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Preface

The lives of members of a Cystic Fibrosis (CF) family are hard, whether you are talking about the person who suffers from this debilitating disease or someone who simply supports a loved one in the fight. As the husband of a woman with CF, the coughing keeps me up at night, but I do not wake with a sore throat. I have to plan around the breathing treatments, but I do not have to do them. I’m worried during the ER visits, but I do not get poked with any needles. I spend hours in the hospital room, but I can leave to grab a snack. I do not have cystic fibrosis and I cannot take away her pain, so the best I can do is to support her and be in her corner.

My nearly thirty years of experience with martial arts has led to national championship matches, full contact bouts, and a focus on continual improvement. Throughout all of this, I learned that nobody can do it alone. I always had someone in my corner. Whether they were encouraging me, coaching me, or simply being present, their support was critical. A good cornerman offers tactical instruction, but more importantly, knows his fighter. He knows the fighter’s strengths and weaknesses and how to motivate them to do their best. He looks at the fight from a unique perspective and helps the fighter refocus when necessary. But the one thing the cornerman cannot do is fight the fight for them. You dodge and move, get riled up, and suffer with every hit they receive, fully aware that they are the one taking every shot.

I wanted to write this book because I believe that the lessons I learned throughout my wife’s battle with cystic fibrosis and her transplant journey can help others. It is meant for those who love and care for a person with CF and those who are caregivers; those who fight CF with the determined attitude that they are known for, those who fight any uphill battle when it comes to their health, and those who dedicate themselves to healthcare. This book is from my point of view, and like a cornerman watching his fighter face down an opponent, this perspective is unique. It is my hope that this book will provide valuable insights to those with any debilitating disease, their families, their friends, their medical team, and their caregivers.

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Introduction

It was December 31, 2014, New Year’s Eve morning, and I woke up with an optimistic attitude. I had completed the self-assessment portion of my job performance review the previous afternoon and rewarded myself with stellar marks. With a few days off for the holiday, I had the morning all to myself and planned to be productive. I was going to watch and delete several shows from our ever-growing DVR hard drive. I even planned to do some dishes while I watched. Maybe I would hit the gym then pack some supplies for our New Year’s celebration in the hospital. Also, my wife was in the hospital.

It’s not that I was unconcerned about her current situation, but we had dealt with this so many times before. Rebecca suffered from cystic fibrosis (CF), and when she started to feel sick, the standard treatment was hospitalization and intravenous (IV) antibiotics. This was so common for someone with CF that it was called a “tune-up.” During the past few years, Becca was averaging two tune-ups per year. But 2014 had been a bit more challenging, and this was tune-up number six.

Rebecca was not rebounding as well as she used to, but that was the progressive nature of the disease. We also attributed some of it to the busy year we had—living apart for several months and ultimately moving to the greater Cincinnati area where I had accepted a new position. It seemed like she would bounce back as she always did, if she could just get a minute to rest. We knew that one day she would need a lung transplant, but that was probably not for another couple of years.

In the meantime, it was hard to break from our routine. We both worked full time and made great money. In fact we both had more success in our careers than either of us had expected. She was a Clinical Research Associate (clinical drug trials monitor) with the opportunity to travel to support testing and approval of new pharmaceutical drugs. I had recently changed from engineering management to product management and worked mostly with utility companies in the electrical sector.

Actually, a major factor in the decision to accept the new position was to position myself for a general manager (GM) role and transition Rebecca to a non-working role. For about a year, I had actually been trying to convince her to shift to part time but had experienced a significant lack of success. Becca loved her job and she was good at it.

One day earlier, Becca had been working from her hospital room and I was working at home. We had both finished around the same time and she had told me not to come in because she was tired and planned to catch up on some sleep. This was a bit unusual but it would be good for her to rest. Besides, she would then be ready for our big New Year’s Eve celebration, which would most likely consist of tapioca pudding and a movie on the laptop that we would pause every twenty-five minutes when the nurse came in.

Scanning the DVR recordings, it was clear we were way behind on our shows, and that I had recorded a large collection of movies that I had little interest in watching. Unfortunately, a day of moderately interesting movies and some minor cleaning was not in the cards. My phone rang and I saw it was Rebecca calling.

To my surprise, it was not Rebecca—it was the doctor. I knew immediately that something was wrong because the doctors never called me. Rebecca knew her condition inside and out, knew her medical history, and knew her treatment plans and medications. I couldn’t add anything of value. The only reason for the doctor to call me was because Rebecca could not.

Becca had a very bad night. The oxygen saturation levels (sats) in her blood had declined overnight, despite increasing the level of her supplemental oxygen. This meant that her lung function had diminished significantly. To counter this, they had placed her on BiPAP and brought her to the MICU. I did not know what those unusual words meant, but I knew that things were bad. When the doctor is clearly uncomfortable giving you news, it is bad news. He asked if I was coming in and I said I was. There was something to his tone that suggested that this could be the last time I would see Rebecca. It hit me right in the chest. There was nothing at all ‘routine’ about this tune-up.

I went upstairs to get dressed. I came downstairs without my pants. I went back upstairs to find a different shirt. I came back down to look for my backpack. After what seemed like an eternity (but was probably closer to five minutes) I was marginally closer to being ready to leave. This was when I made myself stop. In that moment I realized that all I had to do was show up. I did not have to bring a bunch of supplies. I did not need to perform any kind of surgery. In fact, I did not need to know what to do. This was the first of many major lessons I learned as this nightmare began:

Lesson 1: Show up.

