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.

Six Misconceptions about Cystic Fibrosis

I wanted to share – and dispel – six misconceptions, or “myths”, as often believed by the general public – those without intimate knowledge and experience with Cystic Fibrosis. There are many possible items to cover, but these are the big ones.

1. Cystic Fibrosis is a childhood disease.

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Until the 1970s, people with CF couldn’t expect to live into adulthood, but today, nearly half of all CF patients are over the age of 18!

Thanks to newborn screening in the US, more patients are diagnosed at birth, and the earlier patients begin preventive treatment and their caregivers learn about what to expect, plus the newer maintenance medications and brand-new medications designed to work at the source – the genetic defect – there is so much more hope for living longer, fuller lives. Patients are graduating high school and pursuing even further education, perhaps getting married and/or starting a family, building a career, and being able to achieve dreams that others said would never happen.

2. Cystic Fibrosis a “lung disease”.

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Courtesy of the Cystic Fibrosis Foundation cff.org

The fact is that the faulty gene exists every cell in the body. The thick, sticky mucus clogs organs, impeding natural functions, causing a chain reaction of issues.
Therefore, CF can affect most major organ systems. In addition to the lungs, the most effected is the GI system – including the pancreas (which also causes Diabetes), stomach, intestines, and also may affect the  Liver, Kidneys, Heart, and Reproductive system.

 

 

3. You “get” or develop CF.

Cystic Fibrosis is genetic – it’s in every cell in your body.

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Most of us are diagnosed by age 2, but for 25% of patients, diagnoses is delayed by a few years, a decade, or even half a century. Late diagnoses happen because the symptoms weren’t recognized as possible symptoms of CF, or that symptoms were mild enough to dismiss them, or because the patient received a wrong, but plausible diagnosis. I know of someone recently diagnosed in her 60s. Imagine that – going your whole life knowing that “something” was going on, but never having an answer, never finding out why. For some patients, it’s validating to finally have a name put to their symptoms, but they have a lot of education to suddenly wrap their minds around.

CF does not “develop”, and is not “triggered” by occurrence of another illness. However, a doctor may accidentally uncover issues while testing or treating a patient for their existing problem, or it might take the collection of symptoms that sparks a theory in someone’s head to test for CF. And finally, it could take the worsening of previously mild symptoms to consider further testing.

 

4. After a tune-up, we are “all better”.

IMG_1251Many CF patients refer to a two-week hospitalization and/or course of IV antibiotics as a “tune-up”. When done in the hospital, we do 4-6 Airway Clearance sessions a day, extra breathing treatments, eat a high calorie diet, do Physical Therapy, etc. Some patients elect to do tune-ups at home – using a temporary PICC line (long, threaded IV that goes into the upper arm, through a vein that reaches the heart), or wearing a permanent PORT – both which allow the patient (or a family member) to give themselves the IV medications at home.

A tune-up  basically “resets” us to baseline – or as close to it as possible. However, it is not a cure. It’s basically a reset button. I often joke that when normal people go to the hospital, they rest. When CF patients go into the hospital, they work – hours of airway clearance (4-6 hours a day), daily walks and/or sessions on the exercise bike, breathing treatments, eating as much as possible…it really is a lot of work. Once I return home, I usually need several weeks to regain my strength from being so sick and doing so much work.

My biggest hope each time is to gain back the lung function I had lost since the most recent exacerbation (or string of exacerbations).  However, with CF, each year usually means additional permanent damage.

5. Kids with CF should live in a bubble, stay away from other kids our age, and never tire ourselves out.

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This is a huge myth. When I was a child, my parents were very overprotective. I was discouraged from playing outside in any temperature extreme. This meant a lot of missed recesses, field trips, and gym classes.

My parents also discouraged me from playing sports – I remember wanting to play youth soccer when we moved to a new town when I was in 6th grade, and they said the only way I could join is if my brother played, too. Well, that didn’t happen.

