Keeping on keeping on

Welp, I managed to regain a whole 1% in lung function. Go me.

I am feeling a little better. My oxygen has been a little more stable and my heart rate hasn’t been going quite as high with exertion, though I do still need some supplemental oxygen when I’m out and about. I’ve also been a little less congested. Overall though the gains haven’t been too significant, so much to my disappointment my doctor wants to try one more week of IV antibiotics. Thankfully, however, we’re stopping the Zosyn and switching me back to Zerbaxa, aka the Fancy Restricted Antibiotic. This is so that I can get a LITTLE more sleep, since Zosyn is run every 6 hours, whereas Zerbaxa is run every 8 hours. I’ve been an utterly sleep deprived zombie these last couple weeks, so at this point I’ll take whatever I can get! I feel like a cat these days: I don’t actually sleep anymore, I just take naps of varying lengths at various points throughout the day. I think cats enjoy this more than I do though!

On the plus side, my last sputum culture did NOT show MRSA or either of the two fungal infections that popped up previously. Unfortunately, that hasn’t actually translated into any real improvement in my health. If I don’t see any real improvement with another week of IV antibiotics, we’re going to move on to treating the other infection that has been cropping up lately in my sputum cultures: mycobacterium chelonae, a nontuberculosis mycobacterium (aka NTM). We haven’t treated it until now because it’s unclear whether the NTM is actually the source of any of my symptoms, and treating it requires taking multiple antibiotics for a very extended period of time. Apparently it can also be difficult to get approval for some of the medications involved. And, one of the meds used to treat it is Tobramycin, the IV antibiotic which gave me tinnitus and can cause hearing loss. We’re exploring whether I can take the Tobra via nebulizer, which has less risk of causing hearing issues, but unfortunately in the past the nebulizer formulation of Tobra (called TOBI) gave me asthma attacks. I’ve asked whether we can try giving me the IV version of Tobra to inhale, which is what I did for years without any problems before TOBI was developed. It’s unclear however whether we’ll be able to get approval for an off-brand use of injectable Tobra since a nebulizer version is available.

The other main goal now is getting me to be a little more active. I’ve been feeling sort of awful both physically and mentally, which has led to me spending a LOT of time moping on the couch. I’m trying to pull myself together and leave the house a little more, even if only briefly, and to do brief, mild exercise at home a few times a day. I’m obviously not running any marathons, but whatever exercise I can manage is good for both my physical and mental health. I just need to be careful about not pushing my heart rate too high, as unfortunately the “leaky pipes” have still been an issue. Thankfully I haven’t had a full bleed in two and a half weeks, but I’ve been having varying degrees of bloody mucus at least once a week all month. I’m trying really hard to avoid anything that could trigger a full bleed, as that would force me to hold various treatments and prevent me from clearing mucus out of my lungs. On the other hand, I do need to exercise, because not exercising is also detrimental to my lungs as well as the rest of my body. It’s a really fun catch-22.

It has been a really, really difficult winter. Hopefully things will start looking up soon.

Quicksand

WARNING: This is not a happy-go-lucky Eliana post. Proceed at your own risk.

Sometimes it gets hard to keep fighting this fight. All too often battling CF feels like struggling to climb out of quicksand: a lot of effort, but little progress. I’ve been feeling very quicksanded lately. At this point I’ve been varying degrees of very sick for almost 4 months straight. It’s exhausting and demoralizing.

Aside from soul-sucking fatigue, I think I hate the bleeding the most. It’s probably my most visually dramatic symptom, and it’s always been the thing that really made me feel the weight and severity of CF. But it’s also physically uncomfortable on multiple levels. The only way to get the blood out of my airways is by coughing, which can be very draining. Additionally, blood isn’t exactly a substance that’s supposed to go down your throat, so I’m usually hoarse and scratchy afterwards. And most bleeds don’t stop immediately, so I’ll be coughing up thick bloody mucus for hours or days afterwards, which is physically and mentally exhausting.

