Thalassaemia can be prevented:

Future not so ‘pale’

by Sajitha Prematunge

Marriage between carriers

Marriage between a carrier and a normal person Marriage between a Thalassaemic patient and a normal person

Thalassaemia is a major cause of mortality and morbidity in South East Asia imposing enormous strain on health services and economies of many countries. But fortunately it is a hereditary disorder, preventable through screening, clinical matching and marriage counselling says, Dr. Shantha Hettiarachchi, Thalassaemia Unit, Health Education Bureau on Sri Lanka’s position.

“1.5 % of the world’s population is Thalassaemia carriers”, said Dr. Hettiarachchi. “Approximately 70,000 beta Thalassaemic patients are born a year.” According to him 2.2% of the Sri Lankan population is carriers and 2,500 children are Thalassaemic. The highest number of Thalassaemia patients are reported from the Kurunegala, Kandy, Anuradhapura and Badulla districts. “Approximately 100 new patients are reported a year.”

Symptoms

As Dr. Hettiarachchi explained patients suffering from Thalassaemia major or Thalassaemic patients appear normal at birth, but become severely anemic during the first year of life. Poor growth, feeding, intermittent fever, delay in recuperating from recurrent infections and abdominal distension due to enlargement of lever and spleen are symptoms often connected with Thalassaemia.

Treatment

Health care of Thalassaemic patients have taken its toll on Sri Lanka’s economy. As much as seven to ten percent of Sri Lanka’s health expenditure is spent on treating patients suffering from Thalassaemia, which is equivalent to 10 million per one person over a lifetime. As Dr. Hettiarachchi explained alternatives available for developed countries such as bone marrow transplant and gene therapy are not available for Sri Lankans.

Beta Thalassaemia Minor

Beta Thalassaemia Major

Consequently they have to resort to more conventional methods such as blood transfusion. “Thalassaemic patients require regular blood transfusions to avoid fatal complications and to secure optimum growth and development.

It is important to maintain a haemoglobin level of over 10 to 12 g per deciletre”, said Dr. Hettiarachchi. Patients also have to undergo concurrent iron chelation therapy for iron overload, with the help of drugs such as Desferrioxamine.

“One unit of blood (420 ml) contains 200 ml of iron. As the red blood cells in the transfused blood are broken down, iron released from these cells accumulates in the body”, explained Dr. Hettiarachchi. Excess iron gets deposited in vital organs such as the heart, liver and pancreas leading to heart failure, liver disease, hepatic and pancreatic dysfunction and diabetes and could also result in thyroid, parathyroid and adrenalin insufficiencies.

Management

“It could be easily identified by a simple blood test whether a person is a Thalassaemia carrier”, said Dr. Hettiarachchi. “It is very important to know whether they are carriers of the disease prior to marriage, especially those who live in areas with high prevalence of the disease.”

“Although no cure is currently available in Sri Lanka Thalassaemia is easily prevented through counselling and awareness”, said Dr. Hettiarachchi. It is of utmost importance to avoid marriage between carriers he warned.

Marriage two thalassaemia carriers have four possibilities, with a 25% chance a baby may be born Thalassaemic. There is no danger of giving birth to a Thalassaemic baby due to a marriage between a carrier and a normal person, only a 50% chance of giving birth to a carrier and 50% chance of giving birth to a normal baby.

“Even a Thalassaemic patient can marry a healthy person without the danger of giving birth to a Thalassaemic child”, Dr. Hettiarachchi said.

Thalassaemia causes a decrease in the level of haemoglobin - an oxygen carrying pigment essential for body metabolism found in red blood cells. Haemoglobin consists of haem - iron component and globin - the protein component. Through the genetic mutation in Thalassaemia the globin synthesis is impaired, resulting in anaemia.

