Starting Anew

With the promise that Trikafta potentially holds for me, along with starting suppressive Doxycycline for MRSA, and based on the improvements (health-wise) I have had, I feel like I am entering a stage of reinvention.

Life Changes

I was getting ready to die. I have been reading books on “how to live as you die” and “how to accept” death. Less than 2 months ago, my CF doctors discussed lung transplant with me (I do not plan to do so), and also mentioned a feeding tube for nutrition.

I couldn’t even think about anything besides surviving because I was always so sick…every 3-4 weeks 103 degree fevers, going on O2 sometimes for 6 months at a time, hospitalizations only 6 weeks apart, and my CF team telling me they were “out of ideas”.

And now, I have some hope – and have all this stuff that I want to do, but how do I start living again? Doing stuff? Getting out? Seeing friends? Will they have me back? Am I still important to them?

I also have no idea what my interests are at this point, though I love writing, acting, and beading. In the past, I used to read a lot but not lately, I used to sing but not in 30 years bc of a throat condition that killed my pipes. I have spent a lot of time running support groups online, and – this may be a surprise to many – volunteered as a radio DJ for pirate web radio station/chatroom for a year. But what now?

A New Me?

At 48, I am starting over in a way. But I prefer to see it as starting something new, not in place of where I have been surviving.

Just not sure how to start. After my sinus surgery in 3 weeks, I want to hit the ground running. In the meantime, self reflection and decluttering my house as my body gives me bursts of energy.

A Call to Action

To all of my friends, family, and sorority sisters…

If anyone who knows me from any part of my lifetime had any suggestions, clubs, groups or idea of something that I might be able to do (seriously, don’t suggest “town adult soccer team” because, like, have you ever seen me kick a ball?), or anything that you are part of and think I might be interested, please let me know!! I am looking for something to start in the Spring/Summer.

Also, I would love to start seeing people. I live in Central Connecticut but at some point plan to resume driving and would love to meet up with old friends.

Hope, Written in Sand

As I begin the second month of my “miracle drug”, I can’t help but realize that the beginning of the New Year is also the beginning of so much hope that just six weeks ago I did not have.

Although I have not seen the doctor yet to see where my lung functions are, I can share anecdotally what my experience has been.

I have gone from 5 L of oxygen and barely able to walk from my bed to the bathroom, to running up the stairs without coughing, having to only pause for a few seconds – if that – to catch my breath.

I am beginning to be able to do things that I could never do when I was always sick. And I realize that although it’s only been six weeks, and who knows when I’ll get sick again – and I will, because I still have CF – but before this medication, and the suppressive antibiotic therapy I am on at the same time to help get my MRSA under control, I couldn’t stay well longer than three days without some sort of intervention.. That is not an exaggeration.

Something as innocuous as emptying the dishwasher and reloading it would take me three separate 10 minutes sessions, separated by a rest. In between each one. I would then need to rest, lying down, for a few hours after doing it. This was happening as recently as November even after I returned from the hospital. But now, I get burst of energy during which I can accomplish an entire task. What a foreign concept to me!

I can also laugh. And if you know me in any capacity, you know that I have what can sometimes be called an inappropriate giggle. But I find humor in every so many things and the ability to just laugh and laugh until my stomach hurts, and not stop because of coughing, but because I literally have fallen over, my stomach muscles tightened so much from joyful guffaws.

I actually spent several hours celebrating Christmas with my family. I missed Christmas and Easter this past year, and on Thanksgiving, I only lasted long enough to eat and I had to leave.

Typically when I wake up, I wake up to a host of very unpleasant pains and symptoms. Decorum demands that I not go into detail, but let’s just say it usually took me about two hours before I could do anything for myself. Let alone do anything for anyone else.

Now, I’m waking up less encumbered with exhaustion and pain, and although I’m still not a good “waker upper,” I have been able to attend to the kitties, make coffee and even breakfast, and be up with Ken in the morning before he goes to work. What an absolute joy! To start feeling like a wife and the keeper of my house again! To actually have been able to spend time with my family without them worrying how long I would last or if God forbid the next time they saw me would be in the hospital. Because that’s how it was.

I am not saying that I am at 100% of anything. But I feel like I have gained much in the past month, a bucket full of things I have lost in declining health over the past 10 years.

I still have an uncertain journey ahead, as it’s not known how I will progress. I do have side effects that, while bothersome, are not life threatening. I’m getting used to a new body. Basically, the medication is changing the way that every single cell in my body works. My body feels like it doesn’t belong to me. But I will learn. Ken and I are learning together! His engineer mind is so helpful when trying to figure out some of the new issues we are dealing with. and he knows when to reassure me, when to tell me to rest, and when to push me – because he so well knows what I do have inside of me.

As I move ahead in 2020, I have a few medical procedures/surgery that I must get through but I’m hoping by the end of next month, I shall be on my way to starting to accomplish some of the new goals that I dared set in sand.

#ThankYouVertex

It Takes a Village

So much has happened since my post.

