World Sickle Cell Day

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BY MICHELLE SMITH 

June 19th 2016 is known as World Sickle Cell Day. Sickle Cell Disease, also noted as “The Black Disease” due to it being very prevalent among dark skinned people and almost completely absent in “white” populations.  It has been cited that  sickle cell is also prevalent in the following areas: Africa, Mediterranean countries (such as Greece, Turkey and Italy),The Arabian Peninsula, India, Spanish-speaking regions (South America, Central America and parts of the Caribbean) In each region both dark and light skinned people have been found to be Sickle Cell carriers.

Sickle Cell Disease is inherited in an autosomal recessive pattern. This means that a child will not inherit the disease unless both parents pass down a defective copy of the gene. People who inherit one good copy of the gene and one mutated copy are carriers. They are clinically normal, but can still pass the defective gene to their children. According to statistics, if both parents carry a sickle cell gene, with each pregnancy there is a one in two chance the child could receive a sickle cell trait and a one in four chance the child could be born with the actual disease.

I have come to realize that this disease is not understood by many educators, employers and family members; this needs to change. The public at large need to have awareness about Sickle Cell Disease and its effects in the same respect that awareness is being shared about diabetes and hypertension, all diseases serve as a potential barrier for a productive life. A child with Sickle Cell Disease will be an adult with Sickle Cell Disease which means that support and awareness will be required as a lifelong management practice.

Sickle Cell Disease involves the red blood cells, or hemoglobin and their ability to carry oxygen. Normal hemoglobin cells are smooth, round and flexible, like the letter “O,” so they can move through the vessels in our bodies easily. Sickle Cell hemoglobin cells are stiff and sticky and form into the shape of a sickle, or the letter “C,” when they lose their oxygen. These sickle cells tend to cluster together and cannot easily move through the blood vessels. The cluster causes a blockage and stops the movement of healthy, normal oxygen-carrying blood. This blockage is what causes the painful and damaging complications of Sickle Cell Disease. The two main characteristics of SCD are a long-standing anemia and repeated episodes of vaso-occlusion. Anemia is a result of increased breakdown of red blood cells. Your child may appear pale and have yellow eyes from time to time. They may become tired sooner than their peers when doing an activity. Vaso-occlusive episodes are blockages of the blood vessels anywhere in the body by deformed red blood cells. This causes a lack of oxygen in the affected area of the body. Symptoms depend on where the blood vessels are blocked.

The most common symptom of a vaso-occlusive episode is bone pain. Any bone can be affected, including the arms, legs, back and skull. These episodes, commonly called pain crises, are unpredictable.  It is very important for parents to communicate with their children advising them to be aware of when they are not feeling well so that they can get support immediately. Some pain crises happen without a known reason which can make crisiS hard to predict. It has been cited that infection, stress/fatigue, dehydration and exposure to cold and very hot temperatures can be possible triggers for a pain crisis.  Now that it is summer it is very important to keep your child hydrated with a lot of fluids. This rule should apply regardless of illness. Remember healthy habits start early.

Have you been tested and what does this really mean? In 2006, Ontario became the first province in Canada to implement universal newborn screening for SCD in Canada.  Which means that this process gives parents a better opportunity to seek support earlier. For more information about the diseases being screened under this program you can visit https://www.newbornscreening.on.ca/en/screening-facts/diseases-screened.  

More importantly it is very important for individuals to understand that they may have the trait and not know it. The trait very rarely comes with signs or symptoms, which can pose a problem when picking a partner. If you have sickle trait and become involved with someone that has sickle cell trait then you potentially can have a child that will have Sickle Cell Disease. People with sickle cell trait do not have Sickle Cell Disease. They cannot develop Sickle Cell Disease later on in life but they can pass the sickle cell trait to their children. Sickle cell trait is inherited from one’s parents,  If one parent has sickle cell trait and the other parent has normal hemoglobin, there is a 50 percent (1 in 2) chance with each pregnancy of having a child who has sickle cell trait.

If the disorder is not detected at birth, a blood sample can be used in a test called hemoglobin electrophoresis. This test will determine whether a person has sickle cell disease or carries the faulty hemoglobin gene. If you are not sure about your status or your child’s status you can ask your doctor for this specific test. It is the only test that can detect sickle cell trait or disease.

Remember you are the most important part of your health care team. If you need further support with Sickle Cell Disease or management you can contact your health care advocate for further information.

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