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.
