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Care of the patient with Prader-Willi syndrome.

Prader-Willi syndrome (PWS), a neurodevelopmental disorder identified as a frequent cause of genetic obesity, is a common condition encountered in genetic clinics worldwide. The incidence of PWS is believed to be 1 in 10,000 to 1 in 29,000 live births, with a prevalence rate of 1 in 52,000 with a death rate of approximately 3% (Butler et al., 2002; Whittington et al., 2001). In this article, an overview of several aspects of PWS are presented, specifically how the disorder affects cardiopulmonary, integumentary, and gastrointestinal systems; nutritional status and obesity; pain management; and psychiatric or psychosocial manifestations. Practice implications for the nurse are included.

A literature search was conducted for articles on PWS in the CINAHL, MEDLINE, and PUBMED databases covering the period 1995-2010. Inclusion criteria were PWS, nursing and nutrition management, psychiatric diagnosis, and treatment of PWS for adults. The one exclusionary criterion was pediatric PWS. The search yielded a vast amount of information on the genetic cause of the disorder, world-wide prevalence, patient characteristics, and various treatment approaches. A number of articles focused primarily on weight loss and the maintenance of ideal body weight in order to prevent health risks associated with morbid obesity. Very little information was found in the nursing literature specific to the management of PWS in adults, however.

Etiology and Prevalence

Prader-Willi syndrome, a complex, neurodevelopmental genetic disorder is found in all races and both sexes (Vogels & Fryns, 2004; Whittington et al., 2001). It is the most commonly identified genetic cause of obesity, and first of the 10 most common conditions seen in genetic clinics worldwide. The exact relationship between genetics and clinical manifestations of PWS is not conclusive. This has led more recently to the proposal by Woodcock, Oliver, and Humphreys (2009) of a model to describe the "pathway between genetic characteristics to behavior, with scope for interaction with the environment at any stage" (p. 493).

Four genetic forms of PWS stem either from maternal or paternal causation. Of the 23 chromosomes inherited from each parent, the source of acquiring PWS has been isolated at chromosome 15. The first form, believed to be the most prevalent, is attributed to paternal inheritance and involves approximately 70% of the cases. This occurs when genes are missing (also referred to as deletion) from the long arm of chromosome 15 in the area identified as 15q11-q13. The second form, representing 20% of cases, occurs when the individual erroneously inherits two copies of chromosome 15 from the mother instead of one. In other words, he or she inherits both copies of chromosome 15 from the mother instead of one from each parent. This form is referred to as maternal uniparental disomy, and affected individuals present with slightly milder symptoms. The third form, which occurs in approximately 2%-4% of cases, is believed to result from a loss of function due to an imprinting defect in the paternal chromosome without an interstitial deletion (Cassidy & Schwartz, 2008; Gunay-Aygun, Schwartz, Heeger, O'Riordan, & Cassidy, 2001; Keder & Butler, 2004). Imprinting is a "genetic modification of the genome by which only genes in one of two parental alleles are expressed" (Lidegaard, Pinborg, & Andersen, 2005, p. 950). In the fourth form of PWS, which represents approximately 1% of cases, individuals are believed to have a structural chromosome rearrangement (Cassidy & Schwartz, 2008).

Morbidity and Mortality

In a 10-year longitudinal study of 36 patients with PWS conducted 1991-2001, Smith, Loughnan, and Steinbeck (2003) reported 60% of the 10 deaths in their sample were related to coronary occlusion, strokes, or heart failure. Other researchers found PWS deaths due to complications of obesity. These include cardiovascular and sleep apnea, shock secondary to gastric dilation, and pneumonia and hypoventilation associated with aspiration (Schrander-Stumpel et al., 2004).

Acute gastric distention and necrosis have been reported in a number of individuals with PWS (Stevenson et al., 2007a), particularly following an eating binge among those who are thin but were previously obese. These conditions may be unrecognized because of the high pain threshold among patients with PWS, and can be fatal. Choking, especially on foods such as hot dogs, has been reported as the cause of death in approximately 8% in individuals with PWS (Stevenson et al., 2007b).

Concern about the use of growth hormone has been raised by unexpected deaths of individuals within a few months of starting growth hormone therapy (reviewed in Van Vliet, Deal, Crock, Robitaille, & Oligny, 2004). The few reported deaths occurred in obese individuals with preexisting respiratory problems. In contrast, Nagai and colleagues (2005) reported the rate of deaths in persons with PWS did not differ between those who did and did not receive growth hormone. The relationship of the administration of growth hormone to unexpected deaths of patients with PWS remains inconclusive.

