The Story Of A Privileged Parent: Perspectives On Nurturing Two Normal Children Who Happen To Have Cystic Fibrosis.

Welcome to the first of my occasional “guest posters”. I hope you enjoy each of these as they arrive.

Let me introduce you to my mother, Christine. An ol' fashioned Italian Catholic with a warm heart. She has been a major supporter in my life, my rock, and the eternal optimist that never gave up on me or my future.

I asked my mother to answer two frequently asked questions: “What was your family life like with CF?” and “How did your parents care for and empower two children with CF?” What she wrote for me was an open and honest summary of her life as a CF Parent. It is a beautiful story and I thank my mother for laying everything out there for everyone to read. Thank you, Mom. You are the best and I love you...Hope everyone enjoys this post!

Confusion is what you feel when you are told your newborn daughter may die in her childhood years from a disease you've never heard of. Cystic Fibrosis was not well-known when beautiful Angela was born in 1977. I was a faculty member at the University of Minnesota Dental School, a young mother on maternity leave. Since an internet search in the comfort of my home was not available in those days, I spent days researching at the medical school library. The data was limited and depressing. Immense love is what I would need to guide my perspective as a new mother. I was determined that, whatever CF was, we would do everything possible to control its effect. Who knew that two years later we would also be blessed with an amazing son, Joshua, again with this ill-fated disease?

Actually, God knew. I like to believe He chose me as their mother. Here was the opportunity to use my innate gifts to care for these two little souls. It would be up to me how I would handle the special path of privileged parenting for two children needing to find their place in this world. Our goals were to help them develop their own gifts, be good to others, and live their lives joyfully and productively. We would walk this path together. A deep faith from an old fashioned Italian Catholic upbringing, and the example of my own mother, would guide the way.

Angela, my darling daughter, touched so many lives in such lovely ways in her 16 years with us. Sadly, we had to give her back long before we wanted. I am certain she is happy in heaven and is wishing good for all those she loved. Josh, my sweet son, still amazes me after 30 years. How he lives his life bodes him well, and makes everyone he meets happier.

As young parents, their father and I had more added to each day than just watching “the most adorable babies on the planet” grow up before our eyes. We shared hours of hands-on care, pounding on their little chests while they were upside down, hoping to keep those precious healthy new lungs breathing strong and clear. Four times each day, for each child. We had to factor in an hour for each therapy. Yes, every family with children has necessary routines, like eating three meals each day, going to school, doing chores, taking baths, and playing together. Our family had the same focus of having happy children, but our normal routine just included a few more hours of “activities”. We read stories, listened to music, pounded along on their chests, and made them laugh till they coughed. We played games to fill the time while breathing medications in nebulizers (15 minute average for each med) and doing chest therapys (taking 40-60 minutes each time). Until Angie was near age four, it was mostly prophylactic. Then CF began to compromise her lungs. Josh was born with meconium ileus - a CF complication beginning with improper formation and blockage of the small intestine. This required special surgeries and medications, but we were able to maintain his lung functions.

There are mutual benefits to spending more than the average amount of parenting time hugging, kissing, tickling, massaging, and talking with your children from infancy. Their curious minds, intelligence, and personalities blossomed. Their existence was validated. They were naturally authentic individuals who were allowed develop in their own time. Angela was reading and writing before age 4, although she wrote right to left so her name was A-L-E-G-N-A for a while. Josh memorized his dad’s entire Willie Nelson album of golden oldies, and sang them with Grandpa Jerry all the way home from Disney World, just before age 3! Think “Don’t Fence Me In”, “I saw the Light” and “Sioux City Sue”. As preschoolers, they spontaneously sang “Have Yourself a Merry Little Christmas” in the middle of clinic and brought the staff to tears.

We had over 20 prescriptions used daily, including antibiotics, water-soluble vitamins, replacement digestive enzymes, and drugs to open airways and keep mucus from getting stuck in their lungs. Because CF presented itself differently in each child, there was a separate complete cupboard in the kitchen for both of their required medications. We all joked together and said Angie and Josh’s alternate names were “Respiratory Angie” and “GI Josh”, after the old CPR practice dummy, Annie, and, well, you know…GI Joe.

