The author describes sickle cell disease
(SCD) as a genetic condition, which is inherited when a child receives two
sickle cell genes – one from each parent. Sickle cell occurs when the red blood
cells are rigid and sickle-shaped, which causes them to stick together and
block oxygen to the body. A large number of infants are born with SCD annually;
today every newborn is screened for the sickle cell gene. In this article the
author covers the following topics pain, acute chest syndrome (ACS), infection,
stroke, and available treatment options. Fisher mentions that adults and
children with SCD must be continuously checked for pain with the use of the
appropriate pain scale. There are several natural pain treatment approaches
such as, acupuncture, physical therapy, massage, hydration, behavior
modification, deep breathing, imagery, and distraction. Medicated treatment
includes administration of IV fluids, opioids or nonsteroidal anti-inflammatory
drugs and oxygen if hypoxia is present.
One of the conditions is Acute Chest
Syndrome (ACD), it is a rapid deterioration in respiratory function, and it is caused
by vaso-occlusion in the vessels of the lungs. Due to severe pain and using
heavy narcotics, there is a large possibility for the decreased respiratory
effort, which may lead to ACD. Those with ACD are recommended to use an
incentive spirometer to promote deep breathing, and blowing efforts for younger
ages. ACD is the most common cause of death amongst young adults.
Infection, it is one of the major conditions
that cause death in SCD patients called Streptococcus preumoniae sepsis. The
patients who experience fever should be treated as an emergency and require
intervention as soon as possible, including blood cultures, complete blood
count, chest radiographs, IV fluids, and IV Antibiotics.
The author describes Transcranial Doppler
as one the great imagining tools to determine whether a child with SCD is at
risk of a stroke. This tool is used regularly and is only to be used for
children. If the stroke is suspected, the patient must be stabilized to patient
to prevent any brain damage, and an exchange transfusion should be performed right
A Chemotherapeutic agent that has been
shown to decrease vaso-occlusive episodes and incidence of ACS is called Hydroxyurea.
Hydroxyurea therapy is one of the
available pain treatments, which results in less need for blood transfusion and
is not used in the acute state.
The bone-marrow transplant is rarely
used; due to high death rate from complications in children who receive a bone
marrow transplant. Finding a match through the Bone Marrow Registry is