Sickle Cell Disease

Susan Kelly, BSN, RN

King University

Pathophysiology
The functional changes that
accompanies a particular
syndrome or disease.6
What is Sickle Cell
Sickle cell is a disease process that is passed from generation to
generation, when both parents pass the *gene to their offspring that
child will have Sickle Cell Disease (SCD).3
*Cells that determine our make-up such as eye and hair color, blood types,
and other disease processes like heart disease.
Inheritance is the only way an individual can develop Sickle
Cell, it is not a contagious disease process.3
Transformation of Cells
The gene that tells your body to make iron, the compound that makes
your blood red known as hemoglobin, which carries oxygen from
your lungs to other parts of your body is abnormal. Upon the trans-
formation of the altered hemoglobin, oxygen is removed causing red
blood cells (RBCs) to become sickle shaped. Therefore, RBCs cannot
easily pass through blood vessels. 1
Local and national support
groups can be informative
as well as supportive.

Who is at risk of having Sickle Cell

Around the world, millions of people have the sickle cell trait. Sickle
cell is the most common inherited blood disorder in the United States
of America. Risk factors include geographical location, as sickle cell
is more common in Middle Eastern, Indian, Mediterranean and those
Websites are excellent of African heritage. 2
Resources:
Signs and Symptoms

Sickle cell does not typically present until the age of one year.
www.cdc.gov • Increased heart rate know as Tachycardia
• Pale skin color
www.nih.gov • Difficulty breathing known as Dyspnea
• Weakness
www.sicklecelldisease.org • Pain
• Infections
• Delayed growth
• Vision problems
• *Hyperbilirubinemia, yellow tint to skin and/or eyes
*Too much bilirubin (a yellow-orange pigment formed in the liver
by breaking down hemoglobin) in the blood 3
 Complications
Diagnosis • Vaso-occulsive crisis
(Clumping of red blood cells that effects
blood flow causing organ damage)
Sickle Cell is diagnosed by taking a simple blood test. 3 • Severe pain as a result of tissue *ischemia
*(Restriction in blood supply to issue causing
damage)
Treatment • Organ damage as a result of ischemia
• Stroke 5
There is no known cure for Sickle Cell at this time how-
ever, research is on going. Sickle cell can be managed
by:
Non-medical lifestyle
modifications
• Opioid pain medication such as Morphine • Drink 8 to 10 glasses of water daily
• Anti-inflammatory medications such as Motrin or
Advil • Avoid excessive temperatures (hot & cold)
• Oxygen • Rest frequently
• Fluids IV and by mouth • Get frequent check-up 2
• Antibiotics for infection
• Hydroxyurea
• Support groups 3.4
Alternative Therapy
Natural Supplements:
Medical Goals Magnesium
To decrease and or eliminate crisis. Zinc
Vitamin B, C, E 2
Increased intake of spinach, beets, lentils,
and thiocyanate

Risk Associated with Non-Compliance

• Anemia
The most common risk, as the sickle shape RBCs
break apart easily and die, leaving you without enough
red blood cells. Red blood cells usually live for about 120
days before they need to be replaced. But sickle cells usually die
in 10 to 20 days, leaving a shortage of red blood cells 5

• Crisis phase
During the crisis phase patients, will experience extreme episodes of pain in various parts of the body such as bones, joints,
chest, and abdomen. The intensity of pain varies as well as the longevity the painful episodes can last a few hours or a few
days 5