Sickle cell anemia

Sickle cell anemia for nursing student

1. Dharmendra Patel
1
st
Year M.Sc. Nursing
Nootan College Of Nusing
Sickle cell AnemiaSickle cell Anemia

2. Introduction
•Sickle cell anaemia is a serious disease in which the body makes
sickle-shaped ("c"-shaped) red blood cells. Normal red blood cells
are disk-shaped and move easily through your blood vessels. Red
blood cells contain the protein haemoglobin.
•Sickle cells contain abnormal haemoglobin that causes the cells to
have a sickle shape, which don’t move easily through the blood
vessels – they are stiff and sticky and tend to form clumps and get
stuck in the blood vessels.
•The clumps of sickle cells block blood flow in the blood vessels
that lead to the limbs and organs. Blocked blood vessels can cause
pain, serious infections, and organ damage.

3. CharacteristicsCharacteristics ofof sicklesickle cellscells
RBCSRBCS

120 day life span120 day life span

Hb has normal oxygen Hb has normal oxygen
carrying capacitycarrying capacity

12-14 g/12-14 g/dl of Hb of Hb
SICKLED CELLSSICKLED CELLS

30-40 day life span30-40 day life span

Hb has decreased Hb has decreased
oxygen carrying oxygen carrying
capacitycapacity

6-9 g/6-9 g/dl of Hb of Hb

4. Pathological defect.
Sickle cell haemoglobinopathies are a group of diseases in
which normal adult haemoglobin ( haemoglobin A or Hb A) is
partially or completely replaced by a variant haemoglobin:
sickle haemoglobin (Hb S) or haemoglobin C ( Hb C),among
other.the molecular structures of these haemoglobins differ.
Normal haemoglobin a contains four polypeptide chain,two
alpha and two beta chain,each with a characteristic amino acid
sequence.
The alpha chain contains 141 amino acid, the beta chain 146
amino acids.
The precise sequence of these amino acid have been established.

5. The nature of the differnces between Hb A,Hb
S, and Hb C is determined by the one amino
acid in the beta polypeptide chain in the sixth
position.this position is occupied by glutamic
acid in Hb A, by valine in Hb S,and by a
lysine residue in Hb C. in all of these
polypeptide chains,the sequence of the other
145 amino acids is the same.
Cont…Cont…

6. Types of sickle cell disease.
The four main types of sickle cell anemia are caused by
different mutations in these genes.
1)Hemoglobin SS disease

Hemoglobin SS disease is the most common type of
sickle cell disease. It occurs when you inherit copies of
the hemoglobin S gene from both parents. This forms
hemoglobin known as Hb SS. As the most severe form
of SCD, individuals with this form also experience the
worst symptoms at a higher rate.

8. Cont…Cont…
2 ) Hemoglobin SC disease
Hemoglobin SC disease is the second most common
type of sickle cell disease. It occurs when you inherit the
Hb C gene from one parent and the Hb S gene from the
other. Individuals with Hb SC have similar symptoms to
individuals with Hb SS. However, the anemia is less severe

9. Cont…Cont…

3 ) Hemoglobin SB+ (beta) thalassemia

Hemoglobin SB+ (beta) thalassemia affects beta
globin gene production. The size of the red blood cell is
reduced because less beta protein is made. If inherited with
the Hb S gene, you will have hemoglobin S beta
thalassemia. Symptoms are not as severe.

10. Cont…Cont…
4 ) Hemoglobin SB 0 (Beta-zero) thalassemia
Sickle beta-zero thalassemia is the fourth type of sickle
cell disease. It also involves the beta globin gene. It has
similar symptoms to Hb SS anemia. However, sometimes
the symptoms of beta zero thalassemia are more severe.

11. Cont…Cont…
Sickle cell trait
People who only inherit a mutated gene (hemoglobin S)
from one parent are said to have sickle cell trait. They may
have no symptoms or reduced symptoms.

