Sickle Cell Anemia 2007

8/14/2019 Sickle Cell Anemia 2007

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Sickle CellSickle CellAnemiaAnemia


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Sickle cell

anemia is a disease

passed downthrough families in

which red blood

cells form anabnormal crescent

shape.

DefinitionDefinition


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Causes/EtiologyCauses/Etiology Sickle Cell Anemia is an inherited disorder

in which a child inherits one abnormal genefrom each parent. This is known as autosomalrecessive inheritance.


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These abnormal genes produce an abnormaltype of blood hemoglobin called hemoglobin

S.RBC containing HbS loses its round, pliable,

biconcave disk shape and becomesdeformed, rigid and sickled-shaped

Sickle-shaped cells deliverO2 to the

body's tissues. They also can clog moreeasily in small blood vessels, and breakinto pieces that disrupt blood flow, thepatient may have pain, swelling and fever

If the RBC is again exposed to adequateamount of oxygen before the membranebecomes too rigid, it can revert to anormal shape

Cold can aggravate the sickling process


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NON MODIFIABLE

1. Hereditary 2. Race Sickle cell anemia is most common in

people of African, Indian, Middle Eastern, orMediterranean descent. Nevertheless, it canoccur in all peoples.

Risk FactorRisk Factor


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Signs and symptoms of sickle cell anemiausually show up after an infant is 4 months old Includes:

Anemia RBCs usually live for about 120 days

before they die and need to be replaced.However, sickle cells die after only 10 to 20days.

Without enough red blood cells incirculation, your body can't get the oxygen itneeds to feel energized.

1.

Signs and SymptomsSigns and Symptoms


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Delayed growth

RBCs provide your body with the oxygen and nutrients

you need for growth

Shortage of healthy red blood cells can slow growth in

infants and children and delay puberty in teenagers

Jaundice

It is a yellowing of the skin and eyes that occurs

because of liver damage or dysfunction

Sickle cell anemia have some degree of jaundice
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Hand-foot syndrome Swollen hands and feet may be the

first signs of sickle cell anemia inbabies. The swelling is caused by sickle-shaped red blood cells blocking bloodflow out of their hands and feet.

Frequent infection Sickle cells can damage your spleen,

an organ that fights infection. This maymake you more vulnerable to infections.


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1year old and up Vision Problem

Tiny blood vessels that supply your eyes maybecome plugged with sickle cells. This can

damage the retina (portion of the eye that

processes visual images).

Episodes of pain (Sickle Cell Crisis)

Pain develops when sickle-shaped red blood cells

block blood flow through tiny blood vessels to yourchest, abdomen and joints. Pain can also occur in

your bones.

The pain may vary in intensity and can last for a

few hours to a few weeks.


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Test Definition Purpose

Hemoglobinelectrophoresis

Hemoglobinelectrophoresis isa test thatmeasures thedifferent types ofthe oxygen-carryingsubstance

(hemoglobin) inthe blood.

You may

have this testif your healthcare providersuspects thatyou have adisordercaused byabnormalforms of

hemoglobin

Diagnostic TestDiagnostic TestNormalresult

Abnormality withcondition

No HbS Presence of HbS


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Sickle Cell

Test

A sicklecell testlooks forthe presence

or absenceof abnormalhemoglobinin the bloodthat causes

sickle cellanemia.

This test isdone to tellif you haveabnormal

hemoglobinthat causessickle celldisease andsickle cell

trait.

Negative Sickle Cell

Trait

Sickle Cell

Anemia


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CompleteBloodCount(CBC)

-It measuresthe # of RbCs,# of WBCs,

total amountof Hb in the

blood, % ofthe bloodcomposed ofRBCs(hematocrit)

-provides

specificinformationthe size andhemoglobincontent of

individual redblood cells.

It is ascreening testused todiagnose andmanagenumerousdiseases.

RBC:Male:4.7 to 6.1million

cells/mcL

Female: 4.2 to5.4 million cells/mcL

WBC:4,500 to10,000 cells/mcL

Hgb:

Male: 13.8 to 17.2gm/dL

Female: 12.1 to15.1 gm/dL

Platelets:1500,000-400,000/L

decrease(indicates

anemia)

Chronicneutrophilia isoften

present

decrease5-10g/dL

oftenincrease


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Nursing Management Provide information about what the client may feel

Encourage questions and dialogue about fears andapprehensions

Inform the client of the time period before theresults will be available


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ManagementManagement Medical

Patients with sickle cell disease need ongoingtreatment, even when they are not having apainful crisis.

