My Cystic Fibrosis Journey: 2003-2004

Getting to Know You

It may occur to some that I see CF as an entity, almost as a person, who takes joy in making me sick and making us suffer.

There’s a method to that chain of thought. Long ago, I had the idea that we need to give CF a name, a persona, so that we could have someone/something to blame. Someone whose name I could curse and hold responsible. Someone to fight, to stand up to, to proclaim, “you haven’t got me yet!”

And so, CF became “Creepy Fred.”

Creepy Fred was the buzzkill of every party, the kind of person who acts up, looks for attention, and makes everyone around him uncomfortable. Pretty accurate description of CF, isn’t it?

When I wake myself up coughing in the middle of the night, I yell at Creepy Fred to let me sleep. When my stomach is churning in pain and and I am curled up in a ball, crying on the couch, I tell Creepy Fred to f- off. Essentially putting a face on CF reduces it to something I can address, which is very helpful when I am feeling vulnerable and out of control.

The Third, Fourth, or Broken Wheel

I learned more about my health each day, and was able to observe and assess emerging patters. For example, sometimes before a lung infection pops up, I get tired legs walking up the stairs or night sweats. Or when I have the beginnings of an intestinal blockage, I burp much more often. Over time, I have been able to connect certain symptoms that preceded flareups of different organs. In many cases, early detection can help prevent excessive intervention. Without the freedom I now had at home, I probably wouldn’t have noticed those patterns.

However, I had to do a lot to accommodate the many ways CF affected my daily functioning.

It was really evident when it came to having a social life.

I continued to lose more and more friends over time. I had made most of my friends  through work, and 99% of them lived close to the city. We tried to come into Boston to see them, but with a few exceptions, nobody wanted to make the reverse trip to the outer suburbs.

It was getting more difficult for me to get into the city and have a “night out,” because the round trip added at least 90 minutes to the night, depending of traffic or train schedules. It was also more difficult for me to do it myself, partly because I go through periods unstable diabetes (CF related Diabetes is different from Type I or II, and combined with medications like Steroids for the lungs, or other digestive issues, it can be difficult to manage at times). I also have very poor night vision despite wearing glasses, and a few of my medications also cause blurriness, so Ken usually accompanied me into the city, which irritated some people. Lastly, I also had to build in a nap period before going out, and reserved the entire next day to recuperate from even a few hours of “being out”.

Even though people seem to understand the basics my disease, some people think that we use it as an excuse not to do things. What they don’t realize is that just the everyday issues of Cystic Fibrosis, and side effects of treatments, can and sometimes affects nearly every part of my life:

  • Lungs: Pollution/pollen/humidity/damp weather which can cause my lungs to become inflamed; coughing attacks are intrusive and gross.
  • Digestive: Sometimes eating causes immediate pain, discomfort, gas, diarrhea, acid reflux; swelling in my abdominal region makes me need to wear “comfy” versus “appropriately trendy or dressy” clothing.
  • Sinuses: Pollutants, irritants, and infection cause drainage which is gross, messy, makes me cough, causes headaches.
  • Eyesight: Medications and blood sugar levels can cause blurriness, exacerbating the difficulty I already have driving at night.
  • Diabetes: The risk of “crashing” – my blood sugar dropping to inadequate levels – can cause me to lose consciousness, which can make driving risky.
  • Sweating: I get easily overheated, despite my propensity to sweat easily. As a result, I can get heat exhaustion when other people are barely uncomfortable. It makes me have to leave crowded places.
  • Drinking: At some point, my body didn’t like the effects of alcohol, and I also couldn’t combine alcohol with a number of medications, so I was the “boring one”.
  • Energy: I had reached the point to where my tolerance to “active time” – total time spent out and about – had dropped. I could no longer go to a festival and walk around all day, or stay out until 2am dancing. I often got tired easily; when I was sick, it made my legs very tired and “heavy”. I became the “buzzkill” who often had to go home early.
  • Timing medications and treatments: It became difficult to spend large amounts of time without the privacy or opportunity to do mediations and treatments.

Getting to Know Myself in a New Way

My social life kaput, I continued to work on activities that gave me happiness.

