Infusion Day visit with Dr Joanna Nightingale, Head of Scientific affairs BDFA
On Tuesday 4th June 2024 I had the privilege of watching a Brineura infusion at Birmingham Children’s Hospital.
The day started early for everyone. Both families receiving treatment live about an hour’s journey away and arrived before 9am. Suzie Haworth, the nurse in charge, met me at 8:30 and took me to the ward where the infusions took place. There I met Isaac, with mum Aimee, and Florence, with mum Phoebe and Kirsty Darling, another nurse in the team. Other members of the team include nurses Louise Simmons, Rachel Gould and Louise Ball and the pharmacist Siema Akram.
Both children are used to the routine of coming to Birmingham. Isaac was finishing breakfast while Florence was settling in and showing everyone Peppa Pig on her iPad.
The first action after checking in is the observations: blood pressure, heart rate, O2 sats, respiration and temperature. These check that the children are fit and well for infusion and are repeated hourly throughout the day. Once confirmed that everything is ok, Suzie and Kirsty located the device under the skin of the scalp and applied numbing cream to area. Siema was then able to retrieve the vials of Brineura from the freezer and deliver them to the ward.
Brineura is delivered to the cerebrospinal fluid (CSF) by the intracerebroventricular route (directly into the ventricles of the brain) through a surgically implanted reservoir and catheter (intraventricular access device).
To deliver Brineura, the area around the device was cleaned thoroughly to maintain sterility before the device was accessed by inserting a port needle. A small sample of CSF was removed using an empty, sterile syringe and sent for infection monitoring. The infusion set was then attached to the port needle and secured with sticky tape.
In Birmingham the port is covered with a square of sterile gauze secured with orange hair clips! For children who are inclined to remove their access device, a net cap can be used.
Brineura is packaged in vials containing cerliponase alfa 150mg/5ml and children over the age of 2 receive 300mg per infusion. The drug is infused at a rate of 2.5ml per hour. The main part of infusion took place over a period of 4 hours and was a full on Peppa Pig session. Every hour the observations were complete by the nursing team, and they also got their lunch delivered.
Once all of the Brineura in the syringe was delivered, there was still some in the tubing that leads into the port needle. This was delivered by using a flushing solution. The amount used depends on the length of the tubing. It uses the same rate of delivery and took about an hour in Birmingham.
Once complete, de-access took place. The nurses removed the gauze and used a special liquid which “de-stickies” plasters allowing easy removal of the tape that holds the tubing in place. After removal of the port needle the area was sprayed with Opsite, a permeable spray dressing that seals the site and prevents infection.
After de-access was complete the children were observed for a further 30-60 minutes. If there were no signs of an adverse reaction out the children were then able to go home. This is usually about 4pm making infusion a whole day process. The nursing team completed their care by ensuring all material is disposed of safely and that there were no issues from the sampling of CSF from the morning. They also made sure that all notes have been completed and filed correctly.
In addition to their role in infusion, the team at Birmingham can arrange for other medical personal at the hospital to drop by if needed. The children saw neurologists and dieticians while on the ward, reducing the need at attend another appointment. This team can prescribe additional medication where required, write letters for support in the community, offer moral support to parents who reported feeling they were in a safe environment for the day.
It was so good to see infusion day in its entirety. The team in Birmingham made me feel as welcome as the children and filled me in on all aspects of the day. The importance of having access to the regional infusion sites is clear as it has made a positive impact on our families and reduced the stress of dealing with this disease.