St.

Dominic College of Arts and Sciences

Emilio Aguinaldo Highway, Talaba IV, Bacoor, Cavite

College of Nursing

A Case Study
Diabetes Mellitus Type II

“Hinog na Mangga”

Presented by:

Group 1

Agcaoili, Jenalyn
Aranzaso, Christian
Columna, Liezel
Hierco, Rica Bianca
Legayada, Mary Jerah
Manigsaca, Melizen
Paraiso, Joanna
Romero, Jelica
Turla, Jordina

1
 CHAPTER 1

INTRODUCTION

H.S or commonly known as Hereditary Spherocytosis is an inherited blood disorder

where a metabolic defect causes defects in the red blood cell membranes which lead to their

characteristic spherical shape, a condition that is mild and requires no specific therapy.

Hereditary Spherocytosis is a disease that results in the formation of abnormal red blood

cells with fragile cell walls. Red blood cells circulate in the blood and contain hemoglobin,

which carries oxygen to all parts of the body. The shape of a normal red blood cell resembles a

disc. Normal red blood cells easily change shape to move effectively through the small blood

vessels between organs of the body. A person with Hereditary Spherocytosis has red blood cells

that are very round and have difficulty changing in shape. The lack of ability to change shapes

makes moving through the small blood vessels difficult. Therefore, the red blood cells stay in

the spleen longer than normal. This lengthy stay in the spleen damages the cell membranes.

Eventually, the spleen will destroy these red blood cells.

Paleness or yellow color of the skin or eyes, stomach pain, shortness of breath, irritability

in children, fever and vomiting; these are some of the signs and symptoms of Hereditary

Spherocytosis. Symptoms of Hereditary Spherocytosis vary depending on the severity of the

disease

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 Hereditary Spherocytosis (H.S) is an inherited disease that causes anemia. If a child has

Hereditary Spherocytosis, either parent may also have the disease. Occasionally, neither parent

of an affected child has the disease; this is considered a spontaneous mutation.

We choose Hereditary Spherocytosis as our case study because of its relevance to

immunology. Immunology covers the study of all aspects of the immune system in all

organisms. It deals with the function of the immune system in states of both health and disease,

and the inability of the immune system to function in immunological disorders such as

autoimmune diseases; like Hereditary Spherocytosis, in which the immune system attacks its

own host’s body.

BACKGROUND OF THE STUDY

One of the cases that we handled in Bautista General Hospital in Cavite, City is

Hereditary Spherocytosis. Hereditary Spherocytosis is a rare condition that captured the interest

of the researchers to further study the case.

Our patient is a 10 months old baby boy. He was admitted in Bautista General Hospital

with a yellowish color of the skin because of his present condition that has been acquired from

his mother. This condition is passed down from parents to children. A parent with the disease

has a 50% chance of having a child with the disease. It is more common in people whose

ancestors came from Northern Europe. The incidence of Hereditary Spherocytosis is about 200-

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300 per million in Northern Europe populations but this is likely to be an underestimate. In other

parts of the world, the disease is thought to be less common. Unfortunately, the statistics in the

Philippines is still unavailable.

STATEMENT OF THE PROBLEM

1. What is the demographic profile of the client?

• Age

• Gender

2. What are the different assessment parameters of a patient with Hereditary Spherocytosis?

3. What are the different nursing diagnoses formulated based on the client’s situation?

4. What are the nursing diagnoses that should be prioritize?

5. What nursing intervention can be formulated based on the identified problem?

6. What are the client’s responses based on the implemented nursing interventions?

SIGNIFICANCE OF THE STUDY

TO CLIENT/HIS FAMILY

This study is for the family to know and understand better the importance of seeking

medical assistance.

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 For them to understand further, the disease and be able to cope gradually with whatever

changes that the patient will go through regarding his condition.

TO NURSING SERVICE DEPARTMENT

This study will provide facts and nursing managements about this disease; which will be

a great assistance for them to provide the students to have sufficient knowledge about the disease

and how to deal with patients who are suffering from it.

TO NURSING EDUCATION

This study will provide the information about Hereditary Spherocytosis. Because of the

facts and managements regarding to this disease will be beneficial in teaching the students.

TO THE STUDENTS

This study will serve as a reference for the nursing students about patients with

Hereditary Spherocytosis. Also, for them to gain knowledge and be aware on how to give proper

nursing managements to the client suffering to this condition.

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TO THE COMMUNITY HEALTH CENTER and/or CITY HEALTH OFFICE

This study will provide knowledge about this disease which will be helpful for the

services of the community to educate those who are suffering from this condition, the family and

other people who need the information about it.

TO THE FUTURE RESEARCHERS

The result of this study will serve as a guide for future reference about future researches

on the study of Hereditary Spherocytosis.

