Hope 2.0

I have entered a new phase of CF bad-assery. CF has declared war on me and is trying to drag me down to a very low level of lung and body functioning.

So let’s just say that I am now in The Big Fight.

I spent all of February and March sick, trying to fight the infections that I can (MRSA) at home. I waited too long and the bugs that I cannot treat at home* with oral antibiotics went crazy.

My body was overcome with inflammation.

Facing the Piper

I had a doctor appointment and lung function tests. My lungs are operating at 30%.

30%.

If I was interested in a transplant, this is the time docs start approaching the topic. But for a number of reasons, I am not a good candidate (not everyone is), Which in a way, is good because there are not enough organs to go around. Of course, if people increased the amount of organs available in the national organ donation database, this wouldn’t be a problem for those who need it, but for me, I knew that I would not be a good candidate. So that’s off the table.

Thirty percent – if I stayed at this level – is considered “end stage”. But you can live at end stage for a long time. Kind of weird to think about.

Paying the Piper

I entered the hospital with inflammation levels (measured by a marker called C-Reactive Protein) at 238. Normal is 1.0-3.0.

I was on 4.0 liters of Oxygen.

The Carbon Dioxide in my blood is 66, out of a normal 40ish range.

I began a 14-day course of 3 IV antibiotics, around the clock. I ended up with a kidney injury due to the toxicity of some of the meds, so I had to go on extra IV fluids because no human could have drank enough.

I endured a lot of pokes and sticks and have the battle wounds to share. But I had a lot of love from my husband, cats, family and friends to get me through the moments the tears fell.

It made the drudgery of daily work manageable.

I spent so much time and energy doing airway clearance and exercise – 3 hours of vest, an our of a machine my clinic is trialing, an hour (over course of the day) using a handheld blowing device called the Aerobika, walking 1500-3,000 steps a day (starting around day 6), and doing pilates each day. Plus trying to eat and sleep, despite 5 am blood tests and 11pm vital sign checks. And there was no time to nap unless I begged for an hour or two of no interruptions, but every other department (besides nursing and the doctors) decided that request didn’t affect them.

At day 10, they retested my lung function and it had only improved to 33%.

33%.

Granted, I always continue to improve after leaving the hospital, but this was not encouraging.

I left the hospital on day 15, kidneys still irritated, on 3 liters of oxygen, and feeling like this body of mine is not mine.

The Real Work Begins

Coming home was daunting. I knew I had fallen health-wise, but this time was so different. Everything is much slower.

Let me say that I am recovering, but I have a lot deeper of a hole to climb out of, have to take many more breaks, and sometimes have to ride out a plateau every few ascents.

I am making changes and setting daily goals and have to commit to a walking regimen to keep me as strong as possible, endurance-wise. Keeping up meds, fluids, airway clearance, and a good place for my mind to rest. Demanding that negative energy not affect me. Stress and bad energy is so damaging to the mind, heart, body and soul. No more do I give permission for others’ emotions to attach to me.

So… how much is this from a single exacerbation and how much is progression? There’s no way to tell, it’s a combination of both. I try not to blame myself for waiting a couple of weeks too long, because that does nothing positive for me. It is hard when everything to me seems like a gradual change, especially when your body and your mind or not in the shape they need to be to look at things objectively. But I have learned a lesson in recognizing my limitations sooner. This requires me to lean more on Ken and my doctors and notify them of changes that I might deem as small, as “little losses” might not be so little in impact.

Hope 2.0

Since transplant is not an option, and they treated my infections that were active, and I’ve tried a lot of the other treatments out there without much improvement, pretty much all that’s left for the my doctor to try is to put me on the brand new CF drug, Symdeko.

Like its cousin Orkambi, which I took two and half years ago and had a hard time managing due to side effects and medication interactions, it attempts to fix the defect or the mutation in the way that my cells process salt and water.

There may be a period of adjustment as the mucus thins and my body goes through a “purge”, a period of time where the existing mucus thins out and comes out in large quantities.

This can be traumatizing because it mimics the signs of an infection, it’s physically draining, and there are also side effects that may or may not show up.

So there you have it.

Hope. 2.0.

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Hope Sinks

Remember my last post, when I was so full of hope and excitement that if I can get myself as fit as I can, my life could change.

In my head, I had visions of hiking, doing normal household stuff and running errands, going out socially for more than 40 minutes, and even feeling well enough to go on a vacation (it has been 11years…we have planned so many trips that we had to cancel because I had to go into the hospital or was close to it).

Then I got sick. Too sick to start working out. MRSA sick with constant fevers, cough, sinus issues, and fatigue. Oh and the Flu. I took the Tamiflu and also started antibiotics. By the end of week 2 on antibiotics, I told the doctor I wasn’t feeling quite better and that probably I needed another week.

