Prune Belly Syndrome Network, Inc.
"You are NOT the only one!"

~ Nursing Paper~

This is a Family Nursing term paper which discusses Prune Belly Syndrome, written by Vicky L. Earl, SN in 1996; a then third year nursing student at Stanford University. A review of literature is examined including the mode of inheritance, pathophysiology, developmental potential, and review of research, and the medical and nursing management involvement. Family dynamics are discussed which identify the types of crisis and the effects the syndrome has on the family's lifestyle. Types of genetic screening and community agencies are identified. Also, the nursing process is utilized for the care of the hypothetical family.

Contents

I. Hypothetical Family Situation

II. Review of Literature

A. Mode of Inheritance
B. Pathophysiology
C. Developmental Potential
D. Review Research
E. Medical and Nursing Management


III. Family Dynamics

A. Types of Crisis
B. Effects on the Family's Lifestyle
C. Diagnosing
D. Community Agencies

IV. Nursing Process

V. References



Hypothetical Family Situation

The family consists of a 34 year old father, a 24 year old mother, and a newborn baby. The parents have been married for two years. This was their first child and the mother's first pregnancy. The mother is recovering but the baby is sick and has been admitted to the neonatal intensive care unit (NICU).The baby boy was born at 35 weeks and weighed 2700 grams. The newborn infant has a wrinkled abdomen due to the lack of adequate abdominal musculature, a right ventral wall hernia, undescended testes, a dilated bladder, and hypoplastic lungs. The family did not have any prenatal testing done during the pregnancy. After the baby was born, the mother was unable to bond with the baby for several days because of the baby's complications. The doctors were insistent that the baby would not survive. The doctors informed the parents the baby should be given to the state of Alabama because the baby would be a burden on them financially and emotionally. The parents felt pressured and confused in their decisions regarding the baby's welfare. The family lives in Homewood with both of the parents working. The father is an accountant and the mother is a school teacher. Financially the family is considered to be middle class. The family has private insurance that covers most health care after payment of a deductible of $500. The private insurance does provide home health care if the family decides to take the infant home. Economically, the family is under financial strain because of the infant being in ICU and considering the long term care the infant will need.

Review of Literature

Mode of Inheritance

Prune Belly Syndrome is an abdominal muscular deficiency which consists of a triad of congenital defects. These defects include aplasia of the musculature of the lower and medial abdominal wall, anomalies of the urinary tract, and bilateral cryptorchidism. (Terada, Uchide, Akasofu, 1994).

Although the syndrome has been reported to be associated with diverse chromosomal anomalies, the karotype is normal in most cases (Terada, et al, 1994). Prune Belly Syndrome is usually detected after twenty weeks gestation in the fully developed form of the triad of symptoms. Multiple cases of the full syndrome in families have rarely been reported and there is not a clear genetic pattern (Washington University teaching file). The syndrome occurs predominantly in males and no more than 5 % in females which have mild cases without severe urinary tract involvement (Terado, et al, 1994).

Pathophysiology

The exact cause of Prune Belly Syndrome is not yet known, but there are two main theories. First the deficiency may represent a mesodermal defect due to obstructed transition of myotomes from the lateral mesoderm to the lower abdomen at four and five weeks gestation (Loder, Guiboux, Bloom, Hensinger, 1992). The second theory suggests megalocystis. This causes an overdistention of the abdominal wall and induces hypoplasia and defects of the abdominal musculature (Loder, et al, 1992).
The abdominal muscle deficiency may develop secondary to ischemia and reduced nerve conduction. This is due to the overextension of blood vessels and nerves or to muscular overextension (Terada, et al, 1994). The overextension of the muscles is attributed to megalocytis which results from urethral obstruction (Terada, et al, 1994). This causes a urethral atresia and stenotic "kinking" of the posterior urethral valves. The utrethral obstruction arises because the mesenchymal-epithelial interactions are disturbed during fetal growth (Stevens, Gupta, 1994). This obstruction causes hypoplasia of the prostate and the urethra. Cryptorchidism is caused by blockage of the enlarged bladder. The large bladder fills the pelvic cavity which causes an upward displacement of the testes. The presence of the intrapelvic mass from early fetal development causes the descent of the testes to be difficult. (Stephens, Gupta, 1994). The deficiency and laxity of the abdominal muscles do not constitute a "malformation" but rather a "deformation" which results from the distention of the fetal abdomen during early gestation (Terada, et al, 1994). The urethral obstruction may be the primary cause of this syndrome. The abdominal muscles deficiency and the cryptorchidism are the secondary causes of the syndrome (Terada, et al, 1994).