SCOPE AND LIMITATION

This study covers only the information about Hereditary Spherocytosis that has relevance

to our topic; immunology. This study is limited only to Hereditary Spherocytosis which is an

autoimmune disease that can be classified as an immunologic disorder. This study will not tackle

any topic beyond the disease and immunology.

And also this study will not provide the Philippine health statistics about Hereditary

Spherocytosis.

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 CHAPTER II

REVIEW OF RELATED LITERATURE

Diagnosis of Hereditary Spherocytosis in Newborn Infants

The normal range of red blood cell osmotic fragility and autohemolysis was determined in

venous blood of 32 healthy newborn infants. With these normal ranges as a reference, the

diagnosis of hereditary spherocytosis was definitely possible in five newborn infants by

demonstration of increased osmotic fragility of fresh and incubated red blood cells, moderately

increased autohemolysis, and partial reduction of autohemolysis by the addition of glucose.

Most previously studied newborn infants with hereditary spherocytosis have had atypical

hyperbilirubinemia. Inconstant signs are anemia, reticulocytosis, erythroblastosis, spherocytes in

the blood smear, and increased mean corpuscular hemoglobin concentration. In newborn infants

with atypical hyperbilirubinemia in whom blood group incompatibilities are excluded, studies for

hereditary spherocytosis should be done.

Schröter W, Kahsnitz E.

http://www.ncbi.nlm.nih.gov/pubmed/6886914

Neonatal Hyperbilirubinemia due to Heredotary Spherocytosis

Hereditary Spherocytosis is a rare cause of neonatal hyperbilirubinemia and Medline search from

1966 onwards revealed only one case from India. Fifty percent of patients with Hereditary

Spherocytosis give a history of jaundice in the neonatal period but it is often passed over as

physiological jaundice. We present a neonate who was diagnosed after seeing spherocytes on the

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peripheral smear. A term, 2.9 Kg baby born to a B+ve primi gravida mother developed jaundice

at 3 days of age. His serum bilirubin was 18mg/L (direct component 1 mg/L). His PCV was 40,

reticulocyte count was 12%, G6PD levels were normal, blood group was B+ and peri-pheral

smear examination revealed sphero-cytes. Mother gave a history of splenectomy at 10 years of

age and she had been diagnosed to have spherocytosis. She has been asympto-matic with a

hemoglobin of 11 g/dL. Baby’s DCT was negative, MCV was 84.2 fl and MCHC was 36.2%.

Osmotic fragility was significantly increased. Hemolysis started at 0.72% NaCI and completed

at 0.56% NaCl as against the control sample in which hemolysis started at 0.48% NaCl and

completed at 0.4% NaCl. Phototherapy was given for 72 hours during which serum bilirubin

initially rose to 21 mg/dL and subsequently decreased to 10mg/dL. Patient was started on oral

folic acid and parents were counseled as to long term outcome.

Diagnosis of Hereditary Spherocytosis is suspected when sphero-cytes are seen on the peripheral

smear. Sphero-cytes can occasionally be seen in normal newborns, in ABO incompatibility and

autoimmune hemolytic anemia. Blood grouping and DCT help to rule out the latter two. The

mean corpuscular hemoglobin concentrations (MCHC) are elevated (35-36%) and MCV is in the

normal range as seen in our patient. Osmotic fragility may be done using neonatal controls as

the newborn RBC is relatively more resistant to hemolysis. Treatment in the neonatal period is

directed towards treating hyperbilirubinemia. Rarely, packed cell transfusion for symptomatic

anemia may be required. The definitive treatment is splenectomy which is best deferred till 5

years of age.

http://www.indianpediatrics.net/feb2004/feb-199.htm

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Newborn Hematology

Significant red cell disorders in the newborn period may be associated with a family history of

anemia, jaundice, falling hemoglobin and reticulocytosis in addition to abnormal RBC

morphology. The presence of surface antibodies (e.g., anti-A and/or anti-B) may be helpful or

the deficiency of intraerythrocytic enzymes (e.g., G6PD deficiency). Finally hemoglobino-

pathies and thalassemia may offer an almost infinite combination of symptoms and signs in the

newborn. Virtually all red cell problems are isolated and the rest of the hemogram is normal.

Marrow failure resulting in pancytopenia with associated infections and altered hemostasis is

vary rare. Definitive studies on red cells can be delayed for three months when the infant is well

and their blood volume is larger. As in the above case, studies of maternal family members

subsequently confirmed the diagnosis of Hereditary Spherocytosis. It is important to remember

that in the perinatal period (28 weeks gestation to 28 days after birth) there are normal

physiologic changes occurring in red cells. Some of these changes include switching from fetal

to adult hemoglobin production, a 30% drop in hemoglobin, a fall in mean red cell volume

(MCV) as well as changes in membrane pliability and intracellular enzyme levels. RBC survival

is further impacted by acquired infections, medications, and other high-risk conditions.

