TRIBUTE TO DAD
Craigg Cordell was my father and my hero. He was also a Fabrys sufferer. By “sufferer” I mean that he lacked the enzyme characteristic of the disease, not that he experienced pain or ever succumbed to his condition. Rather, he lived life to the fullest and was an inspiration to all who knew him. That is the Craigg Cordell I pay tribute to.
Although he had “the rash,” dad was not diagnosed with Fabry Disease until his early twenties, a couple of years before I was born. Throughout my life, I remember him as a smart, handsome, funny, hard-working and athletic man. He was totally devoted to his family and was a great teacher to his daughters, grandchildren, co-workers, and anyone who had the privilege to talk with him. He always had a wise proverb, old-saying, or appropriate one-liner to fit any situation and to leave the recipient something to ponder and benefit from. These tidbits of inspiration were of great comfort to him, as well, during his last few years of life, when periodic episodes of self-pity would invade his otherwise optimistic outlook.
To look at my dad from the first time I can remember as a child until he was in his early 50’s, you would never have known there was anything in his body capable of bringing him down. He was so handsome and the funniest man I have known. He used to make me and my sister laugh hysterically by wrapping up in a towel after a shower and turning his lean body into Mr. Universe poses. Although he was never a great athlete, he tried every sport and loved the game. I cheered him on in many volleyball tournaments, tennis tournaments, softball games, and listened to him describe his day at the golf course, his favorite pastime. He also coached me and my sister in basketball from the time we were big enough to dribble until we were teenagers. Even after his stroke in 1993 at the age of 52, he was determined to be the master of his body and to return to his favorite activities. He told my mother he would walk, unassisted, out of the rehab hospital, and he did just that one month later. He continued to work until early retirement in 1996 and played golf as long as his deteriorating lungs would allow. Even with his slightly disabled left arm, due to the stroke, he played in many super-ball golf tournaments, often being the member of the group to offer the needed putt. Once he broke a finger on his left hand due to the awkward way he now gripped the club, but continued to play because he couldn’t feel that anything had happened. On our last family beach trip in 1996, he got up the nerve to do something he had always wanted to do by allowing us to pull him behind our boat in an inflated inner-tube (he really wanted to jet-ski, but this was as close as he could get at that time). I’ll always treasure the video-tape of that ride, seeing his great big smile as he bounced over the waves. As I just wrote that sentence, I realized that it kind of described his life – he “bounced over” the waves in his life with a smile, never letting them take him down. Wow!
The biggest “waves” in his life were to come within the last 3 years. In 1997 he had to begin peritoneal dialysis at home. While it was inconvenient having to take treatments 4-5 times a day, dad still tried to squeeze in what he could between them. About the same time that he needed to go on dialysis, it was obvious that Fabrys was affecting many other systems of his body as well, but never his mind or spirit. Due to decreased lung capacity and slowly declining blood pressure, his stamina and strength required him to modify his activities to a less physical nature, going to movies, going out to eat, spending quality time with the grandchildren, and reading the Bible. To illustrate the kind of family man that he was and how his priorities were so unselfish, it was not missing a golf tournament, having to retire early on disability, or being confined to his room hooked to a dialysis shunt that dampened his spirit – even when he could no longer make it down the hallway to the kitchen for meals or to the bathroom for bodily needs, his biggest disappointment came when he was too weak to color with his 5 year old granddaughter. What a man!
Again, while Fabrys was gaining control of his body, his mind and will-power were sharper than ever. His main focus and passion during this period in his life was trying to get help for his condition. Dad knew that an enzyme replacement therapy was in the works and had, himself, taken place in the studies by allowing the participating hospitals to run tests on and evaluate him to collect needed data. However, because he was in the “end-stages” of the disease, he was not considered a “safe” candidate for the clinical trials. As you can imagine, the inequity of this reasoning was troublesome for dad and the rest of our family. In our opinion, his role as a “guinea pig” played a part in the development of the enzyme replacement, and he, therefore, should benefit from it. And he pulled out all the stops! From my review of his meticulous records and filing system, he began writing his doctors, senators, congressmen, and drug company presidents in 1997, urging them to include him in the clinical trials of the new “miracle” enzyme and often educating them, through copies of Federal regulations, on the “compassionate dose” protocol allowed by the FDA in critical situations. Although we were led to believe these players were doing all they could to help dad, it was obvious they were reluctant to offer individual help, waiting, instead, on the comfort of FDA approval.