 

CHAPTER 1

 

Homework for Dating

 

Rebecca and I had learned many lessons prior to this point as well. We had been together for almost seventeen years and, like any relationship, there was a learning curve. We had met at the University of Connecticut in January of 1998. Rebecca told me early on in our relationship that she had CF and asked me if I knew what it was. I told her of course I did…but…perhaps I didn’t remember all of the specific details so it would be fine if she wanted to refresh my memory. Okay, technically, I had no idea what the disease entailed but I had heard of it. Probably. I was certain that she could not see through my façade.

She saw fit to provide me with an eight-page technical article on the disease. This is the challenge with dating a cytogenetics major. I had not previously needed to do homework to date somebody, but she was worth it so I gave it a read. Being an experienced engineering student, I knew how to portray understanding and when I discussed the article with her, I was sure to mention the CFTR gene and its effect on protein production. She was quite impressed.

I learned that cystic fibrosis is a genetic disease caused by the body’s inability to correctly produce a certain protein. This protein, called the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR), is responsible for transporting sodium chloride across cell membranes. As a result, the mucus that the body produces is thicker in someone with CF and causes damage to several organs.

The pancreas for example, often does not release digestive enzymes with food so people with CF usually need to take these enzymes in pill form with their meals and snacks. Even with that support, their bodies often do not absorb enough nutrients from their food and they can be smaller and undernourished. In some cases, like Rebecca’s, they can develop CF-related diabetes (CFRD) as the pancreas can no longer secrete sufficient insulin to counter their carbohydrate intake. She developed this at sixteen years old and had to manage it closely every day.

The mucus in the lungs is also thicker and more difficult to clear (cough out) resulting in an ever-present cough and frequent lung infections. This is the driving factor behind many of the hospitalizations and the need for IV antibiotics. I always felt like I could pick her out of a lineup blindfolded because I knew the sound of her cough so well. It had a base that kicked in about a half second after it started followed by a long exhale.

The sinuses are also impacted, often developing polyps and infection. For Rebecca, this has led to severe headaches and over a dozen painful sinus surgeries. As a result, she lost her sense of smell, requiring me to smell all of her leftovers (including nasty food that I do not like) to inform her if they are still good. This is a burden that I have to shoulder alone.

The life expectancy for someone with this disease has increased significantly over her lifetime, in a large part due to the efforts of the Cystic Fibrosis Foundation (CFF). When Rebecca was born, she wasn’t expected to survive until high school. Advances in care and development of new treatments and antibiotics have continued to prolong the life expectancy of people with CF. By the time we met, her life expectancy was around thirty.

To inherit cystic fibrosis, you must receive a mutated CFTR gene from each parent (the condition is recessive). Parents of someone with cystic fibrosis may not have CF themselves, but each carry at least one copy of the faulty gene. There are different severities along with various mutations of the disease. Rebecca has two copies of the most common mutation, Delta F508.

Becca was able to tell me her specific mutation because she had it tested. She had a strong interest in human biology driven by her own illness. She majored in cytogenetics and molecular biology, graduated Magna Cum Laude and was one of thirty University Scholars at the University of Connecticut (UConn). In other words, she was wicked smart, which explains why she wanted to date me.

We were halfway through our junior year when we met and had great chemistry from the start. We attended UConn and lived in a group of dorms affectionately called The Jungle. We met at a floor party on the day my niece Gabrielle was born. Apparently, Becca liked the fact that I was quite excited about this and bragging about my niece at the party.

This particular night, my friend Rui was “working on” Becca’s friend, Melissa, and his efforts resulted in a group of girls partying on our floor. Contrary to logic, our studly group of friends did not often have large groups of girls on the floor.

During that first night, Becca and I learned that we had matching 1990 Chevy Luminas (surprisingly the least stolen car in America), she learned that I was a bouncer at the Civic Pub (the coolest bar on campus), and I learned that she liked to drink White Russians and had CF.

Over the next weeks, we met for coffee, had a few dates, and came to know each other’s friends better. My favorite times were the meals. I distinctly remember going to brunch early on and thinking she would be surprised by the amount of food I had taken. I had two overloaded plates and four drinks (small glasses). But when we returned to our table, she had just as much food as I did! I looked at this girl who was 105 pounds (literally half my weight) and remember thinking, Who is she trying to impress?

I knew there was no way she could eat that much food and as I finished my first plate, she was only halfway through hers. She was just cutting tiny pieces, chatting, taking her time. I could not tell if she was slowing down, but it seemed like it. By the time I finished my second plate, she was finishing her first. So then I just sat there watching her chip away at it one little tiny piece at a time, just cutting, biting, and talking. When she was halfway done with her second plate, I was thoroughly impressed and began to wonder if she could actually finish it all. She just kept going! Before I knew it, she was sopping up the last remnant of syrup with her last piece of pancake, and it was done. Simply amazing.

Back in her room, I was surprised to find she had Slim Fast shakes. I learned that she used to drink them with meals for extra calories. She also had high-calorie Scandishakes that she would mix up with whole milk or cream. She gave some to our friend Big John when he broke his jaw during intramural softball and had it wired shut.

She began sitting at our table and my other friends got to know her. One morning, she came to the table with one plate of breakfast foods and a second filled exclusively with bacon. My friend, Norm, looked at it and asked so the rest of our group could hear, “Is that a full plate of bacon!?”

To which Bec replied without pause, “Shut up, Norm!”