Repeated monitoring of hemoglobin and reticulocyte counts is warranted in the sick and unstable

newborn. Hydrops fetalis results from severe intrauterine hemolysis and anemia necessitating

emergency exchange transfusion. The cause is usually due to severe alpha thalassemia, red cell

surface antibodies or congenital infections. Rh incompatibility usually presents with rapid

hemolysis and varying degrees of anemia and jaundice (depending on the extent of maternal

sensitization and antibody production).

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White blood cell abnormalities may be asymptomatic and incidental or associated with fever,

infection, and altered host resistance. Wide fluctuation in the WBC count can be noted

depending on marrow production of granulocytes. Infection, antibodies, and medications call all

affect the circulating neutrophil pool. This is the pool of granulocytes sampled with a

venipuncture. Interpretation of a peripheral WBC count can be difficult when trying to

determine the risk of infection or underlying pathophysiology. A bone marrow examination can

be very helpful in assessing granulocyte production and the risk of infection. Most WBC

aberrations are secondary or reactive to a disease process, but occasionally rare hereditary

disorders can present in the neonate. These are usually associated with an increased risk of

infection (e.g., chronic granulomatous disease) or as part of a recognizable syndrome (e.g., Jobs

syndrome). Congenital leukemia is extremely rare but striking leukemoid reactions can occur.

Of note is the transient myeloproliferative condition or pseudoleukemia that may be observed in

neonates with Down syndrome. Of prime importance when evaluating infants with any white

cell aberration is the real risk of infection and the need for antimicrobials.

Platelet problems in newborns present almost exclusively with thrombocytopenia and bleeding.

Thrombocytopenia can be isolated or associated with other cytopenias. A low platelet count

results from either decreased production or peripheral consumption of platelets. By examining

the bone marrow and/or the mean platelet volume (MPV usually higher in younger platelets seen

in consumptive disorders), the underlying pathophysiology can usually be identified. Most

thrombocytopenias are consumptive and due to maternal antiplatelet antibodies, congenital

infections, or part of complex DIC seen in sick newborns. It is paramount to compare the

amount of clinical bleeding to the degree of thrombocytopenia in order to quickly make the right

10
diagnosis and determine the need for transfusion or other therapy. Hemorrhagic disease of the

newborn is usually seen from day 2 to 4 of life resulting from vitamin K deficiency and the

subsequent failure to produce clotting factors II, VII, IX and X. The prothrombin time is

markedly prolonged and serious life threatening hemorrhaging can occur in many organ systems.

This transient deficiency of vitamin K is thought to result from poor placental transfer, marginal

content in breast milk, inadequate intake of breast milk and a sterile gut (lack of vitamin K

producing GI flora). Hemorrhaging can be prevented by the intramuscular administration of

prophylactic vitamin K shortly after birth.

Robert W. Wilkinson, MD

September 2002

Rubella Infection with Autoimmune Hemolytic Anemia

Autoimmune hemolytic anemia(AIHA) is uncommon cause of anemia but must be considered in

differential diagnosis if the patient has a concomitant hymphoproliferative disorder, autoimmune

disease, viral or mycoplasmal infection. Positive direct atiglobulin test ( Coombs’ test)

represents a central criterion for detection of anti-red blood cell (RBC) alloantibodies. High-titer

cold agglutinin (CA) may be induced by certain infectious agents, including mycoplasmal

pneumonia, EBV, CMV, rubella virus or may develop chronic (malignant) B cell

lymphoproliferation.

Autoantibodies against red cells optimally reacting at 0 degree C, i.e. CA, are normally found

with low titers in the serum of human adults. Cold reactive autoantibodies account for 16-32% of

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all immune hemolysi. In children autoimmune hemolysis is a rare event but is mild and mostly

followed by acute infections.

Neerja Agrawal, Rahul Naithani, and M. Mahapatra

Indian Journal of Pediatrics, Volume 74-May 2007

SYNTHESIS

Hereditary Spherocytosis is an inherited disease. It is caused by the formation of

abnormal red blood cells in the fragile cell wall. Red blood cells are being trapped because they

cannot change their shape easily; that is why they stay longer in the spleen and damaging the cell

membrane. After circulating, cell eventually damaged that is destroyed by the spleen.

To confirm diagnosis of Hereditary Spherocytosis; “osmotic fragility test” is being done.

If the patient is having hyperbilirubinemia it is indicated treatment to have blood transfusions.

But when a patient is at the age of five (5) and older he can undergo splenectomy ( surgical

removal of the spleen). Since the Hereditary Spherocytosis is inherited the pregnant mother who

is suspected to have this disease should under go prenatal check up. During perinatal period the

normal physiologic changes occurring in the red cells.

