Janine Furmedge is the Haemophilia Nurse Coordinator at the Royal Children’s Hospital, Melbourne
Prophylaxis with factor concentrates has been, without doubt, a great advance in the management of severe haemophilia and is well established in developed countries such as Australia. However starting regular factor infusions in infants and young children brings many challenges; finding veins is often difficult and upsetting for all involved and the thought of home treatment is a daunting prospect for most parents.
Infusaports (or ‘ports’) are temporary devices that are surgically implanted to allow easy access to a central vein. Ports have a reservoir that is inserted under the skin, usually on the chest. The reservoir connects to tubing (a catheter) that is tunnelled under the skin and feeds into a large vein in the neck. A special needle is inserted through the skin and into the port reservoir and the injected factor travels along the catheter and into the bloodstream.
By establishing reliable vein access, ports make home treatment feasible in small children. But while ports bring considerable benefits, they are not without risk. Infection is the most common complication and parents are provided with comprehensive education so they learn how to administer factor safely and with a technique that minimises the risk of infection. Parents make a decision with the staff at their Haemophilia Centre if and when a port is appropriate for their child. And for children who have a port, the next step is to transition to peripheral veins once the veins further develop.
The Haemophilia Centre at The Royal Children’s Hospital Melbourne wanted to develop a formal port education package. As the first step in the process we carried out a study to explore the experiences and education needs of parents who had learnt to use a port to administer factor to their child.
Firstly, most parents were learning to use the port at a time when they were still dealing with the distress of the diagnosis of haemophilia in their child as well as beginning to experience bleeds, trips to the emergency department and treatment. So a very difficult time!
There were four main themes that emerged from the study focus groups:
Most parents told us they were very anxious when accessing their child’s port for the first time. They described ‘shaking’, ‘sweating’ and feeling ‘nervous’. Many felt very emotional about putting a needle into their child and fearful of hurting them. There was enormous anticipation leading up to the first time they accessed their child’s port but once it was over there was great relief and the feeling that in fact it wasn’t so bad and much easier than expected.
Many parents described the relationship with and the support received from the person teaching port access as one of the most important aspects during their learning. It was important that the teacher could foster confidence in parents during this difficult time and that both parent and child felt comfortable with and had trust in their teacher. Respecting the needs and wishes of the child was significant, as by ‘making my child feel comfortable you [the parent] are automatically in a different place’.
Interestingly, many parents described the importance of having a routine or ritual around how the port was accessed. This helped both parent and child to feel comfortable and confident about the procedure. Children responded well to having some control and the ritual also assisted parents in their learning process. Parents described practices such as their child always sitting in the same place and position, always inserting the needle on the count of three and always setting out equipment in the same way.
Parents described taking over their child’s treatment as empowering. After a life dominated by the uncertainty of bleeding episodes and urgent visits to the Emergency Department for treatment parents could now take control; ‘feeling in control after feeling so out of control … I think that’s the biggest stand out about ports’. Ports were seen as a ‘blessing’, allowing most children to participate in all kinds of activities including sports and school camp.
Parents asked that a teaching package incorporate photos, checklists and ‘step by step’ instructions. They also expressed the desire to hear the voices and experiences of other families and so we included stories, anecdotes and tips from other parents. In addition, consistency of teaching was identified as very important and so a checklist and information directed to nurse teachers was included as a mechanism to address this issue, especially for when several nurses are involved in the teaching process. The education package was developed with the help of parents who were experts in their child’s port access as well as those who were learning for the first time.
The study helped us to have a greater understanding of what it is like for parents learning to give factor to their child via a port and to develop an education package that we hope will address parents’ needs. The ability to give treatment at home was liberating for families but also meant less connection to the hospital and other families. Parents told us the enormous value they found in sharing their experiences and stories. This highlights the importance and great work of our Haemophilia Foundations in providing strong support and opportunity for children, families and adults with bleeding disorders to connect with each other.
If you have any questions about your child and ports, please contact your Haemophilia Treatment Centre.
A special thank you to the families who generously gave their time to share their experiences of learning how to use their child’s port, to write their stories, to be photographed and to review and pilot the port guide. Development and publication of the port guide was supported by a Bayer Haemophilia Caregiver Award. Thank you also to the Educational Resource Centre at the Royal Children’s Hospital, Melbourne for their expert photography and design.
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