Improving Therapeutic Management of Huntington’s Disease in...

View this activity online at:medscape.org/spotlight/huntingtons

Improving Therapeutic Management ofHuntington’s Disease in Long-Term Care CME/CEKaren E. Anderson, MD

Lawrence E. Banta, MD

Nancy Beecham, BS, RNC, CDONA, FACDONA/LTC

Martha A. Nance, MD

Supported by an independent educational grant from

Pg.2

Improving Therapeutic Management of Huntington’s Disease in Long-Term Care CME/CE

This article is a CME/CE-certified activity.To earn credit for this activity visit:

medscape.org/spotlight/huntingtons

CME/CE Released: 5/28/2013 Valid for credit through 5/28/2014

Target AudienceThis activity is intended for family medicine/internal medicine physicians, neurologists, psychiatrists, nurses, nurse practitioners, physician assistants, and those healthcare professionals who work in long-term care settings.

GoalThe overall goal of this activity is to improve the recognition and management of Huntington’s disease in the long-term care setting by providing healthcare professionals with the most recent developments and tools available in their practice setting.

Learning ObjectivesUpon completion of this activity, participants will be able to:

1) Develop strategies to recognize and overcome barriers to the optimal care of residents with Huntington’s disease living in long-term care facilities

2) Identify at least 3 best practice approaches and resources aimed at improving neuropsychiatric symptoms in patients with Huntington’s disease

3) Design a team-based approach to patient management

4) Develop an appropriate treatment plan for chorea associated with Huntington’s disease

Credits Available Physicians - maximum of 0.50 AMA PRA Category 1 Credit(s)™

Nurses - 1.00 ANCC Contact Hour(s) (0 contact hours are in the area of pharmacology)


Pg.3

Accreditation StatementsFor Physicians This activity has been planned and implemented in accordance with the Essential Areas and policies of the Accreditation Council for Continuing Medical Education through the joint sponsorship of The American Medical Directors Association (AMDA) and National Association of Directors of Nursing Administration in Long Term Care (NADONA/LTC). The American Medical Directors Association is accredited by the ACCME to provide continuing medical education for physicians.

The American Medical Directors Association designates this enduring material for a maximum of 0.50 AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

For Nurses NADONA/LTC is an approved provider of continuing nursing education by Georgia Nurses Association, an accredited approver by the American Nurses Credentialing Center’s Commission on Accreditation.

Activity #: 1088 Awarded 1 contact hour(s) of continuing nursing education for RNs and APNs; 0 contact hours are in the area of pharmacology.

Instructions for Participation and CreditThere are no fees for participating in or receiving credit for this online educational activity. For information on applicability and acceptance of continuing education credit for this activity, please consult your professional licensing board.

This activity is designed to be completed within the time designated on the title page; physicians should claim only those credits that reflect the time actually spent in the activity. To successfully earn credit, participants must complete the activity online during the valid credit period that is noted on the title page. To receive AMA PRA Category 1 Credit™, you must receive a minimum score of 70% on the posttest.

Follow these steps to earn CME/CE credit*:

1. Read the target audience, learning objectives, and author disclosures.

2. Study the educational content online or printed out.

3. Online, choose the best answer to each test question. To receive a certificate, you must receive a passing score as designated at the top of the test. Medscape Education encourages you to complete the Activity Evaluation to provide feedback for future programming.

You may now view or print the certificate from your CME/CE Tracker. You may print the certificate but you cannot alter it. Credits will be tallied in your CME/CE Tracker and archived for 6 years; at any point within this time period you can print out the tally as well as the certificates from the CME/CE Tracker.

*The credit that you receive is based on your user profile.

Pg.4


Hardware/Software RequirementsTo access Medscape Education users will need:

• AcomputerwithanInternetconnection.

• InternetExplorer7.xorhigher,Firefox4.xorhigher,Safari2.xorhigher,oranyotherW3Cstandardscompliantbrowser.

• AdobeFlashPlayerand/oranHTML5capablebrowsermayberequiredforvideooraudioplayback.

• OccasionallyotheradditionalsoftwaremayberequiredsuchasPowerPointorAdobeAcrobatReader.

Supported by an independent educational grant from Lundbeck, Inc.

