Roid Rage

PrednisoneI’ve been on high-dose prednisone to  treat my pulmonary fibrosis since I was diagnosed two years ago. I don’t have a moon face or ankle swelling often associated with prednisone use, so I always figured I’d gotten off easy when it comes to steroid side-effects. Silly girl. I should have known nothing is easy or simple, especially for me.

When I broke my ankle two months ago, we discovered that prednisone has been eating away at my bones.  My teeth have suddenly become quite sensitive, another prednisone by-product. And now, the greatest gift of all — lousy healing. As the final cast came off my ankle last week, the top three inches of my incision was a gaping wound. Okay, gaping may be a bit of an exaggeration.  It’s maybe a quarter-inch wide and an 1/8-inch deep. Not huge, but not healed either.  Bone Doc wanted to take me back to the OR to debride and re-close the incision. But that would have required at least a regional block (too deep for a local) and more futzing with the Optiflow, not to mention increased risk of transplant-thwarting respiratory issues.  So, we opted to go to the Wound Care Center at St. Luke’s.  I’m so glad we did!

Wound Doc says it looks like it will heal fine — we just need to start using the appropriate dressing and give it 2 or 3 months. That’s fine by me. Every couple of days I goop the wound with polysporin and apply a hydrocolloid dressing.  The doc says the waterproof dressing creates a nice moist, warm environment to encourage granulation (what my mom used to call “proud flesh”), which forms the underlayment for new skin. The prednisone will slow things down, but healing will occur. Whew!

The other disappointing news from last week’s de-casting session was that I am still non-weight bearing on the ankle. Thankfully, Bone Doc says I can start putting my Frankenboot to use next Frankenbootweek, just in time to help in the kitchen for Turkey Day. But he wants me to hold off on physical therapy for a little longer to give my roid-ravaged bone some extra healing time.

My loathe/hate relationship with prednisone continues. But despite the bumps it’s added to my road, I still have much to be thankful for.  And, after my November 26 visit with the transplant team at Barnes, I hope there will be even more to celebrate.

Tips for Dealing with Steroid Side Effects 

  • Up your calcium intake to at least 1500 mg a day with a combination of calcium supplements and calcium-rich foods such as milk, cheese and yogurt. Remember to up your Vitamin D intake, too — 400-800 IU daily.
  • Weight-bearing exercise (like walking) will help stabilize bone mass and burn calories.
  • Steroids affect metabolism and fat distribution, increasing appetite and adding fat deposits to the abdomen and neck. Focus on eating a healthful low-fat, low-calorie diet to counteract weight gain.
  • Fluid retention is common with steroid use. Reduce (or eliminate) salt-use in cooking and at the table, and look for low-sodium versions of typically high-sodium foods such as: chips and crackers, soups, prepared foods, canned foods, dressings and condiments, and restaurant meals.
  • Steroids suppress the immune system, so get a flu shot, wash your hands frequently, and avoid anyone who is coughing or sneezing.
  • Tell your eye doctor and dentist about your medication, since steroids can cause cataracts or glaucoma as well as increased tooth sensitivity.
  • Other side effects to watch for and discuss with your doctor:  hip pain (may indicate aseptic necrosis of the hip joint), insomnia, mood changes, elevated blood pressure, elevated blood sugar.

Busted!

Image  PF has been an adventure for me in ways I never could imagine.  Not only have I experienced an illness once suffered exclusively by HIV patients (PCP pneumonia), and developed a perforated septum only a serious coke head could appreciate, now I’ve broken my ankle in a fashion befitting a fullback.  The xray above isn’t mine, but it looks pretty darn close to what i did.  And I blame it all on PF — okay, and some stupid choices on my part.

