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A National Approach to Crisis Intervention and Advocacy
David A. Wyatt
Prader-Willi syndrome (PWS) often places great stress on the family and all persons who come into contact with the person with PWS. The Prader-Willi Syndrome Association (USA) national offi ce provides crisis intervention in the areas in which our member families often need advocacy and/or educational information to obtain the local support and assistance they need. Requests for this service come through e- mails and telephone calls to a toll-free number. The Crisis Intervention Counselor and/or the Executive Director respond personally to these requests and with appropriate educational materials about the syn- drome. A “crisis letter” that addresses the issue(s) at hand often accom- panies a “crisis packet” containing the appropriate information. The Association maintains an account of the types of crisis calls received and reported the following summary of calls for one recent year:
36%—Legal and advocacy: court system, schools, insurers, Supplemen- tal Security Income, etc.
17%—Residential placement cases
16%—Medical cases (other than morbid obesity)
11%—Sexuality issues in residential placement or school
10%—Behavior management in the home, school, or residential placement
10%—Morbid obesity
Following is a description of the various crisis issues that are presented by families with a child, adolescent, or adult with the syndrome.
Medical Crisis Issues
While parents initiate a majority of our medical crisis calls, the national PWSA offi ce also receives calls from physicians, nursing personnel, emergency room personnel, and intensive care units when they are in the process of treating a person with PWS because of medical issues related to the syndrome. These are usually critical emergencies. The
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situation is exacerbated when the medical personnel are not familiar with PWS.
PWSA immediately sends acute medical information written for these situations, such as a general medical alert document or articles on respiratory concerns and anesthesia considerations. The informa- tion is faxed or e-mailed directly to the nursing station and/or the attending physician. The national offi ce may notify one or more physi- cians on the Scientifi c and Clinical Advisory Boards about the situation, then inform the medical staff on the scene that they can obtain a medical consultation from the physician who was contacted.
PWSA can often give input and get feedback from several expert sources, thanks to e-mail. The following is an e-mail sent to the medical boards. This case demonstrates how an individual with PWS is often affected in quite different ways, and with more complexity, than other patients because of the syndrome.
11-year-old girl, 4 foot 5 inches and 109 pounds. She had surgery for scoliosis January 29th. The surgery was uneventful, but she has had dramatic cognitive changes since then. She repeats things 60–100 times as if she does not remember just saying it, can barely hold a fork, drops things, thinks she sees people in the room that are not there, and is wetting herself. She is also sleeping a dra- matic amount of time. She was on antibiotics, then off and is on again because they discovered Sunday she has pneumonia. No change while off and the only pain med she is taking is Tylenol. Her long-term memory appears to be still OK. She had a CT of the head, and an EEG. Nothing showed up. She cannot get an MRI right now due to all of the new hardware in her back. The only thing (besides the pneumonia) that shows up is a slightly elevated ammonia level. She went home from the hospital 5–6 days post surgery (they were hoping that the cognitive issues were the result of the anesthesia and would be temporary) and ending up back on Sunday due to the pneumonia, which seems to be responding to the antibiotics, but her cognitive functioning is no better.
Several members of the PWSA medical boards responded to this e- mail with advice on further testing, and two agreed to consult with this girl’s physician. The consultations had two results: First, the medical treatment was modifi ed in addressing the pneumonia according to the consulting physician’s clinical experience with antibiotic medication and oxygen impairment in patients with PWS. Secondly, specifi c psy- chotropic medications and dosages were given, again related to the clinical experience with individuals with PWS, and medication changes began to clear up her cognitive diffi culties.
Morbid obesity is always a threat for the person with PWS. When the obesity is leading to life-threatening medical problems, crisis intervention is needed to reverse the gaining of weight. The initial response to a call from the parents, who are aware and frightened that the eating is out of control, is to support them in instituting means of controlling food access. If this is already being done, the Crisis Counselor can help the parents solve the mystery of where the individual with PWS is obtaining food and support them in getting this source shut down. In some cases, it is more appropriate to assist the parents in getting the person with the syndrome into an
inpatient rehabilitation program, which is often successful in revers- ing the most life-threatening weight gain and/or managing the most diffi cult behaviors (see Chapter 17). Often, the weight and be havior problems occur concurrently. The following case is a typical example:
Call from mother: Son, 16 yrs., 320 lbs. Just offi cially diagnosed with PWS.
