Siena College of Taytay College of nursing Taytay, Rizal

Case Presentation On Autosomal Recessive Polycystic Kidney Disease (ARPKD)

Rizal Provincial Hospital-Morong Branch Morong Rizal
Pedia Ward November 18-21 and December 2-5, 2013
Clinical Instructor: Ms. Jesmar Espiritu, RN, MAN

I. INTRODUCTION A. Background of study

Autosomal recessive polycystic kidney disease (ARPKD) is a severe genetic disorder characterized by enlarged kidneys at birth and kidney and liver failure. Newborns with ARPKD have large, echogenic (highly visible by ultrasound) kidneys, and many of them also have enlarged livers. About one-third of those with ARPKD die within the first year of life due to pulmonary complications and many of those who survive infancy suffer kidney failure before adulthood. Rarely, the condition is diagnosed later in childhood. ARPKD is caused by mutations in the PKHD1 (polycystic kidney and hepatic disease-1) gene and is inherited in an autosomal recessive manner, which means that a person must inherit a mutated copy of the gene from each parent to develop the disease. ARPKD is the most common genetic cystic renal disease occurring in infancy and childhood. However, it is nonetheless a rare disorder and is much less common than ADPKD. A single gene defect leads to differing degrees of renal and hepatic involvement, with very different phenotypes and clinical outcome within even one affected family. Kidneys are bilaterally enlarged and contain large numbers of cysts throughout the organ, due to the dilatation and elongation of renal collecting ducts. At birth, the interstitial and the rest of the tubules are normal but they may later develop interstitial fibrosis and tubular atrophy that can cause renal failure. There may be hepatic as well as renal involvement. Studies suggest a prevalence of between 5-10 in 100,000 births. (Martnez-Fras ML) Our groups decided to take this case because its not as usual as the cases that weve handled before and were able to establish good communication skills with the patient and family.

A. General Objectives
Within 32 hours of exposure at Rizal Provincial Hospital-Morong branch in Pedia Ward, Group 1 of the 4th year nursing students of Siena College Taytay is aiming to acquire the proper attitude with our client. To apply the 11 core competencies and we also aim to enhance our knowledge and hone our nursing skills in order to provide holistic care and support to our client in RPH Morong.

B. Specific Objectives To be able to:

1. Establish rapport with the patient . Assess properly to determine the contributing reasons regarding to the clients condition and identify any deviation. 2. Perform sterilization procedure as well as proper disposal of hospital waste. 3. Give appropriate health teachings needed in the situation. 4. Document all significant data and ensure patients consent with full sign. 5. Respect the rights and beliefs of the patient. 6. Follow the standard hospital policies. 7. Accept criticisms and recommendation from the professional for improvement. 8. Identify researchable problem that is related to PKD. 9. Document precisely all the gathered data about the client. 10. Listens considerately to clients and families about their concerns and requests. 11. Communicate with the medical team effectively.

C. Significance of the Study

The importance of this study is much recognized since it would build up competence and enhance skills to ourselves in dealing with such case again. Moreover, it was observed that perfect way to widen our knowledge in handling with this type of illness.

D. Scope and Limitation

This case study was conducted on November 18, 2013 Rizal Provincial Hospital-Morong Branch to a 6 months old baby who has been diagnosed of PKD r/o UTI.

D. THEORETICAL FRAMEWORK
Erik Erikson's---- Psychosocial Development Erik Erikson developed a theory called the Eight Stages of Psychosocial Development. He theorized that stages are precipitated by crises that all individuals face as they move from birth to death. According to Erikson, each stage presents the individual with a psychosocial conflict that must be resolved. Each stage has a positive and negative pole. For instance, during the first stage the infant struggles with whether to trust the environment or not. If he or she is born into a relatively stable, dependable environment the infant learns to trust and out of that trust hope emerges. If, however, the infant is born into an environment that is erratic, and undependable he or she may grow up considering the world to be an untrustworthy place. This will colour future life experiences and rather than entering the world with a hopeful outlook, the individual may feel hopeless.
Stage Basic Conflict Important Events Infancy (birth to 18 months) Early Childhood (2 to 3 years) Autonomy vs. Toilet Training Trust vs. Mistrust Feeding Children develop a sense of trust when caregivers provide reliabilty, care, and affection. A lack of this will lead to mistrust. Children need to develop a sense of personal control over physical skills and a sense of independence. Success leads to feelings of autonomy, failure results in feelings of shame and doubt. Preschool (3 to 5 years) Initiative Guilt vs. Exploration Children need to begin asserting control and power over the environment. Success in this stage leads to a sense of purpose. Children who try to exert too much power experience disapproval, resulting in a sense of guilt. School Age (6 to 11 years) Adolescence to 18 years) (12 Industry Inferiority Identity vs. Role Confusion Social Relationships vs. School Children need to cope with new social and academic demands. Success leads to a sense of competence, while failure results in feelings of inferiority. Teens need to develop a sense of self and personal identity. Success leads to an ability to stay true to yourself, while failure leads to role confusion and a weak sense of self. Young Adulthood (19 to 40 years) Intimacy Isolation vs. Relationships Young adults need to form intimate, loving relationships with other people. Success leads to strong relationships, while failure results in loneliness and isolation. Middle Adulthood (40 to 65 years) Generativity Stagnation vs. Work Parenthood and Adults need to create or nurture things that will outlast them, often by having children or creating a positive change that benefits other people. Success leads to feelings of usefulness and accomplishment, while failure results in shallow involvement in the world. Maturity(65 death) to Ego Integrity vs. Despair Reflection Life on Older adults need to look back on life and feel a sense of fulfillment. Success at this stage leads to feelings of wisdom, while failure results in regret, bitterness, and despair. Outcome

