Managing Cystic Fibrosis — Hospital Tune Up and A New Goal

This year was a low point, and a high point, for my health and managing my Cystic Fibrosis.  This last year I dealt with more than I have in a while .  For the first time in 11 years, I needed to go into the hospital for a “Tune Up”.

I really do consider myself lucky in that I have a much more mild case than most patients with CF.  Over the course of my 33 years, I’ve only had to go in to the hospital for three tune ups– not counting my initial diagnosis at the age of four months old.  My health, my lung function and digestive system in particular, stayed relatively steady and even for many years.  And, most likely due to the amount of exercise, I maintained fairly well even through college when I was hospitalized.  But since then, very slowly, sometimes almost imperceptibly year to year, my lung function has declined.  This year I hit my lowest point ever in August.

Every three months I go to UVA for their adult CF clinic.  I have been at this same clinic since I graduated from college, and have seen many changes take place in the office.  One of the major changes occurred about a year or so ago when my doctor left and they brought in two new doctors.  The new Doctors, two extremely competent and very nice women, were more aggressive in wanting to treat me.  And honestly, with a disease like CF, you need to be aggressive because it is too easy to let things slide (and your health can go down very quickly).  They expressed concern over the trend in my PFTs (Pulmonary Function Test) and so we started trying different drugs and regimen changes to increase my FEV1 percentage.  FEV1 is the measurement of air I can expel in one second.  I couldn’t see much difference in any one change, and by August I was down to 41% FEV1.  I had been hovering around 50-53%, but this was too low and they strongly urged me to take a course of IV antibiotics.

This was hard to hear, because I really did not want to spend a week in the hospital.  My previous visits were to stay a week in, while they tweaked my medications and monitored any progress, and then send me home for a week and continue treatment.  Adult clinics do things a little differently and my doctor promised that I would only be in for three days max. while they correct the dosage.  In and out, and then on a home course for two weeks.  Sounded manageable, but I was also right in the middle of selling my house and buying a new one.  The timing was awful, and I wanted to think about it, and maybe come back in a month (after everything should be settled) and then start treatment.  Soon after coming home from that visit, my friends and family urged me to reconsider and go in as soon as possible.  I acquiesced.

And so that’s how it happened that I happened to be on IV antiobiotics AND buying a house AND selling a house all at the same time.  And not any one of these things was easy or simple.  I already blogged about buying and selling my homes, and if I had to do it all over again I would.  I LOVE our new house and yard, I LOVE our location, and I LOVE having a short commute (I consider 15 minutes compared to the hour+ each way is a major improvement).  It was all so stressful, but I made it through and with flying colors I think… (at least no one has told me that I was a complete crazy person).

The only easy, simple thing out of the entire process of going in for a tune up at the hospital, is that somehow everything was already set up and all I had to was walk in.  I don’t remember signing more than one liability waiver when they put in the PICC line (as an outpatient) and maybe one to authorize setting up a home health care supply.  No one asked me about insurance (because it was already in the system), and I didn’t have to pay a deductible while I was there.  I thought this is what living in a different country must be like…but as I said, this was the ONLY simple thing.

I called up my doctor to schedule time to go in a week after my clinic visit.  Originally, I was supposed to go in on Friday (they would call first) and I would get my PICC (peripherally inserted central catheter) line in place first, and then go up to my room to get started.  Unfortunately, the bed they thought would be vacant was not, and so a decision had to be made. Put the PICC line in anyway and hope the bed frees up when they expected it to Saturday afternoon OR wait until Monday because the staff doesn’t generally put in PICC lines in over the weekend.  Not wanting to put this off any longer than I had already, I opted to go in and have the PICC line placed and then go home and wait for the call.  Late on Saturday afternoon it came in and we arrived around 7 PM.

PICC line (source)

This is what my PICC line looked like- a statlock device (non-sutured please thank you!) with two lines.  The procedure went well, and I was so happy to find that they were not placing the line directly in my elbow (just above it) so that I could actually bend my arm.  Two plus weeks not being able to bend your arm is NOT fun.  I’m sure anyone who has broken an arm has felt similarly.  And yes the lines are that short- but once I was home they added extensions to them so that I could flush them and administer my medications myself.

