health wise

Thalassaemia - a silent killer:

Prevention, the best option

By Dhaneshi YATAWARA

Protecting the right of your child to enjoy a healthy life is a major part of a responsible parenthood - don’t you agree? Think twice, would you let your child carry the burden?

Facial disfiguration due to enlargedmaxillary and frontal bones

Thalassaemic child with abdominal protrusion

In the case of patients with thalassaemia, their lives would have been brighter if their parents had a slightest idea of how to prevent continuing the physical disorder they inherited. Thalassaemia is an inherited disorder of blood and hence has no cure; unless with a highly expensive bone marrow transplant.

“Yet, this is preventable by simply concerning the appropriate medical conditions in selecting one’s life time partner and heading for a ‘safe marriage’,” said Dr. Ashok Perera, the Medical Officer in Charge of the Thalassaemia Prevention and Patient Rehabilitation Program at the National Centre for Thalassaemia. The Centre is functioning under the Kurunegala Teaching Hospital, having Dr. Dayananda Bandara and Dr. Jayantha Wijesekara as consultants.

Avoid misconceptions

“This is neither a cancer nor a infectious disease. This is strictly a result of a genetical change and only parents with this disorder can pass it to their children. And these children are not mentally retarded,” Dr. Perera added.

“Thalassaemia exists throughout the country but it was found, with the recent researches by the medical community, that North Central, North Western and Uva provinces have reported highest number of cases. Certain parts of the Central Province also reports a considerable number of cases,” Dr. Ashok Perera explained further.

Accordingly among these three main provinces, North Western Province reports the highest and Kurunegala district is prominent. “The number of new cases reported yearly is now at a downturn. In 2006, 50-60 new cases were reported and by 2007 it dropped to 49 new cases and last year it was 39. So there is progress which can continue with proper management,” he stated further.

The safest approach in preventing the birth of a thalassaemia child is a for a carrier to marry a non carrier. “A thalassaemia carrier is totally a normal, healthy person and his or her condition won’t be converted to that of a thalassaemia patient, which is a misconception among the community we have observed,” Dr. Perera added.

Medically, thalassaemia condition is in two parts - i.e. thalassaemia major and thalassaemia minor. Thalassaemia minor category are also known as carriers or traits.

Only those with thalassaemia major will show all the symptoms of the disease. As mentioned earlier, there is no possibility for the thalassaemia minor condition to be converted to thalassaemia major.

A spectrum of conditions

Since no one can predict the sequence of the genes, thalassaemia shows a spectrum of conditions depending on the structure of the haemoglobin molecule. “ Only two have been observed in Sri Lanka - `beta’ thalassaemia and `E’ thalassaemia,” Dr. Ashok Perera said explaining further. Accordingly patients with `beta’ thalassaemia is more in number. “Red blood cells in Thalassaemia `E’ patients shows longer life time - thus, less complications for patients. These patients live longer than patients with `beta’ thalassaemia,” he added. The oldest `beta’ thalassaemia patient living in the country is 32 years old and the less number of thalassaemia `E’ patients live longer.

According to Dr. Perera another level is `alpha’ thalassaemia where the foetus gets affected while in mother’s womb and not born as a result. Either they would face a miscarriage or a still birth. “We don’t have patients with `alpha’ thalassaemia since they do not live till birth,” Dr. Perera explained.

Haemoglobin is not just one component - in our blood it has three categories. i.e., haemoglobin A, Haemoglobin A2 and haemoglobin F (foetus). In a normal adult, haemoglobin A is in high percentage while haemoglobin F is very low. Among the normal thalassaemia patients we mostly find, haemoglobin F at a very high percentage and haemoglobin A is very low. Haemoglobin F gets destroyed very quickly thus shortening the lifespan of a red blood cell.

“Hence, we cannot generalize patients. They should be examined and analyzed by a special consultant doctors and a specially trained staff,” Dr. Perera added.

As it is reiterated by the medical professionals the best option to eradicate thalassaemia is to prevent the birth of a thalassaemia child. To treat an adolescent patient the Government spends 1-1.5 million rupees per year per person.

“People need to conduct blood screening, specially if they are living in areas with high thalassaemia prevalence. This is a less complicated method,” Dr. Perera said stating that it is a major step in their preventioncampaigns.

First step would be testing for a full blood count which could be conducted at any medical lab islandwide, where the volume of red blood cell is measured (MCV).

Three parameters are considered - MCV, MCH and MCHC and if these are below normal levels further testing is required to differentiate patients from those having anaemic conditions. “A chemical analysis of the haemoglobin is needed next specifically to identify thalassaemia patients and this is done through High Performance Liquid Chromatography (HPLC),” Dr. Perera explained.

In the preventative steps taken by the National Centre for thalassaemia high priority is given in taking the blood screening to the village level where the people are gravely struck by poverty finding it extremely difficult to travel to the town just to check their blood. “We need to conduct cascade screening where the immediate relatives of a affected family, i.e., brothers and sisters of the parents, will be tested to check for carriers,” he stated further.