Developmental Potential

The developmental complications begin during fetal development. Problems with this syndrome can affect the physical, intellectual, and emotional conditions in the patient. In the past, the morbidity and mortality in the patients with Prune Belly Syndrome resulted from genitourinary malformations with the majority having renal failure. Since the medical surgical treatment of these patients has been improving, the genitourinary morbidity and mortality rates are decreasing (Loder, et al, 1992).

Other significant long-term functional aspects include orthopedic malformations. The prevalance of musculoskeletal involvement includes 45% of the patients (Loder, et al, 1992). The involvement may be congenital which includes clubfeet, limb deficiencies, teratologic hip dysplasia, or vertebral malformations. The developmental complications include renal osteodystrophy, scolosis, and pectus excavatum or pectus carinatum (Loder, et al, 1992).

Clubfoot is the mot common orthopedic deformity which usually occurs in at least 1/4 of all children with Prune Belly Syndrome (Loder, et al, 1992). Other foot deformities include lower-limb hypoplasia and shriveled legs or feet. There is increased incidence of hip dysplasia due to teratologic causes. Tertatologic hip dysplasia is characterized by the association with other malformations and develops early in utero with severe soft-tissue contractures and the displacement of the femoral head (Loder, et al, 1992). The appearance of pectus deformities and idiopathic like scolosis have an increased incidence in patients with the syndrome.

Other areas of physical complications include pulmonary and gastrointestinal problems. Pulmonary complications include hypoplasia secondary to oligohydramnios which is present at birth (Loder, et al, 1992). Later in life, recurrent bronchitis may become a problem. After an upper respiratory infection or the use of anesthetics, the patients are susceptible to the development of respiratory insufficiency. These respiratory difficulties develop from a mechanical restriction associated with the thoracic cage deformities such as scoliosis and pectus excavatum (Stephens, Gupta, 1994). These deformities develop secondarily from the abdominal wall deficiency. Even without a history of pulmonary problems, a patient may develop postoperative distress. It is important to be aware of the potential pulmonary complications because many of the Prune Belly Syndrome aspects require operative treatment. As the deformity progresses, adjacent ribs are altered, increasing the claudal declination and decreasing intrathoracic volume (Loder, et al, 1992). This progressive chest wall and rib cage deformity affects the spine causing an asymmetric change in the rib and paraspinal muscle support. The paraspinal muscle is less than normal and weakens as the body develops.

Gastrointestinal problems include intestinal malrotation, small bowel stenosis, imperforate anus, and chronic constipation (Stephens, Gupta, 1994). Constipation is the most common gastrointestinal problem. This is related to the weak abdominal muscles with inadequate intra-abdominal pressure for defecation (Stephens, Gupta, 1994). Constipation can be aggravated by aspects such as cast immobilization.

Most children with Prune Belly Syndrome are surviving into adulthood and muscoskeletal aspects are becoming more of a priority in the care of the patient. The healthcare workers involved in the multidisciplinary care of these patients should be aware of these problems.

There has been no documentation of intellectual involvement caused by Prune Belly Syndrome. However, the emotional aspect involved with the syndrome could hinder the child's ability to learn.

In the past, the psychological implications of the disfigurement have been underestimated. The average age of an operation to repair the abdominal wall disfigurement is prepubertal. According to researcher R.M. Ehrlich, there is no reason the procedure can not be performed at an earlier age (1992). These children could have a more normal bodily image of themselves during their years of childhood (Parrott, Woodward, 1993).

Emotional support is needed for both parents and the child. The child will spend more time in the hospital than out the first few weeks of his life. The diagnosis is difficult on the family, especially financially and emotionally. It is important for the parents to bond with the infant as soon as possible. Bonding is important in the development of the physical, intellectual, and emotional aspects of the infant (Whaley, Wong, 1993).

