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| Sunday, 9 May 2004 |
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Features |  |
| News Business Features Editorial
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Compiled by Carol Aloysius International Thalassaemia Day was on May 8 Thalassaemia - a genetically inherited disease Dr. Shantimala de Silva, Consultant paediatrician LRH, answers questions on a killer blood disease in an interview with Carol Aloysius Q: What is Thalassaemia?
The disease is usually severe in these patients and need regular blood transfusion throughout their lives in order to live. (Thalassaemia Major). Q: What are the clinical signs of Thalassaemia? A: They do not grow as well as they should Due to the break down of red cells, they appear to be pale and the eyes tend to look yellowish. These patients also have excess iron in the body and it get stored in the vital organs like the spleen, liver, heart and pancreas causing damage to these organs. Patients' abdomen gets enlarged due to the enlargement of liver and spleen. Q: Is it found only in Sri Lanka? A: No Q: Can Thalassaemia be treated? A: Yes. Q: How? A: With monthly blood transfusion for the rest of their lives. By giving daily subcutaneous chelation therapy And by giving folic acid supplements daily Bone Marrow transplants is a permanent cure for some patients, but it is very expensive. Q: What is Thalassaemia Minor? A: It is not a disease state, i.e. patients have no symptoms The person is only a carrying the disease Thalassaemia. The person is carrying one abnormal gene in the red cell. It is also called Thalassaemia trait. The person appears to be normal, both physically and mentally It could only be detected by blood tests The haemoglobin level will be slightly low 9-10 gm/dl. (normal 11.14 gm/dl) and the HbA2 will be high No treatment is required for this type of person Q: How is it inherited? A: Through genes A person with two normal genes for normal red blood cell. A person with one normal and one abnormal gene is called a Thalassaemia carrier having a Thalassaemia trait. Just as the character of a person is represented by each gene coming from the patient, to form a normal red cell you need to have one good red cell from each parent. A person with one abnormal gene is called a carrier or Thalassaemia minor. When both genes are abnormal he is called a Thalassaemia major. When two carriers get married the results you have a 25 per cent chance of having a normal child, a 25 per cent chance of having a Thalassaemic child and 50 per cent chance of being a carrier. Q: Can Thalassaemia be prevented? A: Yes. Q: How? A: a) It could be prevented if a Thalassaemia carrier avoids marrying a Thalassaemia Carrier. b) Ante natal diagnosis and genetic counselling which is not available here. Q: At what age is Thalassaemia detected in a child? A: Around 5-6 months. Q: What are the symptoms? A: The child becomes pale. Has difficulty in feeding. Has a distended stomach and is lethargic. This is the time most parents bring their child for examination. Q: What do you do then? A: We give a physical examination to see if the spleen is enlarged and then do a haemoglobin test, a blood picture and a haemoglobin electrophoresis. Once diagnosed as Thalassaemic we give the child a blood transfusion and advise the mother to bring the child for blood transfusion every month. Q: How can you help a Thalassaemic Patient? A: By donating blood and by contributing to purchasing an infusion pump needed for chelation medicine. Q: How could you help to reduce the Thalassaemia population? A: Check your blood to detect if you are a Thalassaemia carrier. If you are a carrier avoid marrying a person with Thalassaemia carrier state. Q: How could a person with Thalassaemia Major live? A: A well treated Thalassaemic could lead a near normal life. They can grow up, complete their education, work and perhaps have a family. It is true, that Thalassaemics live with more risks than others, because of the amount of medical treatment they require. But even so, a well treated Thalassaemic at the present day could have an excellent life expectancy. Even enjoying a life span over 70 years. **** A special message from the Minister of Health Care, Nutrition and Uva Wellassa Development, Nimal Siripala de Silva. Some facts about Thalassaemia * Thalassaemia is a disease that is genetically inherited. * It is not contagious * It can lead to early death if not treated * In Sri Lanka 3 per cent of patients (about 2000 children) suffer from Thalassaemia. But around 16 new patients are being found every year. The