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Chikungunya in Florida

15th Dec 2014 Uncategorized

Chikungunya is a viral disease that is transmitted from the bite of an infected mosquito. To date, two specific species have been identified, which include Aedes aegypti and Aedes albopictus. Chikungunya is classified as an alpha virus with positive single-stranded RNA genome. The disease was first described by M. Robinson and W.H.R. Lumsden following an outbreak that occurred in Tanzania in 1952. Since that time, outbreaks have continued to occur in Africa, Europe, parts of Asia, and more recently in the Caribbean. The Pan American Health Organization has reported over 900,000 suspected cases in 40 countries over the last 11 months. From a clinical perspective, Chikungunya has an incubation period of two to twelve days, with the majority of cases occurring between 3 and 7 days after being bitten by an infected mosquito. A fever of 102 degrees (F) and joint pain are the characteristic symptoms. Rash, fatigue, joint stiffness, headache, and nausea/vomiting may also occur. While a majority of patients do feel significantly better within a week of onset, joint pain (arthralgia) may persist for several years following infection. Schilte C. et al (2013) followed 180 patients in 2006 from an outbreak on La Reunion Island and concluded that over 60% continued to experience symptoms of arthralgia 3 years after infection. Young children, elderly adults, pregnant women, and those with pre-existing disease are at greatest risk. The disease has caused mortality in a relatively small number of cases so far.

The disease has been reported in the US since 2006, stemming from travellers who had been to countries where the virus is common. The disease has spread rapidly throughout the Caribbean and parts of Central America. In late June 2014, the first two locally acquired cases were identified in south Florida. Neither infected patient had travelled outside of the U.S. The virus is not airborne or able to be transmitted from person to person. Mosquitos are the primary vector and transmit the disease by biting a healthy person. Following infection, immunity is incurred as protective antibodies have been developed. Unfortunately, the spread of the disease can occur rapidly as it is a new virus to the continental U.S. leaving most of the population susceptible. To date, 11 locally acquired cases have been reported in Florida. Counties affected include Miami-Dade, Broward, Palm Beach, and St. Lucie (http://www.cdc.gov/chikungunya).

Primary diagnosis is made based off of clinical symptoms, travel history, place of residence, and exposures. Confirmation of disease by laboratory testing through serum or plasma can occur with detection of virus, viral RNA or viral antibodies. The CDC and some state health departments perform viral testing.

At present, there are no vaccines or specific anti-viral treatments for Chikungunya infection. Treatment focuses around supportive care including rest, hydration therapy, and non-steroidal anti-inflammatories (NSAIDs) to relieve pain and fever.

There is concern that Chikungunya could affect more than 10,000 people in Florida alone. This projection, from the Florida Medical Entomology Laboratory, is based off statistics from other outbreaks.

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The ‘Kissing Bug’ Disease: What You Need To Know

17th Nov 2014 Diseases, Medical News

Chagas disease, also known as the ‘kissing bug’ disease, is an infection that is affecting more and more Americans every year. Since this disease has primarily been found in places like Mexico as well as South and Central America, many people in the United States, including physicians, are unfamiliar with it. However, the last few years have seen cases turning up in the United States. Most people are believed to have been infected abroad, but recently more of these infections have been contracted locally.

The kissing bug (also known as the triatomine bug, reduviid bug, assassin bug, cone-nosed bug, or blood sucker) includes many different species that can all carry the parasite Trypanosoma cruzi that causes Chagas disease. These insects can be found both indoors and outdoors, particularly in the cracks and holes found in substandard housing. They are found across the southern two-thirds of the United States, but the parasite is generally found in the triatomine bugs from Latin America. There are other bugs that resemble this insect (like the assassin bug or the wheel bug), so if one is found it can be taken to an entomologist for positive identification.

Fortunately, contracting Chagas disease from kissing bugs is rather difficult – an individual needs to contact the insect’s feces through a wound (like the bite wound that the insect makes, often on the face) or a mucous membrane (like the mouth or the eyes.) Some people are allergic to the insect’s saliva, but this does not mean that they have contracted the disease. Signs of an allergic reaction include redness, itching, swelling, and welts or hives. In rarer cases this reaction can cause anaphylactic shock.