In the history of one family, the infant was not able to bond with the parents for several weeks because of his medical conditions. Later, the parents had a difficult time relating to their son. They were embarrassed by their son's appearance and did not want anyone to know their son had Prune Belly Syndrome. They would actually punish the child for telling his friends or anyone else about this syndrome. Buying clothes for the child was an ordeal because it would take hours and hours of getting pants to fit correctly so the suprapubic catheter would not be visible. Also, the parents coped with "the problem," as they referred to it, by drinking alcohol. This was their only way of handling the situation. The diagnosis affected every day of the family's life.


Review of Research

In the past there has been limited research concerning Prune Belly syndrome. This is related to there only being 1 in 40,000 live births affected by the syndrome (Loder, et al, 1992). The prognosis of the infant depends upon the degree of dilation of the urinary tract. Twenty percent of the infants are stillborn and thirty percent die of renal failure or urosepsis within the first two years of life (cpmcnet.columbia.edu/dept/radiology). The remaining fifty percent have varying degrees of urinary complications (cpmcnet.columbia.edu/dept/radiology).

In the past, it was suggested that the abdominal wall was deformed by pressure from a distended bladder due to outlet obstruction in utero (cpmsnet.columbia.edu/dept/radiology). Currently researchers have concluded that Prune Belly Syndrome is a multisystem disease. The complications are derived from a primary defect in the mesodermal development (Loder, et al, 1992).

The present-day research concerns the operative procedures to repair the abnormalities of the urinary system, undescended testes, and renal defects. These operations include the Montfort operation of reconstructing the abdominal wall and the Fowler-Stephens orchiopexy (Parrott, Woodard, 1992).

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Medical and Nursing Management

Prune Belly Syndrome describes the appearance of the abdomen of the neonate, infant, or young child. At adolescence the abdomen assumes a pot-belly shape. Previous management of the abdominal wall abnormality in patients with Prune Belly Syndrome varied from expectant observations to the use of corsets or body stockings (Parrott, Woodard, 1992). In one child, a diaper was worn around the abdomen to bind the stomach. Currently, surgical treatment is very conservative.

Of the various procedures to correct this abnormal physical appearance, the Montfort operation has had the most success in reconstructing the abdominal wall. This operation preserves the umbilicus and strengthens, flattens, and thickens the abdominal wall. It produces a narrow-waisted, more normal physique and provides excellent transperitoneal exposure for concomitant genitourinary reconstructive procedures (Parrott, Woodard, 1992). The general appearance of the abdomen is improved. The umbilicus is preserved and the unwanted scars are excised with the skin overlying the central fascial plate (Parrott, Woodard, 1992).

Another operation in the management of Prune Belly Syndrome is the Fowler-Stephens orchiopexy. This operation manages the intra-abdominal testis. The major problem with the operation is securing adequate length of the internal spermatic vessels. A short testicular pedicle may prohibit scrotal orchiopexy (Redman, Mooney, 1993). The division of the testicular pedicle used by Fowler and Stephens provides an adequate blood supply for successful orchiopexy.

Also, if the infant has complications with a large bladder, megaureters, or hydronephrosis, a suprapubic catheter is placed, usually around three months of age. In one patient with Prune Belly Syndrome, he had a suprapubic catheter placed when he was three months old until he was thirty years old. Then a cecoplasty (bladder augmentation) was done because of the increasing kidney infections and renal calculi.

In nursing management, it is important to be aware of the multidisciplinary problems involved in the infant's care. Management includes areas of emotional support, developmental areas, and social aspects. Management in these areas is needed for a normal development during the life-span of a Prune Belly infant.

When the diagnosis of Prune Belly is given to the family, nurses need to provide supportive care and education about the syndrome. Emotionally, it is a difficult time for the entire family. The different aspects of the syndrome need to be discussed with the family as well as the how the aspects of the syndrome will affect the family's lifestyle. In the supportive care of the infant and family, the nurse needs to be understanding and be available to the family's concerns of the syndrome.

