Shared Care Guideline
RILUZOLE
Treatment ofAmyotropic Lateral Sclerosis (ALS) Motor Neurone Disease in Adults

Dear GP

This shared care agreement outlines suggested ways in which the responsibilities for managing the prescribing of riluzolefor patients with amyotropic lateral sclerosis (ALS) form of motor neurone disease (MND)can be shared between the specialist (i.e. neurologist), the patient and yourselves as the patient’s general practitioner (GP).

You have been invited to participate but if you are not confident to undertake these roles, then you are under no obligation to do so. We would be grateful if you would reply to this request as soon as practicable.

Sharing of care assumes communication between the specialist, GP, patient and their carers. The intention to ‘share care’ should be explained to the patient and their carers by the doctor initiating treatment. It is important that patients and/or their carers are consulted about this treatment and are in agreement with it.

Background Information

Motor neurone disease (MND) is characterised by progressive degeneration of the motor neurones of the brain, brain stem or spinal cord. Depending on the site of the lesions, characteristic signs may include spasticity, muscle stiffness, brisk or diminished reflexes, muscle wasting and fasciculation, and both flaccid and/or spastic weakness.

It is hypothesised that excessive stimulation of glutamatereceptors on neurones may cause or play an important role in the destruction of motor neurones in MND. Glutamate is a neurotransmitter that tends to excite motor neurone cells. In vitro, riluzole inhibits the release of glutamate, decreases firing of motor neurones induced by glutamate receptor agonists and thus protects cells from glutamate-mediated damage.

Riluzoleis indicated to extend life or the time to mechanical ventilation for patients with amyotrophic lateral sclerosis (ALS).

Other pharmacological interventions are aimed at providing symptomatic relief for people with MND. Surgical interventions may be necessary and include percutaneous gastrostomy to enable feeding and tracheostomy with or without ventilatory support to aid breathing as respiratory muscle weakness increases. A wide range of multidisciplinary health and social services are required for people with MND, particularly in the late stages of the disease. These need to be tailored to suit individual needs.

NICE Technology Appraisal 20 recommends riluzole for the treatment of patients with the ALS form of MND and it is on the UCLH formulary for the above indication.

RESPONSIBILITIES ROLES

Consultant Neurologist
  1. Initiate and optimise (stabilise) treatment with riluzole and continue to prescribe until the GP formally agrees to share care (at least for first two months of treatment).
  2. Discuss the benefits and side effects of treatment with the patient. Provide the patient with a Patient Information Leaflet, explain it and ensure that the patient understands the reason for the treatment and dosage regimen.
  3. Write to the GP with a standard letter asking whether he or she is willing to participate in shared care.
  4. Clinically supervise patient by routine clinic follow-ups every 4-6 monthsand monitor response to treatment. Provide a copy of the current patient care plan for the GP.
  5. Measure baseline LFTs.
  6. Inform GP of results of tests performed in secondary care, ongoing monitoring, actions to take in case of abnormal results, and advise the GP on when to stop treatment, or consult with specialist.
  7. Evaluate adverse effects reported by GP or patient.
  8. Report adverse events to the MHRA via
  9. Advise GP on review, duration or discontinuation of treatment where necessary.
  10. Inform GP of patients who do not attend clinic appointments.
  11. Inform GP, by letter, of clinic visits and action taken for management of patient.
  12. Ensure that backup advice is available for patient and GP at all times.

The information contained in this guideline is issued on the understanding that it is accurate based on the resources at the time of issue. For further information please refer to the Summary of Product Characteristics or contact a member of the appropriate team

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General Practitioner
  1. Reply to the request for shared care as soon as practicable.
  2. Ensure that the patient understands the nature, effect and potential side effects of the drug before prescribing it as part of the shared care programme and contact the specialist for clarification where appropriate.
  3. Monitor patient’s overall health and well-being.
  4. Monitor adverse effects and possible medicines interactions.
  5. Monitor LFTs monthly for the first 3 months, 3 monthly for 12 months then annually thereafter.
  6. Refer promptly to specialist if the patient develops signs of liver dysfunction.
  7. Prescriberiluzole therapy.
  8. Report any adverse events reported by the patient to the consultant.
  9. Report any adverse events to the MHRA via
  10. Stop, alter or change the treatment on the advice of the specialist.

Patient/Carer
  1. Report any adverse effects to the Consultant Neurologist and/or GP. Seek immediate medical attention if symptoms such as fever occur. Report symptoms such as dry cough or dyspnoea if it develops.
  2. Share any concerns in relation to treatment with riluzole
  3. Be willing to have regular blood tests undertaken.
  4. Report any changes in disease symptoms to the Consultant Neurologist and/or GP.
  5. Report to the Consultant Neurologist, motor neurone disease nurse specialist or GP if they do not have a clear understanding of their treatment.

Clinical Commissioning Group
  1. To provide feedback to trustsvia Trust’s Medicines Committee.
  2. To support GPs to make the decision whether or not to accept clinical responsibility for prescribing.
  3. To support trust in resolving issues that may arise as a result of shared care.