I continued making beaded jewelry, an activity I had begun quite serendipitously.

On a fluke, one day, I visited a bead shop in a neighboring town. I talked to the bead shop owner and skimmed through a few “basic beading” books. She made some recommendations on how to start, and I came home with a surprisingly small bag compared to the money I spent. Beads are small, but sparkly and just so lovely! I see a bead and I see what it could be.

I opened the beginner’s book and worked through every page, learning the basics. I loved practicing basic techniques and creating new designs out of my head using just simple techniques. I spent a lot of time at the local bead store, and made friends with another fellow “beader” who was interested in opening her own bead shop. She eventually did open her own shop, and I spent an afternoon each week there, drinking coffee, gabbing, and making stuff at the “bead table”.

My family and friends loved my designs, and one of my friends had asked if I could make the jewelry for her entire wedding party.

One of 14 designs for a bridal party.

One of 14 designs for a bridal party.

I designed 14 sets of necklaces and earrings, each a bit different, but using the same color combination of Swarovski crystals and sterling silver components. So many people wanted to meet the person who made the jewelry, and for the first time in over a year, I felt proud of myself.

While practicing different techniques, I ended up with hundreds of pieces, so I decided to sell them online and at craft fairs. I spent a few years trying to make it work, but it just wasn’t a profitable business. I had a lot of stuff to still give away as gifts, and supplies to create stuff whenever I felt like it. However, for that time since I left my job, I finally had something interesting to say when people asked me what I did.

“I make jewelry,” I told them, and handed out business cards.

Time to Just Be

Our Arkansas trips usually coincided with the Thanksgiving and Christmas holidays because that’s when we could both get time off. Now, with just Ken’s schedule to work around, at least from a work perspective, we could visit any time of the year. We flew out there for Easter that year, and it was great to see Arkansas in the warmer weather. Back home, there’s sometimes still snow on the ground, but in Arkansas, everything was in full bloom.

Me and my mother-in-law at Easter.

Me and my mother-in-law at Easter.

My Arkansas nieces, aged 4 and 7.

My Arkansas nieces, aged 4 and 7.

We also got to see Ken’s sister and her family. She now had two girls, 4 and 7 at the time. They were so cute and “little girl giggly”. I love that age. Everything is pink and glitter – stuff that I never grew out of!

Return of the Curse of Sisyphus

In October, Creepy Fred decided to give me  another Kidney stone to fight. As it was my fifth or sixth stone, I knew immediately what caused the initial waves of pain. We hauled off to the ER once again, because it became more painful – if it was possible – than any other stone. I’d find out why later, but for now, I was in constant agony, constant pressure and squeezing.

I remember fidgeting in the waiting room chairs for hours, to try to find a comfortable position. Nothing helped more than a few minutes.

Once they finally called my name, I thought, finally, relief! Ken followed behind me, but they told him he couldn’t come back yet. That had never happened before. As the nurse opened the double doors in front of us, I saw what looked like a war zone. Patients laying on gurneys, lined up against every possible free wall space. Two and three patients in each room. People slumped over in chairs, bleeding.  I was lucky to get a bed against a wall.

As the hours ticked by, I did anything I cold to keep myself calm and distracted. The pain was exhausting. I had to force myself into a trance-like state to not only handle the pain, but the sounds and lights and smells of hundreds of people crammed into one place.

It was then that I invented my “mind game”. To help me focus outside my body, I would pick a topic – let’s say food – and go through the alphabet. For example, Apple, Banana, Cookie, Dumpling. It required me to concentrate. I was probably around topic 10 when a nurse took my vitals and said someone would see me soon. It wasn’t soon, but when they finally rolled me back for an x-ray, it was a step in some direction.

Shortly after being returned to my wall, another nurse came running towards me. She wheeled me into a suddenly available private patient room and shut the door behind us. It turned out that I had a 9mm kidney stone that was blocking my right kidney from passing any urine, and they were loading me up with tons of fluids and pain medications. She apologized over and over for keeping me in pain and alone in a hallway all day.

