UN-Tethered

CIMG2308When the doorbell rang, I was as giddy as a little kid. It was a gorgeous afternoon, just perfect for taking back one more piece of my independence.  At the door was TJ, my awesome oxygen dude and a twice weekly visitor for most of the past year. But TJ wasn’t dropping off this day, he was picking up!

I’d been staring at empty liquid oxygen (LOX) reservoirs and silent O2 concentrators (two of each) for nearly two months, and it was seriously bumming me out. While not

TJ wheels one of my liquid O2 reservoirs!

TJ wheels one of my liquid O2 reservoirs!

long ago all that equipment kept me alive, it quickly become an unwelcome reminder of the struggle that nearly ended me. Since I’m not a dwell-on-the-past kinda person (at least not anymore), I couldn’t wait to get rid of all those tanks and machines so I could fully focus on what’s ahead: rehabbing my lungs and body, eating right, staying active and living the kind of life that will make my donor and his family happy they made the decision they did.

It took me a little longer than most to get rid of the actual equipment. Barnes Jewish, my transplant center, requires patients to keep their home oxygen equipment until after their one-month bronchoscopy. But I had to hang onto my relics until my team could schedule an overnight pulse oximetry test. It’s a simple test — my oxygen company dropped off a special pulse oximeter that recorded my sats while I slept. I don’t know the actual results yet, but I do know that my sats still drop at night, so the nocturnal Os continue (for now).  I did, however, convince my team that a home system designed to deliver up to 35 LPM was kinda overkill (not to mention expensive!)photo 1(1)

So when TJ wheeled the last concentrator out the door, I couldn’t help but shout So long huffers! He left a smaller, quieter 5-liter concentrator to supply the 2 LPM I still use at night —  my “sculpted lungs” have a little extra healing to do. But 2 liters is a whisper after the 15-30 liters I had blasting in my nose before.

Every day I see improvement.  Every morning that I wake up and take a deep breath is another miracle. And every day I give thanks for the many people who have worked so hard to keep me going: the folks at Lincare, who created an O2 system that could feed my need; my pulmonologist, Dr. Dan Potts (and Linda), who recognized my IPF immediately; everyone at St. Luke’s Respiratory Therapy and Pulmonary Rehab, who problem-solved with me and accommodated me in every way; the incredible team at Barnes-Jewish Lung Transplant Center, whose expertise and compassion are beyond compare; and my supporters, who have showered me with prayer, good humor and lots of encouragement. This little victory belongs to all of us!

 

 

 

 

 

The List!

OrganI made it! I’m on the lung transplant list! Well, almost. I will officially go on the list next week after I’ve met with the surgeon and signed the paperwork — that’s when the real waiting begins. In the meantime, I’m busy trying to get everything taken care of so I will be ready when I get “the call.”  And I am hopeful that my wait will not be a long one.

After a full evaluation at Barnes earlier this week, I’ve been assigned a Lung Allocation Score (LAS) of 62. The score is based on a 100-point scale and most people on the list are clustered in the 30s and 40s. A score of 50 is the 90th percentile. My score places me about the 93rd percentile, and that means that next week when I am officially listed, I will be #1 on the Barnes list.

That’s incredibly exciting, however it’s tempered a bit by some pretty serious limiting factors on my side. I’m relatively short (5’4″), so my donor will need to be my size or smaller. And I have HLA antibodies, which I developed during my pregnancies. HLA antigens are part of the immune system and in my case, this specific antigen occurs in 78% of the population. So, 78% of all donors are not a match for me — instant rejection. Among the 22% of donors that are available, we still have to match for size, blood type, etc.  To deal with the size problem, there’s a strong chance I will get only one lung. A single lung transplant would allow the surgeon to use a larger lung than would be possible if he was trying to fit two lungs into my chest. If I do get a single lung transplant, they would leave the less-diseased lung (my left) in as a “place holder” to keep my chest cavity from collapsing.

All of this leads to the essential and yet unanswerable question: how long? Masina, my transplant coordinator gave me the best answer she could — I might get the call the day after I’m listed, or it could take several years to find a match. Of course, I don’t have several years, so I will continue to pray that my match comes through while I am still healthy enough to receive it. I’ve been thinking in the abstract about “the list” for two and half years.  Now that it’s a reality, I am feeling overwhelmed for the first time. A lot excited, somewhat scared, and a nagging sense of guilt that my second chance will come at the loss of another’s life. So many things to sort out. Luckily, the transplant program includes social workers and spiritual counselors to help me work through it all.

Throughout this whole journey, I’ve felt strongly that there is a plan for me.  The first part was to make me a better human being, a better mom, wife, daughter, sister, friend. I think I’ve grown immensely as my priorities have shifted, fallen away and emerged anew to reveal the simple truth about what is really important in life (love, relationships, the things that bring me joy).