The nurse needs to be knowledgeable about the syndrome, especially respiratory conditions. Because of the lack of abdominal muscles, there are no accessory breathing muscles. A typical Prune Belly patient has a concave chest with the ends of the ribs flaring outward (Ewig, Griscom, Wohl, 1996). This is due to the lack of abdominal musles pulling on the ribs which is mainly cartilage. In one case history of a surviving 43 year old male with Prune Belly Syndrome, the patient described his experience of having respiratory arrested. First, he developed ventricular fibrillation. His chest was cracked open and the chest muscles were cut. Then, he developed a pleural effusion on the left side above the diaphragm, developed atrial fibrillation, and a paralyzed diaphragm. If the healthcare team had known the respiratory complications of Prune Belly Syndrome, they would have handled the procedures differently. The patient respiratory arrested four times and was eventually trached. The patient expressed that it was a miracle that he survived.

The fluid and electrolyte balance and nutritional aspect is important in the care of the infant. A low protein diet is needed to reduce the work of the kidneys and is essential to keep the infant well-nourished. When the nutritional status is balanced then the body has more energy to fight infections. This is important because the urinary tract is the major site of infections. The nurse needs to be familiar with the drugs given to fight infections, mainly antimicrobial therapy and prophylactic antibiotics (ami-med.com/peds/scr).

In the case history of the 43 year old, he reported his parents were instructed to lower the protein and calcium intake and give him two to three eight ounce glasses of cranberry juice daily. The patient had strong feelings about drinking cranberry juice. He did report that he drinks four or more liters of water each day. If he does not drink the high volumes of water, his urine sludges quickly.

Prune Belly Syndrome is rare in society, but the family needs to know how to deal with the problem. The nurse may need to help the family in decision making and may need to teach the family about the syndrome. Being able to adjust to the syndrome is difficult, but having to adapt to a new way of life is even more difficult.

Family Dynamics

Type of Crisis

"A crisis may be defined as the state that occurs when insurmountable obstacles prevent the individual from achieving a goal" (Stuart, Sundeen, 1995). Crisis occurs in everyone's life at one time or another. The illness of the infant puts the family into a crisis situation. There are three types of crisis which are maturational, situational, and adventitious (Stuart, Sundeen, 1995). A maturational or developmental crisis occurs as one progresses through the developmental stages. This type of crisis is expected and internally generated (Anatai_Otong, 1995). A situational crisis is an unexpected event that threatens a family physically and psychologically (Anatai-Otong, 1995). This results in role changing and adjustment for each member of the family. The last type is adventitious crisis. It is a major natural event that affects the emotions of the person (Anatai-Otong, 1995). This type of crisis is usually accidental or an unexpected event with multiple losses.

The hypothetical family is experiencing a maturational crisis in having a baby. The family was anticipating a healthy infant's birth, but the adjustment to the infant with Prune Belly Syndrome causes the crisis. Also, the family is experiencing situation crisis because the infant will need long term care, especially caring for infections of the urinary tract. The parents have to decide who will care for the infant while they are at work. All of the problems place a family in a major crisis which is complex and not easily solved (Anatai-Otong, 1995).

Effects on the Family Lifestyle

The family was preparing for their first child. Things were already busy and hectic with the excitement of the firstborn baby. When the mother delivered at 35 weeks, she was expecting a healthy baby. Since there was no prenatal screening done, the diagnosis was a shock to the entire family.

Besides the shock of the diagnosis, the parents were not allowed to bond with the baby for several days. This delayed the bonding between the parents which may hinder the relationship later in life. Since the baby was in the intensive care unit, this placed a strain on the parents because of the many trips to the hospital to see their baby.

Another difficult aspect of the syndrome is the long term care which will be needed for the child. This includes the operations to correct the cryptorchidism, urinary anomalies, and abdominal wall reconstruction. Even though the family has good insurance, the family will need to provide finances for the extra tools needed to give the child a quality of life.

The diagnosis of Prune Belly causes many changes in the family's life. Prevention of urinary tract infections is a major concern. The family will need to stay away from people who are sick with infections and have regular visits to the doctor, especially when the child does have an infection.