We prepared (yes prepared) homemade distilled water for “mist tents” that they would sleep in, a therapy used into the 1980’s. We ripped out the carpeting and draperies and removed all my houseplants because of Angie’s developing mold allergies. Air compressors and bronchial drainage tables adorned our living room. Within 6 years, there appeared IV poles for at home intravenous therapies and gastrostomy tube feedings for our daughter, as CF continued its fierce battle against her. Our son had innumerable abdominal surgeries beginning the day after his birth. Of the resulting scars, he would later declare his tummy had “three belly-buttons, and a zipper!”

Our family were pioneers of at-home CF care. At age 6, Angela was the youngest child from the University of Minnesota Hospital CF Center to go home on IV therapy. We had to convince both the CF doctors and our insurance company to let us do so. We had respiratory therapists come to our home to assist with the exhausting schedule we took on. At about 10 years in, we had to fight for insurance coverage of the newest “lung shaking vest” chest therapy option, that our own Dr. Warren Warwick invented. We were one of the first families to have it here in Minnesota, again meaning more new “furniture” for our living room! The portable device so commonly used today was a noisy, huge metal cube placed like end-tables on each end of our sofa. We combined hands-on bronchial drainage therapy (BD’s) - now called chest physical/physiotherapy (CPT) - with the High Frequency Chest Compression Therapy (HFCC) or "vest" therapy.

Along with other CF families, staff from the hospital and The Cystic Fibrosis Foundation, we participated in parenting groups, pot-luck holiday parties, picnics and camping and CF camps. Wait a minute! What's that you say? Well, indeed, it was not yet the standard to keep the kids apart back then. Angie was often hospital roommates with up to four other CF kids when she was young. This was way before burkholderia cepacia and other bacteria made their nastiness known, and insurance didn’t like private rooms. Perhaps more research would have been helpful?

Creating memories, for the times when sickness might take over the energy for living, was of the greatest importance. We didn’t have much money, but we had an abundance of love and fun. We played games like bouncing on my exercise trampoline while catching a ball, seeing who could do the most somersaults in the pool, as well as swimming laps. Fun for them, was more “therapy” from our perspective. We would pack up and go camping and fishing, up to a cabin in the woods, down to Disney World to visit grandparents and Mickey Mouse, while using a small ironing board for a “BD” table. We had a schnauzer named Charlie that the kids adored, and who learned to jump through hoops as well as step gently over IV tubing. If they were interested, we helped them learn. They were asked to be Poster Kids for the Minnesota CF Foundation and did so for three years. This gave the kids several special experiences, and it gave me a venue to speak at events designed to raise funding. Angie and Josh were and had good friends.

Angie loved ice skating, dancing, basketball, softball, swimming, playing the clarinet, fishing, reading, embroidering with Grandma Carmella, and telling jokes to Grandpa George. She was always rearranging her room (poor Josh was the "voluntary" moving man) and watching soaps with Grandma Arlene. She watched news all day long, went to Space Camp in Alabama, and kept a poster for missing child, Jacob Wetterling, on her door until he would be found (I still have it). She loved running short races when she was young, and enjoyed being at school. It was especially hard for her when she got older and was too sick to attend high school. She missed her friends every day. She would have thrived with the internet, texting and Facebook, but they didn’t exist yet.

Josh was busy all the time trying lots of sports like baseball, football, and wrestling. He loved swimming, so we put in a pool, he loved sports camp for CF kids at Camp Courage. Josh was always playing with the neighborhood kids in our huge back yard, where we mowed “10 yard” lines in for touch football. He and his best friend made funny home videos all the time. He enjoyed writing lyrics, poems and stories, and his love for singing and everything music continued to grow. He had a Muppets drum set when he was only two, and he never stopped playing it! Later, I gave him a portable karaoke machine, as he was missing his sister, and singing was something he loved. He continues with this most joyful hobby to this day. He considers singing another form of therapy because, like dancing with his wife, it is working his lungs, making him cough, and it’s also a good three minutes he is less aware of how he’s feeling inside that moment.