Faculty and DisclosuresAuthorsKaren E. Anderson, MDAssociate Professor of Psychiatry and Neurology, Georgetown University; Director, Huntington Disease Care, Education, and ResearchCenter,MedStarGeorgetownUniversityHospital,Washington,DC

Disclosure: Karen E. Anderson, MD, has disclosed the following relevant financial relationships:

Served as an advisor or consultant for: Lundbeck, Inc.Served as a speaker or a member of a speakers bureau for: Lundbeck, Inc.

Dr Anderson does intend to discuss off-label uses of drugs, mechanical devices, biologics, or diagnostics approved by the FDA for use in the United States.

Dr Anderson does not intend to discuss investigational drugs, mechanical devices, biologics, or diagnostics not approved by the FDA for use in the United States

Lawrence E. Banta, MDMedicalDirector,WestValleyMedicalCenterMentalHealthUnitandKindredofCaldwell,Caldwell,Idaho

Disclosure: Lawrence E. Banta, MD, has disclosed the following relevant financial relationships:

Served as an advisor or consultant for: Lundbeck, Inc.Served as a speaker or a member of a speakers bureau for: Lundbeck, Inc.; Ortho-McNeil-Janssen Pharmaceuticals, Inc.

Dr Banta does intend to discuss off-label uses of drugs, mechanical devices, biologics, or diagnostics approved by the FDA for use in the United States.

Dr Banta does not intend to discuss investigational drugs, mechanical devices, biologics, or diagnostics not approved by the FDA for use in the United States.


Pg.5

Nancy Beecham, BS, RNC, CDONA, FACDONA/LTCCertified Gerontological Nurse Consultant; President, California Chapter of the National Association of Directors of Nursing Administration (NADONA)

Disclosure: Nancy Beecham, BS, RNC, CDONA, FACDONA/LTC, has disclosed no relevant financial relationships.

Ms Beecham does intend to discuss off-label uses of drugs, mechanical devices, biologics, or diagnostics approved by the FDA for use in the United States.

Ms Beecham does not intend to discuss investigational drugs, mechanical devices, biologics, or diagnostics not approved by the FDA for use in the United States.

Martha A. Nance, MDClinical Professor of Neurology, University of Minnesota; Medical Director, Struthers Parkinson’s Center; Director, Huntington’s Disease Society of America Center of Excellence at Hennepin County Medical Center, Minneapolis, Minnesota

Disclosure: Martha A. Nance, MD, has disclosed the following relevant financial relationships:

Served as an advisor or consultant for: Lundbeck, Inc.Received grants for clinical research from: IMPAX Laboratories, Inc.; Juventas Therapeutics; Medivation, Inc.; Neuraltus Pharmaceuticals, Inc.; NeuroSearch A/S; Pfizer Inc; Santhera Pharmaceuticals; Schering-Plough Corporation; SCHWARTZPHARMA,Inc.

Dr Nance does intend to discuss off-label uses of drugs, mechanical devices, biologics, or diagnostics approved by the FDA for use in the United States.

Dr Nance does not intend to discuss investigational drugs, mechanical devices, biologics, or diagnostics not approved by the FDA for use in the United States.

EditorRon Schaumburg, MAScientific Director, Medscape, LLC

Disclosure: Ron Schaumburg, MA, has disclosed no relevant financial relationships.

CME Reviewer Nafeez Zawahir, MDCME Clinical Director, Medscape, LLC

Disclosure:NafeezZawahir,MD,hasdisclosednorelevantfinancialrelationships.

Pg.6


Improving Therapeutic Management of Huntington’s Disease in Long-Term Care CME/CE Released: 5/28/2013; Valid for credit through 5/28/2014

Martha Nance, MD: Hello, I am Martha Nance, clinical professor of neurology at the University of Minnesota, medical director of the Struthers Parkinson’s Center, and director of the Huntington’s Disease Center of Excellence at Hennepin County Medical Center in Minneapolis. I would like to welcome you to this program titled “Improving Therapeutic Management of Huntington’s Disease in Long-Term Care.”

Joining me today are Karen Anderson, associate professor of psychiatry and neurology, and a neuropsychiatrist at Georgetown UniversityinWashington,DC;LarryBanta,aneuropsychiatristandmedicaldirectoroftheWestValleyMedicalCenterMentalHealth Unit and Kindred of Caldwell, a specialized skilled nursing facility, both in Caldwell, Idaho; and Nancy Beecham, certified gerontologic nurse consultant and president of the California chapter of the National Association of Directors of Nursing Administration(NADONA).Shewasalsonamedthe2012NationalNurseAdministratoroftheYear.Welcome.

