sca nsg mgt 3.pptx

8/14/2019 sca nsg mgt 3.pptx

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Sickle Cell

Diseaseanagement in Nursing

Prospectus


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p


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an inherited disorder in which there are

inadequate healthy red blood cells (RBCs) to

transport oxygen to the body's tissues.

In SCD, the RBCs are shaped like a half moon (or

sickle) and rigid, obstructing capillary blood flow andcausing severe pain.


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Sickle cell disease is a multisystem

disease. It is a congenitalhaemoglobinopathy inherited in anautosomal recessive manner.


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STRUCTURE OFHEMOGLOBIN

Hb is a conjugated proteinmolecules consist of twopairs of polypeptide chain( alpha and beta)Each globin chain bears aheme group whose centraliron atom is the site towhich the O2 attaches tohemoglobin


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TYPES OF HEMOGLOBIN

HbA: 2 Alpha and 2 beta chains.Hb A2: 2 Alpha and 2 delta chains.Hb F: 2 alpha and 2 gamma chains.Hb S: Valine substituted for Glutamicacid.Hb C: Lysine substituted for Glutamic

Acid.


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Types of hemoglobinopathies

Sickle cell trait (Hb AS)- adequateamount of normal hemoglobin ispresent, they are carriers, do not have

symptoms of SCDSickle cell anemia (HbSS)-mostsevere form of disease

Hemoglobin C (HbSC)Hemoglobin E (HbSE)Hemoglobin S beta thalassemia-mild

form of sickle cell disorder


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Pathophysiology

SCA is an autosomal recessivegenetic disease that results fromsubstitution of Valine from glutamic

acid in position 6 of beta globin geneleading to production of defective formof hemoglobin (Hb S)

Hemoglobin S a is structurallydefective hemoglobin


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Change in the properties ofSickle cell RBC

Deoxygenation leads to hydrophobicinteraction between adjacent Hb Smolecules

Distortion of RBC into sickle cell formRapid hemolysisDecreased elasticity of the cell wall ofRBCDecreased lifespan to 10-20 daysClogging of RBC in microcirculation


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Traits and Disease


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Diagnosis and screening Hemoglobin electrophoresis of cord blood can beused to screen newborns for SCD. The sickleturbidity test-a finger-stick test with a rapid result-isused to screen children ages 6 and up. Hemoglobinelectrophoresis can then confirm the diagnosis. DNAanalysis provides the most accurate diagnosis, butit's expensive and not widely available.

Routine hematologic tests (CBC and ferritin levels)

are done to evaluate anemia. The child's height andweight are routinely measured to identify failure tothrive. Routine assessment includes a history of painand signs of infection and inflammation. As the childbecomes older, genetic counseling will be needed.


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SCDa

lifetime ofsymptoms.


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anemia . Sickle cells arefragile, breaking apart easilyand dying after 10 to 20 days.Normal RBCs live for about120 days. This results in adecreased amount of oxygendelivery to tissues. Individualstypically experience fatigue,pallor, tachypnea, jaundice,

and shortness of breath whenanemia is severe .

SCD usuallymanifests after 4to 6 months ofage because

initial sickling isinhibited by high

levels of fetalhemoglobin


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s t roke . Stasis and clumping of the irregularlyshaped RBCs can result in cerebral occlusionand stroke manifested by headache, vomiting,

seizure, weakness, paralysis, and motordisturbances

v i s ion p rob lem s . Problems with visionoccur if the sickle shaped cells block theflow of blood in the small vessels in the

eyes. These cells can actually damagethe retina, uvea, and optic nerve.

Proliferative sickle cell retinopathy maycause vitreous hemorrhage and retinal

detachment with loss of vision.


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hepatomegaly . Liverinvolvement is present in40% to 80% of individualswith SCD. Acute attackswith pain in the right upperabdominal quadrant andhemolysis lead to theproduction of gallstones .

pain episodes. Painoccurs when the

irregularly shaped RBCscause stasis of the bloodwith clumping of cells in

the microcirculation,ischemia, and infarction.

This vaso-occlusive crisisis the most common type

of sickle cell crisis andlasts for days or weeks. Itfrequently occurs in theabdomen, back, joints,

and chest areas


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delayed grow th . RBCs provide neededoxygen and nutrients for growth anddevelopment. Children with SCD areoften underweight and have delays indevelopment, including puberty. Frequenthospitalizations may interfere with schooland social development .

f requent infect ion s . Sickle cellscan damage the spleen, an organ

that fights off infections. Childrenwith SCD are more vulnerable tobacterial infections. Infants and

young children can actually die inas little as 12 hours from the onset

of fever.


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hand-foo t synd rom e . Anearly indication of SCD is

swollen hands and feet. This

occurs because the sickleshaped cells prevent blood

flow out of these extremities.Pain, mobility, and activity

impairments result.


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HAND-FOOT SYNDROME


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psyc hos oc ial i s sues . Depressionand disturbed self-conceptassociated with a chronic diseaseoften occur in individuals with SCD.

priapism. Men of any age with SCDmay experience painful erection of

the penis that can last for hours,days, or weeks. This usually occursduring sleep and is often associatedwith dehydration, hypoventilation, or

a full bladder. Men with SCD are alsoat increased risk for erectile

dysfunction.