highest number of patients are in Anuradhapura and Kurunegala districts. * Since Sri Lanka lacks anti-natal pre-testing facilities to detect Thalassaemia in a foetus, and abortion is illegal, prevention is the only way to minimise the number of patients. This can only be done if couples get themselves examined for Thalassaemia traits before marriage. * Raising awareness and education on Thalassaemia will be a high priority in all health labour programmes in the future. * Volunteers who can be trained in raising awareness on this disease are urgently needed. * The Health Ministry, hopes to enlist the co-operation of NGO's and Religious leaders for this purpose. **** Management of Thalassaemia Major General - Provide psychological support. Advise to drink tea with meals and to avoid iron-rich foods. Blood Transfusion - Start if Hb - 8.0g/dL for 1 week, or if impaired growth, enlarged spleen or malformed bones are seen. Immunise against hepatitis B and perform blood typing before first transfusion. Maintain mean Hb at 12.0-12.5g/dL. Transfuse at 4-6 week intervals (1-2 weeks in special cases). Iron Chelation with Desferrioxamine - Start when serum ferritin - 1,000 ng/mL. Maintain level - 2,000 ng/mL. Daily subcutaneous dose: 20-60 mg/kg (3-7 times weekly over 8-10 hours) High doses for a short period only! Intravenous dose at time of blood transfusion: 50 mg/kg over 24 hours. On days of treatment give up to 200 mg vitamin C depending on age. (See page 15, Thalassaemia, guide for doctors and medical staff). Splenectomy - Indications: increased blood requirement, enlarged spleen, leukopaenia, thrombocytopenia. Immunise against pneumococci, meningococci and haemophilus influenza 4-6 weeks before operation. After operation give prophylactic antibiotics for at least 2 years. Monitoring - Keep accurate records of all treatment. Record height, weight every 3 months. Measure serum ferritin, calcium, phosphorus, blood sugar, liver and thyroid function every 6 months. Evaluate progress annually and investigate if unsatisfactory. N.B.: Thalassaemia trait carriers (heterozygotes) with iron deficiency may have a blood film which is similar to thalassaemia. The differential diagnosis here is that in trait carriers both HbF and serum iron are low. ###### The Pacemakers (pacesetters) by Dr. D.P. Atukorale One of the most marvellous of the controls in the human body, is the mechanism that governs the actual control of the heart muscle, the heart itself. This and the act of breathing are the only two bodily functions that cannot be interrupted for long, if life is to continue.
Heart block Disturbances can arise in any part of the conduction system of the heart. The disturbance may be in the sinus node or during its passage down the conduction system to the ventricle. As a result of certain diseases affecting this conduction system, there is slowing of the heart and the heart may beat at the rate of 30 or 40 per minute causing dizzy spells or blackouts. This slowing of the heart beat is called heart block. If the heart block persists for more than 15 seconds the brain in starved of blood and the patient loses consciousness. If the block persists i.e. if the heart does not beat for more than 5 minutes, irreparable brain damage occurs and the patient may get resistant convulsions and dies following ventricular fibrillation or cardiac asystole (cardiac arrest). Majority of patients get only short attacks of heart block lasting for a few seconds, resulting in dizzy spells or giddiness. It should be noted that heart block is a rare cause of giddiness, one common cause of giddiness being constriction of blood vessels to the brain. Pacemakers When the heart beats too slowly to meet the body's demands, the heart rate can be speeded up using pacemakers or drugs. In patients with acute myocardial infarction (heart attack) complicated by heart block, the doctors first try drugs such as intravenous atropine, to speed up the heart, failing which, patient is connected to an external pacemaker in the coronary care unit. An electrode inserted into the right ventricucle (right sided pumping chamber) of the heart is connected to the external pacemaker box and the heart rate can be controlled by turning a knob in the pacemaker box. These are called temporary pacemakers and once the patient's conducting system comes back to normal, pacemaker can be switched off and the electrode pulled out from the right ventricle. Lives of over one thousand patients with heart attacks complicated by heart block have been saved in Coronary Care Unit, Institute of Cardiology, National Hospital, Colombo by means of temporary