Last week I was up late doing my insomniac routine when I decided I’d get ready for bed and attempt sleep one more time.  I was on my way to the bathroom and noticed the bubbler was empty.  The bubbler is a small bottle attached to the concentrator that humidifies the O2, making it easier to breathe.  I bent over (mistake #1), unscrewed the bubbler and filled it, then screwed it back in place (mistake #2).  I was a little breathless but figured concentrator_cropit was because I’d just bent over (all those innards squeezing against my lungs).  What I didn’t realize was that I hadn’t screwed the bubbler on just right. As a result, the concentrator gauge read that I was getting my full quotient of O2, but a few precious LPMs were actually bleeding out of the bubbler. I took a couple of steps and my sats dropped hard and fast.  I got that hurky-jerky feeling in my arms that signals I am about to collapse…and I did.  Usually I end up on my knees or my rump, but this time I somehow twisted my ankle under me.

When I hit the floor, I splayed my legs in front of me, took one look at the odd angle of my ankle, and yelled.  I don’t think I even said words, I just yelled until I had no more breath.  I don’t think I passed out, because I remember thinking hard about…something.  But it did seem like a long time before the family arrived.  It wasn’t of course, they were there in seconds giving me alternate cannulas and making sure the O2 was flowing. Then I told them about my ankle and everyone gasped. The bone wasn’t sticking out, but you could see the contour of it beneath my skin and it was definitely not in the right place.  A quick call to 911 was followed by a bumpy ride to the ER (ambulances are NOT air-cushioned) with a very nice paramedic.  Xrays showed that I dislocated the ankle and had a spiral fracture of the fibula. Before this, the only bones I’d broken were a couple of toes. Two years of prednisone had weakened my joint and my bone.  Have I mentioned I have a loathe-hate relationship with prednisone?  For all that had happened, there wasn’t much pain. Even when the ER doc put the ankle back into position, it really didn’t hurt (okay, maybe the morphine helped a little there).

The most distressing part was hearing that I would likely need surgery to put the ankle back together.  Surgery is a very scary proposition for me because I can’t be “put under” anesthesia. If I am ever intubated, I will probably never go off a vent  (and never get a transplant).  Which meant this surgery had to be done with me awake.  Dr. Wegman, my surgeon, was one month into private practice when I showed up in his office.  He’s part of a highly respected group recommended by my primary doc, so I figured they would only hire the best.  But I have to admit, I think I scared the new guy a little.  I would be his first “awake” surgical patient.  Lucky for him, I’m actually pretty mellow.  Anyway, as soon as the bone guy confirmed surgery, I called Dr. Potts, my pulmonologist and knight in white coat, to tell him what was going on.  He immediately called the head of anesthesia and demanded the best person for this gasless procedure.  He also called respiratory therapy and set up the Optiflow system for surgery.  Optiflow is an oxygen system that heats and humidifies O2 at flows up to 60 LPM.  I was on 55 LPM for surgery and my sats never fell below 93!

Thanks to Dr. Potts and team planning it all out ahead of time, everything went perfectly.  I had a spinal and epidural that left my lower half completely numb for the ORIF surgery (open reduction internal fixation).  A sterile drape over my face ensured I didn’t have to view the hardware application to my bone (one plate, 8 screws, plus a 9th screw to stabilize the tibia and fibula), and the noise of the Optiflow pretty much left me in my own little world.  I chatted with Dr. Settles, the wonderful anesthesiologist, about college-bound kids and career changes (he used to be an OB-GYN).  And when I hit recovery a few hours later, I didn’t have to deal with that awful grogginess.  I spent the night in the hospital to make sure my lungs were functioning well following the “stress” of surgery.  All was fine. In the morning, I got a quick lesson in the fine art of walkers and headed home.

Now, four days post-op, the hardest thing to deal with is that everything is, well, harder.  Walking around the house on two healthy legs uses a lot less energy (and a lot less oxygen) than holding myself up on a walker with two arms and hopping on one leg.  Even a two-hop transition from chair to wheelchair leaves me breathless.  I can’t bear any weight on my ankle for 6 weeks, so I will be dealing with this for awhile.  I’ve started working with a physical therapist to try to keep the blood flowing and get my muscles to be more efficient in oxygen use. Think chairobics.