To take care of myself and be strong enough to do the therapies, I worked out every morning and took bubble baths every night. I expanded on hobbies that did not take me away from home, like gardening, reading, and cooking. I volunteered whenever possible at school and campfire, and taught religion education classes. Their father and I tried to keep our marriage strong, but ended up growing in different directions. I have heard that half of all marriages, with or without children, end in divorce. Sadly, by the end of elementary school age, their father and I parted ways.

I used to say we were “running defense” for our children. In their teenage years, I tried to walk beside them. They chose their physician and discussed their treatment plans as part of the team. My goal was that they would grow up as independent, responsible adults, including their health care, like making appointments and ordering medications, as well as balancing a check book. I needed to trust and respect them. As Josh got older I walked behind him, but he knew I was always there for backup. Josh’s wonderful wife, Carly, came along and now they go arm in arm through the life they have created together.

Our family has been dealing with CF over three decades. We learned we were genetic carriers by having a child diagnosed with the disease. Both parents are carriers of this recessive gene; there is a one in four chance for cystic fibrosis in each pregnancy. There was no confirmed family history, but surely it was there for generations. CF wasn’t even named as the disease “Cystic Fibrosis of the Pancreas” until the 1940’s. Prior to that, infants died from pneumonia or “failure to thrive” or tuberculosis, or no reason at all. The CF gene wasn't discovered until 1989. My children and I have the same ∆F508 gene as discovered in that initial testing, but it took them each down totally unique paths. After our first twelve years fighting CF, the gene was discovered. Add another twenty years and that knowledge has defined many mutations of the gene, but not provided a cure or controlled the effects of CF enough to protect all these children or give these adults their deserved old age. We need more and different research. The scientific process is way too slow for my liking.

The prescribed care has changed, therapies have improved remarkably, gene studies and research for the cure continue, and life span expectations have lengthened to 37 years plus. Those with the disease and those who walk the path with them try to make the best of every day. We are brave. We do not complain. We treasure life.

Today, the newly diagnosed have a much better prognosis; parents have more opportunities to maintain these little ones in better health than we had 30 years ago. Many of these children who were previously given a very limited life span are now awesome adults fighting issues never anticipated at the time of their birth. The problem remains that we are still only treating the complications that come with CF, and trying to prevent every gastro-intestinal and pulmonary exacerbation, and newly defined issues like “cystic fibrosis related diabetes”. Our adult CF population is becoming resistant to the available antibiotics needed for those killer micro-bacterial beasts and panoramic viruses spanning the globe and creeping into our hospitals.

Where is that cure for CF we have been praying for? I have been waiting for a cure since our diagnosis in June of 1977.

My reality is that I buried my daughter as a child. Angela was only 16. She was really sick, and died, because of cystic fibrosis. My son watched his sister lose her energy for this life from the same disease he has. Josh was only 14. This is a sadness I do not wish for anyone. Josh, even so young, had a heart so warm and a soul so wise, it was impossible to not be joyful as we continued our own lives. Together, we slowly moved through the grieving process, keeping our hearts filled with love. We maintained closure and closeness to Angela by visiting the cemetery at significant moments and kept our sanity by doing little things like going to lunch and a funny movie on "Angie's Day."

Life may not have unfolded according to my own agenda, followed the fairytale of my childhood plan, or lived that old so-called American Dream. But my life has filled me with love and a faith that continues to grow. December 1993 was an especially sad moment in my world, as my wonderful mother, Carmella, struggled for 6 months with leukemia and died on the December 10th. She was the best example of what a mother should be, and I modeled my nurturing after her. She was there for our family from day one, learning how to do therapy so her grandchildren could stay overnight, and coming after work once or twice a week to help out or bring gifts and food and love. I needed my mother at my side for her strength and encouragement. During that same period is when Angela’s fight with cystic fibrosis became impossible to win, and she died on December 15th. Their relationship was so close in those final moments; it almost seemed they sensed their future. Losing my mother, and then my daughter within five days, planning two funerals in the same week, and needing my son to have his own major surgery postponed-and ultimately performed after Christmas, was heartbreaking. I felt filled with the grace of God, and was able to move peacefully through it all. My prayers were answered when I found myself with each of them when they were dying. Thank you, God.

