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Million Dollar Donation Funds New ALS Research

Thanks to the generosity of Theresa Patnode Santmann of Babylon, Winthrop-University Hospital has launched an innovative research program aimed at the development of therapies to halt the progression of Amyotrophic Lateral Sclerosis (ALS). Also known as Lou Gehrig’s disease, so named for the New York Yankees baseball legend who was diagnosed with the condition in 1939, ALS is a progressive neurological disease that attacks nerve cells responsible for controlling voluntary muscle movement.

In ALS, these motor neurons, which carry messages from the brain to the muscles in the hands, feet, arms, legs and more, deteriorate and die. As they do, so too do the muscles with which they communicate, which atrophy from lack of movement.


(L.-R.) Louis Ragolia, PhD, Director of Applied Research at Winthrop; Theresa Patnode Santmann; and Thomas M. Jeitner, PhD, Lead Research Scientist.
With an average age of onset between 40 and 60 years old, as many as 20,000 to 30,000 Americans have ALS, and more than 5,000 people in the United States are newly diagnosed with the disease each year.

While Lou Gehrig was diagnosed at the age of 36 and lived only two years following diagnosis, Santmann’s husband John was diagnosed with ALS when he was just 30 and he lived a remarkable 18 years longer.

John Santmann was studying for his PhD in Clinical Psychology at the time; Theresa was pregnant with their second child. As the ALS stole John’s ability to function, Theresa became sole provider – and caretaker – for the whole family.

Life with ALS is not easy. Early numbness in the hands and feet progresses – often very rapidly – to loss of strength and the inability to move the arms, legs, and other parts of the body. When the muscles in the diaphragm and chest become affected, patients lose the ability to breathe independently. Most people with ALS die from respiratory failure, usually within three to five years from the onset of symptoms, and only about 10 percent of ALS patients survive for 10 or more years.

While Theresa’s husband surpassed all expectations with his survival of almost two decades after diagnosis, the disease places extraordinary stress on the patient and family. In order to continue to care for her husband, who soon came to require full-time attention, Theresa did something that many thought was impossible. She secured the funding, property and permits to create an adult home where she could take care of her husband, her family and others while making a living in the process. Ambitious and resourceful, Theresa has since earned a degree as a Registered Nurse and developed her home-grown family business into a home-like 69-bed nursing and rehabilitation facility and opened a second location – a 160-bed nursing home, both in Suffolk County.

Recently, Theresa made the decision to begin funding ALS research. She explored many existing ALS research programs but when she heard about the proposed research initiative at Winthrop she was most impressed. And when Louis Ragolia, PhD, Director of Applied Research at Winthrop, heard that Santmann planned to make a significant contribution to establish an ALS research program at the Hospital, he was thrilled.

“Most research funding for ALS – like many other diseases – goes to established research programs that follow a popular line of thinking,” said Dr. Ragolia recently. “Here at Winthrop, we’ve wanted to take a new approach, a fresh look, at how we might best help patients with this debilitating disease.”

And the private funding from Theresa Santmann provided just the support they needed. Thanks to her generous donation and her commitment to future funding, Winthrop launched a national search for a scientist with the necessary experience and background – but an innovative new approach – to the problem of nerve degeneration to lead the new ALS research initiative at Winthrop.

Lead Research Scientist Thomas M. Jeitner, PhD, was recruited to Winthrop from the Medical College of Wisconsin in Milwaukee, where he had conducted extensive studies on the role of enzymes called transglutaminases in neurodegeneration.

“I came to Winthrop because here I was offered the unique opportunity to investigate ALS with a new approach informed by my 15 years of research on nerve death. Nerves die by similar mechanisms, which have been extensively studied in Huntington’s disease and Parkinson’s disease, but some of these mechanisms have not yet been studied in ALS. We plan to study them here at Winthrop, essentially ‘piggy backing’ on the very exciting developments in the treatment of these other diseases,” said Dr. Jeitner. “The beauty of these studies is that we can build on existing information to develop novel therapeutic strategies for ALS.”

Dr. Jeitner’s experience and research focus are providing a fresh and insightful approach to the problem of ALS and are an excellent complement to the ongoing research of Dr. Ragolia, who has been active in molecular biology and biochemistry research for more than 15 years.

Dr. Jeitner’s research to date has focused in part on a type of abnormal “clotting reaction” that sometimes takes place in motor neurons, which results in the formation of tangled protein fibers that interfere with the nerve cells’ function and viability.

Dr. Ragolia has studied, among other things, a unique form of a naturally occurring enzyme that is found in the cerebrospinal fluid of ALS patients.

Together, Dr. Jeitner and Dr. Ragolia are assessing the characteristics of and changes in nerve cells in ALS – and potential ways of halting the deterioration of these cells.

These studies will range from fundamental investigations of biochemical events that cause ALS to the manifestation of these changes in cells and tissues. In addition, the researchers will be testing various pharmaceuticals as potential treatments for ALS.

There are currently no drugs available to effectively treat this devastating disease. Dr. Jeitner and Dr. Ragolia will test medications that have already been approved by the FDA for other purposes – and which hold promise for treatment of ALS – in mice that have a mouse version of ALS. Once the scientists achieve a successful model of ALS treatment in mice, human trials in ALS patients will be the next step. The researchers are hopeful that this could occur within the next few years.

“I looked at a number of ALS research programs,” said Theresa Santmann recently. “But when I heard about the innovative and very promising approach being undertaken at Winthrop I was convinced that this was the research program I wanted to support.”

“This private grant has provided a wonderful opportunity for us to secure state-of-the-art equipment to tackle the problem of ALS in a truly unique and efficacious manner,” said Dr.

Jeitner. “We realize that patients with ALS and their families are desperately seeking relief from this disease, and we are extremely motivated by that.”

“We are deeply committed to the development of new and effective therapies for ALS,” continued Dr.

Ragolia, “and we feel strongly that between the outstanding funding provided by Theresa Santmann and the staunch support of Winthrop’s administration, we are well-positioned to move forward with this groundbreaking research initiative.”
Vol. 19, No. 3
Fall 2009

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