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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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The Ebola Virus and the Case for Modern Medicine

07th Aug 2014 Ebola, Viruses

Newspapers throughout the world have been carrying news of the recent outbreak of Ebola. According to the World Health Organization 932 people have been killed thus far, the majority of them in Africa. This disease has taken the lives of at least 100 health care workers trying diligently to bring health back to the infected. These efforts have not been in vain as over 1,700 people have been infected since the outbreak began with around 54 percent of these cases resulting in death.

What you need to know about the Ebola virus.

Ebola primarily occurs in Central and West Africa near the rainforest. Outbreaks generally start when an animal infects a human. This could be in the form of a bite, their feces, secretions, bodily fluids, etc. Fruit bats are notorious for carrying the disease. Once it comes in contact with humans, it is passed person to person. This is not an airborne disease so it cannot spread by simply coughing around another person. There must be some type of fluid exchange for the disease to spread. This can happen via direct or indirect contact with bodily fluids, blood, treating people that are infected, and handling dead bodies.

By understanding how the disease is transmitted, it is easier to avoid becoming infected if you are in an area where an outbreak is occurring. Unfortunately, many of these smaller villages do not have access to this information or basics like clean water which would allow them to live in more sanitary conditions where the risk of infection is reduced.

Once a person becomes sick there are several signs that it could be Ebola. They start to run a high fever, feel weak, headache, muscle pain, and sore throat. These symptoms are very similar to the flu which makes it even harder to identify. Unfortunately, the symptoms do not manifest right away. It can take two to 21 days before a person knows that they are sick. The disease can live inside of a person for 61 days after becoming contaminated, creating additional risk for mass exposure. Very often, people go about their lives not knowing that they could be spreading a deadly disease.

Modern Medicine

As a doctor and humanitarian, Dr. Dean has traveled the world providing the gift of hearing to those in need. This is one of the many ways that he is using modern medicine to transform lives of individuals and their community. The Ebola virus demands a similar approach. Modern medical care can prevent many people from dying from the disease. Access to basic solutions like IV fluids in a contained hospital wing can save lives and prevent the disease from spreading.

Dr. Dean is also keenly aware of the need to prevent outbreaks in the first place by training local community leaders on how to identify the disease and ensure that infected individuals are quarantined and treated right away. People in West Africa and throughout the world can benefit from receiving education on how to avoid coming in contact with the disease, then understanding what to do when symptoms develop. Humanitarians, like Dr. Dean, can play a critical role in stopping this disease by developing educational and treatment programs then training local leaders. For those fortunate enough to live in the United States, focusing on good hygiene is an easy way to avoid disease like this since they require a person coming in direct or indirect contact with an infected persons bodily fluids.

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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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Surprise Performers Join The Starkey Hearing Foundation Gala

18th Jul 2014 Starkey Hearing Foundation

The Starkey Hearing Foundation So The World May Hear Gala will take place on July 20th in St. Paul Minnesota. This high-octane annual event will be jammed packed with celebrity performers and humanitarians from throughout the world. The foundation was pleased to announce that this year’s surprise performer is Grammy-Award winning artist, Rob Thomas. This is set to be an incredible and unforgettable evening as Rob Thomas takes the state with Sammy Hagar and John Legend.

Each year the gala raises money to further the efforts of The Starkey Hearing Foundation to give the gift of hearing to children throughout the world. Humanitarians, activists, donors, volunteers, and celebrities come together for one purpose and one cause – helping children to hear. This is a monumental goal, and the foundation has been to over one hundred countries thus far. Each trip, each encounter, is life changing, and this one night honors those that make that mission possible.

Dr. Robert Dean has been privileged to work closely with The Starkey Hearing Foundation and has already been on multiple global medical missions with their group. He has traveled the far reaches of the globe, experiencing firsthand the life-changing gift that hearing brings to a child. It is one thing to be a physician in the United States where healthcare is readily available. It is completely different to work in a country where your help may be the only medical help that child ever receives. He has witnessed how a child, after being fitted with a hearing aid, is accepted back into a society that had previously viewed them as an outcast. Hearing does more than open up a world of sound. It enables children, and adults, to be an active and contributing member of society that will change the course of their entire life.