“A person must identify whether he or she is a carrier or not and that should be a key factor when marriage is concerned. We call it the 21st `porondama’ (horoscope factor),” Dr. Perera emphasised.

To make the process more people-friendly and extend it to the most rural areas the National Centre is at a grave need for a field thalassaemia screening machine and a HPLC.

Medical horoscope

Any married couple would dream of a family with beautiful healthy children. Serious disputes in families is unavoidable if these dreams shatter.

The psychological and social problems these families endure are limitless. “Most of these families are from very remote villages. Transport and such costs for each visit is unbearable for these people,” the medical officer added. Red blood cells destruct prematurely iron deposits in the body in extensive amounts. These iron deposits are toxic. Hence, need to be removed. Removing iron can be treated with drugs whether given orally or injected subcutaneously. The drug, desferrioxamine, injected subcutaneously needs to be given to an adolescent child 8-10 hours per day, five days per week totally covering 25 days per month through a special infusion pump. Basically this pump is eternally connected to their bodies just like an extra organ.

“In addition they need monthly blood transfusion getting admitted to the hospital for few days. We have seen that this adversely affects these families who mostly depend on daily wages and the children unable to attend school,” Dr. Perera further explained.

As this genetic disease is incurable and the cost of management is very expensive, prevention is the best option for Sri Lanka. If a married couple who are both carriers conceive a child, the couple has to take the painful responsibility of raising a child with thalassaemia, restricting the right of the child to have a happy life.

For a successful prevention program detection of all the carriers would be ideal. In Sri Lanka if we promote to make sure that either of the partners of a couple is a non carrier the concept will be more flexible. The carriers have their right to know their problem and solutions available for them.

However the final responsibility lies with the man and the woman who are going to marry and produce children. Are we going to continue to carry this burden to the next generation?

 Thalassaemia is a disorder in the red blood cells. In these patients red blood cells breakdown premature to its full life-span of 120 days. The actual problem lies in haemoglobin - the most important constituent in blood carrying oxygen and giving the blood its redness. Haemoglobin molecule has two components - haem and globin. Globin is a protein. In thalassaemia globin production gets defective as a result of the changed genetic material. Synthesis of protein in our bodies is controlled by our genes which we inherit from our parents. Thalassaemia is a disease that will manifest only if two defective genes were inherited from both parents. That is only a pair of genes with the same ‘change’ can disorder the production of globin. Since carriers or thalassaemia minor category carry only one gene they will experience any sort of a disorder in globin production.

Symptoms

Since thalassaemic patients experience anaemia, in order to fill up the deficiency the body tends to produce more haemoglobin even at extraordinary points. Normally red blood cells are produced in the bone marrow at the end of long bones of our body. To meet the demand in a thalassaemia patient even maxillary bone marrow will start producing red blood cells.

The liver and the spleen are other two organs that get affected due to this excessive haemoglobin production. Hence the two organs enlarge resulting a protruded abdomen. The facial and forehead bones starts to overgrow in trying to give more space for the bone marrow. Hence, protrusions of forehead and cheeks result.

A child would start showing these symptoms from five years onwards.

The initial symptoms becomes visible in a child since 4 - 5 months from birth. Lack of redness under the eye, inadequate weight gain, less active, poor sucking, more prone to infections and eyes get yellow in colour are the most common ones.

As the iron deposits under the skin it becomes unusually dark in colour.

To provide more space for bone marrow for maximum red blood cell production the cavity enlarges and the shaft becomes thin. This results a fragile bone structure.

Naturally these people have a high iron absorption rate to fill up the deficiency as a natural adaptation of the body. In addition to keep haemoglobin at an optimum level monthly blood transfusion (2 - 3 days per month) is required. This results an excessive level of iron deposits in the body which needs to be removed using drugs.

Iron is a foreign element to the body thus it will have negative impacts on body’s most vital organs. The heart tend to get enlarged and later the patient may be prone to a heart failure. As the body endocrine system gets affected, i.e. organs like the pituitary gland, pancreas etc. the body faces a hormone imbalance. With the irregular production of insulin thalassaemic patients are prone to diabetes. Secretion of hormones affecting the secondary sexual growth imbalances reducing the growth of the child.

Economics of vegetarianism - Part II

by Dr. D.P. Athukorale

The cost of living increasing so gradually all around us, it would be nice to find a soft spot in the economic system - a place we can cut costs for a change. Many Sri Lankans are discovering that the vegetarian life - style offers us this opportunity. As far as I am aware per capita consumption of beef, pork and mutton has dropped tremendously in favour of chicken and a thing of the past for all except the most affluent Sri Lankans (Buddhists, Muslims and Christians).

For the average Sri Lankan consumer the drop in the sale of beef, mutton and pork is a blessing in disguise. Many Sri Lankans in the Urban areas, especially Sinhalese (Buddhists and Christians) and Muslims have turned away from high priced beef and mutton in favour of broiler chicken and fish. The poorer Sri Lankans fortunately have been drawn away from poultry and dairy products in search of more economical alternatives. Happily, there is a whole new world awaiting them in the form of green vegetables, roots (manioc, sweet potatoes, “kiiri-ala” etc.) whole grains, legumes (soy, “mekeral”, beans, “dambala”, green gram, dhal etc.) and fruits, and all for so much less than the cost of animal products. The cost of a kilogram of beef, pork or mutton is very much more expensive than a kilogram of beans or soy or lentils which does not contain saturated fatty acids, cholesterol antibiotics, hormones and other poisons such as nitrates as in case of animal products you buy from the supermarkets.