Nancy Beecham, BS, RNC, CDONA, FACDONA/LTC: Thank you.

Lawrence Banta, MD: Thanks.


Pg.7

Dr Nance: I would like to note that this program will include discussion of off-label use of medications that have been approved by the United States Food and Drug Administration.

The goals of this program are to develop strategies to recognize and overcome barriers to the optimal care of people with Huntington’s disease who reside in long-term care facilities; to create an appropriate treatment plan for chorea associated with Huntington’s disease; to identify at least 3 best practice approaches and resources aimed at improving neuropsychiatric symptoms in patients with Huntington’s disease; and to develop a team-based approach to patient management.

Pg.8


Intoday’sdiscussion,we’llcoverarangeoftopics.WhatisHuntington’sdisease?HowdopeoplewithHuntington’sdiseasedifferfromotherresidentsinthelong-termcaresetting?Whatkindofcareplanningisneededtoaddresstheresident’smedical,physical,andsocialneeds?Andhowshouldwedevelopateamapproachforovercomingbarriersandachievingoptimalcare?

The nature of Huntington’s disease and its management can pose many barriers, some of which we’ll cover in this discussion. But tocomprehendthosebarriers,let’sfirstestablishabaselineunderstanding.WhatisHuntington’sdisease?

Karen Anderson, MD: It is a genetic condition, which means that it is inherited, and it is progressive -- the symptoms tend to get worse over time. The main symptom that we associate with Huntington’s is a movement disorder called chorea, which may be very different from the types of neurologic symptoms that the staff are used to seeing in the long-term care setting. There are other symptoms of Huntington’s disease that need to be managed, including aggression, mental inflexibility, irritability, and depression. The condition inevitably leads to death from various causes, including dysphagia or infections.

Dr Nance: Larry, you’re the medical director of a facility that houses as many as 10 residents with Huntington’s disease at any giventime.HowdopeoplewithHuntington’sdiseasedifferfromresidentswhohaveotherconditions?


Pg.9

Dr Banta: First, let me note that many facilities are nervous about taking residents with Huntington’s disease because these residents are a little different from the typical long-term care population. They are often quite a bit younger. Onset of the disease can occur in the very young, even in the pediatric age group, but typically these patients are between the ages of 30 and 50 years.[1] Many of them still have children at home who, because this is an inherited disease, may become affected later. And many of these residents may also have parents who are affected or who have died from the disease. Several family members may even be in the same facility at the same time.

The male to female ratio of Huntington’s disease is approximately 50:50, so the numbers are almost equal.[1] The symptoms of Huntington’s disease are different from those seen in the usual patient population in a nursing home, most of whom have conditions such as Alzheimer or Parkinson disease.

One of the main characteristics of Huntington’s disease patients is that they lose executive function -- the ability to make proper decisions or control their behaviors. As a result, they become quite impulsive, irritable, and inflexible. However, they still retain basic memory functions. They remember who you are. They remember, and are much more aware of, the day-to-day things that are going on.

The other thing that distinguishes them from others in the facility is the chorea, which is a jerking, writhing movement disorder. Chorea affects about 90% of the patients at one time or another in the course of the disease.[2]

Pg.10


The behavioral issues I mentioned earlier, including irritability and aggression, can lead to threatening behaviors. Sometimes these lead to altercations between residents, which can bring the facilities under scrutiny by the government. It can be challenging to have these residents in the same facilities as those with other conditions, such as brain trauma and Alzheimer disease, because of the different types of behavior that they exhibit. They may need a higher level of care. They may need to have more staffing. Often there are family issues that have to be dealt with, such as abandonment, or the guilt that family members may feel about leaving someone in a facility, or the fear that they may end up that way as well.

Another difference is that the duration of stay in the facility is often considerably longer than that of our other patients. Residents with Huntington’s disease typically stay in facilities from 7 to 10 years from the point at which they can no longer take care of themselves at home. It is a challenge because, as I mentioned, many facilities refuse to take these individuals because of the nature of Huntington’s disease.

