something like 105 mmHg. Now it seems to be always measuring below 100 mmHg, around 95 mmHg usually. These are huge shifts. They are still very bad. But they’re huge shifts in the right direction.

I’ve started to feel a bit better on my feet, to the point where for the first time in many months I’ve been able to take a few short 10 minute walks.

I am still severely fatigued and have a lot of brain fog, but I expect that will take the longest time to start improving.

On one of these recent 10 minute walks I went to a coffee shop. Someone next to me was talking graph programming, graph coloring and traversal, etc. on the phone with someone else. I’m such a huge nerd, it really reminded me of the passion I have for networking and how much I’d like to get back to work at some point. It’s hard to believe I’ve been sick now for 20 months. (Most people don’t realize it’s that long, because I’ve only been on STD for 12 months.)

Based on the results so far I have no doubt in my mind now if I were able to find a viable way to continue the IVIG I would continue to improve.

But nobody has yet presented us with the help required to make that happen.

Doctors needed

I am supposed to get regular tests to monitor my liver and kidney function. I’ve not had those in two months because I still don’t have a PCP. Here in Portland, ME there’s a 3 month wait list for PCPs at all locations, forget about all the specialist help I need. It’s absurd. I had a number of doctors in Seattle that were able to intervene with their expertise in any area that was necessary during my ongoing treatment, I have none of those now out here.

  1. An awesome primary care doctor who was wonderful in every which way, followed up on everything, and gave me all the support I needed to deal with this challenging illness. When you have a complex and difficult to understand illness like mine it takes a while to find a good doctor who will learn about it like she did. I miss her very much.
  2. An awesome immunologist/infectious disease specialist, who deals in complex cases and was the one who worked out what was wrong with me. I’d be no where if we hadn’t been lucky enough to see her, she is a god send to people with illness like mine that take some working up to diagnose.
  3. An awesome neurologist who could monitor my neurological changes, understood what was going on.
  4. An endocrinologist who was due to do a bunch of stimulation tests to make sure all my endocrine functions were working fine — tests which we had to cancel due to the move and still haven’t be done.
  5. A cool cardiologist who kept track of my heart condition through the stresses that the illness is putting on it, a specialist that would be very helpful to have right now because one of the things happening after the IVIG infusions appears to be an arrhythmia. Back home, he would be able to have me come in the day before my next round stick on a ambulatory cardiac monitor to wear, and we’d review the results after the round of infusions and know if it was a serious side effect to worry about or not. I have no way to get that here for months.
  6. A sleep doctor, for which there isn’t much to be done now but surgery.
  7. Surgeons, for surgery that now I’m not getting:

Before we left Seattle I had a surgery scheduled to correct the airflow in my nose, turns out of have a pretty insanely deviated septum and enlarged turbinate. Fixing this should help improve my sleep quality since I have mild sleep apnea. Improving sleep quality is always a good thing for a sick person. We had to cancel that of course due to needing to move because the IVIG was denied. So my sleep quality wasn’t fixed due to this situation.

I also was scheduled to start the work necessary for jaw surgery (something called a maxillomandibular advancement) to advance my jaws and pull my tongue forward, again for the sleep apnea, and because for some reason my jaws have become so misaligned I’m biting my tongue (and it constantly hurts). We had to cancel that too of course. It takes a looong time to set that one up, it’s basically never going to happen now unless we go home in the next couple of months and start up the process again. The only problem is the surgery is pretty serious, it’s not advisable for me with my condition to get it without being on IVIG, so how do I do all the orthodontic pre-work if it turns out when it comes time for the surgery I don’t know if I’m going to be on the IVIG? You can’t undo the pre-work! You need to know ahead of time that you’ll be covered for the IVIG through the surgery.

The Snowball

Vi is getting sick. We’re not sure what it is. We don’t have a primary care for her for the same reasons above. So she went to the urgent care, where they suspected Lyme and have run a Lyme test. God I hope it’s not Lyme. I’ve learned way too much about Lyme over the past year and it’s a horrible-horrible fucking thing that is so badly misunderstood and stigmatized by most people.

Vi has suspected fibromyalgia, that has been diagnosed previously by a rheumatologist and neurologist. It can be a serious condition, for her as with a lot of people, it’s been mostly a background thing that hasn’t really caused a major problem. But now she’s having worsening symptoms, pain, and fatigue. She’s been warned about the stress making things worse, and now the stress put on her with this move, having to take care of me full time without any support, and just the emotional toll of not having friends around (which can’t be understated), seems to be making her sick.

Denying my IVIG coverage, forcing us to move so I could get this simple fricken drug, so I could experience what it’s like not to feel like I’m covered in napalm and be able to stand up and walk again, is directly linked to making my wife stressed and sick.

This situation has begun to snowball.

