Sickle Cell Disease: What You Should Know and How You Can Help

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

Photo Courtesy of www.SickleCellDisease.org
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.

Treatments
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.