Prune Belly affects every area of a family's lifestyle. It is important for the family to develop coping patterns which will help in dealing with the diagnosis. Healthcare workers need to be supportive and sensitive to the needs of the child and the family.

Diagnosing

Before birth a fetus may be diagnosed with the syndrome based upon the appearance of the fetus (ami-med.com/peds/scr). Through the use of a fetal ultrasound, Prune Belly Syndrome can be detected as early as eleven weeks (Terada, et al 1994). The fetal ultrasound has given doctors the ability to diagnose the syndrome. Blockage of the urinary system may be visualized through the ultrasound. Newborn infants who have been diagnosed with the obstruction in the uterus can receive prompt surgical correction of the defects (ami-med.com/peds/scr). Also, the ultrasound detects any renal or abdominal defects.

Other tests include x-rays of the kidneys and chest, a renal scan, CT scan of the abdomen and kidneys, and an intravenous pyelogram (ami-med.com/peds/scr). ). The IVP provides information about the integrity of the kidneys, ureters, and bladder. Also the radiograph visualizes the masses which shift position of the ureters (Whaley, Wong, 1993).

The treatment would be based on the results of the screening and diagnosis. There are three surgeries which may be performed consisting of pyelostomy, vesicostomy, and reconstructive surgery (ami-med.com/peds/scr). A pyelostomy is an opening into the kidney with the placement of a drainage tube. A vesicostomy is similar to a pyelostomy in that an opening is made in the bladder, providing drainage (ami-med.com/peds/scr). ). Also, reconstructive surgery may be done for cosmetic reasons or if an area is obstructed.

The counseling provided gives the parents an opportunity to discuss their feelings concerning the diagnosis. This allows clear communication and education of the diagnosis of Prune Belly Syndrome. The counseling allows the parents to understand their options in caring for their infant with this syndrome (Whaley, Wong, 1993). It is important for the family to understand and follow the advice given concerning the care of the infant. The success of counseling is measured by the manner in which the family uses the information presented (Whaley, Wong, 1993).

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Community Agencies

Families living with Prune Belly syndrome have many needs; not only financial, but emotional, social and educational needs which need to be met. There are no community agencies in Birmingham which are specifically for helping families with Prune Belly Syndrome. The reason is because there is not a need in Birmingham.

At Children's Hospital, social workers are very knowledgeable about agencies in the area to help families with finances, shelter, transportation, and support groups. Children's Hospital has their own support groups and financial resources to help the family in need. At the time the child is in the hospital, the social workers can help with the problems the family is having. If the social workers cannot directly help the family, they will refer the family to other organizations.

There is one association which provides information on Prune Belly Syndrome. The organization is called the Prune Belly Support Network. It was started by a 45 year old man with the syndrome and it provides information via the internet for anyone interested in the syndrome. The information is located at http://www.prunebelly.org)

Nursing Process

Nursing Diagnoses

1. Ineffective breathing pattern related to decreased lung expansion
2. Altered nutrition: less than body requirements related to a decreased intake
3. High risk for impaired tissue integrity related to the use of diaper around the abdomen as a binder
4. Altered urinary elimination pattern related to urinary tract anomalies
5. Ineffective family coping related to inappropriate bonding
6. Knowledge deficit of Prune Belly Syndrome related to inability to think clearly
7. Powerlessness of parents related to the diagnosis of Prune Belly Syndrome in their child
8. Anticipatory grief related to potential loss of the child
9. Spiritual distress related to thought of death and afterlife for child
10. Anxiety related to long term care of child

Priority 1: Ineffective breathing pattern related to decreased lung expansion

Assessment
    * Labored breathing with flaring nares, grunting, wheezing, or expiration
    * Tachypnea (69 breaths/ minute, normal 30-60)
    * Tachycardia (155 beats a minute, normal 120-140)
    * Peripheral cyanosis
    * Shallow, decreased breath sound bilaterally
    * ABG's, pH 7.39 (normal 7.11-7.36), Pa02 22 mmHg (normal 31-75)
    * PaCO2 43 mmHg (normal 27-40), Sa02 83% (normal 85-90%)
    * Restless, agitated, lack of sleep (sleeps every 4 hours for 2 hours)

The infant's respiratory difficulty will be lessened as evidenced by:

Long term goals:
1. The infant will maintain an increased lung expansion.
2. The infant will maintain regular respirations between 30-60 breaths/ minute without difficulty breathing.
3. The infant will maintain clear breath sounds bilaterally without the flaring nares, grunting, or wheezing on expiration.

