Jazz and her Incredible Journey

Hi there! I’m Jazz, 25 from the United Kingdom.

I am super excited that Taryn and Jess asked me to write a blog post about my medical life, disability and how I have thrived over the past 25 years.

I write ‘thrived’ as it is a miracle (to me) that my body has endured so much in a short life span and I am here to tell the journey to where I am today. As you read on, you may be wondering ‘how does Jazz remember so much?"; Well, I asked my mom to contribute to the earlier stages of my life, so here we go.

Some facts about my diagnosis: I was born with Cystic Hygroma, a rare condition which affects 1 in 6,000 live births. Due to it being a rare condition, these numbers are calculated by incidence or prevalence rate. There is no official method for tracking these conditions and no official statistics exist, only estimates. My cystic hygroma is located in the head and neck and involves fluid-filled sacs that results from a blockage in the lymphatic system.

They are most commonly located in the neck or head area, but can be located anywhere in the body. Cystic Hygroma can be discovered in a fetus during a pregnancy ultrasound, or it may be apparent at birth as a soft bulge under the skin. When it is identified on pregnancy ultrasound, there is an increased risk for miscarriage. Cystic hygroma may be caused by genetic or environmental factors that cause abnormal development of the lymphatic vascular system during embryonic growth.

It may occur on its own or as part of a genetic syndrome with other features, such as Turner syndrome, Down syndrome or Noonan syndrome. It can occur in adults too but mine was present at birth. Due to my cystic hygroma, they placed a tracheostomy in my neck in July 1996 and I have had this ever since. My hygroma has improved dramatically since I was a child and now have period size hyromas floating around my head and neck. I often have flare ups which are painful and irritating; they rarely lead to me going to the ER but have in the past.

Onto the story:

My mum’s pregnancy went well – apart from the car accident when she was about 6 months pregnant but they never followed that up with a scan so nothing was ‘missed’ beforehand and this maybe could have been at 6 months, but wasn’t. My mum didn’t know if the Cystic Hygroma could/should have been picked up in scans but for whatever reason no medical issues were raised. In a way she was glad of this as otherwise she might have spent months worrying and that wouldn’t have been good for me, in the womb. They never wanted to know girl/boy either.

I was born in a lovely, calm environment (Tameside Hospital) with a normal delivery but shortly after my birth, they changed my mother's birth plan (to pop me on her chest and let her cuddle me,) and I was whisked away to the doctor area whilst staff fussed around her.

They said another Dr was coming to see me - then (time wise, no idea) I went to special baby care. My parents came to see me there – at Tameside, in a normal incubator. I had one massive hygroma around the chin, going down to my chest – but I was breathing by myself! We stayed in Tameside, just one day. I think it was a night but we could have gone to St Mary’s (now part of Manchester Children’s Hospital) that night. I went in an ambulance, Dad and Mum followed. I was born 6lb, 8oz with my hygroma which equates to around 5lb 10oz with no hygroma.

They settled mum in her own room (not on a ward with other new mums) and then we were able to go and see me in SCBU (special care baby unit) ...they’d been settling me in. As my mum recalls, (and maybe I just remember key moments from hearing my mum tell the story) the first time they saw me in SCBU they saw my chest going right in and down – almost like I was being squashed. They asked about this and very quickly they were ushered out and staff went around my incubator. This is when they intubated me and put in the nasal airway ( the blue one in nose.) Due to my hygroma, my intubation was through the nose, not the mouth as my tongue was basically a sac of hygroma. My tongue is still enlarged but a lot less than it was.

They waited in the family room and eventually a Dr. We think it might be Mr Bianchi but we’re not sure if he was just Pendlebury)...Mum liked him so much that maybe she was hoping it was him. He came and explained that I had had trouble breathing and so they had intubated me...that the fluid in the hygroma was squashing my windpipe (she thinks that’s what was said.)

Until this point, they had not mentioned about the cystic hygroma, only that I had cyst. I’m not sure when exactly they explained/ told them/diagnosed the cystic hygroma.

Mum stayed in with me and Dad, Sam (brother), Grandma etc came to visit. I had my first operation at 8 days old to remove the main hygroma sac. Luckily it made a massive difference – I looked just like a new born for a few days. Then the other hygroma sacs started to fill and mis-shape my face again. My mum couldn’t breast feed but expressed milk for me and this went in the NG tube. Sam sometimes visited. In August Sam went away with Grandma/Grandad to give him some special time. Mum stayed with me in hospital for a few weeks but also did come home occasionally, for a day.

Around the start of July, they started to talk about the tracheostomy but that Mr Bianchi (or maybe Mr Rothera?) were away for 6 weeks so they’d arranged for me to go to Great Ormond Street. I was whizzed down in me own ambulance and mum was driven down – because I was in an incubator. The second time we went down in an ambulance, mum came with me.

