16
Residential Care for Adults with Prader-Willi Syndrome
Mary K. Ziccardi
Provision of residential services to adolescents and adults with Prader- Willi syndrome was not seriously addressed prior to the late 1970s, primarily because until then most individuals were not expected to live beyond the adolescent years.3 As a natural outgrowth of earlier diag- nosis and appropriate nutritional and weight management, individuals with Prader-Willi syndrome now live well into adulthood. Thus, from the mid-1980s to the mid-1990s, establishment of adult residential services was a major thrust of both local chapters and the national Prader-Willi Syndrome Association (USA). And, while many additional programs are needed to care for this expanding population, nonethe- less, this experience has taught us that individuals with Prader-Willi syndrome have unique needs that must be addressed in order to ensure a safe and successful living environment. This chapter addresses the issues involved in both the process of selecting a supported living environment and the necessary elements of that environment. These elements are the minimal requirements needed for the success of any supported living situation, ranging from the single-resident apartment to larger, congregate settings.
For many families, exploring residential placement for their family member is an overwhelming and emotionally charged task.1 At the most basic level, placements are diffi cult to locate and secure. Many families face long waiting lists and lack of governmental funding for developing appropriate alternative living opportunities. Families are often forced to wait years for any residential facility that is even remotely willing to assume the challenges of serving someone with Prader-Willi syndrome (PWS).
That said, the family’s full participation and disclosure of their family member’s strengths and needs is the fi rst step in securing a successful residential placement. A residential provider has a signifi cantly improved chance of meeting the individual’s needs if those needs are discussed honestly and openly. Many willingly provide behavioral accommodations and environmental modifi cations to facilitate a lasting placement that includes a clinically sound approach to treatment, an acceptable quality of life, and positive outcomes. The prospective pro-
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vider and the family will be most successful for the individual with PWS if a spirit of openness and cooperative, respectful communications are established early in the relationship.
Questions Families and Providers Should Ask
For most families, the task of selecting a residential provider is a process and experience unlike any other. While families may not know what areas to address and questions to ask, at the same time providers may be unaware of the potential impact on families of an agency’s culture and operational structure. Many families benefi t from training work- shops that both educate and support them through the process of selecting residential supports and services.2 In addition to providing factual information regarding funding, eligibility, and application proc- esses, many workshops provide an informational checklist to help families obtain suffi cient information for making informed placement decisions.
Some of the questions that a family may fi nd benefi cial to ask when interviewing a potential provider are included in Table 16.1.
While not all-inclusive, this list can provide a stepping-off point in seeking and sharing information that may result in a positive, long-term relationship between the family and the provider agency.
Asking and discussing these questions with a potential provider agency can assist in determining if the agency’s program matches the family member’s needs. Further, any potentially contentious issues can be openly and thoroughly discussed. Likewise, the set of questions suggested in Table 16.2 might assist the residential service provider in better preparing to serve an individual with Prader-Willi syndrome.
We will discuss a number of environmental and program areas that are critical to the quality and success of a residential placement.
Program Issues
Provider’s Manifestation of Philosophy
When a family is considering their residential options, the philosophy of the provider agency should also be considered. Agencies, just as families, may consciously or unconsciously develop a set of beliefs and practice accordingly. Guardianship may be one of the more signifi cant areas that merit early exploration. For example, is the family planning to assume guardianship of their adult son/daughter? Some agencies may demand guardian oversight as well as an active role in the seem- ingly smallest occurrences. Providers who advocate this philosophy may solicit a total team approach, involving the families in the home purchase, renovations, staff selection and training, behavioral supports and policy setting. Families may need to consider their own relation- ship dynamics, availability, and degree of interest in participating. Con- versely, other providers may prefer that families practice a more “hands off” approach, only notifying a family for the most major life issues.
Table 16.1. Questions Families Should Ask Potential Providers of Residential Services General
1. How long has the agency been in business?
2. What is the depth of the agency’s experience supporting people with Prader-Willi syndrome?
3. How many people have you discharged from your program in the past year? Regarding those that had Prader-Willi syndrome, in general terms, what were the reasons for the discharge(s)?
4. With permission, can the prospective provider share names and phone numbers of individuals and their families who currently use the agency’s services and would be willing to be a reference for the agency?