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 ANATOMY AND PHYSIOLOGY

Blood is the “river of life” that surges within us. It transports everything that must be

carried from one place to another within the body – nutrients, wastes (headed for elimination

from the body), and body heat – through blood vessels.

Blood Cell Formation

Blood cells are produced, in adults, by the red bone marrow located within the spongy

bone found in the flat bones of the skeleton – skull, collar bones, shoulder blades, sternum, ribs,

vertebrae, and hip bones, - and in the epiphyses of the femur and humerus. The cells that divide

to produce blood cells are stem cells called haemocytoblast. Their derivatives differentiate to

form either red blood cells, granulocytes, agranulocytes, or platelets. While Granulocytes

continue to mature in bone marrow, the precursors of monocytes and lymphocytes move to the

lymph nodes, spleen, and other organs of the lymphatic system and continue their development

there.

Red blood cells are produced, by a process called erythropoiesis at the rate of about 2

million per second. They have a life span of about 120 days, at which point they are worn out

and need replacing. Old red blood cells are processed in the spleen and liver and their iron

content is recycled. Red blood cells production and destruction is regulated by the hormone,

13
erythropoietin, produced by the kidneys. This hormone responds to reductions in blood oxygen

levels, produced perhaps by moving to a higher altitude, by increasing the rate of erythropoiesis.

Production of white blood cells, known as leucopoiesis, is controlled by a number of hormones.

Most granulocytes die within days as a result of combating pathogens; monocytes live for several

months; whereas lymphocytes, the backbone of the immune system, can live for many decades.

Red Blood Cells

Erythrocytes or red blood cells make up about 99 percent of blood cells, with some 5

million cells per cubic millimetre of blood, outnumbering white blood cells by 800 to one. Their

function is to supply oxygen to all body cells. Each red blood cell is flattened disc with a

dimpled centre, giving it biconcave profile. This shape provides a large surface area, relative to

the cells volume, for taking up and releasing oxygen. Red blood cells are also small – at about 7

micrometers, just narrower than the diameter of the smallest capillaries – and have a natural

elasticity, which enables them to squeeze along narrow capillaries in single file. Red blood cells

lack a nucleus; instead they are packed with the protein haemoglobin, which gives them their red

colour. As red blood cells passed through the lungs, where oxygen concentration is high,

haemoglobin picks up oxygen to form oxyhaemoglobin; when red blood cells pass through the

tissues, where oxygen levels are low because of its constant consumption by cells in cell

respiration, oxyhaemoglobin unloads it’s oxygen and becomes haemoglobin. Oxyhaemoglobin

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 gives arterial blood its bright red colour, while haemoglobin gives venous blood its dark red

colour.

Red blood cells of individual with Hereditary Spherocytosis

The shape of a normal red blood cell resembles a disk. Normal red blood cells easily

change shape to move effectively through the small blood between organs of the body. A person

with spherocytosis has red blood cells that are very round and have difficulty changing this

shape. The lack of ability to change shapes makes moving through the small blood vessels

difficult. Therefore, the red blood cells stay in the spleen longer than normal. This lengthy stay

in the spleen damages the cell membranes. Eventually, the spleen will destroy the abnormal red

blood cells.

The size of many of these RBC's is

Normal red blood cells are small cells
shaped like biconcave disks- flattened disks with
depressed centers. Because of their thinner
centers, they look like miniature doughnuts
when viewed with a microscope. Their small
size and peculiar shape provide a large surface
area relative to their volume, making them
ideally suited for gas exchange.
quite small, with lack of the central zone of
pallor. These RBC's are spherocytes. In
hereditary spherocytosis, there is a lack of
spectrin, a key RBC cytoskeletal membrane
protein. This produces membrane instability
that forces the cell to the smallest volume--a
sphere. In the laboratory, this is shown by
increased osmotic fragility. The spherocytes do
not survive as long as normal RBC's.
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 Spleen

Situated to the left of the stomach, and served by the splenic artery and veins, the spleen

is the largest of the lymph organs. It has two main roles. First, it removes aging red blood cells

from the blood and salvages the iron for later use. Second, its macrophages remove pathogens

and cell debris from the blood flowing though its sinuses (blood spaces), while lymphocytes

initiate the immune response. White pulp consists of fibres carrying lymphocytes, while red pulp

consists of blood sinuses and areas of connective tissue rich in macrophages.

Spleen of an individual with Hereditary Spherocytosis

An individual who has a Hereditary Spherocytosis has an enlarged spleen. And cells

destroy quickly and red pulp has a greater amount than white pulp.

IMAGE INSIDE THE SPLEEN, WITH IMAGE INSIDE THE SPLEEN, WITH
NORMAL BLOOD CIRCULATION WITHIN HEREDITARY SPHEROCYTOSIS

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 PATHOPHYSIOLOGY OF HEREDITARY SPHEROCYTOSIS

Atypical blood film?