Ironically affluence may actually be a curse for the wealthy as it helps them to continue to revel in the meat, dairy products, highly refined food stuffs-pastries, pizzas, cutlets and food deep-fried using vegetable oils. Of course, they can afford the food and the subsequent medical bills that come along with a diet like that. But can they physically afford be sick and sluggish and to live shorter and less robust lives.

While we may be lured into a vegetarian lifestyle for economic reasons, we find that we soon develop a taste for these foods. What at first tasted weird, begins to taste rather delightful. Moreover we can get up from the table with more energy than when we first sat down, instead of rolling over to the couch to regroup after an exhaustive onslaught of fats and cholesterol.

As the years roll on we realise that we are getting sick less frequently, have shed past tendencies towards lethargy and lazy apathy, have become more lively and zestful participants in all the wonderful experiences that life offers us and have seriously diminished the likelihood of heart disease, cancer, arthritis, diabetes, hypertension, kidney ailments, obesity and other ailments that cut short our enjoyment of life if not life itself.

You might wonder why we Sri Lankans especially the affluent like to eat meat and dairy products if vegetables and grain foods are cheaper and more nutritious.

The pork producers, meat and dairy product industry organisations spend millions of rupees informing the TV viewers that a glass of milk or pat of butter, sausages, a lean cut of beef is essential to a well-balanced diet, that consumption of beef sausages is essential for children who want to become good cricketers etc., This type of advertisement became a major focus of the meat and dairy industries in recent years. It is noteworthy that these TV ads are not Health Authority ads, and when you buy milk foods and products the customer has to spend money which the meat and milk industries spend for their TV advertisements which comes to millions of rupees.

What flesh eaters may not understand is that they are getting a lot more than protein when they consume animal products. You are getting saturated fats, cholesterol, hormones, antibiotics (used for livestock industry) used by the meat and dairy industry when you buy meat products. You consume for more protein than you need and you are paying five times as much as you would, to get the same amount of plant protein (which does not contain cholesterol, saturated fats nitrates hormones and antibiotics as in case of meat products and dairy products). The hormones and antibiotics costing hundreds of thousands of rupees are prescribed not by veterinary surgeons or doctors but by the agribusiness. Remember the fact that vegetarian foods cost for less than animal products and your body gets fibre (roughage) in addition to essential aminocids and other nutrients without chemical additives which animal products contain.

Remember the fact that vegetarian foods cost for less than animal products is only a bonus. The significant benefit is that you are getting more of what your body needs in terms of nutrients; Most people would be shocked to discover how much more costly meat is than vegetables and grains. In USA, for example supply the same twenty grams of protein that six and two thirds slices of whole wheat bread do. Yet the steak costs 1.17 dollars compared to only twenty-one cents for the bread. You are paying five times more for a food that does not rival its counterpart’s nutritional value and fibre content.

Although hard to believe, the high cost of animal foods, the nutrients you need to sustain life must ultimately come from plant foods. You can get these nutrients in one of two ways, either directly by eating the plant food itself or indirectly by eating the animal that was fed on plant food. To get the nutrients second-hand from the animal means you have to pay for the food many times over. When you pay for beef you are paying for the grains fed to raise the animal, for the ranchers overhead on the animal as well, as processing, packaging and transport of the meat. It makes better economic sense to eat the plant foods directly.

Preventing kidney diseases

Risk Factors for kidney disease

Diabetes and high blood pressure can damage your kidneys and lead to kidney disease. These conditions cause about 70 percent of kidney failure cases.

1. Early kidney disease has no signs or symptoms - the only way to know if you have kidney disease is to get checked.

2. Kidney disease does not go away. It may get worse over time and can lead to kidney failure. If the kidneys fail, the only options are dialysis or a kidney transplant.

3. Kidney disease can be treated. The sooner you know you have it, the sooner you can take steps to keep your kidneys healthy longer.

4. Heart Disease.

5. Family genetics.

Testing for kidney disease:

1. A general blood test to measure your GFR, which tells you how well your kidneys are working. GFR stands for glomerular (glow-MAIR-you-lure) filtration rate.

2. A urine test measures the amount of protein in your urine. Protein can leak into the urine when the kidneys are not working well.

3. You should know your GFR, if you have protein in the urine, your blood pressure and your blood glucose.

How to keep your kidneys healthy:

1. Manage your diabetes and keep your blood pressure below 130/80 mmHg.

2. Eat healthy foods and cut back on salt.

3. Exercise

4. Take medication as prescribed.

5. Get blood and urine tests done regularly for kidney disease, at least once in every 3 months.

6. Ask your doctor about blood pressure medicines that can help slow down kidney disease.

(The article is based on an interview with Dr. P. Ravichanran, Chennai.)