When you say I breathe, the “I” is extra.
I remember the day my dad told me about familial IPF.
I made the walk up a steep dirt road from a Honduran Internet café back up to my concrete block house. I sat on a makeshift bench I had made just for this purpose, to stare out over Trujillo Bay to watch the sunset. I was with my wife Anne and another Peace Corps volunteer Wade.
Recounting to Anne and Wade the phone conversation I just had with my dad, I started to realize just how little I really understood. The basics were that my dad and his brother, uncle Larry, were diagnosed with idiopathic pulmonary fibrosis (IPF) and there was reason to believe this was a genetic disorder.
The news triggered memories of my grandfather (Ojichima). He died at the early age of 56. They said he died of emphysema but now its clear he was misdiagnosed and that this disease was attacking and killing my family.
At the time, there was no way to know whether this would affect me. So, I went on with my life. Anne and I had three beautiful kids, added two dogs to the family, then 2014 came.
2014 marked 10 years from that day in Honduras. My father would pass away in May. My uncle Larry had passed away three years earlier. Both received lung transplants which is the only way to extend life against the battle with IPF. You will never win, you just get a little more time. The statistical norm offers 3-5 years. Dad died the same year of his operation, Larry 3 years post op.
In those 10 years, many things happened. Advances were made by teams in Johns Hopkins University and UT Southwestern which identified the gene that was causing this. You can now take a test to see if you have the telomere syndrome (genetic mutation) which gives rise to familial IPF. Some advances were made with experimental drugs that have shown to extend life for those diagnosed with IPF. My dad was lucky enough to be on the test trial for pirfenidone and is the likely reason why he passed away at 65, while Larry passed at 58.
On a more personal note, over those 10 years, I also saw my dads family, a historically disconnected group, draw closer through newly formed annual reunions. My dad and Larry had a special bond that many brothers share. They were fiercely competitive and held a deep respect for one another. My dad was never an emotional man but, when Larry died, something changed in him. I think this heartbreak is only matched by that of a parent losing a child and when my dad passed, it took only a few months for their mother, my grandmother, to pass. She lost two sons and a husband to this terrible disease.
So, in November 2014, I was in my office, when I received a call from UT Southwestern, to receive the results of the genetic test. I had found out two months earlier that my brother Erik had tested positive for the mutation. After hearing the news, I put down the phone and called Anne, who came up to the office to see me.
We walked outside to a bench and cried.
Anne once beautifully described the moment you experience a true life crisis. She said, it feels like the fabric of life has torn and you don’t know how to put it back together. The effect makes you apprehensive. You are fearful of returning to blind bliss, believing that life may humble you again.
My younger brother Matt would soon feel this in 2018 when he also tested positive for the mutation.
For me, there were obvious echoes of my dad and Larry for Erik, Matt and me. Three brothers with a special bond, facing tough questions on life. Thoughts of the pain for our own mother, for my dear wife Anne. Then the most dreadful…the fact that one of our children may some day have to face this. The statistics look bad.
Moving from crisis to action was the genesis of The Telomere Project. At first, honestly, this was borne from our family story, thinking how we could be proactive about addressing this challenge. But, with time and education, this has grown to something much larger.
As you dig into the root causes of familial IPF, you find yourself at the nexus of leading edge science and medicine, into the world of genes and telomeres, mutations and ageing, and patients and families.
The irony is not lost on me that while I wanted to be a doctor earlier in my life (but changed my concentration after realizing I am embarrassingly squeamish at the sight of blood), life has given me the opportunity to come back and dedicate my energy into healthcare.
I would emphasize the word opportunity because, while knowing that I have a mutation that could lead down the same path of my father, if it weren’t for the research teams at UT Southwestern and Johns Hopkins, I would not have known beforehand. I would still be driving through life, oblivious. Their achievements have been a gift to me and my brother, allowing us to try to do something about telomere syndromes that lead to IPF.
Research and medical breakthroughs happen in step functions, they are not linear. That means that the right investment of time and resources can make a change and can accelerate breakthroughs.
When “it” is raining, what is “it”? There is just raining.
Thich Nhat Han
The greatest gift was the information that there was a mutation passed down in this family. Physically this may mean that the lungs will scar and oxygen will become more and more precious. Although I have not been diagnosed with the disease, I have leapfrogged in my appreciation for my precious breath. Every breath is a moment. And every moment is a guru. At times I am sad for all the breaths, all the moments I had not equally experienced as precious. At times, this journey has already been filled with dis-ease as I grapple with the imagined and perceived future loss and losses. I am grabbed, you might say, with a feeling of suffering of what has not even happened yet. The very definition of insanity.
I hope to better understand that this time lost suffering in this way is the greatest loss. I am happy when I choose to experience each breath as a beautiful moment.
Right now, I really enjoy walking. As I walk in the forest, I look up at the magnificent Live Oak tress that we are blessed with here in the Trinity Valley Forest covering Dallas Fort Worth. As I look up, the magnificent trees look like giant lungs, swaying and breathing, with their outstretched and networked branches in the sky. This reminds of the potential continuity and connectivity in our world. I also experience a true connection with divinity on these walks. Together the trees seem to connect with each other giving the overall appearance of a forest…united. And yet, one can still make out the outlines of each individual tree. Much like each of us with our own unique story, swaying and breathing, in our own unique way…together we are a forest. We are rooted in a common ground, we stand strong and tall as our trunks demonstrate a life of independent growth. There will be differing examples of bark, with some showing tougher skin than others revealing how the outside world has encrusted us.
And still, the branches on top outstretch, reaching to one another. Regardless of the peculiar and original paths, the fruits and leaves of our story weave a tapestry of life that provides a canopy of cover for others walking within this forest of life.
I am hesitant to detail the details of my relationship with IPF. I know all of you have seen this movie. The shock of the news, the setting in of the news, the fight, the struggle, the new baselines, the mind games. Suffice to say, I am invigorated and inspired by my brother and happily join in him at The Telomere Project in our fight to understand this condition. The journey is driven with a passion to find treatment for the pathology, and treatment for the suffering, physical and mental, inherent with Telomeric diseases.
I stand strong with my wife of 20 years, Amy. We have actually been dating for 27 since high school! I also stand strong with my family, my siblings and their families, my mother, my aunts and uncles, and all others out there who are touched by this condition.
I stand for my children and my children’s children, and all those who have children affected. Nothing is impossible. No “thing” is impossible. And this “thing” has met its match.