5. Does the agency have any written materials about the services that we could take with us?
6. How can I obtain a copy of the most recent licensing survey, if applicable?
Agency Staffi ng and Consultants
7. Describe the hiring process used when selecting staff.
8. What initial and ongoing training does the agency require of staff?
9. How are direct support staff supervised?
10. What is the turnover rate for direct support and supervisory staff? What is the average length of employment for a direct support staff at your agency?
11. How does the provider select and access physicians, ancillary medical services, behavioral consultants, etc?
Agency Policies
12. How does the agency handle an individual’s fi nances?
13. Do you provide reports to the individuals and/or their families regarding their fi nances? If so, how frequently?
14. What are your agency’s policies regarding medication and its administration?
15. What contingencies are in place for medical and behavioral emergencies?
16. How can a supervisor be contacted by a family during an emergency situation outside of normal offi ce hours?
17. What is the frequency of your communications with families? What if my family wants to know more/less than the standard?
18. What issues are you mandated to report to parents/guardians?
19. What is your agency’s policy on visitation? Can families visit unannounced? Would we have a private place in which to visit?
20. What is the agency’s grievance procedure for the individuals and/or their families?
Program Issues
21. How does your agency individualize services to meet varying needs?
22. What is your agency’s relationship to the individual’s school or vocational programs? Can or will it change based on my family member’s individual needs?
23. What recreational, religious, and social activities are available? Are the opportunities for participation individualized, accessible, and consistent? What happens when an individual chooses not to participate and the rest of the group plans to go?
24. What kind of transportation is available for the individuals’ use?
25. What is the agency’s relationship with neighbors and the community at large? Describe how the individuals belong and have a community presence.
Source: Adapted from C. Norwood, What Questions Should I Ask? Center for Mental Retardation, 2002.2
An honest self analysis that defi nes the family’s style and comfort level and fi nding a provider that best matches their beliefs can be critical to a placement’s long-term success.
Families and providers also need to openly discuss larger issues regarding their views and practices surrounding the very meaning of normalization and to what depth community integration is expected.
Discussing the negotiables of these issues can be far-reaching to many
signifi cant aspects of the individual’s daily life. Does the son/daughter ride a sheltered workshop bus or independently travel—that is, become trained to use community transportation? How are food and caloric restrictions handled during family visits and holiday celebrations? Is the agency expected to provide staff to accompany and support the individual at family events? Can the individual carry spending money?
A common ground should be realized regarding the extent to which an individual with Prader-Willi syndrome is their “own person,” able to make decisions and reap the benefi ts or suffer the consequences. Or, does the agency advocate that the individual should be supported and afforded freedoms within the confi nes of the developmental disability, while receiving more intensive direction and supports for issues related Table 16.2. Questions Providers Should Ask Families Seeking Residential Services General
1. What is your family’s expectation of this placement? For example, do you consider this a respite or short-term assistance for your family, or do you expect this to be your son’s/daughter’s permanent home?
2. For what issues/incidents would you like to be contacted immediately? What is the best manner in which to reach you?
3. If you are not your (adult) son’s/daughter’s guardian currently, do you intend to apply for guardianship?
4. What are your preferred days/times for team meetings?
5. Do you prefer written communications from the agency staff? Barring any major incidents, how often would you like an update?
6. What assistance, if any, would your family like in preparing for the move and transitioning of your son/daughter to the new home?
Agency Policies and Staffi ng
7. Do you anticipate that you will have input into the agency’s hiring decisions?
8. What are your expectations of how confl icts will be resolved?
9. Describe your preferences regarding medical appointments. Will you be scheduling any or all of them? Attending any or all appointments? With or without agency staff?
10. How much involvement would you like regarding your child’s fi nances?
Program Issues
11. What are your preferred methods of managing the behavioral manifestations of Prader-Willi syndrome exhibited by your son/daughter?
12. What interventions have been consistently successful with your son/daughter? What other interventions have been attempted but proven unsuccessful for your child?
13. Are you using a behavior support plan currently?
14. Should extended family visitation be used as a reinforcer for your child?
15. Do you have a relationship with your child’s current educational/vocational staff?
16. Will you need assistance from agency staff with transporting your child for routine family visits?
Would you like a staff member to assist your son/daughter by accompanying them to other family events, i.e., holidays, birthday celebrations, weddings, etc.?
17. Other than yourself, who else in your extended family can visit your child? Who can take them out of the facility for day/overnight visits?
