Tag Archives: Michael Rivner

Challenge, walk continue for ALS funding

A year after the famous ice bucket challenge, The Augusta Chronicle talks to Dr. Michael Rivner and one of his patients about ALS care and research and the need to keep funding these efforts at Georgia Regents University.

Read: Challenge, walk continue for ALS funding
The Beat Feet for ALS Walk is scheduled for 8 a.m. Saturday, Sept. 26 at Augusta’s Riverwalk. For more information, visit walk.alsgru.com

 

 

Ice buckets are empty, but ALS coffers still need filling

Patients, families, employees and friends will Beat Feet for ALS at 8 a.m. Saturday, Sept. 26, at Augusta’s Riverwalk in an effort to raise money for the GRHealth ALS Clinic.

This annual fundraising walk posted a record year in 2014, pulling in almost $145,000 in donations, perhaps driven in part by the popularity and timing of the ice bucket challenge – a unique dare that several Georgia Regents University leaders participated in to raise financial support for ALS.

But much more funding is needed, says ALS Clinic Director Dr. Michael H. Rivner, in order to explore better treatments and improve the quality of life for patients with this debilitating disease that kills most patients within two to five years.

“With ALS, the muscles start to deteriorate rapidly until you are essentially trapped inside your own body, and there is no cure,” said Rivner, Charbonnier Professor of Neurology at GRU’s Medical College of Georgia. “There’s no way to sugarcoat it; ALS is a death sentence.”

But effects of the disease vary, and many people can live with quality in their last years with the help of nationally accredited clinics like the one at Georgia Regents Medical Center.

The clinic, which opened in 2004 through a partnership between the Georgia Regents Neuroscience Center and the ALS Association of Georgia, takes a multidisciplinary and coordinated approach to patient care. Instead of scheduling multiple appointments and trips, patients are able to see neurologists; nurses; physical, occupational and speech therapists; social workers; dietitians; respiratory therapists; and equipment specialists all on the same day. This is especially helpful for ALS patients because of diminishing mobility.

The Georgia Regents ALS team sees patients on the second Friday of each month in Augusta and the fourth Friday of each month at a satellite clinic in Macon. They assess disease progression, functional status, family concerns, and equipment, transportation and referral needs. In addition, family and caregiver training and support are incorporated into the time spent with each patient.

It could cost as much as $250,000 a year to treat just one patient with ALS, so fundraising dollars are financing medical equipment and therapies – often not covered by health insurance – such as wheelchair ramps, home modifications and speech and breathing assistance devices. Funds are also used to purchase gas cards and other items for patients and families who are under financial strains due to ALS.

In addition, donations are supporting several vital research efforts, including a clinical trial of a new ALS drug that follows disease progression and a study on ALS antibodies.

“We were able to fund a pilot project which allowed us to study LRP4 and Agrin antibodies in ALS. Our research thus far has identified these antibodies in around 10 percent of patients with ALS, generating a lot of excitement in the ALS research community,” Rivner said. “If this allows us to pinpoint the cause of ALS in that 10 percent of patients, then perhaps we can identify these patients more quickly and develop better treatments for them.”

Funds raised from the Beat Feet for ALS Walk also support programs administered by the ALS Associations of Georgia and South Carolina and the Muscular Dystrophy Association for patients and families affected by ALS.

To register for the walk or make a donation, visit walk.ALSGRU.com or contact Brandy Quarles at bquarles@gru.edu or 706-721-2681. You can also make a donation directly to the Georgia Regents ALS Clinic on the website or make a check payable to ALS Clinic (Fund 1078) and mail it to 1120 15th St., BP-4390, Augusta, GA 30912.

ALS, or amyotrophic lateral sclerosis, is more commonly known as Lou Gehrig’s disease, named for the late first baseman and power hitter for the New York Yankees. Gehrig was stricken with the neurodegenerative disease that causes muscular atrophy and forced into retirement at age 36. It claimed his life two years later.

About 6,000 people are diagnosed with ALS each year. The GRHealth ALS Clinic cares for about 150 patients between the Augusta and Macon locations.

