Mismanagement, ignorance cause 7 to 10 deaths every day due to sickle cell anaemia: experts

July 12, 2009

Courtesy by: indianexpress.com

Almost a decade ago, the Baroda Medical College (BMC) became the first institute in the state to initiate research on the genetic disorder of sickle cell anaemia, which has affected a huge portion of tribal population in Gujarat. But in the past four years, the medical college has seen a complete stagnancy with no research proposals in line.

In a five-year project of the Indian Council of Medical Research (ICMR), between 2000 and 2005, the BMC had initiated the project of sickle cell anaemia and Thalassemia screening in antenatal and college students.

The project was jointly funded by ICMR and the state government with Rs 75 lakh for the project and Rs 50 lakh for the infrastructure development of genetic laboratory.

However, after completion of the project, the BMC had proposed to carry out a second phase of the same project focusing on the prenatal diagnosis of sickle cell anaemia and Thalassemia. “Since this is a genetic disorder, there is a 50 per cent chance of passing on of the disease to the progeny. The aim of the project was to study the prevalence of sickle cell at the foetus level so that the genetic disorder can be detected and stopped before the birth of the infant,” said Dr R Z Patel, retired associate professor of the Pathology Department, BMC.

Four years after the proposal for the second phase, the BMC is still waiting for funds and the laboratory awaits further researches on sickle cell anaemia. Interestingly, while Vadodara district was the first to carry out research, the screening in the tribal region started only in February this year. However, screening in south Gujarat had started much ahead in 2003.

“Over 3.15 lakh tribal population has been screened in Central Gujarat. The prevalence of sickle cell anaemia trait in Vadodara, Narmada, Dahod and Bharuch is between 14 and 17 per cent; while in Panchmahals, Sabarkantha and Banaskantha the prevalence is between 10 and 15 per cent. Over 0.2 per cent of the population is the carrier of sickle cell disease,”

said Prakash Parmar, project coordinator of Sickle Cell Anaemia and Thalassemia, Indian Red Cross Society (IRCS) Ahmedabad. The IRCS is carrying out screening of the tribals in the age group of 6 years and 30 years.

Research is not the only area of concern. With the understaffed primary and community health centres in the tribal regions, the patients hardly receive timely and proper symptomatic treatment.

“We generally depend on the health workers in the region to distribute iron folic acid among the patients. In sickle cell anaemia crisis stage, patients are brought to the city-based hospitals. However, though counselling forms the major part of the prevention process, there is a dearth of counsellors in the districts. There is only one counsellor in four tribal districts of Vadodara,” said Dr Vijay Birla, sickle cell anaemia project coordinator, District Health department, Vadodara.

Dr Jayanti Patel, founder of Late Shree Ramjibhai Chaudhari Sickle Cell Anaemia Foundation, Bardoli, said, “Gujarat has over 80 lakh tribal population. Of which, over 28 lakh population is likely to have sickle cell anaemia traits and two lakh are sickle cell carriers. But due to mismanagement and ignorance on the part of the society and doctors, 7 to 10 patients die every day.”

He added: “In all the screening facilities, children till the age of 5 years are not screened properly and so the disease is hardly detected early which keeps them out of early medication as well. The health workers and primary doctors do not have proper understanding of the disease, because of which most of the symptomatic treatment and diagnosis lacks precision.”


Life-saving fluids

July 12, 2009

Courtesy by: thestar.com.my

In emergency rooms and operating theatres, alternatives to blood transfusions keep patients alive by keeping their blood vessels full.

THE circulatory system can be described as a closed network of pipes (blood vessels) that originate from a two-sided, four-chambered pump (the heart).

Prof Joachim Boldt … On one hand we are running out of blood; on the other, we find it more difficult to get blood. In my hospital, we have had to cancel surgeries because we didn’t have enough blood available.

Flowing within these pipes is approximately five litres of blood (for an average adult), which delivers oxygen and nutrients to our cells, carbon dioxide to our lungs, and waste to our kidneys; regulates body temperature; and protects the body from attacks by bacteria, viruses, and foreign organisms.

Therefore, even though the proof of life is determined, by medical standards, with measurable activity of the brain or heart, our survival greatly depends on a functioning circulatory system.

As Fresenius Kabi Asia Pacific medical director Dr Hrishikesh Kulkarni put it in a press release on the Fresenius Kabi Advanced Course on Transfusion Alternatives (FRACTA), “When there is a disturbance in blood circulation due to illness, trauma, or surgery, organ blood flow is diminished, leading to decreased oxygen supply to the organs, and ultimately to organ failure.”

