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

*Average life span 45-65 years

(Brown, 2012).

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)

Histidine LeucineValine Threonine Glutamic acidProline Glutamic acid

*The 6th amino acid in the Beta-globin chain becomes

valine instead of glutamic acid

Valine Histidine Leucine Threonine Proline Valine Glutamic acid

Normal Beta Hemoglobin Chain

Sickle Cell Beta Chain

(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

-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)

(Kiera105.wordpress.com)

Pharmacologic Treatment of SCD (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


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)


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


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)


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)

*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)

(Sciencephoto.com)

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

Experimental Treatments (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

Experimental Treatments (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)

Nursing Implications & Key Points (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)


- 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)


- 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


- 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


- 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)


- 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.

Healthcare

Smith k sickle_cell