In the event that someone does contract Chagas disease there may be a mild swelling at the site where the parasite entered the body – this is known as a chagoma. Romaña’s sign is swelling around the eye if the eye was the parasite’s inoculation point. The chagoma or Romaña’s sign both last longer than an allergic reaction and are not nearly as itchy. Other than the potential swelling and fever, there are very few early signs for Chagas disease. In rare cases there may be severe inflammation around the heart muscle, brain, and lining of the brain.

If a person remains untreated they can enter a chronic phase of disease where very few or no parasites are found in their blood, making diagnosis difficult. Many people will remain symptom free and never know they have the disease, but 20-30% can develop symptoms. These symptoms include heart rhythm abnormalities that can cause sudden death, a dilated heart that doesn’t pump well, and a dilated esophagus or colon that makes it difficult to eat or to pass stool.

Chagas disease can be spread through blood transfusions, organ transplants, and congenitally from mother to child. Many people have been notified that they test positive for the parasite after donating blood, despite not being aware that they had it. Two drugs can be used to treat the disease if it is caught early enough – nifurtimox and benznidazole. Due to the more “exotic” nature of this disease many physicians aren’t familiar with it. If you are concerned that you may have been exposed to or contracted Chagas disease you can talk to your physician or seek one familiar with the disease here: http://www.astmh.org/source/ClinicalDirectory/

 

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Dr. Susan Perlman and Stephanie Magness on American Health Journal Nov 7

11th Nov 2014 Diseases, Friedreich's Ataxia, Hearing, Medical News

Dr. Susan Perlman appeared on American Health Journal this Friday to discuss Friedreich’s Ataxia (FA) along with one of her patients, Stephanie Magness. They educated viewers on the clinical aspects of the disease as well as daily life and clinical studies.

Dr. Perlman is a professor in the Department of Neurology at the David Geffen School of Medicine at UCLA and physician specializing in ataxia, Huntington’s Disease, and neurogenetics. She is the Director of the Ataxia and Neurogenetics Program and the Post-Polio Program at UCLA and has been a primary investigator for many Friedreich’s Ataxia trials over the years.

In this program, Dr. Perlman reviews some of the basic facts of the disease – it is a recessive neurogenetic disorder caused by mutations in both copies of the gene that controls the production of frataxin, it is a disease of childhood, and the symptoms start with balance and coordination difficulties. It begins in the legs and progresses to the hands, speech, vision, and even hearing. The patients may also develop cardiac symptoms, which include hypertrophic cardiomyopathy, heart rhythm problems, and even heart failure. Diabetes is another potential symptom and can cause affected individuals to require insulin.

Stephanie Magness is currently one of Dr. Perlman’s patients and, like all too many people with FA, she was not diagnosed correctly for the first few years of her symptoms. Friedreich’s Ataxia is often misdiagnosed as another recessive childhood neurogenetic disease like Charcot-Marie-Tooth disease, cerebral palsy, and idiopathic peripheral neuropathy. She was eventually diagnosed correctly via gene testing and is now able to manage her symptoms accordingly. Stephanie has also taken part in a natural history study as well as a clinical drug trial – possibly the first approved drug for FA.

According to Stephanie one of the most difficult aspects of the disease is its progressive nature. Even if a patient is able to handle their symptoms well currently they know that it may all change. Her advice was to focus on what you can do instead of things that you obviously cannot and find new things to do that you can excel in.

Now that drug companies have begun to take an interest in Friedreich’s Ataxia and to see it as an important area scientifically there is hope that a drug will be approved to treat FA in the not-too-distant future. Watch the program on Vimeo here: http://vimeo.com/110841193 (the section about Friedreich’s Ataxia starts at 15:39)

 

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Hearing Loss and Veterans

05th Nov 2014 Hearing, Medical News

One of the most common injuries to war veterans is hearing damage. 60% of soldiers returning from warzones experience some version of hearing damage – either hearing loss, tinnitus, or both. This accounts for around 414,000 US veterans from Iraq and Afghanistan to date. Most people are concerned with loss of limbs, post-traumatic stress disorder, and brain injuries while less attention is brought to hearing loss. Despite its prevalence, hearing damage is often overlooked since it is generally does not cause loss of life.