Option 1:

If we go home, we’ll have support, we’ll have doctors, we won’t be alone. But I won’t have IVIG and I’ll continue to get sick, those 10 minute walks won’t ever grow into 20 or 30 or 60 minute walks, and I can forget about hoping to return to work. Maybe the horrible burning pain returns and I sink into regret for making the decision, crying again in emergency rooms as doctors tell me they can do nothing because my health plan won’t cover the IVIG, just as they did in November.

But it might prevent Vi, who I love so dearly, and who after all is my full time care taker from getting sick.

Option 2:

Medicare doesn’t start until July 2020. Realistically that’s how long we have to decide to remain exiled out here, unless someone gives us the help needed to get covered for IVIG so we can go back home.

If we stay, maybe I keep getting better on the IVIG. (But then again maybe I die of a heart attack because I don’t have a fricken cardiologist on my team for the next four months who can throw a simple holter monitor on me, like I do have back home. I certainly don’t get the sleep apnea fixed.)

Maybe even though we stay and continue the IVIG, it’s not enough to get me back to work, maybe all I get from it is a quality of life improvement, I can live pain free, and go for walks, and do basic household stuff to take care of myself. Don’t get me wrong, that is a big deal. Going from being bed bound in pain, completely dependent on someone else, to being able to stand up in the kitchen and make yourself a meal is night and day. If you haven’t been there it’s impossible to relate.

But maybe Vi continues to get really sick. Then who is around to help? That seems like a very bad scenario.

And if I do get well enough to return to work, I still haven’t had anyone explain to me how that will even possible without a return to the west coast, and a return to the west coast wouldn’t be possible without being covered for IVIG on Juniper’s health plan.

Impossible to understand

I still can’t understand the lack of help. I get the world is a shitty, horrible place, and there’s a lot of people suffering in the United States. But I’m lucky enough to be an engineer at a $10 billion Silicon Valley tech company who was covered by their health plan. That health plan had no problems paying around $80,000 for the various diagnostics and emergency room visits and hospital stays leading up to the diagnosis and request for IVIG. But then when it came to that request for the treatment that can stop it all that, the treatment that is working and making me better as we speak. A request that was made by some of the best doctors in the country who treat that specific condition. That health plan parsed it like a firewall, against it’s very short policy, dropped it into the default deny, pointed the finger at my employer, and seemingly didn’t even bother to read the appeal letters from my doctors. They didn’t ask any questions, they didn’t talk to my doctors. Just like that, denied treatment and left to remain severely disabled and in pain without any more thought or care.

This isn’t what anyone would expect. When we have a $10-billion-Silicon-Valley-tech-company health plan, we’re not supposed to be left in torturous pain, crippled and unable to stand and walk, when there’s a simple, safe, and effective drug that reverses that. Sure we might have co-pays, and deductibles, and so on. And I’ve probably spent about $25,000 (excluding this recent move to the east coast) on things that weren’t covered. Or visits to specialists in other states and so on. That’s expected. But not covering the drug that fixes a person. How can this be.

H̶o̶m̶e̶ Alone for the Holidays

Recent inactivity has been due to our move. You see, much like Kris described, Connecticut sucked. We were a half hour away from anything, even the hospital. Our upstairs neighbors loved to do cardio between 10pm and midnight and when they weren’t, they were screaming at each other and throwing boulders (or something) in the kitchen. The local industrial facilities and plants definitely broke ordinances and jackhammered for hours starting 6am. We were exhausted and on edge. It intensified our homesickness. After driving back and fourth to Portland Maine a few times, that’s 8 hours round trip, we found a temporary sublet and I moved everything through our obscenely long hallway over the course of 9 hours and moved us in. Between trying to get settled in, not sleeping, my massive flare-up of Carpal Tunnel in both wrists (9/10 pain scale) and some sort of injury to both my index fingers I’ve been trying to avoid sitting at the computer. Unfortunately, I’ve still had to cook and do pretty much everything as normal so it majorly sucks for me right now.

Kris finished his first round of IVIG and is scheduled for his next infusions next week. His latest batch showed up the other day. Never ceases to amaze me how easy the process of getting it has been.

Other than that, we have DESPERATELY been trying to be normal. I’m able to walk places again, which is really nice. Grocery stores and coffee are only 4 minutes away. While this move hasn’t been easy or everything I hoped it would be, I have no doubt moving here was necessary to our emotional and physical well-being. Now we can do normal things – starting with getting a Christmas tree. I was pretty hellbent on getting a tree this year – We need it for morale now more than ever, something resembling a sense of home, so we even managed to go to Home Depot together. Felt good to just do something together.

I’m grateful to be in somewhere resembling Seattle again and while Kris is too, the emotional toll of all this is still very intense for him. He is really devastated. He wishes he could have a second chance at life and wants to go home – to Seattle, or back home to New Zealand. Because of this illness and our current circumstances, neither may be possible, maybe ever again. It’s really awful and I keep counting on stumbling upon a solution that doesn’t involve abandoning treatment. I’m trying not to get let down by this myself, but I guess being insanely busy and exhausted is keeping me from focusing on it too much. Unfortunately, as we get closer to Christmas, I’m finding myself sad that I couldn’t see certain people or have a nice holiday dinner with our loved ones. I wanted to have a couple of people over this year with holiday catering by Veggie Grill. But alas, it looks like it’s just and the rats this year.