12. Sign & symptomsSign & symptoms
•• excessive fatigue or irritability, from anemiaexcessive fatigue or irritability, from anemia
•• bedwetting, from associated kidney problemsbedwetting, from associated kidney problems
•• jaundice, which is yellowing of the eyes and skinjaundice, which is yellowing of the eyes and skin
•• swelling and pain in hands and feetswelling and pain in hands and feet
•• frequent infectionsfrequent infections
•• pain in the chest, back, arms, or legspain in the chest, back, arms, or legs

13. Other Clinical ManifestationsOther Clinical Manifestations

CNSCNS

HemorrhageHemorrhage

ParalysisParalysis

CVSCVS

CHFCHF

Heart rate increasedHeart rate increased

14. Cont…Cont…

PulmonaryPulmonary

AtelectasisAtelectasis

PneumoniaPneumonia

Pulmonary hypertensionPulmonary hypertension

Resp. rate increasedResp. rate increased

GITGIT

Acute hepatomegalyAcute hepatomegaly

SpleenomegalySpleenomegaly

15. Cont…Cont…

GUGU

HematuriaHematuria

DiuresisDiuresis

MusculoskeletalMusculoskeletal

Painful swelling of hands & feetPainful swelling of hands & feet

OphthalmologyOphthalmology

BlindnessBlindness

RetinopathyRetinopathy

Dermatology Dermatology

Ulcers of hand &feet Ulcers of hand &feet

16. Diagnostic testDiagnostic test
••Blood counts can reveal an abnormal Hb level Blood counts can reveal an abnormal Hb level
in the range of 6 to 8 grams per deciliter.in the range of 6 to 8 grams per deciliter.
••Blood films may show RBCs that appear as Blood films may show RBCs that appear as
irregularly contracted cells.irregularly contracted cells.
••Sickle solubility tests look for the presence of Sickle solubility tests look for the presence of
Hb S.Hb S.
Hb electrophoresisHb electrophoresis
Hb electrophoresis is always needed to Hb electrophoresis is always needed to
confirm the diagnosis of sickle cell disease. It confirm the diagnosis of sickle cell disease. It
measures the different types of hemoglobin in measures the different types of hemoglobin in
the blood.the blood.

17. Medical managementMedical management

Hydroxyurea & erythropoietin increase fetal Hydroxyurea & erythropoietin increase fetal
Hb.Hb.

Transfusion therapyTransfusion therapy

Oxygen therapyOxygen therapy

Analgesics: morphine,meperidineAnalgesics: morphine,meperidine

Nutritional supplementsNutritional supplements

Monitoring vital signsMonitoring vital signs

Genetic counselling.Genetic counselling.

Immunizations can help prevent infectionsImmunizations can help prevent infections

Psychological therapiesPsychological therapies

18. ComplicationsComplications

Increased risk of severe bacterial infectionsIncreased risk of severe bacterial infections
stroke
cholelithiasis (gallstones) and cholecystitis.
 Hyposplenism

Osteomyelitis Osteomyelitis

19. NsgNsg mgmtmgmt

Impaired skin integrity r/t altered circulation to Impaired skin integrity r/t altered circulation to
tissuestissues

Risk for injury r/t joint swelling.Risk for injury r/t joint swelling.

Position pt in sitting position& change positionPosition pt in sitting position& change position

Remove constrictive clothingRemove constrictive clothing

Give frequent & small feedingsGive frequent & small feedings

20. Cont..Cont..

Altered renal tissue perfusion r/t to cell Altered renal tissue perfusion r/t to cell
sicklingsickling

Inspect for edema for signs of impaired renal Inspect for edema for signs of impaired renal
function.function.

Measure wt I/O to assess renal statusMeasure wt I/O to assess renal status

Observe urine for colour,content & odourObserve urine for colour,content & odour

Maintain fluid intake.Maintain fluid intake.

21. Thankyou!Thankyou!