Hides food everywhere, e.g., under his pillow, bed, etc. Becoming aggressive towards her. Took out a knife and tried to hurt himself. Embarrassed about his small penis and his obesity. Others stare and point at him. He has no social life.
He cried and said, “Momma, why am I like this? I don’t want to be this way.”
Has diabetes and sleep apnea and is beginning to demonstrate right-sided heart failure.
Education Issues
Often, major problems arise when the child with PWS begins his/her educational program, even as early as preschool. One issue is obtain- ing an Individualized Education Plan (IEP) that is appropriate for the person with PWS. An IEP meeting can seem overwhelming and intimidating to the parents without experience or thorough prepara- tion. Parents are encouraged to go to the Internet and locate NICHCY (www.nichcy.org), a federally-funded information clearinghouse on disabilities. NICHCY has abundant information on the Individuals with Disabilities Education Act (IDEA), the U.S. federal law governing public schools in the area of programming for persons with disabilities.
They also have information about the IEP process. The national PWSA offi ce can provide parents with educational materials to help prepare them for the IEP meeting and, perhaps, put them in touch with other parents of children with PWS who have had successful IEP results.
One area of the IEP that can cause parents consternation is that of requesting a one-on-one aide to assist the child with PWS. When the child’s eruptive behavior is interrupting the educational process and the school’s response is suspension or expulsion, an experienced aide can often defuse the environment. An aide is also needed when the child with PWS is a serious food forager. This request is often met with resistance because of the added expense for the school and school dis- trict. The national PWSA offi ce has been successful in assisting parents to obtain this aide through the crisis packet and a letter from the Execu- tive Director that points out that the expense of not providing an aide can far exceed the expenditure of having one.
Another problem area of the IEP is in the development of a behav- ioral plan that is appropriate for a person with PWS. Children with PWS usually have diffi culty in meeting the requirements of a classroom because of the effects of their syndrome. They may disrupt a class by having a “meltdown” because of their lack of emotional control. Often, the response to this behavior is based on a false assumption that the child with PWS has a control mechanism or can learn to gain control by receiving a consequence such as a threatened suspension. Another typical behavior exhibited may be called “stubbornness.” This may
play out as being oppositional, sitting down, refusing to move, or throwing something. This often occurs because people with PWS have diffi culty identifying priorities. When they are confronted with stimuli from several different sources, it is diffi cult for them to prioritize or sort out the major from minor issues. This produces anxiety and confu- sion that leads to the behavior.
To assist the administrative staff and the teacher to understand the syndrome and offer suggestions for addressing the various behaviors that may be occurring, the national offi ce provides written material, audiotapes, and videos that give insight and facts about PWS. When this approach does not help, the Crisis Counselor can contact an ex- perienced advocate in the area to go with the parents to the next IEP meeting. If this is not possible, or does not succeed in establishing an appropriate IEP for behavior, PWSA (USA) can provide, through a grant, an educational consultant who is experienced with persons who have PWS. This person usually talks with the school representatives and the parents by telephone to obtain a clear picture of the major issues. This is typically followed by a “speaker phone” conference in which the parents, school personnel, and the educational consultant work toward a suitable behavioral and food control plan.
Legal Issues at School
In the U.S., most children with disabilities are now integrated into regular schools. At the same time, due to the rash of school shootings by students, there is now a “zero tolerance” discipline policy in many schools, which also includes disruptive behavior in general. This is creating many problems for our children with PWS. Placement in a mainstream class brings a faster pace of learning and higher expecta- tions, which heighten the pressure and performance anxiety. It is also much more diffi cult to write a behavior management program into the IEP that is practical for school staff to implement. The response on the part of the school is now weighted toward longer suspensions and, if the behavior continues, may lead to expulsion. Reports of arrest for stealing food, hitting teachers, or destroying property are not uncom- mon. In one case, for example, a teacher fi led sexual battery charges against a 9-year-old child with PWS because he grabbed her breast. In another case, a 10-year-old with PWS was taken to jail in handcuffs for kicking a teacher during a tantrum. And, of course, there are the many, many cases where students are suspended from school for taking food from the lunchroom, from other students’ lunch boxes, or from the teacher’s desk.
