FINDING CURES FOR MANKIND

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Starkey Halo

19th Sep 2014 Starkey Hearing Foundation

On March 31, Starkey introduced a hearing aid that is compatible with the iPhone, iPod touch, and iPad. In keeping with current technology trends they offer the ability to stream phone calls, FaceTime, music, and more directly to their hearing aid using Bluetooth. Users have the ability to control the device through the app (TruLink) or manually. Many hearing aids on the market have had the ability to work wirelessly, but they require a secondary device. Since this is often something that had to be worn around the neck many individuals find it more cumbersome than it’s worth. The Starkey hearing aid does not require any complicated set up or additional devices, making it easier to take advantage of all the features.

In addition to streaming phone calls and music, this hearing aid also has some additional features that make it a top-of-the-line product. They have improved the audibility of high-frequency sounds (like women’s and children’s voices) by lowering the frequency, they’ve developed a state of the art feedback canceller to eliminate buzzing and whistling, they provide sound compression technology to differentiate soft and loud noises, and the device has advanced noise reduction to make speech easier to hear in congested environments. In addition, the app has many different settings to allow for more adjustments and personalization than most hearing aids on the market.

The interface is intuitive and quick to use, giving users the flexibility they need to adapt to new situations. Individuals that have experienced hearing loss report that their hearing is back to “normal” and they can hear parts of conversations they would have missed otherwise. Users can use their iPhone as a microphone to stream sounds directly to the hearing aid as well. An example would be trying to hear a conversation in a noisy restaurant. You can use your iPhone to amplify the conversation you want by placing it closer to the people you want to hear and you can even disable the ear-level mic to block out ambient sounds.More great features include the ability to have Siri read your emails and texts to you, a “find-my-hearing-aid” feature to located misplaced hearing aids, and personalized geo-tags and settings based on coordinates. These geo-tags allows you to set hearing preferences for up to 20 locations of your choice.

The Starkey Halo hearing aids are available through your hearing care professional only, but the app is free to download from the app store.

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Puccio Study FA

19th Sep 2014 Diseases, Friedreich's Ataxia

A recent breakthrough in Friedreich’s ataxia (FA) research gives hope that gene therapy can prove be an effective treatment for the disease. A team led by Hélène Puccio, Ph.D., has demonstrated that gene therapy can be used to reverse heart damage caused by FA and correct mitochondrial metabolism. Dr. Puccio works as the head of a research team at the Institute of Genetics and Molecular and Cellular Biology located at the University of Strasbourg, France, as well as research director at the Insititute de Santé et de la Recherche Médicale (INSERM.) She and her team have concentrated their research on the pathophysiological mechanisms involved in recessive ataxias, like FA, in order to develop new animal and cell models.

Friedreich’s ataxia is rare, but one of the more common recessive ataxias – found in 1 in every 50,000 births. It is caused by a mutation in the frataxin (FXN) gene, which in turn causes a decrease in the amount of frataxin produced. The lack of frataxin disrupts the mitochondria that are responsible for the cell’s energy production, and, unfortunately, heart tissue is particularly vulnerable.

While FA causes progressive neurodegeneration that starts as impaired balance and coordination, it also impairs heart function and can contribute to diabetes. Most patients require a wheelchair after about 10-20 years of disease progression, but complications with the heart lead to death in more than half of individuals, and often before the age of 35.

Dr. Puccio’s team used a harmless viral vector to insert a normal copy of the problematic gene in the heart cells of FA mouse models. Since these mice show the same heart symptoms as human patients suffering from the disease the results are especially promising. The virus used was an adeno-associated virus (AAV) since it is known to effectively and efficiently target heart cells and express therapeutic diseases. This virus was modified to render it harmless and only capable of inserting the normal FXN gene copy into the cells.

One single intravenous injection was not only able to prevent the development of heart disease in the mice, but it quickly and completely reversed the heart damage in advanced cases. Within three weeks the mice appeared to have completely restored heart and mitochondrial function and their heart tissue appeared similar to healthy mice.