18. Does your son/daughter have a religious affi liation? Which church/synagogue does he/she regularly attend?
19. How will your child’s prescription medications be secured, if necessary, and administered during a visit to your home?
20. During visits to your family home, would you like to have menus and/or food packed to assist with nutritional management?
to health and safety? In addition to exploring the daily dynamics iden- tifi ed in Table 16.1, all parties would benefi t from delving into the reality of the practices manifested from the overall philosophical foundations.
Before starting out, families should establish their “must-have” pri- orities, whether it is location, number of housemates, behavioral man- agement strategies, parental involvement, or other issues critical to their values and wishes for their family member, and consider conced- ing some smaller, less important issues. When faced with minor com- promises and small disagreements, it’s important to be mindful of the greater gains to be achieved for and by the person with Prader-Willi syndrome. Families should ask themselves and the agency how the person’s health, their very life, will be improved and enriched by this residential placement.
Once the selection has been made and an individual has been accepted into a program, the family and provider agency must work as a team to provide the best care and support possible. Any obvious disagreements, displayed by either party, can and will most likely be used by the person with Prader-Willi syndrome to create confl ict and to manipulate. If an issue that requires discussion becomes evident, the family and provider representatives should make every effort to discuss it out of earshot of the individual receiving services. Remaining com- mitted to respectful and professional interactions will go far in provid- ing a secure environment in which the individual with Prader-Willi syndrome can learn new skills, remain healthy and safe, and achieve a quality of life.
Environmental Requirements
Several environmental factors are key to providing both a safe and successful residential program for people with Prader-Willi syndrome.
For most, securing food is primary. The methods used to do so are ultimately of less importance than the simple fact that it is secured. To many with Prader-Willi syndrome, the very knowledge that food is inaccessible is a comfort and reduces a great deal of anxiety. Many residential programs choose to utilize a totally locked kitchen; others elect to lock refrigerator and cupboards. Either method is appropriate.
Most importantly, consistency in properly securing these locks must be applied by everyone at all times.
There are a variety of methods to prepare and apportion the meals and snacks. Determining what is best in a particular environment depends on the overall philosophy, the physical structure of the home, and the number of people who live and work there. Constant factors, however, include the need for accurate menus and recipes. In addition, those who cook and prepare food must be adequately trained to measure quantities in accordance with the calories prescribed. The role of the dietitian is later addressed in this chapter under “Medical Care and Ancillary Services.”
Individuals with Prader-Willi syndrome enjoy the comfort and per- ceived sense of control of their own lives associated with the anticipa-
tory knowledge of what foods will be included in meals and snacks.
To this end, a menu board posted daily outside the kitchen may help alleviate some of the ever-present questions and concerns regarding food.
To accommodate the short stature of many adults with Prader-Willi syndrome, especially those who have not benefi ted from growth hormone therapy, providers may want to lower shelves and closet rods when building or modifying a home. This simple adaptation can serve to increase an individual’s independence.
Most people with Prader-Willi syndrome “skin-pick”; the frequency and severity of skin-picking episodes can be especially challenging for all involved. Therefore a range of behavioral interventions and treat- ment approaches may be needed (see Chapter 12). A water-circulating, Jacuzzi-style bathtub can assist with healing open areas and promote skin integrity. Under some funding sources, the Jacuzzi tub may be allowed and reimbursed as medical equipment.
People with Prader-Willi syndrome have unique space needs. A pro- pensity for both collecting and hoarding, accompanied by an extraor- dinary sense of protectiveness for these collections, particularly from others, dictates that the resident must have ample space. Individual bedrooms, which are locked and accessible only to the occupant and support staff, are ideal to reduce both real and imagined incidents of stealing and property destruction. When single bedrooms are not pos- sible, strict guidelines need to be established and enforced with regard to individual space, property, and privacy. Integrating these expecta- tions into the “house rules” may help to prevent disagreements later.
Furnishings in the home should be durable, sturdy, and easily cleaned and maintained. When making purchases, consider items made of wood that cannot be easily overturned. As an example, purchase a console television rather than an entertainment center, which may be tall, may contain several pieces of audio-visual equipment, and would be easier to topple. Unwieldy pole lamps or ceiling-installed track lighting may be better choices than table lamps that can easily be thrown in the midst of a behavioral episode. For decorative purposes, small wicker baskets, items made of soft material, and hanging quilts may be preferable to metal and glass picture frames, large mirrors, and candlesticks. When hanging large frames, consideration should be given to discreetly bolting the frame to the wall. The individual’s pref- erences, safety, and common sense should be balanced when making these decisions. Further, the intensity of environmental safeguards pro- vided should be commensurate with the behavioral needs of the residents.
