The purpose of this document is to provide guidance for the administration of non-cytotoxic injection or infusion via an Intracerebroventricular (ICV) reservoir at Great Ormond Street Hospital (GOSH).
This guideline is to be used for the administration of cerliponase alfa/BMN190 and may need to be adapted for administration of other ICV medications.
For intrathecal or ICV administration of cytotoxic drugs please see the clinical guideline; Intrathecal cytotoxic chemotherapy: administration via a lumbar puncture or Ommaya reservoir.
This document must be read in conjunction with the GOSH Operational Policy for the Administration of Intracerebroventricular Non-Cytotoxic Injection or Infusion (GOSH 2016).
There are designated areas in the hospital for the administration of intracerebroventricular non-cytotoxic injection or infusion stated in the corresponding operational policy.
These areas are:
- Clinical Research Facility
Rainforest and PICU may be used as an alternative providing personnel on the corresponding register are completing the procedure.
Intracerebroventricular medication must only be administered by a suitably trained and authorised nurse or doctor (Rationale 1). The GOSH non-cytotoxic Intracerebroventricular register names all staff involved in the administration of Intracerebroventricular drug therapy.
It is the responsibility of the individuals on the GOSH non-cytotoxic Intracerebroventricular register to ensure that any colleagues they involve in the process are on the register for the task in question.
All staff should receive training in the risks associated with the administration of medication via an ICV device appropriate to their level of involvement in the prescribing, verification, handling, preparation and administration.
Risks associated with ICV administration of Cerliponase Alfa include:
- Hypersensitivity Reactions
- CNS infection
The small volumes of medication administered for the protocol for cerliponase alfa/BMN190 are not felt by the neurosurgeons, to pose a risk of raised intracranial pressure.
Preparation of equipment
- Programme protocol
- Patient’s medical notes
- Sterile gown
- Sterile gloves
- Sterile gauze
- Sterile dressing pack
- 3 x 3ml ChloraPrep®
- 5/8 Inch 22-26G non-coring Gripper needle
- 2 x needle free access device (e.g. microclave®)
- Infusion line with pressure monitor
- Intrapur Paed 0.2 micron filter
- 2 x 2ml luerlock syringe
- 10ml luerlock syringe
- Adhesive strips (e.g. Steristrips®)
- Protective pot (e.g. Posey ®)
- Surgifix® size 5.5
- Op-site® spray
Prior to accessing the ICV device
ICV medication must only be administered by a suitably trained doctor or nurse (rationale 1).
Ensure written consent is in place and the family are aware of the potential risks and side effects of the treatment (rationale 3).
The ward doctor must review the patient and document in the clinical notes that they are fit for the procedure (rationale 1).
Ensure adrenaline (1:1000) is available (rationale 5).
Assess the reservoir site and apply local anaesthesia, such as Emla® or Ametop® over the device site (rationale 6).
Signs of ICV reservoir leakage (swelling of skin around reservoir site, erythema of the scalp, bulging of reservoir device, or extravasation of fluid) will prompt further evaluation of the reservoir for failure prior to continuing with the infusion. Additional surgical consultation including surgery may be required to fix or replace the device (Biomarin 2014).
Check medication in line with the medicine administration policy (rationale 2) prior to accessing the device.
Accessing the ICV device
The doctor or nurse performing the procedure must be deemed competent to access the reservoir (rationale 1). This is a sterile procedure and should always be performed with two people (rationale 11). Do not attempt to access the device more than 3 times (rationale 11).
Position the child comfortably on the bed (rationale 10).
Open sterile dressing pack and place all items on sterile field (Rationale 11).
Put on sterile gown and sterile gloves (rationale 9).
Connect together the infusion set on the sterile field as required and manually prime the line.
Ensure the skin is visibly clean.
Clean the site using 3 x 3ml ChloraPrep®* sticks. Gently press the applicator against the skin and then apply the antiseptic using firm repeated up and down, then back and forth strokes for 30 seconds over the device siteand allow to dry (rationale 11).
*If the child is allergic to ChloraPrep®, Betadine with alcohol is an acceptable alternative. If the child is allergic to Bedatine with alcohol, please phone the infection prevention and control team for advice.
Place sterile sheet around ventricular device (rationale 11).
Locate the reservoir by slightly depressing the dome several times.
Using the Gripper needle, access the reservoir at a 90 degree angle.
If a child is unlikely to be highly mobile throughout the procedure/infusion it may be possible to complete the injection/infusion with the needle in the dome of the device. If the child will not tolerate this or is expected to be mobile, the needle must be secured within the small hole in the centre of the device using steristips/posey/surgifix .
Check the colour and transparency of the CSF. If it is not clear in colour or contains debris, contact neurosurgical team for advice. Do not proceed with the infusion (rationale 23).