Family History of HS; Recent infection; No
typical clinical and family history of HS
laboratory features

Screen proband and

family for abnormal
Dominant Inheritance RBCs

Normal test
HS RBCs result
detected in
siblings but
No further Variable clinical normal parents
Investigation severity in
needed different family
members
protein defect
(causes sphere
shape of RBC)

Search for co-inheriting

haematological disorder

ß-thalassemia or None
sickle cell disease

Paleness Lack of Lack of Stomach Shortness Irritability Fever and

or yellow energy appetite pain of in vomiting
of the breath children
skin
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 CHAPTER III

RESEARCH METHODOLOGY

Research Design

The descriptive method of research was utilized in this study. This study contains only

the facts about the disease which is Hereditary Spherocytosis and the important information

about the patient. The researchers use this kind of study to add further knowledge to everyone

about this disease.

Research Environment

The researchers conducted this study at the second floor ward of Bautista General

Hospital, Cavite City. The institution has a 70-bed capacity catering various services like; CT

Scan service, Internal Medicine, Obstetrical Gynecologist, Pediatrics, Anesthesiologist,

Ophthalmology, Radiology, Urologist, Cardiology, Pulmonology, Orthopedics, Psychiatrics,

Physical Therapy, Rehabilitation Medicine, ICU, Delivery Room, Operating Room and

Emergency Room.

Research Respondent

The research respondent was the father and the mother of a 10 month old baby with a

Hereditary Sperocytosis. They are presently residing at Cavite City.

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Research Instrument

The researchers made use of the following sources of information to gather all the

necessary data needed in conducting this study.

• Interview

The researchers made an initial interview with the father of the 10 month old

baby to provide additional information about the past health history of the infant. It was

done at Bautista General Hospital last August 15, 2008.

• Physical Assessment

The researchers performed physical assessment to distinguish any

manifestation of any abnormalities on the child that can be used as baseline data in

formulating the necessary care plan to the patient.

• Review of Records

The researchers reviewed the secondary sources of data such as the patient’s

chart to further add some details about the patient. And it will help the researchers to find

necessary care.

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 CHAPTER IV

PRESENTATION AND ANALIZATION OF DATA

DEMOGRAPHIC PROFILE

Patient’s name: Baby Face

Age: 10 months old

Gender: Male

Address: Cavite City

History of past illness:

Baby Face was born at Bautista General Hospital on October 3, 2007. After 12 hours

Baby Face was noted “yellowish in color or jaundice” as describe by the father. And after 24

hours he was again noted as ‘hinog na mangga“ as describe by the father. October 7, 2007 four

days after his birth, his first (1st) blood transfusion was done at the same hospital. October 27,

2007 was the second (2nd) blood transfusion at Bautista General Hospital.

Baby Face was brought to Philippine Children Hospital last October 30, 2007 and had

gone under the TORCH test (Toxoplasmosis, Other, Rubella, Cytomegalovirus, Herpes). This

test will determine if the patient is positive in rubella virus. The result is positive; therefore the

patient is a carrier of rubella virus. After a couple of weeks the third (3rd) blood transfusion was

done at Bautista General Hospital on November 13, 2007.

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 November 14, 2007 the client was brought to Asian Hospital for the second opinion

because there is no diagnosis on what is the real disease of the patient. A CBC (Complete Blood

Count) test was done. However there is no improvement in the status of patient so the parents

decided to go to the specialist at Fe Del Mundo Hospital in Banawe, Quezon City for the

monitoring of the CBC (Complete Blood Count). November 30 same year bone marrow test was

done in Fe Del Mundo Hospital.

Due the distance of the Fe Del Mundo Hospital the parents of the client decided to

transfer at Asian Hospital for the monitoring of CBC. Still there is no improvement on the

laboratory test results of the client. December 21, 2007 was the fourth (4th) blood transfusion

done at Bautista General Hospital.

January 2008 at Asian Hospital the reticulocytes test was performed. Where in this test

will determine the production of the Red Blood Cell (RBC). February 1, 2008 the Fifth (5th)

blood transfusion was done again at Bautista General Hospital. Then next (6th) blood

transfusion was done on March 29, 2008.

June 6, 2008 was the seventh blood transfusion at the same hospital. Then July 9, 2008 at

Philippine General Hospital, OFT (Osmotic Fragility Test) was done to detect red blood cells

that are more fragile than normal. This test is performed to detect if it is Hereditary

Spherocytosis or Thalassemia. However the client wasn’t diagnose at Philippine General

21
Hospital but in Asian Hospital last July 25, 2008, under the service of the specialist in said

hospital. And recently August 13, 2008 the eighteenth (8th) blood transfusion was done in our

10-6 night shift duty at Bautista General Hospital.