ALS patient gets diaphragm pacemaker

NeuRx Diaphragmatic Pacing System may improve longevity, quality of life for ALS patients

AUGUSTA, Ga. – For her 67th birthday, Lettie Sue Abercrombie enjoyed a scenic mountain drive with her husband and a surprise party attended by nearly 50 family members and friends. Five days later, the Danielsville grandmother became the second patient at Georgia Regents Medical Center to receive a NeurRx Diaphragm Pacing System – a device that could help Abercrombie, who has ALS, have a better quality of life and see a few more birthdays.

“I am excited to be able to get this new machine. My breathing has been pretty good, but I can’t exert myself like I used to,” said the retired medical assistant who was diagnosed with ALS, or amyotrophic lateral sclerosis, in May 2013. Often called Lou Gehrig’s disease, ALS is a rapidly progressing, incurable and fatal neuromuscular disease characterized by systematic muscle weakness that results in paralysis. “As my diaphragm gets weaker, the device will stimulate it,” reminding her body to breathe, a process that will become more and more difficult as the ALS advances.

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“Most ALS patients develop chronic hypoventilation over the course of their disease,” said Dr. Michael H. Rivner, Director of the Georgia Regents ALS Clinic. “In normal respiration, people breathe deeply and often enough to clear the carbon dioxide carried to the lungs for elimination. But when people hypoventilate, they breathe too shallowly or slowly, reducing oxygen intake and producing dangerously high levels of carbon dioxide in the blood. Traditionally, we could only assist our patients with non-invasive ventilation. Now, we can provide the opportunity for a patient to breathe for a longer period without needing a mechanical ventilator, essentially offering a better quality of life,” Rivner explained.

Implanted through minimally invasive laparoscopic surgery, the Diaphragm Pacing System includes four electrodes for stimulating the diaphragm muscle, and a fifth electrode under the skin to complete the circuit. The other components are a connector holder, a cable, and an external battery-powered pulse generator.

“The pulse generator regulates movement of the diaphragm muscle, creating a vacuum-like effect in the chest cavity that draws air into the lungs. Then, when the contraction eases, the air is expelled,” said Dr. Vijay Patel, the cardiothoracic surgeon who implanted the device. “Put simply, the pacemaker provides electrical stimulation to the muscle and nerves in the diaphragm, contracting the diaphragm and conditioning those muscles to resist fatigue.”

The device is programmed to allow an effective yet comfortable breath. Each patient must begin with a regimen of three 30-minute sessions a day to condition the diaphragm muscle. Then, as ALS progresses and breathing weakens, the conditioning time increases.

“The goal is to provide patients both comfort and longevity,” said Patel.

Approximately 30,000 people in the United States live with ALS, and more than 5,600 new cases are diagnosed each year. An estimated 3,300 ALS patients could benefit from this new pacing system.

“We are tickled that she got it put in,” said Herschel Abercrombie, now a full-time caregiver to his wife who can no longer walk or even lift her legs. She gets around in a power chair and with a special hoist installed in the ceiling of their home. Mr. Abercrombie bathes her, clothes her, and cooks for her.

It’s not quite the retirement – traveling, camping, and fishing together – that he had envisioned with the woman he’s loved since high school. But he’s thankful for every day they are given, days that could increase based on previous outcomes with the device.

“It’s all in the Lord’s hands,” he says. “We pray every day that God will take care of her; but if he heals her or takes her to heaven, she’s a winner either way.”

About the ALS Clinic
The nationally accredited Georgia Regents ALS Clinic serves about 150 patients across the Southeast. The clinic, which opened in 2004 through a partnership with the ALS Association of Georgia, takes a multidisciplinary and coordinated approach to patient care. Instead of scheduling multiple appointments and trips, patients are able to see neurologists; nurses; physical, occupational, and speech therapists; social workers; dietitians; respiratory therapists; and equipment specialists all on the same day. This is especially helpful for ALS patients because of diminishing mobility. The Georgia Regents ALS team sees patients on the second Friday of each month in Augusta and the fourth Friday of each month at a satellite clinic in Macon. They assess disease progression, functional status, and family concerns, as well as equipment, transportation, and referral needs. In addition, family and caregiver training and support are incorporated into the time spent with each patient. For more information, call 706-721-2681.