“Hold on, that plate is piled with only bacon! You must really LOVE bacon.” Norm said as he poked at her some more.

“You shut up, Norm. So…I like bacon.”

“You must like it a lot.”

“I do, and I’m going to eat all of it.” And she did!

Yes, Becca fit into our group pretty well. Actually, that exchange became a running joke between her and Norm who always offered to make bacon for her when the opportunity arose.

It was greatly entertaining to be with a girl who was not sensitive when it came to food or her weight. Her strong love for Ben and Jerry’s New York Superfudge Chunk inspired my first nickname for her—Superchunk. Though she did not like it in the traditional sense, it did not seem to bother her. To me, she was this little superhero who could eat whatever she wanted, was stronger than she looked, and was smart and spunky to boot. We just clicked.

The fact that we got along so well made me worry that she was the one. Early on, I had to make the call whether or not I should go through with a relationship that if successful, could end in heartbreak too soon. Thirty years old seemed like a long time away from twenty…but not long enough. I imagined dealing with the pain of loss and becoming a middle aged widower having to start my life over. That seemed scary and tragic. At the time, I thought early thirties was middle aged…but regardless, it was a very hard decision for me.

Was I assuming too much? Maybe it would not last that long. However, after a few short months, we were spending all of our free time together and not dating other people. I imagined breaking up and feeling like she was the one who got away. That was the point when I figured out the first of many lessons. With a lot of introspection, I decided that a more serious relationship was worth the risk. I did not want to be plagued with regret. I had figured out what I wanted.

 

Lesson 2: Sometimes you have to trust your instincts, take a risk, and not focus on the worst that could happen.

 

Becca had previously expressed that she was not interested in a long-term relationship, but being an expert on women, I understood that she was sure to change her mind. I had come to the conclusion that we should move forward. I knew she was quite smart, so I felt that, logically, she would come to the same conclusion. Having sorted this all out, I had the conversation.

Couple1 98Unbelievable as it was, she turned me down. She had just come out of two back-to-back long-term relationships and would be leaving for Virginia at the end of the year. This would be a six-month clinical where she would work in a laboratory for some relevant real world experience. She was determined to not have the ties and complexities of a relationship when she left, so we continued to date, keeping the status quo.

As the spring semester came to a close, I knew that I did not want to commute ninety minutes up to Storrs if we were not serious. I could meet someone local, save gas, and take advantage of the fact that my license would soon tell the world I was twenty-one. So on spring weekend, instead of heading to a kill-a-keg party together like we had planned, I went there with my friends and gave her a call from the party. It wasn’t that far off campus and she could just meet me. She was…displeased. She did not come out that night and that was fine with me. The plan was in motion.

It was an easy way to start the breakup process. I decided that I was not going to drive up from Brookfield every weekend, so we might as well end it when the semester ended. I would be a little less agreeable, she would get annoyed with me, and then I could break it off nice and clean. It was a flawless plan.

Well, it was a nearly flawless plan. The next day, I felt guilty so I bought her a flower and apologized to her. My intentions had not changed, however. When we arrived at my place, I started “The Talk.” We were going to be living apart during the summer. We would both be busy with work and friends. She was leaving for a clinical shortly after that. Then we would be living at a distance for over six months and we had not even dated that long. It was all coming out so well until she said, “OK. Let’s do it.”

“Uhh…let’s do what?”

“Let’s be exclusive. Let’s agree not to date anyone else.”

I was confused and unprepared because I had not planned for her to throw a curveball halfway through my well-rehearsed, brilliantly executed breakup speech. I had intended to let her down nice and easy (and maybe stay in touch for when she returned from clinical). Needless to say, my nearly flawless plan failed. Or I suppose you could say that my original plan succeeded. Yes, let’s go with that.

Part of what I liked about her was her attitude. She had dealt with so many obstacles in her life yet she was unfazed. Once she left for college, she was on her own and self-sufficient (and had been since she was eighteen). She worked to pay her bills, earned scholarships with her academic performance, and still found time to hang out and enjoy life. She would get her college degree even though the math suggested that she would not live long enough to pay it back. She was determined to achieve her goals.

As in any relationship, the longer we were together, the more I learned about her motivations and who she was. For most of her life through college, Becca had stayed relatively healthy. She dealt with mealtime pills (digestive enzymes), insulin injections, breathing treatments, and coughing fits, but she managed to live an active life. She was incredibly motivated and focused on her academics. This was even more impressive after hearing her story.

Rebecca was born in Milford, Massachusetts, in 1977 to her mother Brenda, who was sixteen years old. Becca’s father Scott was only three years older. In her first months, Rebecca was diagnosed with “failure to thrive.” She almost died and was diagnosed with cystic fibrosis at six months old. At ten months old she weighed 11 pounds and was 24 inches tall (the size of an average two- or three-month-old). At less than a year old, she had experienced pneumonia, a pneumothorax (collapsed lung), and sepsis (organ injury from infection).

By two years old, her parents had divorced and she moved to Florida with her mom. It was there where her mom married Pete and her sister Jessica was born. Becca grew very close to him and he treated her like his own daughter. She always reflected on this time of her life with affection—except for the heat and sweating. Florida is damn hot.

She described herself as precocious and explained that she would always carry a purse around filled with “proper things.” She would make her sister play school and office and was convinced that she was the reason why her sister hated school.

When Becca was seven, Brenda and Pete divorced, which was tough on her. Fortunately, visitations with Pete continued for both her and her sister which helped to ease the transition and provide some stability. She had grown extremely close to him and thought of him like a dad (rather than as a stepdad).