Dr Nance: Nancy, you have worked with clients who have Huntington’s disease since the day you graduated from nursing school many years ago, and you have also worked as a director of nursing facilities. Tell us a little bit about your experience as a nurse dealing with residents with Huntington’s disease, and about some of the challenges in providing care for these individuals.


Pg.11

Ms Beecham: Huntington’sisauniquedisease.Withmostdiagnoses,youcancreateacareplanusingatemplatebasedonthespecific predictable symptoms. But each of the residents with Huntington’s disease is unique. You cannot necessarily predict that certain things will happen in a certain order. Because every case is different, it is essential that you take an individualized approach to each of the residents.

The other thing we have found is that, because we develop long-term relationships with these residents, we see their behavior overtime,andwelearnwhatmighttriggercertainbehaviors;asaresultwelearntobetterunderstandeachresident.Webecomean extension of the biological family because these folks are with us so long. It is important that the certified nursing assistants (CNAs), who are the primary caregivers, are very involved with these patients, and we have consistent assignments. They are consistently with the residents, they know them. They know what is good for them, and what triggers to avoid.

AnotherthingtheCNAslearnisthatwhentheresidentssayno,theymeanno.Withotherconditions,iftheresidentsaysno,youcancomebacktothatpersonandtryagain.WithaHuntington’sdiseaseresidentyoureallycannotdothat.Giventheircognitiveimpairment and their fixations, you have to learn exactly how to work with that person on an individual basis.

Itisalsoimportanttorememberthatwhatweofferispalliativecare.Wehavetorealizethatthisisaterminaldisease.Wearenotgoing to cure them, but we can help them by understanding them and knowing what works for them.

Pg.12


Huntington’s disease residents also have unique nutritional needs. Because of the chorea, they are constantly moving and as a result they burn a lot of calories. The physical act of eating is a challenge for them as well. Providing food of the proper texture is important. Also, these individuals have dysphagia -- difficulty swallowing -- so choking and aspiration are among the leading causes of death in these individuals. Such problems can arise in residents of any age, including people in their 20s, 30s, or 50s.

It is necessary to put together a team that helps residents with their nutritional status. The team includes everyone from the dietitiantotheCNAs.Whenyouhelparesidenteat,itisnotpretty.Thefoodgoeseverywhere,overyou,overthem.Gettingthenutrition into them is a challenge.

Thisisespeciallythecasewiththeyoungerresidents;weprovide“fast”(easytoeat)foodbecausetheyarefastpeople.Wehavefinger foods available for them around the clock. And they love junk food, so that has to be available for them, too.

AnothervitalaspectofmanagingresidentswithHuntington’sdiseaseisprovidingasafeenvironment.Wehavetomakesurethatwe protect them. For example, we shorten the cords attached to lights or to the call buttons and we take down the curtains to reducetheriskthattheymightbecomeentangled.Wepadcornersandbeds.Weusespecialchairs.Asasidenote,wealsohavetobe concerned about suicidal behavior, and we may need to make adjustments in the environment to reduce that risk.


Pg.13

Given the many aspects of dealing with Huntington’s residents in the long-term care setting, it is vital to assemble a care team. Included in that team are the many therapists -- speech, occupational and physical, art, and music -- well as physicians, CNAs, and pharmacists. A team approach is critical.

Dr Nance: Wehavetalkedalittlebitaboutgeneralstrategiesandtheneedforateam.Letusgobackforaminutetothemedicaltreatment.Karen,whatarethemainmedicalstrategiesthatareusedtomanagesymptomsinresidentswithHuntington’sdisease?

Dr Anderson: Wedonotreallyhaveevidence-basedcareyet.Thatresearchneedstobedone.Insteadwerelyonguidancefromexperts, from those of us who have seen a lot of patients over the years and from case studies that have been published.

Pg.14


As Larry mentioned, behavioral issues are often the main barrier that prevents people with Huntington’s disease from being accepted into long-term care. Take depression, for example. Approximately half of people with Huntington’s will have depression at some point, and their depression may be accompanied by symptoms such as anxiety, obsessive-compulsive behavior, and perseveration. They can get very “stuck” on things, as Nancy noted. They may ask the same question repeatedly. They may have a particular action or particular thing they get very focused on. Of course, that can be difficult for staff to deal with when they are trying to go about their business and help the resident. Fortunately, effective treatment is available. Appropriate use of antidepressants can relieve not only the depression but also some of those other symptoms I just described. The selective serotonin reuptake inhibitors, for example, are often used to treat irritability and aggression.[3] Antipsychotic medications are used for aggression, irritability, and also for agitation, and those can be quite successful in these patients.[3] Mood stabilizers such as valproic acid are also used frequently for aggression.[3] Some of these uses are off-label, but they are fairly common in everyday practice.

