Muscular Dystrophy: The need to fight this crippling disease

By Syed Akbar
Hyderabad: Nipun Loya was bubbling with energy and vigour till a few years ago. Today at 14, Nipun cannot stand, write or even wipe out the sweat from his face. And he could barely utter a few words.
Nipun is one of the 50 and odd muscular dystrophy patients who have gathered in the city from different parts of the country to dissolve their sorrow in a unique fun camp. Nipun no longer feels he is alone in suffering. He has found quite a few new friends from places as far away as Solan in Himachal Pradesh.
"Muscular Dystrophy patients do not generally move out of their homes. Most of them are caged in their living rooms for months together. An outing once in a while makes a big difference to them. The present fun-cum-picnic camp is programmed on the lines of "Jerry Kids" camps being held regularly by Hollywood comedian Jerry Lewis for MD patients", says Vipul Goel, himself a victim of the crippling genetic ailment. Vipul's brother Atul and sister Sanjana are also MD patients. Muscular Dystrophy is a common disease affecting mostly children progressively distroying their muscular system. Patients generally die before they attain the age of 25.
The city's fun camp is incidentally the first outdoor programme for MD patients in south India. With 52 patients in attendance, it is also the second biggest fun camp in the country after the Solan's camp held last year in which 92 patients participated. Points out Sanjana, president of Indian Association of Muscular Dystrophy, "no where in the world not even in the USA more than three dozen MD patients turn up for such programmes. Indeed it is an achievement".
The child patients participated in several fun events including infotainment competitions like word building, Antakshari, throw ball and lucky names. According to Sanjana and Vipul, chess player Venkatesh, who died from MD after his euthanasia plea was rejected by government, had inspired them to hold fun camps all over India. "We have made a beginning in Hyderabad and it will continue every year", they said.
Unmindful of what the cruel fate has in store for them, young MD patients spent time in sharing their dreams. "I want to be doctor", says 14-year-old G Srikrishna, who is good at sciences and social but not so good at mathematics. Srikrishna is now studying in 9th class and goes to school in his father's car.
Like Nipun, O Rahul and K Bharadwaja have stopped studying. "It is quite difficult to physically lift the child and put him in the classroom. He cannot maintain personal hygiene and so we have stopped sending him to school", says Nipun's father Narayan Loya. "He eats only one chapathi in the morning and one in the evening. He does not feel hungry. He even cannot change sides on the bed. We have to attend to his every need", he says.
The grown-up patients, however, want to spend the rest of their life in happiness. "We do not want to die early. We want to live and enjoy life to its fullest. We are against mercy killing. Why should one ask for it. Why the desperation" argues Vipul. Our picnic programmes are with a purpose and we want to bridge the gap between normal and disabled people, he adds.
According to Dr R Janardhan Rao, the association has an enrolment of about 3000 patients all over the country. Though the exact number of MD patients in the country is not known, it is believed that there are at least 3.5 lakh people affected by the disease.

Muscular Dystrophy – How To Tackle It?

By Syed Akbar
Muscular dystrophy is a crippling genetic disease. It continues to be an enigma to scientists and researchers all around the world. There is no cure as yet to muscular dystrophy. Clinically speaking, muscular dystrophy falls under the broad category of motor neuron disorders.
In patients affected with muscular dystrophy muscles stop synthesizing a particular type of protein called dystrophin. This protein is vital for all muscles to work perfectly. Unlike polio where disability stops soon after the attack, muscular dystrophy is a progressive disease crippling muscles throughout the life of the patient. Patients, if afflicted with the disease in early childhood, do not generally live beyond 25 years of age.
At present, there is no medicine to treat muscular dystrophy. It is advisable that those with the history of the disease in family should go for genetic counseling before the marriage. They may also prefer amniocentesis and go for abortion if the foetus carries the defective gene. This is the only way to avoid a muscular dystrophy patient from taking birth.
Muscular dystrophy may attack at any age. However, it generally attacks children at young age. The first symptoms of the disease include faulty gait and frequent falls. The calf muscles are bulged and the child cannot stand on its own.
The suffering caused by the disorder is considerable. First, it cause long-lasting disability, secondly there is no definitive treatment, and thirdly it may affect other family members because of its hereditary nature.
Untill a couple of decade ago virtually nothing was known about its pathogenesis. Now we know that it is a genetic disease.
In muscular dystrophy, the muscle fibres are gradually replaced by fatty tissue and the normal function of the muscle is impaired. The rate at which this happens, and therefore the rate at which weakness occurs, varies somewhat between children with the same condition. It is important to realize that some muscles are affected earlier than others, and this upsets the normal balance of strength between the muscle groups.
In children with muscular dystrophy the most frequently seen contractures occur at the ankles, knees and hips. These are partly caused by the child walking on his toes, with the knees at little bent and the feet apart, a position he adopts in order to balance in standing and walking as weakness of the hip. Knee and trunk muscles make it more difficult to keep his balance. The contractures are aggravated by the fact that children in latter stages of the condition spend more time sitting.
It is important to seek advice about physiotherapy and to start treatment aimed at preventing contractures as soon as possible after diagnosis, before there is any tightness. Do not wait untill there is an obvious deformity.
Increasing weakness makes tasks like walking and dressing more difficult but there are ways in which your child can be helped to make the most of his abilities and retain as much independence as possible, either causing distress or disrupting education or recreation.
Remember that worldwide search for a cure is on all the time, but when a therapy is found it will not make stiff or twisted joints move again. So this is another reason for trying to prevent deformities and keeping muscles supple and strong for as long as possible.
Exercises should be done regularly. It helps enormously if the exercises are done in an atmosphere of fun. They can be combined with singing, story telling and a general sense of enjoyment.
The child should not be stressed during exercises. Making the exercises into a game or having a chart of achievement might help towards a cooperative attitude.
It is important to keep a happy balance between encouragement and demand.
Praise and emphasise all the positives. Everything the child does successfully must be complimented and rewarded with a hug or on some other appropriate way.
Breathing exercises should be conducted for older children.
The child should be encouraged to attend school as long as possible. The teachers should be told about the problem in the child as their help is essential to provide the child greater and easier mobility in the school premises.

