Asia ReView

Dr V.K. KHANNA

Thalassaemia major is an inherited blood disorder in which the affected children are not able to maintain haemoglobin (Hb) in the normal range (12-16 grams/dl). As a result, they require repeated blood transfusions to maintain Hb so that they can lead normal, active life. If left untreated, these children survive only up to 3-5 years.

It is believed that thalassaemia originated in the areas around the Mediterranean Sea and spread to different communities over time. In India, 3-17 per cent of our population is estimated to be thalassaemia carrier/minor. Every year 7,000-10,000 children with thalassaemia major are born in India. It is more prevalent in communities like Sindhis, Punjabis, Gujaratis, Marwardis, Bengalis, Saraswats and Gaurs.

kinds of Thalassaemia

Thalassaemia of two types: thalassaemia minor and thalassaemia major. Thalassaemia minors are people who carry one defective gene of thalassaemia.

They are normal except that during stressful situations like severe infection, surgery and pregnancy, they may have low haemoglobin.

Thalassaemia minor causes no problems per se. Many may also have generally low haemoglobin, which can be raised with folic acid intake.

Thalassaemia major is a serious blood disorder that causes severe anaemia and related complications. Patients have two defective genes of thalassaemia, one acquired from each thalassaemia minor parent. To survive, the affected children require repeated blood transfusions (every two-four weeks) for their whole life.

Inheritance

When one parent has thalassaemia minor, there is a 50 per cent chance of the birth of a thalassaemia minor child and 50 per cent chance of a normal child.

When both parents are thalassaemia minors or carriers, there is a 25 per cent chance of the birth of a thalassaemia major child, 25 per cent chance of a normal

child and 50 per cent chance of a thalassaemia minor child. Children born with thalassaemia major look normal at birth. They begin to look pale by the age of three-six months, become irritable and have feeding and appetite problems.

Their spleen and liver get enlarged. If not treated at this stage, infants start to develop facial changes in the form of prominent forehead, maxillary (cheekbo-nes) prominence, flat nasal bridge and protruding teeth.

Thalassaemia minor screening can be done by a simple blood test like complete blood counts (CBC). Further tests like HPLC /Hb electrophoresis can confirm the diagnosis.

Treatment of thalassaemia major includes repeated blood (packed cells) transfusions every 2-4 weeks for the whole life. The amount of blood and the frequency of transfusions increase with age.

As a result of repeated blood transfusions, thalassaemia patients develop iron overload. Unfortunately the human body has no natural mechanism of excreting the excess iron from the body, which gets deposited in and around the various vital organs of the body and ultimately causes organ failure. It is very important to remove the excess iron from the patient’s body by a process called iron chelation. This can be achieved by medicines only.

PREVENTION

Controlling the birth of more thalassemics is the only way to prevent the disorder. Public awareness, carrier detection and antenatal diagnosis are vital. Considering the high carrier rate in our country, the government needs to make testing for thalassaemia carrier status mandatory during pregnancy.

A simple blood test called Hb electrophoresis / Hb A2 will tell you whether you are a carrier or have a trait of thalassaemia minor. If tested on time, i.e., before starting a family, one can prevent the birth of a thalassaemia major child. During pregnancy, if the mother is found to be a thalassaemia minor, then the father is also tested. If two thalassaemia minors do plan a family, there is also a facility of testing the foetus.

Chorionic Villius Sampling (CVS) is done at 10-12 weeks of pregnancy. If the growing foetus is normal or thalassaemia carrier then the pregnancy is continued. If the test shows as thalassaemia major then termination of pregnancy is recommended.

Focus On A Cure

The only cure for thalassaemia major is a bone marrow transplant, which is extremely expensive and the success rate is also not very high. Research continues in the fields of cord blood transplant and gene therapy.

The writer is a senior consultant, Pediatrics Fortis Hospital, Noida

Dr Vinod Choudhary

The next time you find red patches on your baby’s body, blame the water. Patches may not always be indicative of skin infection, but may be caused by bathwater that makes the skin drier.

A new study conducted by Dr Victoria Dizon from the University of Santo Tomas Hospital, Manila, and Carlos Galzote from Asia-Pacific Skin Testing Centre, Metro Manila, Philippines, proves that soaps can have an itching effect on babies, but neither is bathing a baby with just water good enough.

