HEALTH TRIBUNE

Congenital heart disease is curable
by Dr Harinder Singh Bedi
C
ongenital heart defects are structural abnormalities in the heart present at birth. They result when a mishap occurs during the development of the heart soon after conception. Defects vary in severity from simple problems like holes in the heart to more severe abnormalities such as the complete absence of one or more chambers or valves.

Children with PIDs need philanthropists’ support
by Dr Surjit Singh
P
rimary immunodeficiency disorders belong to a group of rare diseases which affect the body’s immune system and render such patients susceptible to infections. Most of these disorders are there at the time of birth and the affected children need to be diagnosed in the first few months of life or else the patient would succumb to infections. Very few centres in India have the wherewithal to diagnose these conditions.

High blood pressure linked to depression
Washington:
A new study conducted by University of North Carolina reveals that high blood pressure is linked to depression, but only in people with a family history of hypertension. People whose parents did not have high blood pressure did not show the same association.



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Congenital heart disease is curable
by Dr Harinder Singh Bedi

Warning signs

At Birth

Difficulty in breathing

Blueness at birth or immediately after

Increased heart beat

A ‘thrill’ felt when a hand is placed on the baby’s chest

Between 2-6 months

Blue nails and toes

Spells of fainting or severe blueness

Difficulty in feeding

Difficulty in sucking

Difficulty in breathing during feeding

Sweating

Inadequate weight gain

Recurrent chest infections

First 3 years

Fainting spells

Abnormal heart beat

Congenital heart defects are structural abnormalities in the heart present at birth. They result when a mishap occurs during the development of the heart soon after conception. Defects vary in severity from simple problems like holes in the heart to more severe abnormalities such as the complete absence of one or more chambers or valves.

There is an alarmingly high incidence of heart defects from birth in India. The incidence is about 8 cases of congenital heart defects in every 1000 live births. Every year about 2 lakh children in India are born with a congenital heart defect, but only 5,000 get treated. Congenital heart disease accounts for 20 per cent of infant mortality. In half of these cases parents never get to know of the problem.

The exact cause of heart defects is not known. They may be due to a genetic abnormality associated with chromosomal disorders or with a condition called downs syndrome where, besides having a heart defect, the child is also mentally challenged. They could ‘also be due to an infection contracted by the mother during pregnancy or owing to drugs taken during pregnancy. It is thus imperative for the mother to be under medical supervision even for a “normal” pregnancy. Any drug taken during pregnancy should be only after medical consultation. Smoking and alcohol intake by the mother are seriously detrimental to the developing baby and should be strictly prohibited. If the above reasons are not there, the mother should not have a feeling that she did something wrong.

This writer has had on extensive training in the management of complex cardiac heart defects, including heart transplant at the Royal Alexandra Hospital for Children in Australia and has successfully implemented international-level cardiac care in Punjab — is of the view that if a child is born with a heart defect, the chances are better than ever before that the problem can be overcome and that a normal life will follow. Recent progress in diagnosis and surgery makes it possible to repair most defects in the heart, even those once thought to be incurable. As a rule, serious heart defects are diagnosed in infancy. However, some defects may remain undetected till the later stage.

Early treatment protects the heart from the adverse effects of volume loading or hypertension, and the body from hypoperfusion (low blood pressure) and the damaging effects to each organ system from cyanosis and polycythemia (blue blood). Repair during the early stage of the disease is expected to yield normal or near normal heart function. Early repair also decreases the potential for electrical instability of the conduction system of the heart.

Though every effort is made to detect and treat hear defects in infancy, a disturbing number of cases go undetected and the problem is still present in adulthood. A very easily curable defect can thus be converted to a high risk case due to the onset of pulmonary hypertension or may become irreversible due to a reversal of a previously left-to-right shunt. Quite often a technically good repair in an adult is not followed up by an expected recovery due to a poor myocardial function, rhythm disturbance or other organ failure. Structural alterations and long-standing damage to the lungs can make a previously curable disease inoperable.

An adult with a congenital heart defect is a rarity in the West. The author knows this from his long experience in the successful management of these difficult cases. Quite often cardiac surgeons from abroad look for guidance from experts in India as they have a much wider experience of handling this difficult-to-treat problem.

