When you’re dealing with a challenging health situation, it can be hard. Hard because of what you are dealing with, and hard because you need to navigate getting and seeking care. That typically looks like going to a doctor, getting the doctor to understand the problem, and then finding solutions to deal with the problem.

Each of those has their own challenges. You may not have a doctor that specializes in the area you need. For example, you may not have a primary care doctor when you have strep throat, and have to go to urgent care instead. Or maybe you develop a problem with your lungs and need a pulmonologist, but that requires a referral from someone and several months’ wait to be seen.

Once you face that challenge and are in fact seen by the specializing provider (and hopefully the problem you have is in fact the one this specialist can address, rather than referring you on to a different kind of specialist), you have to figure out how to communicate and show what issues you have to the doctor. In some cases, it’s really obvious. You have a red, angry throat which leads to the doctor ordering a strep test. Or you go to the dermatologist for a skin check because you have a mole that is changing, and you get a skin check and a biopsy of the mole. Problem identified and confirmed.

It takes identifying and confirming the problem, and usually diagnosing it, to then reach the stage of addressing it, either with symptom management or with curing or fixing or eliminating the source.

But…what happens when you and your doctor can’t define the problem: there is no diagnosis?

That’s a challenging place to be. Not only because you have a problem and are suffering with it, but also, the path forward is uncertain. No diagnosis often means no treatment plan, or the treatment plan itself is uncertain or delayed.

No diagnosis means that even if your provider prescribes a treatment option, it may get denied by the insurance company because you don’t have the clinical diagnosis for which the treatment is approved for. Maybe your doctor is able to successfully appeal and get approval for off-label use, or maybe not.

And then, there’s no certainty that the treatment will work.

So. Much. Uncertainty. It’s hard.

It’s also made hard by the fact that it’s hard to tell people what’s going on. A broken leg, or strep throat, or a suspicious mole: these are things that are relatively easy to explain to other people what is going on, what it means, how it might be treated, and what a rough expectation of timeline for resolution is.

Most stories are like this. There’s a story arc, a narrative that has a beginning, middle, and an end.

With the uncertain health situations I’m describing, it’s often never clear if you’ve even reached “the middle”, or what the end will be…or if there even is an end. Certainly no guarantee of a happy ending, or an ending at all if you have been diagnosed with a chronic, lifelong disease.

I’ve been there (here) many times, now living with more than a handful of autoimmune diseases that I’ll have for life. But the first few were relatively “simple” to diagnose, treat, and understand what they looked like. For example, with type 1 diabetes, the symptoms of weight loss, excessive urination, incredible thirst, etc. led to a blood test confirming high glucose, an A1c test confirming it had been high for months, and a diagnosis of type 1 diabetes. The treatment is managing glucose levels with insulin therapy, presumably for the rest of my life. (22 years and counting, here). It makes things challenging, but it’s something I can explain to other people for the most part what it means and how it does or does not impact my life.

Lately, though, I’ve found more uncertainty. And that makes it hard, because if there is no diagnosis then there is no clear explanation. No certainty, for myself or to give my loved ones. Which makes it feel isolating and hard psychologically, in addition to the physical ramifications of the symptoms themselves.

There’s a saying in medicine: “When you hear hoofbeats, think horses, not zebras.” It’s a reminder for clinicians to consider common explanations first, rather than go straight to explanations of rare conditions. Most of the time, the advice is helpful—common issues should be ruled out before rarer ones.

In my case, that’s what we did. We ruled out every possible common condition…and then pretty much all the rare ones. So what do you do, when your symptoms don’t match the pattern of a horse…or a zebra?

You might have an uncommon presentation of a common disease or a common presentation of a rare disease.

Either way, whether horse or zebra, the symptoms cast a shadow. They’re real.

Whether the animal in question has stripes or not, you’re still living with the impact. What makes this even harder is that many diagnostic processes rely on pattern recognition, yet undiagnosed conditions often defy easy patterns. If your symptoms overlap with multiple conditions—or present in a way that isn’t fully typical—then the search for answers can feel like trying to describe a shadow, not the thing itself.

And shadows are difficult to explain.

This makes a meta-challenge on top of the challenge of the situation, which is trying to explain the unexplainable. This is crucial not just for helping your doctors understand what is going on, so we can improve the diagnostic pursuit of answers or gauge the efficacy of hand-wavy treatment plans meant to do something, anything, to help… and it’s also crucial for explaining to your friends and family what is going on, and what they can do to help.

We often want to see or hear health stories in the format of:

• Here’s the problem.
• Here’s what I’m doing about it.
• Here’s how I’m coping or improving.
• Bonus: here’s how you can help

I’ve seen so many examples of friends and family responding to the call for help, for me and for others in health situations. I know the power of this, which is why when you can’t explain what’s going on, it makes it challenging to ask for help. Because it’s hard to explain the “what” and the “why”: you are only left with the “so what” of ‘here’s what the end result is and how I need help’.

(And if you’re like me, a further challenge is the situation being dynamic and constantly changing and progressing, so what help you might need is a constant evolution.)

You might also feel like you shouldn’t ask for help, because you can’t explain the what and the why. Or because it is ongoing and not clear, you may want to ‘reserve’ asking for help for later ‘when you really need it’, even if you truly do need help then and there at that point in time. As weeks, months, or even years drag on, it can be challenging to feel like you are burdening your loved ones and friends.

But you’re not.

