ALS treatment
adapted for
the real world

CASE STUDY A*

Here we present the case of a male in his early fifties. Emotionally labile and increasingly immobile and without speech, Simon needed the full dose of riluzole but may not have been receiving it properly through his PEG – until a different approach was advised.

Patient pseudonym
Simon J
Age
52
Onset of symptoms
September 2014
Date of diagnosic
June 2015 (approx. 9 months from onset)

Symptoms since diagnosis

  • Emotional lability
  • Deteriorating bulbar function/poor swallow/excessive saliva – PEG in situ since Jan 2016 (now unable to eat/drink)
  • Recently lost speech – uses iPad
  • Upper limb weakness affecting arms and hands, no function in R arm now
  • Lower limbs now affected, unable to walk, in electric wheelchair/hoisted
  • Spasm in legs – treated with baclofen
  • Deteriorating respiratory function, but struggles to tolerate NIV
  • Panic attacks/chest infection/oral thrush

Treatment and support

  • Riluzole: Wife was concerned Simon wasn’t receiving the right amount of riluzole, as she was having to crush his tablets and flush through a PEG. As well as struggling to get the mixture through his PEG tube, she noticed it was also lining the empty container after it had been mixed with water. The specialist neurology nurse put forward Teglutik® as an option in preference to tablets, given that a liquid formulation now exists to remove the need for crushing
  • SALT: Community speech and language therapist provides communication aids and assesses speech and swallowing ability, advising on safety and food/fluid consistency
  • MDT: Under the care of a neurologist, MND specialist nurse, and community dietician
  • MND Association: Visited by a representative of the organisation for advice, support and information

Impact of Teglutik®

Simon’s wife feels a bit happier knowing that Simon is now getting the full dose of riluzole. Both she and the MND specialist nurse are more comfortable with giving a liquid formulation of riluzole that has been designed for this purpose. It is also easier for Simon’s wife to administer, as she can use it as it is without the added complications and concerns of trying to crush tablets evenly, dissolve them as fully as possible, and flush through effectively and without waste.

Teglutik® is not currently indicated for administration by PEG/enteral feeding tube. The compatibility of Teglutik® when administered through tubes, typically used in the UK for PEG feeding, has been studied; please contact Martindale Pharma Medical Information at medinfo@martindalepharma.co.uk for a copy of this information.

*This is an amalgamation of two real patient cases based on details as submitted by healthcare professionals.

CASE STUDY B*

Here we present the case of a retired female. Linda was experiencing severe difficulties with food and choking which became progressively worse. As swallowing tablets was a clear problem, another route of administration was proposed.

Patient pseudonym
Linda F
Age
65
Onset of symptoms
December 2014
Date of diagnosic
March 2015 (approx. 4 months from onset)

Symptoms since diagnosis

  • Progressing weakness in various muscles
  • Breathing difficulties affecting speech, on top of increasing slurring
  • Reduced appetite, weight loss
  • Occasional choking – has had to alter consistency of foods and been advised to thicken fluids
  • Mobility restricted – bed moved downstairs
  • Uses frame for walking – manages only short distances and always under supervision due to frequent falls. Wheelchair used outside the home
  • Constipation, partly due to dietary changes, and partly to lack of mobility
  • Some type II respiratory failure signs; breathless on minimal exertion and when lying flat

Treatment and support

  • Riluzole: Given as tablets initially, then switched to liquid formulation when available
  • MDT: Under the care of GP, consultant neurologist, specialist nurse, physiotherapist, occupational therapist and dietician. The team members provide ongoing monitoring of disease progression, symptom management, advice and support
  • Community therapists: Community speech and language therapist provides communication aids and assesses speech
  • and swallowing ability; community occupational therapists assess need for and provides living aids and home adaptations; district nurse visit for support and monitoring of constipation and pressure area care
  • Hospice: Referred to local hospice for outreach palliative care nurse and day therapy
  • MND Association: Visited by a representative of the organisation for advice, support and information

Impact of Teglutik®

Linda’s husband and medical team much prefer to give the liquid formulation of riluzole to Linda, particularly in view of her occasional problems with choking. While initially after diagnosis Linda was prescribed riluzole tablets, the MDT took the decision to move to Teglutik® as soon as it became available; a decision supported by the community therapists who did not want to have to recommend crushing tablets. Linda was worried that this measure would soon have become almost a necessity had it not been for the release of the liquid formulation, and is relieved her husband does not have to do something that she knew was not advised.

*All details are as provided by the submitting healthcare professional, with the exception of the dates of symptom onset and diagnosis, which have been estimated based on the case notes.

Welcome
to Teglutik®

Teglutik® is the first oral liquid suspension formulation of riluzole, the only licensed treatment for patients with amyotrophic lateral sclerosis (ALS), the most common type of motor neuron disease (MND).

This site provides information on Teglutik® and ALS for UK-based healthcare professionals and patients. Please select from the buttons below to tailor the content to your needs:

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For additional information about MND you can visit the Motor Neurone Disease Association website. The Motor Neurone Disease Association is the only national charity in England, Wales and Northern Ireland focused on MND care, research and campaigning

http://www.mndassociation.org/