Once I had my bed/room at the hospital (thankfully a single room) they immediately started going through the rigamarole.  Lots of questions and histories, and it took the staff about an hour to figure out that my Doctor (who had gone home for the evening) had already put in all the orders. Hospitals, in my opinion, need to have open communication as much as possible. Information needs to be easily and readily available and if there was a way to digitize everything even more I think it could only help. I feel as though they should have been aware why I was there to begin with, but instead I had to explain it again to every. single. person. The weird thing was, I didn’t feel particularly bad. I felt the same as I had in months, if not years- and many people could not understand why I was in the hospital if I didn’t feel bad. I was there because my PFTs were low and I needed a boost.

The first night was awful- I didn’t get much sleep and they treatments schedule was something like 8 PM, 2 AM, 6 AM, 10 AM, etc… Since I was on two different medications, and they were on two different schedules, I was tethered to an IV pole quite a bit.  The food was truly awful. When I find food dissatisfying or unappetizing, I very quickly lose any appetite. After a while, if I haven’t had enough to eat, I get cranky and irritable and stressed out, and then seeing the food from the hospital stresses me out and becomes a cycle of stress. Thankfully, Mr. Lucky was there for me and came to visit every day I was in the hospital. He also went out and brought me snacks so that I could feel a little more like myself. (I, unlike many other kinds of patients, didn’t have a restriction on food because my digestive system is not like most peoples.  Because I don’t absorb all the nutrients or calories from the food I eat (even with my meds) I can lose energy and weight very quickly if I don’t do what my body tells me to eat.) I suppose I could have, but never thought to ask the nurses to bring me anything (can you do that?).

Something I found amusing in a slightly annoying way, was that not only did they NOT have all the medications on hand that I routinely take every day, but the nurses didn’t necessarily understand the labels either. I take a prescription called Creon, which is a pancrelipase, and helps break down the food I eat for absorption. It replaces the digestive enzyme that comes from the Pancreas since most (or all) of that enzyme is blocked in my system from going through the proper channels. (Another common symptom of CF).  I take this medication with my food, when I eat my meals. The nurse thought I should take this medicine with food, meaning, I need to take it whenever she gives it to me (all hours of the day, whenever they get a chance) and eat food with it.  Umm…no.  It’s not a drug I need UNTIL I eat. I’m not sure this one nurse ever got that.  Thank goodness I had my own pills on me so I could take them when I needed them…

So everything was going fine. I was trying to stay upbeat- just a few days and then I could be home!  My parents were coming to visit very soon, and there were some time crucial documents I needed to deal with to buy my current house. The levels of the antibiotics were good–they had adjusted them as necessary. Not surprising (to me anyway) was that my liver does not process Tobi (tobramycin) as fast as expected, so instead of one dose every 24 hours, I needed one dose every 36 hours.  Not a big deal, but it also means they just increased my treatment period for a week.  This was not surprising to me because I felt as though I started having vertigo the last time I took Tobi (inhaled solution).  It may actually not have been the Tobi, but it is a known side effect (it messes with your inner ear when it builds up in your liver- you can become auto-toxic) and I did not want to take chances.  So I had a balance test and an hearing test which pointed to no permanent damage, which means that occasionally if I need Tobi, I can take it.

As the Monday morning became Monday afternoon, I started freaking out because no one was talking about me going home.  Monday was supposed to be the day! Early afternoon they finally came in to explain why they wanted to keep me an extra day. My initial blood results tested for a gram positive stain- meaning (supposedly) I had a blood infection. This was very serious they explained, and they needed to keep me an extra day to keep an eye on me. Now I understand that they were covering their butts on this, because the hospital could be found negligent if they allowed me to go home and then something serious happens to me. However, I felt that it did not make any sense whatsoever, and now I would have to pay for an extra day “just because”. This is why I didn’t buy it: 1. I didn’t feel sick.  All morning I had several doctors (including a gaggle of students) come in and ask how I was feeling. Not only did I not feel sick, I actually felt great! I was getting stir crazy and wanted to go home. I don’t think any of them believed me because my blood sample said differently.  2. It is possible to get a false positive on this test. Admitted to me by the Doctor who was handling my care, sometimes there is a mistake. My sample could be contaminated another way, or there could be a lab error, or sometimes they test positive when they are not.  Considering how confused the nurses were when the took my samples (and took a full five minutes to make sure they got all the labels right and then some…) and how earlier that day someone else’s vial of blood was dropped in my room, I can see human error accounting for many mistakes.  And lastly, 3. I was already being treated for a blood infection. Granted, I was taking IV antibiotics for something else, but the end result would be the same. My blood tested positive on Saturday night when I was first admitted, and started IV’s right away. Theoretically, if I had an infection, it would have been treated immediately and I should have been fine by Monday. The Doctor said that most infections, when treated quickly, usually one takes a couple of days to clear up.