The best meta explanation and response to my attempts to communicate the challenges of the meta-challenge of the unexplainable, the uncertainty, the unending saga of figuring out what was going on and how to solve it, came from Scott (my husband). We’ve been married for 10 years (in August), and he met me when I had two of my now many autoimmune diseases. He knew a bit of what he was getting in to, because our relationship evolved and progressed alongside our joint interests in problem solving and making the world better, first for me and then for anyone who wanted open source automated insulin delivery systems (aka, we built OpenAPS together and have spent over a decade together working on similar projects).

That being said, to me it has felt drastically different to be living with ‘understandable’ chronic autoimmune diseases like type 1 diabetes and celiac, and this latest saga where it’s unclear if it’s an extension of a known autoimmune disease (presenting and progressing atypically) or if it’s a new, rare autoimmune or other type of disease. So much is unknown. So many challenges. When we would adapt and address one problem or challenge, it evolved and needed another solution, or another problem cropped up. I’m honestly at this point exhausted of adaptations and problem solving. I’m tired of asking, seemingly endlessly, for help and support. Amazingly, Scott does not seem exhausted by it or tired of me, whereas many people would be. And he said something a few weeks ago, completely off the cuff and unplanned that really resonated with me. I was talking, again (he’d heard this many times), about how hard this all has been and is, and that I was also aware of the effect it has on him and on our relationship. I can’t do all the things I did before, or in the way I did before, so it’s changed some of what we do, where we go, and how we are living our lives. I’m having a hard time with that, and it would be natural for him to have similar feelings. (And frustrations, because if I feel frustrated with being out of control and unable to change the situation and fix it, so too would he be except worse secondhand because it’s so hard to love someone and not be able to help them!)

But what he said literally stopped me in my tracks after he said it, because we were out walking and I had to physically stop after he said it to process it in my heart (and leak some tears from my eyes).

It was something along the lines of:

“You’re not an anchor, you’re a sail.”

Meaning, to him, I’m not holding him back from living his life (as I was and am concerned about).

He continued by saying:

“Yes, the sail is a little cattywampus sometimes, but you’re still a sail that catches wind and takes us places. It’s much more interesting to let you sail us, even in a different direction, than to be without a sail.”

(Yes, you can pause and tear up, I do again just thinking about how meaningful that was.)

What a hit, in the most wonderful way, to my heart, to hear that he doesn’t see me and all these challenges as an anchor. He recognizes them, and that we are dealing with them, but he’s willing and wanting us to sail in the direction they take us, even when that makes us go in some unplanned directions.

Probably some of this is personality differences: I love to plan. I love spreadsheets. I love setting big goals and making spreadsheets of processes and how I’ll achieve them. In the current situation, I can’t make (many) plans, there are no spreadsheets or processes or certainty or clear paths forward. We’re in an ocean of uncertainty, with infinite paths ahead, and even if I set sail in a certain direction…I’m a cattywampus sail that may result in a slightly different direction.

But.

Knowing I’m a cattywampus sail, and not an anchor, has made all the difference.

If you’re reading this and dealing with an uncertain health situation (undiagnosed, or diagnosed but untreated, or diagnosed but with no certainty of what the future looks like), you may feel like you’re a boat adrift in the middle of an ocean. No land in sight. No idea which way the wind will blow you.

But.

You’re a boat with a sail. Maybe a cattywampus one, and maybe you’re going to sail differently than everyone else, but you probably are going to still sail. Somewhere. And your family and friends love you and will be happy to go whichever direction the wind and the cattywampus sail take you.

If you’re reading this and you’re the friend or family of a loved one dealing with an uncertain situation, first, thank you. Because you clearly love and support them, even through the uncertainty. That means the world.

You may not know how to help or be able to help if they need help, but communicating your love and support for them alone can be incredibly meaningful and impactful. If you want, tell them they’re a sail and not an anchor. It may not resonate with them the way it resonated with me, but if you can, find a way to tell them they and their needs are not a burden, that life is more interesting with them, and that you love them.

This has become a long post, with no clear messages or resolutions, which in of itself is an example of these types of situations. Hard, uncertain, messy, no clear ending or answer or what next. But these types of situations happen a lot, more than you know.

If you’re going through this, just know you’re not alone, you’re loved and appreciated, and you’re a sail rather than an anchor, whether you’re a zebra or a horse or a zebra-colored horse or a horse-shaped zebra shadow.

—

PS – I’ll also share one specific thing, for loved ones and friends, as something that you can do if you find out about a situation like this.

If someone trusts you and communicates part or all of their situation, and they specifically tell you in confidence that they are not sharing it publicly or with anyone else or with X person or Y group of people…honor that trust and request not to communicate that information. They have a reason, if not multiple reasons, for asking. When dealing with uncertain health situations, we can control so little. What we can control, we often want to, such as choosing when and how and to whom to communicate about our challenges and situations.

If someone honors you by telling you what’s going on and asks you not to tell other people – honor that by not disabusing the trust in your relationship. Yes, it can be hard to keep it to yourself, but it’s likely about 1% hard of what they are dealing with. Passing on the word becomes a game of telephone that garbles what is going on, often turns out to be passed on incorrectly, and causes challenges down the line…not in the least because it can harm your relationship with them if they perceive you have violated their trust by explicitly passing on information you asked them not to. And that, on top of everything else, can make a challenging situation more so, and it may then later influence how they want to communicate with others, potentially shutting down other avenues of support for them. So please, respect the wishes of the person, even if it’s hard for you. You can always ask “can I share this with so and so”, but respect if the answer is no, even if you would do something different in your situation. Because, after all, it is not your situation. You’ve been invited on the boat, but you are not the sail.