Diagnosis of Prader-Willi Syndrome

Prader-Willi syndrome is a highly variable genetic disorder affecting multiple body systems. In adults, PWS most notably manifests with intellectual disabilities, hypogonadism, growth hormone insufficiency (Cassidy & Driscoll, 2008) characterized by short stature, hypotonia, dysmorphic features, sleep disturbance (Maas, Didden, Bouts, Smits, & Cuffs, 2008), scoliosis (Odent et al., 2008; Weiss & Bohr, 2008), and behavioral dysfunction, of which the most widely known is food seeking and high risk for obesity (McAllister, Whittington, & Holland, 2011). Obesity-related disorders are associated most with morbidity in adults with PWS (Partsch, Lammer, Gillessen-Kaesbach, & Pankau, 2008). Growing evidence suggests hypothalamic dysfunction (Cassidy, Dykens, & Williams, 2000) causes growth hormone deficiency (GHD), short stature, and hypogonadism.

Consensus diagnostic criteria for PWS first developed by Holm and colleagues (1993) have proven accurate. Major criteria are weighted at 1 point each, and minor criteria at 0.5 point each (see Table 1). The supportive findings listed in Table 1 only increase the level of suspicion of the diagnosis and are not scored. For children under age 3, 5 points are required for diagnosis, 4 of which must be from major criteria characteristics. For individuals age 3 and older, 8 points are required for diagnosis, at least 5 of which must be from major criteria characteristics (Gunay-Aygun et al., 2001).

Genetic testing is recommended to ensure an accurate and definitive diagnosis. Widely available tests include the fluorescence in situ hybridization, which detects microdeletions, translocations, and chromosome rearrangements; and the high resolution chromosomal analysis test, which detects deletions, chromosomal abnormalities, and translocations. Less universally available tests include DNA methylation to detect imprinting patterns on the 15q11-13 region, as well as the DNA polymorphism test to identify deletions and parental source of chromosome 15 (Gunay-Aygun et al., 2001; Keder & Butler, 2004). Methylation testing makes a definitive diagnosis in about 99% of patients but will not identify the specific genetic mechanism. However, simple DNA studies of chromosome 15 (q11-q13) will pinpoint the specific type of PWS (Smith et al., 2003).

Systemic Involvement, Patient Characteristics, And Nursing Interventions

PWS can be manifested in multiple ways, including appetite disturbances, ineffective thermoregulation, mood dysregulation, and a high pain threshold (Smith et al., 2003). In addition, an increase in body fat, hypogonadism, decreased bone mineral density, hyperphagia, cognitive disabilities, skin picking, and other behavioral characteristics (Gunay-Aygun et al., 2001) may be present. Hypothalamic dysfunction is believed to be a source of some features of PWS, such as short stature and hypogonadism (Wattendorf & Muenke, 2005). The role of GHD caused by hypothalamic dysfunction remains a subject of research (Angulo et al., 2007; Carrel, Lee, & Mogul, 2006; Lindgren, 2006), with impairment of growth hormone regulation a characteristic of the disorder (Ho & Dimitropoulos, 2010). Growth hormone therapy has been effective in increasing height and muscle strength, improving pulmonary function, and decreasing muscle fat in patients with PWS (Angulo et al., 2007; Haqq et al., 2003; Hoybye, 2007). However, as previously mentioned, obesity comorbid with respiratory problems may be a contraindication for use of growth hormone.

Hypothalamic Dysfunction

Prader-Willi syndrome is characterized by hypothalamic-pituitary dysfunction (Wagner & Berry, 2007). The hypothalamus defect also is believed to contribute to hyperphagia and disruption in appetite control. These leads to obesity, the major cause of morbidity and mortality in this disorder (Papavramidis, Kotidid, & Gamvros, 2006). Secondary hypogonadism also is believed to be due to this dysfunction, resulting in failure to secrete normal levels of gonadotropins (Verhoeven, Tuinier, & Cuffs, 2003). Impaired hormone production in the pituitary gland of individuals with PWS also impacts growth hormone secretion. Decreased growth hormone accounts for the short stature, hypotonia, low metabolism, and low bone density characteristic of the disorder.