Our friends back home and New Zealand knew how down we were and sent us a lot of wonderful gifts this year. I can’t even express how grateful we are to be remembered when we feel so alone and abandoned. While we’ve opened most things, we managed to put a few under the trees.

Jennifer sent us her amazing Sugar Cookies. Definitely a taste of home.

Earlier this year, I spent MONTHS on the phone with Mayo Clinic trying to get in to see their Neurologist who is world-renowned at treating auto-immune neuropathies like Kris’s. Unfortunately, because our new insurance policy is very restrictive and we can’t afford to fly to Arizona (not to mention, the physical toll) we will not be able to go. I was considering doing a GoFundMe so we could make the January appointment, but because we are probably $17,000 in the hole for our emergency room rescue dose of IVIG, we will need all the help we can get paying that off. It hurts me to think of all the effort I went through to get this appointment and I want Kris to get the best care possible. It doesn’t sit right with me but I guess we need to be realistic about the limitations we will now have for his care being in New England under this shitty HMO.

Meh. I’ll keep wishing for a Christmas miracle.

Vi

Plan B and the Brain Eating Virus

IVIG is a very safe treatment for my condition. At low doses IVIG helps those with immune deficiency by providing the protective antibodies from other people. At high doses used in my case it dampens aspects of the immune system. Reducing the production of antibodies and increasing the catabolism of antibodies. It also does a bunch of things with regard to dampening down T cell immune function as well and affecting cytokine levels. So for autoimmune conditions it works well.

Something like 70% of IVIG today is used for treating autoimmune conditions like mine.

If I can’t get IVIG at home, I have a possible second option I can try, but its more risky, very scary, and less effective. I really don’t want to do it. It’s a chemotherapy drug originally used for cancer treatment called Rituximab, and it’s somewhat affordable.

Rituximab is an antibody that attaches to the immune systems B cells, this causes your own body to kill those cells. The B cells are the ones that produce antibodies, so doing this does mean you no longer have B cells, and thus are no longer producing antibodies of any kind, both the good that protect you from disease, and the bad autoantibodies that are part of your autoimmunity. Rituximab however does not help with the T cell involvement though.

You don’t want to do Rituximab unless you really have to. It’s really unpleasant, it’s obviously very risky because you’re killing your immune system and are going without protection from common things that might kill you. But the scariest risk of all is that it exposes you to a disease called PML. This is basically a virus that turns your brain to mush (as in the picture above), and there is nothing you can do about it but die a very horrible death.

Catch 22

I think I’m stuck in a Catch 22.

I have daydreamed a lot about returning to work. The walk back onto the campus from the Light Rail stop. Reconnecting with people. Laughing to myself when I see those robot security guards. Learning what’s changed and happened with projects that are now over a year on. I’ve been a fan of Juniper ever since touching an M40e for the first time 15 years ago at Telecom NZ.

But returning to work is looking complicated. Recovery will be slow and incremental, requiring accommodation, long before I begin to look anything like my old self.

The catch I can’t work out is: IVIG is only being provided on this very narrow plan here in New England. When we return home we will be back on Cigna and the IVIG will stop. Any improvements I made on the IVIG here will revert. If the IVIG improves my condition then it needs to be continued for at least a few years (though the dosage may be reduced). If my condition is improved enough that I can return to work at some point, then I no longer get disability, and must find work. But if I can’t find work that has an employer who would accommodate me during recovery, and provide insurance that covers the continuation of the IVIG treatment, then I relapse.

Do I have to hope my condition doesn’t improve to the point I can return to work so we don’t end up homeless? Do I have to give up IVIG treatment because finding an employer that has insurance that will continue that treatment is impossible from this position?

I have too much time to reflect, and I think a lot about what if things had gone different. It’s hard to wish for too much to change. For example, I never would’ve met my wife without something bad that happened to me, which created the circumstances for it. I think about the the amazing places I got to work. Coming to the US to work at Amazon is such a great memory. I remember the excitement of the interview. The lunch at Republic Bar (about the only thing around there back then) with a guy who looked too young and too cool to be my manager, he was telling me all about cool sounding shit that was new to me, using merchant silicon, etc. The best team I ever worked with and a lot of fun on a job (between the stress). Working there changed my view on so much that it probably damaged me for future employers. Amazon provides IVIG in these cases, if I had made it work out we might not be in this predicament. Microsoft! That backbone was like my baby, I wish I still had hands on it. Had I never left, would Microsoft have helped me with the IVIG? I don’t know, but it seems more likely. Or where would we be now if I had got lucky with that Google interview 2.5 years ago. My specialist in Mountain View tells me that they’re absolutely the best at handling her other patients like me, it sounds like a dream.