It wasn’t until the 8th grade when I had another chance to DO something. I begged my parents to let me try out for the cheer squad. To everyone’s amazement, I made it. The following year, in high school, I auditioned for, and was selected to appear in the school musical (long was my dream of acting, singing and dancing) – which required a lot of singing and dancing at the same time. To keep up, I begged my mom to let me take some dance and voice lessons, and I also participated in every choir/chorus/play/musical that gave me a role.

It was so amazing to exert my body to its fullest potential, sweating while cheering and dancing and finding my breaths while cheering and singing. I never felt more alive, and never knew that my body COULD do things. I surprised everyone, and I credit my constant rehearsing and performing with maintaining excellent lung function during those years (despite getting sick).

KIDS SHOULD MOVE!!! We all should move!!!

6. Cystic Fibrosis is contagious.

First, CF itself is not contagious. It is genetic. You cannot “catch it”. Are are either born with 2 copies of the CF gene (and have CF), carry one copy of the gene (and are a carrier), or born with no mutated genes.

In addition the germs that we ca13285734_10154040190825973_2138384192_nrry enjoy living in our lungs because they like the thick, sticky mucus that coats our airways, giving them a moist, warm place to grow. They really wouldn’t like to live in the lungs of otherwise healthy lungs.

Think of a lizard relocating from a tropical rainforest where it rains every day and temperatures soar over 100 in the hottest times of the year and that has thousands of species of bugs to eat, to a dry, snowy tundra where it’s too cold for insects to live and the sunshine isn’t abundant or strong enough to warm up the cold-blooded reptile.

Our colonized bacterial infections are the same way – they need a certain type of environment in which to grow and thrive. They don’t want to set up shop in clear lungs.

However, we are very contagious to one another – so there are now contact protocols for staff taking care of patients in care settings – they have to wear disposable gloves, masks and paper gowns, and dispose of them before leaving the room, so that if they see another patient, microscopic bugs aren’t attaching themselves to doctors, nurses, etc., and latching onto and into the next (CF) patient they see.

Between patients ourselves, it’s suggested that CF patients remain at least 6 feet away from one another when outside, and not even be in the same room inside a building.

Have any questions or myths about CF that you’d like dispelled? Share below!

 

The Price of a Few Months’ “Life”

As if my body somehow can read a calendar, I am back in the hospital. I have been averaging an inpatient stay for IV antibiotics every six months or so, and I was last inpatient in December, so it’s about time.

Things Start Brewing

It was only two months after my last hospitalization when I started feeling sick again. It started as a sinus infection – the same mutli-resistant bugs that colonize in my lungs also live in my sinuses.

Three weeks of oral antibiotics didn’t work, so I ended up needing an antibiotic rinse for another few weeks. During that time, the bacteriae in my lungs started florishing, and over the next month or two, we exhausted all of our other options.

I visited my CF clinic several times in the past month to check my lung function, and each time, my numbers went lower and lower.

Decision Time

By the end of April, my FEV1 – a measurement of how much air I can blow out in one second – was down nearly 30% from December.
I was coughing more, had increased junk in my lungs, and got to the point where I was sleeping contantly.

I called my doctors on Monday and they decided to admit me the following day. I had to wait until nearly 8pm for a bed, but started IV antibiotics immediately.

A huge challenge lay ahead of me, however. My doctors usually prescribe three IV antibiotics – medications that are very toxic to the veins. Instead of “regular” IV lines, called “peripheral lines,” we need to use an IV that goes into a much larger vein.

Among the few options I had was a PICC line, an IV that is tunneled into the upper arm and threaded though a vein that empties near the heart. The kind that I use is stitched in place, and has two lumens (tubes).  Another benefit is that the nurses can draw blood from it – like a spigot, which would mean I wouldn’t have to get stuck several times a day.

A PICC line can last several months, which is useful because I am on these antibiotics for 2-3 weeks at a time. However, because of damage to my veins from previous blood clots, inserting a PICC line is more challenging.

It is inserted under x-ray, in the Interventional Radiology department. In addition, due to the amount of work and time involved, I receive “conscious sedation” – a combination of pain and anxiety medications – to make the process less painful and scary for me.