I bled again last night, by the way. I know it’s not over yet because I can still taste blood in the back of my throat. Here’s hoping it settles down sooner than later.

The most frustrating part is that we are soooo close to the release of a new drug that has potential to be extremely beneficial to me. Several years ago they started coming out with drugs that targeted specific CF mutations. It’s not a cure, but for the first time they were able to treat the underlying cause instead of just the symptoms. Unfortunately, none of those drugs treated my specific mutation combination. CF is recessive, which means I hit the genetic lottery and got 2 copies of the CF gene. One of my mutations is the most common one, but my second mutation is rare, which means it won’t be getting much money and attention when it comes to researching new drugs. However, they’ve finally developed a treatment for people who only have one copy of the most common mutation, and people have shown tremendous improvement in the trials. It’s supposed to go for FDA approval by the summer and hit the market by next year.

This should be good news, right? It definitely is! Unfortunately, while the drug can stop or significantly slow the damage CF causes, it can’t undo preexisting permanent lung damage. People have regained significant percentages of lung function, but they can only regain so much. So the lower I fall now, the lower I’ll be even if I get the drug and regain some lung function. Over the summer my lung function was 60%. If I had regained 10% of my lung function then, I’d be back up to 70%. I was still working full time at 70%! But lately, I’ve been struggling to even hit 50%. The more ground I lose now, the less benefit I’ll see – if the drug even works for me at all, of course, which is not guaranteed. It’s extremely frustrating that things have been snowballing out of control now when hope for a real treatment is so close.

So, I’m tired. I’m frustrated. And, honestly, I’m scared. I don’t know what’s going on with my lungs right now, or what’s going to happen over the next year. I’m just trying to push through from one day to the next, one handful of quicksand at a time.

Hanging in there

Lately it seems like every time we do a sputum culture we find a new infection. I think my lungs put up a flyer at the hottest bacteria hangout. “Vacancies available! Low prices! A comfortable place to raise your spawn!” This month’s pest: MRSA. I’m kind of feeling like a leper these days, since I know MRSA is supposed to be highly contagious, but I’m trying to stay calm since healthy people aren’t supposed to be susceptible to it. It’s unclear whether MRSA is the cause of my recent health struggles, but we aren’t taking any chances, so I started IV Vancomycin on Friday to try to eradicate it.

I’ve never taken Vancomycin before, so they also sent out an “anaphylaxis kit,” which wasn’t alarming at all. Fortunately the first dose went well and the anaphylaxis kit remains unused. Unfortunately, after a couple days I developed a rash all over my torso. It took 40 years, but it seems we finally found a medication that I’m allergic to! Due to this we stopped the Vancomycin and are switching me over to an oral antibiotic. In the meantime, the rash still hasn’t gone away and actually seems to be spreading, despite the fact that my last dose was Sunday night and I’ve been taking Benadryl multiple times a day. So that’s been fun. Of course, I also just realized that my Benadryl expired in September of 2017. Maybe some non-expired Benadryl will work better.

Aside from all this, my lungs decided to bring the drama and bleed Friday night. I thought the leaky pipe issue was under control, but apparently not. Thankfully it was “only” 15 mL, which for me isn’t too bad. I did make an appropriately dramatic scene though by spraying blood all over the white bathroom sink. If it wasn’t Shabbos I totally would have taken a picture of the crime scene for posterity. On a related note: squeamish people should never, ever randomly scroll through the pictures on my phone. You never know what random horrifying medical disasters you might find in there.

I’m also still very hoarse and scratchy as well as super congested. I haven’t been sleeping well because I wake up rattling and coughing multiple times overnight. But I’m a little less tired with the higher dose of Prednisone, and I think my oxygen is doing a little better this week.