Shame and Doubt

Ego Integrity vs. Despair Generativity vs. Stagnation Intimacy vs. Isolation Identity vs. Role Confusion Industry vs. Inferiority Initiative vs. Guilt Autonomy vs. Shame and Doubt Trust vs. Mistrust
Patient V is fed when hungry by his mother Patient V cries for attention for compassion or hygienically purposes His mother/grandmother set comfortable mode, tone and sustenance of Patient V. His mother and father are nurturing ability and care for Patient V, especially in terms of visual contact and touch.

We chose this Theory because, this model helps us what psychological work we need to do when there has been difficulty navigating a particular stage in life. And lastly it is appropriate to our patients age and status.

II. NURSING ASSESSMENT

Name: Age: Birth date: Address: Date/ Time of Admission: Diagnosis: Admitting Physician: Chief Complaint: Patient V 6 months April 18, 2013 #65 Rizal Street Baras, Rizal November 12, 2013; 11:48 AM Autosomal Recessive Polycystic Kidney Disease r/o UTI Dra. Santiago Fever x 7 days

History of Past Illness: Prior to admission, Patient V had an on and off fever that lasted for seven days. They decided to go to his pediatrician to meet the client's needs. The pediatrician told them to bring Patient V at the hospital because he needs to be confined. History of Present Illness: The client had experienced low grade fever for 3 days with temp of 37.9. 3 days prior to admission, the client's parents consulted his pediatrician and ordered to take phenytoin amoxcillin. The fever didn't go off. Client was rushed @ the Emergency room. Assessment was done. The doctor advised the client to be admitted @ the Pedia Ward for close monitoring. Immunizations: BCG DPT OPV Measles Vaccine 1 dose 3 doses 3 doses _ At birth 6 weeks old 6 weeks old At birth Not yet done because hes only 6 months old.

Family History:
There was no known sickness among the family on both maternal and paternal side.

III. GORDONS FUNCTIONAL HEALTH PATTERNS GORDONS FUNCTIONAL HEALTH PATTERNS

Health Patterns Before Hospitalization The client had cough and colds for the past weeks. Taking Ferlin (Iron Vitamin B Complex) as ordered. He attended monthly check-up to his Pediatrician; It was his first hospitalization. His mother preferred to breastfed her. Never experienced bottlefeeding. During Hospitalization His medications were administered Intravenously as ordered by the Pediatrician. Analysis

HEALTH PERCEPTION PATTERN

Intravenous therapy may be used to correct electrolyte imbalances, and to deliver medication. It is the fastest way to deliver fluids and medications throughout the body.

NUTRITIONAL METABOLIC PATTERN

He's been bottle-fed during his hospitalization. Milk was Promil.

Breastfeeding helps defend against infections, prevent allergies, and protect against a number of chronic conditions. The ideal room temperature for your baby is between 16 and 20C. It's important to provide them comfortable place to sleep in order for them to have enough rest. Bowel patterns can depend on the type of feeding. Breastfed infants tend to have more frequent bowel movements than bottle-

SLEEP/ REST PATTERN

Client doesn't have any sleeping problems. He usually sleeps at 8pm. Wakes up in the middle of the night when he's hungry.

He's irritated in the hospital's environment. Wasn't able to sleep well; he keeps on waking up every each nap.

ELIMINATION PATTERN

He consumed 2-3 diapers per day. His mother uses towel (lampin) sometimes to avoid skin rashes. The client defecates every day. Stool was yellowish

The client wasn't able to defecate every day. Consumed 3-4 diapers per day. Stool was yellowish green.

green.

fed infants

IV. PHYSICAL ASSESSMENT

System Review Actual Result The patient has fair skin color. The clients skin is light brown in color. Presence of small wounds on the hands and feet. Right side of the head is symmetrical to the left side of the head. No mass present. Symmetric to the face, both eyes coordinated with parallel alignment There is eye opening upon verbal command. Eyebrows are black, evenly distributed and the right side is slightly lower. Breath on his own, no oxygen or mechanical ventilator present. Same color as face. Central position and proportional. No Presence of wound/discharge on both ears. The lips are soft, moist and smooth. Pink in color. Presence of crackles. Pulse rate of: respiratory rate of: The patient receives his food through bottle feeding. Defecate every day. His stool was yellow green in color Patient consumed 2-3 diapers per day for his urination. Analysis Normal

Skin

Due to IVF insertion

Head

Normal

Eyes

The patient has eye opening when commanded.

