Smith k sickle_cell

Sickle Cell Disease (SCD) is a life long
condition with no known cure. It is an
autosomal recessively inherited condition that
affects the structure of the hemoglobin. Within
the United States, SCD occurs most often in
Black American and Hispanic individuals (Brown,
2012; Gill, Lavin, & Sim, 2010).

Introduction (continued)
SCD is a group of disorders:
*Sickle Cell Trait (SCT)
*Sickle Cell Anemia (SCA)-most
common
*Sickle Cell Hemoglobin C
*Sickle Cell-Thalassemia
(McCance, 2010)

Incidence
*1- 400 to 1-500 live births among blacks
*1 of every 36,000 Hispanic births
*In the general population, the risk of two black
parents having a child with SCD is 0.7%
*According to National Heart, Lung, and Blood
Institute, SCD affects an estimated 70,000 to
100,000 people in the United States (Gill, Lavin, & Sim, 2010).

Prevalence
*Tends to occur in people with origins in
equatorial countries, particularly central Africa,
Near East, Mediterranean area, and parts of
India
*Occurs in 7% to 13% of blacks in the
United States
*As high as 45% in East Africans, those
who have the trait, not necessarily the disease
(Brown, 2012).
*Average life span 45-65 years

SCT – The individual inherits HbS (hemoglobin S)
from one parent and HbA (normal hemoglobin) from the
other (AKA: the sickle cell carrier)
SCA – The individual has two HbS genes (HbSS), the
most severe type of SCD, and homozygous form of SCD
(65% of SCD)
Sickle cell HB C disease - heterozygous form in
which the child will inherit another type of abnormal
hemoglobin from one parent (25% of SCD)
(Gill, Lavin, Sim, 2010)

Characteristics (continued)
Sickle Cell Thalassemia – Individual inherits one
copy of HbS gene and one copy of the Hb beta-thalassemia
gene.
*Beta Thalassemia genes result in reduced or
lack of expression of normal HbA
*Individual thus has only HbS or mostly HbS
with very small percentage of HbA (Gill, Lavin, & Sim, 2010)
*Life span of sickled cell—10 to 20 days,
normal RBC—120 days (Brown, 2012)

NOTE: Hemoglobin polymerization,
leading to erythrocyte rigidity and vaso-occlusion
is central to the pathophysiology
of Sickle Cell Disease (Rees, Williams, & Gladwin, 2010).
*Deoxygenation - the most important variable
in determining the occurrence of sickling
(McCance, 2010; Rees, Williams, & Gladwin, 2010)

Pathophysiology (continued)
*The 6th amino acid in the Beta-globin chain becomes
valine instead of glutamic acid
Normal Beta Hemoglobin Chain
Valine Histidine Leucine Threonine Proline Glutamic acid Glutamic acid
Sickle Cell Beta Chain
Valine Histidine Leucine Threonine Proline Valine Glutamic acid
(McCance, 2010; Rees, Williams, &
Gladwin, 2010)

*Mutation of the 6th amino acid produces a hydrophobic
motif in the deoxygenated HbS tetramer
*Flexible, oxygenated, HbS-containing red blood cells
(SRBC) traverse the capillaries and release oxygen
*Once oxygen released, polymerization begins and the
SRBC becomes rigid
(Sangkatumvong et al., 2011)
*Crystallization produces polymer nucleus, growing and
filling the erythrocyte

*Architecture and flexibility of erythrocyte disrupted,
promoting cellular dehydration
(Rees, Williams, & Gladwin, 2010)
*Increased osmolality of plasma draws water out of
erythrocyte
*Decreased plasma volume occurs in states of dehydration
causing increased viscosity of blood
*Cell stretched to elongated crescent
(www.bydeway.com)
McCance, 2012)

*HbS not bound with oxygen forms aggregates of
semisolid gel that become stacked within the
erythrocyte
*Cell changed from flexible, nourishing cell to an
inflexible obstacle that starves and damages tissues
(Sangkatumvong et al., 2011)
*SRBC may become lodged if failing to escape
microvasculature
*Microvascular bed becomes obstructed
(Designerget.com)