I know none of this sounds great, and honestly I’ve been having a pretty cranky week. But despite all this, I did have one really good day, because 2 friends who couldn’t make it to the party made a special trip in from out of state to visit me! Sarah and Anna joined forces to drive in from Cleveland and Pittsburgh for an awesome roommate reunion. And unlike the actual party, we got to spend hours chatting and hanging out together, which was wonderful! I’m so lucky to have such terrific friends!

Also, Sarah wrote a thing about me, so I’m basically famous now. Get in line for autographs everyone!

www.ou.org/life/inspiration/the-40th-birthday-bash-appreciating-each-day

Ups and downs

The good news is, the “leaky pipe” issue FINALLY seems to be under control. During the last 3 weeks I’ve only had one brief incident of coughing up bloody mucus, and that was a week and a half ago. After on and off bleeding at various levels of severity for a month and a half, I’m almost afraid to talk about finally making progress, so here’s hoping I didn’t just jinx myself into a bloody disaster. Listen lungs, you don’t need to prove anything, just be chill!

I had a couple good weeks where my lung function was on an upward trend and seemed to be stabilizing. Despite the bleeding issues, my endurance was better and I barely needed to use supplemental oxygen. Unfortunately, that all came crashing down 2 weeks ago. After a day of increasing hoarseness, fatigue, and shortness of breath, I suddenly spiked a fever. I was convinced that I had caught a virus, but fortunately the fever only lasted one night and I didn’t develop any other viral symptoms. Despite that, since then I‘ve been extremely fatigued and even mild exertion sends my heart rate up and my oxygen level down. So I’ve been back on the supplemental oxygen train while we try to figure out what’s going on and what we want to do about it.

A few months ago if I was feeling like this they probably would’ve thrown me in the hospital. Now, nobody’s rushing to do anything drastic, since I just finished such a long course of IV antibiotics with so little return. I’m also reluctant to increase my steroid dosage, since I finally got the Prednisone down to 10mg and would REALLY like to get off it entirely due to all the side effects. Today we added in some IV fluids to see if extra hydration will help break up the chest congestion and improve my lung function. I have a follow up appointment on Friday, so we’ll see where things stand and figure out our next steps then.

Despite all this, I have been using most of my limited energy for very exciting party planning! Only half a week left until my big blowout 40^th birthday bash! I can’t believe how many people plan to come, and how many people have donated to the GoFundMe! I am so grateful and can’t wait to celebrate with everyone!!

They see me rollin'

I’m still dealing with what I’m now thinking of as Leaky Pipe Syndrome. Thankfully I haven’t had another full bleed, but I keep randomly streaking. Yesterday I finally managed 48 hours blood-free and resumed one of my treatments, only to streak at the end of the day and be forced to start all over again. Previously we wouldn’t react so strongly to just a little streaking, but my recent history of Big Dramatic Bleeds makes everyone anxious so we’re being very cautious. I have to keep reminding myself to Be Very Chill and not do anything to elevate my heart rate, which these days basically means not doing anything at all exerting. I am not enjoying being so limited and feeling this fragile.

Today I got my first experience using a motorized cart at Shoprite. I really wanted to actually go food shopping instead of trying to figure out what I needed online, but I also knew it definitely wasn’t safe to do that much walking. So I swallowed my pride and grabbed a motorized cart. I did feel awkward about looking perfectly healthy while puttering around on a scooter though, so I kept my oxygen on the whole time even though I didn’t really need it once I was sitting. I figured no one would question the girl with the nasal cannula. I managed to only run over Katherine’s foot once, and though I ALMOST knocked over a couple displays, nothing ACTUALLY ended up falling. Using the scooter ended up being a weird combination of fun, annoying, and occasionally slightly terrifying. Those things are harder to drive than you’d think!

Katherine has been amazing through all of this craziness. Since I’m mostly incapacitated, almost all the household tasks have fallen to her. I can put together a shopping list and zoom around on my scooter picking things out, but she’s the one shlepping it all home and putting everything away. Not to mention cleaning, and laundry, and just about all the things that keep a household going. I feel terrible about not being able to contribute much, but she does it all without complaint and reassures me that she’s happy to take care of things. I’m not sure how I got so lucky!