This past summer when dad’s blood pressure dropped into the 40’s over 20’s and his peritoneal dialysis was no longer effective at draining excess fluid (necessitating periodic bouts of hemodialysis – which he hated), we knew it was time to step up the letter writing campaign. This time we wrote to Donna Shalala at the Dept. of Health and Human Services, to our North Carolina senator, and to the presidents of both drug companies manufacturing the enzyme. We outlined how critical dad’s condition was and how we knew that there was a procedure in place which allowed for “compassionate dose” treatment of an unapproved drug. On August 9, 2000, dad got the news that one of the drug companies had agreed to supply the enzyme for compassionate dose treatment if the FDA would approve it for his individual use. Dad called me that day crying, he was so excited and happy – his hard work and prayers had seemingly been answered. The next few weeks were filled with elation and frustration. For an “emergency” situation, the process was filled with hurdles and setbacks. However, I realize that these steps are necessary to ensure that all players and the patient are protected and receive the best possible care, and I thank God for everyone involved in helping my dad. As we called everyone that played a role in this process everyday to check the status of the approval or the preparations for the infusion, it was dad’s faith and will to live that kept his spirits high and his weakened body holding on “just one more week,” as we kept hearing. Dad had a sign, written in his handwriting, on the back of his bedroom door that read, “One Day at a Time (Try to Remember).” These were certainly words to live by during this waiting period!
On September 18, 2000, dad was the happiest man and most famous patient at the hospital. He had been told the week before that this Monday morning would be the day. In anticipation thereof, he showered the weekend before (or should I say he laid on a bench in the shower and my mother scrubbed him). The shunt in his stomach for peritoneal dialysis and the one in his neck for hemodialysis had made bathing almost impossible for many months before, but because he didn’t sweat, he also didn’t smell. This day was special and he was going to be ready! After his “shower,” I leaned him back in his recliner and shaved him – how happy and handsome he looked, just as I remembered as a child. When we arrived at the hospital, he got the royal treatment from the doctors and staff. Everyone rallied around him taking his picture, taking measurements of his bloated stomach and swollen legs, and setting up a special database of his statistics so that his progress from the bi-monthly enzyme infusions could be monitored. When he was finally hooked up to the IV, it was like gold running through his veins. The day he had prayed for had arrived and he was victorious in his unwavering efforts to get the treatment he knew he needed and deserved. From our understanding, dad was the first and only patient to receive the enzyme infusion outside of the clinical trials. Bless his heart, dad was confident by the end of the day that he would have an appetite and need to urinate (he still had some kidney function left). He was eager for any sign of improvement.
Unfortunately, the enzyme infusion never had time to make any difference in dad’s life. We were originally told that after the 4 hour infusion and a brief period of observation thereafter, dad could go home until his next infusion two weeks later. However, because he was “fluid overloaded” when he entered the hospital that morning, his nephrologist felt it would be best to start him on a round of hemodialysis for a couple of days to improve his breathing. As his wheezing was extremely heavy, it was agreed that he would stay at the hospital a while longer to remove the excess fluid from around his heart and lungs. During this extended stay, dad developed an infection, common to dialysis patients, called peritonitis. Because all of his systems were already so compromised from Fabrys, his body never stood a chance against the infection and he died on September 24, surrounded by his family. We were all able to tell him how much we loved him and how proud we were of him.
While he did suffer some abdominal pain from the infection, he left otherwise peacefully in the evening, his blood pressure gradually dropping until it was nonexistent. Based on the damage done to his body from 59 years of the Fabry buildup, it was amazing he lived as long as he did. I am convinced that dad’s faith in God and justice kept him alive until his prayer of treatment was answered. I know dad is with God now and that he is happy and comfortable. I learned from my mother a couple of days after dad died that on the morning of the 24th dad was watching a baseball game (the T.V. was not on). He was in his hospital bed cheering for a baseball team and pointing out famous baseball players who were already deceased. He even reached overhead with both arms to catch a fly ball. My dad had not lifted his left arm above his head since his stroke in 1993. This occurred minutes before he “died” the first time (dad was resuscitated and lived until later in the evening). This taste of heaven revealed God’s mercy in releasing my dad from his tired, weakened body and restoring in his soul the energy and innocence of youth. What a victory!
Although he never personally benefited from the enzyme infusion, I know that dad felt victorious in his fight to obtain it and in the doors his fight may have opened for others. That he lived to see the day when the enzyme was ready for individual patient use was a true blessing in that he could leave this world knowing that others, including close family members, may not have to experience the symptoms he had to endure. The connection I will always feel to my father is even stronger now that I have learned that my 5 year old son also has Fabry Disease. Because of dad’s participation in the development of the enzyme treatment and his fight to help get it approved, I know that my son will live a long, symptom-free life. My dad was truly a hero! Thanks dad for all you’ve done, for all you’ve given to so many people, and for being a wonderful father, role model and inspiration for others.
Written by Tracy Myatt