When I first found out I was going to get IVIG (intravenous immunoglobulin), I was nervous because I didn’t know what to expect. New medical treatments or therapies and the uncertainty of what to expect can make it harder. This is a little bit about my experience, what to expect with IVIG, and some differences in the experience depending on location (outpatient or not).

Preparing for the first infusion – prior authorization and scheduling

First, know that IVIG usually needs prior authorization from your insurance. This is approval not only for the treatment itself; but the volume (how much you’ll get); the schedule (do you do it a single day or multiple days in a row) and the cadence (how often do you get it, if you get it over and over again). And, the location.

The first time my provider prescribed IVIG, my insurance denied it. And again. And again. My provider had to do a round of peer to peer with the insurance company before they approved it.

(Note: IVIG is used for dozens of conditions including numerous autoimmune disease conditions. Sometimes this is the only approved treatment for these conditions; sometimes there are other treatments your insurance company will want you to try before IVIG. IVIG is rather expensive, more on that below, which plays into this.)

I was approved for a handful of infusions at an outpatient location in downtown Seattle. Once that was approved, I was able to actually schedule my appointments. If you get IVIG in multiple sessions (e.g. 2 days in a row, like I do, or 3 days in a row), it may make scheduling a tiny bit harder, but usually the clinics can get you in. Luckily, you don’t have to do the exact same time every session, e.g. you can start at 9am on Tuesday and then do 11am on Wednesday, if that’s what works for your schedule or theirs. Or if you prefer and they have availability, you can do it at the same time each day.

For your first appointments though, they’ll probably schedule you earlier in the morning and tell you to expect it to take all or most of the day. My first appointments were scheduled as 6 hour (!) blocks on back to back days. Gulp.

The first infusion experience

When I arrived for my first infusion, they did the usual check in at a front desk (like a typical doctor’s appointment) and then assigned me to a room/bay. This outpatient infusion center has open room bays with curtains to the hallway (and walls in between the bays), with numbers. Across the hall are several bathrooms. Once I got into my room, a nurse came in and introduced herself and walked me through what we would be doing. She started by asking if I had taken any pre-meds (pre medication) such as Tylenol or Benadryl. I said no (I didn’t know that I was supposed to), so she brought me two Tylenol and a Benadryl. This apparently is fairly common, because headaches are a common side effect and in rare cases (something like <1%) of the time allergic reactions can happen, so the Tylenol and Benadryl can slow down or minimize any allergic reaction that might occur.

I’ll admit, I was nervous about IVIG because of what I had read about allergic reactions, but I’ll say up front: I did not have any allergic reactions to IVIG at the first or any subsequent sessions.

While we were waiting for those to kick in, they put in the order for the IG itself, which had to be sent over from the pharmacy. They inserted the IV into my arm, which involves using a needle then immediately pulling it back out so only a small thing of tubing is left behind. (This is similar to an insulin pump site!) They tape it and wrap it so it won’t get jostled from normal movements, and we waited until the IG arrived.

Note: if you need to have labs run to check anything before or around the time of your infusion, they can pull blood from the same IV first, then they flush it with saline and can then use it to infuse your IG.

When they start the IVIG, they start it at really low (slow) rates. Your provider will have prescribed the rates and the schedule. For example, you start at a slow rate and then it increases every 30 minutes. This way, if you do have any symptoms (sudden headaches or start feeling itchy, for example), you can go back to a slower rate to finish the infusion. That will take longer, overall, than if you went at the planned schedule because as your rate gets higher (larger), more goes in faster.

An example schedule might be 0-30 minutes at 30 mL/hr; 30-60 minutes at 60 mL/hr; 60-90 minutes at 120 mL/hr; 90-120 minutes at 240 mL/hr; 120-150 minutes at 375 mL/hr. If you still have IG left to infuse after 2.5 hours (aka 150 minutes), they continue at the highest rate, eg 150-180 minutes will continue at 375 mL/hr. Note that with this typical progression, your rate starts slow and doubles every half hour, until you get to the max dose. I believe this is calculated based on body weight. I am not sure if this is the number they were referring to (for max rate determination), but I heard the nurses discussing that one of the numbers is calculated based on ideal body weight, not actual body weight. That is one of the factors that will influence how much you get (volume wise, as in how much liquid) as well as the rate schedule.

If you start to feel yucky for any reason, you have a button you can call the nursing team with. But they also have scheduled checks. My experience was that they took blood pressure, pulse, and temperature at the start (0 minutes) and every 15 minutes up to the first hour, then every half hour after that. So with the rates described above, the schedule might look like this table:

Time	Rate	Checks
Start (0 min)	30 mL/hr	BP, pulse, temp
15 min	(no change, continues)	BP, pulse, temp
30 min	60 mL/hr	BP, pulse, temp
45 min	(no change, continues)	BP, pulse, temp
60 min	120 mL/hr	BP, pulse, temp
90 min	240 mL/hr	BP, pulse, temp
120 min	375 mL/hr	BP, pulse, temp
150 min	(no change, continues)	BP, pulse, temp
180 min	(no change, continues)	BP, pulse, temp
(runs until you infuse all liquid)

In the scenario where you had no side effects, and let’s say you had 400mL of IG to infuse, that means you would finish the initial infusion in around 2 hours and 15 minutes. After the main infusion finishes, they will hang a small bag (often 50mL) of saline, and then infuse that at the same highest rate of your IG, which means that all the remaining IG in the tube will get infused in. This may take something like 10-30 minutes, depending on your rate.

