Why Waddle: (by Paul Rainey)

 

The ‘Waddle’ concept came through my personal experience as a parent; its simple aim is to raise awareness of the daily challenges children with Spina Bifida and Hydrocephalus face.  Last year our core team of 6 walked and paddled the 100 mile length of the River Bann over the course of 3 days at the beginning of June 2010. In 2011 we have extended our team to include Robert Ryan and John Harrower whilst also changing our day one route to follow the Newry Canal from Carlingford Lough; therefore traveling coast to coast across Northern Ireland. The aim of Waddle is to  fund-raise for the life changing work of the Royal Victoria Hospital for Sick Children, and the support services provided by the Association of Spina Bifida and Hydrocephallus (ASBAH).

We have been fortunate to receive help and support from a wide variety of channels and special thanks goes to Janet Gray MBE, the serving patron of Waddle.

You can find out a lot more about Waddle at www.thewaddle.co.uk, or you can read my story below outlining the inspiration behind Waddle and my experience as a parent of a child with Spina Bifida and Hydrocephalus.

 

 

Zoe’s Story:

Zoe is a happy, active 14 month old who happens to have Spina Bifida and Hydrocephalus.  Waddle was conceived on Zoe’s first birthday to mark a milestone that we as a family never thought we would have together.

We were given the diagnosis of Spina Bifida at our 20 week scan at the Lagan Valley Hospital.  The news was totally unexpected and my wife Kirsty and I were stunned ; a referral was quickly made to the Royal Victoria Hospital where we received more detailed information about the severity of our baby’s Spina Bifida and Hydrocephalus. 

I remember going to that first appointment at the Royal with the positive expectation of finding out what the next stage would be, and how we would deal with this situation which we were living in that had taken over every waking thought.  What we were confronted with however was an agonising decision to continue or terminate the pregnancy which pulled our emotions in many different directions.

Although none of the medical consultants could say with absolute certainty what level of impairments Zoe would have, the prognosis was bleak.  Foetal MRI scans with the support of ASBAH confirmed that Zoe had a rare thorasic Spina Bifida which meant that her back would be open at birth from between her shoulder blades to her bottom, with no skin, muscle or soft tissue coverage. The spinal cord nerves below the middle of her back would be damaged resulting in paralysis of her lower limbs and problems with continence.  The scans also showed her Hydrocephalus to be severe with little brain tissue present, and that she had a large Chiari Malformation extending to her 5th cervical vertebrae - this is when the Cerebellum or hind brain which controls heart rate, respiration and fine motor control develops in the narrower space of the neck instead of the back of the skull. 

We decided to continue with the pregnancy to give our baby every chance, and prayed hard.  On the 29th December 2008 our second baby girl was born and we named her Zoe, which means ‘Life’. 

The birth was by planned Caesarean section with a specialist delivery team of eight due to the risk of Meningitis and Septicaemia with the Spinal Cord being exposed.  4 hours after birth Zoe underwent her first surgery for 5 and half hours to close her spine and back, and to have a shunt inserted into her brain to drain her Cerebral Spinal Fluid (CSF) and manage her Hydrocephalus.

 

Zoe was recovering well from her surgery but two days later on New Years Eve morning I walked into the ICU to see a hive of activity around Zoe’s incubator - Zoe had crashed and was being put on full life support.  Tests confirmed Septicaemia and we were told the next hours would be crucial; Zoe fought hard through the day and we had her baptized New Years Eve night as her life was still in the balance.  She improved over the next week and was able to come off life support; on day 9 we were able to have our first cuddle with Zoe.

Zoe spent a couple of months in the Neonatal ICU and SCU and had a second Septicaemia infection and pneumonia during that time before we were able to bring her  home.  After two great weeks at home Zoe became ill vomiting with a temperature above 40∞C, and we had to bring her back to the RVH for Sick Children.

Zoe’s shunt had blocked which meant the CSF could not drain and therefore pressure was increasing inside her head pressing her brain against her skull.  Zoe had a second operation to replace her shunt and was monitored in ICU.  After some encouraging improvement 2 days later Zoe deteriated again and was rushed back to theatre for a new shunt. 

Similarly Zoe initially responded well following this 3rd surgery, but again 2 days later she became seriously unwell and needed another emergency operation.  Her fourth operation replaced her shunt again and it was externalised outside her body so drainage of CSF could be monitored and recorded in ICU.

Zoe however did not respond as well following this surgery.  Zoe was not to wake up for the next 6 days despite her externalised shunt showing that CSF was draining correctly.  There is a limited time which a shunt can be externalised due to the risk of infection (Meningitis) so we knew Zoe would have to have another operation within a week, but it now became a question of which operation she would have to undergo.