Swine flu: A deadly cocktail of Human Influenza A and HIV?

Syed Akbar
Hyderabad, June 17: With swine flu gradually turning deadly in many parts of the world, health experts and scientists fear that a mix of swine flu virus with human immunodeficiency virus (HIV) may lead to a cocktail of a severely potent viral strain that could wreak havoc.

The HIV-H1N1 co-infection risk is more in Andhra Pradesh as it has a large number of people living with HIV. The State has 5.4 lakh HIV positive cases, the highest number for any State in India. Since half of the 31 swine flu cases reported in the country are from Hyderabad and other parts of the State, any lax on the part
of health and quarantine authorities may spell a doom.

Since people with HIV have suppressed immunity, it takes quite a long period for swine flu virus to subside. The virus may develop into a potent viral strain by being a co-pathogen with the human immunodeficiency virus.

"People suffering from HIV, particularly those with low CD4 cell counts in their blood, are at higher risk of contacting swine flu. While swine flu may not develop pneumonia in ordinary people, chances are higher among people living with HIV," warns senior researcher Dr MN Khaja, who formed part of the team that
sequenced Hepatitis C virus.

Moreover, the swine flu or novel influenza A (H1N1) virus may emerge into a more potent viral strain in PLWH as in the case of tuberculosis bacteria. The TB bacteria has turned more potent in people with TB and HIV co-infection, causing resistance to available drugs.

"Though we do not have sufficient research data on H1N1 and HIV co-infection and how severe the human influenza virus would behave in people with low or compromised immunity, the World Health Organisation and the Centers for Disease Control and Prevention, USA, have preliminary data that suggests the severity of the problem. The virus may turn more lethal and a new strain may emerge in HIV hosts," Dr Khaja told this correspondent.

Health officials point out that adults and adolescents with HIV infection are known to be at higher risk for viral and bacterial lower respiratory tract infections and for different types of recurrent pneumonia. Evidence that influenza can be more severe for HIV-infected adults and adolescents comes from studies among HIV-infected persons who had seasonal influenza.

The novel influenza A virus has been found to be sensitive to the neuraminidase inhibitor anti viral medications zanamivir and oseltamivir. But whether it works in people living with HIV is not yet clear.

Indian cars will soon have a "star" rating on their fuel efficiency

Syed Akbar
Hyderabad, June 19: Indian cars will soon have a "star" rating on their fuel efficiency so that those intending to buy a four-wheeler will have the option of the selecting the best one in the market.

The Bureau of Energy Efficiency of the Union Ministry of Power has decided in principle to star rate the automobile brands based on their fuel efficiency and performance on road. To begin with, the star rating system will be implemented for cars. A vehicle rated "5 star" is the best in the market. The lower the number of stars the less the fuel efficiency of the vehicle.

Presently star rating is implemented for air conditioners and refrigerators on a pilot basis. Given the tremendous response the star rating system has generated from consumers, the BEE has decided to go in for more products from January 1 next year. They include fans, distribution transformers and tubelights.

"Air conditioners, fridges, fans, tubelights and distribution transformers will invariably have to get star rating from January 1, 2010. These products without star rating will be banned for sale in the country.
Ultimately we will bring 21 products including cell phone chargers, washing machines, computers and geysers," said Sandeep Garg, energy economist from the Bureau of Energy Efficiency, New Delhi.