Remember, though, for your first infusion you may be on slower rates than this progression, to watch for side effects. A typical first infusion might be roughly double the time as this (4+ hours) even if you don’t have side effects, as you progress through the rates. If you do have side effects (feeling yucky, headache, itching, etc), it may take a lot longer because they will go back to the slower rate where you didn’t have side effects. For example, if you had issues at 240 mL/hr but not 120, they will finish infusing at 120 mL/hr, which would take an estimated 4 hours and 45 minutes, rather than 2.5 hours.

Another thing that can slow down the total time is bubbles. If your infusion line develops bubbles, the infusion machine will detect them and sound an alarm and stop the infusion. A nurse will come in, clear the alarm, shake the bubbles back up the tube, and proceed. That might take 2-3 minutes total, but if that happens several times (which is common with IVIG), that may add another 15 or so minutes.

Once that’s complete, you will get the IV removed (which is like pulling a bandaid off, in terms of the experience) and you’re ready to go for the day!

With the slow first infusion rates, bubbles, and saline flush at the end, this is why they schedule you for 6 hours or so, even if the infusion itself may take 4.5 or so hours.

See below though, because the next round of infusions are usually quicker than the original rounds, if you don’t experience side effects during the infusions.

Creature comforts – what to take to an IVIG session for your infusion

Most infusion centers have semi-comfortable recliner chairs. You can put the foot of the chair up; you can lay the chair back. Some of them have a seat heater, although admittedly it doesn’t get super warm. You may want to dress in layers so that if you get cold, you can add items. For example, if it’s summer and you typically wear shorts and a t-shirt, you may want to take loose pants you could pull over your shorts or a jacket to throw over your torso, or socks if you aren’t wearing them. The nursing staff will offer you heated blankets, too.

That being said, my experience with IVIG was freezing. Not from the ambient room temperature, but because the liquid arrives very cold from the fridge, and infusing cold liquid into your arm at the higher rates caused my arm to be ice cold and uncomfortable. I tried warm blankets, throwing a jacket over my torso, wrapping a towel around my arm… and I still had a cold arm and cold hand that took over an hour to warm up after my infusion ended. What finally ended up working best for me was to bring my own heating pad, and place it on a low temp and sit against it. This warmed up my core temperature which then helped keep my extremities (arms) warmer. Now, I regularly use my heating pad every time and don’t need a jacket, long sleeves, a blanket, or anything else! It’s surprising how effective it was to warm my core in order to combat the cold in my arm. Another option is an infusion hoodie, like this one that my sister in law got me. It has zippers in both arms, so you can wear it with your IV tubing.

You’ll absolutely be able to get up and go to the bathroom! At this infusion center, the infusion machine is on a pole with wheels. It’s plugged into the wall with a power cord, but they’ll unplug it for you or show you how to do it, so you can get up anytime and roll yourself to the bathroom, then come back. The battery on the machine lasts 6-8 hours, so you don’t have to bother plugging it back in, and this makes it easy to get up and go to the bathroom whenever you need to, especially if you’re doing a good job staying hydrated (e.g., drinking lots!).

Especially at those longer first infusions, you’re there a long time (4 or 6 hours or more). At my first infusion, they offered me a breakfast menu (because we were there at 8am) and a lunch menu to order food from. At shorter infusions you won’t get a menu if you’re not there at meal time (and at other locations, see below, there are no meals but just snacks), but they still offer to bring you juice, water, snacks periodically.

I have celiac disease which means I’m gluten free and that cuts down on my ability to take whatever from the menu or snacks. (I’m also choosing to mask to avoid infection exposure, and know that masking works, so I’d rather not eat if I don’t have to, and so I don’t). They probably do have some GF options, but I don’t bother with them and bring my own snacks if I want them, and my own drink bags. I have a soft insulated bag I throw water bottles and diet sodas into with an ice pack, so they stay cold and I can have a drink any time I want to, of my preferred choice.

You don’t have to bring your own stuff, but if it would make you happier or more comfortable, you can!

The first few sessions, I brought my laptop, (and phone), headphones, and a tripod for my phone. I tried to get some work done on my laptop, but because the infusion was in the crook of my arm, I risked kinking and stopping the infusion. I eventually gave up on that (but it might be more feasible if it was infusing into my forearm, for example), and stuck to using my phone. Sometimes I watch a show; sometimes I listen to a podcast or audiobook or music; sometimes I’m on zoom calls on my phone with video off, depending on my schedule. But plan to bring entertainment/something to occupy yourself with. If you want, you can nap – they’ll dim the lights, but they’ll likely be doing blood pressure etc checks every 30 minutes so don’t plan to get uninterrupted sleep for the most part.

Side effects from IVIG at first infusion and subsequent infusions

At my first round of infusions, I honestly felt like I was in “time out”. I felt awkward sitting in the chair, tied to the infusion machine. I wanted to be working or out walking and going about my day. I wanted to be normal! (Well, I wasn’t normal, which was why I was getting an infusion). I felt fine during and after my infusions, so I got home and went for my usual walk. It was really hot that day (90 F in July in Seattle), so I felt pretty gross and overdone, and probably overdid it. Then I came home and tried to get some work done, but felt like garbage – I thought because of walking in the heat.

The second day, I did the same thing, but decided not to walk in the heat. I still wanted to try to get work done, and did some, but still felt generally kind of gross and tired and just not great. I had a little bit of a headache, so I kept taking Tylenol (in addition to the dose they gave me before my infusion).

The third day (so the day after my second infusion), I still had pretty strong headaches and felt a little gross. Not sick – I wasn’t infected, all my biomarker data from my wearables was fine (e.g. heart rate, HRV, temperature, respiratory rate etc), but I felt worn down.