This year’s So The World May Hear gala will be full of star performers like Rob Thomas, who believe in the foundations mission. While it is easy to get caught up in the hype of a celebrity endorsed organization, The Starkey Foundation has been very conscious of its primary mission, to bring hearing to the world. As such, it has made a concerted effort to work with local organizations and governments to provide hearing aids and, even more importantly, access to long-term hearing health care in places it would be least expected. Using an effective three-step approach the foundation has worked effectively across cultures and continental boundaries, something that Dr. Robert Dean is an active part of.

This July 20th, the foundation once again hopes to bring a spotlight to the need for the global hearing initiative while commending those who are giving a portion of their lives to making the world a better place. Dr. Robert Dean will be in attendance, once again lending his support to a cause worth fighting for. To learn more about the foundation, its goals or its mission visit www.starkeyfoundation.org.

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Secretary Hillary Clinton to Speak at The Starkey Hearing Foundation Gala

17th Jul 2014 Starkey Hearing Foundation

Dr. Robert Dean has been privileged to support The Starkey Hearing Foundation in their efforts to give the gift of hearing to children throughout the world. This year’s special guest speaker is none other than the renowned Secretary Hillary Clinton. Fresh off her worldwide book tour, Clinton will be lending a hand to a cause worth fighting for.

The Starkey Foundation has been incredibly blessed by the presence of celebrities from all over the world who truly care about giving the gift of hearing. Many celebrities and humanitarians will be attending the annual So The World May Hear gala in St. Paul MN. The Starkey Hearing Foundation has used this function to recognize and honor those who have made hearing possible through their generous donations of time and money, and this year is no exception. This year, the So The World May Hear gala will take place on July 20th, 2014 so that humanitarians, volunteers, and donors can be recognized as those that make the foundations work possible.

This year the gala, and all of those involved with the foundation are pleased to announce a very special guest. For many years now the Starkey Foundation has received a great deal of support in its mission to spread the gift of hearing from the Clinton Foundation. This year the So The World May Hear gala is going to be the host to special guest speaker former Secretary of State Hillary Clinton. Secretary Clinton has been a longtime supporter, through both the Clinton Foundation and the Clinton Global Initiate, but this is the first time she will be joining the Starkey foundation and its guests for the gala. Given her busy book tour and political efforts, the foundation is truly honored by her presence and the commitment it shows to children around the world.

With a list of humanitarians that feature the Archbishop Desmond Tutu, Forrest Whitaker, Prince Seeiso of Lesotho and Sandi Young, this year’s gala promises to be spectacular. Dr. Robert Dean, the Florida-based humanitarian, physician, and journalist has already announced his intention to attend year’s function. Adding Secretary Clinton, as the guest speaker, is both profound and exciting for the foundation.

Beyond the glitz and glamour of a once a year event, The Starkey Hearing Foundation has been a worldwide advocate at the forefront of the global hearing initiative. The founders, Bill and Tani Austin, believe that there is no such thing as a disability only ability and have built an organization that takes this message the furthest reaches of the earth. As of this year the Starkey Foundation has visited over one hundred countries and has delivered and equipped over 1.43 million people with hearing aids. As they traverse the planet helping people hear The Starkey Foundation, and volunteers like Dr. Robert Dean have changed countless lives bringing a whole new world of possibility and opportunity to millions of people.

You too can make a difference by supporting The Starkey Hearing Foundation. Learn more by visiting www.starkeyfoundation.org.

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Dr. Robert Dean to Attend the Starkey Hearing Foundation Gala

15th Jul 2014 Starkey Hearing Foundation

Each year the stars align over St. Paul MN, and the supporters and fans of the Starkey Hearing Foundation will come together to celebrate giving the gift of hearing to children throughout the world. At the So The World May Hear gala the spirit of giving will be once again honored, and those who have taken the time to participate and give back to the community will have a spotlight shone upon their generosity. The event is designed to showcase the tremendous impact caring individuals can have on their fellow man and encourages each person to reach for their full potential. The gala celebrates the achievements of those who are willing to work to make the world a better place, people like Dr. Robert Dean, who has been traveling the world with the Starkey Hearing Foundation to give children one of the greatest gifts of all – the gift of hearing.

This year the So The World May Hear gala will take place on July 20th, 2014. Among the humanitarians that will be honored are the Archbishop Desmond Tutu, Forrest Whitaker, Prince Seeiso of Lesotho and Sandi Young with performances by Sammy Hagar and John Legend. Dr. Robert Dean, the Florida-based humanitarian, physician, and journalist will also be in attendance at this year’s gala. The gala has even greater significance this year as it falls on co-founder Tani Austin’s birthday, and founder Bill Austin has committed to matching all donations, up to $100,000 that come in through Tani’s birthday on the 20th of July. This is a fantastic opportunity for people to contribute and have that contribution make an even greater impact.

