Recommended Treatments And Therapies

Importance Of Exercise In Huntington Disease (HD)

Walking difficulty and imbalance are two of the predominant features in HD. This is caused by both the presence of involuntary movements and the absence of ability to perform voluntary movements. In general, exercise program is found to be highly beneficial for a person with Huntington diseases. The main goal of the exercise interventions is fall prevention/reduction and improvement of cardiovascular efficiency thus achieving a better quality of life.
The falls prevention exercise program contains Range of motion exercise and stretching to maintain joint integrity and prevent contractures. Its better the exercise is performed by the patient themselves than the caregiver passively administer a range of motion and stretching exercises. In severe cases, splinting to keep the joint in optimal position is advised.
Other than improving flexibility and strength, the external causes like clustered living room with furniture and obstructed walkway with telephone cable, poorly lighted room, slippery surface etc. needs to be taken care to ensure prevention of falls. Also, to keep a watch on the effects of medication on mobility.
The attitude of the caregiver should be positive and to maintain calm and composed mental status to avoid provoked responses. It is advised to have a silent, moderately lighted environment to practice the exercises. Any distractive environment brings non-cooperation from the person with HD.Aerobic exercise has its own advantage of keeping the healthy heart thereby keeping the brain healthy too. Simple walking starting from 5 to 10 minutes up to 45 minutes a day is a regime followed universally. Studies have found that other than improving endurance, aerobic exercise can offer neuroprotective effect which means slow down of the severity of the diseases.
Finally, the compassionate approach by the stakeholders indeed is very much in the card for the successful management of Huntington disease.

Dysphagia Management

Swallowing difficulty could be of various types depending on the muscles affected. The oral musculature may be impacted wherein, for instance, due to involuntary movements of tongue muscles there may be difficulty in chewing, inability to form a bolus and swallow it. In addition pharyngeal and oesophageal muscles may also be affected. There can be a delay in the triggering of swallow reflex and hence there may be premature spillage of food or liquid into the passage backwards in the mouth. Food passes through 3 phases before it reaches stomach (oral, pharyngeal and oesophageal). In all 3 phases, there is involvement of voluntary muscle movement and automatic coordination, which can be affected sooner or later in the course of HD. Depending on the severity of impairment, patients are advised to adopt compensatory measures to minimize the impact. Some compensatory measures include eating slowly, eating more semi solids, chewing slowly enough to form a bolus etc. Swallowing difficulty assumes greater significance also for the reason that there can aspiration of food (food entering the wind pipe and then lungs), which could be dangerous. So any difficulty in eating/swallowing should be addressed with a certified clinician before it gets worse. Needless to say it significantly affects the nutritional and hydrational status of the patient with HD.
Abnormal whole body posturing can also affect eating and swallowing. The abnormal posturing, which is due to excessive involuntary movements, often prevents the ease of transport of food or drink from the hand to the mouth and causes spillage.
Educating people with HD and the caretakers (family, others) about swallowing aspects is the first step in management of dysphagia. Safe swallow strategies during eating and drinking include using an appropriate posture, avoiding distraction, not taking in too much quantity at each intake as well as gaining more control over food / water in mouth through feedback.

Dysarthria Management

Quality of speech gradually deteriorates during the course of HD. Again there are many types of impairments depending on the types of muscles involved and the amount of incoordination in each case. Voice production may also be affected with arrhythmic expiratory bursts. Few patients may have slow speech and difficulty in uttering specific syllables, where as other few people may have explosive speech etc. Being patient, attentive to their speech and encouraging them to communicate using verbal and non-verbal modes can act against the progression of the disease especially thinking abilities.
People with HD must be provided with enough safety measures, such as appropriate support and space, during speaking, as their involuntary movements could be enhanced while speaking. Feedback on various aspects of speech like breath support, voicing and pronunciation might help some people with HD to improve their speech quality. Gestures may be encouraged along with speech, if necessary, since the goal is effective communication. In addition assistive devices may be used for effective communication as well. However, these devices need to be individualised given the involuntary movements in people with HD.