But before our organs fail, our bodies will have put up a valiant fight to correct the disturbance, caused by either the loss of blood or fluids, or the escape of fluids from blood vessels to the spaces between our cells (interstitial space).

Knowing blood is vital for survival, the body tightly regulates the amount of blood (blood volume) by monitoring the pressure it exerts on the walls of blood vessels (blood pressure).

Dr Hrishikesh Kulkarni … When there is a disturbance in blood circulation due to illness, trauma or surgery, organ blood flow is diminished, leading to decreased oxygen supply to the organs and ultimately, organ failure.

When the body loses fluids or blood, blood volume decreases (hypovolemia). The lower blood pressure that results will alert pressure sensors, or baroreceptors, parked in the inner walls of large veins and chambers in the heart. The baroreceptors will then send signals to cardioregulatory centres in the brainstem, which will try to increase blood pressure by instructing small arteries to constrict and the heart to pump harder and faster. By these measures, the remaining blood will be pumped through a smaller space, raising blood pressure to normal levels.

Low blood volume also causes granular cells in the kidneys to secrete hormones that promote water reabsorption and decrease urine production.

However, if we lose more than 10% of our blood (about 500ml), this mechanism will no longer be able to compensate for the decreased blood volume, and our bodies may go into hypovolemic shock. Blood pressure will start to plunge as the heart can’t compensate and is unable to pump sufficient blood to muscles and organs.

The body will start to redistribute blood away from the skin, muscle, and gut to the brain, heart, and kidneys to try and preserve their function. Eventually, oxygen-deprived cells will start to die and our organs will start to fail.

To prevent us from going into shock, we need fluids to sustain our blood volume until the infection or blood loss can be stopped. And before the routine use of intravenous fluids, this was usually done with blood transfusions. However, several limitations of blood transfusions had doctors using alternatives as far as possible.

Prof Tong-Joo Gan … If you have low blood pressure because you have lost a lot of blood, there’s not much point in giving a solution that is going to go everywhere (in the body).

“On the one hand, we are running out of blood; on the other, we find it more difficult to get blood. In my hospital, we have had to cancel surgeries at times because we didn’t have enough blood available,” says Prof Joachim Boldt, who is the anaesthesiology and intensive care medicine department head of Klinikum Ludwigshafen in Germany.

Risks associated with blood transfusions such as infections and patients’ rejection of donor blood, and the reluctance of people from certain cultures and religions to receive blood, have also limited the efficiency of blood transfusions. During emergencies, blood transfusions are also difficult to carry out as doctors will need to determine the patient’s blood type before they proceed.

While there is currently no real substitute for blood, alternatives that expand blood volume (volume replacement therapy) include fluids that are categorised as crystalloids and colloids.

Generally, crystalloids like saline and lactated Ringers solution (saline plus additional electrolytes like potassium and magnesium) contain smaller molecules, whereas colloids like starch, albumin, dextran, and gelatine contain larger molecules.

As colloids contain larger molecules, they stay within the blood vessels. Crystalloids, however, do not, as the small molecules can leave the blood vessels and enter the interstitial space.

Prof Datuk Dr Wang Chew Yin … Colloids and crystalloids, including new generation starches, are available and are used depending on a patient’s needs.

Vice chairman of the US Duke University Medical Center anaesthesiology department Prof Tong-Joo Gan explains: “If you have a low blood pressure because you have lost a lot of blood, there’s not much point in giving a solution that is going to go everywhere (in the body).

“You want a solution that stays within your blood so that it actually allows you to perfuse the organs adequately.”

The mainstay of volume replacement therapy is now starch because of its advantages, says Prof Gan. Besides being cheaper than albumin, new generation starches are also more similar to human plasma, says Prof Boldt. “With plasma adapted fluids, we can avoid metabolic derangements and reduce the risk of coagulation disturbances.”

In Malaysia, consultant anaesthesiologist Prof Datuk Dr Wang Chew Yin says colloids and crystalloids, including new generation starches, are available and are used depending on patients’ needs.

Will we be looking at a future without blood transfusion thanks to the advent of newer volume replacement therapies? Not likely, as such therapies can only help those who need to replace blood volume loss, but not those who need blood transfusions due to the damage or lack of functioning blood components, such as people with sickle cell anaemia or thalaessemia).

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