Table 11

Planning in advanced MSA

SymptomTreatment optionsWhen recommendedThings to considerComments
Swallowing difficulties; Inability to maintain sufficient nutrition/hydration, repeated aspiration pneumonia or chokingDiet modification and supplementation, risk feeding, gastrostomy insertionWhen unable to maintain sufficient oral intake to maintain weight, when swallow is unsafe, if chest infections/aspiration pneumonia. Allow time for patient to consider pros and cons and assist patient to document wishes.Can improve quality of life and help manage blood pressure, hydration, constipation, and may not be possible in later stagesPercutaneous endoscopic gastrostomy (PEG) may improve quality but not length of life. There is currently insufficient evidence on PEG or radiologically inserted gastrostomy use in MSA.
Speech and communication difficultiesLow-tech equipment options can help communication access via speech and language therapy (SaLT)—a specific communication assessment may need to be requested.Voice banking as early as possible (can be done through MSA Trust and SaLT). Regular review by SaLT to assess changes to communication and allow time to refer onto specialist services if required.Equipment can be useful and aid communication but use of technology needs to be learnt in good time. It can greatly improve quality of life and reduce isolation. Mobility/dexterity issues and cognitive issues can limit use of communication devices.NHS England fund Augmentative and Alternative Communication services help people communicate as effectively as possible when speech is impaired. Environmental and access to technology services are also available. Get to know what service is in your area and the referral criteria.
MobilityAppropriate equipment and support to maintain safety, provided by occupation therapy and physiotherapy and other services as appropriate, for example, tissue viability, wheelchair services, orthotics.When current methods of mobilising or transferring might become unsafe or uncomfortable. In advanced stages when too fatigued or sleepy to want to be sat out of bed, or when they find they prefer to remain in bed for longer periods.Patients will need appropriate equipment, support and care to maintain skin integrity and avoid pressure areas developing and meet care needs.Patients will need support of occupational therapy teams for equipment. Care services may be involved. District Nurses and Community Matron can provide care, facilitate services and be link between patient and GP. Neurology and Specialist services may need to link with GP and specialist palliative care services.
Respiratory symptoms—aspiration, stridor, vocal cord paralysis, obstructive and central sleep apnoea, respiratory insufficiencyRespiratory support such as CPAP may be needed, or further input from respiratory if already in place. Tracheostomy may be considered if CPAP insufficient.Discuss potential for respiratory difficulties in advance. Refer for investigation and specialist support if symptomatic.CPAP may not be tolerated or discontinued if infection or daytime respiratory insufficiency. Tracheostomy may have impact on care needs.Sudden death can still occur even with CPAP or tracheostomy due to central sleep apnoea. Advance care planning should be in place to understand patient’s preferences in good time and to prevent decisions about for example, tracheostomy being made in a crisis or medical emergency.
Recurrent infectionsAntibiotics for acute infections, discuss treatment preferences with patient.As early as possible; consider ‘rescue’ antibiotics at home and remember people with MSA may not have a temperature in infection due to autonomic dysfunction.Antibiotics may become less effective overtime. Patients may not wish to be admitted for intravenous antibiotics in later stages; discuss and document treatment preferences with patients and family.Respiratory physiotherapy (suctioning and cough assist machines may help with secretion management if problematic) For urinary tract infections, low-dose prophylactic antibiotics or silver-lined catheters may reduce infection—refer to urology and continence services.
Blood pressure managementNon-pharmacological and pharmacological optionsWhen there is symptomatic postural hypotension. Beware of supine hypertension.Can pose significant challenges for example, syncope when opening bowels and postprandial BP drop, altered consciousness and risk of falls if still transferring or mobilising.24-hour BP monitoring can inform management strategies. Specialist advice can be sought if needed.
Advance care planning and referral to specialist palliative careDiscussing treatments options, exploring personal preferences and recording these appropriately. Hospice services can provide practical and emotional support for person with MSA and their families and carers.At all stages, by all healthcare professionals, may include Neurologist, Parkinsons’s nurse specialist, neurology nurse specialist, palliative care team and GP and primary care teams.Advance care planning should be revisited regularly to ensure documentation is accurate, all relevant teams are aware and to ensure documentation of reconfirmation of wishes or changes to preferences recorded and communicated.May include do not attempt resuscitation, Power of Attorney, advance directive for refusal of treatment, treatment preferences for interventions such as PEG or tracheostomy, where they would like to be cared for and end of life wishes, etc.
  • BP, blood pressure; CPAP, continuous positive airway pressure; GP, general practitioner; MSA, multiple system atrophy; NHS, National Health Service.