Large and open common living spaces, such as a family room and living room may serve multiple purposes such as providing opportuni- ties for family visits, individual and small group activities, and social interactions. However, a common television may require supervision to avoid arguments about “who chooses the next show.” Small game tables, for crafts and puzzles, are often used. A computer loaded with games and e-mail access may also be appropriate, when used with some supervision.
The proper utilization of both separate bedrooms and common living areas is essential to containing and managing behavioral tantrums, including possible escalation of physical aggression and property destruction. When the house rule “Leave the room when asked” is enforced, and plenty of space is available, potential chain-reaction behavioral tantrums may be avoided.
Overall, carpeted areas in the bedrooms and general living spaces seem most desirable and comfortable. Wide staircases, with carpet or treads and handrails on both sides, provide additional safety. Due to affected individuals’ propensity to hoard items, storage closets can be very benefi cial in reducing the general clutter in the bedrooms.
These closets can be used for off-season clothing storage, as well as storage of overfl ow word-search books, jigsaw puzzles, crafts, and videos.
If space is available, it is ideal that key supervisory staff have an offi ce area on-site. This will afford a private space to consult with family members, complete necessary paperwork, and train and counsel staff. Ideally, this offi ce space can be locked and inaccessible to the men and/or women living in the home.
Yet another environmental consideration in program location is access to the community. Neighborhoods with recreation centers (e.g., indoor and outdoor paved walking tracks, swimming pools, basketball courts), bowling alleys, churches, shopping, and movie theaters may help to promote exercise and encourage an overall sense of community belonging. Conversely, building a program next to a restaurant or con- venience store might invite food-related incidents and simply provoke already challenging behaviors. While food cannot be completely avoided when in the community, common sense and good judgment should prevail.
No environment is perfect and without pitfalls. Families and provid- ers can work together to prioritize, set guidelines, and defi ne expecta- tions. All involved will need to be prepared to adapt and make changes to meet current and future challenges.
Staff Training and Supervisory Approaches
A well-trained, supported, and empowered staff is the cornerstone of any successful residential program. These attributes are even more critical for supporting people with Prader-Willi syndrome. Creating a successful team of staff begins with the employment application and interview phases. It is helpful to develop some questions specifi c to the program’s structure and needs. Every attempt should be made to assess the applicant’s approach to diffi cult situations, such as being the target of verbal or physical aggression. It is often benefi cial for the applicant to be interviewed by more than one supervisor, either in subsequent appointments or by using a team interview approach. Further, requir- ing the applicant to respond to written questions may help determine literacy and problem-solving abilities, while also providing additional insight as to whether the applicant would be a positive addition to the team of staff.
Once a hiring decision has been made, both initial and ongoing train- ing is crucial. Training sessions on the most basic issues, such as genet- ics, environmental controls, diet, exercise, and behavior management strategies, is best completed prior to an introduction to the residential setting. Once a new staff member has toured the home and been intro- duced to the program, an orientation checklist detailing specifi c require- ments and approaches can be an excellent training tool. Further, a new staff member should ideally be trained by an experienced supervisor or lead staff, working side by side for a full 40-hour work week on the shift they will be scheduled to work. The opportunity to witness and participate in a variety of shift responsibilities and events will only serve to strengthen the trainee’s overview of the entire program.
Hiring and initial training of new staff is only one aspect of the overall training program. Direct support staff encounter challenges daily. Ongoing support and training are required to ensure that the residents are constantly provided with the best possible responses from staff. When a program is new, or when diffi culties arise, weekly staff meetings are benefi cial. A group of work partners who are cohesive and empowered can be solidifi ed through an atmosphere of sharing, honesty, and mutual support. Role-playing actual or potential events is a great learning tool for staff.
The program supervisor is key to a successful environment. This leader must earn the respect of staff, individuals served, and parents.
The supervisor must establish a strong presence on each shift, demon- strate the ability to mediate the issues of both individuals served and staff, and display expertise in training and coaching others. The direct line supervisor will most likely interact routinely with parents and other professionals and be required to lead an interdisciplinary team to consensus on a variety of diffi cult issues.