(Prathama.org/tbds.php)
*Increased viscosity of blood--the final common pathway
leading to multiple pathologic effects
*Viscous blood flows slowly, promotes vascular
obstruction by increasing opportunities for sickling while
decreasing opportunities for reoxygenation in lungs (McCance,
2010)

*An oxygenated sickle-shaped red blood cell
(RBC) returns to biconcave shape…however…
*Repeated cycles of sickling tires the RBC
*RBCs eventually become irreversibly sickle
shaped
*Rigid sickled RBCs easily trapped, circulatory
survival shortened, and chronic hemolysis is end
result (Wells, DiPiro, Schwinghammer, & DiPiro, 2012)

*Autosomal recessive
genetic disorder
*For disease to manifest,
one must inherit two copies
of sickle cell gene (one from
each parent)
*Results from an amino acid
substitution in the Beta
globin chain of hemoglobin
(medical-dictionary.thefreedictionary.com/sickle+cell+disease)

Manifestations of SCD
*General: pallor, fatigue, jaundice, and irritability
*Acute: Crises (4 types)
1. Vascular/thrombotic – sickling in microcirculation, vasospasm
and “logjam” effect, extreme pain
2. Aplastic – transient cessation of red blood cell production,
acute anemia, extremely low reticulocyte count (result of viral
infection)
3. Sequestration – large amounts of blood pooled in liver/spleen
(seen only in a young child)
4. Hyperhemolytic (rare) – accelerated rate of red blood cell
destruction usually in association with certain drugs and
infection (McCance, 2010)

Clinical Manifestations Chart
(uspharmacist.com)

Complications of SCD
(medical-dictionary.thefreedictionary.com/sickle+cell+disease)
Complications (examples of
those visualized)
Acute chest syndrome
-New pulmonary infiltrate with chest
pain, temperature >38.5° C,
tachypnea, and cough
-Lung becomes spleen-like, sickled
red cells attach to endothelium, fail
to become reoxygenated, increased
inflammation, lung infarcts
-Poor prognosis, leading cause of
morbidity, 25% of all deaths in those
with SCD (McCance, 2010)

Complication Examples (continued)
Glomerular Disease
-Characterized by damage to glomeruli
-Protein and often red blood cells allowed to leak into
urine
-Caused by sickled cells in the kidney
-Results in nephropathy progressing to renal failure
Cholecystitis
-An inflammation of the gall bladder from a gallstone in
cystic duct
-Can be caused by hemolysis resulting in increased
bilirubin leading to gallstone formation (McCance,2010)

Complication Examples (continued)
Cerebral Injury
-Common in SCD
-Approximately 45% of patients with SCD have
cerebral infarcts
-One study showed 37% patients had a silent stroke by
age 18
-Result of vascular congestion of sickled cells
(Sangkatumvong, 2011)

Precipitating / Sickle Triggering
Factors
One or more of the following stressors:
*Hypoxemia
*Increased hydrogen ion concentration in
blood, low pH (decreases hemoglobin’s
affinity for oxygen)
*Increased plasma osmolality
*Decreased plasma volume – dehydration
*Low temperature (McCance, 2010)
*Infection
*Pregnancy (growing fetus causes strain,
can lead to crisis)
*Physical/Mental stress (Brown, 2012)

Pharmacologic Treatment of SCD
Note: Pharmacologic treatment is based primarily on symptoms
presented, no know cure for SCD. Dosages of medications are
patient specific (age, weight)
GENERAL
-SCD patients should receive routine immunizations, plus
influenza, meningococcal, and pneumococcal vaccinations
(Kiera105.wordpress.com)
-Children are recommended (up to age of 5) to be placed on
prophylactic Penicillin by 2 months of age
Pen VK (Penicillin V Potassium) 125 mg. orally twice daily
until age 3 then 250 mg. twice daily until age 5 (Wells et al., 2012)