Fourth day? Same.

Fifth day? Same.

It probably took those ~3 days after the two days of infusions of feeling gross, then I started to feel better.

Then I started to feel GREAT. I had more energy than I had had in months! It felt like I went from Eeyore to Tigger. Wahoo! So much energy (and no more headaches). I was thrilled.

(Spoiler alert: this is probably unique to my situation, but this also did not last).

For me, the first week I felt sluggish and had headaches. The second week I felt better. The third and fourth week I continued to have symptom progression like before I was on IVIG. That’s probably unique to my situation – from what my doctor says, this is not a common response to IVIG to have an improvement and then a decrease like I do, in a cyclical fashion. But this pattern has continued in all subsequent months, where week two I see an improvement and then drop back down to the previous progression in weeks three and four.

What I did change in subsequent infusions – my personal approach

The headaches I got from IVIG weren’t quite debilitating, but they were obnoxious and cut down on my ability to work or rest like I wanted to.

Over time, I gradually figured out that for me, working on IVIG days (during, or even only after infusions) tends to cause more headaches for several days after. Sometimes I need to work (well, I choose to work) on those days, but I’ve gradually shifted my schedule so that I’ll do audio-only calls that are listen only while I’m infusing, or after I’m home, but I don’t plan any intensive cognitive work on IVIG days. I also don’t try to go out and do anything anymore because that typically results in several days of minor but annoying headaches, even with Tylenol. I can certainly do those things, but it comes with a cost.

In other words, I went from trying to live my normal life on IVIG days (and feeling like I was in time out) to changing my approach and treating them like rest days (minimal to no work planned; no requirement for physical activity but to do a short, gentle walk only if I feel really great) and also often the day after. That is frustrating when I think about how much time I am losing (3 days every month!), but when I calculated the lost productivity in the next few days after IVIG, I realized I was losing out on even more time by trying to work through it, and so resting up on IVIG days and maybe the day after ultimately retains more productive and energetic time for me in that first week.

I continue to take Tylenol and a Benadryl prior to each infusion, although I have never experienced any allergic reaction symptoms (eg itching, rash, etc), but the cost of a Benadryl is low and it provides peace of mind. The Tylenol before I start does seem to help, and I find that putting reminders on my calendar for Tylenol in the afternoon and evening before bed on IVIG days both helps me more proactively remember to take them, and taking them proactively makes the headaches minimal or non existent, compared to if I wait for a headache to start and then am chasing the headache with the Tylenol. Not sure how common that is for everyone else, but in my experience after several months of testing, this is true for me that Tylenol proactively for 2-3 days can eliminate the headaches, in addition to reducing the cognitive load.

Note that you can also adjust your infusion rates, especially if you get headaches DURING infusion. My headaches are never during the infusion, nor bad enough that I want to adjust my rates to be slower – they instead seem to correlate with cognitive burden after infusions and activity burden on my body, so I choose to address them with less work, more rest, and Tylenol for a few days.

The financial cost of IVIG

My eyes about popped out of their sockets when I looked at my EOB (insurance document that says how much was charged and how much insurance paid) for my IVIG experiences. It was in the ballpark of $30,000 billed PER DAY, so $60,000 or so total for two days of IVIG.

Oh my gosh.

(Luckily, I have health insurance; and in fact I typically hit my out of pocket max and deductible within a few days of the new years, so I was not paying for this directly).

As those of you who have looked at EOB know, that’s not what actually gets paid, but still. Yeeesh.

The insurance company rate was more like $17,000 per day, or $34,000 for the two days. Still yikes, that’s a LOT of money. And to do this every month?!

That’s probably part of the reason why insurance pushes back on requests to cover treatments like IVIG. It. Is. Expensive.

But in some cases like mine, they are literally the only option and often the only FDA-approved treatment option for certain conditions.

But, there are things you can do to lower costs, and your insurance will likely tell you this. For example, my prior authorization was originally for something like 41 days, enough for two rounds of treatment. After that, we would have to resubmit for prior auth. I read that approval letter though and noted that they would be more willing to approve outpatient, ambulatory infusion centers.

…which is what I went to the first time. It’s not at the hospital; it’s in a standalone building across from the hospital. But for some reason, they source from the inpatient hospital pharmacy (I think) and so while they bill outpatient costs for nursing staff and facility, the IG liquid itself gets charged at hospital prices. Sheesh. Thus, the $30,000 per day, the bulk of which is hospital priced IG (which is already expensive).

So I looked around and found another ambulatory infusion center that is equal distance from my house, but actually a shorter drive most of the time due to traffic patterns, plus I wouldn’t have to pay for parking. The downside is that they’re not tied into MyChart so I can’t see their records or the status of my prior authorizations like I could with the other ‘outpatient’ center. The upside is: they are significantly cheaper.

How much cheaper?

They bill $17,000 per day of IVIG for the same volume, and my insurance therefore pays about $5,500 per day, or $11,000 total for two days every month.

$34,000 per month, or $11,000 per month. Same amount of time. Same drug volume. Same concept…but much cheaper.

There was no way to find out in advance how much IVIG would cost at each of these locations. I only know now, after having experienced at both locations, what the relative costs are. It’s the same treatment, the same drug/liquid (IG) with the same cost for the same volume…. Except it’s not. Outpatient doesn’t always mean ‘outpatient’, depending on what services they use such as where they are sourcing via the pharmacy.

Note: this won’t be what anyone else’s IVIG costs, of course. It’s based on the volume of IG I am getting (based on my weight and medical conditions), plus the insurance plan, plus where they source it from, etc. But I’m sharing my costs to give you a ballpark (expect thousands, if not tens of thousands as the “price” that you or insurance won’t pay, but is the upper cap on what to expect).

