We know that Pipers signals are scrambled the messages her brain sends are not telling her body how to behave, Pipers muscles are weak, her tone is quite poor, this is an area where we see incredible gains with Pipes lifting her head to fix and follow,
yet we also see the weight of pipes heavy little head left to withhold a slumped position, it’s evident Pipes needs extra support now Pipers muscles have deteriorated, her body now flops and folds forward if she if left unharnessed in her wheelchair,
unable to withhold that upright seated position with the lateral supports of her chair.
Pipers precious little ballet feet ~ as we like to call them ~ with toes always pointing down have over time formed quite a different position,
so turned rolled are Pipers feet the ankle would now bare the weight of support should she be in an upright standing position.
Piper will continue to use her AFO’s although may require Botox injections to ease muscles, to encourage
and enable the socialization and therapeutic benefits of a standing frame
It seems quite strange, so difficult to understand that Pipers body is so low in tone yet legs are so tight when trying to place into an extended position.
Pipers respiration has become of concern, her recent illness and infection brought a number of things to our attention. We try so hard to keep Piper at home and most situations we are able to handle, however every once in a while there’s something
new, something that’s not our normal we then have little choice but to seek further medical support and aid, in this case iv antibiotics and the assistance of high flow, as Piper has a poor swallow and breathing is laboured there is concern Pipes may
suffer sleep apnea and I guess this may make sense, as Piper can quite happily sleep her days away We just put this down to the exhaustion of over night seizures, the same applies to snoring, at night I lie with my hand on Pipers chest waiting for the rise
and fall or listening for the next breath, again the irregular patterns I put down to seizure recovery.
The above is a draft from from several months ago, I thought it best to include to give a little insight and understanding
into our progress x this may be the longest post in history 🙊💜x I do apologise I really must update a little more often 🙊x
Heading for home what a busy yet lovely time we’ve had with our gorgeous Melbourne family. Pipers
appointments went quite well it was reassuring to know Pipes hips have healed beautifully, the bone growth is perfect 🙌🏻 I think we scored an A+ for outstanding recovery and placement, on the other hand our precious ballet feet still seem to be misbehaving,
Botox no longer an option as there is concern this form of treatment could result in another trip to the intensive care unit for Piper, there is a risk of Botox traveling from the injection site through the body, potentially this could travel to Pipers
chest and airway, causing Pipers muscles to relax further.
The options ~ to leave the feet to continue as they are or allow Piper a surgery to lengthen muscle, allowing the foot and ankle the chance to extend and reach the required position, which enables
Piper to use her AFO’s, to wear shoes, to comfortably stand in a standing frame, to promote the beautiful thing that see Piper so happy and engaged.
We are told in comparison to Pipers hips this procedure is quite small, our surgeon will wait
until after winter ensuring our girl remains healthy and strong for surgery
Pipes spent several days in hospital for video EEG and respiratory monitoring in the hope to witness brain activity as seizures occur, thankfully Piper
displayed different seizure types whilst on monitors, although nothing overly spectacular as far as seizure presentation is concerned, I would have liked for the EEG to have captured the full extent of a seizure, the kind that grabs every muscle
in her body and then just as harshly releases. ~ “if ever there’s a time Pipes it’s now!” I thought to myself bizarrely wishing for another seizure to occur. Due to the minimal activity while the electrodes were in place I had
feared the EEG would not give as much information as Pipers neurologist required, yet there was far more activity than even I was aware.
During the night I would wake to Piper puffing out secretions I assume a small seizure had occurred,
this was actually the seizures end, a bigger more silent seizure, from the data collected we now know it’s during these moments Piper stops breathing.
As Pipers form of epilepsy is not treatable with surgery we are investigating the possibility
of implanting a Vagus Nerve Stimulator, much like a pacemaker for the brain.
A small device is implanted into the chest, a lead attached to the Vagus nerve (located in the neck) as small intermittent impulses send electrical signals to the brain,
the Vagus nerve also controls the muscles involved in swallow, as well as those that control respiration, the trouble with this is Pipers muscles are already weak, Piper has a poor swallow, saliva pools to the back of her throat, the muscles are not strong
enough to fully protect the airway, this allows secretions to trickle down causing the reaction of choking, coughing and eventually vomiting and/or aspiration.
The difficulty for Piper with VNS is for those twenty seconds of electrical impulse
muscles/airways will relax, the VNS may help to settle the severity, the quantity of seizures, yet it may also put Piper at further risk, there is much to consider.
Lucky for us we have a fabulous team of specialists, together we
will find the best way forward and continue to allow a beautiful happy little girl every chance to shine 💜x
and as one gorgeous little girl would say ~ If plan A fails, there are 25 more letters in the alphabet 💞