My faith continues to guide me, but I remain totally amazed that God had so much faith in me! I am so blessed to still have Josh, and Angie remains a vivid part of our lives to this day. Josh was not an only child; he grew up with a sister who filled his heart. Creating the happy memories and living a joyful daily life is still paying off. Josh has grown up and is happily married. I have since remarried and am blessed with the opportunity to continue growing and sharing my love and my life with my new husband. He is a great guy who quietly loves me and all that comes with me.

The privilege of parenting can be overwhelming at times, but looking into my son’s eyes and seeing that twinkling brightness even when he doesn’t feel well-that is his joyful soul, the soul God trusted I could nurture.

Please, pray for the cure. Please, help by donating for research to make CF go away. Oh, and please, enjoy my wonderful son. He is a gift. From God. And me.

Peace and Love,

Christine

XOXOX

Quick Thoughts And A Request

Here is a quick post about some of the hulabaloo happening in Joshland...

There's been such a positive response about The Breathe Event over the last few weeks. Thank you to everyone who took part in this amazing tribute to Matt Scales, especially CFvoice. I wrote about my experience on the project last month. You can check that post out by clicking here.

Because of the impact "Breathe" has had on the CF community, I was asked to introduce the song at the 2009 Breathe of Life Gala for the Minnesota/Dakotas Chapter of the Cystic Fibrosis Foundation. What a great honor and privilege! I got a little emotional while I was speaking, but everything came together nicely at the end. They raised nearly 1 million dollars towards a cure not including some VERY generous donations before the gala took place. Thanks CFF and all the compassionate donors. Whether it's a dollar or a million dollars, it all helps!

"Breathe" has introduced me to many unknown friends in the CF community who are reading this blog for the first time. My new friend Kat just added Welcome to Joshland to her wonderful non-profit website. The Blooming Rose Foundation was created to help new and "experienced" CF Warriors, as well as their friends and family, find positive support, resources, and information about CF in this vast wasteland we call the internet. Kat, I look forward to working together and giving hope to everyone who needs it. She rocks beyond all reason.

To my new readers...I hope you find my stories entertaining and honest. That is all I've ever hoped they'd be. These stories aren't always about CF topics. A lot of them are simply things that have happened to me...but CF seems to always sneak in there for a moment or two in every story. That is all I will ever give it...a moment or two. My life is far more than this disease and I refuse to give it top billing. I am Josh who happens to have CF. It has taken away more than it has given, but what I've received are lessons that will stay with me forever.

To my loyal readers....You'll be seeing a few different things when you walk through the gates of Joshland. I'm attempting to gather some special people to be occasional guest posters (an idea I got from Ronnie Sharpe and his blog Run Sickboy, Run,which is full of great CF info and topics that cover anything imaginable. Plus, he makes me chuckle and is a goof like I am. How can I not like this guy?) I hope these guest posters will be willing to talk about their relationship with me or perhaps cover a topic that I have received questions about. That is...if they are willing to write. ;-)

I am also opening up the floor to questions or ideas for stories you'd like to hear about. I am always looking for inspiration and who better to help me out than my readers?

I will do my best to answer any questions you have honestly and will gladly take any story suggestions. I may not use all of the suggestions, but don't hesitate to send me your ideas because it may help me pull a different story out of my forgetful brain.

You have a few ways to send me questions:

1. You can comment anonymously on this post with a question or suggestion. Just write "Question or Suggestion" in the comment.

2. If you have no worries of anonymity, you may comment with your name on this post or write me at welcometojoshland@gmail.com.

I look forward to hearing from anyone in the near future. ;-)

Peaceful Things,

Josh

Life Lessons For A Mat Rat

"I can do this...I can do this...I can do this. I can pin him in the first period. He'll underestimate me because I'm scrawny and he sees me coughing."

That was what went through my head every time I got on the wrestling mat in high school. I knew if I could get my opponent on his back in the first period, I had a shot at a win. Anything past that and I ran out of gas. But winning wasn't always that hard for me...