Around the middle of July, I had my tracheostomy tube put in. Mum was encouraged to try breast feeding but it just didn’t happen as I couldn’t latch on due to the hygroma. We came back to Pendlebury on Victoria Ward – it became a very familiar place!

They warned my parents that I would make no sound but mum said she will never forget the times I was crying my little heart out with no sound whatsoever. She can’t remember when I started making sounds – she guesses when the tube allowed some air around it.

I came home for Sam’s birthday for the day – a trial of us being confident with my tracheostomy. I then came home properly a couple of weeks later. I was in hospital for 18 weeks (125 days)- May to September 1996.

We then went back down to GOSH for an operation on my hygroma – we didn’t know this wasn’t part of Mr Bianchi’s doing so went – and forever more have wished we hadn’t. It wasn’t a good experience and we would never go back there again.

We had amazing support from the pediatric home nursing team following discharge from Pendlebury. I had a lot of infections around my tube (e.g. Strep group A) and a really sore/ ulcerated tongue. I was tube fed – until 2+. My mum remembers that they wanted to give me a gastronomy button but she and my father refused. One extra hole was enough for me! Ultimately it was determined I would get to eat eventually. A huge triumph. I was able through my and my parent's resilience to eat and drink and thrive!

My parents then went to the local authority for a Statement of Educational needs when I was 18 months, and Chris from PANDA playgroup helped with that by allowing me to go there without us. Tameside didn’t know what to do with me and suggested a special school for deaf children, but my parents were adamant on mainstream – eventually, by the time I started nursery, the statement was although it took another term (maybe slightly less) before staff were trained up in tracheostomy care and were confident enough that Dad or mum, I wouldn't be on the premises!!

Large tongue operation – March 2002

Due to my hygroma, from my tongue being large, fat, ulcerated and dry, and down almost to my chin, it was reduced so we could see my bottom lip and teeth. I looked like a different child and it obviously helped my speech. I always just got on with life after my operations – painting, playing etc.

I hated having the NG tube in again hence I think I really wanted to eat. Only 5 days post-op so tongue, my tongue had swollen with the trauma (it did go back down) and I couldn’t control the excessive dribbling – so tissue after tissue was used!

Apart from spending time in hospital for a large chunk of my life by this point, I went to regular school, had friends and attended out of school clubs. When in (and out) of school, I was not allowed in the water or sand due to my tracheostomy, but as you may have gathered by now, I stuck to no rules or odds regarding my medical life or disability. Due to my hygroma, I learnt BSL and Makaton to communicate and hit every communication milestone on time due to this. If I had to have speak, I would not have met these as I could not talk.

In between surgeries,

I had regular check- ups with my ENT, plastic surgeon, orthodontics, consultants, doctors, speech and language etc. I had so many services involved, it was crazy! Around the age of 10 (before I went to high school), I was switched to a silver tube with a speaking valve (flappy) and a sleeping valve (open). I remember the day when I went onto the ward (no pre-warning considering, I associate wards with pain and them holding me down in order to do an IV, NG or anesthetic) and I screamed, cried and wandered round aimlessly for about 2 hours before my ENT came and put the new tube in. I was so anxious and scared about what was happening -lack of communication, big no for me! Once it was in, I hung around for half an hour or so and off I popped home with this shiny new tube.

Around this time was when I went to Birmingham Children’s Hospital to discuss the possibility of re-aligning my jaw to help my quality of life.

At 10, this sounded so scary and the odds of having to have it done more than once were too high for both myself and my mum- we left and did not look back.

In 2012, this topic approached again. By this time, I was 16 and ‘fully grown’ (or would be, by the time the surgery came around.) I had orthodontics heavily involved, plastic surgeons from different areas of the country, speech and language. I was on a first name basis of the photography people by the end of 2013, so many different specialists, doctors and consultants involved in my case, I felt overwhelmed and like ‘it’s not that big of a deal’; but it was, I just did not realize it at the age of 16. For the next 3 years and 10 months, I was in an MRI machine every month, hooked up to every wire you can think of, had nerve testing, muscle testing- you name it, I probably had it. Even an ECHO, to check how my heart was functioning. I still hated IVs, so putting dye into me was no easy task. The plan was to take my jaw apart, take bone out and reconstruct it so my jaw would shut and my lips would be closer together. It was the biggest anterior bite recorded. I am in a medical journal out there due to this.

Jaw surgery- September 1st 2016.

I was anxious, scared, apprehensive and excited about the future. I was optimistic at the thought that this would ‘cure’ everything. How wrong I was, I just did not know it at the time. This is the first surgery where I HAD to sign for myself as I was classed as an adult. I had my regular chat with the staff about waiting until I’m asleep to put any IV into me. I cried. They would not let my mum come into the room with me, so I froze and said no until they allowed her to come with me. I then waited in a room outside the anesthetic room, which was so nerve wrecking and I had the urge to run.