Difficulty in obtaining the required volume of CSF required for pre-infusion will prompt further evaluation of the reservoir by the neurosurgical team prior to continuing with the infusion.
Administer the medication as per drug prescription and drug specific guidelines (rationale 10, rationale 14).
Secure the needle as required using steristips/posey/surgifix or similar whilst ensuring the device site is visible throughout the infusion (rationale 15).
During the Infusion
The child should be on continuous heart rate monitoring and have hourly blood pressure readings throughout the infusion (rationale 20). These should be recorded in the child’s health care record.
If the infusion set becomes disconnected during the infusion do not reconnect (rationale 11).
If the medication needs flushing, ensure the infusion set is clamped, change the syringe containing medication to the syringe containing the flush and continue the infusion at the same rate.
If the infusion pressures go above 20, stop the infusion and check for kinks in the line. If this does not resolve the problem, stop the infusion (rationale 21, rationale 22).
De-accessing the device
Remove the protective pot and adhesive strips from the head.
Remove the needle.
Apply pressure with sterile gauze pad. When CSF stops leaking/site stops bleeding spray with Op-site spray® (rationale 17, rationale 11).
If CSF continues to leak, apply a small dressing with gauze and tape. This dressing must be removed within 24 hours post the procedure, or as soon as the pressure dressing is no longer required (rationale 17).
Clear away equipment according to hospital waste management policy (rationale 18).
The doctor/nurse and the nurse must sign for administration of medication on the patient's prescription chart after administration is completed (rationale 10).
The doctor/nurse will document procedure in patient’s health care record. (rationale 19).
Post procedural care
The nurse caring for the patient should monitor the patient for side effects of the medication, and record these in the nursing notes (rationale 19). The nurse must also remind the parents to be aware of potential side effects (rationale 19).
Give analgesia and antipyretics as required (rationale 10).
Complete observations as required (rationale 20)
Rationale 2: To adhere to NMC Standards for medicines management (NMC 2008) and Trust policy (Medicines Administration Policy 2014).
Rationale 3: To adhere to NMC Code (NMC 2015).
Rationale 4: To obtain a comparative baseline which will allow monitoring of changes.
Rationale 5: To ensure patient safety due to the risk of anaphylaxis associated with enzyme replacement therapies (Bailey 2008).
Rationale 6: To minimize the risk of pain during the procedure.
Rationale 7: To ensure the child stays still during the procedure.
Rationale 8: To minimize the risk of pyrexia post procedure.
Rationale 9: To reduce the risk of cross infection and adhere to standard precautions.
Rationale 10: To facilitate comfort.
Rationale 11: To minimize the risk of infection. Allowing any cleaning solution to dry is vital in order for disinfection to be completed.
Rationale 12: To avoid analyzing CSF located within the device and ensure you are in the device.
Rationale 13: To monitor cell count, glucose and protein for safety.
Rationale 14: To adhere to trust guidelines.
Rationale 15: To ensure the infusion site can be monitored for signs of needle displacement.
Rationale 17: To ensure that there is no further leakage of CSF.
Rationale 18: To maintain a safe environment.
Rationale 19: To adhere to the NMC code of conduct (NMC 2015)
Rationale 20: To monitor for any signs of deterioration or any signs of a neurological problem.
Rationale 21: Normal Intracranial pressure should be 1-20 mmHg (Chesnut, Temkin , Carney et al 2013).
Rationale 22: To alert nursing staff to a problem with the infusion line, needle or device.
Rationale 23: To monitor for potential complications, such as infection or bleeding.
Bailey, L., (2008) "An Overview of Enzyme Replacement Therapy for Lysosomal Storage Diseases" OJIN: The Online Journal of Issues in Nursing Vol. 13, No. 1, Manuscript 3.
Biomarin (2014) Pharmacy Manual. A multicentre, multinational, extension study to evaluate the long term efficacy and safety of BMN190 in patients with CLN2 disease.
Chesnut RM, Temkin N, Carney N, Dikmen S, Rondina C, Videtta W, Petroni G, Lujan S, Pridgeon J, Barber J, Machamer J, Chaddock K, Celix JM, Cherner M, Hendrix T. (2013) A trial of intracranial-pressure monitoring in traumatic brain injury. New England Journal of Medicine. PMID: 23234472 [last accessed on 05.01.2017]
Great Ormond Street Hospital for Children NHS Trust (2014) Medicines Administration Policy, Great Ormond Street Hospital, London.
Great Ormond Street Hospital for Children NHS Trust (2016) Operational Policy for the Administration of Intracerebroventricular Non-Cytotoxic Injection or Infusion, London.
Nursing and Midwifery Council (NMC) (2008) Standards for the medicines management. London, NMC
Nursing and Midwifery Council (NMC) (2015) The Code, Professional standards of practice and behaviour for nurses and Midwives. London, NMC