Palliative Radiation to Induce Xerostomia in Patients with Bulbar Dysfunction Secondary to ALS

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Palliative Radiation to Induce Xerostomia in
Patients with Bulbar Dysfunction Secondary to ALS

• Eric Anderson, Sean Collins, Michael Sirdofsky,
  and Kari Esbensen

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Background

• Patients with amyotrophic lateral sclerosis (ALS)
  often suffer from bulbar dysfunction
• can be initial manifestation
• dysarthria, dysphagia, impaired gag reflex,
  pseudobulbar affect
• inability to coordinate swallowing and absence of
  spontaneous swallowing lead to profuse salivation
  and drooling (sialorrhea)

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Sialorrhea

• Complications of excessive salivation or drooling
• Perioral chapping
• Maceration and secondary infection
• Dehydration
• Odor
• Dermatitis
• Increased risk of aspiration
• Social stigmatization (patients and caregivers)
• Increased dependency
• Reduced QOL

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Management of Sialorrhea

• Anticholinergics
• often effective in reducing or eliminating
  excessive oral secretions in these patients
• the side effects of these medications are often
  intolerable and lead to the discontinuation of
  these drugs or a decrease in their dosage to the
  point that salivation is not adequately
  controlled
• severe constipation
• urinary retention
• blurred vision
• diminished QOL

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Management of Sialorrhea

• Injection of botulinum toxin type A into the
  parotid and submandibular glands
• safe and effective in controlling drooling
• effects fade in several months, and repeat
  injections are necessary
• very expensive

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Management of Sialorrhea

• Surgical intervention denervation of salivary
  glands
• performed through the middle ear, where the
  tympanic plexus and chorda tympani travel before
  entering the major salivary glands
• does not require general anesthesia
• salivary function returns within 6-18 months

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Management of Sialorrhea

• Surgical intervention salivary gland excision,
  salivary duct ligation, duct rerouting
• effective and permanent treatment of significant
  sialorrhea
• invasive
• non-ideal surgical candidates

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Background and Rationale

• To our knowledge, this is the first attempt to
  use radiation treatment as a means of decreasing
  problematic salivation in patients unable to
  swallow due to bulbar dysfunction.
• Radiation therapy provides a therapeutic
  alternative that avoids producing constipation
  and other anticholinergic side effects of
  standard medical therapy and is less invasive
  than surgical interventions or injections.

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Background and Rationale (cont.)

• Studies on patients with head and neck cancers
  treated with external beam radiation have shown
  xerostomia (dry mouth) to be one of the major
  side effects of radiation to the parotid glands.
• In our population of patients, this side effect
  of radiation was used to therapeutic advantage to
  reduce or eliminate salivation and thereby
  improve their quality of life.

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Background and Rationale (cont.)

• Side effects of radiation noted in head and neck
  cancer patients also include diminished taste,
  sticky saliva, stomatitis, oral candidiasis, and
  chewing and swallowing difficulties.
• ALS patients with bulbar dysfunction already
  experience significant problems with chewing and
  swallowing, and many receive nutrition via tube
  feedings.
• XRT might provide a viable treatment alternative
  with minimal disadvantages, especially for
  patients already experiencing many of the
  possible side effects as a result of their
  underlying conditions.

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Objectives

• To evaluate the occurrence of side effects from
  radiation in our patients as well as to examine
  whether the benefits of this therapy outweigh the
  burdens of treatment in ALS patients
• To assess patient satisfaction with the results
  of treatment, the need for further treatment
  following initial radiation therapy, and whether
  this new treatment modality can serve as a useful
  palliative measure in similar patients suffering
  from bulbar dysfunction

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Study Design

• Retrospective chart review of ALS patients
  treated in MDA clinic who received palliative
  radiation for control of sialorrhea
• 6 patients thus far and 2 more scheduled for tx
• Patients received 700 cGy x 1 fraction of
  conventional radiation to the parotid glands.
• Patients were routinely followed every 2-3 months
  in the MDA clinic and questioned about
  improvement in their symptoms.

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Study Weaknesses

• Retrospective rather than prospective
• Limited data for comparison of symptoms before
  and after treatment
• Patient questionnaire
• System for assessing degree of sialorrhea

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Amyotrophic Lateral Sclerosis (ALS)

• Majority of cases are sporadic/idiopathic
• Mean duration between the onset of symptoms and
  death in sporadic ALS ranges from 27 to 43 months
  (range from lt1 yr to gt20 yrs)
• The average 5-year survival is 25
• Death from progressive respiratory failure
• End-of-life decision making
• less than 5 of ALS patients choose long-term
  ventilatory support by tracheostomy

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MDA Multidisciplinary Clinic at GUH

• Neurologist (Dr. Sirdofsky)
• Pulmonologist (Dr. Anderson)
• Spirometry (usually asymptomatic until lt50 FVC)
• BiPAP
• End-of-life discussions
• Ventilator support
• Speech and Occupational Therapists
• Nutritionists
• Social Workers
• Assistive Devices
• 1st Wed of every month

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References

• Braam, P.M., et al. Quality of Life and Salivary
  Output in Patients with Head-and-Neck Cancer Five
  Years After Radiotherapy. Radiation Oncology
  200723-10.
• Hockstein, N.G., et al. Sialorrhea A Management
  Challenge. Am Fam Physician 2004692628-34.
• Jellema, A.P., et al. Unilateral versus
  Bilateral Irradiation in Squamous Cell Head and
  Neck Cancer in Relation to Patient-Rated
  Xerostomia and Sticky Saliva. Radiotherapy and
  Oncology 2007 doi10.1016/j.radonc.2007.03.002.
• Thomas-Stonell N, Greenberg J. Three Treatment
  Approaches and Clinical Factors in the Reduction
  of Drooling. Dysphagia 1988375.