Dr Nance: Is there a specific antidepressant or a specific antipsychotic that we should be using in people with Huntington’s disease?

Dr Anderson: There really is not a specific antidepressant. The choice should be based on the potential side-effect profile of a particular agent used for a particular patient. For example, mirtazapine tends to cause weight gain and is somewhat sedating, so it is one that we often chose for patients who have a real problem keeping weight on and who may be having issues with sleep.[4] Similarly, with those who are neuroleptic, you may want to consider starting by prescribing a more-sedating drug for someone who is more aggressive, and then consider switching to one that is not quite so sedating.


Pg.15

Dr Nance: Howabouttreatmentsforchorea?

Dr Anderson: There is one medication, tetrabenazine, a dopamine-depleting agent, that is approved in the United States for treatment of chorea.[5] It is effective in most people who have chorea.[6] It reduces their movements by about 25%, which can make a significant difference, especially if chorea is interfering with activities of daily living or is keeping the staff from being able to assist a resident during the day.

Tetrabenazine comes with a boxed warning for depression.[5] If somebody is using tetrabenazine, there is a chance that he or she can become very depressed. There have been cases of people becoming suicidal and acting on those impulses while on tetrabenazine. If that happens, it is very important to reduce the dose of tetrabenazine or, in some cases, discontinue the medication. Antidepressants can also be used concomitantly with tetrabenazine to treat depression and suicidal thoughts.

There is no target dosing with tetrabenazine, dosage is individualized. The medication should be slowly titrated upward until benefit (reduced chorea) is seen. The dosage should be reduced if the side effects that can occur with tetrabenazine, such as depression, sedation, increased falls, suicidality, or dysphagia, are observed.

It is important to emphasize that titration should be slow, especially in people who are frail or who have other medical issues, and that it can take several weeks to reach a therapeutic dose. Prescribers should start with low dosing and increase very slowly to insure that the dosing is safe, but also to achieve a dose that is effective for suppressing chorea in each individual.

Another important thing to remember is that suppressing chorea does not affect the underlying condition. Huntington’s disease will worsen and will progress regardless of whether or not the chorea is treated.

Pg.16


Dr Nance: Are there other medications that might be used to treat the chorea or manage the other symptoms of Huntington’s disease?

Dr Anderson: Tetrabenazine is certainly not appropriate for every patient. Another dopamine-depleting agent, reserpine, is sometimes used.[7] Alternatively, dopamine-receptor agonizing neuroleptics, such as the antipsychotics haloperidol or olanzapine, can also be used off-label for chorea, and in some cases those may even be preferable for specific patients.[6] One should bear in mind that neuroleptics may worsen other features of the disease, such as bradykinesia and rigidity. Antiseizure drugs have also been used for irritability and aggression.[7]

In some cases, Huntington’s disease residents have severe difficulty with sleep. It is important to evaluate this carefully to see if the chorea is keeping them awake because they are moving constantly and are unable to fall asleep. It may be that they have primary insomnia, or insomnia due to depression, or insomnia simply because they are hungry, or because some other environmental issue is preventing them from sleeping.

In some cases, I might use a small dose of a benzodiazepine, such as clonazepam, to suppress the chorea just long enough so that the resident can fall asleep but not so as to have an effect during the daytime, when use of the drug might increase their risk of falling.[4]

Dr Nance: You are emphasizing the importance of individualizing the treatment based on the pattern of symptoms the resident exhibits.

Dr Anderson: Absolutely. It is very important to ensure that you treat the individual’s particular symptoms, and also that you think about the side effects of any of the psychoactive medications you may prescribe.


Pg.17

Dr Nance: Larry, what concerns do you have based on your experience with the medical management of residents with Huntington’sdisease?