The biggest hurdle is getting on the schedule. I woke up Wednesday morning hoping to get it placed, but I got bumped.

Playing the waiting game.

The next hurdle is dealing with the delicate “regular” IVs and having blood drawn several times a day until I get my PICC line. The smaller veins in my hands/wrists were getting irritated and flimsy. I was running out of places to stick.

The aftermath of several blood draws and a blown IV .

On Thursday, they attempted to place the line, but couldn’t get the line in after nearly 3 hours of work.

Attempt #1 failed.

 

I was despondent.

I was told that they would try again on Friday, and I waited impatiently. I was miserable. Since Tuesday, each morning, I had to abstain from food and drink after midnight because of the sedation (aspiration of food and drink is possible when sedated).

Friday came around and I was starving, getting dehydrated (which makes it harder to place IVs and draw blood), and completely exhausted.

Was it ever going to happen?

As the day went on, I kept getting mixed reports that I was on the schedule. Finally, a note popped up on my calendar slating my PICC placement for 6:15pm. Six o’clock came and went. 7pm, too.

Interventional Radiology department (IR) rarely places PICC lines at night or on the weekend unless there are emergencies, so I was getting really worried that they’d bump me again.

Wasn’t this urgent?

I needed these medications and I was quickly losing access options!! I was at risk nutritionally and feeling really scared.

Around 8:15 pm, as I was frantically texting my husband and a few of my CF friends for support, there was a knock at my door.

A transport rep showed up at my door, ready to take me down to IR. I couldn’t believe it!

My chariot awaiteth.

I was wheeled downstairs, moved, and prepped.

For some reason, instead of trying to place a line in my right arm, they decided to try my left arm again, requiring the doctor to perform an angioplasty. They blasted and plowed their way through my clotted vein, finally achieving success after 2 1/2 hours.

Scary looking, but a success!

 

Despite medications and numbing shots, I was very uncomfortable and scared. I must have reactively winced a dozen times.

But it was finally done. No more pokes for the rest of my stay (as long as the PICC line remains functional)!!

Like the Heavens Opened

I slept like a log Friday night, and woke up Saturday morning finally able to eat breakfast for the first time since I had come into the hospital. Pancakes and bacon never tasted so good!

I am thrilled to finally have a PICC line, making it much easier to get my antibiotics and for the nurses to draw my blood twice a day.

Thank you, God for helping me hang onto the last drops of faith and patience I could find, my amazing nurse, my doctors who harassed IR, my hubby/family/CF friends who kept me sane and entertained.

The Blessings Keep Coming

Another bonus of this hospitalization is that from now on, CF patients will be cared for on a particular floor. That means continuity of nursing staff – the nurses will see us much more frequently, building more experience with CF patients, thus creating an all-around better patient experience.

The Nurse Manager even remembered me from a 3-week hospitalization from fall 2012. She gave me this cute little bear for the nurses to sign!

I have already seen and felt the positive changes taking place as a result. Today, I walked 5 laps around the unit – a total of 1,650 steps, and when I got back to my room, Father Jeff was waiting to give me Communion. I am feeling very loved and lucky today!

And by sheer coincidence, a fellow CF patient – a.k.a. a “Cyster” (a word we use to refer to other women with CF) – is in the room right next to me. I recognized her cough and sent a text message to her, asking if she was in the hospital and on this floor.

This is the third time we’ve been hospitalized at the same time, so it was a crazy coincidence!

And, yes, it’s possible to recognize someone by their cough!

Although we’re not allowed to hang out or be near each other due to contact precautions, we can wave to each other and have quick doorway conversations.

Thankfully, we can send text messages to one another!

It’s nice to have someone else in the trenches with me (although I hope she feels better ASAP!!).

Lastly, and most importantly, I am thrilled to notice overall improvement since the week began.

My cough has decreased, I am getting more junk out, I have more energy, and I feel like my lungs can hold more air. I am sure that my doctors will repeat my lung function tests sometime in the next week, so I really won’t know how far I’ve come until then.

