RBC’s Hemoglobin

What is Hemoglobin?

Hemoglobin is found in all RBC’s and carries oxygen from the lungs to tissues and organs throughout the
body.

Normal RBC – soft, smooth, round and can move easily through the body

A WOMAN WITH SICKLE-CELL ANEMIA

Definition:

 Sickle-cell anemia is a recessively inherited haemolytic anemia caused by an abnormal amino

acid in the beta chain of haemoglobin.
Signs and Symptoms:

 Anemia
 Episodes of pain
 Swelling of hands and feet
 Frequent infections
 Delayed growth or puberty
 Vision problems

Pathophysiology:
Change of one base-
pair in DNA molecule

Valine produced instead

of glutamic acid at
position 6 in Beta-chain

Abnormal
haemoglobin
molecule

Change in surface
tension of RBC

Sickling of RBC

Destruction of many
sickle RBC

ANEMIA
Nursing Management:

a. Nursing Assessment

Assessment data for a sickle cell anemia patient should include:

 Factors causing previous crisis. The patient is asked to identify factors that precipitated
previous crisis and measures the patient uses to prevent and manage the crisis
 Pain levels. Pain levels should always be monitored using a pain intensity scale.
 Characteristics of pain. The quality, frequency, and factors that aggravate or alleviate the
pain are included in the assessment.
 Infection. Because patients with sickle cell anemia are susceptible to infections, they are
assessed for the presence of any infectious process.

b. Nursing Diagnosis

Based on the assessment data, major nursing diagnosis for the patient include:

 Acute pain related to tissue hypoxia due to agglutination of sickled cells within

blood vessels.
 Risk for infection.
 Risk for powerlessness related to illness-induced helplessness.
 Deficient knowledge regarding sickle cell crisis prevention.

c. Nursing Goals

The major goals for the patient are:

Relief of pain.

Decrease incidence of crisis.

Enhance sense of self-esteem and power.

Absence of complications.
 d. Nursing interventions

for sickle cell anemia include:

Managing Pain

 Use patient’s subjective description of pain and pain rating on a pain scale to
guide the use of analgesic agents.
 Support and elevate any joint that is acutely swollen until swelling diminishes.
 Teach patient relaxation techniques, breathing exercises, and distraction to ease
pain.
 When acute painful episode has diminished, implement aggressive measures to
preserve function (eg, physical therapy, whirlpool baths, and transcutaneous
nerve stimulation).

Preventing and Managing Infection

 Monitor patient for signs and symptoms of infection.

 Initiate prescribed antibiotics promptly.
 Assess patient for signs of dehydration.
 Teach patient to take prescribed oral antibiotics at home, if indicated,
emphasizing the need to complete the entire course of antibiotic therapy.

Promoting Coping Skills

 Enhance pain management to promote a therapeutic relationship based on

mutual trust.
 Focus on patient’s strengths rather than deficits to enhance effective coping
skills.
 Provide opportunities for patient to make decisions about daily care to increase
feelings of control.

Increasing Knowledge
  Teach patient about situations that can precipitate a sickle cell crisis and steps to
take to prevent or diminish such crises (eg, keep warm, maintain adequate
hydration, avoid stressful situations).
 If hydroxyurea is prescribed for a woman of childbearing age, inform her that the
drug can cause congenital harm to unborn children and advice
about pregnancy prevention.

Monitoring and Managing Potential Complications

 Management measures for many of the potential complications are delineated in

the previous sections; additional measures should be taken to address the
following issues.

Medical and Surgical Intervention

Medical Management/Intervention:

1. Peripheral Blood Stem Cell Transplant

 PBSCT may cure sickle cell anemia. However, this treatment modality is available to only small
subset of affected patients, because of either the lack of a compatible donor or because severe
organ damage (eg., renal, liver, lung) that may be already present in the patient is a
contraindication for PBSCT.
2. Pharmacologic Therapy
 Hydroxyurea(Hydrea)- a chemotherapy agent, has been shown to be effective in increasing fetal
haemoglobin (ie, haemoglobin F) levels in patients with sickle-cell anemia, thereby decreasing
the formation of sickled cells
 Arginine- has antisickling properties and enhances the availability of nitric oxide, the most potent
vasodilator, resulting in decreased pulmonary artery pressure.
- May be useful in managing pulmonary hypertension and acute chest syndrome
3. Transfusion Therapy
- RBC transfusions have been shown to be highly effective in several situations: in an acute
exacerbation of anemia (eg, aplastic crisis), in the prevention of severe complications from
anesthesia and surgery, in improving the response to infection (when it results in exacerbated
anemia), and in severe cases of acute chest syndrome.
- in pregnant women, transfusions are also effective in diminishing the episodes of sickle cell
crisis but this does not improve fetal survival
4. Supportive Therapy
- Supportive care is equally important
A WOMAN WITH THALASSEMIA

Definition:


 Thalassemia are a group of autosomal recessively inherited blood disorders that lead to poor
haemoglobin formation and severe anemia.

G-6-Phosphate Dehydrogenase Deficiency

- G-6 PD is the source of the abnormality in this disorder, this gene produces an enzyme within
the erythrocyte that is essential for membrane stability.