In the meantime, I feel like a total invalid. The family freaks if I try to do anything on my own, because let’s face it, there’s a very real chance I could fall again.  And if I break anything else, they might as well just rig me up in a hoist and swing me from bed to bathroom.  Heck, they might prefer that now, but I do have a little dignity left. Not much, but some. Then again, the hoist idea may not be so bad.

The thing I have to keep in mind is that this is just another bump in a very windy, bumpy road. I will heal, and I will go on. And I will heed the lessons I’ve learned:

  1. Trust how I feel, not what I see. The concentrator said I was getting the right amount of O2, but my body told me I wasn’t. Next time I will sit down and take time to figure out what’s going on.
  2. Check the bubbler, check the bubbler, and check the bubbler. The same is true with all of my equipment. Everything is so routine that it’s easy to get careless. But my life literally depends on my oxygen apparatuses.
  3. Be grateful everyday for my caregivers.  My family are the unsung heroes in all of this. People tell me how brave I am, but I wouldn’t be here (literally) if my family wasn’t here to save my sorry ass when I do stupid things. They are brave for living this with me. I love them to the moon and back and will never be able to express just how much they mean to me.
  4. Trust the team. I called Dr. Potts because I wanted to make sure he was in the loop early. Because I contacted as soon as I did, he was able to work out a plan with my surgeon that ensured both of my issues were handled appropriately.

I wonder what the next bump will bring?

Is This Progress?

ImageIs this progression or an infection?  A reaction to the weather or some other episode? These are the questions I ask every time my cough gets worse or I have more shortness of breath, or my sats just won’t stay up.  They’re the same questions everyone with PF asks. Because this is a ninja disease.  You may be stable for months, years even, and then BAM!  Without warning, your condition worsens almost overnight.

That’s what I’m wrestling with right now.  Is this progression, or just another infection?  I was in the hospital with bacterial pneumonia in early July — 6 days, including 2 in ICU.  But I responded quickly to treatment and came home almost at baseline.  Almost, but not quite.  I had been resting on 6 LPM and found I was more comfortable at 7 LPM.  And before the infection, I’d been moving on 10 LPM, but after I definitely needed 15 LPM.  My doc told me not to worry; it takes a long time to fully recover from pneumonia, and since this was my third bout, it would take longer still.

I settled in for a quiet summer of resting and steering clear of potential infection sources (i.e. crowds!)  But I still felt like I just wasn’t getting better.  We played with my prednisone but even that didn’t help.  Then, two weeks ago, I was having trouble making it down the hallway without getting short of breath. My sats weren’t holding, either, so I kicked up my oxygen another notch — 8 LPM.  When I woke up with a productive cough (my cough is ALWAYS dry), I called my doc.  He put me on oral antibiotics and bumped up my prednisone again.  After a week of antiobiotics, I wasn’t coughing up gunk anymore, but I still felt lousy.  And my sats were falling again.

As it was, I had maxed out the oxygen system I had available at home.  My 10 liter concentrator only yields 10 LPM if I use a 7 ft. hose — that’s the same length you use with a portable tank.  A 25 ft. hose — which allows me to move around my bedroom and to the bathroom — yields 8 LPM.  My portable liquid tank goes to 15 LPM, which means that anytime I want to move more than a few steps, I have to use my portable.  I talked to my doc and we added a second 10 liter concentrator.  They are Y’d together and with a 25 ft. hose, I get 16 LPM.  That’s perfect right now — sitting on 16, I can keep  my sats right around 90%.  I still need to use my portables if I want to go anywhere else in the house.  I’ve Y’d two of them together to give me 20 LPM so I can make it out to the kitchen to eat dinner with the family.  The problem is, at these high flow rates, even liquid portables don’t last long — maybe half an hour at 15 LPM.  I can probably get 45 minutes to an hour when I Y two together (10 LPM each for a total of 20).  That means excursions beyond home are impossible.  I can do doctor visits, but lunch with friends is off the table.