Other infusion experiences including a different outpatient infusion center clinic experience

My second series of IVIG, due to the cost, was at this other outpatient center. This is in an ambulatory infusion clinic that’s basically plopped inside an older medical office or business building. It’s less shiny and new, but works the same. There are rows of bays with infusion chairs (so you’re in your own ‘room’), the infusion still works the same, etc.

I ended up in this location because I was actually trying to get home infusions. If I was going to be doing this two days a month, I thought, I’d rather have a home health nurse come in and do it and save me the travel time. But it turns out they won’t do mine as home  infusions because I do two days in a row. Stupid policy, and they lied to me up front about it, which made me really mad, because I was stuck going to this outpatient location. (I’m less mad now that I see the comparable cost, so it’s worth saving $25,000 a month to go to the less great location, because it’s not $25,000 worth of value to go to another location or fight prior authorization again to find another outpatient ambulatory site that may or may not be any better.)

They’re pretty efficient. You are basically scheduled directly with a specific nurse each time and you know who it is. They text you a reminder and some questions the day before, and you walk in and say hi and immediately get seated. They set up your IV and ask some questions and off you go. It’s the same infusion time (eg 2.5 hours, in my case), plus the infusion set up time which is usually pretty quick (15-20 minutes) and the flush at the end (~15 minutes), plus any bubble time, which means about 3 hours at the clinic, plus my ~30-40 minute drive each way depending on traffic. Times two days in a row. So, not as bad as those first infusion appointments (6 hours estimated), but it’s still 4-5 hours round trip of time for two days in a row. The nursing staff is great, and like the other clinic you’ll get offered warmed blankets, snacks, and drinks. So in terms of experience, it’s not too different. The main functional difference is that your infusion machine is on a pole on the wall, so you can’t get up and go to the bathroom with your infusion set up. Instead, you have to ask (or time it so when they come in to do a rate change) for them to pause the infusion, unhook you, then you go to the bathroom and come back and get your infusion hooked up again and you’re back at it. (This adds another 5 min or so to the total time, so I usually try to time it so I am doing it when they’re coming to change for the saline flush, or make it until the end. Sometimes I make it, sometimes I don’t because I’m also trying to hydrate really well and drink a lot, because that helps reduce headaches, too.)

At the end of each appointment round, they also take on the hassle of calling the scheduler for me and getting my appointments scheduled. I generally stick to roughly the same times of day and prefer earlier in the week appointments so that I can feel better by the weekend (again, perhaps my unique response timeline to IVIG).

I like this location a little bit less than the downtown location, but not $25,000 less, so I continue to use them every month for my IVIG.

Final tips – infusion rate spreadsheet to estimate time of IVIG

You’ll maybe have picked up that I don’t like uncertainty and not knowing what to expect. The vagaries of time uncertainty related to infusions bother me, because I like to know roughly how long things will take so I can schedule the rest of my day. After the first round of IVIG once we figured out what my rates are (and that I don’t have to slow them down for side effects), it should be predictable for how long things take every time, with margins for getting set up and flushed, etc. I ended up making a spreadsheet with my rates and time increments (every 30 minutes) and since I know my total volume of IG, I can see roughly what time my infusion will take. I also estimated the flush time of the 50 mL of saline (which is given at my max rate), and added that in. My spreadsheet therefore is set up so each time I change the start time and it auto-populates every 30 minutes how much will be given, how much has been given cumulatively, and including the estimated flush time at the end, what time the infusion will finish. This way I can estimate what time I’ll be leaving and when I’ll get home. For example, if I have a 10:30 appointment time, my infusion might start flowing at 10:48 (usually plus or minus 5 minutes from this, depending on if they unboxed all my stuff before I arrived). I might finish at an estimated 1:22pm, plus flush time, which means I can expect to be walking out the door at 1:35pm.

This way, if it takes a lot longer (say, start infusion at 10:57 and I’m having tons of bubbles), I know that I’m likely to be a half hour behind my typical schedule.

(If you make your own spreadsheet, it won’t be exact: it takes them a minute or two after the rate change alarm goes off to come and change it, so I actually add a minute to every 30 minute estimate to adjust for this time. I also add ~3 minutes buffer to the overall time to account for bubble pauses, too, as usually I average 2-3 rounds of bubbles that pause the infusion each time.)

Schedule may change (and IVIG may not be forever)

I had some existential angst when I realized that I needed IVIG. In part, due to the situation that caused the need for IVIG. But in part, for the idea that I would be on this monthly schedule for IVIG for the foreseeable future. Two days a month seemed like a LOT, especially with not knowing how long I needed it for. (At the point in time I’m writing this, it has been six months, and I’m still getting it for my situation.)

But if you need it, it’s usually worth it. (If not, you’ll stop it). So that my final piece of advice for folks:

• If you need it, it’s worth the (time and usually financial) cost
• It may not be forever. Your situation may improve to the point where you can space out your infusions more than your initial schedule. For example, you might first get it every 4 weeks, then do well enough to push it out to every 6 or 8 weeks, or more.
• You might get to eventually discontinue it.

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That’s been my experience with IVIG so far, after 6 months of sessions where I get IVIG two days in a row every four weeks. There’s not a lot out there about IVIG, and I’ll caution that my experiences may not be universal, but this may help others know what to expect when they’re going for IVIG for the first time, regardless of condition. You’ll note I did not specify what condition I have or the reason I’m getting IVIG, and hopefully that helps people understand this has been my n=1 personal experience.