The two main reasons for hearing damage from war are short-term exposure from high-intensity noise and long-term exposure to loud ambient noise. This can involve everything from loud trucks and helicopters to machine guns, artillery fire, and blasts from explosives. The prevalence and intensity of damaging wartime noises has increased over time, particularly with the use of improvised explosive devices (IEDs), which has resulted in more hearing damage for soldiers than in the past. Hearing protection is provided by the military, but many soldiers will forgo using it in order to listen carefully for signs of danger. In addition – if soldiers find themselves in a combat situation suddenly, there is no time or good way for them to grab hearing protection.

One of the problems with diagnosing hearing loss is that many veterans don’t seek medical attention when they first start to notice problems. Military culture can be partially to blame since hearing loss is so prevalent. Hearing loss has often been seen as a necessary evil or even an honorable sign that a soldier has seen action. However, this delay in treatment is not unique to veterans. Many individuals try to live with their hearing loss, despite the fact that damage to hearing cannot be reversed. On average it takes 7 years for someone to talk to a doctor after they first start noticing changes in their hearing.

Hearing loss is costly for the U.S. Department of Veterans Affairs with around $2 billion in benefits dispersed annually. The VA purchases one in every five hearing aids sold in the United States and 25-30% of all VA disability claims involve hearing. There is a push to get more funding for research in hopes that they can achieve the same innovations that the prosthetic limb field has seen. All veterans are encouraged to have their hearing evaluated upon discharge and medical attention should be sought as soon as hearing loss is noticed.

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Saint Louis Encephalitis

31st Oct 2014 Diseases, Medical News, Viruses

Over the summer there have been mentions of Saint Louis encephalitis virus (SLEV) in regards to Pinellas County. This virus is a flavivirus – a group that includes West Nile – and has the potential to cause severe illness or death in older adults. The name of this disease comes from a particularly extensive outbreak that occurred in St. Louis, Missouri and the surrounding St. Louis County in 1933. Due to the high number of cases NIH was able to investigate and isolate this previously unknown virus. It is thought to have originated in northern Mexico and have been carried north by birds. Pinellas County Mosquito Control uses sentinel chickens to monitor for Saint Louis encephalitis and other arboviruses, since birds are usually the source of infection for mosquitos. Blood is drawn from the chickens weekly and tested for antibodies to the viruses of concern. Fortunately for the chickens they do not develop any symptoms, although once they test positive they have to be removed from the program since they will always carry the antibodies. Sentinel chickens from Pinellas County have tested positive for Saint Louis encephalitis in late July as well as August and into September. Regions with confirmed SLEV include Cross Bayou, Walsingham Park, Sawgrass Lake Park, and Lake Maggiore. While it is more common during hot and humid months, Saint Louis encephalitis can occur year round. Most individuals that are infected have no symptoms, but in rare cases it can manifest with such signs as fever, headache, nausea, vomiting, and fatigue. Severe infections can result in high fever, neck stiffness, disorientation, confusion, tremors, coma, and sometimes convulsion, spastic paralysis, and death. Older adults are at a greater risk for fatal disease. Since there is no cure for Saint Louis encephalitis the only treatment available is supportive care. If you or anyone you know shows symptoms of this disease be sure to talk to your health care provider for proper diagnosis. Since this virus is spread by mosquitos you should take the appropriate precautions. Wear insect repellent and protective clothing and be sure to avoid areas with high mosquito populations. Drain standing water, change pets’ water dishes and birdbaths regularly, and maintain your pool’s water balance to prevent mosquitos from breeding. If you use rain barrels be sure to cover them with fine mesh. Ornamental ponds can be stocked with fish that eat the mosquito larvae and any ornamental bromeliads should be flushed with fresh water regularly and treated with a larvicide. Mosquito Control field technicians can respond to concerns by use of the Mosquito Control Request form found on the Pinellas County website. http://www.pinellascounty.org/forms/mosquito_form.htm