Since this is the first time that gene therapy has produced such a fast and complete remission of heart disease in an animal model work is rapidly being done to begin clinical studies. There is also hope that this technique can be used to prevent or even correct damage to the spinal cord and cerebellum.

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FARA Energy Ball Research Panel

19th Sep 2014 Diseases, Friedreich's Ataxia

There were many excellent speakers at this year’s FARA Energy Ball that covered a wide variety of topics – from recent scientific breakthroughs to first-hand experiences of those suffering from the disease. Three leaders in the Friedreich’s ataxia research community spoke regarding current genetic approaches to therapies and shared some of their most recent findings.

One of the speakers was Joel Gottesfeld, Ph.D. Dr. Gottesfeld currently works as a professor in the Department of Molecular Biology at the Scripps Research Institute. He studies several genetic human diseases – one of which is Friedreich’s ataxia. In addition to studying general characteristics of this disease Dr. Gottesfeld and his staff are working to develop new treatments.

Their research has led to the discovery of a new group of histone deacetylase (HDAC) inhibitors. These small molecules were found to reverse the heterochromatin-mediated silencing of the frataxin (FXN) gene. Further research with mouse models has shown that these HDAC inhibitors can cross the blood brain barrier, do not appear to cause toxicity at expected therapeutic levels, and do act as HDAC inhibitors in the mouse brain. Even more promising, they demonstrated that the HDAC inhibitors increased the FXN gene expression and protein in the blood cells of FA patients.

Another speaker was Hélène Puccio, Ph.D. Dr. Puccio is the head of a research team at the Institute of Genetics and Molecular and Cellular Biology located at the University of Strasbourg, France, as well as a research director at the Insititute de Santé et de la Recherche Médicale (INSERM.) She has concentrated her research on the pathophysiological mechanisms involved in recessive ataxias, like Friedreich’s ataxia, in order to develop new animal and cell models.

During one of her studies she and her colleagues were able to use mice with no frataxin in their heart to demonstrate that gene therapy can be used to reverse heart damage and correct the mitochondrial metabolism. This finding leads to the possibility of using gene therapy as a treatment for the disease.

Finally, Massimo Pandolfo, MD works for Université Libre de Bruxelles at the Hôspital Erasme and has been the principal investigator of the European Friedreich’s Ataxia Consortium for Translational Studies (EFACTS.) Dr. Pandolfo led an international collaboration that discovered the FA gene back in 1996 and has continued to contribute in many ways over the years. He has been working with everything from research trials to clinical care for patients. In addition, he has contributed to our understanding of the disease process, clinical characteristics, and has helped to advance drug development.

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FA Dyclonine Study Review

19th Sep 2014 Diseases, Friedreich's Ataxia

Freidreich’s ataxia (FA) is a recessive disease causing progressive damage to the nervous system. It is the most prevalent inherited cause of ataxia and affects about 1 in 50,000 people in the United States. Despite its prevalence, there is no cure or treatment beyond supportive care.

A recent article was published in the Human Molecular Genetics journal in regards to a potential new therapy for FA. The drug, dyclonine, was developed for use as a topical anesthetic and is found in the over the counter throat lozenge, Sucrets, as well as some Cepacol throat sprays. FA is caused by a genetic deficiency in the mitochondrial protein frataxin (FXN) and dyclonine was noticed to increase FXN protein in animal models. When the drug was given a mouse model of FA it prevented a performance decline during balance beam tests. A proof-of-concept clinical study was performed on eight patients, which showed an increase in buccal cell FXN.

Mice used in this study were tested with level balance beams of varying thickness that required them to walk from one end to the other in order to seek shelter. Mice were timed and video recorded in order to count the number of foot slips. The individual in charge of recording the data was blinded to which treatment group the mouse was in. Mice that were dosed with dyclonine had statistically significant improvements. Not only did their performance on the beams not worsen like the affected controls, but they even improved their time to cross.