In elementary school I was one hell of a wrestler. With all the physical and personal problems I had complicating my life, I needed an outlet for my anger. I had been watching the fake stuff on TV for a while and figured I could pick up the "other kind" of wrestling without any problems, so I asked my mom and dad to sign me up. Turns out I was right. I had built up a lot of upper body strength to compensate for my lack of leg strength and balance from cerebral palsey and spastic diaplagia. I beat people twice my size because I was deceptively strong, extremely quick, and very smart. I was always thinking two or three steps ahead of my opponent and would end my matches before they knew what was going on. In fact, I rarely lost a match and a lot parents would complain to the coach because their kids had to wrestle me. Looking back now, I can see why. All of my anger and frustration came out when I wrestled and that probably scared them a little. What a bunch of babies!

By junior high school, only the best kids continued on in wrestling and improved with practice. I improved as well, but not by the leaps and bounds that other guys did. A lot of it had to do with my Cystic Fibrosis-infected body not maturing as quickly as most of my peers. Then there was the ever present problem that wrestling season was always during the worst time of the year...flu and cold season. CF compromises your immune system which meant all through junior high (and high school for that matter) I got sick frequently and missed practices and meets. On top of all the CF garbage, I developed a mild asthmatic condition. It was kind of embarrassing...then again, so were the spandex singlets we had to wear. No one on the team could understand why I was gone all the time, but I never blamed them for that. Even if I explained CF to them, it's hard to know what it's like unless you live with it.

To be honest, there was a much simpler explanation for my decline. Even though I never stopped busting my ass to get better, I wasn't a superior athlete. When the cream of the crop rose to the top, I stayed at mid-level. Some people are born to be athletes and others find their talents elsewhere. I was an "other" and that was okay. I stuck with it because I loved wrestling and it kept me healthy.

By the time I got to high school, wrestling was a struggle. I did everything I was asked, but it wasn't very pretty. I went to almost every practice, worked out in the weight room three times a week, and attended every meet my body would allow. Winning matches had become a rarity, but I managed to win a few exhibition matches at different meets which made me happy. Plus, my teammates were always there cheering me on which was cool. They knew how hard I was trying and I appreciate that to this day.

There were several guys in my 112 lbs.weight class that were much more talented than I was, so it was going to be near impossible to ever reach my goal of wrestling on junior varsity team. Thank goodness for wrestle-offs which allowed every guy on the team to actively try to win their roster spot. I always went in thinking...just once...maybe I could pull off the upset. The coaches had initially let me compete for a spot, but one day after practice the coaches pulled me aside to talk:

"We think it might be better if you don't wrestle-off for a while because you have missed a lot of practices and we want to do what's best for the team. But we definitely want you there to cheer us on because you are one of the most supportive guys."

I was shocked. On one hand, I understood that the guys that had been there every week deserved their time to shine. It was a very "win, win, win" mentality in high school and, as with any sports team on any level, you want to put your best athletes out there for competition. I was not a superstar athlete, so from that perspective it made sense. On the other hand, I had no control over my health and didn't think that should have been held against me. Plus, it was junior varsity wrestling for goodness sake! The coaches had always been so supportive of me...where did this whole opinion come from? I've never liked being coddled or patronized. Rather than make a big deal out of it, I kept my mouth shut and begrudgingly agreed to not wrestle-off anymore.

For the next two years I was the unofficial rookie trainer of the team and the "wrestling dummy" for the guys that needed a partner. I was pretty good at my new role. I took pride in teaching them the basics. A lot of the new guys thought wrestling was easy. The first time the set their eyes on me they were convinced they could take me down. Always the gentleman, I invited them to "take a shot and see what happens." When the rookies tried to take me down, they always shot for my legs with their heads facing down toward the mat instead of looking at me like they should have been. Instinctively, I'd hop back and slam their face into the mat. One time, I accidentally made a guy's nose bleed for a good hour and he let me know with some very colorful language that he was not happy about it. Settle down rookie...you'll learn.