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Starkey Hearing and Doctor Robert Dean Afghanistan Mission

22nd Oct 2014 Hearing, Medical Mission Trip, Starkey Hearing Foundation

There are many civilians living and working in war zones and, despite not being military personnel, they are exposed to many of the same hazards. There has been significant research done into the effects of war on soldiers, but very little has been done for those not enlisted. Recently it was reported that the number of children and other vulnerable Afghan civilians injured or killed has increased dramatically since the beginning of 2014. There has been a rise in the number of ground engagements in areas with higher civilian populations, which has accounted fro this steep increase in causalities. While civilians can be accidentally injured or killed during war, there has been a disturbing trend of attacks directed toward them – particularly tribal elders or government officials in this case. The number of children and women causalities has increased the most as the fighting moves into areas with higher population density.

Afghan civilians are exposed to everything from gunfire, IED attacks, mortars, and grenades, to loud noises from trucks, helicopters, and aircrafts.  All of these sounds can damage hearing and lead to tinnitus or permanent hearing loss on top of any other injuries.

Hearing damage can occur in one of two ways – long-term exposure to a loud environment (driving a loud truck,) short-term exposure to a high-intensity sound (gunfire or IEDs.) Exposure to loud noise causes destruction of the hair cells in the inner ear, but the degree of destruction is based on the intensity and duration of the noise. These hair cells are responsible for carrying sound vibration information to the auditory nerves, so without them available to convey this information the individuals experience hearing loss.

Hearing loss is irreversible, but there are methods to cope. Hearing aids are the most recommended method to restore some, if not nearly all, hearing to an individual. Starkey Hearing Foundation has been working with the Bayat Foundation  in order to distribute hearing aids to Afghan civilians, many of them children affected by war, who would otherwise be left unable to hear.  We were able to fit over 600 people with hearing aids while on our mission to Afghanistan.

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Beware of Poisonous Caterpillar in Florida

25th Sep 2014 Diseases, Medical News

Recently there have been countless news stories about a spectacular caterpillar – the puss caterpillar. Children from Florida to Virginia have been stung and the excruciating pain has been landing them in the hospital. Some of these children had actively sought out to pet the caterpillar due to its “cute” fuzzy appearance. In fact, the caterpillar gets its name due to the fact that it resembles a “fluffy house cat” or even a “tiny Persian cat.” Other children came into contact when the insect fell from a tree and landed on them.

These caterpillars are generally an inch long at the largest and are covered in a thick coat of fur that can range from greyish-white to orangey-brown to charcoal. In addition to the fur their body tapers at the end, which can give it the appearance of having a tail. The fluffy hairs that cover the puss caterpillar hide a series of tiny, venomous spines that can stick in your skin.

The feeling is said to be similar to a bee sting, except worse, and immediately starts increasing to the point where it can even “make your bones hurt.” The degree of pain depends on where you’re stung, but it has been reported to take as many as 12 hours to begin to subside. On top of the intense pain, contact with the caterpillar’s bristles can cause fever, vomiting, convulsions, rashes, swelling, abdominal distress, blisters, and even chest pain, numbness, and difficulty breathing. However, each individual reacts to a different extent – some require medical treatment and other report only a mild itching or burning.

It may seem like more and more people are being stung recently, which is partly true. Like many other insect species, the puss caterpillar populations go through a “boom-and-bust” cycle. This means that the population can increase or decrease quite dramatically as environmental conditions change. Right now these conditions appear to be ideal for the caterpillars so the population has been on the rise.

The Poison Center of Tampa recommends that you use cellophane tape over the affected area and remove repeatedly to strip the spines from your skin. In addition, you can apply ice packs to reduce the stinging sensation and follow it up with applying a paste made of baking soda and water. Oral antihistamines, calamine lotion, hydrocortisone cream, and juice from the stems of comfrey plants can also be used to treat symptoms. It is recommended to watch for the caterpillars around trees like oak, elm, and citrus. They even tend to be found around common garden plants like rose bushes or ivy. Additionally, teach children what they look like and why they need to avoid touching them since the puss caterpillar’s fluff-ball appearance can be quite appealing.

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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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