The information contained in this guideline is issued on the understanding that it is accurate based on the resources at the time of issue. For further information please refer to the Summary of Product Characteristics or contact a member of the appropriate team

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SUPPORTING INFORMATION

Licensed indications (relevant to this shared care guideline)

Riluzole is indicated to extend life or the time to mechanical ventilation for patients with amyotrophic lateral sclerosis (ALS).

Dosage and Administration

The licensed dose is 50mg BD. If patients have difficulty in swallowing, the tablets may be crushed and administered via a PEG tube.

Riluzole is not recommended for patients with impaired renal function. (See below for hepatic impairment)

Riluzole is also not recommended for use in paediatric population.

Cautions and Contraindications

Contraindications:

  • Hypersensitivity to the active substances or to any of the excipients.
  • Hepatic disease or baseline transaminases greater than 3 times the upper limit of normal
  • Pregnancy and lactation

Cautions:

Hepatic impairment

Riluzole should be prescribed with care in patients with a history of abnormal liver function, or in patients with slightly elevated serum transaminases (ALT/SGPT; AST/SGOT up to 3 times the upper limit of the normal range (ULN)), bilirubin and/or gamma-glutamyl transferase (GGT) levels. Baseline elevations of several liver function tests (especially elevated bilirubin) should preclude the use of riluzole.

Because of the risk of hepatitis, serum transaminases, including ALT, should be measured before and during therapy with riluzole. ALT should be measured every month during the first 3 months of treatment, every 3 months during the remainder of the first year, and periodically thereafter. ALT levels should be measured more frequently in patients who develop elevated ALT levels.

Riluzole should be discontinued if the ALT levels increase to 5 times the ULN. There is no experience with dose reduction or rechallenge in patients who have developed an increase of ALT to 5 times ULN. Re-administration of riluzole to patients in this situation cannot be recommended.

Neutropenia

Patients should be warned to report any febrile illness to their physicians. The report of a febrile illness should prompt physicians to check white blood cell counts and to discontinue riluzole in case of neutropenia.

Interstitial lung disease

Cases of interstitial lung disease have been reported in patients treated with riluzole, some of them were severe. If respiratory symptoms develop such as dry cough and/or dyspnoea, chest radiography should be performed, and in case of findings suggestive of interstitial lung disease (e.g. bilateral diffuse lung opacities), riluzole should be discontinued immediately. In the majority of the reported cases, symptoms resolved after drug discontinuation and symptomatic treatment.

Riluzole may cause dizziness and somnolence, if affected patients should not drive or operate machinery.

For a full list of cautions and contraindications, refer to the Summary of Product Characteristics.

Clinical Monitoring

Possible adverse events should be reported to the GP and consultant neurologist.

The information contained in this guideline is issued on the understanding that it is accurate based on the resources at the time of issue. For further information please refer to the Summary of Product Characteristics or contact a member of the appropriate team

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Adverse Effects

Very common (≥1/10): Nausea, abnormal liver function tests, asthenia.

Other reported adverse effects are headache, abdominal pain, vomiting, dizziness, tachycardia, somnolence and cicumoral paresthesia.

For a full list of adverse effects, refer to the Summary of Product Characteristics for riluzole

Drug Interactions

There have been no clinical studies to evaluate the interactions of riluzole with other medicinal products.

Medicines which inhibit CYP1A2 (e.g. caffeine, diclofenac, diazepam, amitriptyline and quinolones) or induce CYP 1A2 (e.g. cigarette smoke, charcoal-broiled food, rifampicin and omeprazole) may affect the elimination rate of riluzole.

Pregnancy and Lactation

Riluzole is contra-indicated in pregnancy and breast feeding. Clinical experience is lacking in pregnant women. It is not known whether riluzole is excreted in breast milk.

Contact Details

University College Hospitals NHS Foundation Trust
Consultants:
  • Dr Robin Howard
  • Dr Richard Orrell
  • Dr Katie Sidle
  • Dr Pietra Fratta
Motor Neurone Disease Nurse specialist / Consultants’ secretary:
Tel: 0203 448 3517
References
  1. Summary of Product Characteristics for Rilutek® accessed via Date of last revision of text 6th January 2014
  2. BNF April 2014 accessed via the trust intranet.
  3. NICE TA no 20. Guidance on the use of riluzole for the treatment of motor neurone disease.

This document represents only a brief summary and is as accurate as possible. Always refer to the Motor Neurone Disease team at the National Hospital for Neurology and Neurosurgery (NHNN) for further information.

Shared Care Guideline updated by: Sheetal Sumaria, Senior Clinical Pharmacist - NHNN/UCLH April 2014
Update agreed with lead commissioning CCG: Camden Clinical Commissioning Group: 25 June 2014
Ratified by North Central London Medicines Optimisation Network: 02 December 2014
Date of next review: April 2017

The information contained in this guideline is issued on the understanding that it is accurate based on the resources at the time of issue. For further information please refer to the Summary of Product Characteristics or contact a member of the appropriate team

Riluzole SCG – ALS – MND in adults - 2014 – Review date April 2017Page 1 of 4