pacemakers. Another common condition where pacemakers have been used to save life is "Kaneru" poisoning in which digitalis like substance in "Kaneru" cause heart block. Permanent pacemakers Permanent pacemakers are used in a condition called chronic heart block which usually affects elderly people. In these patients the damage to the conduction system is permanent and they present to the doctor with recurrent syncopal attacks or dizzy spells. Although drugs such as long acting Isoprenaline can be used on these patients, doctors prefer to use permanent pacemakers which can transform and prolong their lives drugs used for heart block. There are 2 parts to a pacemaker, the box which is of the size of a match box that generates the electrical pulse and the electrode or the wire that conducts the impulse to the heart. The pacemaker box contains 2 Lithium batteries, an electrical circuit and a plug which takes one or two leads to the heart. The whole system is implanted by a Cardiologist. The operation is performed under local anaesthesia. The cardiologist positions the electrode or the wire in the right ventricle and connects this to the box which is buried under the skin of the chest wall. It takes about 30 minutes for the whole operation. Permanent pacemakers are very expensive and each pacemaker implantation costs about Rs. 100,000 to the Health Ministry. In Colombo Institute of Cardiology more than 200 permanent pacemakers are implanted every year. In the private sector a pacemaker implantation casts about Rs. 200,000. The type of pacemakers used nowadays are called demand pacemakers and these fire only when the heart misses a beat and do not come into action when the heart generates its own beats. Majority of the pacemakers used nowadays are programmable pacemakers. Using an external electrical gadget called programmer, the doctor can change the heart rate long after the pacemaker operation. There are more complicated and sophisticated pacemakers called dual chamber pacemakers where 2 electrodes are used, one in the right atrium and the other in the right ventricle. These are used in a special type of heart block called sick sinus syndrome. Rate responsive pacemakers are able to increase the rate of discharge in response to the needs of the body e.g. by sensing the rate of breathing. Activities such as walking and running are sensed by a crystal incorporated into the pacemaker and sometimes these are over-sensitive and may stimulate the heart to beat too fast if you travel in a private bus or a three wheeler. Anti-Tachycardia pacemakers (Automatic implantable defibrillators) These are special pacemakers which help patients who are prone to life threatening palpitations such as ventricular tachycardia and ventricular fibrillation. The pacemaker can detect abnormal fast rhythms and can deliver a shock restoring the normal rhythm. Several thousands of these are used in U.K. and U.S.A. and each pacemaker costs about 1.5 million rupees. Life with a pacemaker After pacemaker implantation a person is fit to live a normal life as in majority of these patients the coronaries and the heart muscle are normal. One is able to drive vehicles one month after discharge from hospital. One of the patients known to me climbed Adam's peak two months after pacemaker implantation. The programmable pacemakers implanted in the Institute of Cardiology are guaranteed for 8-10 years. If the batteries show signs of failure, batteries are replaced. Patients have to attend the pacemaker clinic once in six months to test the pacemaker and impending pacemaker failure can be detected by the pacemaker analyzer. Pacemaker patients are advised to avoid certain types of electrical apparatus such as microwave ovens and electrical shavers electrical cauterization should not be used during surgical operations in case of pacemaker patients. Certain detectors used in public libraries, supermarkets abroad and airports can detect the lithium battery and you may be accused of shoplifting or carrying an explosive as you are about to board an aircraft. Therefore always carry your pacemaker card wherever you go, especially, when you go to the airport to board an aircraft. Pacemaker patients should lead a fit and normal life indulging in all the physical activities they enjoy, including sexual activity. The function of the pacemaker should be monitored regularly though infrequently (perhaps once in six months) at the special outpatient pacemaker clinic where sophisticated equipment is used to measure pacemaker function such as its rate, very accurately. ###### Effects of fluoride on teeth by Dr. Srilal