I was livid. I now felt as though I was being held hostage to their insecurities. They had failed to approach me about this earlier (or purposely waited) so that I would have no choice but to stay–unless I wanted to go without IVs for a couple days because it was too late to set up the home health care team. I was very tense and terse with the Doctor and gave her a piece of my mind. I tried to be kind to everyone else though, because I know that it’s not their fault I was still there. I know it’s not really the Doctor’s fault either (it’s probably hospital policy) but I felt like she should know better. I told them I would not be coming in again for any kind of treatment, but I might revisit that absolute in the future.

With my parents arrival the next afternoon, I made it clear that I was to get out of there ASAP the next day, and wanted to leave by noon. The Doctor agreed upon my new lab results being clean (which I told her I was 100% sure they would be- and they were).  To their credit, everyone did pitch in to help my make that goal (I ended up waiting close to an extra hour though for my first doses of take home IVs).  Finally free! Mostly…sort of.  I had some limitations including not being able to lift anything over 5 pounds with my PICC arm or lift anything over my head. My boss very generously told me to just stay home the whole time, but three weeks is crazy and I knew there was stuff I could do.  So on days that I did not have an 11 AM Tobi treatment, I went to work.  While doing the IVs we also closed on the house, and I managed to hire movers (since I couldn’t lift anything). The PICC line came out the day the movers showed up.  Also, Mr. Lucky did a whole bunch of moving stuff for us as well (thankfully!).  I felt weird and useless for not being able to do more.

The day after we moved in, I had my follow up clinic appointment.  They were very happy with my results- I gained 10 points on my FEV1 and I was back up to 51%.  That’s better than they hope for, and even told me that they expect that number to dip back down again at my next clinic visit.  In the meantime, we decided not to follow up with any Tobi inhaled solution to just see how I do. Fast forward three months to mid-December at my next visit, and my FEV1 is still at 51%.  That’s great!  They are ecstatic about my numbers, because my weight is also up a few pounds and the big kicker is my total lung volume. Before hospitalization, my total lung volume was hovering around 65% I think. After the tune up it shot up to around 80%. This December that number was at 97%.  That means I have a total capacity is 97% predicted of someone in my height/weight/age range which is almost science fiction sounding. BUT my FEV1 is still low- remember that’s the number for getting air out fast.  My Doctor explained it by saying that it means I have obstruction in my lungs that is preventing that air from coming out. So for now, the only change to my regimen is to increase the strength of Advair. I’m also going to have an albuterol test (before and after) to see how much of it could be asthmatic conditions.

But how did my total lung volume jump up? One word- exercise. Feeling better after my course of IVs, I started moving at least 20-30 minutes at least 5 days a week. My respiratory therapist gave me an A+ and I am encourage to do even more. I know I can, I can feel it. My goal this year is to try to get my FEV1 up to at least 60%.  It may not happen- it may not be able to happen- but I’m still going to do everything I can! My routines vary every day- some days I do yoga (which is great for working on the lungs and breathing), other days I do Wii Fit, and other days I walk on our treadmill. I recently ordered Zumba for the Wii and hope to rotate it in two days a week (hopefully). I do feel better overall, and I know that I can tolerate more now– walks outside in the colder air, running through the airport to catch my flight, and generally recovering faster when I have a coughing fit. I am hoping that if I get sick- with a cold or some relatively minor ailment- that I will be able to recover faster as well. Thankfully, I haven’t had to deal with many changes in my digestive system– although I am trying to incorporate more vegetables in to my diet.  And I was recently tested again for CF related Diabetes, and passed my 2 hour glucose tolerance test with flying colors.

Whew. Thanks for letting me get all that off my chest. I know it was a long read, but I just wanted to say it. Looking over it all, it keeps hitting me how fortunate I am that I am in a position to do these things for myself, that I could go into the hospital (and afford it), and that my health really is OK. Just last week I met a girl (whose father I know in a professional setting) who is about ten years younger than me (maybe a little more) and she’s doing OK too, but she had a lung collapse this last year. It didn’t get better on its own and she had to have surgery. I wanted to give her a hug (but that’s a no no for CFers to get too close to each other – what with the communicable pathogens and all).  I am so thankful for all that I have, and I hope never to forget it.