Of course, she did get entertainment value watching me almost take out an entire cereal display with my scooter, so maybe it’s a fair trade. Here’s the photographic evidence she took of my Shoprite experience. Maybe next time she’ll get video!

Not again...

I guess I should’ve gotten an update out yesterday before anything had a chance to go wrong…

I felt noticeably better over the course of the weekend. My heart rate didn’t shoot as high with mild exertion, I was able to maintain oxygen without using the concentrator, and I had a little more energy. At my appointment on Wednesday my pulmonary function was up to 52%, which made me very happy. Overall we were pleased with how things were going and I seemed to finally be on a stable, upwards trend.

But apparently this rollercoaster isn’t over QUITE yet. This morning, after 2 full weeks with not the slightest hint of blood in my mucus, I suddenly coughed up 15 mL of pure blood out of nowhere. I literally yelled “NO!!!” as soon as it happened, because I was so frustrated to once again take a step backwards right when things were looking up. Fortunately it was less than the bleed from 2 weeks ago and wasn’t close to ER level, but I still can’t help feeling that my lungs are like dilapidated, rusty pipes right now, just springing leaks all over the place without warning. So once again I’m holding various treatments until I’m completely blood free for 48 hours. Unfortunately this time I’m not on any home infusion, so I don’t have that extra support to help make up for the missing treatments. Hopefully the bleeding will stop soon and I won’t lose too much ground.

I also got to do a super fun barium swallow yesterday to see whether I’m aspirating things into my lungs when swallowing. Fortunately it turns out that I’m swallowing fine, but I’m definitely having significant reflux (which we already knew), and there’s concern that I may be messing up my lungs by aspirating that. I’m already on a bunch of reflux meds, which control the acid level but can’t stop the reflux from coming up. We started exploring surgical options a few months ago, but put that on the backburner while dealing with respiratory issues. Once we get things stabilized the plan is to continue testing and consultations to move towards surgery and see if that helps with some of the respiratory issues.

On the plus side, before things went south, Katherine and I took advantage of the good weather and my relatively decent health to go to the Mummers Parade! I’ve been watching this Philadelphia New Year’s Day tradition on TV since I was a kid, but never actually made it there in person. We shlepped the wheelchair on the train and joined the crowds at Broad Street and Sansom to watch the string bands perform, and had a great time! And one of the mummers even gave me his parasol, which was super cool!! Apparently looking pathetic in a wheelchair has its advantages!

I’m definitely frustrated about backsliding once again, but I’m glad it at least waited until after I squeezed in a great experience! Hopefully that was the last hurrah for this CF exacerbation, and I’ll get back on a steady upwards trend soon!

Almost free

B”H I finally stopped bleeding and was able to gradually resume all my treatments without incident. I was scheduled to finish my IV antibiotics on Friday, but we ended up stopping a day early because the ringing in my ears got slightly worse. The tinnitus originally started back in January following a long course of Tobramycin, and gradually improved over several months, but never faded completely. We knew doing another round of Tobramycin was risky, but we also really needed an antibiotic that might make a difference. Fortunately the ringing is still mild and mostly unobtrusive, and hopefully will improve again now that I have stopped the Tobramycin.

The plan now is to finish the last of my IV fluids on Friday and see how I do at my follow up appointment on Wednesday. I had the option of continuing just the IV fluids, but after 2 continuous months of IV infusions I needed a break. Aside from Prednisone interfering with my sleep schedule, I never sleep well while on IV antibiotics since I have to run infusions every 8 hours. So 2 full months of IV antibiotics also means 2 full months of poor sleep, even after my symptoms improved and I got out of the hospital. I am very much looking forward to NOT waking up to any alarms!

