Diagnosis

On June 9^th, 2016 I received the following from my friend, Desiree, about her baby daughter, Daphne:

All-

I have been a little silent this week not knowing how to process the news we received last Friday from Daphne’s genetic test.  Daphne was diagnosed with Leukoencephalopathy with thalamus and brain stem involvement with high lactate.  This is a rare mitochondrial disease.  The chances of her inheriting two of the EARS2 mutated genes (the cause) from my husband and I (both recessive carriers and we did not know it) is around 1 in 4.3 million.  In 2014 when it was discovered there were only 12 cases in the world.  Unlike other mito diseases that have been researched for years there is not a lot of research on this type. Just like other mito diseases there is no cure, just treated with vitamins, supplements and co-factors. They do know there are two phenotypes: mild and severe.  All cases to date, even with overlapping similarities have been very different.  I do not know much more at this point.  This will likely be my last update; we have a blog where you can follow along if you want at https://www.lifeisradd.com/. 

Thank you for all your support!

Desiree

My response to Desiree was:

Desiree,

I certainly don’t know God’s plans, and I can’t fathom why your beautiful baby daughter would come into this world carrying such an incomprehensible burden.

What I do know is that each child is unique. So much so that we routinely tell children that they are “one in a million”. In Daphne’s case, she is easily “one in 4.3 million”! There is no way for me to predict them, but I’m sure that God has bestowed some really special gifts on Daphne that you are certain to discover as she grows.

I’m not a medical researcher, so there is little I can do for Daphne or your family, except pray. So…

• I pray for declining lactate levels in Daphne’s brain.
• I pray for medical research and cures for LTBL advance rapidly.
• I pray that you find strength and comfort in friends and community.
• I pray that you delight in Daphne, and she in you.
• I pray that Daphne will help your family grow closer and stronger, together.
• I pray that Daphne will cause Rich and Adelaide to astound you.
• I pray that Daphne will cause you to astound yourself.
• I pray that as she grows, Daphne will open your eyes to God’s grace and miracles.
• I pray that God strengthens and keeps you, Rich, Adelaide, and especially… Daphne!

Your brother in Christ,

Dave

These were the prayer prompters that I was thinking of as I responded to Desiree. You may have better ones. Either way, perhaps you can add little Daphne and her family to your prayers. I’ve decided to follow Desiree’s blog, so every time she posts something I’ll get an email, and be reminded to pray for Daphne. If you’d like to keep little Daphne in your prayers and you’d like to follow Desiree’s blog, too, click on the link in Desiree’s email (above), and then use the FOLLOW icon in the lower right of her blog.

Your brother in Christ,

Dave

Eulogy for Stephen Raymond Hoag

On June 1^st, 2016 I gave the eulogy at my father’s memorial service. Certain immediate family members demanded that I leave out references to what my dad worked on while employed at Lockheed. After much argument, I finally edited what I had written to remove the “offending” references. Later that day, while watching a video picture collage at the reception, I discovered that they had included pictures of the SR-71, along with a shot of Dad in the cockpit of an SR-71. With that obvious reversal of their purported beliefs, I’m publishing the unabridged eulogy, below:




Eulogy for Stephen Raymond Hoag

June 29th, 1929  -  May 19th, 2016

By David Alan Hoag

For those of you who don't know me, I’m David Hoag, the eldest of Ray and Barbara Hoag’s 4 children. On behalf of our family, I’d like to thank all of you for coming today to celebrate Ray’s life. I’m honored to be able to briefly share with you some of my recollections of his life.

Born Stephen Raymond Hoag on June 29th, 1929 in Oakland, California, he was known to all as “Ray”. Ray was in the ROTC while at Berkley, served in the Navy, and ended up missing both the WWII and Korean conflicts. Ray’s high-school sweetheart was the girl across the street, whom he married in 1949. This August would have marked the 67th wedding anniversary for Ray and Barbara. My father was completely devoted to my mother… and she to him.

Ray was fiercely proud of his Irish heritage, and loved all things Irish. He could proudly trace his Irish genealogy, was in contact with relatives in the old country, and even went so far as to provide a headstone in Ireland when he discovered that the grave of his grandparents was unmarked.

Ray also worked tirelessly behind the scenes for the church, was a past president of The Holy Name Society, and had the honor of being the very first lay lector at his home church of St. Pius V in Buena Park. He was a member of the Knights of Columbus, where he rose to the rank of 4th Degree Knight while he served the organization with selflessness and quiet dedication.

Dad liked to tell stories, share jokes, and loved puns to the point of elevating them to a high art form, like:

“When cannibals ate a missionary, they got a taste of religion.”

This seemingly dominant genetic defect is something I have been accused of inheriting. He was quick to fire up his pipe, and even quicker to offer you advice, a statistic, or… quite often… both. He was surprisingly well versed in the poetry of Alexander Pope, and usually quoted the following to me when I argued with him:

We think our fathers fools, so wise we grow.

Our wiser sons, no doubt will think us so.

It took quite a bit of maturing for me to appreciate the irony of his favorite poem.

From early on, Ray had a passion for electronics, electrical circuit design, and aircraft. He eventually followed his passions into a lifelong career with Lockheed, supporting the F-104 Starfighter for 6 years in Germany, and ultimately working in Lockheed’s famed “Skunk Works” on the super-secret SR-71 spy plane until he retired after more than 30 years with Lockheed. Ray never shared with his family exactly what he did at Lockheed or exactly what he was ever working on, answering all of our questions with: “I could tell you, but then I’d have to kill you.”

It turned out that dad’s retirement from Lockheed coincided with the government’s decision to retire the SR-71, so his retirement party was a huge who’s who of politicians, generals, famous test pilots, Lockheed top management, and other luminaries. Eventually, the stories began to flow as freely as the alcohol and I heard not only about what a great engineer, and even greater guy my dad was, and how everyone held him in highest esteem, but also dozens of top-secret stories about his job and the SR-71. I could tell you a few of the juicier tidbits, but then I’d have to… well, you know…

Dad taught me how to use, AND… how to care for tools. He taught me to use the right tool for the job. He taught me how to fix things, rather than just toss them aside. He taught me how to “measure twice, and cut once”, and he taught me to plan to succeed while being prepared for the unexpected. He taught me that whether digging a ditch, or designing an aircraft… that you always gave your best effort, and that neither task was to be held in any higher esteem than the other. He modeled a way of approaching life that that I came to know as “common sense”, and constantly drummed into me the fact that common sense was the most underutilized, and most UNCOMMON thing on the planet.

As you are no doubt aware, Ray had been diagnosed with Parkinson’s disease in his late fifties. Studies show that life expectancy for patients with Parkinson's disease is poor, with barely one-third of patients surviving six years. But with an amazing force of will and a positive attitude, Ray struggled against, and continually adjusted to, the debilitating disease for 27 years.

Over the last 3 decades, one of Dad’s greatest joys was to experience the visits of his grandchildren and greatgrandchildren. When they were around, even the toughest physical challenges seemed to melt away in the presence of their innocent laughter and enthusiasm. They seemed to connect him to memories of better, earlier years and good times. If you knew Ray, it’s those memories of better, earlier times that ultimately define his life, as well as yours.

Now Ray is finally and forever at peace in God’s merciful… and loving hands. 

Rest in peace, Dad.

Your brother in Christ,

Dave