cell disease (SCD) is a genetic condition that 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 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.
stated by the author 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.
Chest Syndrome (ACD) is a rapid deterioration in respiratory function caused by
vaso-occlusion in the vessels of the lungs. Due to severe pain and using
narcotics as opioids, there is a potential for the decreased respiratory effort,
which may lead to ACD. Patients are encouraged 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.
is the most common condition that causes death in SCD patients called Streptococcus
preumoniae sepsis. 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
of the great imagining tools to determine whether a child with SCD is at risk
of a stroke is Transcranial Doppler, this tool is used regularly and is only to
be used for children. If the stroke is suspected it is important to stabilize
the patient to prevent further brain damage, and an exchange transfusion should
be performed immediately.
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.
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 extremely difficult.
Prior to Surgeries petient need
to be checked for any signs of fever, infection, dehydration, and pain, which may
trigger or cause a sickling crisis. Those receiving blood transfussion maybe in
the risk of suffering from complications. Because SCD is a hematologic
disease, it is important to track blood loss and to watch the patient’s Hgb
closely in case he or she requires an intraoperative or postoperative blood
transfusion. Postoperative care includes controlling pain, administering
supplemental oxygen if needed, and maintaining fluids. The nurse should
continually monitor the patient after surgery for ACS, pain crisis, and
infection. Surgical patients should be encouraged to use incentive spirometry,
deep breathing, and ambulation when it is allowed.