I’ve been extremely fatigued this week, but I don’t know if it’s from lack of sleep or if it’s because we’re slowly decreasing the Prednisone and I’m not quite as hopped up on steroids. My appetite has also slipped without the Prednisone munchies, but unfortunately I’m still stuck with uncomfortable Prednisone bloat. If only Prednisone would let me choose my side effects! We’re currently in the process of getting me started on a monthly injection for severe asthma, which we hope will control my asthma-like symptoms enough to allow me to get off the steroids completely for the first time in 2 years. Hopefully soon I can finally say goodbye to Prednisone side effects!

The big question is what will my pulmonary function numbers look like on Wednesday after a few days without IV infusions. Here’s hoping that I managed to regain a few more percentage points, and that a few days of sleep will do me a world of good!

One more week

As I predicted, we’re keeping me on IV antibiotics for another week. I was surprised, however, to hear that my doctor originally expected to send me back into the hospital at yesterday’s appointment. Fortunately, I looked better than she anticipated, and my chest didn’t sound any worse (though it also didn’t sound any better). She would have stopped the IV antibiotics yesterday, but I could use the extra support while recovering from the recent bleed, particularly since I’m still holding chest PT and half my nebulizer treatments. If I can just stay blood free for 48 hours I can gradually resume those treatments and get back on track with clearing the congestion out of my chest.

The good news is that there is FINALLY an end in sight. As long as nothing stupid happens within the next week, we will finally stop the IV antibiotics next Friday. Depending how I’m feeling we might continue the IV hydration, but we might also just give me a break from everything, since by then I will have been on IVs for more than 8 weeks. The nice thing about the port is that giving me a break is a viable option since it’s so easy to just re-access it and resume IVs if necessary. Though I wish I had seen a stronger response to treatment, I am very much looking forward to moving on from endless IVs and trying to put my life back together.

Yesterday, for the first time, I went into a store with my new oxygen concentrator. It was an interesting and conflicted experience. I definitely appreciated the fact that the oxygen made it easier for me to move around without huffing and puffing. But I also had conflicting thoughts and feelings about making my until now mostly invisible illness suddenly and inescapably visible. On the one hand I appreciated and on some level even enjoyed the validation of having something external to match the internal struggle. There have been many, many times over the years when various every day tasks were extremely difficult for me, and yet you would never know it from the outside. It can be very frustrating to push through your day feeling like a train wreck while looking like the picture of health. On the other hand, it felt very odd to walk through the store with a blatant sign of severe illness strapped to my face, knowing that everyone who saw me instantly knew that something was very, very wrong. I found myself wondering what people saw when they looked at me, this baby-faced girl afflicted with some terrible mysterious illness, a tragic image to tug at your heartstrings. I wasn’t entirely comfortable with strangers being able to immediately categorize me as the sick girl without knowing anything else about me. But that won’t stop me from living my life and using the medical supports I need to do so. It was an interesting experience, and I’m sure I’ll get used to it with time.

Hopefully my lungs will behave themselves and stop. freaking. bleeding. so I can get back on track with treatments and exercise and continue clawing my way back to health!

Frustration

Things seem determined not to go smoothly this time around.

During the last few weeks I’ve been sporadically coughing up small amounts of blood. This week the bleeds were a bit more frequent and slightly more severe, which is concerning as previously that pattern led up to a big bleed that landed me in the hospital. The normal response to bleeding is to hold some of the treatments that can be irritating to my airways, but we’ve been hesitant to do that as I’m still very congested and really need those treatments to help pull me out of this exacerbation. It’s a catch-22: the treatments could provoke a bleed, which is bad for my lungs, but holding treatments increases congestion, which is also bad for my lungs. Increased congestion can also lead to increased infection, which can provoke bleeding. There’s no good answer here, and I’ve pretty much been walking a tightrope, hoping not to fall off.