Hypothalamic dysfunction also is suspected as a cause of daytime sleepiness (Wagner & Berry, 2007). Fronczek, Lammers, Balesar, Unmehopa, and Swaab (2005) found decreased hypocretin neurons in persons with PWS compared to age-matched controls, suggesting a possible cause of daytime sleepiness in this population. However, the exact role of the hypocretin system in producing daytime sleepiness in PWS remains unclear. Nixon and Brouillette (2002) identified sleep disturbances that were independent from the obesity-related sleep disturbances associated with PWS. Variable thermoregulation frequently is identified as one of the characteristics of PWS. Williams, Rooney, Williams, Josephson, and Pauli (2005) studied temperature regulation of persons with PWS compared to three control groups: siblings of patients with PWS, neurodevelopmentally disabled persons, and age-matched well persons. Their findings revealed no difference between persons with PWS and the neurodevelopmentally disabled controls. Temperature regulation difficulties did not necessarily reflect syndrome-specific hypothalamic abnormalities. Additionally, knowledge of a patient's usual body temperature was important because the patient might have had no fever in an acute illness or a high temperature during a minor illness, often indicating hypothalamic dysfunction (Eiholzer, 2001).

Another complicating symptom associated with PWS is an unusually high pain threshold. Priano, Miscio, Grugni, Baudo, and Mauro (2007) found the presence of impaired thermal and pain stimulus perception in patients with PWS compared to obese patients in a control group. Researchers questioned the presence either of a hypothalamic dysfunction or complex disturbance of the neurotransmitter balance. The lack of normal response to painful stimuli can interfere with early diagnosis of infections and injuries. Nurses therefore must perform frequent assessments to note changes. This highlights the need for conducting a more complex and thorough ongoing assessment of vital signs and behavior, and performing a comprehensive physical exam to note any signs and symptoms of patient problems, including identification of risk factors and subtle changes to prevent problems from developing.

Knowledge of the patient's medical history may be an effective tool in formulating a patient-specific pain scale. Numerous questions should be considered. How has the patient previously reacted in painful situations? Does the patient exhibit restlessness? Does the patient have difficulty assuming a particular position? Is there an increase in repetitive behaviors? The nurse should observe for these symptoms as part of the pain assessment. Pain assessment in the patient with PWS is difficult due to a heightened pain threshold, but it is complicated further by the potential for respiratory depression if analgesics are administered. Evaluating the effectiveness of pain management while maintaining homeostatic mechanisms is essential to ensure patient comfort and safety.

The Impact of Obesity

Adults with PWS are typically morbidly obese (see Figure 1). Morbid obesity is the major medical problem for persons with PWS. Commonly known obesity-related problems include diabetes, high blood pressure, chronic venous insufficiency with associated leg and foot ulcers, cellulitis, and hypoventilation. Common sleep issues related to obesity include excessive sleepiness and sleep-disordered breathing (Williams, Scheimann, Sutton, Hayslett, & Glaze, 2008). In a population study in England, 70% of persons with PWS had significant oxygen desaturation, apnea, and hypoventilation while sleeping (Harris & Allen, 1996). Williams and coauthors (2008) reported a correlation between the body mass index (BMI) and apnea-hypopnea index in their U.S. sample of 37 patients. With 76% of genetically confirmed cases of PWS having abnormal sleep (Gunay-Aygun et al., 2001), patients must be monitored for symptoms of sleep apnea and, in particular, obstructive sleep apnea.

Obesity and resulting sleep apnea present as risk factors for heart disease. Because the main cause of mortality in one sample of individuals with PWS was cardiovascular events, all patients with PWS should be screened for heart disease (Smith et al., 2003). Evidence supported the possibility that repetitive airway collapse during sleep resulted in sleep apnea and sleep fragmentation for persons with PWS (Camfferman, Lushington, O'Donoghue, & McEvoy, 2006). This mechanism could result in additional cardiovascular complications, including cor pulmonale (Marzullo et al., 2005). Alveolar hypoventilation also caused pulmonary vascular obstruction with pulmonary hypertension and finally cor pulmonale (Haqq et al., 2003), in which right ventricular enlargement leads to heart failure.

Nurses must be skillful in identifying signs and symptoms of cardiopulmonary changes. Care plans should include daily weights and assessment of changes in skin color, blood pressure, heart rate and rhythm, and temperature. In addition, daily pulse oximetry should be obtained and respiratory auscultation should be performed, noting presence of adventitious sounds, and symmetry of lung expansion. Follow up on the most subtle changes is imperative due to the increased risk factors related to hypotonia and obesity because these changes could signal a life-threatening situation for the patient (Smith et al., 2003; Wattendorf & Muenke, 2005).

