What Is Sickle Cell Disease
Sickle Cell Disease is a serious, inherited
disorder that affects red blood cells,
causing the cells to change their shape. It
can be a very painful disease.
Red blood cells contain hemoglobin, a
substance that carries oxygen from the air
in the lungs to all parts of the body.
Normal red blood cells contain hemoglobin
"A". they are soft and round and can easily
pass through tiny blood vessels.
People with sickle cell disease have red
blood cells that contain mostly hemoglobin
"S", an abnormal type of hemoglobin. these
cells become sickle (crescent) shaped, stiff
and sticky and have difficulty passing
through small blood vessels.
When sickle shaped cells block small blood
vessels, less blood can reach that part of
the body. Tissue that does not receive a
normal blood flow eventually becomes
damaged. This is what may cause the
complications of sickle cell disease.
Normally, red blood cells live for about 120
days before new ones replace them. Red
blood cells containing mostly hemoglobin
"S" do not live as long as normal red blood
cells. The early breakdown of the red cells
decreases the amount of hemoglobin in the
blood to carry oxygen, this causing anemia
of which the disease is also know as, Sickle
Cell Anemia.
Any child with sickle cell disease needs
close medical attention from their local
doctor. The child's caregivers should
receive instructions about the disease and
understand his/her special needs.
Understanding Genes
Genes are the instructions that determine
various characteristics and traits such as:
eye and hair color or diseases such as
Diabetes. We have two copies of every
gene in our bodies. We receive one copy
from our father and one copy from our
mother at birth. There is now way to control
which genes are passed from parents to
children. the sickle gene (S) affects the red
blood cells.
What is Sickle Cell Trait?
Sickle Cell Trait occurs when a person has
one working gene (A) and one altered gene
(S), they are also called a carrier. Sickle
cell trait is not a disease. People with sickle
cell trait cannot tell that they have it without
being tested.
How is Sickle Cell Disease Inherited?
Sickle cell disease is inherited when a
person receives an altered Sickle gene (S)
from each of their "carrier" parents.
Carrier parents have a one-in-four chance
of having an affected child and a one-in-two
chance of having a child who is a "carrier".
Carriers of sickle cell are not affected
themselves because they have a working
(A) gene as well as an altered gene.
Hospitalization and Related Issues
Many children are hospitalized as a result
of the complications of sickle cell disease.
Hospitalization is a hard time for children
and parents of children with sickle cell
disease
The infant/toddler or small child will need
constant advocacy. The parents are
expected to provide this for their child until
they can advocate for themselves. Some
your adults find it difficult to navigate the
system and advocate for themselves.
The school age child, on average, will miss
29 days of school per year due to
hospitalizations and home recovery. This
creates a dilemma for the children, parents,
social workers and educators.
Financial support to these families
becomes necessary because of the
economic hardship that often accompanies
hospitalization. Meals and parking are two
of the most commonly reported expenses
that strain the budget while a child is
hospitalized.
Many families experience employment
related issues such as medical leave. some
have limited understanding of their rights
and responsibilities during these trying
times.
Living with Sickle Cell - Spoken Word Video
CBS Cares - Sickle Cell PSA
Sickle Cell website for Kids
links