Last night things finally gave way, and I coughed up 25 mL of pure blood. Thankfully that’s not ER level, but it’s significant, and left me no choice but to hold treatments. Currently I’m under orders to hold treatments until my mucus has been COMPLETELY blood free for 48 hours. The 48 hour countdown hasn’t even begun yet, as my mucus remains blood streaked, though thankfully far less so now than this morning. So I’m already 1 day into holding treatments, and have at least 2 more days to go, if not more. Each day without these treatments means I’m less able to clear the congestion out of my lungs, and makes it more likely that I’ll lose the little ground I’ve managed to gain over the last several weeks of IV antibiotics. I also can’t exercise or do anything to elevate my heart rate, as the extra pressure on my weakened blood vessels could trigger a bleed, so any efforts to regain my strength and get back to normal life have also been put on hold.

Honestly, I am extremely frustrated right now. I have now been on IV antibiotics for 7 weeks straight, and will probably be going into week 8. I have a follow up appointment tomorrow, but we can’t even do pulmonary function testing because the effort involved could provoke a bleed. I’m pretty sure they’re going to keep me on antibiotics for at least another week while we get the bleeding under control, so that will be 2 full months of treatment. It was already upsetting that with all this treatment I’ve made such limited progress. To now be forced to stop the treatments and exercises that could help me move forward and watch at least some of that progress be unraveled is just infuriating. On the other hand, I’m also terrified of having another massive bleed like I did in February and July, and I REALLY don’t want to go back in the hospital. I’m caught between a rock and a hard place with no way to win here.

I’m tired. Hopefully things will get better soon.

Holding steady

Well it was definitely a FRA-lichen Chanukah (thank you Shira Rubin for the wonderful wordplay)!

It is sooo good to be home! I’m still not getting excessive amounts of sleep due to coughing and needing to run an IV every 8 hours, but I am far more rested than I was in the hospital. And of course the food situation is greatly improved! Week #2 of the Fancy Restricted Antibiotic, however, hasn’t been quite as dramatic as I would’ve liked, and medically I’m basically at the same place as I was when they discharged me. I’m still dealing with a fair amount of congestion and tightness. My oxygen levels mostly hold steady when I’m sitting still, but slide with the slightest exertion. Unfortunately for me, they only slide to juuuust over the cutoff point for supplemental oxygen. Which means it’s difficult for me to manage basic daily living tasks, but I also don’t qualify for the in-home supplemental oxygen that would make things easier. It’s a bit of a catch-22: I know I’m deconditioned and need to exercise, but I don’t have the supplemental oxygen that would make exercise easier. I do have a possible lead on that issue though, and it may be resolved within the next couple days… Stay tuned for further developments!

Another catch-22 is that for the last week I’ve been having sporadic episodes of coughing up small amounts of blood. The usual response to that is to hold some of my more irritating treatments to allow the wound to heal – but those treatments are also clearing the congestion out of my chest. Clearing congestion helps reduce infection, which helps reduce the risk of bleeding. So we’re walking a delicate balance between not provoking a bigger bleed – because G-d knows I don’t need any more drama in my life! – and not undoing the progress we’ve made in clearing things out. Oh, and all my bleeds in the last week have followed more significant exertion, since an elevated heart rate puts more pressure on the blood vessels and makes it more likely that any weak spots could give way. Apparently I need to just be very chill for the next however long until things finish healing… which doesn’t exactly help with the whole trying to exercise and not become even more deconditioned thing.

Of course I can’t just be boring and straightforward about things, everything has to be complicated. I’m so high maintenance.

My follow-up appointment is on Wednesday, and I fully expect at least another week of treatment, possibly with yet another antibiotic. My lungs are being so very stubborn this time around (which I guess shouldn’t be a surprise, they are MY lungs after all)! I’m super curious to see what my pulmonary function testing looks like. I know I’ve improved from that dismal 45% a month ago, but I also know I’m not back to my usual 60%. The question is how much progress have I managed, and how much more might I be able to accomplish.

So, not exactly the glowing update I would have liked to share, but also not terrible. Holding steady is definitely better than going down! And being home is definitely, DEFINITELY better than being in the hospital!!

