My Story
Gunner Lewis-Vale

Our beautiful Gunner arrived three weeks ahead of schedule on 9th October 2019 after a straight forward pregnancy weighing 6lbs 4oz.

After a speedy labour throwing my body and mind into shock, I looked down at Gunner and felt besotted with the little boy in my arms. It was the early hours of the morning so we both got a little sleep and were soon having checks before being sent home.

 Gunner passed everything other than the newborn hearing test which we would later discovered was severe glue ear. This would lead to a hearing band at the age of 18 months and open his world to what he’d been missing. As he had only ever experience hearing of 50db in both ears.

Gunner took to our family of four like he was always meant to be there, the final piece to our family jigsaw. Gunners Dad, Big Sister and I couldn’t believe our luck and enjoyed his first smile, first roll, crawl, words and Christmas these all passed by without a worry.

Fast forwarding to April 2020 we are in lockdown and I myself am still on maternity leave. Gunners belly button is starting to push out and look a little larger than your regular belly button. We notify the doctors to be informed this is nothing to be concerned about. If it continues to get larger as Gunner grows to call back.

Gunner at 8 months old June 2020  started to become aware that Gunners head seems to be growing much larger and quicker his  forehead is starting to protrude. I start to buy bigger clothes that make allowances for this, I then start to question it with close family and friends. We tried several times to contact the HCV but due to Covid -19 we got no further.

Later in the year of 2020 when the colder months settled in October 2020 Gunner starts to have a continuous runny nostril (right). His snoring becomes much louder than before, his sleep became more broken and chest infections became a regular occurrence. From October – December Gunner is prescribed 5 different types of antibiotics without being seen due to covid-19. As a family we avoid going out in cold or wet weather as Gunner will get a further infection and my worries as a mum really start to take its toll. Gunners father and I are calling the medical practise weekly at this point in desperation to get him seen. We manage to get a Doctor to send a referral for all concerns Hearing, Breathing and Hernia through sheer persistence.

January 2021 my worries for Gunner have started to consume my days, I begin to call Gunners ENT consultant as he hasn’t been seen for over a year. In hope that we can get his hearing assessment updated and I can have an opportunity to bring up my other concerns. It pays off Gunner has an appointment  12th January at the local community hearing centre. We arrive and follow all Covid-19 guidelines, we are seen and the team can’t seem to fathom why Gunner has been left so long. They agree that my concerns over his head size and continuous infections are justified and they themselves question his runny nostril and point out his very flat nose bridge. I live here elated I can’t believe someone has seen him and agrees with me, I cry through sheer shock and happiness.

They call Gunner back on 23rd February to ensure results are consistent that there has been no change from birth. On this second visit however Gunner snot is out of control this day and due to Covid-19 the team don’t want to get near his as the pandemic seems to be getting worse so do peoples worries. They seen alarmed by it and we are swiftly in and out with no conclusion or information. I leave upset and frustrated we then get a further prescription of antibiotics and we still aren’t leaving the house in bad weather. (Which at this time of year is most days)

7th Match 2021 Gunner is learning to walk and takes a tumble he seems fine but is later sick and has a high temperature. We call 101 and off we go to our local hospital PRH we are seen straight away and the paediatric team are concerned about his temperature that at home I was unable to keep down. A consultant is called down to also look at his irregular heart rhythm which they are trying to shock Gunners body out of. He has and ecg machine attached, bags of ice all around his head and this point they are filling a cold water bath to again achieve this shock dunk effect.

Luckily it doesn’t come to this and Gunners body (heart) starts to behave. The consultant was called during this panic and arrives. I discuss with him my concerns through a whaling crying state Gunners head, breathing, nose and belly button is all spilling out. The consultant slowly listens and informs me that Gunner is to be admitted and he will help us get to the bottom of all this and find the reason. In the moment I felt relieved but had no idea that Gunner could have MPS Type 1 Hurlers.

We spend the next 3 Days with many consultants and teams coming in and out of our room, tests began and we got a small insight into the possible diagnosis. Mucopolysaccharide  a life changing diagnosis which would impact Gunners life expectancy massively. I’m in total shock and have to deliver this possibility to my husband and family who are unable to see us due to Covid-19. 

We are sent home while blood samples are biked across to Manchester and urine samples are sent to Birmingham. Both of these hospitals specialise in this condition and therefore will be the ones who will identify if it is in fact this. We are aware that it is a genetic condition but are told to avoid looking at any further information until this is confirmed due to the enormity of it.

We dont have to wait long and within 3 further days we get the call from Bimingham from the metabolic team. They can confirm that Gunner does have Mucoplysaccharide Type 1 the most aggressive from and need to see us just 4 days later.

Sat in the room with the consultant, Specialist nurse, junior doctor, Gunner, Jamie and I discussing what feels like a heart breaking diagnosis. Gunners condition is explained his body doesn’t have the ability to break down the body’s natural sugars. His body is therefore storing it throughout his organs, bones and brain. This means that his enlarged head is due to fluid that the storage of sugars is causing. His herniated bell button is because the body is storing sugars in his liver and spleen causing them to be enlarged. This storage is causing the continuous  infections as well as his flat runny nose, large tongue, hearing loss and snoring.

Gunner needs to start weekly 5 hour enzyme therapy treatment transfusions. This will start to prevent further build up of the sugars in his bones and organs. However we are made aware this has no effect on him neurologically therefore damage will still continue. The only option we have is for Gunner to undergo  a BMT before he is 2 for which Jamie, Daisy and I need to be tested to see if we are a match.

We are tested and our daughter Daisy is so brave but very keen not to do it ever again. We now wait several weeks to find out we are not a match and there is no UK register match either. So an urgent worldwide  appeal is launched

Gunner starts his weekly transfusions he has to be canulated this is a nightmare and some weeks takes up to seven attempts. He then has to stay within a cot in his yellow outlined bay due to Covid-19 for the whole 5 hour transfusion. I supply him with as many toys as I can carry to the hospital but this is no easy challenge. However he does start to get used to the nurses and smiles at everyone around him. He is regularly the youngest on the ward and keeps everyone entertained.

We are pleased to see that the enzymes are starting to stop his runny nose and reoccurring infections. Gunner under goes surgery to fit a central line with three lumens these will administer all medication from transfusions, to chemotherapy and all medication required for his bmt once a donor is found. This is now a visible line going down his neck, and out of his chest, this is all connected to artery in Gunners hear.  Watching him be put to sleep for this and a full body MRI was what can only be described as horrendous, however Gunner copes with both extremely well.

We now visit hospital daily the 3 hour round trip feels like nothing, we’ve also mastered the multi story car park (we are from a village). Gunners slowly goes around each department having a full body MOT this is to look at damage already caused to ensure his body is fit for transplant. Also to look at where he is regards to development physically and mentally so this can then be monitored post transplant. Most go well apart from ophthalmology where we find corneal clouding in both eyes and a long sighted rx.

Our donor journey throughout this is turbulent we have two countries come forward but donor matches aren’t strong enough to put Gunner through it. A third is found in Germany but the donor doesn’t respond to the hospitals correspondence so with time of the essence we move on. Finally 4th time lucky a further donor is found again in Germany and they respond. A 34 years old male who has agreed to donate from his pelvis who is the strongest match possible a 10/10 for Gunner. Honestly even now writing this I can’t believe our luck and how amazing this man truly is.

Dates are set and our bmt journey begins ….