Further neurological tests were done that week and we worried about the possibility of brain damage having occurred while the pressure in her head was high, as the cause for her now comatose state.  The through tests systematically ruled out possible causes and pointed towards her Chiari Malformation.

 

The surgical option for the Chiari malformation would involve removing the base of the skull and part of each of the 5 cervical vertebrae affected to relieve pressure on the Cerebellum.  This was viewed as the absolute last resort by the consultants as the survival rate of this surgery is extremely low in babies less than 3 months old. 

After long and supportive discussions with the Neurosurgeons’ on the Friday we agreed to take time out for the weekend and think about whether to go ahead with the decompression surgery for the Chiari Malformation.  The hospital staff were very sensitive and arranged to allow Zoe’s sister Cara, and her grandparents to see her on the Sunday afternoon, in affect to say their goodbyes.

 

Prayer works! Out of blue Zoe began to wake up that Sunday morning before the family visits.  Based on her improvement he decision was then taken on the Monday morning to hold off on the decompression surgery, and to do the originally scheduled operation to internalise the shunt.

Unfortunately Zoe became clinically unwell a few hours after this fifth surgery with seizures, increasing frequency of Apnoea’s (stopping breathing), and CSF leaking from the back of her head.  Zoe deteriated further that evening and after more CT scans she was taken to theatre for a sixth operation in the early hours of Tuesday morning.  Zoe’s shunt was replaced this time with a digital programmable shunt were the CSF drainage pressure levels can be adjusted. 

Over the next few weeks Zoe continued to have periods of Apnoea but steadily improved and began to gain weight.  After a couple of months in hospital again we were allowed to bring Zoe back home.

Zoe has been back in hospital twice since her series of operations thankfully for only a week on both occasions and like many babies she is happy, eats, sleeps and keeps Pampers in business.  She has made great progress physically and based on this a decision on a Spinal fusion surgery has been deferred.  Her daily routine differs a bit however from our experience with her older sister at the same age. 

To protect her Kidneys Zoe has to be catheterised every 2 hours each day with an indwelling catheter through the night, along with several medications. She also gets physiotherapy through the day to help her flexibility, mobility, postural control and circulation.  Zoe was missing some muscles in her lower back when she was born along with the spinal nerve damage, and at present is working hard with a goal of being able to sit unaided.

She loves working with her Occupational therapist Sabina and is showing really positive improvement with her play development and language.  Looking ahead she has a small operation planned to protect her kidneys, and will require some orthopaedic intervention but never stops smiling; we are so lucky as a family to have her.

 

I often look at Zoe’s daily challenges, and effort & determination to do her exercises when at Physiotherapy, and it puts my sometimes less than positive attitude about my own sport injury rehabilitation in perspective.  Spina Bifida however is not an injury, children born with this condition have no choice; it is a neural tube defect that occurs with the first 2 weeks of conception. How different would any of our childhood activities have been if we where in a wheelchair? - just a thought.  

As I review Zoe’s story it would be remiss of me not to say thank you to those people who cared for Zoe and supported us as a family.  I apologise for not mentioning everyone by name and for any incorrect medical titles used, but particular thanks goes to:

1.  
   • • All our family and friends for their support, thoughts and prayers.
   • • My daughter Cara for keeping me smiling.
   • • Mr Dave McCauley (Consultant Neurosurgeon) for his skill, surgical expertise and humour.
   • • Mr Hill (Consultant Plastic Surgeon).
   • • Mr Philip Weir (Neurosurgical Registrar) for all the ‘needles’.
   • • Mr Clifford Mayes (Neonatal Consultant) and team.
   • • Barbara Palmer (Neonatal Intensive Care Nurse).   
   • • Marie (ASABH) for her support to Kirsty and I, pre and post-natal.
   • • Rev Brian Anderson for being there at ICU often till the early hours.
   • • Medical staff at RVH for Sick Children - ICU, Special Care Unit & Paul Ward.
   • • Spina Bifida Clinic - Dr Hill (Head of Clinic), Mr Cosgrove (Orthopaedic Consultant), Mr Baillie & Mr Marshall (Consultant Urologists), Laura & Emma (Continence Nurses).
   • • Child Development Clinic - Sabina (Occupational Therapist), Jane (Physiotherapist).
   • • Moira Surgery, Dr’s, receptionists and Mona for their continued support and all the repeat prescriptions.
   • • Ricky & Barry for helping make Waddle a reality.