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Cystic Fibrosis

Cystic fibrosis (CF) is an inherited, chronic disease that interferes with the respiratory, digestive and reproductive systems. Mutations of a specific gene (the CFTR) affect the cells that line the organs of these three systems. This defective gene causes the production of thick, sticky mucus. It builds up and clogs the small airways and passageways which carry harmful bacteria out of the body. Because the bacteria is trapped, serious infections develop that weaken the organs and place constant stress on the immune system.

Living With Cystic Fibrosis
Medical advances mean better, more effective treatments are available today to help children and adults with CF live more comfortably. But effective management of the disease requires a variety of medications that are costly, and the newest drugs available are even more expensive; treatment regimens are demanding, and depending on the extent and severity of the disease, normal childhood activities are often disrupted by daily therapy and lengthy hospital admissions.

How You Can Help
Though the research is very promising, there is no cure yet for Cystic Fibrosis. Your gift to the Take A Breather Foundation helps brighten the lives of children struggling with the disease today. Through direct support of individual wishes, we help families to do exactly what our name suggests, take a breather from the psychological, emotional and the financial burdens that accompany living with cystic fibrosis. Children with CF have dreams and wishes and it’s for these reasons the Take A Breather Foundation exists, and why our mission is so important to these children and their families

For more information about cystic fibrosis, please visit the CF Foundation website

A Mother’s Story

There I was, a 30 year old, questioning, debating, doubting the wisdom of our children’s pediatrician, a man who had been practicing medicine for  as many years as I had been alive.  Dr. Will had been my husband Tony’s pediatrician and was a very well respected doctor.  He was also the doctor for our three other children, John 6, Michael 5, and Ame, 2 as well as for our newborn son, Matthew.  My concern in previous “new baby visits” was focused on Matthew’s slow weight gains, compared to his brothers and sister at the same age. Dr. Will focused instead on his alertness, his movements, his smiles and overall well being. During these previous visits, Dr. Will in his very kindly manner tried to reassure me that babies differ in growth patterns, even those in the same family. In response to my concern, he had suggested trying different formulas as well as possibly adding a supplement to his feedings. Tony readily saw this as a possible solution and in his quiet way tried to reassure me that we just had to give Matthew time to catch up.

The basis of my anxiety at this time was the fact that four years prior to Matthew’s birth, our third son, Mark was born. Within a day after his birth, Mark was taken to the NICU, where he remained for two months. After three surgeries to correct an intestinal blockage, our baby son died. It was not until a follow-up post partum appointment did we learn that Mark had Cystic Fibrosis (CF). Prior to this, we had no awareness that Tony and I were carriers of the gene for this inherited disease. Needless to say, we were shocked.  Our families were shocked. Our doctors were shocked.

Despite our desire to trust in Dr. Will’s experience and suggestions, the weeks of watchful waiting and weighing, became months with slow progress in Matthew’s ability to gain weight, and my concern grew. The fear in my heart was another baby who would be diagnosed with CF.  This fear, admittedly based primarily on a mother’s instinct, had been growing, and on this particular day, with my continuing questions, Dr. Will agreed to set up an appointment for us to have Matthew seen at Children’s Hospital of Philadelphia (CHOP).

Within the next week or two, we met with Dr. Sam at CHOP and after a preliminary examination of Matthew, he also questioned my fears about Matthew having Cystic Fibrosis and stated without hesitation that he thought I was just “a nervous mother”. Oh how I wished he was correct!  However, after a “sweat test”, the standard test for CF at the time, my fears became a reality. Matthew tested positive for CF, and we began a new life journey.

So what is it like living with a child with CF? My immediate answer is that in many ways, it is just like living with any newborn, toddler, pre-schooler and grade school child. You love them, you watch their first steps, first teeth,  first funny and endearing words, first not so nice words,  first attempts at toilet training, first fights with their siblings, first days at school and first disappointments, and first group of best friends, …(who have  remained best friends 40 plus years later).

You also live with fear, fear of the unknown, fear of your child catching a cold or being near someone with a cold, fear of how your child will adjust to the routines of chest percussions, fears related to taking the necessary enzymes and gaining or not gaining weight…fear of being able to make life as normal as possible for your CF child and not imposing harsh restrictions on him or your other children in order to protect him…fear based on the fact that at the time of Matthew’s diagnosis, the life expectancy for someone diagnosed with Cystic Fibrosis was six years!