At ten, Rebecca was given the choice to stay with her mother in Florida or move in with her biological father Scott and Viola, her stepmother, in Maine. She chose Maine, partly because the situation was more stable. She remained there through high school. During the summers, she would often visit her mother, who had remarried and moved to Idaho.

Living in Maine was a major change from Florida. Her father and Vi were very religious and often strict. Scott was a quiet man who had learned the farrier trade (shoeing horses). This is tough work in the cold winters and he often put in many hours. Vi stayed home and was very involved with Becca’s upbringing.

Becca was a good kid but often butted heads with Vi. She had come from a much less rigid environment and now she was not allowed to listen to her favorite Kenny Rogers and Heart cassettes–only Christian music. She had many chores maintaining the house along with taking care of the horses. In school she managed to maintain all As and, despite her lung disease, excelled on the cross country team.

From a young age, Becca developed a strong focus and the character that would shape her into the woman she became. In addition to all of her medical issues, Becca had a speech impediment. She had worked to overcome her stutter since childhood but still had good days and bad. Then at sixteen, she was diagnosed with cystic fibrosis related diabetes (CFRD). She became insulin dependent. She now had another daily challenge to face.

She struggled with the fact that she did not have the same social freedoms that other kids her age enjoyed. At one point, Vi told her she could not attend her senior prom with her boyfriend. She was relieved when her father stepped in and allowed it.

When describing her upbringing, Rebecca was always sure to mention that the stability of her home in Maine was good for her. She never regretted taking the steady route because she knew it was what she needed most at that time.

While Bec was in high school, her mother had two more children, Jordan and Hannah. Rebecca was thrilled at the idea of a little brother and another little sister. Though Brenda divorced again a few years later, Bec made sure to call them and send gifts on every birthday. She had a sense of responsibility that was impressive. By the time of her high school graduation, Becca was more mature than most others her age. She was determined to pay her way through college, and was completely independent once she arrived at the University of Connecticut. She had received several scholarships and student loans. She also got a part-time work-study job during the semester and worked full time over the summers. She was taking care of herself at eighteen years old while taking a tough course load and excelling. She was a force to be reckoned with.

At the same time, Brenda struggled through several failed relationships, turbulence at home, and the subsequent loss of custody of Jordan and Hannah. This created a challenging environment for Rebecca’s teenaged sister Jessica. After hearing some concerning stories from Jessica about their living situation, Rebecca did not want to cross her mother, but felt she needed to do something to watch out for her sister. She knew that Jessica should not stay in that environment and required something more safe and stable.

Though they had lived apart for several years, Rebecca adored her sister. They were different in many ways because Jessica was relaxed and more of a free spirit while Bec was very goal-oriented and focused. They were like a mini female Odd Couple.

Knowing that it would risk her relationship with her mother, she told her stepdad Pete about the situation. He immediately stepped up and took Jessica in to live with him in St. Louis. It took a lot of courage for Becca to stand up for Jessica and to involve her dad, but she saw it as black and white. Even though she was young and barely on her own, she was resolute and knew what she had to do. As a repercussion, her mother did not talk to her for about a year. Becca dealt with this in her typical, realistic fashion. She missed her interaction with Brenda, but knew that her actions were necessary. It was soon after that I met Rebecca. We did not talk about it much since Bec knew she had done nothing wrong and was so focused on what she needed to do at college. Meanwhile, she continued her efforts to stay in touch with Jordan and Hannah and was even able to plan occasional visits with them. I remember learning her story and being surprised that she was so well grounded.

Just before our one year anniversary, she left for her clinical in Virginia. It seemed unfair that she needed to pay full tuition (even though she was not taking traditional classes), work a forty-hour-per-week clinical for no pay, and then get a part-time job on top of that to cover room and board. She managed it by pushing herself past her limit. Up to that point, I knew her signature cough but I had not really seen her sick. Her body was tired from working so many hours while getting too little sleep. As the spring arrived, I listened over the phone while she coughed so much more and had almost no voice. I tried to convince her to work less but as I would come to learn over the years, I am less convincing than I give myself credit for.

Pushing past limits makes for great inspirational posters, but loses its luster when your girlfriend ends up in the hospital with pneumonia 400 miles away. I traveled down to visit her in the hospital and was surprised to see how truly sick she had become. I remember walking toward her room and seeing an incredibly skinny arm reach up toward a nurse. As I got closer, I realized it was Becca. She was petite to begin with, but the weight loss from pneumonia made a significant impact on how she looked. I realized that I could not leave her there. She would work out the details of the final weeks of her clinical with UConn and the institution, but her health was first and foremost.

She was discharged with home IVs and I asked my parents if she could come and stay with us for the last weeks of the summer. They had only met Rebecca a few times at that point but they were happy to help. They knew her story, clicked with her personality, and had a great deal of respect for her. As soon as she was healthy enough, I loaded up our two Chevy Luminas (mine being the Eurosport edition) with all of her things. We set up Bec with her IV antibiotics and I duct taped her insulin pen to the AC vent (which I later learned was unnecessary) and we started the drive back to my parents’ house in Danbury.

Typical expectations would be that a twenty-one-year-old college student renting a pre-furnished apartment would not have a lot of things. This was not the case. Rebecca had THINGS. She loved to shop and get discounts and introduced me to the term “retail therapy.” Needless to say, the packing of our Luminas was an amazing engineering feat, only possibly rivaled by Edison or Da Vinci. We could even see out of the car windows!