Although the week was pretty brutal, I am glad that all of this work is beginning to pay off. I view this hospitalization as an investment – if I want to have a fun summer, I have to pay the cost. It sometimes doesn’t seem fair, but that’s the way my life works.

CF SUCKS!! Update

Just a quick update. CF has really been beating me up lately.

I developed a sinus infection about six weeks ago. I culture Pseudomonas Aeriginosa and MRSA bugs in my sinuses as well as my lungs. I tried to manage it but the infections started causing symptoms in my lungs, so my doctors put me on oral antibiotics about 3 weeks ago. The medications didn’t work well enough, so we addressed it yesterday at my CF Clinic for Pulmonary Function Tests and checkup.

My lung function is down by about 20%, so I am back on oral antibiotics and will also begin using an Antibiotic rinse for my sinuses (gross, but effective). The appointment ran several hours, and I have to return in 2 weeks for a repeat lung function test. You’d think they’d get tired of seeing me, no? 😉

Patiently waiting to see my doctor.

Patiently waiting to see my doctor.

In addition, I have to see my ENT (Ear, Nose, Throat doctor) tomorrow morning to make sure that there isn’t more going on. What a huge pain!! I wish I didn’t have issues driving, but some of my medications make me drowsy, and I don’t always have the option to not take them. But we do do what we have to do. We find a way.

I pray that this plan works because it’s only been 3 months since I was last on IV antibiotics. So I’m doubling my usual Vest (airway clearance therapy) and doing everything else I can to increase my chances of avoiding more intensive intervention.

Just another valley in this bumpy road. CF SUCKS!!

Holding Out for a Hero

In less than two weeks, I will be traveling to Boston to see a doctor who specializes in Pancreatitis in Cystic Fibrosis.

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I have had Chronic Pancreatitis for over seven years now. The genetic defect that causes CF creates thick, sticky mucus in the organs. When this mucus clogs the Pancreas, it blocks the release of digestive enzymes and insulin. After four decades of constant inflammation, mine is useless, and I have to take supplemental digestive enzymes – Pancreatic Insufficiency, and inject insulin any time I eat – Diabetes. I have nothing left to lose.

I need a hero.

I spend hours a day with active pain and nausea. My symptoms occur around the clock, worsen when I eat, and become severe if I eat protein or fat. Even with medication to treat pain, nausea and vomiting, I spend a lot of time incapacitated and on the couch.

The medications also have side effects: drowsiness, which affects my ability to drive; I have difficulty focussing or concentration; dystonia (involuntary muscle contractions), etc.

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Deconstructing the Web of Living with and Treating Cystic Fibrosis

Sometimes, effectively treating the various symptoms of Cystic Fibrosis is like juggling chain saws. One hand is trying to catch the first one, the other hand is trying to catch the second one, and here comes another and you just have to hope they all go where they are supposed to go.

Each person with CF has their own  complex combination of issues, and while treatments are meant to manage symptoms and help slow down the progression of permanent damage and prevent further intervention, sometimes they can exacerbate other existing problems, or even cause additional ones.

The combination of so many variables means that no two CF patients are the same. As a result, our symptoms, and their severity and frequency, dictate medications and treatments. What works for one person may not work for another.

I’ve created a video explaining the basics of my particular disease. It’s like a web, really. Everything is interconnected and sometimes you just have to hope for the best.

Please visit my Youtube CF channel.

As always, if you have any questions or comments, please share below.

Finding Restful Sleep and Solving Other Problems through Self Hypnosis

Hello readers! First of all, I want to wish you all a Happy New Year. I hope that your holidays were full of joy and love.

As always happens this time of year, we’re in the winter doldrums. The next six weeks will feel like the “day after Christmas” as we’ve moved on from the busyness and socializing from the holidays to a state of questionable weather and days that still get dark in the afternoon (although we get lighter by two minutes every day!).

The New Year is a time when we cast away the negative and try to focus on the positives in life. It’s a chance to make resolutions, change habits and generally live a happier, healthier, more successful life. Of course, however you measure success is up to you. Continue reading