Drama free and moving on

You know how some places have those signs, “This workplace has been incident free for x days?” Katherine and I decided that I should have one for bleeding. Current count: 17 days!

Since I’ve managed to stop bleeding and my lungs finally sound clearer, I’m finishing up one last day of IV antibiotics and fluids and then calling it quits for this round. After being on IVs for a full month, I am very much looking forward to freedom and a full night’s sleep! Next task: Getting back to pulmonary rehab and rebuilding my stamina. I’ve been trying to keep somewhat active since the bleeding settled, so hopefully I haven’t deconditioned too much. I’m also hopeful that maybe I’ll manage another few months of relatively good health like I did after the last round of IV antibiotics. Fingers crossed!

One less exciting bit of news is that I’m starting to show side effects from long-term steroid use. A scan showed that my bones are thinning, not quite to the point of osteoporosis, but definitely on the borderline. It’s not a huge deal, but it’s something to be aware of. That should become less of an issue as we slowly taper the Prednisone down, and hopefully I’ll be able to get down to a maintenance dose that’s low enough to have minimal long-term impact. In the meantime, I’ll be starting medication to help rebuild bone density and hopefully prevent osteoporosis.

On the other hand, I also got some unexpectedly good news! Apparently by the end of May I reached the out-of-pocket maximum on my medical benefit, which means medical is now covered at 100% and I no longer have any medical copays (though I still have prescription copays, as the pharmacy benefit has no out of pocket maximum). I was surprised, as I have spent less than half of the $6,700 I was anticipating. It turns out that some of my more expensive specialty medications are covered under the medical benefit instead of the pharmacy benefit, so those copays made a significant dent in my out-of-pocket maximum. Even better, I have a grant that covered those copays, so I didn’t even have to pay them myself! Having my out-of-pocket maximum essentially halved was a HUGE relief and will make the rest of the year MUCH easier. Additionally, I got two other prescription copay assistance programs set up, and my pharmacy expenses finally hit the catastrophic coverage phase, which means that my prescription copays have dropped significantly. Now that I have everything set up, next year should be much smoother financially!

It’s been a difficult summer, but B”H things are finally looking up. Looking forward to getting back on track and hopefully squeezing in a few good months before the next round!

Enough already!

Oh for %@&#$ sake…

Yesterday I pulled myself together and left the house to run a few brief, low-key errands. I walked slowly in two stores carrying nothing heavier than my purse. I didn’t do anything that should have been overly stressful for my body. But after leaving the second store, before I could make it to my 3^rd and final errand, I suddenly started coughing up blood again. So there I was, sitting in my car in a random parking lot, spitting into a cup, hands and face streaked with blood, praying that no one would notice me and freak out. Fortunately I could tell that it wasn’t going to last long, and 3 minutes and 20 mL later it was over.

You know your life is weird when your first reaction to coughing up blood is to be immediately, over-the-top FURIOUS. If I had the energy or lung capacity I would have been swearing at the top of my lungs. Instead I had to be content with raging inside my head and in my text messages to poor Katherine. Since it was a relatively small bleed, once things settled I was able to clean myself up and drive home, and I did not need to go to the hospital. But now I need to once again hold various treatments to avoid causing further irritation to the wound. And apparently I need to basically put myself on house arrest for the rest of the week until I’m more certain that everything has healed.

I’m not really sure how I’m supposed to move or kasher a kitchen when I can’t even lift anything or leave my apartment. It has been an extremely frustrating month, with no end in sight. I’m usually pretty zen about most of this stuff, but my patience is wearing thin, and I am not a happy camper. Here’s hoping this was my lungs’ last hurrah for this round and that I can get back to my bizarro version of normal life soon.