Synthesis of the main globin chains - either alpha or beta is markedly reduced. There are two types of Thalassaemia. Alpha Thalassaemia or beta Thalassaemia, depending on the production of which globin chain is impaired. “Although alpha Thalassaemia is the more common form, it is a mild form of Thalassaemia, with little or no clinical manifestations”, said Dr. Hettiarachchi.

Moreover according to the level of clinical manifestation, Thalassaemia is two fold - Thalassaemia minor and Thalassaemia major. People with Thalassaemia minor, also known as carriers are healthy and normal with the exception of a mild anemia.

With a history of over 50,000 years, Thalassaemia is the commonest inherited haematological disorder as well as the commonest single gene disorder in the world. Thalassaemia is derived from the Greek word Thalasa, which means ‘sea’, because it was found in a valley south of Italy and Greece covered by the Mediterranean Sea.

Add colour to your diet

Contd. from last week

Last week Healthwise discussed recommended food habits for children under five and diabetic patients, with Dr. Renuka Jayatissa, Head Department of Nutrition, Medical Research Institute. This week we are going to focus on patients affected with heart diseases, high blood pressure and how to improve the diet of vegetarians.

Heart disease

Monounsaturated fat can be derived from avacodo

One teaspoon of salt per day is sufficient for patients suffering from high blood pressure

Eggs are relatively harmless and contains only 250 mg of cholesterol

The aspect most heart disease patients and people who have a genetic tendency towards heart disease should keep in check is their oil consumption, explained Dr. Jayatissa.

Oil or fat can be divided into two categories - saturated and unsaturated fat. Unsaturated fat can further be divided into two categories as polyunsaturated and monounsaturated.

Sources of saturated fat include full cream milk, meat and coconut.

Peanut, cashew, ginger and contains polyunsaturated. Monounsaturated fat reduces the risk of dying from heart disease and can be derived from fruits like avocado and olive.

It is advisable for patients suffering from heart disease or people who are at high risk of getting heart disease to minimise - not avoid - cholesterol rich food. “But avoid full cream milk at all cost” warned Dr. Jayatissa. “If you stick to non fat coconut milk, it will cause no problem.”

“Patients suffering from heart disease or people who are at high risk of getting heart disease should obtain their energy 10% each from saturated, polyunsaturated and monounsaturated fat” said Dr. Jayatissa.

She recommended that heart disease patients and people who are at high risk eat a lot of vegetables. Their diet should consist of two vegetables, two green leaves and a minimum of two fruits per day.

Compared to other meat items eggs are relatively harmless and contains only 250 mg of cholesterol which falls way below the 300 mg recommended for a heart disease patient and a person at high risk. “It is safe to eat three eggs a week” said Dr. Jayatissa.

Other than recommendation for food, Dr. Jayatissa also recommended 45 minutes of exercise at least five days a week.

“People who are genetically at a risk of getting heart disease and are over weight should always keep their weight in check and go for annual cholesterol check ups.” She emphasised the importance of minimizing sugar and sault intake because excess sugar gets converted to cholesterol within the body. That means cutting down on sugar rich cola drinks and biscuits; salt rich sauces, soup cubes and dry fish.

“These should be occasional additions into your diet.”

High blood pressure

“As a nation our salt intake is very high” said Dr. Jayatissa. “It’s important to reduce salt intake at an early age.

In fact no salt should be given to children under the age of one” Dr. Jayatissa recommends only one tea spoon of salt per day for patients suffering from high blood pressure.

She suggested that lime juice and spices can be used to enhance taste instead of salt.

People at high risk of getting high blood pressure should avoid fast food, pastries which have a high content of salt and trans fats.

“They should avoid eating out and stick to home cooking which would facilitate the regulation of diet.” Exercise would relieve stress and therefore help to reduce blood pressure.

Vegetarians

Dr. Jayatissa explained that vegetarianism is not very advisable for children. “Prevention from eating meat causes iron deficiency. Twenty percent of children in Sri Lanka suffer from it.” She explained that erratic food behaviour cause anaemia.