The second part of that plan — getting me to this point — has progressed quickly. Until I saw my LAS score, I just didn’t realize (or didn’t accept) how sick I am. I’ve been dancing on the knife edge, but no matter what hurdle appeared before me, somehow I’ve maintained equilibrium. In fact, every hurdle has actually pushed me forward.  My broken ankle helped to bust through a weight plateau and I’ve dropped 23 pounds since September.  Even my evaluation at Barnes, dramatic as it was, turned out for the best.  The 4-day eval started off on Monday this week with blood tests, a chest xray and an EKG. All went well until the EKG.  The tech ran a strip, left the room, came back to run another strip, left the room, then came back to run a third strip. She kept asking how I felt — I was fine.  The tiny room suddenly filled with people and someone was shoving baby aspirin at me. The EKG said I was having a heart attack. I knew I wasn’t, but we went to the ER anyway. While everyone in the ER agreed I wasn’t having a heart attack, they admitted me for observation and to do a heart cath the following day. The cath went smoothly and my arteries are beautiful. Thank God for statins and a timely change in diet!

Since I was already there, it made sense to simply complete my work-up as an in patient. Over the course of two days, they finished all of the testing and delivered my transplant patient handbook to me before I left the hospital. The blessing here? Four days of outpatient testing, running from floor to floor, sitting in crowded waiting rooms and driving back and forth in frigid weather would have done me in. As it was, I got to breathe that lovely high pressure 100% pure hospital oxygen and be escorted from test to test with a nurse in tow (because of my high liter flow, a nurse was with me all times).

Blessings have surrounded me throughout my journey, and I know they will continue. I have faith that when the time is right, everything will happen just as it should. Until then, I will wait patiently and prayerfully (okay, we’ll see how long the patience lasts!).  As I get into the routine of being on “the list”, I will post about my experiences.

In the meantime, for those of you who hope to one day get on the list, here are a few things to think about:

  1. Work hard to get to your goal weight as quickly as you can. My weight is what held me back from being listed earlier.
  2. Get active. Start pulmonary rehab, or at the very least, start walking.  Fitness and strength will be essential to get you through the transplant and recovery.
  3. Stay positive. Your attitude is paramount. Celebrate every victory, no matter how small. Focus on what you can do rather than what you can no longer do.
  4. Surround yourself with a team as committed as you are. If your doctors don’t know anything about PF, find ones who do. Join support groups to talk with people who are in your situation and can answer your questions (check out the Resources page for links). Ask your family and friends to help.
  5. Have faith. You can control your own behavior, thoughts and attitude, but some things are simply beyond us. My faith has given me the patience to accept and deal with these unknowns.

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.

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.

It’s (Not) All About Me

not_meChronic illness has a way of redefining who you are if you aren’t very careful.  It engulfs every waking moment:  medicine schedules, treatments, therapies, doctors appointments, research, dealing with symptoms, support groups, thoughts, prayers …. you get the idea.  It’s a full-time job. And for awhile there, I fooled myself into thinking it was my full time job, and mine only.  But I was wrong. Very wrong.

As my disease has progressed, I’ve watched my family become “sick-centric”.  My illness worms its way into decisions as mundane as who will drop off my daughter at the library, to bigger things, like where (and if) we go on vacation this year. Too often, family activities are dictated by whether I’m having a good day or a bad day.  And, since most household chores leave me gasping for breath, my husband has taken over all the shopping as well as most of the housework — in addition to all of the yard work.  I see the strain on his face and it tears me up inside. Like so many people I’ve met with PF, the guilt can be overwhelming at times.

Yet, even though our activities have changed drastically, I still clung to the hope that my kids were relatively unscathed by all of this.  We’ve talked about my illness, and I’ve told them I’m always here if they have questions or want to talk.  Since both have remained fairly mute on the subject, I figured they each viewed all of this as just a bump in the road.  Again I was wrong.  So very wrong.

My daughter had to write about a personal experience for an English assignment.  She chose to write about when we learned of my diagnosis.  We sat in my bedroom, talking about that day.  She distinctly remembered  a frigid winter landscape, bare trees, gray skies and icy streams.  I was diagnosed in early fall — September.  The trees were still green, the sun bright in the sky and the temperature was almost balmy.  She became very upset, convinced I was wrong, so I told her to write it the way she remembered.  When I read the finished essay, I realized why she was so adamant about the weather.  Her memory of a bleak winter day was so vivid because that’s exactly how she felt.