Integumentary

Skin picking is a common behavior associated with PWS (Gunay-Aygun et al., 2001; Ho & Dimitropoulos, 2010), and can lead to infected wounds. Skin picking is attributed to the obsessive-compulsive tendencies of persons with PWS. Poor peripheral perfusion and inactivity increase the likelihood of skin breakdown due to skin picking. Ineffective thermoregulation also contributes to the potential risk for infection because individuals with PWS may not have a febrile response to infection. The nurse should perform and document a detailed skin assessment frequently. Consultation with a wound specialist also may be beneficial for patients who have broken skin or pick at hard-to-protect areas at risk for infection. Of particular concern is picking in the area of an incision or skin that shows signs of inflammation and loss of significant skin integrity. It is important to enhance healing and deter further skin damage. Interventions may include using dressings or other barriers at the site, keeping fingernails short, and avoiding excessive attention to the behavior (Forster & Gourash, 2005; Nolan, 2003). For patients who engage in rectal picking, management should include use of soft baby wipes and if necessary one-piece pajamas as a deterrent to picking (Nolan, 2003). Based on their experience, the authors also suggest hourly application of a greasy antibiotic ointment so picking is not as effective in damaging the skin.

Gastrointestinal

Growth hormone deficiency is seen in adults with PWS (Grugni et al., 2005; Hoybye, Barkeling, Naslund, Thoren, & Hellstrom, 2007). A lack of adequate growth hormone in PWS is believed to promote accelerated protein synthesis, resulting in decreased use of carbohydrates. This causes an increase in fatty-acid metabolism which, coupled with hyperphagia, is partially responsible for the obesity seen in PWS (A. Angerio, personal communication, December 8, 2010; Goldstone et al., 2001). Studies also have revealed a dysfunction in satiety as a problem. Hoarding, stealing, and hiding food are major problems to be addressed when caring for a person with PWS. These behaviors contribute to compulsive overeating (Hiraiwa, Maegaki, Oka, & Ohno, 2007; McAllister et al., 2011).

Inability to vomit has been identified as a characteristic of PWS. Alexander, Greenswag, and Nowak (1987) surveyed 313 individuals with PWS; 36% reported experiencing at least one episode of vomiting but, in general, the sample reported decreased ability to vomit. Their work suggested an alteration in the physiologic set point for vomiting in patients with PWS. Pain in the upper stomach and/or vomiting should be viewed as possible signs of acute intestinal dilation; intravenous support might be life saving (Schrander-Stumpel et al., 2004). It was long believed the stomachs of patients with PWS did not empty well. Hoybye and colleagues (2007) recently found the gastric emptying rate in patients with PWS was similar to those of lean subjects and normal weight individuals. Along with a diminished pain sensation, patients can experience gastric dilation and a heightened risk for gastric necrosis and subsequent rupture. These potential complications are life-threatening and may require urgent interventions, including surgery. Surgical interventions can lead to additional concerns because of increased risk for morbidity and mortality relating to anesthesia. Careful patient assessment is necessary, including monitoring for abdominal distension and rigidity, change in character of bowel sounds, and the frequency and quality of bowel elimination. Patients may need medications, such as stool softeners, fiber supplements, or motility agents. Equally important are dietary interventions, such as adequate water intake, a diet rich in fiber, and an exercise routine designed to establish and maintain regular elimination patterns.

Musculoskeletal

A great deal of attention now is devoted to research on the use of growth hormone to improve the quality of life for persons with PWS. Growth hormone can boost muscle tone and metabolism, normalize height and weight, give higher energy levels, improve respiratory function, and increase bone density to diminish the risk of osteoporosis (Staffer & Wallis, 2008). Odent and co-authors (2008) conducted a retrospective longitudinal study of 145 persons with PWS; 43.4% were found to have scoliosis. The occurrence of scoliosis increased with age and was not affected by genotype or growth hormone treatment. Patients with higher BMI values also had an increased risk of kyphosis. Screening for scoliosis throughout the life cycle with an annual back assessment is essential for early recognition and treatment. Conservative management of scoliosis in persons with PWS is suggested (Weiss & Bohr, 2008) to reduce the potential risks associated with surgical management.

Hypotonia is another lifelong issue for persons with PWS (Cassidy & Driscoll, 2008). One study suggested gait abnormalities in patients with PWS also existed in patients without PWS who had similar BMI values (Vismara et al., 2007). Further study is needed to determine if gait contributes to activity limitations and increases the BMI in patients with PWS. The dysmorphic musculoskeletal features of persons with PWS, such as short stature, muscular hypotonia, acromicria, and scoliosis, need to be examined as possible contributors to gait differences.

