Sensitive grounds

I was in a situation a couple of weeks ago with a patient who had no idea of the severity of the condition her child had. I was on my paediatric placement and the baby had been referred for plagiocephaly, but also for severe gross motor delay. These weren’t her main problems unfortunately. The child had a diagnosis of Wolf-Hirschhorn syndrome. Both my supervisor and I were not familiar with this condition. The syndrome is caused by a chromosomal abnormality. The common characteristics are; severe to profound mental retardation, microcephaly, seizures, heart defects, hypertonia, cranial asymmetry and various other facial abnormalities. There is an estimated mortality rate of 34% in the first 2 years, but this rate is underestimated due to many affected children dying without being diagnosed. It is a rather serious condition.

The mother of the child is of foreign decent. She does not speak very good English. The father is Australian, however, works during the week and is finds it difficult to get time off work for his child’s medical appointments. The mother usually goes to the appointments and relays what she gathered from them to her husband. The child has been seen by various allied health staff and the diagnosis has been explained to the parents.

However, I am not sure the diagnosis has been explained well enough to the parents. Either that or the mother is in a bit of denial about the severity of her child’s condition. She came to the initial assessment with her baby and had information written on a sheet that mentioned some of her problems, such as heart defect, microcephaly, poor brain development, low muscle tone, and several other things. The mother seemed aware that her baby had these things, but was surprised to hear that her gross motor development was extremely delayed.

This baby was around 3 months old, and my supervisor said that a newborn baby has more tone than this baby had. The child had pretty much no control of its head, poor social interaction – would not focus vision at all throughout the session, extremely low tone, and severe delay in gross motor skills. The prognosis is very poor.

The mother had the impression that her baby was just small, and this was relatively normal and she would catch up with her development soon. I don’t think she had any idea of the severity of the syndrome and that if her baby survives the next few years, she will be severely impaired.

It was very difficult to try to inform the mother that the child has a very poor prognosis and will never be what she sees as normal. We tried to get her to bring her husband the following session two weeks later but he was unable to attend. We informed her of a few things regarding her baby’s condition throughout both sessions, yet it still seemed as though she was not aware of the impact that these would have on her child’s life.

I was very hard trying to inform someone that their baby had such a terrible prognosis. I think the mother may have been in a bit of denial regarding the diagnosis and that may have been affecting her views. I’m not sure how well it was explained to her previously, but I feel as though eventually she did come to terms from a gross motor perspective that her baby would not be very good.

I hope I am not in a similar situation many times again as it was very depressing and difficult to deal with, however, as hard as it may be for me or someone else to tell a parent of a condition like this, it is no where near as hard as hearing it. I do feel now that if you have to deliver bad news if you go about it in a sensitive way, parents (or patients), while obviously depressed are still grateful that you spent the time to discuss with them and help with any questions they may have had.