Tomorrow marks the start of my second month on the new CF drug, Orkambi.
It’s been one crazy month. Just as I started the Orkambi, I ended up having a flareup of my bugs. It came on kind of slowly.
Prior to starting Orkambi, my doctors warned me that I could experience side effects, so for the first week, I chalked up most of the symptoms to the new medication. But my husband, in his usual crazily-accurate assessment of my symptoms, tried to convince me to contact my doctors. I finally did, when my fever spiked to 103.5, which definitely wasn’t listed on my list of symptoms to monitor.
I contacted my doctor – according to my recent sputum test, MRSA was the biggest culprit. The other bugs that showed up – two strains of Pseudomonas Aeriginosa – are growing, but in lower quantities (measured in number of colonies that grow in a petrie dish). However, there are NO oral antibiotics that can treat those strains, so until/unless they start overpowering me, we have to hold off on treating them until I really need them. In other words, until they are the cause of me being very sick.
My doctor wanted me to immediately have Pulmonary Function Tests* (PFTs) and blood work done to check the level of inflammation in my body (the test confirmed I had a high level of inflammation). Doing that blood test would help discern if my symptoms were mostly due to infection or possibly from my new medication.
*Pulmonary Function Tests measure various levels of lung functioning.
We made the trek to the hospital and I sat down for my PFTs. I usually do 3 attempts – blowing into a machine that checks the amount of air I can inhale, exhale in 1 second, how long I can exhale, and a few other variables. I almost burst into tears when I saw my results. My overall lung function had plummeted to the same low level as they did prior to my last hospitalization. I had dropped 34% of my lung function in just a few weeks.
I was devastated.
- I was so sick. Cough, junky lungs, fever, exhaustion, headaches, shortness of breath, oxygen deficits, chest tightness. My oxygen/blood was focusing on my heart and lungs, and I could feel parts of my cognitive thinking lacking. A few times, I got confused and “off”. I was clumsy. I was a mess.
- I was scared that I would not get back the level of independence I felt I was losing. One day, taking a shower wore me out so badly that I almost passed out. My lungs were on fire. I needed my inhaler which was two rooms over. I was alone, and very scared. I experienced something similar to a very scary episode of respiratory distress, and I was terrified. Once I got my inhaler and inhaled, I was better, but I knew that I was too weak to take a shower alone. I needed Ken in the next room, just in case. And after I finished, he’d help me get dressed, lead me down the stairs with both hands, and get me back downstairs onto the couch (it was easier to sleep there for a while because of my breathing).
- I was very nervous that I would end up needing IV antibiotics, especially as it had only been 8 weeks since I finished my last dose in the hospital. If you recall, there was a huge problem finding anywhere to get IV access. The first few days, I blew several regular IVs and then my veins rejected 3 attempts; this was while I was waiting to get a PICC line. However, while in Interventional Radiology getting my PICC line placed, my scarred veins prevented them from finding a place in my arms to put my PICC. So they had to do a “tunneled PICC line” which is a bit more invasive, time-consuming, painful, and scary.
*Interventional Radiology is a team of experts who use minimally invasive
image-guidance technology to diagnose and treat conditions.
One of their specialties is placing hard-to-place IV lines such as PICC lines.
And Then Some Hope Arrives
Thankfully, my doctor offered me an antibiotic that often treats my MRSA well. I was happy for so many reasons, especially as I noticed some improvement in my symptoms over the first few days. At this point, MRSA had knocked me down with scarily-low dips in oxygen, severe exhaustion, and a cough that kept me up at night and exhausted during the day.
Fast forward two weeks of some slowly improving symptoms, and some issues that weren’t resolving.
I had a CF checkup on Monday, and approached it with great trepidation.
I had just finished two weeks on my antibiotic and felt I still had a way to go. I had no idea what would happen during my lung tests. But during an exciting round of PFTs, I found out that I have regained about 1/2 of what I lost in the past month – essentially the amount of air equal to the amount of water in a 500mL Poland Spring bottle. That is a LOT of air!
I still have a lot of work to do, and some hurdles to jump (still haven’t conquered the stairs yet!), but I left my appointment with hope.
I’ve had very few moments of optimism the past month. But now, my hope is renewed.
My doctor prescribed an additional week of antibiotic because I still had some way to go in getting over this infection, which was a great relief, as I really want to get back to where I was last month (which is still down quite a bit from my baseline). He also offered some other suggestions that might help with my still-pressing symptoms like exhaustion, drops in oxygen level, and chest discomfort/pain. Lastly, he told me that he hoped that I stick with Orkambi, because we are already seeing some benefits and expect to see more…
Oh, I haven’t got to that yet. YES! Thanks to Orkambi, the junk in my lungs, which is usually the consistency of glue, is now as thin as water. I also have been sleeping through the night, most of the night – something I rarely do – without having to beg for “10 more minutes” or worse, sleeping until 10 or 11 a.m. (Don’t judge, lol.)
During about 75% of the nights since I began Orkambi, I actually woke up feeling rested, and able to “get up” almost immediately. (Cuddling kitties or hubby doesn’t count.)
I am experiencing some side effects, but they are manageable (and for privacy reasons, I am not sharing them publicly, but I have reported them to the manufacturer.)
So now what?
So now, I continue to take all of my meds, do my Vest treatments, work on regaining lost strength (starting the treadmill tomorrow), and trying to get back to a level of functioning that allows me some independence.
Hope is an amazing thing. It has helped me recognize the progress I have made, and given me motivation to continue working on things and most importantly, continue to take Orkambi.
You never know how far you can go, and why would I limit myself?