optiflow

MyAirvo/OptiFlow

One of the big side effects of pumping such high O2 levels is nose bleeds, lots and lots of nose bleeds.  Even with a bubbler on the concentrator, my sinuses are parched.  I use Ayr gel and Ayr saline spray throughout the day, which help.  But the assault on my mucous membranes is vicious.  My doctor prescribed a new machine called the MyAirvo to help with this.  I was on one in the hospital (where it’s called OptiFlow).  It humidifies and heats the oxygen, so it is much easier to breathe.  It’s also quieter because you don’t get the “rushing air” sound through the cannula.  On the downside, however, the tubing is only 3 ft. long, so I have to switch off to a portable to do anything.  And I will need to add a third concentrator to get enough oxygen flow to keep my sats up.  Right now, when I use the MyAirvo, I have to Y in a 3 LPM from my liquid to keep me sats even close to 90%.  And then there’s the machine rental itself.  It’s an expensive piece of equipment, and it’s so new that most insurance companies (including mine) don’t cover it yet.  So I’m deciding whether this is right for me just yet.

When I look at my oxygen numbers, they just seem ridiculous.  From what I’ve seen, most people don’t use oxygen at this level until the bitter end.  But I’ve always seemed to require higher O2, and my body still feels like it’s raring to go (okay, not raring, but eager).  I’ve had a couple of crazy instances where my liquid ran out and walked 20 feet or so without O2 — I crumpled on the spot like a marionette without strings.  I was still conscious and talking, and as soon as I got oxygen and brought my sats up, I was up walking again like nothing happened.  At least for now, I’m only limited by the Os I can get up my nose.  I pray the body hangs in there with me.

Travels with My Reservoir … Or Gasping Across State Lines

Image For more than two decades, my family has made an annual pilgrimage to Sanibel, Florids. It’s our own slice of island paradise for one week a year — sun, sand, shelling and the luscious smell of sea air.  In the past, we also flew to Sanibel to maximize our short time there. But, since my diagnosis with PF, our travels have grown increasingly complex.

We were still able to fly for our first visit post biopsy. I rented a portable oxygen concentrator (POC) that worked for both the flight down and my activity while on vacation. At the time, I didn’t require oxygen while at rest, so the relatively low flow rate of a POC was just fine.

Six months later, we made a second visit to Sanibel. This time I was on oxygen 24/7, but my requirements were still fairly low — 3 LPM (continuous) at rest, 6 LPM (continuous) with activity.  Since portables offer only pulse settings at 6 LPM (and only a couple of models go that high), I knew a POC would not work for the entire trip.  We had to come up with a different alternative, and it was immediately clear that we had to drive.

While my husband and kids psyched themselves up for a 21-hour drive, I worked on piecing together my vacation Os plan. Usually, your home provider works with a provider at your destination to coordinate services.  My home supplier, however, is a local company with no ties to national suppliers. They said they were unable to arrange any support — I’d have to carry everything with me.  So, I rented a POC that we plugged into the car and provided me with a continuous 3 LPM for the drive.  We also brought my full-size 10L oxygen concentrator plus a home fill unit and 4 bottles of varying sizes.  I filled the bottles before we left, and used those for meal and potty stops along the way.  Once we arrived, the full-size concentrator met my needs at the condo, and kept my bottles full for jaunts around the island. All in all, it worked beautifully.

This year however, my oxygen needs were much greater — 6 LPM at rest and 8-15 LPM with activity. The same plan wouldn’t work. I still use my 10L concentrator at home, but I’ve graduated to liquid O2 for my portable needs because I get much more time out of a liquid portable (about 3 hours). Liquid isn’t perfect, though.  It boils off over time, so a portable bottle that was full the night before will be only half-full in the morning. So, filling a bunch of liquid portables for the trip down wouldn’t work.  Taking my full-size 41L liquid reservoir wasn’t an option either — it stands about 4 feet tall and weighs in close to 200 pounds when filled. There was no way we were going to heft that breathy beast into the minivan, and then drag it up a flight of steps to the condo in Sanibel.

Not my van, but this is what it looked like.