The second part of the study involved using dyclonine as an oral rinse, since that is already FDA-approved, and then swabbing the cheeks for buccal cells. The cells were compared to pre-treatment cells from the same patients. Six of the eight showed an increase in FXN, two showed no increase, but two healthy controls showed increased levels. Interestingly, the patients with the most neurological impairment were the ones that showed the highest increase from the dyclonine mouth rinse. Neurological impairment was determined with a FARS (Friedreich’s Ataxia Rating Scale) score.

What’s next? Further clinical studies are required to see if the results achieved from the oral rinse can be duplicated systemically and show the same improvements as observed in the mouse model. Additionally, optimal dosing, toxicology, and pharmacokinetics all need to be studied and better understood before testing can proceed. Despite the questions that still need addressed, dyclonine could be a new treatment option for people affected by Friedreich’s ataxia.

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Enterovirus 68

19th Sep 2014 Diseases, Enterovirus 68

A rare respiratory illness has been all over the news recently, especially with the addition of two new states – Oklahoma and Pennsylvania. These states have recently been added to the growing group with confirmed cases of enterovirus 68. The other states include New York, Alabama, Indiana, Louisiana, Colorado, Illinois, Iowa, Kansas, Kentucky, and Missouri. Georgia, Michigan, Ohio, and Utah suspect that they have cases, but it has not been confirmed yet.

Enteroviruses are incredibly common, however enterovirus 68 is a rarer strain that can cause breathing problems in children. It was first isolated in California in 1962 and has generally turned up in sporadic, isolated instances. It usually surfaces later in the enterovirus season, which is equivalent to August-September in the Northern hemisphere. Outbreaks have become more common in the 21st century and have included countries like the Philippines, Japan, the Netherlands, and in multiple US states.

In general, children under 5 years of age are the most at risk, but the disease has been found in older children and adults with asthma as well as immunosuppressed adults. The disease causes typical respiratory illness symptoms.

There is no vaccine or treatment beyond supportive care, so it is recommended to use common sense and good hygiene for protection. Since the virus is spread through saliva, phlegm, and stool you should wash your hands regularly, cough or sneeze into your arm (not your hands) and avoid prolonged exposure with sick people. If you are ill try to avoid prolonged contact with immunocompromised individuals, very young children, and infants. The disease generally runs it course without any complications and very few people need to be hospitalized.

While enterovirus 68 has been a hot topic recently, it appears that the number of children with the illness has peaked toward the end of August. More states may be added to the list, but it should not be taken as a sign that the disease is suddenly spreading to new locations. Since only the CDC and a handful of other laboratories are able to test for this particular strain there is a backlog of samples.

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Patients with Friedreich’s Ataxia Are Fighting Back

25th Jul 2014 Friedreich's Ataxia

Friedreich’s Ataxia impacts one in every 50,000 people within the United States. This neurological disorder can impact people of all ages but typically starts in childhood. The disease ravages the body to the point that most people only live ten to twenty years after being diagnosed before they need help of a wheelchair and eventually become incapacitated. This is a terrible way to live, and Dr. Robert Dean has been working with the Friedreich’s Ataxia Research Alliance (FARA) to help find a cure.

Recently, several people diagnosed with the disease have decided to fight back. Rather than being held down by the disease, they are stretching the boundaries of what it means to be someone diagnosed with it. In so doing, they are raising needed awareness and funds for the cause.
Barry Rice, a 34-year-old man from Ireland, has participated in putting together the first race for Friedreich’s Ataxia in the area. Cycle Ataxia will take place on August 10th in Ashbourne, and Mr. Rice is hoping that 500 cyclists will participate. He will be on hand in his wheelchair, participating in the event as much as possible. He was diagnosed with late onset Friedreich’s Ataxia and is able to do more things that many people who suffer from the disease. Still, he is confined to a wheelchair and has difficulty doing simple tasks like tying his shoes. He wants to raise awareness so that others suffering from the disease know they are not alone, and more money can go towards research.