Not long after, they took me straight to the surgical suite to insert a stent in my ureter, to allow the urine to flow out of my blocked kidney. Once I was stabilized and given a prescription for pain medicine, they discharged me with orders to call my urologist.

Well, my urologist couldn’t get me in for 6 weeks, so we looked online and found a kidney stone expert in Boston. He saw us within days, and he immediately scheduled me for Lithotrypsy – a procedure during which they send shockwaves to the kidney to break up the stone. The procedure went well, but I had ot keep the stent in to allow all of the pieces to come out. I spent about six weeks with the stent, and had to travel back into the city to get it removed. Needless to say, I recommend that you never, ever, have your stent taken out in the doctor’s office.

Getting to Know new Branches on My Family Tree

My sister Chrissy, who already had two boys, had her third baby, Trice, in June. My other sister and I actually assisted at the birth. It was a miracle to see new life actually coming into the world. Aside from my wedding, it was the best day of my life.

Flying baby!

Flying baby!

Trice was the final niece/nephew in my family, and we heralded her arrival with as much pomp and circumstance as we did the first – the Queen Sarah. Trice would be the Princess, but only because she was born later. The two of them would become as close as sisters – which made sense considering their mothers are twins and they are genetically half-sisters. I love all of my nieces and nephews, but the youngest always gets the most attention!

Having a New Adventure

That summer, we took our first “real” vacation… to a tropical paradise. We spent an entire week in Puerto Rico!! Our room overlooked the ocean, and the sound of the crashing waves lulled us to sleep. We explored the rainforest – El Yunque – the only one in North America.

A leaf bigger than me!

A leaf bigger than me!

We ate delicious food and sipped freshly-made rum drinks in mom & pop shops. We dressed up in our fancy duds to visit the infamous San Juan Hotel. We strolled through Old San Juan, including the Fort (Castillo San Felipe del Morro) at the tip of the island. Interestingly, the four years I spent in High school Spanish class gave me enough of a foundation to have entire conversations with locals. Oddly, after just a day or so, I was thinking in Spanish. I am by no means fluent, but somehow, that little area of my brain that stored Spanish somehow reactivated enough to get around the island.

Back in the CF Saddle

The holidays came around and were pretty uneventful health-wise, but as soon as the season changed to Spring, I was getting sick again. I am usually on some sort of oral or inhaled antibiotic about 1/2 of the year, collectively.

Given that I had several colonized strains of bacteria living in my lungs, any one or several could flare up at any given time. In order to manage them effectively, the doctors make decisions based on how each bug responds to antibiotics, so they test the Sputum (mucus from the lungs that we cough out) and test each one that grows, for Resistance or Sensitivity. Over time, the bacteria gets smart and starts to biologically build defenses, such as a hard-to-permeate mucoid wall. They literally built fortresses around them. So as they learn to outsmart one antibiotic, the doctors ty another. At a certain point, the strains that don’t respond to oral or inhaled medications require strong IV antibiotics only available in IV form.

A typical course is 2-3 different medications over 2 weeks (a “cocktail”), and is often called a “Tune Up” as it contains around-the-clock aiway clearance and other assistive therapies. The amount of time I spend attached to my medications varies from 4 hours a day to 18 or more. It all depends on the medications and how many of them I have to take.  If I am not stable –  low oxygen, low blood pressure, etc., or if the number, length and frequency of medication infusion takes a lot of work and monitoring, I would spend the entire time in the hospital.

However, if I my vital signs were stable, I would  stay in the hospital for a few days, then  go home to finish the rest of the IV course at home. During those several first days, whether or not they planned to keep me in the hospital or send me home to finish, I’d get a PICC line placed.

Click to enlarge for descriptions.

Click to enlarge for descriptions.

A PICC line is a long IV that is inserted into a major vein on the arm, and threaded to the heart, where it dumps the medication. The meds are toxic to small veins, and a PICC line lasts much longer than typical IVs placed in hands or arms that can blow the vein after a day or two. During a previous hospitalization, I required 11 new IV insertions of regular IVs, and as many as 30 blood draws. A PICC line can eliminate all of those sticks.

