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 Innoway is good because there are not enough organs to go around. Of course, if people increased the amount of Oregon’s available in the national Oregon 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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Relinquishing Control in the Hospital

I just received a letter in the mail which included a six-page questionnaire about the care I received while in the hospital for 2 weeks in September.

They ask questions about the care I received, the hospital environment, my hospital experiences, tests and treatments, etc.

I am under no delusion that my care is very complex. At home, I manage 15-20 medications, test and monitor my blood sugar for diabetes, and perform airway clearance (lung therapy). It’s not just a matter of taking a bunch of pills each day. Many of them have to be taken at specific times, in relation to waking up, eating, performing certain activities (like driving), avoiding potential interactions, managing treatment side effects, etc.

So when I’m admitted to the hospital, I have to turn in all control to a series of more than a handful of different departments to manage.

Every hospitalization is a challenging experience. I’m awakened early (sometimes 4am) to get blood taken, doctors from 3 or 4 different specialties visit me daily, each asking all of the same questions, poking and prodding me according to their focus. I feel like I have to give up agency over my own body. I succumb to the authority and power of mostly strangers. Continue reading

Keeping Organized with Cystic Fibrosis

Having Cystic Fibrosis almost demands some level of organization.

When my health changed in my early 20s, I only had a handful of medications. Now, my list runs between 15-20 different medications, many of which I have to take daily (or more often). I have to manage daily doses, maintain an inventory so that I know when to refill medications, and keep it all accessible and organized. Continue reading

The Road to Orkambi

You may have heard that there is a new medication for people with a certain gene mutation of Cystic Fibrosis.

The FDA has approved a new drug that treats patients with two copies of the most common gene mutation, Delta f508. Forty-five percent of CF patients fit this profile.

The Basics of the Cystic Fibrosis Gene Mutation

Each of the more than 1,500 known mutations affects protein responsible for the proper transport of salt and water through each cell, creating a thick, sticky mucus that clogs the lungs and digestive system. Each of those mutations in the CFTR (transmembrane conductance regulator) protein has a different faulty mechanism.

Think of the protein as an electronic garage door. Consider some ways in which the door can malfunction: Continue reading

Dealing With Emotions When I’m Sick and In the Hospital

I just came home from a week in the hospital for an exacerbation of my Cystic Fibrosis. Several rounds of oral antibiotics and increased airway clearance/chest physiotherapy weren’t enough to keep me healthy, so I headed back into the hospital for IV Antibiotics.

My temporary IV posing with 3 different IV Antibiotics. It only took 3 tries to get this one in.

My temporary IV posing with 3 different IV Antibiotics. It only took 3 tries to get this one in.

Continue reading

Ten Things that Make Hospital Stays More Comfortable

Like many people with Cystic Fibrosis, I have spent a lot of time in the hospital. To be exact, I have had 27 different inpatient hospitalizations that lasted more than one day/night, and with each one, I learned something new about how to make my stay a bit more comfortable.

10. A blanket from home. It’s more of a comfort thing since I don’t like to pile on blankets because I sweat easily, but I can put it over my shoulders when doing a Vest or breathing treatment, and cover myself on my wheelchair or gurney when I’m being wheeled to and from tests, procedures or surgeries. I get lots of comments on this one:

A friend made this for me years ago, and it is one of the blankets that have traveled to the hospital with me.

A friend made this for me years ago, and it is one of the blankets that has traveled to the hospital with me.

Continue reading

Navigating the World of Health Care (pt 2): The first Steps to Getting Help

In Part 1, we discussed the idea of being sick. It’s a scary place to be, and it’s important to have a clear mind when seeking medical care. In Part 2, we will guide you through the first steps of seeking answers.

“What could it be?”

Whenever we get new a troubling symptom, usually out of nowhere, such as chest pain, stomach pain, difficulty breathing or numbness in parts of your body, or infections that aren’t heeling, or discomfort and pain after an injury, it’s hard to think straight.

Sometimes, you’ll have a symptom that can be pretty severe while it’s happening, but happens so intermittently, you it’s hard to establish patterns. You can’t even remember when it first happened. Doctors can be dismissive if you can’t provide much more info than “it hurts when I…”. It is common to see several doctors before one will actually try to figure out the problem, even if it’s severe.

steth

Doctors are so jammed with patients, you’re lucky to get 10 minutes of time with one. And when you do, you have precious few moments to get your history and your reason for being there. And when it comes down to it – medicine is just a job. One we attach noble intents to, but still a job, done by a regular person with the same stresses, emotions, frustrations, flaws and potential to make mistakes as everyone else does. Continue reading