Dr Banta: IthinkKarenandIlookatitverysimilarly.WhenIassessapatient,Ilookatthewholecatalogofsymptomsthatarepresent. I try to design a multilevel approach, usually involving polypharmacy, to address the medical issues as a part of all thethingsweneedtodo.Weshouldalsoemphasizetheimportanceofwatchingforpotentialadverseeffects,notjustfromtheindividualdrugsweareusing,butfromtheinteractionsamongthevariousmedications.Wemustalsorememberthatthedisease evolves over time, and we may have to alter what we are doing to manage those changes. At one stage of the disease, for example, the resident may be on 6 or 7 different medications, whereas at another point they may not need much medical management at all. And sometimes they will need more treatment later on, especially in the preterminal stage.

Wealsowanttoinvolvetheothermembersofthecareteam--theoccupationaltherapist,physicaltherapist,andsoon--whenspecial needs arise. The residents may need particular help with food and eating, or extra help with positioning in their chair, or theymayrequireaspecialchair.Weneedtoinvolvethewholeteaminordertoknowwhattheresident’sspecificneedsare.

Pg.18


Dr Nance: Let’s talk a little more about this idea of the care team. Nancy, who might be among the other people that are importantmembersoftheteam,andwhataretheirroles?

Ms Beecham: Of course there is the medical staff, the medical director (if he or she is not the attending physician), other physicians, and nurses. And there are the CNAs, who really are the hands and the eyes of the operation. They are our foot soldiers, the ones who are at the bedside, developing those crucial relationships with the residents. Also, there is the pharmacy consultant and, as Larry said, the speech, occupational, and physical therapists. You may also have family counselors or psychotherapists. Our residents love music therapy and art therapy, which are good outlets for them. Social workers are part of this group, as are financial planners, and end-of-life planners. And I also think of the residents and their families, when possible, as part of the team. As we discussed earlier, unfortunately, many families are not involved with the resident because of fear or guilt, and therefore the resident may feel abandoned. In some cases the facility staff becomes the family.

Dr Nance: HowdoyougetallthosefolksonboardtounderstandthecomplicatednatureofHuntington’sdisease?Larry,youhavealotofexperienceinhelpingtoeducatethestaff.Whataresomeofthebestpracticesyouhavedevelopedovertheyears?


Pg.19

Wealsostresstheneedforthestafftodevelopstrongrelationshipswiththeresidentsbecausetheyareworkingwiththemdayinand day out.

Ms Beecham: Over the years, we have found that allowing our residents with Huntington’s disease to be anywhere in the facility is agreatidea.Wehadoriginallythoughtabouthousingtheminonededicatedarea,andwhenwediscusseditwiththeCNAstheyadvised against it. They advocated that we integrate them throughout the community. There are some good reasons for doing this. They are younger people when they come in, they can be very helpful, they are engaging, and they often develop bonds with other residents.

Dr Banta: Yes.

Ms Beecham: Because they are going to be with us a long time, it helps when the staff can learn from the residents, and from each other, with regard to their triggers. The staff develops a respect for these residents; they discover what they can and cannot do, which makes it easier to accept them. It is not as frightening if the staff knows what to expect and can regard the patient as a long-term resident.

Dr Nance: I think we agree that continuity of care is important. For example, it can be valuable to have the same staff member get to know the residents and to note in the care plan what some of their unique likes and dislikes are.

Ms Beecham: That is critical. Knowing what will trigger a behavior and what it takes to avoid it is valuable information to write down and pass on.

Dr Banta: Often when the facility staff learns that a patient with Huntington’s disease is coming in, there is a lot of fear and anticipationofdisaster.Theyworry,“Whatarewegoingtodo?”Ofcourse,oneofthecuresforfeariseducation.Anotherisproviding the tools that the staff needs to be able to handle something that sounds overwhelming but that can be managed. Itisimportanttoemphasizethattherearemanythingswecandotohelptheseresidentsthroughtheirparticulardifficulty.Weneed to help the staff understand the nature of the disease, and what parts of the brain have failed and are causing the problems the resident is having. They cannot make decisions. They are inflexible. They cannot communicate. They have numerous physical challenges.

Weemphasizetheimportanceofworkingtogethertoaddresstheseissues,toprovideoptimalcareandthebestpossiblequalityof life for these individuals. Our care is palliative. It is proactive. It acknowledges individuals and engages their families.

Pg.20


Dr Nance: WhatareyourfinalthoughtsaboutwhatthebarriersmightbetoprovidingoptimalcareforpeoplewithHuntington’sdiseaseinlong-termcareandhowtoovercomethosebarriers?