Feel free to drop any questions below, or share your own experiences or any tricks you’ve come up with to improve your own IVIG experiences (like I do with my heating pad to fight the cold arm effect).

The canary in the coal mine was my swollen eyelids. No one (including my providers) seemed to care over the last 3 or so years, but all along, I knew it was a matter of time before my body decided to speak up even more loudly and tell me what else was wrong and what was causing the swollen eyelids. And in January, it was time. My body decided to speak up, and it did so loudly.

I woke up one day in January with a sensation of wool wrapped around my lungs. I could breathe…but not normally. It felt like oxygen was not flowing effectively out of my lungs. My VO2max also dropped suddenly and unexpectedly (given my activity levels) by about a point. And sometimes when I would exercise, my SpO2 levels would drop well below where they were supposed to be, and take a while to come up. Some days were better, but some days I could not walk and talk on the phone at the same time without feeling like I needed to gasp for breath in between sentences (and much shorter sentences than I would normally say). My lungs, in other words, have problems.

I’ve spent the last ~3 months trying to figure out what’s wrong with my lungs, but we are still not to the point where we can define exactly what is wrong. Or, how to make them better. Yet, at least. (I’m pessimistic about “fixing them” but I’d sure like to stop them from getting ‘worse’). Right now, they’re not TOO bad. I did pulmonary function testing and some of my numbers are in ‘normal’ range (although they’ve dropped anywhere from 5-17% from baseline in 2015, when I had a bunch of above-normal results). Other numbers, however, are very below normal (<80% is not normal): some are in the 65-70% range. Bleh. Some are even lower. Collectively, they show TWO possible types of things going on, one of which is restrictive lung disease of some sort; the other is some possible small airways-related disease (which means albuterol might help that). No obstruction, which is good, in part because it crosses off one area of concern off the list, but I still have two other areas of concern. I also did a follow up high-resolution CT (aka HRCT) on my lungs which showed no obvious inflammation or fibrosis (scarring) which is both really good news – nothing terribly wrong yet to the naked eye – but also frustrating because clearly there is something wrong. My doctor and I are both concerned by the level of symptoms, and my doctor managing my autoimmune condition has suggested that the lung stuff, whatever it is, is now a separate disease/on a separate path than the rest of my autoimmune disease activity, and it should be managed by a pulmonologist.

So we are still waiting until my pulmonologist appointment to get more answers (every specialist referral means another 6 weeks to schedule the next new appointment, stringing out the problem solving process over time), and in the meantime I try to do what exercise I can do. Which is still exercising every day, but slowly and with lungs that hurt. Some days with lower blood oxygen availability; some days with OK oxygen (as measured by SpO2). Some days also hurt more to physically breathe, and on those days that often starts from the moment I wake up before I’ve even sat up in the bed. I have not been running a lot, which makes me sad. When I do run, it’s not joyful most of the time, although I try to appreciate the ability to run at all on those ‘bad’ days and savor the few moments of joy on the few better days. Not because of my legs, but because of my lungs. Sometimes just walking, even super slowly, also sucks.  But sometimes, I can still ski or hike like I used to, and other days I ski or hike like a snail pushing its way through a sea of peanut butter (very slowly and usually with a lot of vocal complaining about it, when I can afford to catch and waste my breath on words to complain). I’ve had to shift my focus from training with a schedule and a plan to trying to figure out a list of what I can do on a “good lung day” (which is <50% of the time). On “bad lung” days, I just focus on moving at all, however seems feasible that day.

Meanwhile, it’s not just my lungs. At the same time the lung stuff developed, I also developed pretty serious and sudden joint pain. It particularly affects the vertebrae in my spine, hips, and upper spine between my shoulders and neck. Did you know you have ‘joints’ where your ribs connect to cartilage and your cartilage connects to your sternum? I’m re-learning some of the many places we have joints that we usually don’t pay much attention to. Heat helps, so I spend a lot of time sitting against a heating pad, which thankfully means the biggest areas impacted by this issue can get regular heat therapy. But it also will sporadically “pang” with pain in other random joints such as in my hand, the middle of my foot, a toe joint, or my shoulder. Oral NSAID does nothing for the pain, but when the pain is in easy-to-reach spots, topical NSAID gel does help some, as does the application of heat to those areas. It can get pretty problematic, though, to the point where rolling over in bed at night wakes me up taking my breath away because I rolled over and the joint pain in my spine was so painful it woke me up. (And thus I don’t sleep well those nights, which also stinks.)

What the joint pain, like the eyelids cycling in and out of inflammation, is telling us is: the inflammation is spreading in other parts of my body and my body is attacking itself elsewhere, too. My eyelids continue to swell, but now there’s also a red patch particularly outside of my right eye that provides a visual cue to Scott when my inflammation is particularly at peak. But the joint pain and the lungs collectively mean my fifth autoimmune disease is angry and kicking in protest, lashing out at the rest of my body. And we need to do something about that, and so we are.

Treating autoimmune diseases is not without risk due to the treatment itself. There’s the short-term risk of side effects and the long-term risk of side-effects. When autoimmune disease gets as problematic as mine is (attacking my lungs; the escalating joint pain); it warrants risking the side effects of the medication, even though it’s scary. Also scary is the knowledge that we are trying to tweak my immune system. Even though I know my immune system is *too* strong and *overreacting*, it’s what I know, and it’s scary to think about trying to turn it down, because there is the risk that it overshoots and turns down too far in the process of trying to take it down just a notch.