Making progress

B”H I’m recovering much faster this time than I did in February. The congestion already sounded better just one day later, and I have way more energy this time around.  We think the fact that I was already on IV antibiotics for 4 days beforehand really helped. We added chest PT back in yesterday afternoon and I’ve tolerated it well, so we’re adding in a nebulizer med today. If that goes well, we’ll add in the last nebulizer med tomorrow, and if THAT goes well I could be discharged on Sunday. Fingers crossed!!

I did get a great deal of twisted pleasure in showing off my Super Dramatic Cup o’ Blood (sometimes referred to as my Cup o’ Drama) to various medical staff for the first couple days. Hearing about 80 mL of blood is one thing, SEEING it is another, and it’s so much more fun to answer the question “So what brought you here?” with eye-opening props. I also learned that I get an even better reaction when I tell them the time frame, as in “That all came up within 5-10 minutes,” for maximum shock value. Hey, I’m bored, gotta get my kicks where I can!

Before this year I only ever had severe bleeds twice in my life, with the most recent one being a few years ago. Given that, I didn’t expect to repeat February’s drama any time soon. But apparently horking up mass quantities of blood is my fun and exciting new hobby (why yes, I DO need to find better ways to occupy my time). It’s particularly exciting in that it’s COMPLETELY unpredictable and can happen at any time without warning. Unfortunately, the only thing you can really do to prevent bleeding in the lungs is try to prevent infection. Obviously that’s already one of our primary goals and is the basis for most of my daily medical routine, but we’re probably also going to be a little more attentive to smaller bleeds from here on out. While previously we could take the time to monitor small bleeds to see if they became more significant or were accompanied by additional symptoms, now we’re more likely to throw me right onto IV antibiotics as soon as I start showing any pattern of bleeding in order to reduce the risk of having another major bleed. I’m not thrilled about that, but I’ll take a couple weeks of home infusion over all this drama any time!

In other news, what’s a hospitalization without… Pole People! I hope you enjoy the latest crew!

One step forward, two steps back

Just when things were heading in the right direction…

On Monday I finally started getting some of my energy back. Prior to that I had been so utterly wiped out that I often napped twice a day, and even with that I still felt completely drained. But on Monday and Tuesday I actually woke up a bit and began to feel like a semi-functional human being again. I was hopeful that things were turning the corner and that I was pulling out of the CF exacerbation.

And then the other shoe dropped.

All month I’ve been having mild issues with coughing up blood. I had a couple days of blood streaked mucus, then a small bleed of 5 mL… a couple more days of streaking, then another small bleed of 10 mL. I was a little concerned that maybe I was ramping up to something bigger, but I was NOT expecting to be hit with an early morning massive bleed out of nowhere. It was close to what happened back in February, and I almost filled a specimen cup with 80 mL of my own blood within the span of 5-10 minutes. Fortunately, though I knew it was bad, I could feel that this one was a little more under control. I was pretty sure that it would stop fairly quickly – and I was right. But I also knew that it was bad enough that it was time for another trip to the ER. So Katherine got to wake up to a really fun 5 am phone call and take me to HUP. Don’t I take her on the BEST dates??

Based on February’s drama, I knew they were going to admit me, so this time I came prepared with a suitcase and supplies. Fortunately, a CT scan did not show any active bleeding, though it did show a small amount of pneumonia. Presumably the infection wore away at a blood vessel in my lungs, but B”H it seems to have sealed itself right up, so hopefully I won’t have any further drama. Just like last time, we’re yanking a bunch of my daily treatments that could irritate my airways and cause further bleeding, and will monitor how I do as we slowly add them back in over the next few days. We’re also continuing the IV antibiotics that I had already started to address the pneumonia as well as reduce the risk of additional infection.

Unfortunately, HUP is pretty busy, and they aren’t sure they’ll be able to get me into a bed upstairs until tomorrow. Which means spending the night in an ER stretcher. At least I was lucky enough to get a cubicle; they’re so busy today that some people are on stretchers in the hallway. I will be very glad to finally get upstairs and settle in, hopefully soon!