ALS Walk donations will stay local

AUGUSTA, Ga. – The ice bucket challenge that swept the nation this summer helped generate more than $113 million in donations to the national ALS Association.

“That’s great news,” said Dr. Michael H. Rivner, Director of the Georgia Regents ALS Clinic, “but what we really need are local donations. By giving locally, you can have a direct impact on the lives of patients treated right here in Augusta.”

One of the ways to support the Georgia Regents ALS Clinic is by participating in the Beat Feet for ALS fundraising walk at 8:30 a.m. Saturday, Sept. 27, at Augusta’s Riverwalk. Proceeds from the walk will help fund additional resources and equipment to enhance the lives of area patients with this terminal disease.

“Amyotrophic lateral sclerosis, or ALS, is devastating. The muscles start to deteriorate rapidly until you are essentially trapped inside your own body,” said Rivner. “There is no cure, and, sadly, most patients will die within five years of their first symptoms.”

About 6,000 people in the United States are diagnosed with ALS each year. The nationally accredited Georgia Regents ALS Clinic serves about 150 patients across the Southeast.

The clinic, which opened in 2004 through a partnership between the Georgia Regents Neuroscience Center and the ALS Association of Georgia, takes a multidisciplinary and coordinated approach to patient care. Instead of scheduling multiple appointments and trips, patients are able to see neurologists; nurses; physical, occupational, and speech therapists; social workers; dietitians; respiratory therapists; and equipment specialists all on the same day. This is especially helpful for ALS patients because of diminishing mobility.

The Georgia Regents ALS team sees patients on the second Friday of each month in Augusta and the fourth Friday of each month at a satellite clinic in Macon. They assess disease progression, functional status, family concerns, and equipment, transportation, and referral needs. In addition, family and caregiver training and support are incorporated into the time spent with each patient.

“It could cost as much as $250,000 a year to treat just one patient with ALS, and insurance companies don’t necessarily pay for all of that. But studies have shown that being seen in a multidisciplinary clinic improves the longevity of patients – their health and quality of life, and that’s what we want to achieve for our patients and their families,” said Rivner, Charbonnier Professor of Neurology at the Medical College of Georgia at Georgia Regents University. Donations also support clinical trials – such as an upcoming study on magnetic stimulation – and other ALS research efforts being conducted at GRU.

To register for the walk or make a donation, visit walk.ALSGRU.com or contact Brandy Quarles at bquarles@gru.edu or 706-721-2681.

You can also make a donation directly to the Georgia Regents ALS Clinic on the website or send a check payable to the clinic by mail to:

ALS Clinic (Fund 1078)
1120 15th St., BP-4390
Augusta, GA 30912

 

New cause found for muscle-weakening disease myasthenia gravis

MeiresearchwebAn antibody to a protein critical to enabling the brain to talk to muscles has been identified as a cause of myasthenia gravis, researchers report.

The finding that an antibody to LRP4 is a cause of the most common disease affecting brain-muscle interaction helps explain why as many as 10 percent of patients have classic symptoms, like drooping eyelids and generalized muscle weakness, yet their blood provides no clue of the cause, said Dr. Lin Mei, Director of the Institute of Molecular Medicine and Genetics at the Medical College of Georgia at Georgia Regents University.

“You end up with patients who have no real diagnosis,” Mei said.

The finding also shows that LRP4 is important, not only to the formation of the neuromuscular junction – where the brain and muscle talk – but also maintaining this important connection, said Mei, corresponding author of the paper in The Journal of Clinical Investigation.

Mei and his colleagues first reported antibodies to LRP4 in the blood of myasthenia gravis patients in the Archives of Neurology in 2012. For the new study, they went back to animals to determine whether the antibodies were harmless or actually caused the disease. When they gave healthy mice LRP4 antibodies, they experienced classic symptoms of the disease along with clear evidence of degradation of the neuromuscular junction.

LRP4 antibodies are the third cause identified for the autoimmune disease, which affects about 20 out of 100,000 people, primarily women under 40 and men over age 60, according to the National Institutes of Health and Myasthenia Gravis Foundation of America, Inc.