Short term goals:
1. The infant will maintain decreased respiratory (30-60bpm), heart rate (120-140 bpm), and ABG's of pH 7.11-7.36, PC02 27-40 mmHg, P02 31-75 mmHg, Sa02 85-90%.
2. The infant will exhibit decreased respiratory difficulty by restful sleeping patterns of 8-10 hours of sleep.

Nursing prescriptions:
1. Assess respiratory status for evidence of nasal flaring, grunting, wheezing, rate, depth, and chest movement.
2. Elevate head of crib to promote breathing and ease the work of breathing.
3. Auscultate breath sounds and note any adventitious sounds.
4. Monitor level of consciousness every hour.
5. Provide a calm environment to allow the infant to rest.
6. Monitor vital signs, ABG's, and pulse oximeter every hour.

Priority 2: Altered nutrition: less than body requirements related to decreased intake

Assessment:
    * Weight loss of 680 grams since birth (2700 grams at birth)
    * Decreased intake-drinks less than 2 ounces every 4-3 hours or on demand * Poor suck
    * Does not retain feedings
    * Constipation

The infant will have an improved nutritional status as evidenced by:

Long term goals:
1. The infant will have a strong suck.
2. The infant will gain at least 1360 grams.
3. The infant will not be constipated.

Short term goals:
1. The infant will drink 2-3 ounces of formula every 3 to 4 hours or on demand.
2. The infant will retain the feedings.

Nursing prescriptions:
1. Assess strength of suck and swallowing.
2. Offer 2-3 ounces of formula every 3 to 4 hours or on demand.
3. Observe stool pattern.
4. Weigh daily.
5. Provide low protein and calcium to prevent renal calculi from developing.

Priority 3: High risk for impaired skin integrity related to use of diaper around the abdomen as a binder

Assessment:
    * Redness around abdomen
    * Dry, warm skin
    * Restless, agitated, uncomfortable

The infant will have an improved skin integrity as evidenced by:

Long term goals:
1. The infant's skin will not have any redness around the abdomen.
2. The infant's skin will have no signs of dryness or cracking around the abdomen.

Nursing prescriptions:
1. Turn the infant every 2-3 hours.
2. Assess areas of redness or dryness around the abdomen.
3. Bathe the infant daily to increase circulation to the areas.

Priority 4: Altered urinary elimination pattern related to the urinary tract anamolies

Assessment:
    * Decreased urine output
    * Less than 8ccper shift (normal 1cc/kg/hr)
    * Decreased fluid intake
    * Less than 2 ounces of formula every 3-4 hours
    * Palpated full bladder

The infant will have an improved urinary elimination pattern as evidenced by:

Long term goals:
1. The infant will have a balanced urine output with the intake.
2. The infant will have a maintained intake of fluids.
3. The infant will maintain a urine output of 1cc/kg/hr.

Short term goals:
1. The infant will not have a full bladder. 2. The infant will have an intake of more than 2 ounces of formula every 3 to 4 hours and the output will be in balance with the intake.

Nursing prescriptions:
1. Monitor vital signs. Normal: temperature 37.5, blood pressure 92/72, respiratory 30-60, heart rate 120-140.
2. Percuss and palpate bladder ever 2-4 hours.
3. Provide formula ever three to four hours or on demand.
4. Monitor intake and output.