Not only should direct care staff be supported, they must also be empowered to make the multitude of decisions that are needed on any given shift. Therefore, if a staff member needs to seek advice or direc- tion from a supervisor, it is best done out of earshot of the individuals with Prader-Willi syndrome. This simple approach may help to estab- lish the credibility and authority of the direct care staff.
A career supporting people with Prader-Willi syndrome is not the best match for everyone. Successful staff must possess maturity, display consistency, and not need the “last word” in a debate. Finally, staff must demonstrate forgiveness and know that, regardless of what happened today, tomorrow is a new day. Nationwide, the most successful pro- grams have little staff turnover. Staff possess integrity and maturity and are highly trained and competent. Constant staff turnover is a key indicator of programmatic problems.
Programmatic Components: Behavioral Management and Skill Development Issues
The content of the program is as critical as staff training. People with Prader-Willi syndrome require—nay, demand—consistency and struc- ture in all areas including programming.
Generally, a basic set of house rules, applied equally to all, will estab- lish baseline standards for acceptable behavior. Depending on the structure and uniqueness of the residential program, some examples of house rules may include the following:
1. Keep your hands and feet to yourself.
2. Leave the room when asked.
3. No food is to be brought into the house.
4. No trading, loaning, or borrowing items.
5. Keep your bedroom door locked at all times.
Based on overall cognitive abilities, including understanding of cause and effect (i.e., if I do x, then y will occur), a person with Prader-Willi syndrome may function best with a behavior plan that offers rewards and consequences. A young lady with Prader-Willi syndrome was once overheard to say, “We don’t do something for nothing!” Capitalizing on that approach may be easier said than done, because, historically, reinforcers used in MRDD (mental retardation/developmental dis- ability) programs have been food-based. While it is indeed a challenge to identify potential nonfood reinforcers, it is not only possible, it is the responsibility of caregivers to do so. Behavior support plans must be individualized and must address the unique needs, strengths, and desires of each person. Many years of successful programming coupled with practical, anecdotal experience of many professionals have established that response cost programs, level systems, and point systems work well. A brief explanation of each follows:
1. Response Cost
The basic premise of this strategy is that, while an individual has the opportunity to earn “tokens” for compliance (e.g., absence of target behaviors or completion of daily expectations), previously earned tokens can also be forfeited for negative behavior. The reinforcement schedule of the tokens can be for task-based compliance or at pre- determined intervals. This may work best if the token is a tangible item so that it can be visibly presented and retained for desirable behavior, as well as functionally returned for negative, less desirable activity.
2. Level Systems
This program strategy requires that privileges be earned and available relative to the type and amount of compliance and positive, desirable behaviors. More coveted and diffi cult-to-attain privileges are rewarded at the highest level; that is, when the greatest amount of compliance is shown the most desired reinforcers are available. This approach may work best with an individual who can cognitively grasp future goals, plan accordingly, and make informed choices about willingness to comply in order to earn desired rewards.
3. Point System
This approach can be designed much like the strategy used with the level system. Various expectations are assigned a point value, and points translate into privileges and reinforcers. This plan may work
best with someone who doesn’t require a concrete “token” as a symbol of their compliance but for whom the level system is too abstract.
Behavior support plans for individuals with Prader-Willi syndrome have the best chances for success when they are soundly designed, supported by parents and signifi cant others, and consistently imple- mented. Because individual needs can be constantly changing, behav- ior support plans should be regarded as perpetual works in progress.
Accurate and complete data recording of behavior plan compliance is an essential responsibility of each and every shift. Many of the most common behavioral strategies used with people with Prader-Willi syn- drome build on each other. For example, if a staff member who works on a Tuesday afternoon shift leaves without documenting program compliance, the staff working on Wednesday may not have all of the information needed to determine the individual’s privilege status.
While this certainly does not qualify as an acceptable clinical practice, lack of documentation is also problematic because it leads to inconsis- tent program implementation, staff not working as a team, and possible manipulation of the behavior plan by the person with Prader-Willi syndrome. Conversely, if this lack of documentation has resulted in the loss of an earned reward, which subsequently precipitates a behavioral incident, it may appear that the behavior plan is unsuccessful, when in fact it was working. Staff must be held accountable for consistent and thorough completion of basic program documentation. The uncanny ability of some individuals to determine the weakest point of any behavior support plan further compels the interdisciplinary team to continuously assess the plan’s strengths and weaknesses and modify accordingly.