If an IEP does not have an appropriate behavioral plan for the child with PWS, it increases the likelihood of legal problems. The scenario may be that when the student with PWS has a “meltdown” the school calls the local police department. Then the child kicks the police offi cer!
The person with PWS might be placed in handcuffs and taken to jail.
We have known children as young as 7 years old being subjected to this procedure. Criminal charges may be consequently fi led because of the destruction of property or physical assault. The PWSA Crisis Coun-
selor will respond to this incident, when contacted by the parents or the school, with a telephone call to the law enforcement representative or a crisis letter addressed to the defense attorney, or to the court if there is a scheduled hearing of the case, and a crisis information packet.
If the incident does not go to court, the option to return to writing an appropriate IEP is usually productive.
There have been a few occasions when the school staff person who has been struck by the person with PWS fi les an assault and battery charge. There have also been instances in which a sexual assault charge is fi led because the person with PWS has touched an adult or another student in an inappropriate place. None of these cases was pursued after PWSA (USA) responded with calls, educational materials, and a crisis letter.
In PWSA’s experience, these cases can be resolved out of court, and both the parents and the school can work for a more suitable response. Everyone must be made aware that the individual with PWS may be driven by the insatiable appetite and may not have the usual mechanisms to exercise control over their eating and emotions. It is always emphasized that individuals with PWS usually do not have the ability to stop anti-social behavior by themselves, but with assistance, they can be helped to learn socially acceptable means of dealing with the classroom stress. For further discussion of school dis- cipline issues, from the perspective of an educational consultant, see Chapter 20.
Legal Issues in the Public Arena
On rare occasions, crisis support may be needed because the young adult or adult with PWS may get into a major legal situation related to sexual behavior. Court cases are complicated because the adult with PWS may appear to know the difference between right and wrong sexual behavior. The prosecution usually believes that the individual is competent to stand trial and is often not willing to consider leniency due to mental and social capacity. This results in a long, drawn-out legal process and months of agonizing worry for the adult with PWS and his/her family. To date, all of these cases have eventually been dismissed and the records expunged. See Chapter 21 for further discus- sion of sexuality issues.
Young adults with PWS can also be charged with theft. This occurs when they have tried to walk out of a store with merchandise that has not been paid for or have eaten food merchandise in the store. In these instances, the national offi ce has been able to intercede with crisis intervention, resulting in the charges being dropped and an equitable resolution obtained. A recent example was a 21-year-old male with PWS who entered a clothing store, picked up two shirts at the back of the store, and brought them to the cashier. He explained they were gifts and he wanted to return them and get a refund. His plan was that he could then use the money for food. The clerk refused since there was no receipt and called the police. A charge of shoplifting was fi led. The
case was dropped after the prosecuting attorney received a crisis letter and information packet from PWSA (USA).
Supported Living Issues
The national crisis intervention program can also be helpful at a time when the family of an individual with PWS is desperate to fi nd either residential services or a rehabilitation facility. The national offi ce keeps contact with group homes and other facilities that are designed to provide a safe environment for persons with PWS. National offi ce staff have written letters about the individual and the syndrome that have assisted families to obtain funding necessary for an appropriate place- ment, where the individual with PWS can live a happy and successful life within the parameters of the syndrome.
What has become a major dilemma in residential placement is: What is the least restrictive environment for an individual with PWS?
Consider the following:
• Under age 18—parents can be charged with neglect for allowing a child with PWS to become so obese that it is a life-threatening situation.
• Over age 18—a judge can rule that a person with PWS can have the right to unrestricted access to food.
• Mixed supportive living homes in the United States often cannot lock up food due to the rights of other clients in the home who do not have PWS.
PWSA’s position is that the least restrictive environment for a person with Prader-Willi syndrome is a home with restricted food access. The rationale is clear:
• It frees their mind from the constant striving to secure food.
• If everyone in the home has the same restrictions, they now feel more
“normal” and less singled out, which reduces frustration. Less frus- tration equals fewer behavior problems.
• An extremely obese body with related health problems is very restrictive!
• Would you hand a person who is suicidal a gun? Food will kill our children and adults with PWS just as assuredly as a gun.
• The children’s right to life is more essential than their right to free access to food.