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SICKLE CELL CRISISVaso occlusive crisis

Aplastic crisesHemolytic crisisSplenic sequestration crisesInfectious crises


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The crisis is a common complication insickle-cell patients and can beassociated with one or more symptomsincluding fever, cough,excruciating pain,sputum production, dyspnea or hypoxia

TreatmentBroad spectrum antibiotics to cover commoninfections like strep pneumoniae andmycoplasma, pain control, and bloodtransfusion. Acute chest syndrome is anindication for exchange transfusion.PrognosisIt may result in death, and it is one of the mostcommon causes of death for sickle cell patients


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Aplastic crises

Pallor, dyspnea, lethargy, stupor andpossibly coma

Aplastic crises are acute worsening of

the patient's baseline anaemia,producing pallor, tachycardia, andfatigue.

triggered by parvovirus B19 Mostpatients can be managed supportively;some need blood transfusion.


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Infectious crisis

Infectious crises is due to functionalasplenia and decreased level of serumImmunoglobulin M increasingsusceptibility to infectionHaemophilius influenzae,streptococcuspnemoniae, mycoplasma pnemoniae,salmonella typhimurium, staphylococcusaureus, escherichia coliPneumonia, bronchitis, pyelonephritis,cystitis,osteomyelitis, meningitis,andsepsis


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Splenic sequestration crisis

Blood volume pools in the spleen causingsplenic enlargement, and in severe cases,hypovolemic shock acute, painful enlargements of the spleen,

caused by intrasplenic trapping of red cellsand resulting in a precipitous fall inhemoglobin levels with the potential forhypovolemic shock. Sequestration crises are

considered an emergency. If not treated, patients may die within 1 2hours due to circulatory failure.

Management is supportive, sometimes with

blood transfusion.


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Supportive and preventivetreatments


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B. ADMINISTER FOLIC ACID REPLACEMENT, IF

INDICATED TO SUPPORT ERYTHROPOIESESC. PROVIDE SUPPORT TO CLIENT AND FAMILYReview genetic implication and refer forcounselling as necessary

Encourage participation in support groupsEncourage verbalization from the client andfamily

D. MANAGE PRIAPISM. Instruct the client to

empty his bladder at onset of attack, exercise andtake a warm bath. .INFORM about experimental medications such ashydroxyurea, pentoxifylline, citidiel citrate) and food

additives(VANILLA) which is evaluated in treatingSCA


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.TO CLIENT IN SICKLE CELLCRIS ES

ADM INISTER PRESCRIBEDMED ICATIONS(OPIOID

ANALGESICS)

INCREASE ORAL OR IV FLUIDINTAKE.MONITOR I&OUSE SMALL GAUGE NEEDLE TOPRE VENT TRAUMA

ADM INISTER BLOODTRA NSFUSION AS PRESCRIBEDENC OURAGE TO REST AND

SUPPORT AFFECTED JOINTS
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PRINCE FAISAL CANCER CENTER

Buraidah, Al-Qassim

PAIN ASSESSMENT - RE ASSESSMENT FORM

Patients Name: _____________________________ Age /Sex/Nationality: ______________ Consultant: ______________________

Medical Record Number: _____________________ Diagnosis : __________________________ Date of Admission: ___________

LOCATION QUALITY THERAPEUTIC ADVERSE EFFECT

1. Head 7. UE or LE A Aching R- Radiating N Nausea R Respiratory Depression2. Neck 8. Operation Site B- Burning S Stabbing V Vomiting A Allergic Reaction3. Shoulder 9. Genital area C Cramping Sh - Sharp D Dizziness H- Hemodynamic change4. Chest 10. Flank H- Heavy T - Throbbing C Confusion5. Abdomen 11. Generalize E Electrical Co - Constipation6. Back 12. Others

DATETIME

PAIN SCORE

109876543210

LOCATIONQUALITY

PHARMACEUTICAL **ALTERNATIVE

INTERVENTION*

THERAPEUTICADVERSE EFFECTREASSESSMENT

SCORENURSE NAME &

SIGNATUREKEY: UE Upper extremities LE Lower Extremities ** See Medication Sheet * See Nurses NotesNOTE: REASSESSMENT of pain post intervention must be c ompleted within 15 minutes to 1 hourPOST ANALGESIC: ORAL Analgesics in 1 hour IV - Analgesics in 15 minutes SL - Analgesics in 15 minutes

IM - Analgesics in 30 minutes SC - Analgesics in 20 minutes PR - Analgesics in 15 minutes

PFCC.Med.Fm.39

Pain Rating Scales


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ALGORITHM Assess pain severity

Use splints/slings/dressingsetcConsider other causes of

distress*Consider regional block s

MILD PAIN (1-3)Oral paracetamol

orOral ibuprofen MODERATE PAIN (4-6)

As for mild painplus oral

diclofenac (unless alreadyhad

ibuprofen)or ibuprofen

orcodeine phosphate

SEVERE PAIN (7-10)

Consider Entonoxinitially

IV diamorphine ormorphine

0.1-0.2 mg/kgor

Rectal anti-inflammatory

Supplemented byoral

analgesics


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Strategize for success

Nursing strategies for SCD include:

Manag e acu te cr ises . Teach effect ive pain m anagem ent

measures . Teach effect iv e l i fes ty le m od if ication s . Ins t ruc t w hen to co ntac t the heal thcare

prov ider . Teach s t r ess m anagem ent tech niq ues . Encourage the ackn ow ledg m ent o f

feel ings .


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Thank you

sca nsg mgt 3.pptx

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