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Hereditary Spherocytosis

Hereditary Spherocytosis

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03/20/2013

St.

Dominic College of Arts and Sciences
Emilio Aguinaldo Highway, Talaba IV, Bacoor, Cavite

College of Nursing

A Case Study

Hereditary Spherocytosis
“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

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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 hemoglobinopathies 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

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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,

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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.

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.

The size of many of these RBC's is 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. 15

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

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PATHOPHYSIOLOGY OF HEREDITARY SPHEROCYTOSIS Family History of HS; typical clinical and laboratory features Atypical blood film? Recent infection; No family history of HS

Dominant Inheritance

Screen proband and family for abnormal RBCs

No further Investigation needed

Variable clinical severity in different family members

HS RBCs detected in siblings but normal parents

Normal test result

protein defect (causes sphere shape of RBC) Search for co-inheriting haematological disorder

ß-thalassemia or sickle cell disease

None

Paleness or yellow of the skin

Lack of energy

Lack of appetite

Stomach pain

Shortness of breath

Irritability in children

Fever and vomiting

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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. transfusion was done on March 29, 2008. Then next (6th) blood

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

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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 Characteristics Temperature Elasticity Lesions Pale looking Cold to touch Normal, no signs of edema No lesions Findings

HAIR Areas to assess Characteristics Findings Thin and evenly distributed

SCALP

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Areas to assess Characteristics Deformities Redness or scaliness

Findings Shiny and smooth without lesions, masses or mumps No trauma deformities No redness or scaliness

SKULL Areas to assess Characteristics Symmetry of facial features and movement Findings Rounded and smooth skull contour without any sings of enlargement. Symmetrical in facial features and movement

EYES Areas to assess Conjunctiva and lens Findings Conjunctiva and lens are yellow in color

Nails Areas to assess Capillary refill Findings Delayed capillary refill

ABDOMEN Areas to assess Skin color Findings 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

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Hemoglobin Hematocrit Red Blood Cell White Blood Cell Neutrophils Lymphocytes Monocytes Platelet Count

7.4-9.9 mmol/L 0.37-0.48 4.2-5.9 x 10 12/L 4.3-10.8 x 10 9/L 0.54-0.75 0.25-0.40 0.02-0.08 150-350 x 10 9 /L

7.6 mmol/L 0.40 4.5 x 10 12 /L 8.9 x 10 9 /L1 0 .55 0.43 0.02 310 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

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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.

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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.

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NURSING CARE PLAN ASSESSMENT Subjective: “namumutla at nanghihina ang anak ko,” as verbalized by the father of the patient. Objective: • Pale and weak looking • Cold to touch • Irritable • Fearful to stranger • Delayed capillary refill Vital Signs: PR –120 bpm RR –25 bpm Temp. –36.2 C NURSING DIAGNOSIS Impaired gas exchange related to decrease hemoglobin level secondary to abnormal red blood cell structure EXPECTED OUTCOME INTERVENTIONS RATIONALE  To note for any changes in the vital signs of the patient  To reserve patient’s oxygen consumption  To prevent patient from being stimulated  To assess for allergic reaction and give immediate intervention EVALUATION Goal met. After 8 hours of nursing intervention the patient return his normal skin color and becomes livelier

After 8 hours of  Monitor vital signs nursing interventions, the patient will be able to demonstrate improvement in  Encourage limitations of skin color and activities within patient’s becomes livelier. tolerance  Promote calm environment

 Monitor patients condition during blood transfusion and refer to nurse on duty for any adverse reaction

Dependent:  Assist with the procedure of blood transfusion as indicated  Monitor CBC (complete blood count) as ordered by the physician.