During our interview to the father of the patient he said that the disease was inherited

from the mother who also undergone a TORCH test (Toxoplasmosis, Other, Rubella,

Cytomegalovirus, Herpes) at National Kidney Institute in which the result is positive in Rubella.

History of Present illness:

Admitted last June 2008; underwent Blood transfusion

(+) pallor, (-) DOB, CBC due ended, Decrease Hemoglobin level

Patient Clinical Record

Admitting diagnosis: Hereditary Spherocytosis

Chief Complain: Pallor

Reason for Admission: for evaluation and management

Special treatment done in Bautista General Hospital:

Transfuse 80cc of Packed red blood Cell type “O”

Then After 4 hours give another 80cc of Packed red blood cell

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Patient’s blood type: O (Rh +)

Blood component: PRBC (Packed Red Blood Cell)

Source: PNRC (Philippine National Red Cross)

Physical assessment

General survey

Skin color: Pale looking

SKIN

Areas to assess Findings

Characteristics Pale looking

Temperature Cold to touch

Elasticity Normal, no signs of edema

Lesions No lesions

HAIR

Areas to assess Findings

Characteristics Thin and evenly distributed

SCALP

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 Areas to assess Findings

Characteristics Shiny and smooth without lesions, masses or

mumps
Deformities No trauma deformities

Redness or scaliness No redness or scaliness

SKULL

Areas to assess Findings

Characteristics Rounded and smooth skull contour without any

sings of enlargement.
Symmetry of facial features and movement Symmetrical in facial features and movement

EYES

Areas to assess Findings

Conjunctiva and lens Conjunctiva and lens are yellow in color

Nails

Areas to assess Findings

Capillary refill Delayed capillary refill

ABDOMEN

Areas to assess Findings

Skin color Pale looking

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 After physical assessment there were no abnormalities expect for pale skin and delayed

capillary refill due to decrease in the level of Hemoglobin.

BASELINE MEASUREMENTS

Temperature: 36.2 °C

Respiratory rate: 25 cpm

Cardiac rate: 120 bpm

Hematology

Hematology Normal values Result

Hemoglobin 7.4-9.9 mmol/L 6.4 mmol/L
Hematocrit 0.37-0.48 0.19
Red Blood Cell 4.2-5.9 x 10 12/L 2.2 x 10 12 /L
White Blood Cell 4.3-10.8 x 10 9/L 8.9 x 10 9 /L1
Neutrophils 0.54-0.75 0 .55
Lymphocytes 0.25-0.40 0.43
Monocytes 0.02-0.08 0.02
Platelet Count 150-350 x 10 9 /L 310 x 10 9/L
White blood cell, neutrophil, lymphocytes, monocytes and platelet count is normal. The

red blood cell, hemotocrit and hemoglobin are decreased which indicate that the patient is

anemic. This is due to hemolysis that happened in the spleen area. To correct this result blood

transfusion is needed.

Hematology

Hematology Normal values Result

25
Hemoglobin 7.4-9.9 mmol/L 7.6 mmol/L
Hematocrit 0.37-0.48 0.40
Red Blood Cell 4.2-5.9 x 10 12/L 4.5 x 10 12 /L
White Blood Cell 4.3-10.8 x 10 9/L 8.9 x 10 9 /L1
Neutrophils 0.54-0.75 0 .55
Lymphocytes 0.25-0.40 0.43
Monocytes 0.02-0.08 0.02
Platelet Count 150-350 x 10 9 /L 310 x 10 9/L

After the blood transfusion the result in the Complete Blood Count (CBC) returns to

normal range.

CHAPTER V

CONCLUSION AND RECOMMENDATION

CONCLUSION

This case study will help significant individuals to better understand Hereditary

Spherocytosis. What it is, how it will affect the normal process of the lymphatic system to one

individual, and what are several changes it can bring to a person’s life? Based on the case

presented, with the support of literatures and research study on Hereditary Spherocytosis, the

researchers firmly believe on the following concepts.

An effective and comprehensive nursing management should be formulated and

implemented in both clinical and home setting; in order to provide an optimum care for a patient

with Hereditary Spherocytosis. A proper health teaching is an important tool for nurse’s and its

26
primary responsibility should always be prioritize and should be given an emphasis for its

management.

The treatment for Hereditary Spherocytosis, in young children (up to five years of age)

should take folic acid supplements. Blood transfusions may help with severe anemia and

surgical removal of the spleen (splenectomy) in children five years of age or older. This does not

cure that patient of spherocytosis; rather it allows red blood cells to live longer. Without a spleen

a person has an increased risk for some serious infections. Therefore, patients who have their

spleen removed need to take penicillin for the rest of their lives. Several special immunizations

(pneumococcal and meningococcal) are also required to help prevent some infections.