The study evaluated the safety of a surfactant-based baby cleanser against other cleansers on 180 infants ageing one-day-old to a year old. Results showed that plain water was not capable of increasing skin moisture to the desired level. Moreover, it caused dilatation and congestion of blood capillaries on the skin, a condition called erythema/redness, after two weeks of usage. Neither is it capable of effectively removing fatty residues from baby’s skin (e.g. faecal matter, dirt, food, etc), and can actually be more drying than ultra-mild cleansers (antiseptic disinfectants).

Bathing babies with just water is thus not advisable. It is better to use a mild liquid disinfectant than a conventional soap as bathing babies with soap is slower and babies can loose substantial body heat in the meantime. Not all cleansers available in the market are good for babies. Try avoiding conventional soaps and opt for an ultra-mild cleanser that can be applied head to toe.

Dr Vyakarnam Nageshwar

Approximately 40 lakh passengers travel by air every month in India. Many are increasingly falling ill post air travel, causing serious concern on the number of infectious diseases associated with air travel. Outbreaks such as the one of SARS virus in the Far East in November 2002 have also been linked to aircraft transmission.

One of the primary risks is when the aircraft is delayed on the ground and the doors are closed. If the built-in ventilation system is not running, one faces the risk of breathing contaminated air. Outbreaks are facilitated when passengers remain inside the grounded aircraft for longer than 30 minutes without the ventilators functioning.

During flights, aircraft cabins have systems that control air exchanges. At cruising altitude, the ambient air outside is virtually free of microorganisms. Hence, most the aircraft built after the late 1980s re-circulate the cabin air. 10-50 per cent of the cabin air is filtered and mixed with the outside air, then introduced into the HEPA (High Efficiency Particulate Air) filters before reentering the passenger cabin. HEPA filters remove 99.99% of particles (bacteria, fungi and larger viruses). Outbreaks do occur in spite of these measures and cause great concern.

Illnesses associated with air travel are generally related to the changes in air pressure, humidity and oxygen concentration, and to relative immobility and close proximity to other passengers. Tuberculosis, influenza, plague, Pulmonary thrombo-embolism/ DVT, HANTA virus infections and viral haemorrhagic fever are some of the common respiratory illness that can spread during air travel.

Flying around 35,000 ft, the aircraft cabin pressure is equivalent to atmospheric pressure at approximately 1,500-2,500 metres above sea level, therefore within the pressurised cabin, the inspired oxygen pressure is lower than oxygen pressure at sea level that most healthy travellers do not notice. But passengers with cardio pulmonary disease, cerebro-vascular disease, anaemia and sickle cell disease can have a high risk of exacerbations of their underlying conditions.

A pulmonologist can conduct preflight evaluations of patients, which should preferably become a mandatory process for travellers suspected to have respiratory disorders. This can include a physical examination, routine blood test, Spirometry, chest X-rays and the like. A simple and effective measure of fitness to fly is whether or not you can walk 50-100 yards at a normal place, or climb one flight of stairs without becoming severely short of breath.

Seek medical help if you suffer from any of these symptoms post air travel:

Cough dry/productive

Shortness of breath

Fever on and off

Loss of appetite

Running noses/ sneezes/cold

Rashes/skin lesions

Nausea/vomiting

The writer is a consultant Pulmonologist Wockhardt Hospitals, Hyderabad

BY AMITA VERMA

LUCKNOW

Sept. 5: It was Teachers’ Day on Friday but not even a single pupil turned up to wish the four best-known "teachers" of the state.

The "teachers", on their part, were unconcerned and their day was packed with the usual politics, meetings, appointments — none of which had anything to do with academics.

Mulayam Singh Yadav, Mayawati, Kalyan Singh, Rita Bahuguna Joshi — all key players in present-day UP politics — have been teachers before they took to politics.

Though these teachers-turned-politicians have virtually severed all their links with previous career, they have left an indelible mark on their pupils.

Samajwadi president and former UP chief minister, Mulayam Singh Yadav, was a lecturer in Jain Inter College, Karhal, Mainpuri before he quit his job to enter politics.

