Speech and Swallowing Issues in Wilson Disease Kristin Larsen, MA CCC-SLP Communication Sciences and Disorders Northwestern University

Speech Problems in Wilson Disease   Common sign of neurological involvement Dysarthria: refers to speech disorders of a neurological origin resulting from disturbances in muscular control of the speech mechanism  May be hypokinetic, spastic, ataxic--usually a combination

Hypokinetic Dysarthria   Caused by damage to the basal ganglia control circuit Most frequently found in Parkinson’s Disease or other related CNS degenerative disorders  Hypokinetic refers to decreased mobility or range of motion--decreased “amplitude” of speech

Hypokinetic Dysarthria Characteristics     Reduced loudness Monopitch Monoloudness Imprecise consonant articulation  Fast speech rate     Short rushes of speech Lower pitch Palilalia Breathy voice quality

Hypokinetic Dysarthria-patient perceptions    People can’t hear me People don’t understand me I can’t communicate well in large groups or in public places  My spouse/parent needs a hearing aid!

Hypokinetic Dysarthria Treatment    Generally focuses on increasing loudness/effort, reducing speech rate and improving articulation Key focus: “Think loud, Be loud” LSVT: Speech/voice treatment program developed for Parkinson’s disease, but has proven useful in related diseases

Spastic Dysarthria  Caused by damage to direct and indirect activation pathways of the CNS-bilaterally  Found in vascular disorders, inflammatory diseases and degenerative disorders  Spastic refers to excessive muscle tone

Spastic Dysarthria Characteristics  Strained-strangled voice quality    Harshness Slow rate Imprecise consonant articulation  Distorted vowels     Hypernasality Short Phrases Pitch breaks Excess and equal stress   Monopitch Monoloudness

Spastic Dysarthria-patient perceptions       It takes more effort to speak I speak so slowly I get tired quickly from talking My speech sounds nasal Difficulty controlling emotional expression Often complains of difficulty chewing or swallowing as well

Spastic Dysarthria-Treatment     Reduce muscle tone Relaxation techniques Easy onset of phonation Gentle stretching/range of motion exercises not to the point of fatigue

Ataxic Dysarthria  Caused by damage to the cerebellar control circuit  Found in degenerative diseases, vascular disorders, neoplastic disorders, toxic metabolic conditions and trauma  Characterized by reduced coordination of speech

Ataxic Dysarthria Characteristics       Irregular articulatory breakdown Vowel distortions Prolonged sounds Slow rate Monopitch/monoloudness Excess and equal stress

Ataxic Dysarthria-patient perceptions     Slurred speech “Drunken” sounding speech Stumbling over words Reduced coordination with chewing

Ataxic Dysarthria Treatment    Focuses on modifying rate and prosody Slow down!

Pitch control

Speech Therapy  Diagnosis of speech problem: will determine treatment plan  Treatment: will focus on compensation, augmentation or exercise program as appropriate  Compensations must be practiced frequently to be habituated

General Communication Strategies for Dysarthria      Slow down Take a breath before you start talking Pause for a new breath as needed Exaggerate your speech Control your environment--avoid competing noise when possible

General Communication Strategies for Dysarthria     Set the context: what is the main idea?

Modify the length of the utterance Monitor listener comprehension Use letter/word/picture board or gestures to supplement verbal communication

Strategies for the Listener  Modify the environment-reduce excess noise/distractions, maintain adequate lighting   Maintain eye contact Repeat or clarify the message--let the speaker know what parts you understood  Ask focused questions to clarify message

More Strategies for the Listener     Establish how and when to provide feedback Encourage use of appropriate strategies Model appropriate strategies Encourage use of augmentative communication as needed

Augmentation-when useful speech is limited:low tech      Writing Letter/picture board Personalized communication book Develop consistent yes/no response Use gestures

Augmentation-when useful speech is limited: high tech    Alternative and augmentative communication (AAC) devices  Computer systems: variable expense, level of difficulty Speech software for existing computers Smart phone applications

AAC Device Considerations      Input or access features: how to select letters/words/pictures--direct or scanning.

Output features: voice or readable Portability Cost/funding and insurance coverage Training or learning curve: how easy is it to operate?

Dysphagia   Difficulty with any phase of swallowing May result in aspiration:food or liquid entering the airway-can lead to pneumonia  May result in inefficiency-can lead to longer mealtimes, weight loss, malnutrition

Dysphagia in Wilson Disease    Swallowing difficulty is a common complaint with neurologic manifestation of Wilson Disease Can vary from mild to severe May or may not be accompanied by difficulty with secretion management/drooling

Dysphagia in Wilson Disease     Can involve any stage of swallowing: oral prep/chewing, oral transit, or pharyngeal Involvement of the basal ganglia can impair the coordination of chewing and swallowing Dystonia affecting head or neck muscles can affect ability to swallow safely Pseudobulbar palsy-weakness in lips, tongue or throat muscles can reduced efficiency and lead to aspiration

Role of SLP in Dysphagia Management  Assessment: clinical, endoscopic or videofluoroscopic  Develop appropriate treatment plan: compensations (postures, maneuvers), diet modifications  Monitor progression of swallowing problems  Monitor need for possible non-oral nutrition

Early Signs of Dysphagia     Longer mealtimes Coughing with liquids Difficulty with chewier foods Difficulty with mixed consistencies (cereal in milk, chunky soups)   Feeling food or pills sticking in throat Coughing during or after meals

Signs of Advanced Dysphagia     Aspiration Decrease in caloric intake (weight loss, malnutrition) Decrease in fluid intake (dehydration) Fatigue or excessive inefficiency with mealtimes--unable to meet nutritional needs

Swallowing Guidelines: Posture   Sit as upright as possible Keep head in a neutral or slightly chin down position if indicated/possible  Stay sitting upright for 30 minutes after meals to allow time for all the food to go down (if any food remaining in mouth or throat

General Swallowing Guidelines    Eat and drink slowly-allow plenty of time for meals   Chew thoroughly Focus on the task of eating-eliminate distractions like TV Don’t talk with food or liquid in your mouth Swallow everything in your mouth before taking a new bite/sip

Diet Modification Guidelines      Caution with mixed consistencies May need to choose softer foods May need to thicken liquids Smaller, more frequent meals if fatigued Nutritional supplements--drinks or puddings (try to avoid ones with added copper)

Non-oral Nutrition     If aspiration, malnutrition, dehydration or inefficiency become a problem… Surgical placement of a gastrostomy or jejeunostomy tube for nutrition Highly personal decision, quality of life considerations May still be able to take some foods/liquids by mouth

Drooling/Saliva Management       Medications Botox Radiation to salivary glands Maintain adequate hydration Use suctioning as needed Secretions management techniques

Secretion Management Techniques    SWALLOW! Remind yourself to “slurp and swallow” throughout the day--especially before you speak If able, try to sip water frequently If able, chewing gum or sucking on a hard candy can increase swallow frequency