Many persons with PWS have osteoporosis, possibly related to growth hormone and sex hormone deficiencies. Hypotonia is probably a contributing factor as well (Prader-Willi Syndrome Association, 2008). Physical inactivity and reduced weight bearing also may contribute to osteoporosis. Vestergaard and colleagues (2004) studied bone mineral status, body composition, and biochemical markers of bone turnovers in patients with PWS. Due to high bone turnover, patients with PWS had significantly lower bone mineral density than controls. Authors identified this high turnover of bone as probably linked to sex steroid deficiency. Additionally, nutritional deficiencies of vitamin D and calcium were believed to play a role. Either dietary sources or supplemental calcium and vitamin D thus should be included in the plan of care.

Nutritional Concerns: Overeating and Appetite

Obesity is an underlying characteristic of PWS owing to an insatiable desire for food. Holland, Whittington, and Hinton (2003) hypothesized that the "syndrome should be redefined as one of starvation that manifests as obesity in a food-rich environment" (p. 989). The individual with PWS does not experience a loss of hunger following food intake. Appetite mechanisms do not seem to mirror those of normal subjects, which in all cases are complex and varied. To some extent, meal-regulating signals originate from gastric peptides that act on the brain center and control energy intake. Ghrelin, one such peptide, stimulates appetite during fasting and decreases after satiety. Somatostatin, another hormone circulating as a gastric peptide, decreases ghrelin in both normal persons and patients with PWS. Recent research on ghrelin concentrations in patients with PWS (Chanoine, 2005) indicated high plasma concentrations of the hormone. Little evidence is available, however, to determine whether ghrelin elevation or an imbalance of somatostatin is responsible for the overeating. Further evidence is needed before concluding these two hormones would be effective as appetite suppressants in patients with PWS.

Used to examine hyperphagia, positron emission tomography and magnetic resonance imaging suggest a dysfunction in satiation controls that accounts for the overeating and subsequent obesity (Holsen et al., 2006). Additionally, scans indicated food preferences, especially for sugary foods, were outweighed by the desire to eat due to a lack of satiety (Hinton, Holland, Gellatly, Soni & Owen, 2006).

Nutritional Intervention

Very little information was found in the literature regarding nutritional management for patients with PWS. Hiraiwa and co-authors (2007) reported patients with PWS who have lower body weight and BMI tended to have more psychological and behavioral problems when placed on severe caloric restrictions than patients with higher BMI. Therefore, severe caloric restriction was not recommended for patients with PWS.

Nurses should emphasize to caregivers and adult patients that energy-dense foods, such as cakes, cookies, carbonated beverages, and other snacks, should not be kept in the home of patients with PWS because they have been known to seek and devour food in abnormal quantities or become aggressive when denied ready access to food. See Table 2 for an outline of specific nutrients of concern for persons with PWS, with related rationales. In addition Table 3 provides a sample therapeutic meal plan specific to the needs of patients with PWS, and can be used by nurses and dietitians. The high-fiber, high-fluid intake with several between-meal feedings was designed to increase satiety while still providing the nutrients of a healthy diet and offering a template for the patients' use upon hospital discharge. As with any eating behavior, prevention or slowing of weight gain may well be the only viable approach until further research provides other options (Vogels & Fryns, 2004).

Psychiatric and Psycho-Social Symptoms, Assessment, and Management

According to Smith and colleagues (2003) and Boer and co-authors (2002), the prevalence of psychiatric problems in adults with PWS is S% to 15%. Other researchers who chronicle the disorder in children and adolescents reported higher prevalence rates of behavioral symptoms (Clarke et al., 2002; Hiraiwa et al., 2007; Steinhausen, Eiholzer, Hauffa, & Malin, 2004). Psychiatric and psychosocial symptoms are known to vary and are manifested differently among individuals. Severity of presentation might differ depending on the following factors: the specific genetic origin of PWS, the family's understanding and management of the disorder, comprehensiveness of interventions, age of the affected individual, intelligence quotient (IQ), and level and quantity of stressors experienced by the individual. IQ scores can range from 60 to the mid 90s, indicating mild retardation to normal level intelligence (American Psychiatric Association, 2000; Eiholzer, 2001).

