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Lou Gehrig’s disease

Photo from Christine Pendergast

Reviewed by Jeffrey Sanzel

The name of the book is Blink Spoken Here. It is written by Dr. Christopher Pendergast and Christine Pendergast of Miller Place.

That’s really all you need to know.

That, and please buy the book.

Blink Spoke Here. Dr. Christopher Pendergast and Christine Pendergast.  Buy the book.

You don’t need to finish reading this review.

You just need to buy the book.

Blink Spoke Here. Dr. Christopher Pendergast and Christine Pendergast.  Please buy the book. Now.

For those who want to know more …

It is easy to say that this is an important book — because it is. It is about exceptional bravery in the face of unfathomable adversity.  It is about a man who has defied the odds and lived with one of the single most difficult and devastating diseases:  ALS (amyotrophic lateral sclerosis), also known as Lou Gehrig’s disease. Emphasis on lived with. It is told in his words, with the assistance of his wife.

Authors Dr. Christopher Pendergast and Christine Pendergast

The title refers to how he wrote the book, with an eye-controlled device, as he does not have the use of his hands or his voice. His journey began with the diagnosis in 1993 and continues to this very day — to the very moment that you are reading this sentence. The average lifespan with ALS is two to five years; Dr. Pendergast has survived for twenty-seven. There is no medical answer as to why. But perhaps the Universe has chosen him for bigger reasons. Two of them? First: his bringing awareness to this monstrous affliction through his inspirational Ride For Life. Second: He has written this book.

In 1993, Dr. Pendergast had been a teacher for twenty-three years, married to his childhood sweetheart, Christine.  At the time of his diagnosis, he was in the Northport school district, and he continued to teach in the classroom for as long as possible. When that was no longer an option, he continued as a teacher for the world. Blink Spoken Here is a portrait of a teacher in the best sense of the word.  His passion to impart knowledge has infused his entire life.

Beginning with a description of the disease’s arc, he brings us into his world:

“It was not a dramatic event like a building collapse but a more steady deterioration similar to a bridge failure. I was imploding. In 1993, my physical presence began shrinking before my very eyes. My contact with the world was severing, one function at a time.  Angry, scared and saddened I was like a stubborn mule fighting with tenacity for each inch I surrendered. First it was dressing, followed by grooming, driving, toileting, walking, feeding, and breathing. Now I cling to my last vestiges of talking. It forced me retreat towards within. The exterior husband, father, and friend was left behind.”

Dr. Pendergast is unflinching in his brutal honesty about the pains and the challenges. He shares some of the darkest moments in his life. But, just as often, he speaks of hope and appreciation and deep faith. Many of the simplest things that we take for granted have been taken from Dr. Pendergast.  And yet, in all of this, he manages to find not just the good in life but the lessons that are offered every day. 

If these are not good enough reasons to read this book (and they should be), it is also a beautiful piece of writing. Dr. Pendergast writes with extraordinary eloquence and sincerity, with humor and insight. His prose is exquisite. He shares anecdotes and parables, free verse and personal accounts. The craft is equal to the art and both are worthy of the humanity that created it.

The memoir is split into two sections.  The first focuses on his coming to terms with the disease and its myriad challenges. (The first half even concludes with a wicked send-up of Dr. Seuss.)

The second half of the book focuses on the Ride for Life, which began in 1998 as the Ride to Congress. It follows his goals of bringing national awareness to ALS as well as an increase in services, knowledge, and fundraising. Taking his cue from the activism of the late 1960’s and early 1970’s, he finds his inspiration:

“For me, the remarkable results of these movements underscored the power of choosing to make a difference. The activists of those movements did more than complain about these wrongs; rather they opted to fight for change.  This activism formed a model in my subconscious. I followed this model 40 years later.” 

The initial support of his home school in Northport proves that it takes a village — or at least a district. Over the years, the Ride has evolved and has focused its activities in New York and Long Island.

From the “weight of secrecy” to his global advocacy, this is an odyssey that is both far-reaching and personal. His love for his wife and family and for his community comes through at every turn. This is a man who does not curse the darkness but moves towards the light. 

“Life is too short to spend wishing things were not so. Things are what they are. Some occurrences are not our choice. However, we do choose how to respond. We decide how to live the life we get.”      

There are too many incredible moments to enumerate. Even the description of the challenge of opening an envelope is a revelation. There is a particularly telling incident with his son and church. It is a lesson in forgiveness and perspective, and its reverberations reflect his own continuing journey.

The final chapter, entitled “The First Amendment,” is a crushing account of his loss of the ability to speak: “To the educator, the voice is a powerful tool. It commands respect, informs and on occasion, inspires. The voice becomes our signature for the world. Losing it is catastrophic.” 