On the flip side, one comes across some cases where a murmur is detected by a doctor and the child is labelled as a heart disease patient. On detailed investigation, no structural defect may be found. The murmur is due to the turbulence of flow and has no significance — it is, therefore, called an “innocent” murmur. Such a child must be treated as an absolutely normal person as there is nothing wrong with his/her heart and it does not need “fixing”. In fact, the author has come across some cases of such murmurs being detected at a medical checkup before joining the armed forces. The cases are rejected on these grounds. On a detailed examination, no defect was found and the young man was cleared for the Army and is now a decorated Major.

In the case of a child with a heart defect, while waiting for surgery, rigorous activity should be avoided. The child should be protected from dental infection and sore throat as this infection may travel to the heart. Treatment modalities have to be tailored to individual patient needs. Improved perfusion methods, better surgical and anesthesia techniques, postoperative intensive care and early surgical intervention have made cardiac surgery in infants safe and predictable.

The writer is Executive Director and Chairman, Sigma Newlife Heart Institute, Ludhiana.
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Children with PIDs need philanthropists’ support
by Dr Surjit Singh

Primary immunodeficiency disorders (PIDs) belong to a group of rare diseases which affect the body’s immune system and render such patients susceptible to infections. Most of these disorders are there at the time of birth and the affected children need to be diagnosed in the first few months of life or else the patient would succumb to infections. Very few centres in India have the wherewithal to diagnose these conditions. The PGI, Chandigarh, has been on the forefront in this field for the last many decades. The Advanced Paediatric Centre had been running a weekly clinic where such children are being regularly followed up.

One of the common types of PIDs is known as Bruton’s disease which affects only boys. Such patients lack a vital set of proteins in their blood — the immunoglobulins. If these boys are left untreated, they rarely survive beyond the first one or two years of life because they are unable to fight infections. Fortunately, however, treatment is now available for such patients. This involves monthly injections of immunoglobulins via the intravenous route. Treatment is very expensive, but if the family can somehow afford to continue giving injections of immunoglobulins regularly, the results are very gratifying. Such children can then lead absolutely normal life. Injections of immunoglobulins have to be continued life-long, and the cost of therapy increases in proportion to the rise in the weight of the child.

Immunoglobulins are very expensive and cost approximately Rs 800 per gram. Children have to be given 5 grams of immunoglobulin for every 10 kg of weight. This turns out to be an expenditure of approximately Rs 50,000 Rs. 70,000 per year in the pre-school years and near Rs 1,00,000 per year in the school-aged children. Needless to say, none of the families that we have encountered can afford such expensive treatment. The result is that many of these babies soon die in front of our eyes from a disease that is eminently treatable. It is tragic for the parents to see their young son dying from a condition which can be so easily treated but for which they do not have the resources. If only our society could come together and help such parents in arranging resources, Bruton’s disease would no longer be a killer that it is today.

There are three families from Ludhiana, Nawanshahr and Ganganagar on our follow-up list who have decided to somehow garner resources for the continued treatment of their children. These families have a lower middle class background and are in no position to afford such expensive therapy. However, they seem to have extraordinary determination and will power and are somehow managing to make both ends meet, while at the same time arranging for treatment of their children. They have been helped by philanthropic organisations (sometimes anonymous donors) but at most times they are not in a position to know how and when the next life-giving immunoglobulin injection will be arranged.

Privileged people should come forward and adopt these children so that their parents’ burden can be shared. We need family trusts and other private trusts to support these unfortunate children whose very survival is dependent on immunoglobulin injections. It is understandable that the annual expenditure being what it is, no single individual may be in a position to support one child completely. However, if the logistics can be arranged, surely a group of individuals (say a market committee, social clubs) can collectively adopt one such affected family and support it.

The writer is Additional Professor of Paediatric Allergy & Immunology, PGI, Chandigarh.
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High blood pressure linked to depression

Washington: A new study conducted by University of North Carolina reveals that high blood pressure is linked to depression, but only in people with a family history of hypertension. People whose parents did not have high blood pressure did not show the same association.

The roots of the relationship between high blood pressure and depression lie in a mix of genetics and social factors with the family, says Karen M. Grewen, Ph.D., of the department of psychiatry at the University of North Carolina at Chapel Hill.

“The impact of parental hypertension on future risk is most likely the result of shared genes, learned behaviors, shared environments, or various combinations.” Grewen said.

Hypertension is a major risk factor not only for heart disease but also for stroke and kidney disease. Doctors have known for some time that depression may presage heart-related disease and death in both healthy people and those who have had heart attacks. But the connection of depressive symptoms to parents’ blood pressure status had not been documented. — ANI

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