I finished week 3, and felt marginally better. Stronger. And as fate worked out, the weather was warming up enough to use my treadmill in the basement (it is not heated).

Three days after finishing the antibiotics, I started coughing, but figured that it was just left over stuff from the recent infection.

After another three days, I suddenly began coughing uncontrollably, felt my fever coming on, was feeling very junky in my lungs, and I knew that my MRSA was ramping up again.

I spoke to my doctors on Monday, who put me on a different antibiotic, and tonight – 24 hours later, laying on the couch unable to sleep, coughing which sounds like a pipe has burst, and having to wear my oxygen (which is a huge bummer because it had been weeks since I needed it more than a few hours at a time).

In the meantime, several members of my family have had their own issues to deal with, and I find myself having difficulty keeping my head above water.

I am tired of being sick. I’m tired of coughing. I’m tired of fevers ravaging my body with aches. I’m tired of having to judge whether doing a little laundry is going to force me to nap for three hours later That day or have to rest the entire day tomorrow.

I’m sorry that this is not a positive sounding post, but it’s almost midnight, I feel too awful to sleep, and my mind is racing with all the things that I am probably never going to get to do. Thanks I’ve been trying to do for years, thinks that any 46-year-old should be able to do. I’m sorry that this is not a positive sounding post, but it’s almost midnight, I feel too awful to sleep, and my mind is racing with all the things that I am probably never going to get to do. Things that I’ve been trying to do for years, things that any 46-year-old woman should be able to do.

The cliché about having your health being everything, is so damn accurate that it hurts. And what makes it worse is that I am watching the world go by. Outside, kids are playing and people are walking their dogs. The movies and TV shows I watch because I’ve read everything that I have and honestly cannot concentrate on anything else at this point – keep telling me where to go for vacation, parties I should be attending every weekend, planning for summer, etc., On Facebook, and in articles, I get to read about life as a parent, exotic places to travel to, and the cool/ fun/ amazing adventure others are experiencing.

I feel like there really isn’t much more to look forward to, when every time I try to plan something, whether it’s to go to the store myself, to work out, or to do something big like visiting my in-laws, my health blocks me from having a life.

Losing hope again.

Reclaiming myself

It’s been two years since I ended my three-month stint on the then-new CF drug targeted at my particular mutation(s). If you recall, I had a lot of side effects and medication interactions that forced me to call a cease fire.

I lost a lot of lung function during that time, and have not yet been able to get back to where I was, prior to the medication.

Now, it could have been coincidence. I could still have had the series of CF lung exacerbations and hospitalizations had I not taken the medication. I might still have ended up needing Oxygen at home after one particular infection flare-up, and still need it when I am sick. I might still have lost a lot of strength during those hospitalizations. And certainly, it had nothing to do with having Sepsis a year ago.

All of that, plus my existing CF complications, have driven my overall strength, stamina and unfortunately, my willpower, to an all-time low.

I have decided, however, that I am not giving in. Strength and energy have lost in this war. Now it’s time to go to battle to regain what I have lost. It’s time to reclaim myself.

I was never an athelete, but I had strong lower body muscles from dancing in high school, going through ACL reconstrtion rehabilitation, and doing Pilates for the past 20 years. I very suddenly lost my calves of steel during a September 2016 hospitalization, and my overall muscle tone disappeared.

So I have decided the one thing I can do that can help support my body and improve my energy and strength is a solid commitment to fitness. I used to be a gym rat, but too-frequent infections, symptoms – pain and nausea – from chronic pancreatitis, and side effects from several medications, have collectively rendered my gym membership useless.

So I’m starting from scratch.

It all starts with walking. I need to build up my cardiovascular system to get help my lungs and strengthen my legs. I will then return to light-to-moderate weight training to increase my overall muscle mass and give me that strength boost hat I currently lack. And then move onto hiking outside. Maybe on the flats, but getting my groove on – outside in the Vitamin D and fresh air – will be amazing.

Yeah, I’m sure I’ve written about this stuff before, but I feel that so many of my systems can benefit from this new commitment. I have to do this. So I got out my whiteboard and started writing down all the possible benefits.

• Better moods. Helping my depression and anxiety.
• Strength to carry me when my lungs are working harder when sick.
• Conditioning. Keeping my heart pumping efficiently.
• Lung clearance. Nothing like a good cough to get that junk out!
• Digestive support. Keeping things moving.
• Energy to live. Housework, socializing, driving. Holidays.
• Confidence. I’m tired of being weak and on the sidelines, feeling unworthy to contribute.

I am going public with my plan for accountability.

I may not move mountains, but at least I will be able to climb a few hills. And to me, right now, that can make all the difference in the world.