Another benefit of the PICC line is that the nurses can draw blood from the arm without another stick. Some of the medications can injure the kidney, so they take blood every 12 hours make sure the medication levels are at a therapeutic and safe range.

If I only stay in the hospital a few days and come home to finish the course, I do all of the medication infusions myself  – including “flushing” the line with Saline and Heparin (to prevent clots), and the visiting nurse comes once a week to change my dressing and take blood samples.

In Aril, I needed another round of IV antibiotics, but it wasn’t planned. We try pre-plan my Lung hospital admissions with a few days to a week’s notice. I had been feeling sick and on oral and inhaled antibiotics for several days – certainly long enough for the meds to start turning things around. However, I felt no better, and called my CF clinic to discuss treatment plans for pneumonia. When the nurse I spoke to heard my breathlessness, she told me to get down to the ER IMMEDIATELY.

I felt poorly, but it didn’t feel emergent – yet. When we got to the hospital, they did the typical registration process, and told me that it would probably wait a while. However, when I next went to the trauma nurse, while measuring my Oxygen levels, she asked “are you Toni?” and I answered, yes.

“You’re back here with me,” she said, pulling open a curtain. She hooked me up to a pulse oximeter, blood pressure cuff, and EKG monitor.

“You’re in respiratory distress. Your doctors are on their way.”

Wow. This was the first time I needed Oxygen outside of surgery. I was terrified. I remember that once I got into my room, I called my sister who lived in MA – only 30 minutes from the hospital – and the second she heard my voice, she asked where I was, and said that she was coming to see me. During this time, I went downhill quickly.

The doctors ordered X-rays and said that my lungs looked very different from typical CF pneumonia X-rays. The white lines on lung x-rays – called infiltrates – usually show a specific pattern when I’m sick with the pneumonia strains I colonize. This pattern was unlike any x-ray I had ever had. The infiltrates were all over my lungs, indicating a far-reaching infection.They did sputum tests to check to see which bacteria strains were active in my lungs but those take 3-7 days for results. They put me in total isolation, and after the Infectious Medicine people checked me out, they ordered in the CDC because their own tests couldn’t identify the cause of the infection.

My FEV1 – or the amount of air you can blow out in a second – dropped to 42% – the lowest ever for me, and a stark change from one earlier in the year in the low 90s. I was inpatient on IV antibiotics, steroids, and any support interventions possible. Ken and I were petrified, but somehow, I got off the Oxygen, pulled up my lung functions to around 60%, and actually discharged “on time” – 14 days later. We had gone from hell and back. I’d have to come back to the doctor for repeat lung function tests, but we knew that this would take time. Sometimes, when you go down fast, it can take a while to get back up to where you were before it all happened.

Three Weeks Later

After all was said and done, after leaving the hospital, my arm where I had the PICC line ached. It was normal to have a bit of stiffness, due to not using that arm too much for two weeks, but it didn’t seem to stop. I had a follow-up with my CF doctor three weeks after discharge. I told him what I felt and he thought I might have a Blood clot.

A what?

He walked me right down to the Cardiovascular department and had them scan my arm. I had a Deep Vein Thrombosis (a.k.a. DVT or blood clot) from the middle of my upper arm all the way up to my neck. I got this feeling of panic. What if it breaks off? Will I have an aneurysm? A pulmonary embolism? Apparently, the risk of those events happens in the earlier stages of clot formation, so I was probably safe, but I needed to start Anti-coagulation therapy immediately.

I spent six months on Warfarin (a.k.a. Rat poison), and had to get my blood tested every 3-6 days (that’s a 40-minute drive each way) to make sure that the medication was keeping my blood at a particular clotting level. It was scary, because there was still a remote change of the clot breaking up and moving elsewhere, and then there was the new risk of bleeding too much should I have some sort of accident or injury.

And Time for a Break

The rest of the year was pretty uneventful. We saw a few Red Sox games, went to an animal show, and just tried to enjoy the breaks bewtween crises. We had a busy holiday season with all sides of the family, which was really special. It’s funny because as crazy as holidays can be, when I miss them, I really feel lost. I was lucky that season!

Life with Cystic Fibrosis was kind of like a roller coaster, except the ride UP is so much better than the slide down.



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