A. Silva The inter-relationship between fluorides and the enamel of the teeth was discovered in the twentieth century. Fluorine in its ionic form is a strong inhibitor of dental caries but with higher doses a disturbance of enamel formation called mottling or dental fluorosis occurs. Tooth decay or dental caries begins when the outer surface of the tooth is attacked by acid. The bacteria which live on the surfaces of the tooth as a layer (called the plaque) produce the acid. When any food or drink containing sugar enters the mouth these bacteria rapidly convert these sugars into acids. The plaque holds this acid in contact with the tooth surface for about 2 hours before the saliva neutralises the acid. During this 2-hour period, some of the calcium and phosphate minerals (which are the main compositions of the enamel), are dissolved out of the enamel and into the plaque. This process is called de-mineralization. Once the plaque acid is neutralised, the minerals can return to the tooth surface. This process, called re-mineralisation, has a limited capability and not all of the dissolved minerals are returned back to the tooth. If sugars enter the mouth too frequently there is a permanent loss of enamel resulting in a cavity through which bacteria can penetrate and destroy the inner structure of the tooth. Fluoride arrests tooth decay in three ways: I. It alters the structure of developing enamel in children up to 7 years of age making the surface of the enamel more resistant to acid attack. II. During the periods of alternating de-mineralisation and re-mineralisation, the presence of low levels of fluorine in the saliva and plaque encourages re-mineralisation and improves the quality of the enamel crystals that are laid down. III. The fluoride on the tooth surface inhibits the plaque bacteria from producing certain enzymes which are essential for the bacteria to produce acid. The best and most cost-effective method of supplying fluoride is by the fluoridation of the public water supply. The optimum fluoride content of the water should be between 0.9 and 1.2 parts per million. Another way of delivering fluoride to the teeth is by the use of toothpaste containing fluoride. In countries where water fluoridation is not available other more costly methods are used such as topical applications with fluoride gels. Harmful effects Excessive ingestion of fluorides causes mottling of teeth. Hence, fluoride, supplements should not be made available in areas where the water already contains more than the optimum recommended level. In certain areas of Sri Lanka such as Embilipitiya, Kekirawa and Hambantota the water contains more than the recommended level - i.e. over 1.2 parts per million. The people who live in these areas have discovered an ingenious way to remove the fluoride. Their intent was not to remove the excess fluoride but to remove the ingredient causing an offensive taste. They keep the water overnight in an earthenware pot into which is added "Ingini" seeds. The next morning the water tastes fine. Certain dental surgeons working in these areas had samples of water (taken before and after it was treated analysed. The results were amazing. The sample of water before the addition of the seeds had a high content of fluorides. The samples after treatment showed no signs of fluoride ! There are theories linking fluoride to causing or leading to Alzheimer's Disease and Dementia, and that excessive intake can cause adverse changes to the bone structure. |
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A: Thalassaemia is a disease which affects the red blood cells in the
body. It is genetically inherited from parents and spread from generation
to generation The red blood cells in these patients are abnormal and short
lived and they breakdown easily Thus reduces the amount of haemoglobin in
t e body and causes anaemia. A person having the disease usually manifest
symptoms by the age of 5-6 months
The heart like a car engine has an electrical system which controls the
speed of the heart. A small group of highly specialized tissue situated in
the right atrium (right sided receiving chamber) known as the sinus node,
serves as the pacemaker. Each heart beat originates in the sinus node as
an electrical impulse. From the sinus node the electrical impulse passes
on to the ventricles (pumping chambers) through the special conducting
system and this electrical impulse stimulates the ventricular muscle to
contract, resulting in a heart beat. In normal people at rest, heart rate
is about 70 per minute and it is common knowledge that your heart rate is
same as your pulse rate.