If you read my previous post, you know that the the FDA approved the new CF medication for use in CF patients with at least on copy of a particular gene mutation. Known as “the triple combo” in CF circles and formally named “Trikafta”, it is designed to help up to 90% of CF patients.

The CF world has been waiting for this news, which was announced months before anyone expected. Since then, I have read dozens of stories by CF patients taking Trikafta that it is as close to a miracle as possible, without being a cure.

I began to read more and more social media posts using phrases like “life changing” and “second chance”. So my medical team submitted my prescription, and we were met with a swift “no”.

The insurance company was denying patients based on a “6 month wait period”, regardless of the patient’s need, or the medication’s presumed benefits.

Three Weeks Ago

I didn’t think much of it, because I had complete faith the decision would be overturned quickly.

As I was finishing my 3-week course of antibiotics, I was enjoying the most productive and energetic week I’ve experienced in at least a year. I achieved many small victories – I made it to all of my doctor and lab appointments, got to have a nice lunch out with Ken, had a movie date, and got my hair done (it had been 5 months). I also had begun Pilates again, achieving 4 days in a row.

That Saturday morning, I was up at 7am, cleaned the kitchen, ran laundry, and brewed a pot of coffee.

Ken had somewhere to go that afternoon, so I figured I would spend that time resting from all of my activity.

And then it hit me. Four days after finishing antibiotics, I spiked a fever and started going downhill. By the next morning, I needed 3 liters of Oxygen and had a 102.9 fever. i was becoming powerless over my lung infections, especially MRSA.

A few days later, I was admitted back into the hospital – only 6 weeks after my last hospitalization.

One Week Ago

Fast forward to last week. i spent 14 days on several antibiotics and very close observation. Doctors were stunned that I got so sick so frequently. We agreed on a basic plan, part of that including Trikafta.

I was discharged on my 48th birthday, just 2 days before my favorite holiday – Thanksgiving. I had been sent home to continue my antibiotics. We also scheduled sinus surgery for the beginning of the year, and a plan for suppressive therapy.

I was also neck-deep in appeals for my insurance to cover the new CF drug, Trikafta. My CF team and I had been fighting for five weeks to get insurance to cover it. Even my patient rep at Vertex, Trikafta’s manufacturer, was trying to push things through.

My Favorite Day of the Year

Amazingly, I was able to actually attend my sister’s Thanksgiving dinner – my first family holiday since last Thanksgiving.

I also have been able to cuddle with my babies while recovering from my hospitalization. It takes a while to regain strength after such a rigorous treatment regimen.

Meanwhile, I wondered how Tuesday’s fourth appeal would work out.

Six Days Ago

Imagine how dejected I was when the very next day, Friday, I received my fourth rejection letter.

Three Days Ago

And then everything changed.

Monday morning, my clinic called me to tell me that my insurance had changed their decision over the weekend, and that I was finally approved!

Tuesday, the specialty pharmacy called me to set up delivery, and on Wednesday afternoon, it arrived via Fedex.

The Start of a Second Chance

Today, I take my first dose of Trikafta. So many people played a role in this victory and literal second chance at life.

I am eternally grateful to God for guiding the minds and hearts of every person involved…

… on the clinical side, from scientists to researchers to doctors and nurses and pharmacists, my entire CF team, and my fellow CF patients who participated in clinical trials.

… on the personal side, from family and friends who encourage me, lift me up, and who know just what will lighten a heavy load (Snapple! Captains! Turkey!)

… and my forever love, Ken, who every day puts me first, who supports me and how I want to manage my CF, to working so hard and keeping us insured, for doing triple duty, to FaceTiming with him and my kitties every morning and bedtime when I am inpatient (63 days in the past 11 months).

There is also someone whose name I cannot share, but this very dedicated person will forever have my gratitude for the role they played behind the scenes.

Sometimes, miracles require a village – and I feel so incredibly loved and blessed to have mine.

I want to Keep Going, and Going, and Going

I thought it was a good time to write an update.

It is long, so I apologize in advance.

I had a CF doctor appointment 2 weeks ago, and I had coincidentally started feeling sick two days before that – only 12 days since I was discharged from the hospital!

Not Even Two Weeks Later

I had not had the time nor energy to recover from those two weeks, and I got knocked down again. I never made it off Oxygen completely after my hospitalization, and suddenly I was once again needing it at rest. Of course, Based on lab testing, they confirmed it was MRSA and my Pseudomonas (a strain that actually responded to an oral medication), so I went back on meds.

I have been pretty faithful doing my airway clearance. I spent the past two weeks on my vest, then eating, then trying to force myself to eat. My appetite had been doing some wonky things lately, and I have been very nauseated, so it is disheartening when a basic life function becomes difficult.

Some Hope

I also saw my ENT doctor last week. It’s a pretty gross experience upon which I won’t elaborate, but he was able to clear out some of the madness of my sinuses. He also wants me to have CT scan to check on some abnormal structures that might be contributing to my constant infections. Another sinus surgery might be in my future.