Dr Banta: Placing an individual in a facility is the first barrier. In many cases, finding a place for these patients is very difficult. Most communities have limited resources and may not have a specialized behavioral unit in a long-term care facility. But providing appropriatetrainingandexpertiseinregularskillednursingfacilitiescanallowthesepatientstodowellthere.Weneedtobeableto educate the staff of the regular skilled nursing facilities as well.

Dr Nance: Theverynatureofthedisease--theirritability,thebehavioralproblems--canbeabarrier.Canthatbeovercome?

Dr Anderson: Absolutely. If you take the time to really work with these residents, using not only the psychopharmacologic interventions that we discussed, but also the behavioral interventions that Nancy and Larry mentioned, you really can manage these behaviors very well over time.

Another important thing to bear in mind is that this is a progressive condition. As we discussed, a medication that was great at one time may not be so great at another time. It is very important to know that side effects can show up after someone has been on a medication for a period of time, or as additional medications are added, or as the resident becomes more frail and more susceptible to adverse effects.

Dr Nance: One of the things I have learned as I talk to people around the country is that when patients move to long-term care, they often quit going to the doctor they have been seeing for many years. How can we help patients have good medical care and goodcontinuityofcareoncetheymovetolong-termfacilities?

Ms Beecham: You are right. Often these residents have had a long-term relationship with their physician before they come to thefacility.Whentheydocomein,theymaybeassignedtoanadmittingphysicianwhomayormaynothaveanyknowledgeorexpertisewithHuntington’sdisease.Whenpossible,ourgoalinlong-termcareistomakearrangementsforthoseresidentstobetransferred periodically so they can be seen by their original clinician. The reality is that, as the disease progresses and the person’s movements become more difficult, it is difficult to transport them. I encourage their previous physicians to come and visit the residents in their long-term care facilities.

Dr Nance: That provides an opportunity to promote communication and education between the physician who has treated the residentformanyyearsandthenursinghomephysicianorthegeriatricphysician.Whatresourcesareavailabletohelpthestaffandthephysiciansatthesefacilities?


Pg.21

Dr Banta: One organization that helps with a lot of issues in long-term care is the American Medical Director’s Association. Our nurses have a similar association, NADONA. There is also a lot of help available from the Huntington’s Disease Society of America (HDSA).TheirWebsiteisparticularlyvaluableinprovidingupdatesaboutcurrentresearchandthemanyissuesthatmustbeaddressed in the management of the disease.

Dr Nance: Although HDSA is primarily for patients and their families, it can be very useful for facility staff as a resource to learn about the disease.

Dr Banta: Absolutely.

Dr Nance: Karen, you were involved in an effort several years ago to review medication use.

Dr Anderson: That’sright.WesurveyedexpertsintheUnitedStates,Canada,Australia,andEuropeandcameupwithguidelinesfor treatment of irritability, and also for perseveration and obsessive and compulsive symptoms. These were published in PLOS Currents and are available free of charge on the internet.[4]

Dr Nance: Nancy, you have been involved with support groups in your area.

Ms Beecham: Right. It can be valuable to encourage facilities to become involved with patient and family support groups. There are opportunities for staff to learn from those who participate. One other point I would like to make is that often regulatory agencies come in to examine our facilities and they see bruises on our residents. They believe that the residents have fallen and they begin asking questions, such as, “How many times have theyfallen?”Thisbringsthefacilityunderthescrutinyoftheregulatoryagencies.Icreatedabookletthatwegivetoeachofthesurveyors to explain about Huntington’s disease and the physical problems the residents might experience, because, the fact is, they do not know about Huntington’s disease. They certainly do not understand the challenges we deal with. I think it is a good idea to be proactive before the surveyors come in.

Dr Nance: And a final resource that I was involved with is a book published by the HDSA, A Physician’s Guide to the Management of Huntington’s Disease.[8]ThisisavailableinprintandasadownloadfromthesocietyWebsite.Thebookisusefulforphysiciansandalso for nurses. Patients and families often use it, too. So there are a number of resources available to help patients, families, and staff at care facilities.