Liquid reservoir, concentrator, E tanks for reserve

Still, liquid was the only way to go to meet my breathing needs for the ride down. I borrowed a  21 L reservoir from my home supplier. It’s about the size and shape — and looks amazingly like — R2D2, and holds enough to last me almost 48 hours (1 liter of liquid O2 converts to 814 liters of gas).  Now, I just had to find someone near Sanibel to refill the reservoir during our week’s stay.

After a few calls to oxygen suppliers in the area, I began to understand why my home supplier had given up the year before. No one wanted to supply oxygen to someone who wasn’t their patient, especially someone from out of state. Many said they didn’t offer liquid O2. Finally I tracked down one supplier that grudgingly agreed to refill my reservoir, if and only if I gave them 24 hours notice, arrived at their location between 8 and 8:30 in the morning (the only time their driver was available), and agreed to pay cash.  A girl’s gotta breath, so I said yes.

When I called to make my final confirmation two days before the trip, the person who answered said they had no idea what I was talking about. Panic started to dance around the edges of my brain. After a bit of begging, she agreed to the previous plan.  But their office would be closed both Monday and Friday, and she couldn’t guarantee that the driver would even be there that week. With some more begging, I got the name of their O2 supplier.  While commercial suppliers can’t service patients directly, maybe they could suggest another retail supplier. I got two names and an unsolicited promise of further help if those didn’t work out. Finally, a glimmer of that famous southern hospitality! The first company I called was Rotech, and Todd said he could fix me up no problem. They even drove out to island to pick-up and deliver my refilled reservoir, and let me borrow one of their reservoirs for our stay. (Rotech didn’t show up on my initial internet searches for local suppliers — work on your SEO guys!)  The trip was a success, and breathed easy the entire time.

Traveling is getting more complicated and requires a lot more planning, but it is still possible. With a little tenacity (and a bit of human kindness), I know I’ll be able to continue traveling as long as I have the strength to go.  I look at my oxygen as I do eye glasses, a pacemaker or a wheelchair — it’s a tool that helps me live my life to the fullest.  It’s my choice, not my circumstance, that dictates whether I use the tools available.  And I choose to enjoy what I have with whatever support makes that possible.

TRAVEL TIPS:  Start early to make plans for traveling with oxygen — at least 4 – 6 weeks out:

  • Airlines do not allow oxygen in liquid or gas form. You can, however, use a portable oxygen concentrator — contact your airline to get specifications and necessary paperwork.
  • If you use a national oxygen supplier, they should be able to coordinate your oxygen needs at your destination, and run the costs through your insurance. Keep a copy of your oxygen prescription with you at all times.
  • Make sure to plan for more than your estimated travel needs (airlines require 150%) so you will be covered if you get stranded or waylaid.
  • Call to confirm before you leave.
  • Plan early, then enjoy yourself!

Retooling My Attitude

tool_belt

“If the only tool you have is a hammer, you tend to see every problem as a nail.”

Abraham Maslow (1908-1970)

The right tool makes every job easier, whether you’re a cook or a carpenter, a seamstress or a surgeon.  And sometimes, the simplest tool can make a world of difference.

A few days ago I received a grabber as a gift.  You’ve seen them advertised on TV — a trigger at one end operates a claw mechanism at the other.  You can get them as fancy as you want — foldable, extendable, super heavy duty.  I opted for off-the-rack at Walmart, less than $15. But what a little miracle it is!

You see, the stiffness and scarring caused by my disease limits my lung capacity.  That’s why any activity causes my O2 saturation to drop like a rock.  When I bend over, I get even more breathless because my innards compress against my lungs, further reducing my breathing capacity.  This makes simple tasks, like picking up clothes off the floor, a feat that requires planning. Bending twice makes me dizzy. A third time requires a rest break and pursed lip breathing.  grabber

As a result, I let things pile up.  I’d kick clothes into a mound for several days and then gather them up all at once. Errant kleenex and nebulizer ampules dotted the floor around my chair.  When I did get around to picking up, it took half a day of crawling, reaching, gathering and breathing breaks to finally see the carpet again. Not anymore!  With my grabber, I spend less than 30 seconds each morning picking up my room without a huff or a puff.  If something slips off the hanger in my closet, no worries!  Even the long lost socks behind the dryer are back in play.

