JEDDAH, 30 April 2003 — Saudi Arabia has one of the highest percentages in the world of people who are either carriers of sickle cell trait or with sickle cell disease, in some regions reaching 30% of the population.
The main cause of getting sickle cell anemia is through inheriting the abnormal hemoglobin from both parents who may be carriers without knowing it, and once born with the sickle cell hemoglobin it is present for life.
Abeer Almanabri is the director in the western region of the project for the prevention of inherited blood diseases, a non-government national project by volunteers that work under the Arab Gulf Program for United Nations Development Organizations.
She and her husband are both carriers of the trait and have a13-year old son suffering from thalassemia, “we both chose to marry from outside our families to avoid the known hereditary diseases in our families but we didn’t know that we were both carriers of this disease because we didn’t take a blood test before marriage,” and now she is on a mission to educate people and inform them about the importance of taking the blood test.
Sickle cell disease is a group of inherited red blood cell disorders. Normal red blood cells are round like doughnuts, and they move through small blood tubes in the body to deliver oxygen.
They become sickle when a change occurs to the hemoglobin, the oxygen-carrying component of the red cell, and thus causes it to form long rods in the red cell when it gives away oxygen.
Sickle red blood cells become hard, sticky and shaped like sickles used to cut wheat. When these hard and pointed red cells go through the small blood tube, they clog the flow and break apart. This causes extreme pain, organ damage and a low blood count, or anemia.
There is no cure for this disease and patients have to take pain killers for life, “they need to be hospitalized 2 to 4 times a month due to complications, which means they cannot have a regular learning and working life,” said Almanabri.
There are different types of sickle cell disease. One of the most severe is thalassemia, when the body does not produce enough alpha or beta protein in the hemoglobin and thus the red cells do not form properly and cannot carry sufficient oxygen. A person who carries the genetic trait of thalassemia will usually experience no health problems other than a possible mild anemia. Physicians often mistake the small red blood cells of the person with thalassemia trait as a sign of iron deficiency anemia and incorrectly prescribe iron supplements, and this is what happened with Abeer. The only way to detect the presence of the trait is through a simple blood test called the hemoglobin electrophoresis.
“The problem is that in Saudi Arabia, unlike many other countries including Arab, we are not required to do this test or told about it before marriage,” said Almanabri. “When the man and woman are both carriers of the trait, every time she is pregnant there is 25% chance that the baby will have the disease and 50% chance they will be carriers,” explained Almanabri.
When the lack of beta protein in the hemoglobin is great enough it causes moderate to severe anemia and significant health problems, including bone deformities and enlargement of the spleen.
This condition requires regular blood transfusion and extensive ongoing medical care.
These extensive lifelong blood transfusions, every two to three weeks, lead to iron-overload which must be treated with chelation therapy to prevent early death from organ failure. This therapy involves the patient undergoing the difficult and painful infusion of a drug, desferal, through a needle attached to a small battery-operated infusion pump and worn under the skin of the stomach or legs five to seven times a week for up to twelve hours.
“This is a very painful treatment but it’s the only one available now,” said Almanabri whose son has thalassemia. The Saudi government spends SR100,000 a year on treatment per patient and it gives SR200 million a year in financial support to the families of these patients.
“Every day in Saudi Arabia, 12 babies are born with the disease and 154 are born carrying the trait,” said Almanabri.
The Project for the Prevention of Inherited Blood Diseases has done a lot of research on the issue and it’s conducting a national campaign to educate people, all through the work of volunteers and with the support of donations.
It’s trying to persuade the Ministry of Justice and the Ministry of Health to implement a system that requires people to take the blood test.
“Our goal is not to prevent the marriage but to require from the marriage official to offer the couple to be married the choice to take the blood test and leave them the decision to get married or not and to require from the hospitals to conduct the test free of charge and give them medical advice,” said Almanabri.
Last year the Council of Ministers issued a decision for the Ministry of Health to provide free blood test to who ever requests it and to conduct an awareness campaign and it allocated the Ministry SR5 million a year for three years to do this, but so far they haven’t started on it or implemented the process for enforcing the decision even though the hospitals are already equipped with the necessary labs and personnel to do the test.
The Ministry claims that people will not be willing to take the test for social and religious reasons, “but we have spoken to many of the religious leaders in the country and they were all convinced and issued statements in support of doing the test for the good of the society,” said Almanabri. “If we don’t start now, in 50 years there will be 12 million Saudis either carrying the trait or with the disease,” said Almanabri.