That whole experience drove the point home that getting enough rest was essential to her staying healthy. Over the years, I would see it occur again and again. Her CF exacerbations would often be preceded by a period of insufficient sleep and too much stress.

It was back at my family’s house in Danbury that Rebecca saw her mother for the first time in several years. They had never really settled what had happened, and they had just started talking again. She did not have much money, so my parents said she could stay with us as well.

A peek into Becca’s closet triggered a lecture from Brenda who told her that she was too materialistic and should spend less on her clothes. She compared Bec’s budget to her own, which she suggested was better managed. By this time, Becca had been paying for both her college tuition and living expenses for three years with very little financial help from anyone. Her father Scott helped as much as he could with tuition, but for the most part Becca managed on her own. Brenda was not completely wrong, Rebecca had a few too many sundresses from T.J. Maxx, but it came across as a parent scolding a reckless teenager. Rebecca was an adult who had handled everything life threw at her, and this lady who had not played a prominent role for eleven years was preaching at her.

I prepared myself for the intense Becca response that I had come to know, but it never came. If anyone else had done that she would have told them where they could stick it. Nobody was helping her with her bills and she was clearly capable of managing them. She did not come back with the bold confidence that I had come to expect, she only acknowledged that Brenda was probably right.

Perhaps it was because she had seen her mother so infrequently since she was ten, maybe it was the year of Brenda’s silence, but it seemed that Becca was willing to deal with it to maintain the relationship. Perhaps I was an engineering student and Psych 101 did not give me a full enough arsenal to psychoanalyze their relationship over one long weekend. Ultimately, it was their relationship and I was not about to pry.

In any case, we would only see Brenda one more time before we graduated. She had just gotten married again, and was passing through with her new husband. I cannot remember his name; just that they seemed like a poor match on so many levels. It was clear to me that Brenda was very driven by emotion and impulse. I think this had been good for Rebecca years earlier, when Brenda kept pushing until the doctors diagnosed what was wrong. I did not need to understand Brenda to know that without her, Becca would not be here. Beyond that, she was fun to hang out and have some drinks with. She was quite a character and it was nice to feel that I could talk freely and did not have to watch what I said. We got along well. Interestingly, her actions drew a sharp contrast with those of Rebecca’s. I found it impressive that Rebecca turned out as grounded as she did in the face of such an unusual personality.

Couple2 00Graduation came faster than we could imagine. More time together had meant more time to see how this progressive disease worked. Fortunately, it worked slowly during that time. I saw her through several sinus surgeries, but that was typically less than once per year. It was still tough to hear her coughing fits but I became more used to it with time. Early on, she would comment, “I’m dying.” I knew she meant nothing by it, but her mortality was so much on my mind that the comment was piercing. My mind would instantly flash forward to this perceived future loss. This feeling gradually decreased with time as her situation became part of the norm for us.

 

 

CHAPTER 2

 

Getting Serious

 

As with any relationship, it is natural to get into arguments or have disagreements over each person’s roles and responsibilities. In that respect, our relationship was no different. We both had very strong opinions but we also did a good job in finding common ground. “Pick your battles” became our unofficial mantra in this area. I felt that in many ways, Rebecca’s illness gave me perspective through these times, helping me to keep my eye on the bigger picture. However, there was still a learning curve.

Initially, empathy for someone you care about makes you want to give them everything they want. The logic is that time is precious and there is no need to waste it arguing about the little things. In the end, it is all little things. As bad as it sounds, I would often try view it from the perspective of myself looking back after she was gone. Would this have mattered? Was it worth arguing about the fact that she wanted to try some new, expensive wine with dinner? It was better to just agree with her and let it go.

That logic would work for a little while, then the feelings of resentment would kick in. When I reasoned that it was not worth an argument, I was also reasoning that it was not worth arguing for my opinion. During times that I took that stance out of guilt or to avoid regret, frustration would build. That feeling could continue for only so long before I found myself re-starting an argument on the subject because I needed to have my side understood. The feeling that I had to withhold my words because she was a delicate flower did not hold water. She did not present herself that way nor did she argue that way. In fact, she prided herself on being tough, as do so many people with CF. She was half of a partnership but I was the other half.

 

Lesson 3: Pick your battles but argue for the things that matter to you; it is better than holding a grudge.

 

The perspective that CF brought was that there was little time. We needed to approach each other with respect for the other’s perspective. Maybe we would never see eye-to-eye but if we could develop a running rule, we would be fine. For example, we could try to find new, good wines at the store but when we were out for dinner, we would just find a good pairing. That way it doesn’t have to ruin the next date night.

There is a possibility that these were not groundbreaking relationship developments, but it worked because we worked on it. Ultimately, I wanted to look back at our time together and feel I had given it my best shot and had no major regrets. This may be a morbid way to look at a relationship but whenever I did, I could use reason to guide me toward the best path. It seemed to work for me.

When it came to her health, the most immediate issue was often her blood sugar. Bec put a lot of effort into managing this and she would try to keep her blood sugars in a tight range—80 to 120 mg/dl. She was also very small with little body fat. As I would later learn, this would contribute to her being a brittle diabetic, which meant that her blood sugar could change quickly. Seemingly minor mistakes could have fast and severe effects. She carried a glucometer to test her blood sugar and over the years, used needles, an insulin pen, and finally an insulin pump to manage her injections. Every three months, she would have a blood test called hemoglobin A1c that would indicate how well her blood sugar was controlled.

