Mr. Speaker, I am pleased to rise in the House to speak about awareness of sickle cell disease. One of the reasons I think it is important to talk about is that I had to treat people when I worked as a nurse intern in a small, remote village in Senegal. This disease is particularly prevalent in sub-Saharan Africa, so it was a big concern for us. I also studied this disease when I did additional training in the field of international health. I therefore think it is important to talk about it. This is the most prevalent genetic disease in the world. It affects approximately 100 million people worldwide and 5,000 to 7,000 people in Canada.
Although people from all ethnic backgrounds can have this disease, it tends to be more prevalent among those with ancestors from India, the Middle East, the Mediterranean, particularly Greece and Italy, sub-Saharan Africa, and the Caribbean. There is a connection between this disease and places where malaria is prevalent; however, since it is a bit complicated to explain, I will not elaborate any further right now. If anyone would like more information, I would be happy to explain it to them.
It is an autosomal recessive disease. That is kind of a complicated genetic term. Basically, it means that people have to inherit the defective gene from both parents to get the disease. A child who gets the gene from just one parent is a carrier but will not have symptoms. People who might seem perfectly healthy, who have no health problems, no pain, nothing, can have a sick child because they do not know they carry the gene. That is important, and I will come back to it later.
I will try to explain the disease in simple terms so that most people can understand. Red blood cells are usually round, kind of like a donut that has been squashed in the middle. They flow freely through blood vessels. When they enter capillaries, donut-shaped red blood cells fit together well and flow unimpeded. Normal red blood cells usually live for about 120 days.
Diseased red blood cells are sickle shaped. They do not flow through blood vessels properly. Although it may seem obvious, I still want to point out that a sickle shape has two points. When these blood cells flow through capillaries, they can cause tears and create problems. Also, these red blood cells live for only 20 days, so anyone with this disease becomes anemic. Anemia is a condition that happens when an individual does not have enough red blood cells. Oxygen does not circulate properly in the blood, because oxygen is usually carried by red blood cells.
As it has already been said, a lack of oxygen can cause all kinds of health problems and a lot of pain. It can also cause localized problems, such as a higher risk of infection wherever the problems are. Depending on the seriousness of the problem, people with this disease often have to receive blood transfusion and take medication for pain and other medication for infection, if any infection occurs. This can also cause dehydration, in which case an IV can be administered to rehydrate the patient. That about covers all the treatments.
While some patients might actually manage to live normal lives with very few hospitalizations, others will be hospitalized frequently. As this is a genetic disease that patients are born with, some very young children, and even infants, will have to be hospitalized.
This can be especially worrisome when a baby is suffering and crying endlessly and the parents cannot figure out why, especially if the parents did not even know they were carrying the gene and that they may have transmitted the disease to their children. That is why I think it is important to raise awareness about the disease, especially among health professionals.
One of the problems we have concerns people in remote regions in particular. Since the disease is more prevalent among certain cultural groups, most doctors and nurses in remote regions will have never seen a patient suffering from sickle cell disease. It is therefore not the first disease that will spring to mind.
They have studied and read about every disease in the book, but after a while, they often rely on their experience and on what they have already seen. It can be hard to have a patient who is suffering so much. A doctor might be quick to diagnose such individuals with fibromyalgia or any health problem other than sickle cell disease, especially when it comes to adults. Health professionals assume that if the adult had sickle cell disease he or she would have been diagnosed long ago. It is not something that health professionals will think of, especially in regions where there are very few people from the different cultural groups that I already mentioned.
One of the most important things to do is to raise awareness among health professionals, ensure that they know about the disease, and that they think about it in order to make a diagnosis more quickly and avoid misdiagnosis problems that delay treatments. That was the first important point I wanted to raise.
Another very important point concerns raising the public's awareness. Prenatal screening is now offered in six provinces and two territories. Three provinces are considering it. Thus, we can hope that, in two or three years' time, most or all provinces and territories in Canada will have their own prenatal screening program.
The problem is that older individuals have no idea whether or not they are carriers because the screening was not done when they were born. It would be advisable to offer the test to couples thinking of having children, especially those from the cultural groups most susceptible to this disease. This would let them know whether or not they risk giving this disease to their child and allow them to make an informed decision.
Generally speaking, if both parents carry the gene, there is a 25% chance that the child will have the disease. If both parents know that they are carriers, they might choose to have only one child. If that child does not have the disease, the couple might not take another chance and instead limit the size of their family thereby avoiding having children with the disease. In order to make these decisions, couples must have that knowledge.
In addition to prenatal screening, health professionals need to be able to talk to couples who are thinking about having a baby. These couples need to be told that the test exists and that there is information available. In order for that to happen, health professionals need to know about the disease and must be able to give people that option.
The last point that I wanted to raise is that people with sickle cell disease, particularly children, who live in remote areas may find it difficult to receive care. These young patients are often referred to specialized centres for children, which can cause a lot of problems for parents. It is therefore important to provide more support for family caregivers, particularly parents. It is often women who make the sacrifice to take care of the health of their children, who stay with them, and who do all the travelling. Often, these women are not compensated as they should be for all the help they give to society by making that choice.
We need to realize that there is an extremely high rate of divorce among couples who have a child with sickle cell disease because of the onerous care such children often require. We need to do more for family caregivers.
It is important to raise awareness, but we also need to go further and figure out who should be targeted for education and how this would actually apply in a health practice.
I was pleased to speak to Canadians to help them to learn more about sickle cell disease.