In the beginning with everything about Cystic Fibrosis so new to us, we attended a few CF parent support group meetings. In many ways these groups can be very helpful, as well as comforting, to others who share the same experiences and concerns. For us, many of the stories were sad, too hard for us to hear, and we more often came away fearful and upset about the future. We did not continue to attend these meetings.  At that time, there was no Internet, which depending on the quality and reliability of the reporting, can serve today as a helpful resource.

What was actually more helpful for me was when we went for our scheduled CF Clinic visits at CHOP. While in the waiting area if Matthew was 4 years old, I was looking to see a 6 or 7 year old; when he was 6 or 7, I was looking to see a 10 or 12 year old; when 10….. a 15 year old.  To see these older children, many looking for the most part well and fairly healthy, was always encouraging.   When possible, I might speak with a parent and ask a question or two. Also very helpful was the fact that over the years, and continuing up to today, Matthew has been fortunate in having had excellent, caring doctors.

There were the times during those CHOP visits when a blood test or a chest ex-ray was necessary, and while in the waiting rooms of these various departments, Matthew and I saw many very sick children, some in wheel chairs, some with  physical handicaps, others hooked up to IV’s, etc.  Many times after having seen some of these children, while driving home along West River Drive, aside from talking about the river, the boat houses, (Tony had been a rower) and the changing colors of the Fall leaves, Matthew would often mention something about the obviously sicker or disabled children we had seen. The words were unspoken …but each in our own way, we knew Matthew was luckier than these children.

As the weeks, months and years passed, Tony and I just “paid attention” to this diagnosis and to the impact of CF on Matthew, while integrating as best we could his treatments as part of his normal life…….it became for Matthew just part of his day, inhaling “mist”, getting his “hits”, and taking the enzymes with his meals.

When Matthew was 2 ½, we had another baby, a daughter, who like her older siblings did not have Cystic Fibrosis. Life for us continued with the usual challenges of trying to maintain some kind of order in a household of five children….. plus a dog…..and for the most part, it was like any other active, loud, lively household with young children. Our routine became one of Matthew doing his “mist” while watching whatever happened to be the favorite kids TV show at the time. Tony would then do Matthew’s “hits” upstairs, while I was downstairs in the kitchen getting breakfast and making lunches for whomever was going to be in school all day.

When Matthew was in first, second, third grades, I would go up to school and speak to his particular teacher in the first days of class and explain about his having CF and that he might cough more, but that it wasn’t a cold; that he might need to use the boys rest room more often, and that it wasn’t just an excuse to leave the class; and that his lunch included the taking of enzymes, and that they were necessary for his ability to digest his food. When the older ones came home with colds, upset stomachs, a virus and/or something like chicken pox, we tried as best we could to keep them somewhat isolated. Sometimes it worked, sometimes not…..with the chicken pox, it did not work…. everyone but Molly, the baby at the time, got them.

When it came to telling others about Matthew having Cystic Fibrosis, we would mention it when/if we thought it necessary……to people like the parents of his friends, to neighbors, to his early teachers or a coach, depending on the situation.  We did not hide it, nor did we make it a focus. Throughout all these years, Matthew has always accepted CF, and what that entails, as just part of his life.  Perhaps, that has something to do with Matthew’s easy going personality, or perhaps, it has something to do with his having been diagnosed so early that the CF routines are all he has ever known. Matthew never rebelled against this aspect of his life.  However, when it came to normal teenage rebellion, that might be a somewhat different story!

When Matthew was in grade school, he was nagged, as were his siblings, if his grades slipped, grounded for broken curfews, and obliged to write a poem if the offense was breaking some family rule…this form of discipline I owe to my sister…and Matthew was not appreciative …… even though his cousins had to endure the same punishment. On any given school day Matthew spent most of his after- school time outside playing with neighborhood friends. On weekends, he and his brothers and sisters would generally all be out by mid-morning, either going to a practice related to a school sport or just biking or playing ball, etc in the neighborhood….often coming home only for lunch, as well as for multiple snacks throughout the day, either alone or with half the neighborhood. On rainy or cold days, it was the basement with friends.

While in grade school, Matthew played soccer and football.  In those days, the rule in grade school was you could not play on a team until you were in fifth grade. Football was Matthew’s sport of choice, as it had been for his brothers John and Michael, who had also played in grade school.  With the “fifth grade rule”, we suggested, ‘strongly suggested’, that Matthew play soccer, which was a relatively new sport in this area at that time.  For us, it was about the benefits of the exercise…. the running….. which we thought would be the best thing for him.  To this day when talking to others, Matthew insists “my mother made me play”! Anyway, as soon as he could, Matthew joined the grade school football team, and he continued to play football throughout high school.