My docs also added a week to my MRSA antibiotics, since I had only started to really improve closer to the end of the two weeks. I feel this should help further push down my infection levels and give me a longer stretch of time before it flares up again.

In The Meantime

So now I continue my aggressive treatment, have to schedule some tests, and get a lab tests every few days to make sure my antibiotic isn’t messing up my body chemistry.

I also asked Ken to ask me to do little things as much as possible – helping the cats, grabbing something he needs, etc., to help build my strength. I find that the up and down motions of getting up and down more frequently is helping me. I need to also work on my cardiovascular health.

Oh and By the Way…

BIG NEWS. The FDA approves the newest CF drug, Trikafta, months ahead of scheduled.

It is being touted as a “life changer” and “miracle”. I remain cautiously skeptical at the hype, because:

  • This is NOT a cure, as too many people are asserting. It is a treatment for up to 90% of CF patients with at least one copy of the most common gene, Df508.
  • Every patient is different, and as we saw with its predecessors, not everyone (or even most) patients had huge gains.
  • Time will tell.

However, I am very excited to hear some of the stories coming from members of the CF community. I just don’t want to get my hopes up too much like I did with the other medications.

  • One More Thing
  • I had an awesome Small Victory yesterday. I needed something from upstairs, and for the past 2 months, I have had to plan such an exertion, or ask Ken to grab something next time he went up there. But before I could think, how badly do I need this, I was halfway up the stairs when I realized what I was doing.

    Just a week ago, a walk up the stairs would sent my oxygen levels to a very uncomfortable 86% (It is recommended to wear oxygen when your Oxygen Saturation level is 90 or below.) that usually meant I needed several minutes of purposeful breathing to get enough oxygen.

    I needed no such break!! I zoomed right up the stairs and then (carefully) down the stairs (I am a klutz).

    I feel “plugged in” right now. Charging my batteries for continued use. Now if could just find that energizer bunny!!

    Holding On

    On Friday, in addition to an EKG (to check for medication side effects), I had to visit the town vampire for lab work.

    I was bummed to find out that my inflammation levels had increased since I last had them on Monday. But I am hoping that perhaps they had risen between the first one and when the antibiotics started to hit the infections, and that they were actually coming down.

    I am feeling improvements on several fronts, but that inflammation number – measured by something called C-Reactive Protein (CRP) is very important to my doctor. It doesn’t tell the whole story, but lets him k ow if I am going in the right direction.

    So as I continue to do my treatment regimen, I am praying that the improvements I am feeling are confirmed with another blood test in the next day or two. Hoping the doctor agrees, and that I my theory is confirmed.

    I appreciate the prayers and love. It gives me strength, which I could really use right now. It is daunting to have such uncertainty and fearing where I will land.

    Ode to a Marriage

    Twenty-two years ago, when I was just 25, I married a man.

    A man with whom I’ve grown into adulthood, shared laughter and tears, trudged up mountains, and got lost in valleys.

    We have weathered storms and rainbows, and learned that you cannot have happiness without knowing pain.

    We’ve walked through heaven and hell on earth, and found one another again whence inside the darkest places.

    Twenty-two years ago, did we anticipate the journey we were about to take? Like all newlyweds, we prayed for the best. But nothing in life is perfect.

    We have walked through the past quarter of a century, together, although sometimes, I feel like that’s somewhat of a miracle. We forced ourselves to communicate when it seemed impossible. When our paths would begin to diverge, through faith and a sense of commitment, one of us would reach for the other’s hard, pulling them close again.

    I am no expert, and we don’t claim to have any of the answers, but I am proud of how we have been able to keep returning to the path when challenged.

    And for that, I am truly thankful.

    Measuring Progress?

    It’s sometimes hard to measure progress when I am in the middle of treating an exacerbation.

    There are at least a half dozen clinical symptoms that I can consider, but because changes happen gradually, I can’t always trust my own perspective. Am I coughing less? Bringing up less junk? Are my fevers improving? Are my lungs tolerating more activity? Am I feeling better?

    The doctors rely heavily on my anecdotal evidence, but also maintain close control on my lab work. They want to know if my inflammation levels are better, if my lung function is improving, and if my vital signs are more aligned with my baseline. For example – my oxygen levels, blood pressure, temperature, etc.

    In the meantime, I look for little clues that I am improving. How long does it take my lungs to recover after getting up for the restroom or get something to eat? How am handling stairs? How productive is the cough?

    It’s always questions, questions, questions. Having to self-assess is hard when you so badly want to be better. There is nothing harder than making the call to say I’m not improving.

    So, 2 ½ days into antibiotics, what is the story?

    I am measuring small victories – fevers are down, coughing seems a bit less. I still need my oxygen if I do anything other than sitting. But I still feel very crummy. And it’s really hard to eat, but I am trying. I will be getting labs drawn in the next couple of days to see if things are going in the right direction.