Professional Documents
Culture Documents
Sickle Cell
Sickle Cell
and
Womens Health
the Caribbean,
the Eastern Mediterranean,
Middle East and
Asia.
Low-birthweight baby
Toxemia
Acute chest syndrome
Pneumonia
Fetal death
Antepartum hemorrhage
Causes of Complications
People with sickle cell make a different form of hemoglobin
A called hemoglobin S (S stands for sickle)
This is due to a mutation in the -globin chain of
hemoglobin, replacing glutamic acid with less polar
valine at the sixth position of the chain .
The association of two wild type a-globin subunits with
two mutant -globin subunits forms hemoglobin S, which
polymerizes under low oxygen conditions causing
distortion of red blood cells and a tendency for them to
lose their elasticity.
http://www.sicklecelldisease.org/about_scd/index.phtml
www.worldofmolecules.com/ disease/hemoglobin_S.htm
Sickling
Red blood cells containing mostly hemoglobin S live
only about 16 days versus 120 days of normally
shaped cells. They also carry less oxygen, become
stiff, distorted in shape and have difficulty passing
through the bodys small blood vessels.
When sickle-shaped cells block small blood vessels,
less well oxygenated blood can reach parts of the
body. Tissue that does not receive a normal blood
flow eventually becomes damaged.
Symptoms of SCA
Symptoms appearing at six months of age may include
enlargement of the abdomen and heart and painful swelling
of the hands and feet. Growth is often delayed.
In adolescence, sexual maturation may be delayed.
The disturbances in blood flow associated with the disease
also dispose affected people to infections and leg ulcers.
These symptoms are due to the altered hemoglobin, which
changes shape when the amount of oxygen in the blood is
reduced for any reason. The red blood cell in which the
hemoglobin is contained also changes its shape, from round
to crescent (sickle shaped).
http://www.fortunecity.com/greenfield/rattler/46/Anaemia.html
Medical Complications
Sickle cells are destroyed rapidly in the body of people with
the disease causing anemia, jaundice and the formation of
gallstones.
The sickle cells also block the flow of blood through vessels
resulting in lung tissue damage (acute chest syndrome), pain
episodes (arms, legs, chest and abdomen), stroke and
priapism (painful prolonged erection).
It also causes damage to most organs including the spleen,
kidneys and liver. Damage to the spleen makes sickle cell
disease patients, especially young children, easily
overwhelmed by certain bacterial infections.
Acute Chest
Syndrome
http://europa.redjupiter.com/images/pediatriceducation/110104SickleCellAcuteChestC.jpg
Crisis Types
1. Vaso-Occlusive common (Painful)
2. Aplastic usually associated with infections
(Parvovirus B19)
3. Sequestration particularly in infants and
children; in those with SC and S-beta Thal
(cause of death in 10-15% under 10 years of
age)
4. Hemolytic rare
Vaso-Occlusive Crisis
Results from infarction due to sickled, poorly
oxygenated blood
Symptoms are diffuse and bilateral pain
Organ damage includes:
Spleen to the point of becoming nonfunctional by age 2-4
CNS
Cerebral infarction in children
Hemorrhage in adults
Leg Ulcers
www.meddean.luc.edu/.../ pulmonar/pd/step52b.htm
http://www.cdc.gov/ncbddd/hbd/women.htm
http://www.dshs.state.tx.us/newborn/sickle.shtm
erasmeinfo.ulb.ac.be/.../ English/sickle_diag.htm
Treatment Tips
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Aim for the patient to live as normal a life as possible but be prepared
to take immediate action if he or she becomes ill. Treat infection
early.
Maintain general health and nutrition. Make sure that the patient
keeps warm and dry.
Avoid situations likely to precipitate crisis e.g. dehydration, acidosis,
general anesthesia, and sports such as skydiving and scuba diving.
Arrange regular blood tests. These are needed for reference in a crisis
and to monitor kidney and liver function .
Consider folic acid supplements.
All patients with sickle cell disorders should be offered prophylactic
penicillin.
Treatment Tips
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Treatment
Health maintenance starts with early diagnosis, preferably in
the newborn period and includes penicillin prophylaxis,
vaccination against pneumococcus bacteria and folic acid
supplementation.
Blood transfusions help by reducing recurrent pain crises, risk
of stroke and other complications.
It is important to remove excess iron from the body, because
it can gather in the heart, liver, and other organs and may
lead to organ damage.
Treatments are available to eliminate iron overload.
Promising Treatment
Developments
In search for a substance that can prevent red blood cells from
sickling without causing harm to other parts of the body,
Hydroxyurea was found to reduce the frequency of severe
pain, acute chest syndrome and the need for blood
transfusions in adult patients with sickle cell disease.
Droxia, the prescription form of hydroxyurea, was approved
by the FDA in 1998 and is now available for adult patients
with sickle cell anemia. Studies will now be conducted to
determine the proper dosage for children.
Other treatment options in clinical development include new,
more convenient options than current therapies to eliminate
iron overload caused by repeated blood transfusions.
Transplantation can convert patients into carriers.
Local Resources
Pennsylvania SCDAA
Philadelphia / Delaware Valley
4601 Market St.
Philadelphia, PA 19139
Stanley A. Simpkins, Executive
Director
(215) 471-8686 Office
(215) 471-7441 Fax
E-Mail:
stanley.simpkins@verizon.net
Website:
www.sicklecelldisorder.net
HRS: 9:00am 5:00pm