RECOMMENDATION

The research study brought about a great deal, gives some additional information in

enhancing nursing care practice and deep responsibility with regards to our nursing practice. For

this reasons, this case study recommend the following concepts which may be consider vital in

the care management of Hereditary Spherocytosis.

For client and family, to be able for them to understand the disease and to know what

are the factors they need to consider for seeking some medical assistance for the patient suffering

to Hereditary Spherocytosis.

27
 For nursing department, this study will provide them to have idea and sufficient knowledge

about this kind of disease that the patient was suffering.

For nursing education and nursing students, this study will provide some important

information about Hereditary Spherocytosis and this research study will serve as references for

the nursing student to be guided and to have an idea on how to provide a proper nursing care

management for the client having this kind of disease.

For the community health center and city health office, which will benefit to this study to

provide some information and idea about this disease and will serves as a references that will

help for them to have knowledge about this uncommon disease.

For the future researchers, it will be beneficial to have knowledge regarding to the

Hereditary Spherocytosis; which is an auto-immune disease that may inherit from parents to their

children. It is also necessary to have a background regarding to this kind of disease which is

very difficult to have and we should familiarize ourselves on the signs and symptoms of this kind

of auto-immune disease.

We should support our nursing management with vital health teaching by spreading basic

necessary information regarding predisposing factors that can lead of having Hereditary

Spherocytosis. Clients must be fully aware of their condition and also to the public.

28
29
 NURSING CARE PLAN

ASSESSMENT NURSING EXPECTED INTERVENTIONS RATIONALE EVALUATION

DIAGNOSIS OUTCOME

Subjective: Impaired gas After 8 hours of  Monitor vital signs  To note for any Goal met.
“namumutla at exchange nursing changes in the After 8 hours of nursing
nanghihina ang anak related to interventions, the vital signs of the intervention the patient
ko,” as verbalized by decrease patient will be able patient return his normal skin
the father of the hemoglobin to demonstrate color and becomes
patient. level secondary improvement in  Encourage limitations of  To reserve livelier
to abnormal red skin color and activities within patient’s patient’s oxygen
blood cell becomes livelier. tolerance consumption
Objective: structure
• Pale and weak
looking  Promote calm environment  To prevent patient
• Cold to touch from being
• Irritable stimulated
• Fearful to
stranger  Monitor patients condition  To assess for
• Delayed capillary during blood transfusion and allergic reaction
refill refer to nurse on duty for any and give
adverse reaction immediate
intervention
Vital Signs:
PR –120 bpm
RR –25 bpm Dependent:
Temp. –36.2 C  Assist with the procedure of  To replace blood
blood transfusion as components that
indicated are loss (RBC)

 Monitor CBC (complete  To monitor if

blood count) as ordered by there is any
the physician. changes after
blood transfusion

30
 ASSESSMENT NURSING EXPECTED INTERVENTIONS RATIONALE EVALUATION
DIAGNOSIS OUTCOME

Objective: Risk for infection After 8 hours of  Monitor vital signs  To note for Goal met.
• Pale and weak related to inadequate nursing interventions, any changes After 8 hours of
looking secondary defenses the patient will be able in the vital nursing interventions,
• Cold to touch (decreased to maintain a condition signs of the the patient was able to
• Delayed capillary hemoglobin level) free from signs of patient maintain a condition
refill infection that is free from signs
 Maintain the IV site  To prevent of infection
sterile invasion of
Vital Signs: pathogens
PR –120 bpm
RR –25 bpm  Keep the bed of the  To provide
Temp. –36.2 C patient clean comfort

 Change linens as  To prevent

needed soiled linens
which may be
a carrier that
may cause
infection

 Stress proper hand  To avoid

washing techniques to cross-
all caregivers before contamination
and after handling the
patient

 Encourage adequate  To promote

rest and provide comfort and
conducive environment wellness

31
 CONCEPT MAP

Name: Baby Face

Age: 10 months
Gender: Male

Pale and weak looking

Cold to touch
Irritable
Delayed capillary refill
Fearful to stranger

Vital signs:
PR – 120 bpm
RR – 25 bpm
Temp. – 36.2 C

1. Impaired gas exchanged related 2. Risk for infection related to

to decreased hemoglobin level inadequate secondary defenses
secondary to abnormal red blood (decreased hemoglobin level)
cell structure

32
 INTERPRETATION OF CONCEPT MAP

1.) Based on the assessment done, the patient is pale and weak looking and has a delayed

capillary refill and so the researchers came up with the diagnosis of Impaired Gas Exchanged.

With regards to the patients’ disease, the blood circulation is mainly affected. Due to the

abnormal structures of the red blood cells the patient was experiencing inadequate oxygenation

internally which is manifested in his body externally. In refer to the principle of ABC, (Airway,

Breathing and Circulation) circulation should be one of the prioritization. It is the main problem

that was seen on the patient that needs to be attended immediately. Proper independent and

dependent nursing interventions were done in able to attend the immediate needs of the patient in

order to bring him back to his normal status and prevent further complications.

