Sickle Cell Anemia -An inherited disease in which the body makes SICKLE-SHAPED RBC (C-shaped RBC).

Normal RBC Round (moves freely through the vessels) SICKLE-SHAPED RBC C-shape (Abnormal hemoglobin forms a strand that causes the C-shaped RBC) - Sticky, unable to move freely through the vessels. - tends to form clumps leading to OBSTRUCTIONS in the vessels. Life span------120 days 10-20 days

Red bone marrow produces RBCs to replace the old RBCs in the body. | Due to molecular instability and decrease life span of the SICKLE-SHAPED RBCs | There is FASTER RBC BREAKDOWN than the PRODUCTION of NEW RBCs | DECREASED RBC in the bodys circulation | DECREASED tissue perfusion | DECREASED Oxygen supply in the muscle tissues Example of an Inheritance Pattern for Sickle Cell Trait FATHER MOTHER

Normal gene (Normal Hbg)

Sickle cell gene (Abnormal Hbg)

Male

Female

Male

Female

NOTE: 1 copy of sickle cell gene form each parent is needed to form abnormal hemoglobin that could make a sickle-shaped RBC. WHO ARE @ RISK: Families in: Africa, South or Central America Mediterranean countries (Italy), India, and Saudi Arabia. HOW ITS DIAGNOSED: Hemoglobin Electrophoresis detects the presence of Abnormal Hemoglobin. CBC counts the number of RBC and checks abnormal shapes of RBCs.

TREATMENT:

THERE IS NO CURE FOR THIS DISEASE---only prevention of complications.

Infections Painful (vaso-occlusive)crisis Folic Acid & Penicillin NSAIDS OPIOIDS PCA(Patient-Controlled Analgesia) Diphenhydramine (counters itching of opioid use) O2 Therapy Blood transfusion Hydroxyurea (for severe painful crisis) Normal gene is put in the bone marrow of the person with sickle cell to cause the body to produce normal RBC.

Acute chest crisis Bone Marrow transplant Gene Therapy

AVOID! -Decongestants (vasoconstrictor) -Extreme heat and cold -Stress -Excessive physical labor -avoid traveling in air w/out extra O2 pump (high altitude increases O2 demand of the body)

By: ChronoZ