To counter this one needs to consume meat stuff.

For vegetarians two types of pulses such as Soya, dhal and cowpea are a must in addition to the normal three types of vegetables and two types of fruit.

“Vegetarians should take large quantities of vegetables - up to four table spoons at a time” said Dr. Jayatissa. All main meals should be followed by a vitamin C rich fruit - such as guava, lime juice and tamarind - to enhance iron absorption.

Dr. Jayatissa emphasized the importance of regular check-ups of haemoglobin level in vegetarians. She further explained that exhaustion is a sign of anaemia and should take an iron supplement if necessary.

-SP

Rice: The healthy basics

by Susan Brady

Did you know that worldwide there are more than 40,000 different varieties of rice? Rice comes in a rainbow of colours and flavours and almost every culture on Earth has incorporated rice into their diet.

For more than half of the world’s population rice is a staple and, according to the World Food Grid, 20% of the total food energy intake in the world comes from rice. In Asia alone, more than 2 billion people get up to 70 percent of their daily dietary energy from rice and its by-products.

Rice hit American shores in the late seventeenth century and was an East Coast crop until the mid-1800s. There is currently about 3 million acres of rice cultivated in the U.S. by about 9,000 farms located primarily in Arkansas, California, Louisiana, Mississippi, Missouri, Florida, and Texas. While this is only 1% of the world’s total production, the total annual sales hit $1.2 billion in the year 2000.

One cup of cooked medium-grain white rice has 169 calories, 3.5 grams protein, 1.7 grams fibre, while the same amount of cooked medium-grain brown rice has 218 calories. 4.5 grams protein, and 3.5 grams fibre.

But the brown rice contains much higher quantities of thiamin, niacin, B6, magnesium, phosphorus and manganese. Healthier for you, brown rice does take more than twice as long to cook. (HINT: The easiest way to cook rice is to buy a rice cooker/steamer. You measure the water and rice, set it and forget it. White rice cooks in about 20-30 minutes, depending on amount, while brown rice takes about an hour and a half.

But you can make a double batch and reheat in steamer or microwave when needed.)

A recent study conducted over 22 years with 197,000 participants found that those who ate more refined white rice had a higher risk of type 2 diabetes, while those eating more brown rice had a lower risk of the disease. Researchers at Harvard School of Public Health and Brigham and Women’s Hospital assessed three different studies and found across the board that having more white rice in the diet was associated with an elevated risk of type 2 diabetes.

In addition to white and brown rice, there are a number of other rice alternatives that are healthy for you. Break out of your rut and try something new.

Typical U.S. Rice Varieties

Long grain white rice has a long, slender kernel, and when cooked the grains are separate, light and fluffy.

Medium grain white rice has a shorter, wider kernel and is more moist and tender, clinging together more than its long counterpart.

Short grain white rice has a short, plump, almost round kernel which becomes soft and clingy when cooked.

Brown rice has only the hull removed. (Further milled, it becomes white rice.) slightly chewy texture with a nut-like flavour.

Aromatic red rice has a deep, honey-red bran and is minimally processed to retain the bran layers. This makes it slightly chewy and nutty in flavour.

Basmati rice is an aromatic long grain rice with a flavour similar to that of popcorn or roasted nuts. Cooked grains swell lengthwise and separate and fluffy.

Black japonica is also an aromatic rice but with a dark black bran.

Also minimally processed, it is slightly chewy and sweet.

Jasmine rice is another aromatic, with long grains that cook soft and moist, but cling together. Like Basmati, the taste is similar to popcorn or roasted nuts.

Arborio rice is characterized by a white dot at the centre of each medium-sized grain. This is the typical rice for risotto because of its ability to slow cook, absorb various flavours, and become creamy and slightly chewy.

Wild rice is not really rice at all, but a grass seed. Taking longer to cook and with a distinct flavour, it is a bit more costly but is richer in protein and other nutrients.

- Healthnews