Priority 5: Ineffective family coping related to the diagnosis of Prune Belly Syndrome of their child

Assessment:
    * Subj.- The mother said, "I do not want to hold my baby right now. Maybe tomorrow."
    * Subj.-The mother said, "I cannot feed my baby."
    * Subj.- The father said, "I cannot come every day. I may be able to come this weekend, but I do not know."
    * Subj.- The father said, "I am confused. I do not understand. He is our first child. Will he always have this problem?"
    * Subj.- The father said, "I cannot hold the baby. I do not want to hold the baby."
    * Object- Inappropriate bonding * Object.-Parents not involved in care of baby
    * Object.- Mother will not feed baby or change baby's diapers
    * Object.- The mother has no eye contact with the baby
    * Object.- Father is not interested in holding the child
    * Object.- Parents do not speak directly to the infant

The parents will maintain appropriate parental interaction as evidenced by:

Long term goals:
1. Visit the infant daily.
2. Respond to the infant's needs.
3. Parents will be able to speak directly with the infant.

Short term goals:
1. The parents will be interested in holding the baby.
2. The parents will feed the baby.

Nursing prescriptions:

1. Assess the parent's knowledge of Prune Belly Syndrome and provide teaching.
2. Explain all procedures and treatments to the parents.
3. Encourage the parents to assist in the infant's care such as feeding, bathing, and diaper changing.
4. Provide support for the parents by listening to their feelings and concerns.

Teaching needs:

Because of the long term care the infant will require, the family has several teaching needs. The first priority is the respiratory concern. Because of the decreased lung expansion, the parents need to be taught appropriate respiratory status of their baby. The parents need to be taught the nutrition of their baby. It is important for the parents to decrease the protein and calcium in the diet. This will help prevent renal calculi from forming. Also, giving the baby water will help with preventing renal calculi.

It is important for the parents to be aware of the multiple surgeries their child will need during the long term care. Finally, the parents have needs to be educated about Prune Belly syndrome and all the areas of life that the syndrome affects.

REFERENCES

    1.Psychiatric Nursing. Philadelphia, PA:W.B. Saunders. 2.Bilateral hydronephrosis. (1996.) [On-line]. Available: http://www.ami-med.com/peds/scr).
    3.Doenges, E., Moorhouse, M. & Geissler, A. (1993). Nursing Care Plans, Philadelphia, PA
    4.F.A. Davis, Ehrlich, R.M., Lesavoy, M., & Fine, R. (1992). Total abdominal wall reconstruction in the prune belly syndrome. Journal of Urology, 136, 282.
    5.Ewig, J. (1996). The effect of the absence of abdominal muscles on pulmonary function and exercise. American Journal of Respiratory & Critical Care Medicine, 153, 1314-1321.
    6.Latifi, H. (April 14, 1995). Prune Belly Syndrome [On-Line]. Available: http://www.gamma.wustl.edu
    7.Loder, R., Guibous, J.,Bloom, D., Hensinger, R. (1992). Muskoskeletal aspects of prune belly syndrome. American Journal of Diseases of Children, 10, 1224-1229.
    8.Parrott, T., & Woodard, J. (1992). The Monfort operation for abdominal reconstruction in the prune belly syndrome. Journal of Urology, 148, 688-690. Prune belly syndrome (1996). [On-line]. Available: http://www.cpmcnet.columbia.edu/dept/radiology.
    9.Redman, J., & Mooney, d. (1993). Fowler-Stephens orchiopexy in a patient with prune belly syndrome and segmental artretic vas deferens. Urology, 41, 130-131.
    10.Speer, K. (1992). Pediatric Care Plans. Springhouse, PA: Springhouse.
    11.Stephens, F., and Gupta, D. (1994). Pathogenesis of the prune belly syndrome. Journal of Urology, 152, 2328-2331.
    12.Stuart, G., and Sundeen, S. (1995). Psychiatric Nursing. (5th ed.). Boston: Mosby.
    13.Terada, S., Suzuki, N., Uchide, K., Ueno, H., & Akasofu, K. (1994). Etiology of prune belly syndrome. Obstetrics & Gynecology, 83. 865-868.
    14.Wong, D. (1993). Pediatric Nursing. (4th ed.) New York: Mosby.

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*Provided for informational purposes only via the Prune Belly Support Network. All information found in part or in whole within the context of this research paper does not imply endorsement by the PBSN of any given method of treatment. Medical care MUST be sought in regard to the treatment and management of Prune Belly Syndrome. The objective of the network is to supply anecdotal evidence, and support, only.

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