Waving the White Flag

Tomorrow, Tuesday, my doctors are admitting me for severe lung infections due to my cystic fibrosis.

Interestingly, it has been eight months since my last admission. I have been taking oral antibiotics to treat my MRSA infections, but after three months and several courses of these antibiotics, my lung function continues to drop and I continue to be actively sick. Aside from the MRSA, I have a handful of other bacterial strains that often Colonize CF lungs.

What stinks is that every time I go on antibiotics, I would start to feel a bit better, but within a week of finishing my 2 to 3 week course, I get sick again. I have to say it’s really hard to know when to wave the white flag and admit to my doctors that yes, I do need their intervention.

I will get a PICC line IV (long term, semi permanent) inserted into my arm or chest, which allows medication to go into my bigger veins were they won’t irritate the smaller veins closer to the surface.

The PICC line is usually a double lumen kind, which means that there are two tubes entering my vein so that I can have two medications or fluids running at the same time.

I will likely be taking a cocktail of three IV antibiotics, each with their own schedule.

Considering one of my medications runs for 12 hours a day, there are two other medications that have to run on their own schedules, and I usually receive IV fluids 24 hours a day to protect my kidneys, both IV lines are close to constantly being used.

Hence is the typical process of the 2 to 3 week hospitalization for a CF lung exacerbation.

It’s uncomfortable on so many levels, it can be painful, I get a lot of undesirable side effects, and I become sleepless and invariably stressed out. I feel bad for my nurses and my physician team because I get very emotional when I’m inpatient.

Depression and anxiety make things worse, as it does for many CF patients. Imagine being stuck in a 10 x 10 room, being allowed to leave the room only when scheduled for a test, having to eat, sleep, and do everything else from a lumpy bed in a tiny room with a window that often faces a bleak city scape. Oh, and going no longer than 10-20 minutes without someone walking into my room.

Despite all of the things I can and often do go wrong, however, I know that I am going inpatient to get better and to slow down the progression of the past few months. I am to the point where even getting up to shower by myself is a huge challenge, let alone being able to do do anything like cooking or cleaning or the basic stuff that people do when they live their lives.

So for the next few weeks, I will be doing everything in my power to stay calm and sane. I still have this option in my arsenal, so I am thankful. Not ready to think ahead to a point when no more antibiotics work for my bugs. Until then, I continue to fight.

Where do I go from here?

NOTE: There is a medical photo of an IV that may upset some readers. If thhis makes you uncomfortable, please do not continue.

Wow, where do I begin?

I am at the end of day 12 in the hospital. I knew that it was coming, my doctors have been trying to get me back in here since November because my infections kept flaring up.

First few days…most of which I do not remember

It’s always hard to tell when to pull the trigger and make the decision to come into the hospital and go on IV antibiotics.

Two and a half weeks ago, however, this flareup came on fast and furiously, and it was the Pseudomonas aeruginosa bugs that were in charge this time. These are the big bad bugs that I can no longer treat with oral antibiotics. So I have to go on at least two IV antibiotics for those strains, as well as an additional IV antibiotic to treat my MRSA.

Since my last hospitalization in September, I had been on oral antibiotics for my MRSA almost consistently. And now, everyone else wanted to come out to play.

When I was admitted, my inflammation levels were the worst that I have ever experienced. It freaked out my medical team. 

There is a number that they look at that can measure information, called C Reactive Protein. 

Normal range is 0 to 10. When I was admitted, I was at 156. Twelve days later, it is finally in the 20s. Getting closer.

Typically, after the first few days of antibiotics, I start to show some progress, but my numbers really weren’t going down. So my doctors repeated tests on my bugs, which basically involves me coughing up mucous and then putting it in a petri dish to grow it and see which anabiotics kill it. 

It turned out that one of them was now resistant to one of the medications I had been taking for an entire week. So they had to start a new medication for me, one that I have only had once in the past, but I had it via nebulizer, inhaling it instead of receiving it in my vein.

So I had a new drug added to my cocktail, and stopped the one that wasn’t helping.

Colliston is an old antibiotic that is rarely used these days because of its effects on the kidneys and how hard it is on the body. However, it is the only antibiotic to which one of my bugs will respond. It is a last resort.

My “jetpack” – the unit attached to the wites attached to me, monitoring my heart

 

The good news is that it started to work, the bad news is that it’s affecting my heart. So I had to stop taking a bunch of other medications that can cause the same heart injury, which sucks because they control a lot of the more unpleasant side effects of heavy duty IV antibiotics.

I also came into the hospital with a possible blood infection. I have had three series of double blood cultures and it appears to have subsided thanks to one of the IV antibiotics that also treats my MRSA. There’s a possibility that perhaps I did not have a blood infection and that one of the blood samples was simply contaminated, but they cannot take that chance and not follow protocol to the end.