The following case is an example of how complex some of the prob- lems can be:
A young woman called in urgent crisis. Her sister with PWS weighs 346 lbs., is 37 years old and 5’2” tall. She was in a respite program but now is in the hospital. The case manager is skeptical of the PWS diagnosis, although she tested positive for PWS. The agency stated, “She has the right of unrestricted access to food.” Her sister stated, “Her ‘rights’ are killing her.” In a series of calls over the next 6 months, the sister reported that the case manager manipu- lates her sister and is making up charges of abuse against their mother. The
sister with PWS has been in and out of the hospital. In May, she weighed 440 lbs., having gained 140 lbs. in 2 months in a placement. She now has a tra- cheotomy. Sister stated, “It’s out of my hands and I have to watch it happen.
It is really horrible.” In July, her sister was in the hospital on a ventilator. Crisis packets, referrals, and counseling were provided to the family, hospital person- nel, and physicians.
Advocacy for Services
Children with the syndrome are eligible for various services offered at the local level by national and state governments or their designees.
When the child is diagnosed with PWS, it is important to obtain the assistance of a speech therapist, occupational therapist, and physical therapist to intervene in the area of low muscle tone, or hypotonia. A majority of the states offer these services through a local ARC organiza- tion or through the state Division of Disabilities/Mental Retardation.
Usually it is not a problem to establish eligibility for these services, but in some cases services are denied. However, if the service is denied, it is always possible to appeal this decision. When making an appeal, the resources of the national PWSA offi ce can often be very helpful in resolving the issue and gaining the services. A call from the Crisis Counselor to the agency social worker or administrator can provide that person with the information they need to qualify the applicant. In most instances, a letter and a crisis packet are also sent to the agency, often through the family making the application, and this provides specifi c details about PWS and the reasons the child is eligible for the services requested.
There have been instances in which the services have been provided in a timely manner, yet when an evaluation of the results occurs, usually at the age of 3 or 4 years, the agency proposes to stop the services because the child is doing so well. Families have been successful in achieving the continuance of these services after the agency receives appropriate educational materials and their knowledge of the syn- drome is broadened.
Supplemental Security Income (SSI) is available to persons with qualifying disabilities who have income and resources that do not exceed certain limits set by the U.S. Social Security Administration.
This usually means that the person with PWS who qualifi es for SSI payments is also eligible to receive Medicaid or a similar state medical insurance coverage. Sometimes the national PWSA offi ce assists in an SSI appeal because this is another instance where benefi ts have been denied because the person with PWS is not offi cially “mentally retarded”; that is, their IQ is 70 or above. To win an appeal, one must demonstrate that PWS is lifelong and very debilitating. A family with a young person with PWS should obtain information about these services even if they feel that they are not fi nancially eligible. When the child reaches the age of 18, the income used for evaluation of eli- gibility is based on the individual’s income only and not the family’s income. See Appendix D for further guidance on SSI application and appeal.
Other organizations can sometimes provide assistance to families of children with PWS, such as special equipment that may be needed. The Shriners’ Hospitals have been helpful in providing medical care for orthopedic issues, and the March of Dimes, Elks, Kiwanis, and other service organizations have assisted with equipment and other specifi c needs.
Conclusion
The PWSA (USA) Crisis Intervention Program began in January 2001, thanks to a 5-year grant from the Alterman Family Foundation. It has become an invaluable service to hundreds of PWS families and profes- sionals, as refl ected in the following quotes:
• “I called the PWSA offi ce today. They are sending me information and a video. The counselor was very helpful with issues about guardianship and educating the school staff. I feel a little bit better;
actually it’s unbelievable, and I am overwhelmed. Looks like there may be a light at the end of the tunnel after all.”
• “First of all, I can’t thank you enough. Your work in helping me deal with this matter—or shall I say, nightmare—has been so supportive and informative, even at times when I just wanted to give up.”
• “Thank you so very much for your recent letters and information you sent on behalf of our daughter and our SSI ‘battle.’ They were very nice and hopefully will make our problems with the SS Admin- istration go away.”
• “We would like to thank all of you for the time and assistance you offered us to achieve our goal of getting our son appropriately placed. We will always remember you in our thoughts and prayers.”
• “Thank you so much for your letter. It helped more than you could know.”
• “Thank you so much for the excellent material you sent me recently.
It was a great help to us and to the judge who was hearing our case.”