"Masterji (Mulayam Singh Yadav) had strong likes and dislikes as a teacher. His favourite pupils were those who practised wrestling. However, he never had an air of arrogance and was always accessible to his students. As a teacher, he would constantly tell us that democracy was important for the survival of mankind," recalls Sarvesh Tiwari, one of his former students who now runs a flourishing business in Kanpur.

UP chief minister, Mayawati’s students, on the other hand, remember her as a teacher with sharp mood swings.

One of her pupils, who is now an office bearer in the Bahujan Samaj Party, told this correspondent on condition of anonymity that the "Behenji of today is vastly different from the Behenji of those days".

"As a teacher, she would laugh easily and lose her temper at the slightest provocation too. We used to be wary of her moods even then and none of us dared to talk to her unless she called out to us. However, she was always very dedicated and serious about her work even then and never got distracted by anything. Today, of course, she is not accessible to any one of us," says the former student.

BJP leader and former UP chief minister Kalyan Singh’s ex-pupils remember him as a stickler for principles. "More than the subject, he would insist on moral science. He always appreciated if we spoke the truth and his lectures were unique because of his oratory skills. He was knowledgeable and had an answer for every question," says Pramod Rajput, a past pupil of the BJP leader.

UPCC president Rita Bahuguna Joshi was a professor in Allahabad University and is, perhaps, the only politician who has maintained a contact with her past pupils.

"She speaks to us fondly if we approach her and is still a phone call away for her colleagues," says Veena Pathak, an ex-student.

BJP president Rajnath Singh and former Union minister Murli Manohar Joshi were also teachers in UP before they turned politicians.

The not-so-famous students of these famous politicians are, however, saddened at the fact that after rising to top positions in politics, their "teachers" have not done much to improve the standards of education in their state.

"In fact, they have made education poorer. Mulayam Singh has encouraged students to take to politics on the campus, Kalyan Singh has taught them the politics of religion and Mayawati is now teaching the politics of casteism," says Mr Tiwari.

By Sudhir K. Singh

BHOPAL

Sept. 5: As the state polls draw near, what is becoming increasingly clear to both the ruling BJP and the Congress leadership is that the choice of candidates will substantially determine who comes to power. While the BJP may have got off to an early start in the stumping, thanks to chief minister Shivraj Singh Chauhan’s ongoing Jan Ashirwaad Yatra, failure to drop at least 70-80 non-performing MLAs could cost the saffronists the election. Going by current prognostications, the winner is not expected to get a simple majority of more than 5-10 in a House of 230. Quite obviously, the BJP stands to lose much more in view of its brute two-third’s majority.

Sources in the both parties admit that gone are the days when the symbol or the influence of locally powerful satraps was enough to guarantee victory. The rebellious streak in both outfits has taken a sharp uptick over the years. Nomination of undeserving candidates arouses the ire of both voters and party workers who have no qualms in ensuring their defeat. Not many care a whit about the damage caused to the party. A classic example of public ire was when five BJP legislators whose seats fell within the Khargone LS segment were driven out of their constituencies during the campaigning for the bypoll last year. It was largely due to their incompetence and arrogance that the Congress went on to wrest the seat from the BJP.

Predictably enough, fixing the criteria for candidate selection is posing to be a severe headache for the state Congress. This was clear from the deliberations of the party’s election committee on Friday in which all the biggies (viz. Kamal Nath, Suresh Pachouri, Digvijay Singh, and Jyotiraditya Scindia) were present. AICC observer Narayan Samy took pains to clarify that a candidate’s "winnability" would be the sole yardstick in the ticket distribution. Party insiders argued that this ought to have been the criteria in any case, but the old "jagirdari" system (ie. rule of regional overlords) ensured that it was seldom put in practice. MPCC chief Suresh Pachouri said there was a demand that 40 per cent of the nominations go to new faces. Also broached was an upper age limit of 65-70 as in Rajasthan, exclusion of those who had lost twice in succession, or who were humbled by more than 15,000 votes.

With hordes of ticket seekers thronging the MPCC office, Mr Pachouri informed that confabulations were still on, and that a clear policy had still to emerge.