Behaviors can include pervasive developmental alterations, such as autism, intellectual disabilities, learning disabilities, and difficulty with language processing. Other psychological and physiological symptoms include obsessive-compulsive behaviors, psychosis, mood dysregulation, oppositionalism, aggression, self-injurious acts such as skin picking, and impulsive eating (Milner et al., 2005; Soni et al., 2008; Steinhausen et al., 2004; Wigren & Hansen, 2003). In research using large samples of individuals with PWS, Dykens (2004) and Dykens and Roof (2008) concluded behavioral and psychiatric symptoms became less problematic as affected individuals entered later adulthood and with out-of-home placement. In the population of persons with PWS resulting from maternal uniparental disomy, reduced skin picking was seen but there was an increase in affective, psychotic, and autistic symptoms (Boer et al., 2002; Soni et al., 2007). Individuals whose PWS etiology stemmed from deletion or imprinting displayed more temper tantrums that decreased with age, an increase in obsessive-compulsive behaviors, and more skin-picking behaviors that put them at greater risk for skin and systemic infections (Clarke et al., 2002; State, Dykens, Rosner, Martin, & King, 1999). A study by Dykens (2004) indicated individuals with PWS had the most difficulty during adolescence and early adulthood.

Socially, individuals with PWS lack the skills to relate appropriately to others. They can be impulsive, react poorly to changes in routines, display impaired judgment, lack the ability to filter social cues, exhibit poor decision-making skills, and have difficulty delaying gratification (Koenig, Klin, & Schultz, 2004; Nolan, 2003). As a result of possible cognitive limitations and/or behavioral symptoms, assessment data should be obtained both from the patient and adults familiar with the affected individual. This ensures access to the most comprehensive and accurate information that can be used to guide treatment. Forster and Gourash (2005) identified five domains of behavioral symptoms seen in persons with PWS: (a) food-related behaviors, Co) oppositional behaviors, (c) cognitive inflexibility, (d) insecurity associated with increased anxiety, and (e) skin picking. Ideally, nursing interventions should provide a structured, predictable environment where expectations are clear and a reward or recognition system is in place (Dykens & Shah, 2003). Because of the potential for complex behaviors from patients with PWS, a multidisciplinary team approach including the patient and family should be implemented, with a comprehensive treatment plan developed early in the treatment and revised as needed. A genetics expert should be an active part of the team. As for other patients with psychiatric and behavioral symptoms, consistency, modeling appropriate behaviors, patient teaching, and relationship building are important nursing interventions (Nativio, 2002).

Patients with PWS may present with co-morbid symptoms of anxiety, obsessive-compulsive behaviors, depression, inflexibility in thinking, and mood instability. In a study conducted in Japan, questionnaires were mailed to parents of individuals with PWS to assess presence of behavioral problems and psychiatric symptoms. Of the 369 surveys mailed, 177 were returned; 29 of the responses were from young adults ages 18 to 31. Data from this subset revealed higher rates of behavioral and psychiatric symptoms, such as self-injurious behaviors, delusional thinking, temper tantrums, and emotional liability, than in other age groups (Hiraiwa et al., 2007).

Prevalence of associated psychiatric symptoms might warrant the use of psychotropic medications. However, responsiveness to these medications has been inconsistent and in some cases has actually increased severity of symptoms. Antidepressants such as selective serotonin re-uptake inhibitors (SSRIs) (e.g., fluoxetine [Prozac[R]]) have been very effective in treating aggression, irritability, and perseverative symptoms (Soni et al., 2007; Whitman & Greenswag, 1995). However, both persons with PWS and those without the disorder are biologically at risk for mood disorders, and use of SSRIs can exacerbate the level of mood instability and lead to full-blown manic behaviors (Forster & Gourash, 2005). In a recent study of 119 individuals with PWS in England, Soni and co-researchers (2007) found the presence of more affective symptoms in the study population. Antidepressants and atypical antipsychotics, such as olanzapine (Zyprexa[R]), risperidone (Risperdal[R]), and aripiprazole (Abilify[R]), were more effective in managing symptoms than mood stabilizers such as carbamazepine (Tegretol[R]). Nurses need to be aware that psychotropic dosing in patients with PWS should be lower than in patients without PWS to avoid toxicity and rebound symptoms that might be due to sensitivity to medications. In addition, atypical antipsychotics have been implicated in weight gain in many patients (Mauri et al., 2006; Newcomer, 2007), a factor that is particularly troublesome with this population.

Discharge Planning

If the patient with PWS is hospitalized, discharge planning should include pre-discharge meetings with the patient, family, health care providers, and staff from out-of-home placement facilities if that is the post-discharge destination. An agreed-upon plan of care and community resources should be provided to the patient, family, or out-of-home placement staff in writing. Using a consistent case manager to support access to services and adherence to treatment recommendations in the community also could prove beneficial. Pre-discharge assessment of out-of-home placement should be conducted by social service personnel to ensure appropriateness of the facility as well as adequate knowledge and management skills on the part of the staff, because individuals with PWS pose difficult and complex management needs. Whether the discharge plan indicates discharge to home and family or out-of-home care, attempts should be made consistently to include family members in the life of persons with PWS, with their permission. Individual and family support groups exist specifically for persons affected by PWS in many communities. In addition, the PWS Association has a very active web site to answer questions, and supply referral information and materials about PWS (www.pwsausa.org).