I never realized something so simple could have such an impact on my day.  Breathing easier is a definite plus.  But I think the biggest benefit is that I feel just a little more in control. When I walk into my bedroom, it isn’t littered with the detritus of my disease.  It’s neat and clean, no longer screaming a constant reminder of the little things that seem to get harder and harder to do.  I know that my disease will take its course, there’s really nothing I can do to change that. But I can affect my attitude and my outlook — and those are the most powerful tools I have.

Tethered

I use supplemental oxygen 24/7.  That means wherever I go, I have a cannula strapped to my face and a plastic tube connected to my oxygen source.  When I’m on the go, I use a short tube attached to a portable oxygen tank. But at home, I have a 100 ft. “umbilical” that snakes between me and my oxygen concentrator.

My oxygen supplier frequently warns me I shouldn’t use anything longer than 50 ft. because the longer the tube, the lower the O2 flow.  But with my 100-foot tube, I’m free to roam just about anywhere in my house. So, I’ve worked out a compromise — for bed, I plug in with a 25 ft. tube and save the ultra long cannula for daytime.  Convenience has its price, though.  Like when I get myself all wound around the island in the kitchen.  Did I go right or left?  I can’t tell you how many times I’ve gone to the kitchen for something and started back out, only to have my head snapped around as the cannula pulls tight. The kids still smile when they see me backtrack and do a reverse circuit.

Feet pose another obstacle.  It’s not tripping (for me at least) so much as it is standing, as in, “please move, you’re standing on my cannula.”  Standing on my tubing doesn’t hinder the oxygen flow, but it does stop me in my tracks. I thought when I switched to a green high-flow cannula, this little annoyance would be rectified. To my surprise, the family tromps even more on the highly visible green tubing.  My guess (hope) is that they’ve grown so used to my O2 set-up, they don’t really notice the cannula anymore.  Or it may be self-defense on their part.  Whenever my cannula goes taut, I give it a yank, figuring it’s just caught around a corner.  Recently, I’ve been hearing squeals when I tug on my tubing. Seems I’ve been tripping up the kids a lot more than I realized!

Then there are the frequent snarls. Everyone has grown used to me standing in the hall outside my bedroom and growling as I work through another knot. My tubing gets caught on drawer pulls, chair legs and just about anything left on the floor. The dog, of course, takes it all in stride.  He’s become quite adept at disentangling himself whenever my cannula slides by.  And I usually manage to stay fairly unencumbered myself…except for that time I twisted one too many times while making the bed and did a face plant into a pile of pillows. Yes…there were witnesses. We all burst out laughing.

I’ve often thought a portable tube reel (kinda like those retractable dog leashes) would solve a lot of my problems. Unfortunately, nothing like that exists and I am no engineer. Of course, the real answer is “tubeless” oxygen, like wireless Internet.  Oh wait, that’s called “normal breathing”.

In the meantime, while I still have these lungs, I’ll be tethered.  But being tethered isn’t so bad.  The limits of my cannula have forced me to slow down and refocus. I spend a lot of time talking and hanging out with my kids — unhurried and undistracted.  And I’m closer to my brothers than ever.  It’s amazing what happens when you stop thinking about where you should be and concentrate on where you are.

I’ve heard lots of people say they are “grateful” to their disease for waking them up to what’s important in life.  I’m not grateful to IPF — it sucks big time.  But it is part of my life,  a big honking bump in the road. And like all the other bumps, twists and turns I’ve encountered, I’m trying to learn as much as I can from the experience.  So far I’ve discovered that I am surrounded by amazing people eager to help — I just didn’t notice until I slowed down and let them in.  And I’ve realized that if you get stuck heading down one path, you can always backtrack, unwind yourself and try another route.

Photo courtesy of 123rf