1- Inherited red blood cell disorder
o Abnormal hemoglobin
o Affects bloody carrying oxygen to the body
o RBCs live 10-20 days vs 120 days
o Cresent shaped cells, can get suck and restrict blood flow
sickle cell disease
2most common and severe sickle cell disease
o Hemoglobin SS
3sickle cell diseases that iare more common in Mediterranean regions
Beta thalassemia 0
Beta thalassemia +
42nd most common sickle cell disease, moderately severe
Hemoglobin SC
5what is the etiology of sickle cell disease
- Autosomal recessive genetic disorder
o Receive an abnormal gene from both parents
6sickle cell disease can link as a protective agent against ______ and more common in regions with high ______ risk
Malaria
malarial
7how many people in the US are affected by sickle cell disease
100,000
8how many people in the US carry the gene for sickle cell disease
2 million
9how many births per year are born with sickle cell disease
3000
10o 1 in ___ Black/African American babies
1 in __ carry the gene
o 1 in 365 Black/African American babies
1 in 13 carry the gene
11o 1 in ______ Hispanic American babies
1 in ____ carry the gene
o 1 in 16,300 Hispanic American babies
1 in 144 carry the gene
12___% carry the gene worldwide
7
13how many births worldwide are born with sickle cell disease
312,000
14how is sickle cell disease diagnosed
blood test at birth
15what are the signs and symptoms of sickle cell disease
- Symptoms appear later due to fetal hemoglobin
- Chronic anemia
o Fatigue, weakness
- Pain and swelling in hands/feet
- Infections, splenic complications
- Respiratory complications
- Visual deficits
- Blockage of blood flow
16what can blockage of bloodflow cause
pain
tissue damage
organ damage
stroke
17what is the course and prognosis for sickle cell disease
- Frequent pain, hospitalization, and decreased academic achievement.
- Mental health
- Life expectancy varies greatly based on early diagnosis and medical intervention.
- Nearly all African children born with SCD die during childhood, and most by age 5.
18what are the medications used for sickle cell disease
penicillin used prophylactically in children under 5. Hydroxyurea used to decrease risk of stroke. Need to be cautious with other medications due to side effects.
19what are some things being researched to cure sickle cell disease
- Bone marrow transplant and gene therapy being researched as a potential cure.
20how does sickle cell disease impact occupational performance
- Chronic pain, anemia, progressive cognitive decline adversely impact occupational performance across areas.
- Begin in childhood and progress.
- Improve symptoms
21where might you work with someone with sickle cell disease
any setting throughout the lifespan
22what are the precautions for sickle cell disease
stroke risk
23what are the contraindications for sickle cell disease
medical
24what are the considerations for sickle cell disease
cognitive decline, mental health