Low blood sugar was risky and could cause someone to slip into a coma with the worst case being death. If her blood sugar fell too low (hypoglycemia), she would get disoriented or find it hard to concentrate. She would typically know if it was going low but not every time. This was a scary reality and became scarier when considering that it could happen when she was driving. I could sometimes tell it was low when we were talking. If she had a tough time trying to understand a simple concept, we would stop and test.

Going high meant that she was experiencing hyperglycemia (not marijuana). It did not lead to the same immediate concern as a low blood sugar but over time, it could lead to neuropathy (nerve damage) or even amputation of the extremities. Not taking enough insulin with meals was the major cause, but sickness and certain medications—like prednisone or other steroids—would also drive her blood sugar high. High levels made it difficult to gain weight as well. She could usually tell if her blood sugar was high when she had extreme thirst that she could not seem to quench. Though she would do her best to manage it, sometimes she would get focused on work and forget to eat or occasionally eat and forget to take insulin. Forgetting insulin is one thing but I never understood how she could forget to eat. I often remembered to eat hours before I even required food.

When she would go low, she would get to go through the cabinet and find the junkiest, most sugary deliciousness and eat it in front of me. In a show of support, I would also eat whatever junky delight she would discover. Since I attempted to eat healthy most of the time, this was clearly a selfless act on my part.

She had one instance of low blood sugar when I was driving. She argued she would just have some candy when we got home in ten minutes. I stopped anyway and dropped her at the door of the grocery store to quickly grab some snacks while I parked. In my rearview mirror, I could see her walking with an odd limp. I hopped out of the jeep and raced into the store to find her sitting on a bench at the entrance. She had forgotten why she was there. I bought some skittles and as she ate them she slowly came back to reality. I imagined what could have happened if she was driving and decided to go home. She could be in an accident somewhere and even if it was just a fender bender, her blood sugar would just continue to drop. We needed a better plan.

The next day, we went to Costco and bought a case of skittles. We put them everywhere: her pocketbook, night stand, both of our cars. We always had skittles with us and we would bring them out at the slightest sign of a low blood sugar. She came to hate skittles. We eventually switched to juice boxes, but from then on we had a plan.

With college behind us and over three years as a couple, there was a new challenge on the horizon: marriage. I still held one main concern as I made this decision. I knew that with CF, there was a strong chance that she would not be able to have children. I had always wanted kids. Of course if we had problems, there was always in vitro fertilization (IVF). Additionally, if we had kids and she passed away when they were young, I knew it would be tough on them and I would be left alone to raise them. In the end, I knew I had no guarantees that this would not be the case even with somebody else. The only difference was that here I knew the odds. Knowing that we could not plan for everything, we would just have to figure that out when the time came. She was worth it to me, so it was just a matter of when to pop the question.

We both had our plans as far as the timing. I was going to give it a little time and enjoy going out with my friends since we had just graduated. Rebecca had moved into a nice place up in Branford with a roommate, buying me plenty of time to make my decision. When I was ready, I would find the right moment and ask. It was a solid plan.

Rebecca’s plan was similar but had several more specifics. She decided that she wanted a fall wedding. She also determined that she needed eighteen months to plan appropriately, and then presented me with a date by which time I should propose. She had also made it clear that she wanted a princess cut diamond with two smaller stones on the side and a platinum ring. The carat weight was not important, but ideally, it would look “antique-ish.” She said the rest was up to me, though it did not seem like there was much else. She provided me with the specifications frequently and would even quiz me to ensure I remembered the details. Her ring size, however, was provided only once. As the time came closer for me to propose I could not ruin the surprise and ask.

Her proposal target date came and went. When her birthday arrived in June, I thought a mountain bike would be the perfect gift. We could spend time together riding on the trails and it would be a great workout. Surprisingly, she was not as excited about my gift as I had expected. Perhaps it was because she was expecting something else.

Not long afterward, I was ready to buy a ring. The challenge was that I did not know her ring size and could not ask her without arousing suspicion. Like a good engineer, I snuck into her jewelry box with my digital calipers and measured the inside diameters of several of her rings, averaged them together, calculated the circumference, then used an online chart to determine the size. The result did not feel right because the standard deviation was too large and the average was quite far from what I had anticipated. It turned out that they were rings worn on all different fingers. So in the end, I guessed. I went to New Jersey with my brother-in-law Michael, to where he had purchased my sister’s ring. I found the perfect one.

I decided to propose at a party she was hosting with her work friends one Friday night. I had the ring shipped to my sister Rachel’s house and had to pick it up before arriving. She had given me a rose from her garden, some champagne, and two flutes for the big moment. It was going to be a big night with a private proposal and a party afterward to make it public. It was another perfect plan.

Traveling on I-95 on Friday at rush hour was not exactly ideal and I showed up late to an annoyed girlfriend. As I got dressed, she came into her room and started cleaning up after me. She grabbed my shirt and went to put it in my duffel bag. The ring, rose, and all of the goodies were on the top of the bag and she was seconds away from ruining her surprise. As she reached for the zipper, I had to act quickly. I stopped her with, “Why do you always have to clean up? I’ll pick up my own stuff, just leave me alone and let me get ready!”

Success and failure. She tossed my shirt down with a, “Fine!” and stomped out. Good job, me.

The night went on and I knew the time was not right. Between getting a subtle sense of the mood and feel of the event coupled with the fact that she got super drunk, I brilliantly determined that it would be best to delay the proposal and focus on catching up to her buzz level.

