Difficult discussions

Today was a real down to earth with a bump day from the excitment of the FLM. I took William to see his local paediatrician this afternoon. These appointments are more about me telling him what I am concerned about and asking him of his ideas about William in general. The specialist consultants sort out the specifics of his treatment. I was hoping to get to the bottom of what is causing abnormal patches on William's chest x-ray but got the usual, no-one really knows, kind of response. I told him I am concerned about the fact that William has less energy than usual these days. It may just be that he has had so many infections lately or it may be that there is some chronic infection that we have not yet identified. The difficult thing is that it may be that the degenerative aspect of William's syndrome progresses more quickly when he is unwell. Unfortunately, this is most likely as his examination showed that his reflexes are less brisk and his foot deformities and associated high tone in his tendons have worsened.

Everyone is waiting on his bowel transplant assessment. He can't go on getting poorly with infections if this is causing general deterioration. There is also a very real danger of running out of sites for his hickman line if he carries on getting so many line infections. I hope that they will accept him for transplant and that, perhaps, he may even go on the list straight away as, although his liver is OK at the moment, his lungs are being damaged by the bugs in his gut and there is a very real threat to his long term feeding if we run out of line sites. After the appointment, I had a meeting with William's community nurse and a nurse from the hospice to discuss what we want for his end of life care, should this be needed. We all hope this won't be for a very long time but plans need to be made while we are thinking straight. It all needs to be right and it is the last thing we would be able to do for him. In many ways, we are lucky because we know that there is a chance William will die in childhood. We can make plans to ensure, if this happens, it happens in the best possible way for William and the family. We now have a plan about getting William to the hospice if we reach an end of life situation. Once there, they would help and guide us through everything and we can stay there as a family until it is time to say a final goodbye. I know Hope and Ellie read this blog so will stress again, there is no reason to think this plan is needed in the near future. It is just in case. William is on good form at the moment and has just finished his latest course of IV antibiotics. Fingers crossed, he will stay well for a good long time now.