The longest surgery performed on a child at PCH!

Hi,

My name is Alexis and I am the mum of a positive, strong young man named Zachary.

Zachary is suffering from a rare disease known as Neurofibromatosis Type 1. He has thousands of tumours scattered around his body that wrap tentacles around major nerves and arteries. Zachary’s NF1 also is complicated as his tumours are strangling his Spinal cord and stopping the free flow of cervical fluid.

October 29th 2020

Copied from my Facebook post -

Unfortunately, Zachary is not well. He has a very large tumour growing on his Cervical Spine that is blocking the canal that the spinal cord and cervical fluid run through. This is causing him symptoms such as acute behaviour issues, gait/mobility issues, head/body aches and high temperatures. As of 29.10.2020, Zachary will not be returning to school or physical activities/therapies for the rest of this term/year as he has Neurosurgery coming up on the 23rd November 2020. He is a priority 1 (urgent) case. Unfortunately, the Neurosurgeon cannot remove the complete tumour as there's major arteries running through it, but she will excise as much as she can to allow the spine and cervical fluid to flow freely. Due to the severity of this procedure and complications associated with this issue, it is best for Zachary to stay home where he will be under constant supervision and out of harm's way.

November 23rd 2020

Zachary is admitted for priority 1 surgery to cut away a large tumour in C1/C2 that was impinging on his Spinal cord and causing him mobility and behavioural issues. The surgery went very well over six hours. Seeing our son in ICU with tubes draining bloody fluid from his spine, tubes feeding pain reducing medications into his system to keep him comfortable, and tubes to help him urinate. Heartbreaking scenes for any parent to go through. NDIS review for change of circumstances. Hiring of his own personal wheelchair in a reclined position to take pressure of the spine. Adjustable beds, shower chairs, rails installed, bath lifts, 24 hour supervision ......, he was discharged from hospital 5 days later with pain medications, a wheelchair to support his body and a scar.

December 8th 2020

A follow up MRI to see how the laminectomies are healing.

December 10th 2020

C2/C3 are now blocked by a neurofibroma larger than the last. Zachary was a priority 1 again. Tears falling from my eyes. Why could they have not done this in the surgery before?

Copied from my Instagram post - Friday we were called in to see Dr Omera at Perth Children's Hospital to talk about the MRI Zac had on Monday. Unfortunately, there was other tumours found in all joints of the spinal cord (Atleast 2 on every bone)that they could not see when they had him open on the 23 November 2020. This is very rare, and Zac is the only boy in Australia to have it! And to top it off, there have been abnormal cells found in the first tumour which they don't know if it is NF1 or something stating else with G (I think)

Zac is scheduled to go into surgery again on Tuesday to remove the next tumour and bits of bone to allow the spinal cord and cervical fluid to flow easily. If we don't do surgery then it is very likely that the drug trial won't work , but cutting away at bones means he is losing strength in his cervical spine and will most likely be braced to support his head, neck and upper body.

I am devistated and crying writing this. If God only gives us what we can handle, then why is it always painful! Have Zac in your Christmas prayers as it's not the best gift under our Christmas tree xxxxxxxx

December 15th 2020, we were back in surgery. Another successful surgery to cut away another plexiform neurofibroma and free the spinal cord as much as possible, this one was also wrapped around the main artery of the spine. We were home just in time for Christmas! Although surgeries went well, the complexities of Neurofibromatosis are unforgiving.

January 2021

Zachary turned 13. Although he could no longer enjoy the Abyss and Goliath at Adventure World, he did enjoy a steak and chips at South Yunderup pub, many presents, Testo Sushi Train and a night out!

February 2021

Zachary returned to school in a wheelchair. No more physical sports can be played. Intense physical therapy every Tuesday. Occupational therapy each fortnight. Pain medications every day. Individual Education Plans in place. Full time Education Assistant employed. It was tough. New complications to make contingencies for. Hospital appointments as an outpatient continue. Many hours of schooling missed to regain his independence.

2nd March 2021

A follow up MRI shows his spine is steady. The intense rehab is giving him strength back. He is walking taller short distances. Still in a wheelchair for school, shopping and long distances. But he is happy and positive and loving life again. Transperth Trains had become his obsession!

(My Facebook post) - Fantastic news received today. My 13 year young Zac is confirmed to be diagnosed with NF1 (Neurofibromatosis Type 1) exacerbated by puberty! We have had 5 months of surgeries, MRIs, blood tests and scary names being thrown around because of tumours growing on every vertebrae of his spine. Pathology in three different states in Australia were working together to figure out what it was as it's characteristics didn't match with NF1!! Now just a watch and wait patience game to see what these tumours do.

April 2021

Zachary developed a decline in his mobility and is fatiguing easily. He was having pain spikes, headaches and sleeping issues. Back to PCH we returned. Medications changed or improved and back home.

May 2021

Late night ferry rides across the Swan River, caryards on a Saturday morning. Tesla test drives with automated driving through the Northbridge Tunnel. Weekends packed with adventures, living each day as it came, fulfilling his every desire. Night photography from Kings Park. Cruising the streets in comfort, music loud and proud, singing Backstreet Boys "I Want It That Way". Living, laughing, loving every moment and memory made.

June 2021

Another MRI to check progress. We are told that everything is stable. Although we are concerned his mobility is still declining and his tiredness it concerning. Oncology are trying to think of other ways to make him comfortable.