The piece was beautifully written, and brutally honest.  And in it, I discovered that the news that day crushed my daughter’s innocence.  The mother who was supposed to always be there, no matter how much teen angst she tossed my way, was suddenly fragile and broken. She could no longer be certain when or if I’d be there. I’ve sobbed over that essay, especially as I see the same theme pop up in other projects and assignments.

My son, who is pre-law at Saint Louis University, has been deeply affected by my disease in his own way.  He’s a born debater, and unlike most kids his age, he uses his Facebook updates for serious discussion of issues that concern him most.  During the Presidential election, he posted a plea asking people to stop their political mud slinging for just a moment and take a wholly humanitarian view of “Obamacare”.  He used me as an example of the importance of universal health care.  My PF bretheren and I are uninsurable.  In my case, I’m looking at four years before I will have insurance to cover a lung transplant.  Since ability to pay is a prerequisite for getting on the “the list”, I risk the very real possibility that I may never make the list, or be dropped from it when I am most in need.  It happens, I’ve seen it.  And my son worries about that.  While I’m proud of his understanding and vision, I also know that he’s just 18.  Right now he should be focused on his studies, his girlfriend and hanging out with friends.

It’s not all about me.  And yet it is.  My disease is indiscriminate. Though it attacks my lungs, it devastates the whole family.  I can only hope (and pray) that whatever I learn/understand/accomplish through this illness, will be shared by the people I love most.

So Long, Farewell, Auf Wiedersehen, Adieu

goodbyeAt the stroke of midnight on New Year’s Eve, as the world greeted 2013, I closed the book on a huge chapter in my life.  As of January 1, I officially joined the ranks of the disabled.  The transition was more symbolic than anything, since I have actually been on medical leave since June.  Even so, I found myself swallowing hard at the finality of it.

The decision to leave the work force was not easy for me.  I’ve always worked. Though I was a stay-at-home mom when my kids were young, I still clocked 30 – 40 hours a week (sometimes more) as a freelancer. The thought of not working was anathema to me. In fact, until just a few weeks ago, I was convinced I would return to my job once I’d fully recovered from my bouts of pneumonia last spring. But each time I drew close to reaching the “magic number” (10 mg prednisone), I had a flare.  After a few long discussions with my doctor and a sobering look at how my disease has affected my daily life, I realized that returning to work was impossible. The constant threat of infection is a danger I can’t afford. And, how can I expect my co-workers to keep picking up the pieces for me when a sudden flare lays me out for weeks at a time?

When I entered the hospital in early December with my third case of pneumonia in just nine months, I knew I’d made the only decision I could.  Thankfully, I had purchased Long Term Disability insurance through my employer.  If you have the opportunity to buy LTD, do it NOW.  Someone gave me this advice at the beginning of my career and I am so thankful I listened.  This is our family income for now.  Every penny spent over the years was worth it.

I also immediately applied for SSDI (Social Security Disability Insurance).  My main purpose for wanting SSDI is the Medicare option.  After two years of SSDI benefits, you are automatically eligible for Medicare. Unfortunately, you have to be on Medicare an additional two years before it will pay for a transplant. That’s four years total before Medicare will cover a lung transplant. So, it was important to me to apply and get accepted as quickly as possible.

While researching the application process, though, I read that some 70% of SSDI applications are turned down the first time. Thankfully, there is something called the Compassionate Allowances List.  The CAL is a list of 165 medical conditions that are considered so severe, they are sure to qualify for disability benefits.  Applicants who have CAL conditions are fast-tracked and benefits are decided upon within a matter of days rather than months (or even years). IPF is on the list, and I received my approval three weeks after I’d submitted my application online. Finally… IPF is good for something!

Now that my benefits are in place (and the Medicare clock has started its slow count-down), I am faced with what to do with myself.  I’ve been gobbling up books by the dozen. My husband and I have started hunting down healthier recipes to help my ongoing weight-loss effort.  And I am even eyeing my knitting basket with its snarl of remnant yarn, trying to come up with something fun and creative.  But in the end, this is all just idleness.  What can I do that will make a difference for someone? What can I explore that will teach me something new?  I’m still figuring that out and I’m wide open to suggestions.

In the meantime, here are a few helpful links if you are looking into applying for SSDI:

Social Security Disability Page   The application is two parts: the benefit application itself and the Adult Disability Report. The report is where you will list your doctors, medications, tests, hospital stays, etc. Download the checklist first and gather up all of your info before you start. It will make it much easier (and faster) to complete the report online.  Remember to use the “Remarks” box at the end of the report to paint a picture of what your life is like because of your condition.

SSDI and COBRA Extension   When you are approved for SSDI benefits, you may be able to extend your COBRA coverage an additional 11 months (for a total of 29 months). This article explains it all.

Why You Want SSDI   This is another “lawyer” site, but it does give a great summary of the benefits of receiving SSDI.

 

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