Another time, I had to wrestle a guy in the 150 lbs weight class because they were short a teammate. Of course, this guy got cocky and started to take it easy on me. That pissed me off, so I decided to embarrass him for taking pity on the "sick kid". Before he knew what I was doing, I scooped him up in a single leg and drove him to the ground. He was humiliated and beat the living crap out of me for the rest of practice, purposely stretching my limbs in a way the human body should not bend. It was worth the pain to teach him this lesson: There is only one way to learn about wrestling...the hard way. If you aren't ready for the mental and physical pain, then you aren't ready to wrestle. The rookies caught on and sooner than later were eventually pinning me and moving on to junior varsity and sometimes the varsity team.

By my senior year of high school, I felt I had earned the right to wrestle-off for a spot on the JV team. I walked into the coach's office and cut right to the chase. "Listen, I've busted my ass for this team and I deserve the opportunity to be something more. Please let me wrestle-off. Why won't you let me to wrestle-off?" The coaches gave me this run around answer that they needed me as a motivator. The last thing they ever said to me was.. "If you aren't happy, then you should go home." That was all I needed to hear. After 11 years of wrestling with all my heart and soul, I just walked away. I'd never quit anything in my entire life, but if all my effort didn't warrant the opportunity to earn a spot on junior varsity team, then it wasn't worth my time and energy. I thanked them for my time on the team, cleaned out my locker, and headed home with my head held high. I was proud of what I had done throughout my wrestling career and if this was the way I needed to end it, with my pride in tact, then so be it.

Life isn't always happy endings. It's about learning from experiences. I am disappointed that I never fulfilled my dream of wrestling on the junior varsity team, but I'm not mad anymore. It was worth every moment. I attribute my phenomenal health for many years to the workout regimen that you're put through. Living with CF is sometimes painful, but I've trudged through with the mental toughness I learned from wrestling. But more importantly, I learned a major lesson about of living a good life. A fulfilled person needs to stand by their principles and believe in themselves. Those are the things worth living for. In the end that made me one hell of a wrestler.

Peaceful and Prideful Things,

Josh

The Voyage To Lambeau Field

This past weekend I was lucky enough to be in attendance for Bert Frave...I mean Brett Favre's return to Lambeau Field to face the Green Bay Packers.

If you are not a sports nut I'll sum it up: Future Hall of Fame quarterback Brett Favre ended up leaving the Packers after a sixteen year career there under...shall we say...extenuating circumstances. Through a series of events, he eventually became the Minnesota Vikings QB. As a result, November 1, 2009 was the date for possibly the most hyped game in recent football history . I had always wanted to go to Lambeau for a Packer/Viking game and had bought my ticket before Brett was even a factor. Now the pieces were in place, the intensity level was off the charts, and I was going to be there!

One of my best buds, Randy, was my co-pilot on this voyage. We have been friends since we were little kids and enjoy being stupid together whenever possible. Since I knew how difficult it was going to be put this trip into words, we took the opportunity to be stupid one more time and create a video blog that would capture how electric the atmosphere truly was.

We interviewed several fans from both sides and everyone we talked to loved the idea of a video blog. Randy and I were pleasantly surprised how willing people were to participate in our buffoonery. I intended to put a longer version with more footage on here, but it was too large of a file and I couldn't upload it. Oh well. The chopped down version is still pretty damn good. We also made it on Fox 9 News, but didn't want to deal with any copyright issues, so I didn't post it on here. ;-( I channeled my inner pro wrestler and said the game was going to be insaaaaaaane!

Before this trip I wasn't completely convinced that we needed a new stadium in Minnesota, but after this weekend I have changed my mind. Outdoor football is awesome and I hope the people of Minnesota can figure out a way to make it happen.

From the stadium tour (which was awesome and a must if you are going to make the trip), to tailgating, to the actual game itself, it was a once in a lifetime experience. Lots of love to Randy for all the help and being a great friend and also to Dan and Liza and their crew for feeding us and showing us a good time...and then feeding us some more. ;-)

Here is the video blog of our trip. Enjoy it and look forward to the normal format next post. SKOL VIKINGS!

Peaceful and Gridiron Things,

Josh