Conclusion

The vast array of abnormalities associated with PWS make the syndrome difficult to manage. The disorder takes a significant toll on the patient and the family. In treatment of PWS, the primary focus should be on weight control because obesity and its subsequent co-morbidities are risk factors for mortality. Each patient, however, requires an individualized plan of care. The nurse and support staff are faced with the challenge of dealing with PWS in environments where little is known about effective management of the syndrome and where management results have been inconsistent. A multidisciplinary approach to management of PWS has been highlighted in this article, along with elements that should be included in a treatment plan, to help nurses deliver comprehensive care to meet the complex biopsychosocial needs of adults with PWS.

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Edilma L. Yearwood, Myrtle R. McCulloch, Michelle L. Tucker, and Joan B. Riley

Edilma L. Yearwood, PhD, RN, PMHCNS, BC, FAAN, is an Associate Professor, Georgetown University School of Nursing and Health Studies, Washington, DG.

Myrtle R. McCulloch, EdD, RD, is Assistant Professor, Nutrition, Department of International Health, Georgetown University School of Nursing and Health Studies, Washington, DC.

Michelle L. Tucker, MSN, RN, is a Nursing Instructor, Radians College, Washington, DC.

Joan B. Riley, MS, MSN, RN, FNP-BC, is an Assistant Professor, Georgetown University School of Nursing and Health Studies, Washington, DG.
TABLE 1.
Clinical Manifestations of PWS

Major Criteria            Minor Criteria            Supportive Criteria

* Characteristic facial   * Decreased fetal         * High pain
  features: narrow          movement and              threshold
  bifrontal diameter,       infantile lethargy,     * Decreased
  almond-shaped             improving with age        vomiting
  palpebral fissures,     * Typical behavior        * Scoliosis and
  down-turned               problems,  including      /or kyphosis
  mouth, strabismus,        temper tantrums,        * Early adrenarche
  thin upper lip            obsessive-compulsive    * Osteoporosis
* Developmental delay/      behavior,               * Unusual skill
  mild-to-moderate          stubbornness,             with jigsaw
  intellectual              rigidity,                 puzzles
  disabilities/             stealing, and lying     * Normal
  multiple learning       * Sleep disturbance/        neuromuscular
  disabilities              sleep apnea               studies (e.g.,
* Hyperphagia             * Short stature for         muscle biopsy,
* Hypogonadism manifest     the family by age 15      EMG, NCV)
  as genital                years
  hypoplasia; small       * Hypopigmentation
  labia minora and        * Hands and feet small
  clitoris in               for height age
  females; hypoplastic    * Narrow hands with
  scrotum and               straight ulnar
  cryptorchidism            border
  in males; incomplete    * Esotropia, myopia
  and delayed puberty;    * Thick, viscous
  infertility               saliva
* Neonatal and            * Speech articulation
  infantile central         defects
  hypotonia with          * Skin picking
  poor suck, improving
  with age
* Feeding problems
  and/or failure
  to thrive in
  infancy
* Onset of rapid weight
  gain between ages 12
  months and 6 years,
  causing central
  obesity

Note: Adapted from Cassidy & Driscoll, 2008; Gunay-Aygun
et al., 2001; Holm et al., 1993; Wattendorf & Muenke, 2005

TABLE 2.
Main Nutrients of Concern Specific to Patients with PWS

Water          Vitamins and           Carbohydrates      Protein
               Minerals