2.) The second prioritized nursing diagnosis is Risk for Infection. The researchers believed that

the patient is more prone to infections due to his present condition. Because of the decreased

level of secondary defenses of the patient, he is more susceptible to acquire certain infection that

may cause further complication to his condition.

33
 DRUG STUDY

DRUG NAME MECHANISM OF CONTRAINDICATION NURSING

ACTION INDICATION ADVERSE EFFECTS RESPONSIBILITY

GENERIC NAME: - essential for TREATMENT - undiagnosed GI: - monitor vital

production of FOR : megaloblastic signs
FOLIC ACID certain coenzyme in anemia - disturbances
many metabolic - anemia - pernicious -assess for the
BRAND NAME:
systems such as aplastic or - hypersensitivity capillary refill
FOLVITE
purine and normocytic reactions
pyrimidine anemia - monitor skin
CLASSIFICATION: synthesis. It is also - bronchospasm appearance
essential un the
SUPPLEMENTARY synthesis and - instruct parents to
VITAMINS maintenance of encourage to have
nucleoprotein in healthy lifestyle
STOCK DOSE: erythropoesis. It
also promotes WBC
1 mg and platelet
production in folate
DOSAGE
deficiency anemia
0.05 mg/day

ROUTE:

PO

35
 DRUG NAME MECHANISM OF CONTRAINDICATION NURSING
ACTION INDICATION ADVERSE EFFECTS RESPONSIBILITY

GENERIC NAME: - antagonizes the relief of - hypersensitivity CNS: - monitor vital

effects of histamine allergic - acute attacks of signs
CETERIZINE at H1-receptor sites; symptoms asthma - dizziness
does not bind to or caused by - instruct
BRAND NAME:
inactive histamine. histamine - drowsiness relatives/parents to
ZYRTEC
Anticholinergic release take medication as
effects are minimal including: - fatigue directed
CLASSIFICATION: and sedation is dose
related EENT: - advise parents to
ANTIHISTAMINE - Seasonal and have their child a
perennial - pharyngitis good oral hygiene,
STOCK DOSE: allergic frequent rinsing of
rhinitis GI: mouth with water
5 mg to minimize dry
- chronic - dry mouth mouth
DOSAGE :
urticaria
2.5 mg

ROUTE:

PO

36
 BIBLIOGRAPHY

Wikipedia

emedicine

http://www.emedicine.com/med/topic2147.htm

healthline

http://www.healthline.com/galecontent/spherocytosis-hereditary

medline plus

http://www.nlm.nih.gov/medlineplus/ency/article/000530.htm

pubmed

http://www.ncbi.nlm.nih.gov/pubmed/6886914

http://www.uptodate.com/patients/content/topic.do?topicKey=~ssGsCp44Dl1KDex

http://www.uptodate.com/patients/content/topic.do?topicKey=~cJmJmzii/tSB/gJ&selecte
dTitle=1~30&source=search_result

http://cancer.seattlechildrens.org/conditions_treated/hereditary_spherocytosis/

http://ashimagebank.hematologylibrary.org/cgi/content/full/2001/1205/100214

http://www.itppeople.com/splenectomy.htm

http://www.cincinnatichildrens.org/health/info/blood/diagnose/spherocytosis.htm

http://hereditaryspherocytosis.org/whatisit.html

http://www.medterms.com/script/main/art.asp?articlekey=3724

37
 PATHOPHYSIOLOGY OF HEREDITARY SPHEROCYTOSIS

Patient Presenting with Haemolytic

Anaemia

Family History of HS; Atypical blood film?

typical clinical and Recent infection; No
laboratory features family history of HS

Dominant Inheritance Screen proband and

family for abnormal
RBCs

No further Variable clinical HS RBCs Normal

Investigation severity in detected in test result
needed different family proband &
members siblings but
normal parents

Not membrane

Search for co-inheriting SDS PAGE for ?

haematological disorder protein defect thalassemia
?CDA ?MDS

ß-thalassemia or None DNA analysis for low- expression

sickle cell allele Recessive or non dominant
disease inheritance in proband with no family
history

38
 Presentation Presentation Presentation Presentation
about about about about

Hereditary Spherocytosis Hereditary Spherocytosis Hereditary Spherocytosis Hereditary Spherocytosis

Entitled: Entitled: Entitled: Entitled:

“hinog na mangga” “hinog na mangga” “hinog na mangga” “hinog na mangga”

By: By: By: By:

BSN III-D BSN III-D BSN III-D BSN III-D

Group 1 Group 1 Group 1 Group 1

On: On: On: On:

September 19, 2008 September 19, 2008 September 19, 2008 September 19, 2008