In the summer, our family was lucky to be able to spend 6 weeks at the Jersey shore. For many of these summers, my sister, Peggy, who lived in New York would join us for a week.  Peggy also had 5 children……the combined age range of our 10 children when we began this annual visit was 1 year through 9 years old.  Lest you think this was a recipe for chaos, you would be correct!  However, once the Dads left on Sunday, Peg and I fell into our ‘boot camp routine’ which included standard mealtime menus, ……with little variation;   the four block walk back and forth to the beach…..twice a day; the organizing of whose turn It was to be in the shower…..first, second, third etc.; and the deciding of who would sleep  where, and with whom, in the upstairs dormitory with multiple old beds of various shapes and  sizes.

Just as we had our routine, Matthew continued his routine of “mist and hits”, now surrounded by his cousins who would either be watching TV, playing cards or some other games. After the first or second time and a curious question or two, neither Matthew nor his cousins thought anything about this twice daily event going on in the middle of our small living room. Years later, Peggy’s youngest son Greg’s second baby was diagnosed with Cystic Fibrosis. For Greg and his wife there were the natural concerns, questions and fears that any parents would have upon receiving this news. However, Greg later told Peggy that having seen Matthew all those years at the shore just routinely doing what had to be done and then joining the cousins in whatever activity was going on, had helped him to be less anxious and more hopeful for his son.

During these summers, our children had also by coincidence become friends with two families in particular. One family had a son Matthew’s age, Phillip, who had hemophilia…….which meant no rough-housing for fear of Philip getting cut and bleeding. In the other family, Matthew’s new friend had a younger brother who also had CF.  One afternoon, after returning home from the shore, I was driving Matthew to football practice when he was in either 7th or 8th grade.  While I don’t remember the gist of our conversation, something prompted me to ask him what it was like for him having CF and his quick response was, “it’s OK, at least I can play football, which I couldn’t do if I was Philip.”

And in many ways it was OK for Matthew and for us. We felt so blessed…since life was fairly normal and Matthew’s first hospitalization for CF did not occur until his freshman year of college.  In conclusion, I would add, this is our story, our family’s way of dealing with CF, as well as Matthew’s attitude and way of living with CF. Every family has its own strengths and challenges and its own particular way of living with CF. This was just our way.

Matt’s Message

Thank you for taking time to visit our website. I hope you find it both educational and inspirational. I am forever grateful to my family, my friends and my community, who have collectively embraced the mission of Take A Breather, by supporting my efforts to make a difference in the lives of those touched by cystic fibrosis (CF).

Born in 1968, very little was known about cystic fibrosis, other than it was an early death sentence for most. Children with CF rarely lived long enough to attend elementary school; the CF Foundation was still in its infancy and there were very few medications available to treat this disease. Needless to say, the odds were stacked against me from the beginning, but I was unaware of this, because of the conscious decision my parents made to raise me with the same amount of love, guidance and expectations they had for my siblings. I grew up in a small town outside of Philadelphia; one of six children raised in a middle class, Irish catholic family. My older brother, Mark, whom sadly none of my brothers, sisters or I got to know, passed away from a CF related bowl obstruction at 2 months of age…Mark never came home from the hospital. Looking back, I think Mark’s short battle with CF was the catalyst for my early diagnosis.

As my mother began noticing some of the same symptoms in me as she had in Mark, she voiced her concerns until the doctors agreed to give me a sweat test. Fortunately, cystic fibrosis played a very limited role in my childhood as I kept very active in sports, school, and boyhood mischief. Six times a year, my mom and I would make our drive into the city to The Children’s Hospital of Philadelphia where I went for my “CF Check-up”. We both looked forward to these visits for very different reasons; my mom always looked for the older kids in the waiting room and I remember this one particular gentleman who was probably in his early twenties and her always pointing him out. Seeing him gave her encouragement and in turn made me happy. I looked forward to these visits because it got me out of school for a few hours, and I was always treated to a McDonald’s cheeseburger and soda afterwards. One of the first memories related to my disease would occur over the summer going into 6th grade.

My parents had come to pick me up after spending the week with my cousins and they gently broke the news that my friend John had died. I remember crying in the back seat of the car as I processed this news. Was I crying because I was sad, or crying because I was scared…… scared that this would happen to me as well. You see, John was a friend of mine who also had Cystic Fibrosis. My classmates and I sadly watched as John’s disease slowly robbed him of his childhood and ultimately took his young life. Today, we honor John’s memory annually by presenting the John A. Nardi, Jr. Memorial Memorial Award at our signature event. John would be proud to know his name is bringing smiles to other kids with CF.