An adequate    For maximum            High-fiber         Adequate
intake         fiber intake,          foods provide      lean meats,
may ensure a   fresh  fruits          few calories,      eggs, and
decreased      and vegetables         have a high        poultry are
risk for       should be used         satiety            important
fecal           rather than           value, maintain    for their
impaction.     canned varieties.      a healthy          protein quality
               Juices with            gastrointestinal   and quantity
Recommended    no added sugars        tract, and         and to maintain
amount is      should be              help maintain      musclemass,
6-8 glasses,   limited to no          blood glucose      observed
with           more than 6 oz.        levels. These      to be
quantity       per day. Fruit/        include whole      significantly
based          vegetable juice        fresh fruits       lower in
on age.        combinations should    and                PWS obesity.
               be avoided             vegetables,
Monitor for    because most are       and whole          Meats should
electrolyte    low in fiber and       grain              be grilled,
balance        high in added          breads and         broiled, or
due to         sugars.                cereals.           baked with
risk  for                             Cereals, breads,   no breading
hyponatremia.  Foods in the milk      rice, and          or sauces.
               group are included     pastas             Poultry
               to provide calcium     should only be     should
               and vitamins A         whole grain,       be skinless
               and D. This is         because most       and mostly
               especially             of these foods     from white
               important because      have a low         meat. Eggs
               patients with PWS      glycemic index     should be
               exhibit signs of       and may be         boiled or
               low bone mineral       helpful in         fired using
               density and problems   preventing         cooking
               with dentition.        the onset of       spray in a
                                      prediabetes        non-stick
                                      or Type 2          frying pan.
                                      diabetes
                                      mellitus.          All protein
                                                         sources
                                      Caregivers         should be
                                      should note        cooked
                                      "whole             thoroughly
                                      grain"             to prevent
                                      or "stone          food-borne
                                      ground" as         illness.
                                      the first
                                      or second          One whole
                                      ingredient         egg may be
                                      in the list        served two
                                      of a food          to three
                                      label; this        times a
                                      indicates a        week, but
                                      high-fiber         egg whites
                                      food. In           may be used
                                      bread products     as often
                                      a brown color      as necessary.
                                      is not an          Control of
                                      indication         cholesterol
                                      of whole           intake would
                                      grains             help
                                      because            prevent
                                      molasses           cardiac
                                      imparts            problems
                                      color              related
                                      without            to obesity.
                                      ensuring
                                      food
                                      is high
                                      in fiber.

Note: Developed by Myrtle R. McCulloch. Adapted
from Bots, Schueler, Brand, & van Nieuw Amerongen,
2003; J. Heineman Personal Communication, 2006;
Sell et al., 1999; Vestergaard et al., 2004; Yigit,
Estrada, Bucci, Hyams, & Rosengren, 2004.

TABLE 3.
Sample Meal Plan (1,800-2,000 calories) with Dietary
Reference Intakes for Vitamins and Minerals

                           Quantities Should Be Age Dependant Using the
Typical Meal Plan          Food Pyramid Guide for Serving Sizes

Breakfast 7:00-8:00 a.m.   Whole grain cereal, oatmeal, or whole
                           grain toast with low-sugar fruit spread
                           or low-fat ricotta cheese; 4 oz. skim
                           milk.

Snack 10:00 a.m.           6 oz. of skim milk; whole grain, low-fat
                           crackers with low-
                           sodium; reduced-fat peanut butter.

Lunch Noon                 Homemade vegetable soup with a chicken
                           broth base. Lean poultry (no skin)
                           broiled or baked and well seasoned
                           to provide taste lost through
                           preparation. Steamed or raw
                           vegetables, whole grain brown
                           rice or pasta, fresh fruit.

Snack 3:00 p.m.            Water, baby carrots, grape tomatoes,
                           cucumber slices,
                           broccoli florets with low-fat yogurt
                           flavored as a dip.

Dinner 6:00 pm             Vegetable soup in beef or chicken
                           broth. Lean poultry, salad with
                           a no-calorie dressing, small whole
                           grain pita bread, rice, or pasta.

Light Snack 8:30 p.m.      Walnuts or almonds and fresh fruit.

Typical Meal Plan          Rationale

Breakfast 7:00-8:00 a.m.   Whole grains provide fiber to increase
                           satiety without calories. Milk-based foods
                           provide calcium and vitamin D.

Snack 10:00 a.m.           Juices should not exceed 6 oz. per day.
                           Grains and fat in peanut butter' provide
                           satiety value.

Lunch Noon                 High-fluid, low-calorie soup provides the
                           nutritional benefits of vegetables, fiber,
                           and fluid to curb the appetite.

Snack 3:00 p.m.            To provide nutritional and satiety value.

Dinner 6:00 pm             Adequate protein and a high-fiber
                           carbohydrate  source for nutrients as well
                           as the fiber benefits

Light Snack 8:30 p.m.      This last meal is designed to ward off
                           hunger in the early morning hours.

Source: Developed by Myrtle R. McCulloch.
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Author:Yearwood, Edilma L.; McCulloch, Myrtle R.; Tucker, Michelle L.; Riley, Joan B.
Publication:MedSurg Nursing
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Date:May 1, 2011
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