Many people with Prader-Willi syndrome possess and can display adequate skills in completing personal care and household tasks. While compliance may be an issue, skills are often at acceptable levels. There- fore, creating meaningful skill development program ideas can be challenging. A comprehensive functional assessment could assist in determining need areas. The interdisciplinary team can use this infor- mation to determine priority areas and may want to consider integrat- ing compliance into the reward system of a behavior support plan.
While most people with Prader-Willi syndrome require high levels of supervision throughout their lives, greater independence and auton- omy can be achieved through increasing skills and abilities in many areas.
Medical Care and Ancillary Services
Locating, informing, and working cooperatively with physicians is a major responsibility of a residential provider. In some cities where resi- dential services for people with Prader-Willi syndrome exist, physi- cians are knowledgeable, experienced, and well versed in treating the multifaceted needs arising from the syndrome. Unfortunately, this is often the exception rather than the rule. More often, the provider and parents must work to develop relationships with physicians willing to
provide primary care as well as specialists for psychiatric oversight, podiatric care, dental treatment, and a host of other specialty areas.
A nurse employed by the residential provider can have a positive impact on the relationship between physicians and the individuals requiring medical care. In addition to ensuring that the regulations of each state are met, the nurse can be the “eyes and ears” for the physi- cians. The nurse’s knowledge of Prader-Willi syndrome, familiarity with each individual, and excellent assessment skills may save unnec- essary trips to the physician’s offi ce. Although faced with many signifi - cant medical conditions and risks, people with Prader-Willi syndrome also regularly create or embellish medical complaints. A skilled nurse can assist the physician by decreasing time spent on hypochondriacal complaints, thereby assuring that the person with PWS will receive proper attention and care when brought to the physician for a true area of need.
The nurse also plays a critical role in educating others. As earlier indicated, a well-trained staff is imperative for a successful program.
The agency’s nurse should be involved in both the initial and ongoing staff training. This training can include descriptions of the physical manifestations of the syndrome, as well as standards and expectations for dietary needs, weight control, and exercise. The nurse’s role in educating others may extend to neighbors, teachers, and vocational support staff. A nurse who enjoys an overall case management approach of coordinating, assessing, and educating would likely succeed in this role.
In addition to nursing, other ancillary services are important to a residential program supporting people with Prader-Willi syndrome.
An approachable, informed dietitian is essential to create menus, discuss food preferences, and determine what food items are permis- sible for special occasions. Oftentimes, several people living together may have differing caloric requirements. A creative dietitian can assemble menus that are tasty, healthful, and follow the physician’s prescriptions. The successful dietitian will be well versed in all aspects of Prader-Willi syndrome, in part to avoid potential manipulations by the individuals regarding their diet plans. Further, it is benefi cial that staff receive training related to the food aspects of the syndrome directly from the dietitian. This training can include basic dietary guidelines and requirements, discussion of specifi c diets, practice with weighing and measuring portions, and role-playing that addresses the inevitable “what ifs.” Staff armed with knowledge and confi dence are more likely to make competent and reasonable decisions when unusual situations present themselves. While a dietitian may not always be immediately accessible, the training he/she has provided may enable the staff to alleviate the immediate concern until offi cial clarifi cation is obtained.
Lastly, a dietitian may make regular house visits to monitor weights, make necessary dietary modifi cations, and adapt menus. Because food is of utmost importance to the person with Prader-Willi syndrome, a strong relationship with the dietitian may help to provide assurance that the prescribed diets are indeed being followed.
An exercise physiologist also has a major role in a residential program.
At the onset of a new program, the exercise physiologist’s role is to assess each individual’s overall status. Once the assessment is com- pleted, an appropriate exercise schedule can be created and individual- ized. Periodic review of progress and necessary modifi cations in type and amount of exercise can assist individuals to safely achieve weight loss goals. Similar to the nurse and dietitian, the exercise physiologist can play a supportive and teaching role to the direct support staff. This can be achieved by answering staff’s questions about therapeutic exer- cise and supporting staff in their quest to encourage the individuals with their required exercise regimen.
A residential program’s nursing, dietary, and exercise physiology staff play key roles in the success of the individuals receiving services.
