wolfhirschhorn.org

From the first time we heard Mia’s diagnosis and read through the seemingly endless list of possible complications that Wolf Hirschhorn can present, the one thing that made my blood run cold with fear was the thought of having to witness my beautiful baby daughter having a seizure.

On Sunday evening I fed Mia her dinner, took her upstairs for a bath and it was only when I was drying her that I noticed she wasn’t quite herself. It started fairly subtly, in that her head was turned to the right and her eyes were also fixed in that direction. I tried to get her attention but she seemed distant, so I took her downstairs and woke Vicki, who was dozing on the couch, to see what she thought. Although Vicki has a medical background she understandably finds it very difficult to be objective when it comes to Mia, so we agreed that we would try giving her her bedtime bottle and see how she was after that. It was then that Mia vomited and it became obvious her condition was deteriorating. Vicki stripped her down and got her in the recovery position while I called for an ambulance. The next 10-15 minutes went past in a slow-motion blur, with me relaying messages to and from the operator and Vicki doing a great job of keeping Mia’s airways clear and making sure she was breathing.

The paramedics arrived and got Mia and Vicki into the ambulance, ready for a fast ride to Yorkhill Children’s Hospital, which is about 8 miles from where we live. I set off in the car before them and will never forget the helpless terror I felt seeing the ambulance screaming past me with lights and sirens going, knowing my baby girl was in the back. When I arrived at Accident & Emergency Vicki came out and took me through to the resuscitation ward where Mia was on a bed with six or seven medics round her. In the ambulance her oxygen saturation had dropped dangerously low and, possibly as a result of the Midazolam she was given to control the seizure on arrival at the hospital, her respiratory effort had now fallen to the extent that she had been ‘bagged and masked’ – she was also wired for ECG, oxygen saturation, nasal oxygen and had a cannula in her left ankle to administer any further drugs and fluids. All in all it was a pretty terrifying scene to walk in on and we were asked to sit in an adjoining waiting room, where a nurse brought us tea and the consultant gave us reassuring updates on Mia’s progress. This was the scariest part of the whole experience for us both, every scenario plays through like a movie in your head, including the worst possible outcome.

Thankfully Mia stabilised fairly quickly and we were invited back through to the ward, where we were encouraged to hold and comfort her, while the nurses and doctors did what they needed to around us. By this point over an hour had passed since the whole episode started and, although she was coming out of the seizure state, Mia was still struggling with breathing and the doctor suggested taking her to Intensive Care so she could be intubated. Thankfully just the threat of this seemed to be enough to spur Mia into increasing her efforts to the extent that she was taken off the oxygen and we were moved along the corridor to a quieter, single-bedded recovery room.

We waited in this second room for just over two hours with a nurse coming in every half hour or so to check Mia’s heart rate, pupil dilation, blood pressure and oxygen saturation. It was getting pretty late, we were exhausted and I think the three of us fell asleep at one point, but when Mia woke she was unusually unsettled and cried like I’ve never heard her cry before, probably due to a combination of the medication she had been given and the post-seizure acidosis, which is something the doctor told us about. Shortly after midnight we were moved upstairs to a third ward for observation where there was fold-down parent bed for Vicki to sleep on. By this point Mia was starting to look as if she might be hungry, so we offered her a bottle and she wolfed the whole 6 oz down in double time. She was also starting to act more like herself again which was so reassuring for Vicki and me to see, especially given the length of the seizure she had endured. I left the girls at about 2am and managed a couple of hours pretty restless sleep.

The following day Mia’s strength continued to return, although she slept more than usual and it was obvious she was drained by the whole affair. As it was her first episode it was deemed that she should stay in for another night to be on the safe side. The next day the epilepsy neurologist came to discuss a management plan and we settled on a low dose (0.5 ml twice per day) of Keppra to start off, with an emergency supply of Midazolam to be given in a tiny quantity (0.1ml) after 5 minutes of seizure activity if and when it happens again – both in oral solution. He reckoned what Mia had experienced was a localised (or partial/focal) seizure, which would explain why her head was stuck looking to one side for much of the time.

And that brings us up to yesterday afternoon, at which point Mia was letting us know she was ready to come home and generally getting pretty cranky at being stuck in hospital. So it was with much relief all round that we packed up her bits and pieces and headed home. While I dearly hope that we never have to go through another event such as the one we have just experienced, having a clearer understanding of what to look out for and how, if it does happen, to manage it we are certainly better prepared than we were this time a few days ago. And, most importantly, Mia does not seem to be any the worse for her ordeal.