(continued)
GENERAL (continued)
-Or Benzathine penicillin 600,000 units IM (Intramuscularly)
every 4 weeks from 6 months to 6 years old
-Adults, pregnant women and all patients with chronic hemolysis
should take Folic acid 1 mg. daily (Wells et al., 2012)
SPECIFIC
1. Pain (severe)
Opioids: Morphine or Dilaudid – first line of management – PCA
(patient-controlled analgesia) appropriate method for continuous
dose of analgesic, reducing peaks and troughs of pain

(continued)
Other analgesics: Fentanyl, Oxycodone (2nd line opioids)
Non-steroidal anti-inflammatory: Ibuprofen, Ketorolac
(Brown, 2012)
2. Pain (moderate)
Weak Opioid: Codeine or Hydrocodone (Wells et al., 2012)
Mild Analgesic: Acetaminophen
3. Dehydration
3-4 liters of 0.9% Normal Saline for the adult with accurate
fluid monitoring to avoid overload (Brown, 2102)

(continued)
4. After 3 or more vaso-occlusive pain crises/acute chest
syndrome/severe symptomatic anemia
Hydroxyurea - chemotherapeutic agent, stimulates HbF (fetal
hemoglobin) which correlates with decreased RBC sickling
and adhesion
-Dosage begins at 10-15 mg/kg daily as single dose,
can be increased 5 mg/kg/day every 8-12 weeks
Maximum dosage 35 mg/kg/day (Wells et al., 2012)
5. Acute Chest Syndrome
Broad-spectrum antibiotics

(continued)
Macrolide (Azithromycin 500 mg twice daily,
Clarithromycin 500 mg twice daily, Erythromycin 500 mg
twice daily)
Quinolone (Ciproflaxin 400 mg IV every 8-12 hrs.
depending on severity of infection, or Levoflaxin 500 mg IV
daily)
6. Infection-Fever >38.5°C (Empiric antibiotic therapy with
coverage against encapsulated organisms recommended)
Cefotaxime 1-2 gms every 8 hours IV for moderate to severe
infections (for inpatients)
Ceftriaxone 1-2 gms daily IM or IV (for outpatients)
(Wells et al., 2012; Alexander et al., 2012)

(continued)
OTHER
1. Antipruritic & Antiemetics: Hydroxyzine, Compazine
2. Opioid Antagonist: Naloxone (to counteract effects of
respiratory depression caused by opioids)
3. Laxative: (reduce constipation due to opioid usage)
Docusate, Lactulose, Senna (Brown, 2012)

Non-Pharmacological Interventions
*Heat application to areas of pain
*Limiting movement of painful extremity
*Regular check ups with eye doctor, watching for vessel
damage to vessels in eye and retina
*Oxygen therapy when hospitalized
*Hydration

Non-Pharmacological Interventions
(continued)
(Sciencephoto.com)
*Blood transfusions – in children to maintain HbS <30%
and prevent stroke and recurrence of stroke
(Wells et al., 2012)
*Well-balanced diet high in protein and fiber (will
facilitate tissue repair and decrease risk of constipation)
*Distraction Therapy – television, music (Brown, 2012)

Experimental Treatments
*Gene Therapy – Researchers exploring possibility of
inserting normal gene into bone marrow of children
with SCD to promote production of normal HgB
*Butyric Acid – A food additive that increases amount
of fetal hemoglobin (HbF) in blood
*Clotrimazole (Mycelex) – An OTC medication used
to treat fungal infections helps prevent loss of water
from RBCs and may reduce formation of sickle cells

(continued)
*Nitric Oxide – Helps keep blood vessels open and
reduces stickiness of RBCs, those with SCD have
lower levels of nitric oxide
*Nicosan – Herb used in Nigeria to prevent episodes
of sickle cell crisis
*Decitidine – A medication that increases the HgF
levels

(continued)
*GMI-1070 – Experimental pan-selection inhibitor
that treats vaso-occlusive crises, inhibiting a key
early step in the inflammatory process involved
with cell adhesion
*Bone Marrow Transplant – A potentially curative
treatment, must come from matched donor, usually
a family member who does not have SCD
(Gill, Lavin, & Sim, 2010)

