PARLIAMENTARY SPEECH

S-211 – Sickle Cell Awareness

December 1, 2016

Mr. Len Webber (Calgary Confederation, CPC): Mr. Speaker, I am pleased to be able to rise today to contribute to this debate on Bill S-211. The proposed legislation before us today would recognize June 19 on an annual basis as national sickle cell awareness day.

    There are approximately 2,000 people living in Canada today with sickle cell disease. Increased awareness of their disease would be beneficial, given the low level of public knowledge at this time. It is a hereditary disease. It is not contagious. Carriers are usually not sufferers of the disease, but in combination with a carrier spouse, the disease usually becomes apparent in their offspring. This disease is most common among those with ancestors from India, Saudi Arabia, the Mediterranean, the Indian Subcontinent, and the Sub-Saharan countries in Africa. However, it is still found in other cultures as well. Just to give members an idea, there are an estimated 43 million carriers, with 3.2 million people having the disease because both parents were carriers.

What is sickle cell disease? It is a group of red-blood-cell disorders. Those with the disease have abnormal haemoglobin. As many know, haemoglobin is the part of the red blood cells that carries vital oxygen throughout the human body. We know how important it is that tissues in the body receive a steady and life-sustaining supply of oxygen to work well. Haemoglobin takes the oxygen from the lungs to the parts of the body that need it. Normal cells are a disc shape, sort of like a donut. This shape allows the cells to be flexible. This flexibility in shape allows the cells to travel easily through blood vessels throughout the body. Sickle haemoglobin is different. It forms stiff rods within the red cell, and this changes the cell’s shape to something more like a crescent or sickle shape. As members can imagine, this creates enormous problems. The sickle-shaped cells result in blockages because the cells are stiff and unable to pass through the vessels easily. These resulting blockages mean that vital oxygen stops reaching the parts of the body that need it.

What impact does this have on the person with the disease? A lack of oxygen results in attacks of sudden and severe pain throughout the body. It is a horrible condition. These pains occur without warning, and often result in hospitalization. The pains usually last five to seven days. While not always the cause, it has been noted that pain crisis can be triggered by temperature changes, stress, dehydration, and even living in high altitudes. Of course, any infection that normally causes a rise the number of red blood cells triggers the disease as well. Fortunately for most children with the disease, pain usually subsides between pain episodes. Nonetheless, many children known to have the disease take penicillin daily to help the immune system, and face a life-long regimen of daily folic acid. For teens and adults, the pain is usually chronic. The effects of chronic pain are well know. It has a huge impact on the education, the employment, and the human mind of the sufferers.

Due to the lack of oxygen to vital organs on a regular basis, sickle cell disease often begins to cause long-term damage to vital organs. It is common for those with the disease to develop serious issues with their skin, their brain, their bones, their spleen, their heart, their kidneys, their liver, their lungs, and even their eyes. The spleen is particularly susceptible because of its narrow blood vessels and its basic job of clearing old red blood cells.

If this was not enough, there is another layer of cruelty to this disease. Normal red blood cells have a typical 90- to 100-day existence. Sickle cells last only about 10 to 20 days. Members can imagine what a toll this takes on the human body when it has to replenish red blood cells at 10 times the normal rate. When the body cannot keep up, which is often, there is a shortage of red blood cells and this results in the sickle cell anaemia. The most visible side effect is fatigue. As I mentioned before, this also adds to the pain, the long-term organ and tissue damage, and the toll the disease takes.

Sadly, this disease is a lifelong illness, and when I say lifelong we must not kid ourselves. Lifelong is not a happy story either.

Sickle cell disease shortens lives but it depends currently on where one lives and access to help.

In first world countries like the United States, life expectancy can range greatly from 40 to 60 years. This is about four times longer than it was 40 years ago. Now, about 90% of those with the disease can expect to see their 20th birthday and 40% of those will die by age 50.

Is there a cure? There is a treatment and it is called hematopoietic stem cell transplantation, or HSCT. HSCT is the best known option at this time. Unfortunately, most people with the disease are either too old for a successful transplant or do not have a genetically matched person able to make the donation. The success in this type of treatment is heavily dependent on having a great match.

For HSCT to be successful we need an early diagnosis and good medical treatment. Those who are willing to donate bone marrow should consider the positive effects that their donation could have. Given the need for the best match possible, I specifically suggest that those in affected cultural communities help promote donation, organ and tissue, bone marrow, and blood donation.

In the meantime, the disease takes it toll. There are increased chances of stroke, infection, gall stones, joint pain, low immunity, erectile issues, bone infection, leg ulcers, vision problems, preeclampsia in pregnant women, and heart and kidney failure.

The pain of the disease often means patients are prescribed opioids to deal with the pain. The good news is that addiction among sickle cell patients to opioids is not any higher than the general population. However, having said that, opioid addiction is a reality for many with sickle cell disease. We have heard a lot in the House recently about the affects of opioid addictions and it is alarming. This type of addiction is often deadly and even when it is not, it results in many other significant problems for patients and their families.

There are an estimated 5,000 Canadians living with the disease and the rate is increasing. There is prenatal screening, but with the knowledge comes the difficult decisions that parents must consider, and I cannot imagine.

The Sickle Cell Disease Association of Canada does a lot of advocacy and awareness work and I applaud it for its efforts. Its efforts have gone a long way toward bringing this disease into the fore and making it better known in our society. The association faces an uphill battle in finding a cure. Research dollars are not easy to come by, especially for a disease that is most prevalent in parts of the world that do not have the ability to attract the attention of major pharmaceutical companies. It is a discase that is still very much not discussed, even with the communities most affected. However, things are improving. There is an increase in research funding and awareness that is slowly building.

Passing this legislation would go a long way to normalizing discussion about the disease. It would show those with the disease that we care and help to educate those around them about their disease.

I will be honest. I knew nothing about this disease until I prepared for this speech. As I learned more about sickle cell disease and the thousands of Canadians who suffer from it, I wanted to share this with others. I applaud the hon. member for presenting this legislation and the work that he has done on this. I give him my thanks. I am happy that I was able to talk a bit about it here today to help share awareness.

Let us support this legislation and keep spreading awareness and education.