The next morning while she was cleaning downstairs, I laid out a rose on the bed and set the champagne and flutes on her night stand. At this point, the champagne was just for show because we were both way too hungover to partake. I called her up and she ignored me, still unhappy about my lateness and attitude the night before.

I asked her roommate to call her and she came right up. She stopped in the doorway, squinting at the bed in disbelief. I pulled her into the room and closed the door. I intended to get down on one knee, but she was so stunned that she was shaky on her feet and looked like she might tip over. As I stood there holding her up, I asked her to marry me, she just cried. I knew that meant yes even though I did not get my answer for a minute or two.

The next year flew by. Even though Rebecca did not have eighteen months to plan the wedding, it was a success. The day came and Rebecca looked beautiful. My best man Norm gave an amazing wedding speech that nobody could forget. The slideshow I put together was a hit. Not a detail was missed and everybody was there. Rebecca danced with her dad and her stepdad. I danced with my mom and had a dance off with my dad. It was perfect.
Couple3 03

 

CHAPTER 3

 

False-Starting a Family

 

We had an amazing honeymoon in Aruba, but not long after we returned, it was announced that the Remington Shaver plant in Bridgeport, Connecticut, where I worked, was being shut down. This meant I would either have to accept a corporate relocation to Wisconsin, or join my recently laid off wife on the couch. We opted for the move.

Becca found a job quickly. She had decided that lab work was not for her and set her sights on becoming a clinical drug trials monitor. She was able to get an entry level position at a company in Madison and after a year and a half, she was offered the position she had wanted.

The University of Wisconsin (UW) hospital provided great CF care and Rebecca was a compliant patient. She did all of her breathing treatments, managed her blood sugar, paid close attention to sinus issues, took her digestive enzymes, while at the same time working full time. It was around this time when she started with her tune-ups. UW focused on prevention and when she would start to feel sick, they would act quickly to put her on IV antibiotics.

At the time, this would happen about once per year. She would go in the hospital for about two weeks and come out much healthier. The only problem was that it is tough to get good rest in the hospital because the nurses come in the room around the clock for a variety of reasons. She would occasionally do these IVs at home. This was much more work on our end (mostly Rebecca’s end) depending on the timing of her meds. She would often have to wake up in the middle of the night to infuse a dose. The benefit was that she was in the comfort of her own home.

My biggest complaint was the fact that she could not slow down. She was supposed to be home resting and she would be puttering around the kitchen, cleaning her office, checking emails, basically trying to check things off of her to-do list. I found myself chasing her back to bed ALL the time. She recovered the quickest when she was getting her sleep and not overexerting herself, but she had a busy mind.

During these tune-ups, they would typically place something called a Peripherally Inserted Central Catheter, or a PICC line. This is a long catheter placed intravenously that allows for infusions for a prolonged period of time. It would be placed in her arm and the catheter, which was about two feet long, would extend to just over her heart.

Toward the end of one tune-up, Becca had to leave for a business trip the morning after her last infusion. She was done with her regimen of antibiotics and ready to get the PICC line out. If she kept it, she would have to flush it daily with saline and heparin (anti-clotting medication). She decided that this was too much of a hassle and that we could pull it out ourselves.

I am not sure where she got this idea but she proposed it to me with a great deal of confidence. “You just have to put pressure on the insertion point and pull it out at a slow consistent rate,” she told me. It took some convincing but I finally agreed to help her with it. She sat on the toilet and I positioned myself in front of her. First I removed the dressing and clipped the stitches holding the end in place. Becca was such a seasoned patient and do-it-yourselfer that she sat there with all of the confidence in the world. I put pressure on the site then started to slowly pull. Then things went bad.

When a little of her warm blood dripped onto her leg, she started to get light headed. She tipped forward toward me with her eyes still open, beginning a slow moaning exhale. I had one hand holding gauze at the insertion, and the other hand pulling the PICC out (at a slow steady pace) so I was forced to catch her between my neck and shoulder. I still had to keep pulling but now in this new position, I loosened my grip on the gauze and more blood started flowing out. Had The Walking Dead begun to air, I would have been more cautious about exposing my neck to her, but her moans were unnerving regardless. I finally got the rest of the line out and increased pressure on the site. I waited and watched while she slowly stopped moaning and started blinking a little more. As she was looking at me I could see her slowly reappear behind her eyes. She was confused, wondering what had happened. There was blood in her lap and all over the toilet. Her PICC line was on the floor as I knelt in front of her in our bathroom waiting for her to be alert enough to tell her, “NEVER AGAIN! I WILL NEVER DO THAT AGAIN!!! EVER.”

“That was weird.” she told me.

“WEIRD?! That was weird!?! Do you know what happened? I will never, ever, ever do that again. That was awful!”

“I’ve turned into a weenie.” She told me.

“You’ve missed my point entirely. If the apocalypse comes and you have a PICC line, we will roam the earth looking for saline, heparin, and alcohol pads because I am never pulling one of these again.” I was stuck in a precarious situation with nobody else around performing a pseudo-medical procedure that SHE had talked me into…and she was reflecting on how she used to be tougher!?!

From that point forward, I stuck to my no PICC line removal stance and have never compromised. Some days when I would think of it, often when she got a new PICC placed, I would proactively remind her of my uncompromising position and that she would never convince me to remove it. She would roll her eyes at me unfazed by my bold stance. I would later learn that she had experienced a panic attack. This info would do me no good but at least I had an explanation.