An antibody to the acetylcholine receptor is causative in about 80 percent of patients, said Dr. Michael H. Rivner, MCG neurologist and Director of the Electrodiagnostic Medicine Laboratory, who follows about 250 patients with myasthenia gravis. Acetylcholine is a chemical released by neurons which act on receptors on the muscle to activate the muscle. More recently, it was found that maybe 10 percent of patients have an antibody to MuSK, an enzyme that supports the clustering of these receptors on the surface of muscle cells.

“That leaves us with only about 10 percent of patients who are double negative, which means patients lack antibodies to acetylcholine receptors and MuSK,” said Rivner, a troubling scenario for physicians and patients alike. “This is pretty exciting because it is a new form of the disease,” Rivner said of the LRP4 finding.

Currently, physicians like Rivner tell patients who lack antibody evidence that clinically they appear to have the disease. Identifying specific causes enables a more complete diagnosis for more patients in the short term and hopefully will lead to development of more targeted therapies with fewer side effects, Rivner said.

To learn more about the role of the LRP4 antibody, Mei now wants to know if there are defining characteristics of patients who have it, such as more severe disease or whether it’s found more commonly in a certain age or sex. He and Rivner have teamed up to develop a network of 17 centers, like GR Medical Center, where patients are treated to get these questions answered. They are currently pursuing federal funding for studies they hope will include examining blood, physical characteristics, therapies and more.

Regardless of the specific cause, disease symptoms tend to respond well to therapy, which typically includes chronic use of drugs that suppress the immune response, Rivner said. However, immunosuppressive drugs carry significant risk, including infection and cancer, he said.

Removal of the thymus, a sort of classroom where T cells, which direct the immune response, learn early in life what to attack and what to ignore, is another common therapy for myasthenia gravis. While the gland usually atrophies in adults, patients with myasthenia gravis tend to have enlarged glands. Rivner is part of an NIH-funded study to determine whether gland removal really benefits patients. Other therapies include a plasma exchange for acutely ill patients.

The Journal of Clinical Investigation study was funded by the NIH and the Muscular Dystrophy Association. Mei is a Georgia Research Alliance Eminent Scholar in Neuroscience.

ALS Walk slated for Sept. 28

AUGUSTA, Ga. – Patients, families, employees and friends will Beat Feet for ALS at 8 a.m. Saturday, Sept. 28 at Augusta’s Riverwalk in an effort to raise money for the Georgia Regents ALS Clinic.

ALS, or amyotrophic lateral sclerosis, is more commonly known as Lou Gehrig’s Disease, named for the first baseman and power hitter for the New York Yankees. Gehrig was stricken with the neurodegenerative disease that causes muscular atrophy and forced into retirement at age 36. It claimed his life two years later.

Nearly 6,000 people in the United States are diagnosed with ALS each year, and the life expectancy is just two to five years. But effects of the disease vary, and many people can live with quality for five years and more with the help of nationally accredited clinics like the one at Georgia Regents.

Formed in 2004 through a partnership between the Georgia Regents Neuroscience Center and the ALS Association of Georgia, the clinic serves about 100 ALS patients across the Southeast.  Because ALS patients lose the use of their muscles, it becomes difficult for them to make multiple trips for several appointments, so the clinic features a comprehensive, multidisciplinary and coordinated approach to patient care.  Patients can see their neurologists and nurses as well as physical, occupational and speech therapists, social workers, dietitians and respiratory therapists on the same day.

The clinical team sees patients on the second Friday of each month in Augusta and the last Friday of each month at a satellite clinic in Macon in order to assess disease progression, functional status, family concerns, and equipment and referral needs. Family and caregiver training are incorporated into the time spent with each patient. In addition, an ALS Support Group meeting is part of the monthly clinic experience.

“Insurance doesn’t always pay for multiple visits and additional services at the clinic, but studies have shown that being seen in a multidisciplinary clinic improves the longevity of patients – their health and quality of life,” said Dr. Michael H. Rivner, Medical Director of the ALS Clinic and Professor of Neurology for the Medical College of Georgia at Georgia Regents University. “We hope to raise additional funds to meet our patients’ needs through this walk.”

To register for the walk or make a donation, visit walk.ALSGRU.com or contact Brandy Quarles at 706-721-2681 or bquarles@gru.edu. The walk is sponsored by Accredo, Allergan and Integrity Medical.