Classification: Analgesic

Action: It is used for the relief of fever as well as

aches and pains associated with many

conditions.

2.1.


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2 . Hydroxyurea

Classification: Antimetabolite

Action: inhibits production of Hb F, which inhibits sickling

Indication: to reduce frequency of painful crises and

need for blood transfusions in adult

patient with Sickle Cell Anemia with recurrentmoderate to severe painful crises

Generic (Brand) names: Hydroxyurea (Droxia,

Hydrea)


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3.Blood Transfusion

In a red blood cell transfusion, red blood cells are

removed from a supply of donated blood. These donated

cells are then given intravenously to a person with sickle cell

anemia.

Blood transfusions increase the number of normal

red blood cells in circulation, helping to relieve anemia. In

children with sickle cell anemia at high risk of stroke, regular

blood transfusions can decrease their risk of stroke.
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4.Supplemental oxygen

Breathing supplemental oxygen through a breathingmask adds oxygen to your blood and helps you breathe

easier.
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Non Pharmacological-Highlights

1.Supplements of Folic Acid(Vitamin B)

2.Fluids

3.Heating pads


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SurgicalBone marrow transplant

This procedure replaces bone marrowaffected by sickle cell anemia with healthybone marrow from a donor who doesn't havethe disease. It can be a cure, but theprocedure is risky, and it's difficult to find

suitable donors. Bone marrow used for a transplant must

come from a closely matched donor. This isusually a close family member who doesn'thave sickle cell anemia.

Bone marrow transplant requires alengthy hospital stay. After the transplant,you'll need drugs to help prevent rejection ofthe donated marrow.


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Diagnosis: Acute pain related to tissue hypoxiaInterventions: -promote calm and relaxing environment to

divert clients awareness to pain -divertional activities like watching television,

use of imagery etc. -perform warm compress on the site of pain

to provide relief -encourage families to maintain a normal life

for the child with SC AnemiaOutcome: Client will be able to relieve pain.

Nursing DiagnosisNursing Diagnosis


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Diagnosis: Risk for infection related to inadequatesecondary defense

Interventions: -promote childhood immunization program -encourage adults to update immunizations to

reduce the transmission of global infection -emphasize necessity of taking

antiviral/antibiotics -review individual nutritional needs,

appropriate exercise program and need for rest -encourage frequent proper hand hygieneOutcome: Client will be able to prevent the spread of

potentially infectious microorganism.


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Diagnosis: Impaired gas exchange related tooxygen carrying capacity of the blood

Interventions: -review oxygen-conserving techniques -instruct in the use of relaxation, stress

reduction techniques -encourage appropriate activity and

exercise to improve quality of life -emphasize the importance of nutrition in

improving stamina and reducing the work of

breathingOutcome: Client will be able to have an

adequate oxygenation of tissue.


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In the past, sickle cell patients often diedfrom organ failure between ages 20 and 40.

Thanks to a better understanding andmanagement of the disease, today, patients can

live into their 50s or beyond.

Causes of death include organ failure andinfection. Some people with the disease

experience minor, brief, infrequent episodes.Others experience severe, long-term, frequentepisodes with many complications.

PrognosisPrognosis


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Red grape skin may help sickle cell sufferers(October31, 2009)

"AUGUSTA, Ga. An extract in red grape skin maybe a new treatment for sickle cell disease, Medical

College of Georgia researchers say.Study Using Viagra Ingredient to Treat Sickle CellCut Short (July 28, 2009, 2:03 PM) Viagra treats erectile dysfuntion by widening arteries.

Viagras active ingredient also has been approved, under thename Revatio, to treat pulmonary hypertension, the conditionraising blood pressure in the lungs. And thats why researcherswere studying whether Revatio might also work in patients withsickle-cell disease, a genetic blood disorder.

www.newstin.com.uk

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Brunner and Suddaraths Textbook of Med-Surgical Nursing 11th Edition

Kozier ans Erbs Fundamentals of Nursing

Tabers Cyclopedia Medical Dictionary, F.A. Davis Disease and disorders Nursing Therapeutic

Manual

Http://en.wikipedia.org/wiki/Sickle_Cell_Disease

http://www.nlm.nih.gov/medlineplus/ency/article/000527.htm

http://www.mayoclinic.com/health/sickle-cell-anemia/DS00324

Nursess Pocket Guide 11th Edition

RReferenceeference

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Sickle Cell Anemia 2007

Documents

Transcript of Sickle Cell Anemia 2007