Nursing Implications & Key Points
*Education (Personal)
- Important due to misconceptions about
patients with SCD, usually perceived as “drug-seeking”
- Pain management (i.e.: Morphine vs. Demerol)
- Health maintenance/promotion
- Assessment skills for signs & symptoms of crisis
- Psychological factors and SCD (Valentine et al., 2010)

(continued)
*Care of the Patient with SCD
- Adequate pain management
- Enhanced communication skills
- Familiarity of cultural dynamics
- Utilization of the nursing process (assessment,
intervention, re-assessment, and evaluation)

(continued)
- Recognition of SCD patient in crisis
(cardiovascular accident, acute chest syndrome,
organ damage from SCD)
- Monitoring labs (CBC, Fe, TIBC, renal function
electrolytes, bilirubin, ALT)
- Vital Sign monitoring (for early detection of
respiratory failure, shock, cardiovascular
accident, acute chest syndrome)

(continued)
- Neurologic assessment (noting any changes
in behavior, slurred speech, headaches,
vomiting)
- Infectious screening (mid-stream urine,
sputum
culture) Note: infections are common
precipitants of acute vaso-occlusive crises
- Promotion of well-balanced diet--high in
protein and fiber

(continued)
- Psychological support--patients experience
fear, anxiety, and stress caused by having an
unpredictable condition
- Fluid monitoring (Accurate intake & output)
- Empathy, compassion, a non-judgmental
attitude, and taking time to listen are extremely
helpful in assisting a patient’s recovery

(continued)
- Identify patient’s mental capacity and
willingness to adjust when changes in health
circumstances make that necessary
- Patient education—medications, coping
mechanisms for pain, early recognition of crisis
- Advocate for patient in accessing other services
(Social Services, Occupational Therapy, Support
groups)

(continued)
- Discharge planning (rest until energy levels
increase, pain management, hydrate, well-balanced
diet {dietician may be advantageous
in relation to cultural foods}, review of
medications, follow-up appointments with
primary physician and/or hematologist)
- Achievable goal setting for patient (pain
management, coping strategies)
(Brown, 2012)

Conclusion
SCD is a very complex genetic condition,
showcased by episodes of excruciating unpredictable
pain. Many people can and do lead active lives and
cope well with their illness, having developed a
number of coping strategies. “Patients can live with
their condition and not live by it” and should be
encouraged to do so (Brown, 2012, p. 96).

References
Alexander, J. et al. (2012). Drug information handbook
for advanced practice nursing (13th). Hudson,
OH: Lexicomp.
Brown, M. (2012). Managing the acutely ill adult with
sickle cell disease. British Journal of Nursing,
21(2), 90-96.
Gill, V., Lavin, J., & Sim, M. (2010). Managing sickle.
Nursing Made Incredibly Easy, 24-31.
doi:10.1097/01.NME.0000388522.79370.d2

References (continued)
McCance, S. (2010). Pathophysiology: The biologic basis for
disease in adults and children (6th). Maryland Heights,
MO: Mosby.
Rees, D., Williams, T., & Gladwin, M. (2010). Sickle-cell
disease. Lancet, 376, 2018-2031.
doi:10.1016/S0140-6736(10)61029-X
Sangkatomvong, S., Khoo, M., Kato, R., Detterich, J., Bush, A.,
Keens, T., Meiselman, H.,…Coates, T. (2011).
Peripheral vasoconstriction and abnormal parasympa-thetic
response to sighs and transient hypoxia in sickle
cell disease. American Journal of Respiratory and
Critical Care Medicine, 184, 474-481.
doi:10.1164/rccm.201103-05370C

References (continued)
Valente, S., Alexander, J., Blount, M., Fair, J., Goldsmith, C., &
Williams, L. (2010). Sickle cell disease in emergency
department: Education for emergency nurses. JOCEPS:
The Journal of Chi Eta Phi Sorority, 54(1), 11-14.
Wells, B., DiPiro, J., Schwinghammer, T., & DiPiro, C. (2013).
Pharmacotherapy handbook (8th). New York:McGraw-
Hill.

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