July 2021

We take a break from rehab as he is too tired and won't cooperate. His mobility is tripping him, his head has slipped down to his chest and we are worried, but the doctors say he is okay.

August 2021

Zachary's mobility is declining. His gait is not passing through smoothly. He finds it difficult to stand for long periods of more than ten minutes and his head is resting on his chest again with a slant to the right. He is tripping over himself and again becoming aggressive easily. Every doctor we see are confirming he is stable, but our intuition is saying otherwise. He has become a falls risk as his head keeps moving more forward.

September 2021

Quarterly MRI check. Bloods also being taken for Oncology observation. Zac's head was still slipping further down his chest with his right ear sitting on his right shoulder so physiotherapy was recommenced and to determine a plan for physical improvement. Medications are upped to Tramadol three times a day and a higher dose of amitriptyline to help Zac sleep.

October 2021

Zachary has had numerous appointments over the month of October. We achieved:

Restarting physiotherapy/hydrotherapy with Christina at PCH

Being fitted for a custom manual wheelchair with Indigo at Motum

Restarting Autism Association Occupational Therapy with Shannon - focusing on cooking related therapy for strengthening of wrist muscles, resilience and daily functioning

Being seen by Dr Khoo (Neurosurgeon Registrar- PCH) and feeling that finally someone understands the decline of Zachary's health and cervical spinal strength in keeping his head upright off his chest and right shoulder. Dr Khoo left the room to speak urgently with Dr Saha (Neurosurgery Consultant - PCH) about bracing Zachary with a cervical thoracic orthosis to return and ordering us to attend L4 Orthotics clinic to have the brace fitted immediately.

Zachary was fitted with a soft collar cervical brace that bought his head up and supported his neck, showing signs of improvement immediately.

Left side MRI taken 08.09.2021 / Right side MRI taken 02.03.2021

November 2021

This month is an anniversary month with twelve months elapsed since the first cervical surgery to remove a large plexiform neurofibroma from the C1/C2 spinal canal. This month has seen Zachary achieve:

Ophthalmology - Dr Gardner (Consultant - PCH) is happy Zachary's right eye stigmatism is resolving itself and retracting to the right position quickly and normally. Zachary has 'driver's license' approved vision. Follow up appointment for more extensive testing in six months.

Unfortunately in November, Zachary's mobility declined. He is a severe fall risk as he is very top heavy with his chin slipping back to his chest as his soft collar has not refrained falling of his head. The attached MRI shows the difference of six months between slight cervical muscle deterioration (March 2021) and a major cervical muscle and bone deterioration - Kyphosis (September 2021). Thursday 11th November 2021, Zachary attended a scheduled Neurosurgery appointment. Dr Saha (PCH - Neurosurgery Consultant) was very concerned with the deterioration of Zac's mobility and cervical spine alignment. She called Dr Derrick (currently on long service leave - Rasopathy PCH) to discuss an emergency admission as the vital signs of the complexity within the spinal cord and ongoing mobility issues were of immediate discussions that could not be achieved in outpatient status as the timing is of great importance to the ongoing health and overall wellbeing of Zachary and his family. 5pm Thursday evening, Zachary was admitted to Ward 1B under a complexity team involving Neurosurgery, Rasopathy and Orthopaedics.

Monday 15th November saw Zachary have the majority of medical visits from teams such as:

Neurosurgery

Orthopaedics

Physiotherapy

Social Worker

Kind Kids

Psychology

The outcome for this admission is to be submitted into traction (gardner wells tongs) to straighten the cervical spine and stretch it to a somewhat average curve. This procedure will take approximately 7 - 10 days to achieve a desirable result for fusion. Following the traction, Zachary is being fused with rods and pins to hold his cervical spine (from base of the head to centre of the back) in a permanent position. Recovery from this surgery may take weeks as the opening of scar tissue and repositioning of the spine is a tedious operation. Later to this, Zachary will be placed on a drug trial to shrink the tumours in his body in hopes of relieving some pain and abnormal functions that currently obstruct his daily life movements. During this time Zachary will remain an inpatient of Perth Children's Hospital under the observant care of:

Dr Derrick - Rasopathy

Dr Saha / Dr Rosh - Neurosurgery

Dr Silberstein / Dr Narjaran - Neurology

Dr Khosa - General Medicine / Surgery

Dr Hetal / Dr Gottardo - Oncology

Dr Seresti / Dr Ben - Orthopaedics

Dr Adam / Dr Dylan - Spinal Orthopaedics

Dr Somerfield - Chronic Pain Management

ENT Team

A picture of the Gardner Wells Tongs - the instrument being fitted to Zac 19.11.2021 to correct the Kyphosis

It is with great anticipation of success that I am sending you this donation request to enlighten you with what Zachary and us are facing at this time. Zachary's condition is very complex and rare and not easily prevented, treated and maintained. We, together, have a long road ahead and appreciate all the prayers and well wishes. I pray you never have to face the complexities of these such decisions and the weight of an entire lifeline of contingencies. May this year's holiday period be spent with those you treasure and hold them close as circumstances change in an instant. I do believe we will succeed. In the meantime, please help us raise funds to fix my vehicle so we can transport Zachary in comfort and security and upgrade certain parts of our homes and cars that NDIS do not cover, to make daily living more adaptable to his needs. Your donation is very much appreciated.