The ECHO stickers went on as per usual and I looked up at my reading and they said it was a little high, so they had to calm me down before putting me on general anesthetic- it was to be expected. Once it had gone down, they told me to say goodbye to my mum and I squeezed her hand tight until I was asleep. It took me around 2 minutes to go this time as I was ‘resisting the gas’; I remember them saying that to me. It stinks, anyone would resist the urge to breathe it in!

10 hours later my surgery ended. I did not wake up. September 2nd, I woke up at 5pm. I had writing on my arm, wires hooked up, IVs in every part of my body imaginable. I had one in a vein in my head (this vein has only just gone down.) I looked around, expecting it to be 9/1/16 and in the High Dependency Unit, instead I was in the ICU over 34 hours after I went to sleep. My mum was crying next to me, my brother was around vaguely, I was high as a kite on hospital drugs. I needed water; I couldn’t drink. I couldn’t breathe, they had me hooked up to the trach oxygen machine. I was trying to signal to take me off as I was panicking. You may be thinking ‘but its helping you’; you would be right, when I couldn’t breathe for myself, but I could by this point, and extra air into my already extra airway body was sending my lungs into distress. Scary times! I spent the next 7 days resenting this surgery. They said it would be 3-4 days in hospital and I would be eating soft foods and drinking. However, due to the extent of my anterior bite and my jaw construction, this did not happen. I was in hospital, with drains behind my ears, IVs still in as I couldn’t drink.

My face and eyes were swollen to no end, in my eyes it was a disaster. By day 5, my leg had swollen up and was solid. We spoke to the ward who said it’s fine- it was not. Always trust your body and instincts. Fight for your body, no one else will. I fainted every time I stood up or walked. I ended up going home with 2 DVTs, which are life threatening. Back to the ER and I was on injections for 30 days in my stomach. It was black and blue; I had no skin that was not bruised left. Traumatic experience turned out good.

Due to this surgery, I had to learn to eat, drink, talk and walk again. I went back to being a ‘baby’ because I couldn’t do anything that I used to be able to do. I have only just learnt to drink from an open cup again- 5 years later! I still have difficulty eating which I get embarrassed about but this is part of me and I pray that people accept me for who I am and not what they ‘see.’ My speech has improved drastically and I have just learnt that I sound ‘weird’ because I have a paralyzed vocal cord- so I only have 1 instead of 2. Fun fact! I recently saw a ENT in NY and their comment was ‘you are funky, your whole presence is funky. Your mouth, throat, nose, trach and cords are just funky; I love it.’ This made my day, no one has ever said my body is funky, they just say deformed.

Muscle and nerve surgery

December 2018: This failed. Drastically. Like my jaw surgery, I had scans, MRIs, ECHOs, nerve and muscle pins and photographs to see what is going on. From what they saw, everything was as it should be. Except my jaw- one side does not have a socket, only a ball, so this is the only thing they had to work with. We went in to have surgery, I took my fidget toy (highly recommend for anyone who is anxious to take this if their child or you have surgery, it focuses the mind) and went with the anesthetic within 10 seconds this time, I was not messing around, I wanted out. I woke up 3 hours later to the news that it had failed. I threw up blood and acid where I eventually (and reluctantly) had an anti-sickness injection which made me throw up more but eventually stopped. I went back to the ward where I ate and drank (after a poke or two from the nurses as I was shattered and coming off the anesthetic. I stayed in overnight for monitoring as I had a drain in under my ear again for bleeding.

People say:

‘I’m a miracle, a warrior, a fighter, a kind hearted soul’; I used to think "no I’m not, I’m just me." and I still think this to this day. Slowly, I’m realizing how much my life so far has consisted of trauma, pain and anger. Even though, my body and mind has never given up, not once. I’m so grateful to my body for allowing me to live this life. I have never had the chance to advocate like I do now. A different place, environment and people have allowed me this avenue to do so and I am beyond grateful.

Moral of the story:

Always trust your body, gut and instincts. Don’t let anyone ever tell you or your child, you cannot do something- YOU CAN! You work with difficult situations everyday and YOU overcome them because you advocate for your child or yourself or other people. Just because your child cannot do something today, it does not mean they never will- it takes time. Learning and developing is not an overnight process. You and your child are the most important people in your story. Advocate, fight to no end and never stop.

You are the best for your child and yourself. You are the best version of you, even when you have bad day. It has taken me a long time to realize that I have these medical and disability quirks and I still resent my body for having them but that is also OK...we are all human. I am a miracle that I have survived Cystic Hygroma where the statistics do not show that survival rate is high. That is OK! I am constantly improving myself and with this blog, I hope to have told my story.

Feel free to reach out to me on Instagram at @jbe1996 as I am always willing to answer questions or chat about anything and everything.

Keep advocating. Keep strong. Keep fighting. Keep loving this amazing community that we are building together.

Love, Jazz