September is National Sickle Cell Awareness Month. Sickle Cell Disease is a group of diseases that affects hemoglobin, the molecule in red blood cells that delivers oxygen to cells throughout the body. People with sickle cell disease have hemoglobin S, which can distort red blood cells into a sickle, or crescent shape versus the normal round shaped red blood cells. This disease is inherited from your parents. If both parents have the sickle cell trait but not the disease there is a 25% chance each pregnancy that the child will have sickle cell disease.
Sickle cell disease is an incurable disease that is more common in our community than any of our counterparts. The disease is usually found in people of African, especially Sub-Saharran region and Caribbean descent. Sickle cell anemia is passed on to new generations through genetic lineage. Even if both parents do not have sickle cell anemia, it is possible one of them may carry a dormant gene for sickle cell anemia. It is very important that before you have children you find out if either you or your partner carries the sickle cell trait. I would recommend genetic counseling before you start a family if you both carry the trait.
Signs and Symptoms
Children who have sickle cell disease usually do not display signs or symptoms until 4 months of age.
Infections – Sickle cell disease often affects the spleen, which is the organ that fights infections in the body. Both children and adults who have the disease will have a hard time fighting infections. Infants and children with damaged spleens are more likely to get serious infections that can kill them in hours or days.
Anemia – Sickle cells die after an average of 20 days versus normal red blood cells that live for an average of 120 days. A shortage of oxygen will cause fatigue.
Pain crises – These are the most common symptoms of sickle cell anemia. The sickle shaped red blood cells block blood flow through tiny blood vessels to the chest, bones, joints and abdomen causing pain. Some people will only have a few episodes and others will have multiple crises a year and will need to be hospitalized for medical intervention.
Delayed growth – Due to the decrease in oxygen and nutrients needed for growth. The shortage of healthy red blood cells can slow growth in infants and children and delay puberty in teenagers.
Vision problems – Sickled cells can affect the small blood vessels in the eye and damage the retina. The retina is responsible for taking the images you see and sending them to your brain, so there can be serious vision loss possible blindness.
Stroke – Due to the deformity of the red blood cells a blockage can occur in the vessels going to the brain causing one type of stroke and they can also cause a vessel to burst causing another type of stroke.
Sickle cell anemia has no widely available cure. Treatments can help relieve symptoms and treat complications that occur in the disease. Pain crises can be treated with over-the-counter pain medication and fluids. If the pain continues or becomes severe it may be necessary to go to the hospital. Antibiotics are regularly given to prevent infections in sickle cell sufferers. It is also important that children with sickle cell disease have all of their routine vaccinations. Preventing complications is also a vital part of treatment. Routine scans of the brain can help doctors determine if the patient is high risk for strokes. Many patients receive routine blood transfusions to help prevent, stroke, infections etc.
People are living well into their 40s, 50s and older with sickle cell disease… so it is very important that if your child or family member is diagnosed, to watch very closely for the different symptoms so they can be treated promptly. It is very important that people with sickle cell disease stay well hydrated and limit stress. It has been found that dehydration and stress can exacerbate the symptoms and cause pain crises.
How Can We Help?
It will take a lot of family support to help a loved one to live with this disease. If you are not directly affected by this disease but have a friend or friend’s child with the disease, try and help them especially during hospitalizations because they can be very stressful and unfortunately plentiful. Perhaps you can sit with a friend at the hospital or volunteer to watch their other children while they are at the hospital with their child. You can also get involved by donating, volunteering and/or participating in their fundraising events.
If you need more information or support with Sickle Cell Disease please visit the Sickle Cell Disease Association of America
BMWK – do you or someone you love have Sickle Cell? How can we best support families that are dealing with this disease?