Chapter 3-Phsyician's Guide to Management of HD

Movement Disorder

Falls

Falls are common in persons with HD, and can be a source of significant morbidity. Usually seen more in the moderate to advanced stages, they often result from the combination of spasticity, rigidity, chorea, and loss of balance.

Pharmacotherapy to prevent falls could include treatment of chorea, rigidity, spasticity and dystonia, while minimizing the use of drugs such as neuroleptics and benzodiazepines, whose side effects include sedation, ataxia, or parkinsonism.

Most efforts at prevention, however, involve not drugs, but modification of the environment and behavior of the patient.

• Occupational and physical therapists can instruct patients in how to sit, stand, transfer, and walk more safely.
• Installing handrails in key locations, and minimizing the use of stairs can help to reduce falls.
• Some families convert a ground floor office or den into a bedroom.
• Furniture such as tables and desks, particularly items with sharp corners, should be arrayed along the periphery of the room, where they will present less of an obstacle.
• Floors should be carpeted to lessen the impact when falls do occur.
• Patients who fall out of bed may have a mattress placed beside the bed at night, or may sleep on a mattress placed directly on the floor.

HD patients will eventually become unable to walk and will need to be transported in a wheelchair. A weighted and padded chair, perhaps with a wedge to keep the hips tilted, or a pommel between the legs, may minimize the chance of a severely choreic or dystonic patient falling or sliding out, or knocking over the chair (see Appendix 3).

Use of a wheelchair is not an all or nothing proposition. Mobility may be extended by using the wheelchair for longer excursions and using other assistive devices such as a walker for shorter distances, or in the home.

Walkers with front wheels may be particularly useful when rigidity or loss of balance is a problem. Patients who are particularly prone to falls sometimes wear helmets, or elbow and knee pads to minimize injury. Physical therapy may also help by teaching patients how to minimize injury in a fall and how to get up again after a fall.

General Safety Measures

A number of other environmental interventions may reduce the risk of injury.

• Patients who smoke should do so in a room without flammables, such as rugs, curtains and overstuffed furniture.
• Patients may need to stop using sharp knives and to switch to microwave cooking to prevent burns and spills.
• Falls in the bathroom are particularly dangerous, but there are a variety of assistive devices that can be installed.
• Consultation with a visiting nurse, or a visit from a physical or occupational therapist may be very helpful for any mid-stage HD patient being cared for in the home.
• A sample home visit consultation form is provided in Appendix 4.  (not available on line)

Chapter 6-Other Issues

Driving

All patients with HD eventually lose the ability to drive. This can be a severe blow for some patients, who see driving as a sign of competence and a way of maintaining independence. In many cases, patients, with the help of their families, will realize the time has come and will voluntarily stop driving, often before their physician has come to this conclusion. Other times, however, the issue of driving can become a source of contention between patients, families, and physicians.

People with HD can be divided into groups on the basis of their driving abilities.

• Some mildly affected patients may have no significant problems and simply need to remain alert and not drive when very tired, after drinking, or under hazardous conditions.
• Most moderately to severely affected patients are not safe behind the wheel.

A large number of patients occupy the middle ground; they may have mild symptoms, but the safety of their driving is uncertain.

The physician should ask family members who have driven with the patient for their impressions, and should inquire about recent accidents and traffic citations, including those that were "someone else's fault." Some patients minimize their disability.

A formal driving evaluation, at an occupational therapy or rehabilitation center may be available and can help both physician and patient by providing objective information about the individual's performance.

In a situation in which a patient has become a hazardous driver and is unwilling to stop, or lacks insight into the degree of impairment, the doctor must intervene forcefully for the protection of the patient and others. We have found it useful at such times to give the patient a "doctor's order" rather than a suggestion, and to tell the patient that the instruction to stop driving will be documented in the record.

Some states may require physicians to notify the Department of Motor Vehicles if a patient is no longer safe to drive. In other states, physicians may be held liable if they make such a report without the patient's consent.

Family members, however, are not bound by such constraints and should contact their Department of Motor Vehicles if they feel the patient is dangerous and will not listen to reason. This is a very unpleasant responsibility, but it must be shouldered. Such reports have been made anonymously at times, to preserve harmony.

Smoking

Smoking sometimes becomes a problem for people with HD, for two reasons. Changes in the person's behavior related to disinhibition, personality changes, and perhaps boredom may turn smoking into a consuming passion, leading to irritability and even violence if thwarted.

Simultaneously chorea, impairment of voluntary movements, impaired judgement, and diminished capacity for self observation may make the act of smoking unsafe.

A variety of approaches have been helpful in decreasing the behavior and improving safety. Non-pharmacologic interventions include the establishment of smoking schedules and general safety measures such as ensuring that the patient does not smoke in bed, limiting smoking to rooms without rugs, and use of adaptive devices, such as a flexible tube smoker or a "smoker's robot," available through rehabilitation supply and safety product catalogs  (see Appendix 3-http://hdlighthouse.org/see/pg2/a3-1.htm).

We have also used nicotine patches with some success. The goal is not necessarily to wean the patient completely off cigarettes or patches, but to decrease the drive for cigarettes, and the periods of nicotine withdrawal, which may worsen irritability. A variety of the antidepressant buproprion has also recently been marketed for use in smoking cessation and may be worth a try.