 To replace blood components that are loss (RBC)  To monitor if there is any changes after blood transfusion

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ASSESSMENT

NURSING DIAGNOSIS Risk for infection related to inadequate secondary defenses (decreased hemoglobin level)

EXPECTED OUTCOME

INTERVENTIONS

RATIONALE

EVALUATION

Objective: • Pale and weak looking • Cold to touch • Delayed capillary refill Vital Signs: PR –120 bpm RR –25 bpm Temp. –36.2 C

After 8 hours of  Monitor vital signs nursing interventions, the patient will be able to maintain a condition free from signs of infection  Maintain the IV site sterile  Keep the bed of the patient clean  Change linens as needed

 To note for any changes in the vital signs of the patient  To prevent invasion of pathogens  To provide comfort  To prevent soiled linens which may be a carrier that may cause infection  To avoid crosscontamination

Goal met. After 8 hours of nursing interventions, the patient was able to maintain a condition that is free from signs of infection

 Stress proper hand washing techniques to all caregivers before and after handling the patient  Encourage adequate rest and provide conducive environment

 To promote comfort and wellness

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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 to decreased hemoglobin level secondary to abnormal red blood cell structure

2. Risk for infection related to inadequate secondary defenses (decreased hemoglobin level)

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 GENERIC NAME: FOLIC ACID BRAND NAME: FOLVITE CLASSIFICATION: SUPPLEMENTARY VITAMINS STOCK DOSE: 1 mg DOSAGE 0.05 mg/day ROUTE: PO

MECHANISM OF ACTION

CONTRAINDICATION INDICATION ADVERSE EFFECTS

NURSING RESPONSIBILITY

- essential for production of certain coenzyme in many metabolic systems such as purine and pyrimidine synthesis. It is also essential un the synthesis and maintenance of nucleoprotein in erythropoesis. It also promotes WBC and platelet production in folate deficiency anemia

TREATMENT FOR : - anemia

-

undiagnosed megaloblastic anemia pernicious aplastic or normocytic anemia

GI: - disturbances - hypersensitivity reactions - bronchospasm

- monitor vital signs -assess for the capillary refill - monitor skin appearance - instruct parents to encourage to have healthy lifestyle

35

DRUG NAME GENERIC NAME: CETERIZINE BRAND NAME: ZYRTEC CLASSIFICATION: ANTIHISTAMINE STOCK DOSE: 5 mg DOSAGE : 2.5 mg ROUTE: PO

MECHANISM OF ACTION

CONTRAINDICATION INDICATION ADVERSE EFFECTS

NURSING RESPONSIBILITY

- antagonizes the effects of histamine at H1-receptor sites; does not bind to or inactive histamine. Anticholinergic effects are minimal and sedation is dose related

relief of allergic symptoms caused by histamine release including: - Seasonal and perennial allergic rhinitis - chronic urticaria

-

hypersensitivity acute attacks of asthma

CNS: EENT: GI: dry mouth pharyngitis dizziness drowsiness fatigue

- monitor vital signs - instruct relatives/parents to take medication as directed - advise parents to have their child a good oral hygiene, frequent rinsing of mouth with water to minimize dry mouth

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; typical clinical and laboratory features

Atypical blood film? Recent infection; No family history of HS

Dominant Inheritance

Screen proband and family for abnormal RBCs

No further Investigation needed

Variable clinical severity in different family members

HS RBCs detected in proband & siblings but normal parents

Normal test result

Not Search for co-inheriting haematological disorder SDS PAGE for protein defect

membrane

? thalassemia ?CDA ?MDS

ß-thalassemia or sickle cell disease

None

DNA analysis for low- expression allele Recessive or non dominant inheritance in proband with no family history

38

Presentation about Hereditary Spherocytosis
Entitled:

Presentation about Hereditary Spherocytosis
Entitled:

Presentation about Hereditary Spherocytosis
Entitled:

Presentation about Hereditary Spherocytosis
Entitled:

“hinog na mangga”
By:

“hinog na mangga”
By:

“hinog na mangga”
By:

“hinog na mangga”
By:

BSN III-D Group 1
On:

BSN III-D Group 1
On:

BSN III-D Group 1
On:

BSN III-D Group 1
On:

September 19, 2008

September 19, 2008

September 19, 2008

September 19, 2008

1

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