The Fryatt family will be climbing Mt. Kilimanjaro this October to raise awareness for the disease. This would be a feat in and of itself but is made more so by the fact that Iain Fryatt has Friedreich’s Ataxia. Climbing a mountain would seem out of reach for most people suffering from a neurological disorder but the Fryatt family is determined. He will be using a Mountain Trike to ascend the mountain. The device will allow him to steer using a lever, and it will be the very first time someone has attempted to climb Kilimanjaro using one. The climb will take them over a week, and they are hoping to raise over $10,000 for the climb. The money will then be given to research.

Both Barry and Iain are not letting Friedreich’s Ataxia slow them down or define their life. They are true heroes that are committed to using their abilities to help others suffering from the disease. Instead of focusing on what they cannot do, they are focusing on what they can do.
As a humanitarian and physician, Dr. Robert Dean believes in the power of joining together to support a cause. Since working with FARA, he has witnessed money going into research with clear results. They now know what causes Friedreich’s Ataxia and are testing ways to implement a cure. There is still a long ways to go but with scientists, community leaders, patients and physicians like Dr. Dean working together, anything is possible.

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Dr. Robert Dean and the Friedreich’s Ataxia Research Alliance (FARA)

08th Jul 2014 Diseases, Friedreich's Ataxia

Dr. Robert Dean is actively involved with the Friedreich’s Ataxia Research Alliance (FARA) and working with them on ways to help find a cure. Friedreich’s Ataxia (FA) is a condition that causes damage to the nervous system. It will often start in childhood and worsen over time until it begins to impair movement. The disease impacts around 1 in every 50,000 people within the U.S. Unfortunately, the disease did not get very much attention, funding, or research until FARA was founded. The organization has led the awareness and research efforts since 1998 and continues to do so today.

What are the symptoms of Friedreich’s Ataxia?

The disease impacts people in different ways but generally speaking the symptoms will start to occur between the ages of five and fifteen. It typically starts by making it difficult for people to walk with the Ataxia spreading into the arms and core. Some patients will also lose their tendon reflexes and suffer from scoliosis. As time goes on patients may start to have their speech slur and develop hearing and vision loss, as well.

In addition to making large impacts on their ability to move, patients find it difficult to recover from common illnesses like colds and the flu. They become fatigues more easily and frequently suffer from chest pain, shortness of breath, and heart palpitations, common symptoms that result for accompanying heart disease.

Once the symptoms have started manifesting most patients only have ten to twenty years before they are confined to a wheelchair and eventually become incapacitated. This is incredibly devastating considering that most people start to suffer from Friedreich’s Ataxia when they are small children. By the time they are teenagers they could be permanently confined to a wheelchair.

How FARA Helps Patients

FARA was established in 1998 by a dedicated group of scientist and patient families that were determined to learn everything they could about the disease, what causes it and how to cure it. Their efforts have been successful in that millions of dollars have been poured into FA research and clinical trials throughout the world. With drugs in clinical trials, FARA continues to work on funding and creating public, private partnerships to further research and development.

Dr. Robert Dean and FARA

When Dr. Robert Dean became aware of Friedreich’s Ataxia, he was moved beyond measure. Witnessing small children suffer from an incurable genetic disease was something he could not stand by and watch. Listening to patients tell stories of heartbreak and learning to overcome their condition was inspirational and motivating. When children and young adults can be so confident in the face of a debilitating disease it brings what needs to be done into focus and Dr. Dean had to get involved. He has since been focused on bringing awareness to the disease, FARA, and the patients that are impacted by it. Through participation in various programs and fundraising activities, Dr. Robert Dean continues to push forward and partner with FARA to find a cure for those that need it most.

He invites you to join him in these efforts by learning more about FARA, volunteering, or donating so that those suffering from the disease can lead longer and fuller lives. To contact Dr. Dean, email contact@doctorrobertdean.com.

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Dr Dean Blog – Special Column in Tampa Bay Times for World Health Day

08th Apr 2014 Uncategorized

The Tampa Bay Times is running a special column I wrote in honor of World Health Day:

Column: Health Agency’s Flaws Hurt the Hearing-Impaired

As a physician who has devoted significant time to traveling the world recently on a volunteer mission to help people with hearing impairments, I have a new sense of the significance of World Health Day, which was celebrated Monday. I also have a new sense of the limitations of the organization associated with this day: the World Health Organization.