The education of community members and support to individuals and staff can be invaluable tools in the multidisciplinary approach toward achieving safe, healthy, and enriched lives for people with Prader-Willi syndrome.
Current Residential Options Across the United States
The implementation and operational practices of residential programs throughout the country are diverse. This is due, in part, to the require- ments of the funding sources and philosophies of the provider agen- cies. While environmental controls remain paramount, the physical structure of the program, whether an apartment, single family home, or dormlike setting, may be less important than the care, consistency, and treatment that takes place inside those walls.
Segregated programs, which provide services only to people with Prader-Willi syndrome, can be managed in several ways. One example is a small (i.e., three-to-fi ve-person) single-family home that blends into a residential neighborhood. Additionally, residential homes exist in which larger numbers of individuals (i.e., up to 16) are supported. In this type of program, general house rules and overall philosophies are the same for all, while medical care and behavior support plans can be individualized. This model promotes and capitalizes on community presence and belonging, yet provides the environmental structure and treatment that the syndrome demands.
Similarly, some individuals with Prader-Willi syndrome are best served in a segregated program on a large, campuslike setting. Individ- ual or shared sleeping rooms are contained in a cottage or dorm-style building on the grounds. Many children and young adults who live in this setting attend school or a vocational training program that is on the campus; others are enrolled in school or work settings in the comm unity. In this type of program, services are also provided that address exercise, nutrition, activities of daily living, and behavioral supports.
Some persons with Prader-Willi syndrome live in homes with others who do not share the syndrome. This may occur as a result of the
family’s or individual’s choice. Unfortunately, however, this residential option may often occur out of necessity or an emergency placement need, and when no segregated programs exist or are readily available.
This arrangement presents particular challenges for those with Prader- Willi syndrome as well as for those with whom they live who have other developmental disabilities. While this situation may indeed be successful for some, signifi cant attention must be paid to food access as well as to the unique privacy and property issues and behavioral manifestations of the syndrome.
Due to tight state and federal funding and budget constraints, it is rare to see a residential program that requires 24-hour staffi ng in which three or fewer individuals live. While this may be philosophically pre- ferred, it is simply not deemed cost-effective by many funding sources.
As a result, many states now encourage families to keep their adults with developmental disabilities in the family home with supportive services provided as needed. This option, even when desired by fami- lies, is rarely successful since most families fi nd that, by the time the individual with Prader-Willi syndrome reaches adulthood, the family is no longer able to adequately meet the demands of 24-hour vigilance and care.
A variety of approaches occur within the walls of a residential program supporting people with Prader-Willi syndrome. All existing programs that were researched for this chapter provide dietary man- agement, food restrictions, and education, as well as exercise, access to ancillary services, and behavior support. One unique approach to dietary management involves a base calorie amount to be consumed each day, with the opportunity for earning additional caloric incentives for compliance in areas like exercise, attending work, and exhibiting appropriate behavior. Ultimately, an argument can be made that there is no absolutely “right” or “wrong” approach, but the key to a success- ful residential placement may lie in the matching of the individual’s needs to the program strengths.
Conclusion
Many people with Prader-Willi syndrome will face signifi cant chal- lenges and diffi cult decisions throughout their entire lives. Deciding on the appropriateness and type of residential supports is one of the most important considerations facing any person with the syndrome and their family. Guidance from family and signifi cant others, national research of the options available, and seeking answers to probing ques- tions will greatly assist with making the best possible decision. Suc- cessful residential programs, equipped to manage and support the unique needs of individuals with Prader-Willi syndrome, have contin- ued to be developed over the past two decades. It will be through the relentless efforts of both parents and professionals that all people who desire a residential program will have a safe home in which to learn and achieve a quality of life.
References
1. Greenswag L, Singer S, Condon N, et al. Residential options for individuals with Prader-Willi syndrome. In: Greenswag L, Alexander R, eds. Manage- ment of Prader-Willi Syndrome. 2nd ed. New York, NY: Springer-Verlag; 1995:
214–247.
2. Norwood, C. What Questions Should I Ask? Cleveland, OH: Center for Mental Retardation; 2002.
3. Thompson D, Greenswag L, Eleazer R. Residential programs for individ- uals with Prader-Willi syndrome. In: Greenswag L, Alexander R, eds.
Management of Prader-Willi Syndrome. New York, NY: Springer-Verlag; 1988:
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