2 Types of Thalassemia

1. Alpha – is the result of changes in the genes for the alpha globin component of haemoglobin
- Occurs when there is disturbance in production of α-globin form any or all four of the α-
globin genes
2. Beta- characterized by a genetic deficiency in the synthesis of beta-globin chains
- beta-globin is a component(sub unit) of hemoglobin

Signs and Symptoms:

 Fatigue
  Weakness
 Pale or yellowish skin (jaundice)
 Slow growth
 Abdominal swelling
 Dark urine

Pathophysiology:

Due to E/F

Disturbance of ratio
between the alpha
& beta globin chain
synthesis

Decrease
production of one
or more globin

Formation of
abnormal HB
structure

Ineffective
erythropoiesis

Excessive RBC
destruction

Iron overload

Extramedullary Thalassemia
hematopoiesis
Nursing Management:

1.

a. Nursing Diagnosis- ineffective tissue perfusion related to reduce cellular components as

evidence by cyanosis
b. Goal – To improve tissue perfusion
c. Nursing Intervention – monitor sign of hypoxia such as cyanosis, hyperventilation, increase
pulse rate, breath frequency and BP
- provide frequent rest periods to reduce oxygen consumption
- provide oxygen therapy to patients
- monitor vital signs
- observe any restlessness and confusion

2.

a. Nursing Diagnosis- activity intolerance related to imbalance of oxygen supply and consumption
need as evidence by fatigue
b. Goal- To improve the activity tolerance
c. Nursing Intervention- Assess the capability of doing activity.
- Monitor vital signs before and after the activity
- Create a schedule to perform the activities
- To stop activities if pulse, BP, respiration, fatigue or dizziness will increase
- Provide frequent rest periods to reduce oxygen consumption
- To administer blood products
a. Nursing Diagnosis- imbalance nutrition less than body requirements related to lack of appetiteas
evidence by weight and skin integrity
b. Nursing Goal- To maintain the nutritional level
c. Nursing Intervention-
- Allow to eat food that can be tolerated to improve the nutritional quality as the appetite
increase
- Provide nutritious food which include high caloric, high protein,, vitamins and minerals
- Check the weight daily
- Provide IV fluid if not able to take by mouth

Medical and Surgical Intervention

- The treatment is to arrest the source and stop the offending medication. Transfusion is
necessary only in the severe haemolytic state, which is more commonly seen in the
Mediterranean, variety of G-6-PD deficiency
- Treatment focuses on combating anemia through such measure such as folic acid
supplementation and perhaps blood transfusion to infuse haemoglobin-rich red blood cells.
- Women with thalassemia do not usually take an iron supplement during pregnancy because
they could receive an iron overload because iron is infuse with blood transfusions.
A WOMAN WITH MALARIA

Definition:

 Malaria is a protozoan infection that is transmitted to people by “Anopheles”mosquitoes. The

infection causes red blood cells to stick to the surface of the capillaries causing obstruction of
these vessels and resulting in end-organ anoxia and blood not reaching organs effectively.
 The incubation period for the most frequently occurring type is 12-14 days.

Signs and Symptoms:

 Elevated liver function tests

 Fever
 Chills
 Malaise
 Headache
 Nausea and Vomiting

Complications:

During pregnancy malaria can lead to :

 Premature delivery
 Spontaneous abortion
  Stillbirth
 Low-birth-weight infants

Etiology/Pathophysiology:

Mosquito bite from an

infected Anopheles
mosquito

Saliva is injected under

the skin

Will travel to the

bloodstream as
MEROZOITES

Migrate to the liver as SYPTOMATIC INFECTION

HEPATOCYTES

TISSUE SCHIZONT Released in the bloodstream as

Chills (Cold Stage)
MEROZIOTES

Remain dormant in liver RBC invasion and destruction Low RBC Count
as HYPNOZOITES
TROPHOZOITE

Will undergo maturity

Relapsing Malaria

(P. vivax; P. ovale) SCHIZONT

Production of the next

generation of MEROZOITES

Invasion of previously WOF Blackwater fever

uninfected RBC (Hematuria)

Blood will be taken up by an

anopheles mosquito

GAMETOCYTES

Will undergo maturity

Will produce male and
female GAMETES
Nursing Management:

a. Nursing Diagnosis – activity intolerance

- Acute pain
- Anxiety
- Decreased cardiac output
- Deficient fluid volume
- Fatigue
- Hyperthermia
- Impaired gas exchange
- Impaired physical mobility
- Impaired skin integrity
- Risk for infection
- Risk for injury
b. Nursing Intervention
- assess the patient on admission and daily thereafter for fatigue, fever, orthostatic,
hypotension, disorientation, myalgia, and arthralgia
- enforce bed rest during periods of acute illness
- institute standard precautions
- to reduce fever, administer antipyretics as ordered
- administer analgesic as ordered
- keep a strict record of I/O
- Observe blood chemistry levels for hyponatremia and increased BUN, creatinine, and
bilirubin levels
- Monitor urine output hourly
- Watch for and immediately report signs of internal bleeding, such as tachycardia,
hypotension, and pallor
- Watch for adverse effects of drug therapy and take measures to relive them
- Provide emotional support and reassurance, especially in critical illness

Medical and Surgical Intervention

 - Treatment is with a combination of antimalarial drugs, which will both stop the course of the
disease and help reduce the incidence of low birth weight and preterm birth

DRUGS:

 Sulfadoxine/pyrimethamine – is safe to administer during the last trimester of

pregnancy
 Chloroquine- is safe to administer all during pregnancy and so is the drug of choice
 Quinine, atovaquone and proguanil, or tetracyclines – although effective against the
disease, should not be used at any point in pregnancy or with women who are
breastfeeding as they are teratogenic

Sources:

1. www.mayoclinic.org
2.