Dr. Pendergast describes the gradual decline in his vocal power and the various methods of communication. His frustration is honest and palpable just as his deep belief that his and all voices should be heard in one form or another.  He advocates for those who are desperately ill with ALS and that this basic human right should not terminate at the hospital door.

“Speech is freedom. Communication is the connection to the outside world. We all have a right to be heard … I want to be able to speak, even if it is only one blink at a time.” 

This chapter brilliantly closes the book. Because while he may have lost the physical voice, his spiritual voice continues. It is powerful. It commands respect. It informs. And, truly and always, it inspires.

Once again.

Blink Spoken Here. Dr. Christopher Pendergast and Christine Pendergast.

Don’t wait. Please buy this book. Now.

Blink Spoken Here: Tales From a Journey Within (Apprentice House Press) is available at Book Revue in Huntington, Amazon.com and BarnesandNoble.com.

Chris Pendergast celebrates his 70th birthday in April at 89 North Music Venue in Patchogue with family and friends. Photo by Elliot Perry

By Chris Pendergast

On a cold rainy Columbus Day, my life forever changed. Nothing would be the same. The life I knew ended. My wife, children and I embarked on a new road, one less traveled by. That has made all the difference.

During the Columbus Day weekend of 1993, I was diagnosed with amyotrophic lateral sclerosis, also known as ALS or Lou Gehrig’s disease. Most people now know it is an incurable and uniformly fatal disease. Average life expectancy still hovers near the two or three-year mark. Lucky ones can live longer. A rare few survive longer than 10 years.

Remarkably, l am entering my 27th year with my unwanted road companion. I am here today due in large part to my loving wife, Christine, and our wonderful kids, Melissa and Buddy. They keep me well cared for and motivated. They have, literally, gone the extra mile for me. My grandson Patrick is the most effective medicine I take. My son-in-law Rich and step-grandson Ryan round out my terrific family.

I am blessed with caring, competent and compassionate caregivers, Tiana, Amanda, Marquita and Lena. They are in the trenches every day and night battling right beside and fighting the good fight with me. I am fortunate to have an outstanding mental health therapist, Dr. Melnekoff. He has kept me balanced, focused and headed in the right direction. My life is extended through the phenomenal care provided by my incredible respiratory therapist, Monty Rivera from Millennium. I receive expert medical treatment at the ALSA Clinic, which bears my name at Stony Brook University Hospital. Marvelous friends surround me and help with so many things to make our life easier. My ALS Ride for Life charity has countless volunteers led by staffers Maureen and Marilyn. Because of all their collective work, we have raised more than $8.5 million.

Along the road with ALS, I witnessed amazing things and met incredible people. I was able to participate in wonderful experiences which otherwise would not have happened.

Do I wish I never got ALS? Honestly, I am not sure. I am certain that at some point in almost everyone’s life, a fatal disease will arise. The timing and circumstances vary but the ending does not. I have no corner on the market — everyone will get a turn. My turn came earlier than expected and became more public.

It has been a great life so far. I wonder what new adventures and joys lie around the bend. Besides the joys to come, there are also the challenges and corresponding sorrows. However, I have faith and optimism that everyone one in my life will collectively help me triumph.

To paraphrase Gehrig’s farewell speech at Yankee Stadium, as he did, I also say, “Look at these grand people. Which of you wouldn’t consider it the highlight of his life just to associate with them for even one day? Sure, I’m lucky. Today I consider myself the luckiest man on the face of this earth.”

Chris Pendergast, 70, is a former Northport teacher, who lives in Miller Place. He is the founder of ALS Ride for Life, the Stony Brook University-based nonprofit dedicated to raising awareness about ALS, funding research and providing patient services.

Ride For Life presents CSHL with $300,000 for ALS research: from left, CSHL Director of Annual Giving and Donor Relations Karen Orzel, CSHL Assistant Professor Molly Hammell, Ride for Life Founder Chris Pendergast, Stony Brook Associate Professor Josh Dubnau and Ride for Life board member Frank Verdone. Photo by Jessa Giordano, Cold Spring Harbor Laboratory

By Daniel Dunaief

The past can come back to haunt us, even in the world of genetics. Over the course of millions of years, plants and animals have battled against viruses, some of which inserted their genes into the host. Through those genetic struggles, explained Molly Hammell, an assistant professor at Cold Spring Harbor Laboratory, cells develop “elaborate ways to fight back,” even as they continue to make copies of these pieces of DNA.