Ultimately, the first-line treatment my doctor and I chose is a mild version of immunosuppression, which is an immunomodulator designed to modulate only part of the immune system. This is more mild than what most people think of when they think of immunosuppression (e.g. to go with organ transplant), and while they don’t know exactly how it works, it seems like it causes an effect of turning down *part* of the immune system, but not all. (In theory.) While it’s not been very well tested in studies in my autoimmune disease (this is the story line through allllll the medical literature about my condition), there’s a “cousin” autoimmune disease for which there are a lot of studies and data showing it helps some people, and particularly has a good chance of helping the joint pain. However, the risk of negative outcomes is also not zero. In the medicine we decided was best for me, there is a risk of it accumulating in my retina and causing vision loss. Yikes. The risk in the first 5 years is about 1%, meaning that in people who take this medication every day for 5 years, 1 in 100 will begin to have vision issues. At the 10 year mark, however, this goes up, and 10% (10 in 100 people) begin to have vision issues. And the problem is, this vision damage is not reversible. Plus, the medication has a half life of months, meaning it takes time to ramp up, but also that if you start to notice problems and need to stop the medication, there is another set of weeks to months before the medication levels actually begin reducing in your body, so you get additional damage possibly during that time. As one might imagine, I am very nervous about this risk profile, but this possibly helps illustrate exactly how bad the situation is that I am in: this joint pain is not ideal for quality of life and it is a serious symptom of a serious problem, which is a rampaging immune system that needs to be treated.

While I am not currently fully immunosuppressed, it does mean that I am choosing to continue to be careful to limit my risk in exposure to infectious diseases. For me, that means continuing what I’ve been doing since early on in the pandemic: I am already using a portfolio of risk mitigation strategies including seeking better ventilated air in indoor spaces (using CO2 monitor); masking in any indoor spaces outside home (unless I am at family’s house where everyone confirms no symptoms, tests negative, etc); using portable air purifiers and far-UVC lights when feasible; getting vaccinated and boosted against all the infectious diseases I can, etc. That feels right for me, although it’s not necessarily what others might choose to do in my situation. But again, my risk profile not only includes 5 autoimmune diseases, for which we are trying to turn my immune system *down*; I also have some kind of lung disease now, with lowered lung capacity that is directly influencing my daily living ability. So I am happy to do all the easy things that I can to further lower the risk of hurting my lungs more, such as by limiting exposure to acute respiratory infections that could lead to all kinds of further issues and complications that I just don’t need or want to deal with. So, the biggest change is mostly to what I will continue to ask of family members, which is reporting any and all symptoms of any type of illness, even if it’s “just” a cold, because a “cold” to someone else is likely going to be a lot more serious to me, given my immune and lung status. Plus, too, I may end up needing to switch to a different level of immunosuppression, which further changes the calculation of short- and long-term risk of acute illness exposure.

I started the medicine a few weeks ago, and I managed to avoid the worst of the short-term side effects that cause people to discontinue it. In weeks 2 & 3, it seemed like it was helping reduce the joint pain. I got excited, but then very un-excited when the joint pain manifested again (along with re-swollen eyelids and the spreading red patch outside of my eye). I was hoping this medication would permanently depress the inflammation and systemic attacks, but instead, so far, it looks like it might slightly dampen the inflammation cycles (and thus symptoms), and they haven’t yet completely gone away. And my lungs continue to be problematic, which matches my doctor’s prediction so far that they will not be impacted by this medication and I need some ideas from the pulmonologist to treat my lungs.

So here I am. 5 (or 6) autoimmune diseases; still acutely aware of the stigma that comes with living with (so) many chronic diseases; entering new territory of immunomodulation; and possibly in the future, maybe needing more immunosuppression. I don’t have a lot of answers or even a good understanding of what is going on with my body (for example, what exactly the problem(s) are in my lungs), which is frustrating. More acutely, some days just stink either due to bad lungs or due to joint pain. When I get really unlucky, I have a bad joint day on the same day as a bad lung day. The challenge with bad lung days is that it impacts my ability to exercise, whereas my joint pain doesn’t keep me from exercising (because it’s no worse during exercise and sometimes exercise distracts my brain from the pain signals). So during bad lung days, some of my “treatment” options for the bad joint pain are reduced. Bad lung days also make me feel really fatigued and short of breath even sitting down doing nothing, so I have some days where I’m doing normal workloads of things I want to do, and other days where I’m doing less than I would like. I have been slow to respond to emails related to non-time-sensitive-projects because basically, I’m over here just trying to breathe, and that’s hard. So: sorry to anyone who’s read this who has happened to email me – consider this an auto-responder about why I have not yet responded.

Stay tuned for more posts: I’ll be using this framework to talk about exercise strategies for exercising with ‘bad lungs’ and strategies that I’ve found to be effective (see this post as an example for strategies) so far on the days where I’m struggling to breathe and exist but also want to exercise so I don’t decondition my body so I can do the things I want to do on the “good days”.

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If you’re a family member or friend who’s read this and wants to acknowledge what I’m going through but doesn’t know what to say – it’s ok. I don’t know what to say, either, which is in part why I haven’t said anything to most people! But it felt like it was time to start sharing some of what I’ve been going through. Feel free to send me a purple heart emoji (or a cat picture), no caption/text needed, and that’s an a-ok way to acknowledge that you’ve seen this. 💜

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PS – one of my friends described my lungs as “sad balloons” and for some reason, that analogy felt really appropriate to how they physically feel inside my rib cage. Sad, and ineffective. I gave ChatGPT some prompts to illustrate “sad balloon” lungs and ended up with these, which feels cathartic to illustrate.