Since November, I have visited five developing countries as a volunteer physician with the Starkey Hearing Foundation, a pragmatic, results-based organization committed to fitting 1 million hearing-impaired people with hearing aids worldwide. My travels have taken me to the Philippines, Haiti, Senegal, Liberia and Mexico.

Founded in 1984 by William and Tani Austin, the Starkey Hearing Foundation has given the gift of hearing, and changed lives, for more than 1 million children and adults over these past 30 years.

Seeing the excited smiles of children who are hearing their mother’s voice for the first time has been incredibly satisfying, but it has also been terribly frustrating. Growing up the son of an immigrant and a pharmaceutical research scientist, I have always had a passion for finding cures for diseases in the developing world. During my travels, I’ve come to recognize that a significant share of the hearing loss in these countries is entirely preventable…
Click here to read the whole thing.

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Dr Dean Blog – Talking Starkey for What’s Right in Tampa Bay

08th Apr 2014 Uncategorized

I had the pleasure of being featured on Fox13, WTVT, last night. Kelly Ring put together a story about my recent travels with the Starkey Hearing Foundation for a segment called “What’s Right in Tampa Bay.” The Fox13 crew were a pleasure to work with, and the story explains what my work with the Starkey Hearing Foundation means for my mandate as a physician. You can watch the story on my media page: http://www.doctorrobertdean.com/media/, or at http://www.myfoxtampabay.com in their “What’s Right” tab: http://www.myfoxtampabay.com/category/235258/whats-right-with-tampa-bay.

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Dr Dean Blog – Starkey Hearing Foundation Press Release

06th Mar 2014 Uncategorized

Florida-based Dr. Robert Dean Continues Spreading the Gift of Hearing Worldwide With the Starkey Hearing Foundation
Destinations include Haiti, Senegal, Liberia, and Mexico

Tampa, FL (March 6, 2014) – Thousands of people across the world are hearing sounds for the first time, thanks to the Starkey Hearing Foundation. Robert Dean, MD, a Tampa, Florida-based physician and journalist, is personally responsible for fitting nearly one thousand people with their first hearing aids.

Dr. Dean just returned from a trip to Mexico, where he continued his volunteer work for the Foundation. He began traveling with the Starkey Hearing Foundation in November of 2013, spending two weeks in The Philippines during the aftermath of Typhoon Haiyan. He followed that up with trips to Haiti, Senegal, and Liberia. In each location, the Starkey Hearing Foundation provided thousands of people with free hearing aids, making sure they fit each individual ear.

Hope 2 Haiti director Scott Bonnell says: “It is because of sponsors like Dr. Robert Dean, here fitting children with their new hearing aids, that hundreds of orphans in remote villages like St. Marc are able to hear for the first time.”

Studies confirm that children born with hearing loss become isolated from society, and rarely develop the speech that is so essential for their social and intellectual growth. The Starkey Hearing Foundation is not just committed to helping people achieve that connection to society, but also sheds a light on how to reduce instances of preventable hearing loss. The Foundation shares research into which types of medicines can contribute to deafness, and encourages groups like the Gates Foundation to continue their work bringing vaccines to developing nations, in order to reduce cases of congenital hearing loss.

The Starkey Hearing Foundation was founded in 1984 by William and Tani Austen. They recently made a commitment to the Clinton Global Initiative to distribute one million hearing aids over the next ten years.

Dr. Dean, a physician with a long record of exploring the natural origins of medicine, will share commentaries and videos from his travels in advance of World Health Day. He blogs about his journeys at www.doctorrobertdean.com. You can also follow him online at www.twitter.com/drrobertdean or www.facebook.com/drrobertdean.
Dr. Dean in Senegal Dr. Dean in Senegal with the Starkey Hearing Foundation

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