So here I am…typically I would be close to discharge around The two week mark, but my doctors have shared that this is not going to resolve as quickly and smoothly as my other flareups.

PICC line with double lumen, so I can receive two medications at the same time

I am facing additional time with my PICC line, which makes me nervous because I have had three separate blood clots as a result of picklines over the years. It’s also very uncomfortable and limits a lot of things including the way I sleep, using my arm, etc.

I have also been on oxygen and might not be off of the oxygen when I leave, whenever that is. I am trying to wrap my head around the fact that I might have to go home on 24/7 oxygen. Hopefully it would be temporary, but with CF, you never know what’s going to happen.

In my head, I realize how very very sick I am – how sick I was – and how far I have to go in order for my lung function to increase. But my emotions have been riding a roller coaster that has not stopped since before I was admitted.

Yes, my inflammation numbers are going down. I am feeling a little bit stronger each day, and I actually was able to start walking in the hallways again. But I’m dragging my IV pole, my oxygen tank, wearing a heart telemetry monitor, and taking very small, very calculated steps.

This is not how I imagined progression of cystic fibrosis.

I have felt things that I’ve never felt before. Physical symptoms. Fears. Abject uncertainty.

I am 45 years old and I have cystic fibrosis. According to all of the statistics, I really shouldn’t even be alive. Perhaps that’s what’s making this harder, having beat the odds for so long and feeling confident about that, and now feeling so vulnerable and fragile.

It breaks my heart that my husband has to see me go through this and feel helpless. It hurts that I am more of a patient then a partner at this point in our lives together. I hate that my niece is a nephews have to see me so weak. I hate that my mother cries because she feels guilty for passing on this gene to me. And now that my sisters have had to help bathe me because I could not do so myself.

But on the other hand, I am more grateful for every breath that I do have. I have felt love with my husband that carried me in the most desperate moments I’ve ever experienced. I have also opened up and let my family see me vulnerable and allow them to help me. I have also allowed myself to ask for prayers and positive thoughts online. Are used to be embarrassed and felt like I was throwing a pity party, but I realize that shared experiences are why God put us on the earth together. 

Don’t get me wrong, I am not thankful for cystic fibrosis in any way, shape, or form. But I am thankful to God for every joyful moment.

I am offering my struggle to God as my penance during this Lenten season. I usually don’t talk a lot about my faith, but I know that I am still here for a reason and that God is not done with me yet. I pray for strength, courage, grace, and to still think of others, even when I’m hurting. 

I apologize for the babbling nature of this post, but there’s just so much that I needed to share. I don’t know where this path will take me in the coming days and weeks and months. I have to be patient and take each moment as it happens.

Thank you for spending your time reading my story.

Random Thoughts on Living with Cystic Fibrosis

I dream of feeling free, to engage myself with all that I see.

I dream of taking steps that lead further than my own front yard.

I dream of laughing without choking, and singing or dancing without coughing.

I dream of waking up each morning and bounding out of bed, and just taking off, somewhere, anywhere.

I still remember how it used to be when I could dance without gasping for air or my heart feeling like it was going to explode… times when my heart raced out of excitement and joy versus out of desperation to support my lungs with the oxygen they need.

It wasn’t too long ago that I had bigger accomplishments each day than simply walking up the stairs or taking a shower.

I no longer sing made-up songs about the cats or the weather or anything else that tickles my fancy, because I can’t spare the oxygen.  

I no longer dance when I hear my favorite songs, because that, too, requires my very precious oxygen. 

Instead, I shake my hands and shoulders with as much energy as I can afford to give to them.
I cannot laugh easily anymore. It robs me of my breath.

I work hard to strengthen my muscles so that they can take on some of the effort when I’m doing something strenuous like folding laundry or walking up the stairs or taking a shower.

I have to monitor my oxygen levels when I eat, because even a partially full stomach can make it hard for me to breathe.

Why has God taken my ability to dance, sing or laugh?

Today I am having a hard time seeing the glass as half-full. We, as humans, have the gift of such pleasures, and I feel robbed.

College and Cystic Fibrosis: Who/ What/ When/ How/ Why to Tell People about your CF

I came across some amazing news recently – nearly half of all CF patients are older than 18.

Courtesy of the Cystic Fibrosis Foundation

That means the patient population is growing up!! How amazing is that? It means so many things, and for some of us, it means:

No matter where you are in your self-identity journey, it’s never easy to know when to “come out” with your CF. And now that you’re going to college, you wonder, who do I tell? Who should I tell? Who do I need to tell? Who needs to know, really? It’s nobody’s business, right?