Hope Love Cure

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 πŸ™ŠπŸ’œx I do apologise I really must update a little more often πŸ™Šx 

 

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 πŸ™ŒπŸ» 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 πŸ’œx

 

and as one gorgeous little girl would say ~ If plan A fails, there are 25 more letters in the alphabet πŸ’ž 

Time has just flown and we find ourselves only now settling back into routine and home, suitcases remain half packed from our back and forth travels to Melbourne, although appointments are finally starting to distance a little. I’m so sorry we’ve been MIA these past few months during Pipers recovery, our amazing little girl has had some rotten days, and at times I wondered just how I could have put her through something so traumatic, we've had some difficulty with pain management, and as Piper tried to move her legs the muscles would spasm causing a great deal of discomfort, nappy changes and transfers just broke our heart, and seizures would again cause involuntary movement to Pipers legs causing further discomfort, frustration and pain. Piper appeared to be healing beautifully, then suddenly became increasingly uncomfortable, we later discovered the exceptional healing and new bone growth may have been responsible for this, as the ball and socket reconnect with each other the friction begins to create further inflammation. I felt so relieved to view the final x-ray, to actually see Pipers bilateral hip dislocation, how the hips had deformed over time, understanding there was certainly no other option. still I wanted to ask “Do we really have to do this?” as Piper was wheeled into the operating theatre, her smiley little face staring back at me. still I'm unable to comprehend the fact that Pipers femur bones were cut through to create the required angle. It just amazes me! The extraordinary work our surgeons do. I guess all’s well that ends well :) and things are finally looking wonderful once more. Our care team are simply amazing from hospital to school! Piper has achieved two full weeks back at school (our record!!) and has made some incredible progress, enjoying hydrotherapy, even returning to her standing frame! I have so much admiration, praise and thanks for the place which brings to Piper so much joy, stimulation, therapy and love. Piper is starting to move onto her side for play, which is magical to see, banging her hands and kicking her legs! We’re finally back on track! and just watch this space! Because anything is possible! Xxx A photograph .. or a few ;) tells a thousand words! - 💜xx pipers past months of moments missed 💜x

Some dreams are worth holding on to..... 

The long road to a beautiful blessing  

 

For so long Piper was known amongst her many doctors as the little complex girl with so much going on, the little girl they'd rather keep comfortable than make uncomfortable with surgeries, we felt deflated and defeated with each hospital visit for we thought surely if the seizures would just go away, surely piper would then go ahead in leaps and bounds, I guess that was our wake up, our realization came with the explanation of the syndrome (what ever that may be), that itself is what's taking piper away, not the seizures, the syndrome is what causes Piper to deteriorate  

Most children like piper will not see their tenth birthday 

I hear most! I understand I'm in a complete state of denial and I don't care, if I don't believe in my little girl then who will, we also now understand Piper's epilepsy is merely a symptom due to an underlying cause. A hidden disease which no one can find is trying to take our baby away. What we don't understand is how the world could be so incredibly cruel 

We have the best in the business, their words at times may come across a little harsh, our reaction and response is mostly driven by fear, we know our doctors are doing all that they can, we know piper is a very complex little puzzle.

 

The next words to leave us numb come with the suggestion that my husband and I may perhaps like to consider having another child, to ensure our eldest child a sibling to grow up with, the risk of SUDEP (sudden unexpected death in epilepsy) much higher in a child like piper, We try to process what is being said... I could not  imagine how I would offer this type of advice to a parent should the shoe be on the other foot… It was suggested so gently yet I could not help feel overwhelmed with grief, a little anger, guarded I guess, did they really expect me to replace one child with another. My husband and I walked out of this appointment silently not daring to look at the expression of the other, perhaps because we'd be overcome with emotion, perhaps because we were each processing the conversation in our own way.  

Why does everyone think she's likely to die! Why is everyone just giving up! Of course this being far from the case, yet emotions so raw that day could no longer see the sense in any conversation. 

 

As for another child we had resigned to the fact that decision had been stolen from us, as much as we would love for another.

 I felt frustrated, how can they tell me that! I would not change my little girl for the world, I love every little piece of our precious miracle, however I could not knowingly allow another human being to endure the poking, prodding, pain, testing and tears that piper has had to.

 As time went by we were able to fully appreciate the conversations brought to us, yet we were also able to witness a little girl proving her medical world wrong, defying odds, gaining small yet great milestones along the way, as we relished in each moment with our beautiful girls we continued to dance with the idea of another child 

 Two years later we discuss our concerns with a genetic counsellor he explains our situation,

Pipers DNA has been sent across the world studied and tested for countless conditions, to this day countless common and rare conditions have been eliminated.

In addition to this Michael, Piper and I underwent a trio of  genome testing, a process of elimination comparing our genes against each other to assist in the complex puzzle that is our little girl.

Our geneticist explains although the aim of Pipers years of testing is to establish diagnosis some comfort may be found knowing the alteration is not from mum or dad. 

the fact that we are still to discover this rare condition tells us the condition is likely de novo meaning an alteration in pipers individual make up, so the comfort lies in knowing future pregnancies are quite safe, our chances we are told are that of any other couple starting out, we are thankful, shocked and excited by what this news could bring 

I feel elated for a decision that felt stolen from us is ours again, the possibility is there should we decide to complete our little family with the third child we'd always longed for.

we feel an overwhelming relief a deep appreciation and then realize there's nothing left except to make the decision, the decision to begin the next beautiful chapter. 

 

We are delighted to announce our family of four, will soon become a family of five, including a gorgeous, strong little girl who continues to amaze us all, an incredible, caring beautiful big sister and a little blessing.

Another precious child to cherish and love.

 

 

Which Willow just can not stop thanking us for 😊❀️ We simply can not wait to see Piper respond, develop and grow along side her new little sibling πŸ’™πŸ’– 

 

Latest comments

22.05 | 13:21

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07.02 | 09:55

Oh Summer, you are doing an amazing job with Pipes & all of your family. Keep at it. The same goes for you too Piper. Loads of love from over here. Rikki. Xxx

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06.02 | 05:38

Piper is one tough little cookie, I have no doubt at all that she’ll continue to grow and surprise us all. Love you Princess πŸ‘‘ Piper. πŸ’œ

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13.10 | 11:42

Love yo piper to the moon and back

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