The Starkey Hearing Foundation has become a global force to help people achieve their full potential. Believing that there is no such thing as a disability, only ability, the foundation has travelled the globe delivering people from a world filled with silence. To date the Starkey Hearing foundation has visited over one hundred countries, partnering with local and international organizations to bring their vision of hearing for everyone to the most remote parts of the world. Over the course of these travels the organization has delivered and equipped over 1.43 million people with hearing aids, opening up the world to people who otherwise were forced to live in complete silence. Countless lives have been changed by the organization and Dr. Robert Dean has felt privileged to participate with on the ground activities.

Despite its celebrity appeal, The Starkey Hearing Foundation has taken a realistic and community-based approach to hearing. As part of its model, the foundation works with local and regional leadership in the community and has been striving to empower local residents to take ownership of their own local hearing health services. The team then approaches each situation in a three-phase model that has worked effectively in over one hundred countries across the globe. First patients are identified, then the patients are fitted with custom made ear molds and taught how to care for and operate their hearing aids, and finally the foundation provides after care services to patients. The aftercare is an ongoing support system that remains in place at a central location, giving patients access to information, batteries, and repair services.

This July, the foundation once again hopes to highlight the contribution of those who make a difference in the world. Previous honorees have included President Bill Clinton, Sir Richard Branson, Ashton Kutcher, and Mohammed Ali. Dr. Robert Dean will be attending the event and this year’s gala promises to have a host of deserving people, who for a moment are given the spotlight of a grateful world.

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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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Fighting Against Hearing Loss in the Philippines and Throughout the World

13th Jun 2014 Haiti

The right to hear is basic to who we are as human beings. We were made to enjoy the song of the birds, communicate with others, and listen to the wind rustle through the trees. In the United States and other wealthy countries, we have been able to fight against hearing loss by using vaccines and other basic medicine. Unfortunately, there are millions of people throughout the world that never benefit from modern medicine. They are unable to have access to even the most basic vaccinations. They and their children suffer as a result, often leading to preventable hearing loss.

I traveled to the Philippines with the Starkey Hearing Foundation, on a mission to help improve the hearing of people in poorer communities. While there, I was greatly saddened to find so many people suffering from hearing loss that was completely preventable. One mother, for example, had a little boy that was born unable to hear because she had the measles while pregnant, something that could have been prevented with an MMR vaccine. On this trip, we were able to provide free hearing aids for over 5,000 children. Those children now have the gift of hearing and can rejoin society and their peers at school and other activities. Rather than growing up isolated, they can now enjoy the fullness of life and the sweet sounds that fill it.

I was so moved by this experience that I have continued to travel with the Starkey Hearing Foundation to developing countries around the world. While, in Haiti, I experienced another eye opening moment. We found that children and people with disabilities, hearing loss included, were labeled as worthless and tossed aside by society to die. These children, at no fault of their own, were cast aside and made to believe that they didn’t matter. Every child, every life matters, regardless of a disability and by providing them with hearing aids we were able to directly impact their lives and show that people with disabilities have value. It is amazing how a piece of medical technology, in this case a hearing aid, can turn a child’s life around and give them value again.

After our trip to Haiti, Hope 2 Haiti director Scott Bonnell issued a statement saying: “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.” I believe that it is because of local activists and partners we are able to make a difference. Organizations like Hope 2 Haiti are working with these poor and impoverished communities on a daily basis, paving the way for people like myself and the Starkey Hearing Foundation to come in and provide resources like hearing aids. Without their work it would be impossible for us to help so many.

I have been privileged to continue my work in countries throughout the world and will continue to do so. On each trip, I learn new things and my eyes continue to be opened to the challenges of people living without hearing. As a doctor, I am committed to making a difference in the lives of people at home and throughout the world because there is no difference between us. As people, we are all the same though some have been privileged enough to grow up in the world of vaccinations and preventative medical care while others, through no fault of their own, suffer in silence, cast aside by society. It is up to us, those that can, to stand up and help. I for one am happy to respond to that call and invite others to join me in doing the same. To learn more about the Starkey Hearing Foundation or my work 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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