Nose Ears

Normal Normal

Mouth Cardio/Respiratory

Normal

Gastrointestinal Tract

Normal

Genito-Urinary Tract

Normal

Neurologic/Muscular

The patient responds actively to our spoken words.

Normal

V. ANATOMY OF THE URINARY SYSTEM OF AN INFANT AND PATHOPHYSIOLOGY

The urinary tract is the bodys drainage system for removing wastes and extra fluid. The urinary tract includes two kidneys, two ureters, a bladder, and a urethra. The kidneys are two bean-shaped organs, each about the size of a fist. They are located just below the rib cage, one on each side of the spine. Every day, the kidneys filter about 120 to 150 quarts of blood to produce about 1 to 2 quarts of urine, composed of wastes and extra fluid. Children produce less urine than adults. The amount produced depends on their age. The urine flows from the kidneys to the bladder through tubes called ureters. The bladder stores urine until releasing it through urination. When the bladder empties, urine flows out of the body through a tube called the urethra at the bottom of the bladder.

VI. LABORATORY EXAMINATION

Hematology

Date ordered Nov. 12, 2013

Diagnostic lab exam Hemoglobin Hematocrit

Normal Values 120-170g/l 0.38-0.48

Result

Analysis

123 0.37

Normal Normal

WBC Lymphocytes Segmenters

5-10 x 20/l 0.10-0.20 0.40-0.60

0.50 0.50

Indicates a viral infection Normal

Date ordered Nov. 15, 2013

Diagnostic lab exam Hemoglobin Hematocrit

Normal Values 120-170g/l 0.38-0.48

Result

Analysis

123 0.38

normal Normal

WBC Lymphocytes Segmenters Platelet

5-10 x 20/l 0.10-0.20 0.40-0.60 150,000450,000 McL

7.8 0.43 0.57 185,000 McL

Indicates infection Indicates a viral infection Normal Normal

Date ordered Nov. 16, 2013

Diagnostic lab exam Hemoglobin Hematocrit

Normal Values 120-170g/l 0.38-0.48

Result

Analysis

127 0.36

Normal Slightly below normal. May be caused by loss of blood or nutritional defieciency Normal Normal

WBC Lymphocytes Segmenters Platelet

5-10 x 20/l 0.10-0.20 0.40-0.60 150,000450,000 McL

156

Urinalysis Date ordered Nov. 12, 2013 Diagnostic lab exam Result Analysis

Color

Yellow

Concentrated urine Normal

Specific gravity

1.010

WBC Epithelial cells Transparency

0-1 few Turbid

Normal Normal Normal

Chest X-ray APL

Radiological Report

Patients name: Age: Sex: Examination: Date: Findings:

Patient V 6 months Male CXR APL 11-13-13

Perihilar adenopathies are noted. Heart and other chest structures are unremarkable Conclusion: Perihilar adenopathies non specific

VII. NCP
Assessment S-Mother verbalized mainit ang anak ko. Diagnosis -hyperthermia related to inflammatory process secondary to diseased process as manifested by increased temperature. Planning Short term goal: after 2 hours of nursing intervention, the patients temperature will return to normal. (37.2) Nursing Action -Establish rapport with guardian. Rationale - establishing rapport with the mother promotes cooperation in the nursing care. -monitoring helps the nurse to identify the development of the patients temperature. -ventilatory effort may be impaired due to hyper metabolic state and possible seizures. -to decrease body temperature. -to decrease body temperature. Evaluation -Goal met: after 2 hours of nursing intervention the patients body temperature decreases from 38.3 to 37.2.

O- body temperature is above normal range (38.3) -Tachypnea (73 bpm) -warm, flushed skin

-Monitor temperature.

Long term goal: after 3 days of nursing intervention, the patients temperature will return to normal. (37.2)

-monitor ventilatory effort

-do tepid sponge bath.

-administer antipyretics as ordered.

VIII. DISCHARGE PLANNING

MEDICATION EXERCISE No prescribed exercise since the patient is 6 mos old. TREATMENT pain medication ,surgery to shrink cysts and relieve pain,treatment for high blood pressure, treatment for urinary tract infections, dialysis and kidney transplantation . HEALTH TEACHINGS If patient is in pain go to the physician to have check up with the new born. Take the medication properly if the physician prescribed medication. OUTPATIENT Since the patient is new born tell to the mother of the patient to go back in the hospital in OPD after 1 week to have check up and prescribe medication to the patient. DIET Since the patient is new born he is in milk feeding diet. SPIRITUAL Encouraged the mother of the patient to have faith in God, and always pray. Go to the church.