Pg.22


To summarize the key points that we made today: Huntington’s disease is very different from other conditions that are commonly seen in long-term care facilities. The treatment of Huntington’s disease is palliative. It is very helpful to have consistent assignments for the nursing staff at a facility. Teamwork is critical, involving physical, speech, and occupational therapy, the dietitian, the psychologist, the social worker, and the consulting pharmacist, as well as physicians. The treatment of Huntington’s disease has to be adjusted as the disease progresses. Sometimes medications that are useful early in the disease are not necessary later in the disease, or vice versa. It is important to understand that the symptoms, including the chorea and behavioral symptoms can be treated. And finally, I always end a discussion by saying there is never nothing you can do for somebody with Huntington’s disease.

I would like to thank the panelists for being here today; Karen Anderson, Larry Banta, Nancy Beecham. Thanks so much.

Dr Banta: Our pleasure.

Dr Anderson: Thank you.

Dr Nance: Thank you for participating in this activity. You may now take the CME/CE posttest by clicking on the Earn CME/CE Credit link. Please also take a moment to complete the program evaluation that follows. Thank you.

This transcript has been edited for style and clarity.

This article is a CME/CE certified activity. To earn credit for this activity visit:medscape.org/spotlight/huntingtons


Pg.23

ResourcesAmerican Medical Directors Association http://www.amda.com/Huntington’s Disease Society of America http://www.hdsa.org/National Association of Directors of Nursing/Long Term Care, Inc. http://www.nadona.org/A Physician’s Guide to the Management of Huntington’s Disease http://www.hdsa.org/images/content/1/6/16692/HDSAPhysDeskRef_11_web.pdf

DisclaimerThe educational activity presented above may involve simulated case-based scenarios. The patients depicted in these scenarios are fictitious and no association with any actual patient is intended or should be inferred.

The material presented here does not necessarily reflect the views of Medscape, LLC, or companies that support educational programming on medscape.org. These materials may discuss therapeutic products that have not been approved by the US Food and Drug Administration and off-label uses of approved products. A qualified healthcare professional should be consulted before using any therapeutic product discussed. Readers should verify all information and data before treating patients or employing any therapies described in this educational activity.

Medscape Education © 2013 Medscape, LLC with permission.

This article is a CME/CE certified activity. To earn credit for this activity visit: medscape.org/spotlight/huntingtons

AbbreviationsAMDA = American Medical Director’s Association CNA = certified nursing assistantHDSA = Huntington’s Disease Society of AmericaNADONA = National Association Directors of Nursing AdministrationSSRI = selective serotonin reuptake inhibitor

References1.Huntington’sDiseaseSocietyofAmerica.WhatIsHuntington’sDisease(HD)?Availableat:http://www.hdsa.org/about/our-mission/what-is-hd.html. Accessed May 3, 2013.2. Haddad MS, Cummings JL. Huntington’s disease: neuropsychiatry of the basal ganglia. Psychiatr Clin North Am. 1997;20:791-807.3. Videnovic A. Treatment of Huntington disease. Curr Treat Options Neurol. 2013 Feb 16. Epub ahead of print.4. Groves M, van Duijn E, Anderson K, et al. An international survey-based algorithm for the pharmacologic treatment of irritability in Huntington’s disease. PLoS Curr. 2011; doi: 10.1371/currents.RRN1259. 5. Xenaxine [package insert]. Deerfield, IL: Lundbeck; 2012.6. Armstrong MJ, Miyasaki JM; American Academy of Neurology. Evidence-based guideline: pharmacologic treatment of chorea in Huntington disease: report of the guideline development subcommittee of the American Academy of Neurology. Neurology. 2012;79;597-603. 7. Paleacu D. Tetrabenazine in the treatment of Huntington’s disease. Neuropsychiatr Dis Treat. 2007;3:545-551. 8.NanceM,PaulsenJS,RosenblattA,WheelockV.A Physician’s Guide to the Management of Huntington’s Disease. 3rd ed. New York, NY: Huntington’s Disease SocietyofAmerica;2011.Huntington’sDiseaseSocietyofAmericaWebsite.http://www.hdsa.org/images/content/1/6/16692/HDSAPhysDeskRef_11_ web.pdf. Accessed April 9, 2013.

Pg.24


Medscape, LLC825 Eighth Avenue, 11th FloorNew York, NY 10019

1-888-506-6098

Improving Therapeutic Management of Huntington’s Disease in...

Documents

Transcript of Improving Therapeutic Management of Huntington’s Disease in...