The Central government plans to save about 10,000 mw of power by going in for star rating for electric and electronic consumer products. Star rating for card will help save consumption of fossil fuels.

Referring to high transmission and distribution losses in the country, Sandeep Garg said the Chief Ministers' meeting in New Delhi on January 23 would discuss the issue, including the need for star rated distributing transformers. Already five States including neighbouring Karnataka have made star rated transformers
mandatory for their power distribution companies and electricity boards.

Meteorite Grains Divulge Earth’s Cosmic Roots

The interstellar stuff that became incorporated into the planets and life on Earth has younger cosmic roots than theories predict, according to the University of Chicago postdoctoral scholar Philipp Heck and his international team of colleagues.

Heck and his colleagues examined 22 interstellar grains from the Murchison meteorite for their analysis. Dying sun-like stars flung the Murchison grains into space more than 4.5 billion years ago, before the birth of the solar system. Scientists know the grains formed outside the solar system because of their exotic composition.

“The concentration of neon, produced during cosmic-ray irradiation, allows us to determine the time a grain has spent in interstellar space,” Heck said. His team determined that 17 of the grains spent somewhere between three million and 200 million years in interstellar space, far less than the theoretical estimates of approximately 500 million years. Only three grains met interstellar duration expectations (two grains yielded no reliable age).

“The knowledge of this lifetime is essential for an improved understanding of interstellar processes, and to better constrain the timing of formation processes of the solar system,” Heck said. A period of intense star formation that preceded the sun’s birth may have produced large quantities of dust, thus accounting for the timing discrepancy, according to the research team.

Scientists Discover Magnetic Superatoms

A team of Virginia Commonwealth University scientists has discovered a ‘magnetic superatom’ – a stable cluster of atoms that can mimic different elements of the periodic table – that one day may be used to create molecular electronic devices for the next generation of faster computers with larger memory storage.

The newly discovered cluster, consisting of one vanadium and eight cesium atoms, acts like a tiny magnet that can mimic a single manganese atom in magnetic strength while preferentially allowing electrons of specific spin orientation to flow through the surrounding shell of cesium atoms. The findings appear online in the journal Nature Chemistry.

Through an elaborate series of theoretical studies, Shiv N. Khanna, Ph.D., professor in the VCU Department of Physics, together with VCU postdoctoral associates J. Ulises Reveles, A.C. Reber, and graduate student P. Clayborne, and collaborators at the Naval Research Laboratory in D.C., and the Harish-Chandra Research Institute in Allahabad, India, examined the electronic and magnetic properties of clusters having one vanadium atom surrounded by multiple cesium atoms.

They found that when the cluster had eight cesium atoms it acquired extra stability due to a filled electronic state. An atom is in a stable configuration when its outermost shell is full. Consequently, when an atom combines with other atoms, it tends to lose or gain valence electrons to acquire a stable configuration.

According to Khanna, the new cluster had a magnetic moment of five Bohr magnetons, which is more than twice the value for an iron atom in a solid iron magnet. A magnetic moment is a measure of the internal magnetism of the cluster. A manganese atom also has a similar magnetic moment and a closed electronic shell of more tightly bound electrons, and Khanna said that the new cluster could be regarded as a mimic of a manganese atom.

“An important objective of the discovery was to find what combination of atoms will lead to a species that is stable as we put multiple units together. The combination of magnetic and conducting attributes was also desirable. Cesium is a good conductor of electricity and hence the superatom combines the benefit of magnetic character along with ease of conduction through its outer skin,” Khanna said.

“A combination such as the one we have created here can lead to significant developments in the area of “molecular electronics,” a field where researchers study electric currents through small molecules. These molecular devices are expected to help make non-volatile data storage, denser integrated devices, higher data processing and other benefits,” he said.

Khanna and his team are conducting preliminary studies on molecules composed of two such superatoms and have made some promising observations that may have applications in spintronics. Spintronics is a process using electron spin to synthesize new devices for memory and data processing.

The researchers have also proposed that by combining gold and manganese, one can make other superatoms that have magnetic moment, but will not conduct electricity. These superatoms may have potential biomedical applications such as sensing, imaging and drug delivery.

This research was supported by the U.S. Department of the Army.

EDITOR’S NOTE: A copy of the study is available for reporters in PDF format by email request from the Nature Publishing Group press office by contacting press@nature.com.

About VCU and the VCU Medical Center: Virginia Commonwealth University is the largest university in Virginia with national and international rankings in sponsored research. Located on two downtown campuses in Richmond, VCU enrolls 32,000 students in 205 certificate and degree programs in the arts, sciences and humanities. Sixty-five of the programs are unique in Virginia, many of them crossing the disciplines of VCU’s 15 schools and one college. MCV Hospitals and the health sciences schools of Virginia Commonwealth University compose the VCU Medical Center, one of the nation’s leading academic medical centers