It was not long after we moved to Wisconsin that we decided to start a family. Rebecca was the first to say she wanted a baby, though she worried about her fertility as a result of CF. She figured the sooner the better while she was still healthy enough. Our main concern was her well-being. Would she be healthy enough to carry a baby and if so, would the pregnancy cause any negative long-term ramifications?

My other concern was her life expectancy. I had to be ready to take on full responsibility of this child at any time. We were twenty‑six and the average life expectancy for someone with CF was in the early to mid-thirties. I needed to determine why I wanted a child. It could not be about appeasing Rebecca because I would ultimately be a single father with all of this responsibility on my shoulders.

I wondered if it was fair to bring a child into the world knowing that its mother might not be around to see it off to school. I reasoned that life is a blessing and that we would do our best for this child. I remembered that they thought Becca would not make it to high school and here we were in our mid-twenties. I also remembered my reasoning from when I decided to get together with Rebecca. It is impossible to predict the future so I did not want that to paralyze myself in the present. Anyone can get hit by a car tomorrow but that should not stop you from living today. We had heard that there were people with CF who were in their fifties and some who had children. We needed to live our lives. I was ready.

We went to her CF doctor and discussed this with him. He told us that there was not any major risk to Rebecca at the time. She was healthy, and as long as she continued to be compliant, he would expect her to do well. He commented on how much help she would need from me which was something I had expected and would gladly provide. He eased our fears about the process but stressed that she would need to let others help her. She was accepting of this and we even talked about the potential of asking her mother to stay with us for a few months after the birth.

We were aware that many women with CF had trouble getting pregnant but we were going to give it our best shot. Rebecca and I were excited about the possibility of a child. As always, she did her research—lots and lots of research. She took temperature measurements to know when she was ovulating, she took prenatal vitamins, went to an acupuncturist…whatever we could do to make this happen, we were going to try. My job was pretty simple but being a good husband, I was willing to “help” when called upon. In fact I had a standing offer of “help.”

At the same time, her mom and sister were trying to convince her not to do it. Despite what the doctor said, they worried that this was going to weaken her and shorten her life. Brenda told her it was selfish of me to push her toward this and Jessica worried she would not accept anyone else’s help. Technically, it was Rebecca pulling me toward this but I suppose the in-law is always going to be the bad guy.

After trying for several months, we recognized that we would need the support of a fertility doctor. The issue appeared to be CF related. We decided to start with a process called intrauterine insemination (IUI). The process itself was simple but waiting two weeks to see of it had worked was draining. They told us that if it did not work by the third try, we would need to try something else.

Then on the first try, it worked! We were so thrilled at the possibility of having a baby. It all seemed so surreal. We started to really imagine what it would be like and we were anxious for the first trimester to be over so we could start to tell people. Unfortunately, our celebration would be short-lived. We lost it soon after, and Rebecca was heartbroken. We tried to focus on the positive side: it had worked. If it worked so easily this time, we were bound to succeed eventually. We went back for a second and third round but to our disappointment, nothing came of it. At this point the doctors told us that our best option was in vitro fertilization (IVF).

We went for two rounds of IVF with no results. We planned on doing a third round, but our insurance would no longer cover the procedure so we didn’t go through with it. It felt like we were so close, but positive results were elusive. Time between procedures had stretched longer and longer because of the time it took to recover emotionally from each failure. We began to realize that we had two real options left—surrogacy or adoption.

I had considered adoption several times and struggled with a final decision. Do I want children? was not the right question. It was WHY do I want children? It took a lot of reflection, but I decided that I wanted children because I wanted a part of us to live on. I also wanted to always have a part of Rebecca with me. I admired those loving parents who chose adoption, but it did not line up with the reason why I wanted a child. Maybe one day those feelings would change, but at that point and in our situation, I did not want to adopt.

The other option was surrogacy. To our excitement Jessica agreed to be our surrogate. We still had some embryos in storage from the IVF sessions and once we were ready, we could pull the trigger. Jessica already had a son Cole, who was now a few years old. She was getting married and told us that she would want to wait until after she had a child with her husband. A few months later, she told us that she had changed her mind. It was tough news to take.

She had conflicting feelings as to the reasons for her decision which included the concern that Rebecca would wear herself out with a child. Rebecca knew that she needed a lot of support with this child but that was something that would have to be taken at her word. I also knew that she would do better as a stay-at-home mom rather than continuing to travel and work crazy hours, particularly because we could help her with a child. On the contrary, when she had to get a report filed by midnight then pack a suitcase for the next day, she was on her own.

Jessica also felt that I should be willing to adopt first. She changed her mind a few more times but ultimately she decided not to do it. This was an emotional roller coaster for the two of us as we switched back and forth from having a plan to not knowing what was next. Our friend Meg also offered and so did Rebecca’s other sister Hannah. It was incredibly selfless for Jessica, Meg, and Hannah to offer to make one of the biggest sacrifices someone could make for someone else. But as time passed we questioned whether this was what we still wanted.

Rebecca had become so much more involved in a career that she loved and I started to worry about our chances of success with the procedure. A surrogate could cost tens of thousands of dollars and after paying for IVF medications, I was in no hurry to gamble on another “chance.” Time passed and Rebecca had more frequent exacerbations and tune-ups. She had little energy outside of work, which consumed so much of her time. We spoke about it less and less as her health began to decline.

 

Hope you enjoyed the first 3 chapters! Click here to purchase Lessons from a CF Cornerman.

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