What is Sickle Cell Anaemia?

The disorder affects the red blood cells which contain a special protein called haemoglobin (Hb for short). The function of haemoglobin is to carry oxygen from the lungs to all parts of the body.
People with Sickle Cell Anaemia have Sickle haemoglobin (HbS) which is different from the normal haemoglobin (HbA). When sickle haemoglobin gives up its oxygen to the tissues, it sticks together to form long rods inside the red blood cells making these cells rigid and sickle-shaped. Normal red blood cells can bend and flex easily.

Because of their shape, sickled red blood cells can't squeeze through small blood vessels as easily as the almost donut-shaped normal cells. This can lead to these small blood vessels getting blocked which then stops the oxygen from getting through to where it is needed. This in turn can lead to severe pain and damage to organs.

Everyone has two copies of the gene for haemoglobin; one from their mother and one from their father. If one of these genes carries the instructions to make sickle haemoglobin (HbS) and the other carries the instructions to make normal haemoglobin (HbA) then the person has Sickle Cell Trait and is a carrier of the sickle haemoglobin gene. This means that this person has enough normal haemoglobin in their red blood cells to keep the cells flexible and they don't have the symptoms of the sickle cell disorders. They do however have to be careful when doing things where there is less oxygen than normal such as scuba diving, activities at high altitude and under general anaesthetics.

If both copies of the haemoglobin gene carry instructions to make sickle haemoglobin then this will be the only type of haemoglobin they can make and sickled cells can occur. These people have Sickle Cell Anaemia and can suffer from anaemia and severe pain. These severe attacks are known as Crises. Over time Sickle Cell sufferers can experience damage to organs such as liver, kidney, lungs, heart and spleen. Death can be a result.

Another problem is that red blood cells containing sickle haemoglobin do not live as long as the normal 120 days and this results in a chronic state of anaemia. In spite of this, a person with sickle cell disorder can attend school, college and work. People with sickle cell disorder need regular medical attention particularly before and after operations, dental extraction and during pregnancy. Many hospitals arrange follow-up appointments and it is advisable to discuss with the doctors questions concerning schooling, strenuous exercise, family planning, suitable types of employment and air travel. When a person is found to have a sickle cell disorder it is important that all members of the family be tested. They will not necessarily have sickle cell disorder but may be healthy carriers of a sickle cell trait.

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Bone Marrow Cells Regenerate Heart in Brazil Test

RIO DE JANEIRO, Brazil (Reuters) - Infusing patients with bone marrow cells can reinvigorate their dying hearts and grow tiny new arteries and heart muscle tissue, a treatment that may one day make many heart transplants unnecessary, Brazilian researchers said on Friday.

Dr. Hans Fernando Dohmann, coordinator of the research carried out at the Pro-Cardiac Hospital in Rio de Janeiro, told Reuters four patients out of the five studied no longer needed transplants after being treated with stem cells.


"It was the first time we saw that stem cells actually generate new arterioles, although we have indirectly observed that before via tests. That eliminated the need for transplants in four patients who had had indisputable transplant indications," he said.


The experiment, to be detailed to a weekend meeting of heart researchers and submitted to the journal Circulation, adds to a growing body of research that suggests such treatments can someday avoid the need for many transplants.


When one patient died of a stroke after 11 months of treatment, Dohmann's team was able to do an autopsy and actually look at what had happened to his heart.


They could see the tiny new arteries in the treated area and also saw what looked like new muscle tissue.


"This is the first documented development of cardiac muscle tissue in humans ... while the accepted concept is that cardiac muscle cells, just like nerve cells, do not regenerate," Dohmann said.


He said his team would continue testing other patients.


"But this one leaves us quite convinced, as we have real proof that is a human heart," he said.


Dohmann's team treated 14 patients with bone marrow cells between December 2001 and late 2003. Seven other patients in the control group were treated with conventional methods, and doctors said their recovery was not as good.


During the period, two deaths occurred in the stem cell group and one death in the smaller control group, which did not receive the stem-cell treatment.


The research was carried out jointly with the Texas Heart Institute, which designed the stem cell injector, and with the Rio de Janeiro Federal University.


Bone marrow is a rich source of adult stem cells, which are blood cells that retain the capacity to grow into a range of cells including white blood cells, blood vessel cells and heart cells.

Source:
By Andrei Khalip http://story.news.yahoo.com/news?tmpl=story&u=/nm/20040924/hl_nm/health_heart_brazil_dc




 
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