As I became a teenager, doing treatments and taking meds was never an issue for me. They had become part of my daily routine, just as brushing my teeth, getting dressed and showering were. I didn’t know any different. I did my treatments twice a day mainly to appease my mother, as my outdoor activity was my real therapy. In high school, I continued to thrive as I played on the football team, worked part time jobs and kept an active social life. Self admittedly, I was not an academic over-achiever, however I went on to graduate from St Joseph’s Prep and La Salle University here in Philadelphia. Fortunately, or unfortunately, depending on how you look at it, my first hospitalization didn’t occur until my freshman year of college. I credit this in part to my new lifestyle…. c’mon, I was in college and enjoying my freedom a wee bit too much. It was over the summers that my friends and I would rent houses at the at the Jersey Shore, no, not that “Jersey Shore”. I came away from these summers with some of the best memories and some pretty good stories, but those I can’t get into here.

The beach has always been a big part of my life. As a child, my family would spend our summers in Longport, a seaside town in southern N.J. Today, I live there with my wife. I find being near the ocean very therapeutic, and often wonder if spending my early years breathing in the salt air had a positive effect on my developing lungs. After college, I married my wife Chris, who coincidentally I met at the beach. I had a career in advertising production; we purchased our first home, and traveled to all the warm islands we could find until we found our favorite, St John, USVI. We don’t have children, but love spending time with our nieces and nephews and are blessed to be godparents.

Cystic Fibrosis never stood in my way, and I never tried to hide my disease. I figured if I made it an issue, it would become an issue. However, though I didn’t go around telling everyone I met that I had CF, I did tell those who needed to know. My family, friends and colleagues have always been supportive – and to me, that’s all who matter. When I hit my forties, I noticed my disease progression, this was also the time I was diagnosed with CFRD – Cystic Fibrosis Related Diabetes. In July 2015, reality of the situation finally set in and after a long internal struggle, I finally surrendered to my failing lungs and was listed for a bi-lateral lung transplant. My lung function had decreased to 10% and my freedom was tethered to an oxygen tank. My decision although hard was helped knowing that I had lived an awesome life surrounded by love, support, and the greatest of friendships. I knew as I entered this unknown world of transplantation, I was finally at peace with all outcomes. On November 15, 2015, I received my new lungs and was given the greatest gift – the gift of life. This, all because my donor made the ultimate selfless act a human can make. Every day I am reminded of this with every breath of fresh air I breathe into my new lungs.

I am also extremely grateful to my pulmonary team, transplant team and everyone at the Hospital of the University of Penn for the great care I received. Reflecting on my journey with cystic fibrosis, a story most of us have heard comes to mind. It is the legend of the “Basket of Burdens”, which describes a group of people at a meeting, who were asked by an elderly moderator to write down the three biggest problems/burdens they were facing, and then they were told to fold and drop the papers in a basket. Each person was then asked to pick a paper from the basket, and decide if they would want to switch their problem for the one they chose from the basket. Without exception, no matter how severe they believed their own problem was, no one wanted to exchange their problem with someone else’s. The message this gentleman left them with was “in light of everyone else’s problems, your own worries and problems seem manageable…..and if nothing else, that’s something to be grateful for.”

Each of us in our lifetime is given a cross to bear; cystic fibrosis happens to be my cross. What I choose to do with this cross, has taken on more meaning in my life recently. Most aren’t lucky enough to find life’s answers, and I still have many questions, but I believe I have found one answer, and that is to take my life experience with Cystic Fibrosis and use it to positively impact the lives of others living with this disease. Staying true to this mission, I hope to use my voice to inspire magical moments of hope, strength, and love through the fulfillment of Wishes for those battling CF. The medical advances I have seen in my lifetime are nothing short of amazing. As the scientific community brings us to the doorstep of a cure for Cystic Fibrosis, my greatest Wish is that every child and adult touched by CF may find peace and comfort in their lives and that one day, all will experience a life free of this disease.

I leave you with this, when I was 4 months old the doctors told my parents that I had Cystic Fibrosis and if I was lucky I might live to age 6….this past August, I turned 50. If my story can bring hope to just one person out there, then it’s been a pretty good day. Breathe easy, Matt McCloskey.