Sometimes, when our defenses break down, these retrotransposons, or jumping genes, can become active again. Indeed, that appears to be the case in a fly model of amyotrophic lateral sclerosis, also known as ALS or Lou Gehrig’s disease.

Working on a fruit fly model of ALS, Joshua Dubnau, an associate professor at Stony Brook University, Lisa Krug, who earned her doctorate at Cold Spring Harbor Laboratory and is now working at Kallyope in New York, and Hammell showed that these ancient genetic invaders play an important role in the disease amid activation by a protein often linked to ALS called TDP-43.

A recent study, published in PLOS Genetics, “really proves that retroviral reactivation (as a consequence of TDP-activity) is … central to either causing or accelerating neuronal cell death when TDP-43 inclusions are present,” explained Hammell in an email. If TDP-43 plays the same role for humans, this would suggest that targeting this protein or the jumping genes, it activates could lead to potential treatment for ALS.

These collaborators showed that an aggregation of this protein turned on jumping genes. These genes can make copies of themselves and insert themselves in other parts of the genetic code. In this case, TDP-43 expression disrupts the normal immune-like system that silences retrotransposons such as gypsy, which is a particular type of jumping gene in the fruit fly.

When gypsy was activated, the fruit fly exhibited many of the features of ALS, including protein pathology, problems with movement, shortened life span and cell death or glia and neurons in the brain. The scientists were also able to turn gypsy off, which improved the health and extended the life span of the fly.

Mimicking this protein results in broad activation of several retrotransposons. If this also occurs in people, the disease may activate a retrotransposon that is the human analog to gypsy, called HERV-K, as well as other retrotransposons. The study also suggests that DNA damage caused by retrotransposons may active a cell suicide mechanism. Finally, this effort showed a means by which the protein disrupts the normal immune surveillance that keeps retrotransposons quiet.

To be sure, Dubnau cautioned that animal models of a disease may not translate when returning to people. Researchers need to look at more patients at all the retrotransposons in the human genome to monitor its prevalence, Dubnau suggested. If the link between retrotransposon activation and the development of ALS is as evident in humans as it is in the fruit fly, scientists may take an approach similar to that which they took to battle the human immuno-deficiency virus, or HIV. Retrotransposons have an RNA genome that needs to be copied to DNA. This, Dubnau explained, is the step in the process where researchers attacked the virus.

In a small subset of HIV patients who have motor neuron symptoms that are similar to ALS, Avi Nath, a senior investigator at the National Institutes of Health discovered that treating patients with the typical HIV medication cocktail helped relieve their ALS symptoms as well.

“What is not known is whether, for some reason, this subset of patients had an ALS syndrome caused by HIV or they were curing them” by treating HIV, Dubnau said. Nath is currently involved in one of two clinical trials to see if HIV medications help ALS patients. The next step for Dubnau and Hammell is to screen the tissue of numerous ALS patients after their death to see if their retrotransposons were elevated.

In addition to NIH funding, the scientists received financial support from Ride for Life, which is a not-for-profit organization started in 1997 that raises funds for research to find a cure for ALS, supports patients and their families through patient services and raises awareness of ALS. Every May, Ride for Life conducts a 12-day, 100-mile patient wheelchair ride across Long Island. Dubnau and Hammell, who received a $300,000 grant from Ride for Life in 2015, said they have been inspired by Ride for Life founder Chris Pendergast.

Meeting Pendergast “has had a big impact,” Dubnau said. “He’s a force of nature. He’s an incredibly strong and intelligent person.” Receiving funds from Ride for Life created a sense of personal obligation to Pendergast and many other people who “had raised that money through sweat and effort.”

Without funding from the Ride for Life Foundation, “We would not have the resources to obtain these samples and do the sequencing experiments necessary to prove that this is a clinically relevant phenomenon in a large number of ALS patients,” Hammell said.

Through an email, Pendergast explained that Ride for Life chose to fund the work by Dubnau and Hammell because the research met several criteria, including that it might lead to new strategies to treat ALS and the research was on Long Island, which is a “powerful affirmation for our generous donors.”

Pendergast emphasized the importance of funding basic ALS research. “We need to know why it develops, how it progresses [and] how it can be diagnosed and monitored,” he urged.

A resident of Huntington, Dubnau and his wife Nicole Maher, who works at the Nature Conservancy as a climate scientist, have a nine-year-old daughter, Caitlin. Reflecting both of her parents’ professional interests, Caitlin is going to a statewide science fair, where she is presenting her work on how temperature affects the life span of insects.

As for his research, Dubnau hopes a further exploration of TDP-43 might reveal an important step in the progression of ALS. He hopes this discovery may suggest a strategy researchers and clinicians can take that might “stop the cascade of events” in ALS.