Sickle-cell anemia

By:

Cliff Stoutenburg

Chemeketa Paramedic Program

Sickle Cell

History of the disease

• Explained in 1904 by James B. Herrick

• Disease named in 1922

• High infant mortality rate, history of condition traces back to 1670s

• African americans using tar soap

• Demonstration of SCA in 1949

What is sickle-cell anemia(SCA)

• Common? – Affects 72,000 Americans – 1 in 400 African Americans have SCA

• Blood disorder – Mutation of hemoglobin – Decreases cell flexibility – Destroyed in liver/spleen

• Sickle cell traits

SCA

What is SCA, cont.

• Who does it affect?– More common in regions with high case of

malaria

• How do you get it?

– Can you get it from touching a person with it?

• Does it make carrier immune to malaria

What is SCA, cont.

• Specific form of sickle-cell disease

– Other forms of sickle-cell disease

• HbSC

Signs/Symptoms

• Signs and symptoms– Hypoxia – Increased acidity – Dehydration – SOB– Tachycardia – Delayed growth

S/S– Jaundice – Chronic abdominal pain– General weakness– Joint pain– Fever – Vomiting – Hematuria(blood in urine)

S/S

– Priapism

– Decreased fertility

– Excessive thirst

– Chest pain

Complications

• Vaso-occlusive crisis

• Severe Infections

• Spleen Crisis

• Stroke

Complications (cont.)

• Acute Chest Syndrome

• Anemia

• Aplastic Crisis

• Bone Necrosis

• Kidney Infarct

Complications (cont.)

• Renal failure

• Pulmonary Hypertension

• Congestive heart failure

• Hand and Foot Syndrome

• Cholecystitis

• Average life expectancy

Treatment • Can you be tested?

– Hemoglobin electrophoresis

• Is there a cure?– Some success with bone marrow transplant

(Blood)

Treatment

• Severe cases – Managed with opioid administration

• Diphenhydramine

– Antibiotics – Bone marrow transplants– Hydroxyurea – Vanillin– EPO– Supplement oxygen

Treatment

• Mild cases – Acetaminophen – NSAIDs– Folic acid – Supplement oxygen– Avoid extreme hot or cold weather – Avoid exertion – Stay hydrated!!!

Future treatment

• Studies – Nitric oxide – Clotrimazole – Butyric acid

Myths

• SCA contagious?

• Affects only African-Americans?

• People with SCA cannot get malaria?

Work cited page

1. http://en.wikipedia.org/wiki/Sickle-cell_disease

2. http://www.nhlbi.nih.gov/health/dci/Diseases/Sca/SCA_WhatIs.html

3. http://www.mamashealth.com/sickle_cell.asp

4. http://www.ncbi.nlm.nih.gov/books/bv.fcgi?rid=gnd.section.98

5. http://www.drspock.com/article/0,1510,5054,00.html