Not Usually Associated with a Full Life.

By the time Teddy was 6 months old he was racking up a long list of things that weren’t going his way and we were now on first name terms with plenty of staff in our Alder Hey Children’s Hospital. We knew he had hearing loss and although he had his hearing aids, they brought no obvious miracle or ‘fix’ to the things he wasn’t doing. To say he wasn’t a great feeder is an understatement; he was gaining weight but not enough; he struggled to hold his head for more than a few seconds, he wouldn’t fix and follow a toy and he wouldn’t smile on demand- I know, it’s hard to believe given I am basically a comedian.

Plenty of people would tell me to stop looking for issues. To them, it was an attempt to offer reassurance and comfort, to me, I didn’t need to look for them, it was obvious.

In July 2018 I noticed one side of his tummy was slightly firmer than the other. An ultrasound scan showed his liver was a lot bigger than it should have been and blood tests confirmed it was functioning OK but he had high enzyme levels- a sign of liver disease. 

Teddy has spent a lot of the weekend hysterically crying, (and I mean bright red face, wide eyes and screaming!) and was due for more blood tests on the Wednesday. By now we were averaging several hospital appointments every week so we had to strategically pick what ones Adam would come out of work to attend. I wasn’t brave enough to face blood tests alone so my Mum came with me. I didn’t know what to do with Teddy, something wasn’t right- I refused to take him home until something was done to help him. ‘Luckily’, he put on a good show of just how unhappy he had been that weekend and was admitted to hospital. The General Paediatric team were now liaising with liver specialists in Leeds after a further increase in the deranged blood tests. I had already imagined my worst case scenario of a liver transplant which, similar to when I lost my eye, was nowhere near as bad as what I had coming.  

Because it was still functioning well, it was established that it wasn’t actually his liver that was causing the problem- there was something else that was making his liver unhappy….. Que the metabolic team…..

I felt their introduction almost came with a warning. Although these were not the words of the Metabolic Consultant, my interpretation was ‘let’s hope it’s not a metabolic issue’ and ‘don’t panic yet’. 

Adam left work and arrived at the hospital when he heard Teddy was being kept in, and as luck may have had it, was just in time to don his super hero cape and be with Teddy while he had ANOTHER  bucket full of blood taken. These samples were shipped off to various venues around the UK to be tested; these tests were so specialised and rare that they only ran once a week, and as our luck seemed to go, we had missed this week’s cycle so had to wait for what felt like forever to get some answers. 

Bizarrely, I done zero research into what metabolic condition this could be, which is very out of character for me. The same can’t be said for Adam though. I walked into the hospital room one evening to find Adam crying, he had taken to google and found a metabolic condition that Teddy ticked almost every box to. He told me that it basically came with a death sentence, lots of babies don’t see their first birthday, good ones see their second and if you’re lucky they might make five. I tried to reassure him and me that we didn’t know this is what we are dealing with- nothing was confirmed. To be honest I don’t think I could comprehend it. I chose to file that information.

The following week, the Consultant Paediatrician came into Teddys room with his Registrar and closed the door behind them. He asked if I wanted them to come back when Adam was there. Obviously, the answer was no, if there is something to be said I want to hear it now. 

The doctor stood with his back leaning against the toilet door, trying to be as unthreatening as possible. I sat on the not so comfortable sofa bed as he told me the result of the blood test we were back and they confirmed the diagnosis of a Peroxisomal Biogenesis Disorder (PBD), AKA Zellweger Spectrum Disorder (ZSD)…. The metabolic condition I was choosing to block out. And here I was with that familiar feeling of trying to keep my insides from falling out as I heard the words ‘very serious condition that’s not usually associated with a full life’.

I was trying my best not to do my ugly crying while I had an audience, so I asked the doctors to leave and thanked him for coming to tell me the news! I THANKED HIM- can you believe that?! The Consultant knew that that was ridiculous and gestured not to, with a sympathetic face on his way out.

I stood over the hospital cot staring at Teddy, asleep in his cute beige Peter Rabbit dungaree shorts. Is this real? Standing there looking at him and knowing he is going to die; we won’t have him forever. What have we got to come? Am I really going to have to plan another baby’s funeral? 

I rang Adam at work. I was trying my best to keep my shit together on the phone but clearly that was impossible. I was crying down the phone; I told him he needs to come now, that the results were back and it was the condition he had googled. 

He was with me in about ten minutes, slightly mad that I had been given this news alone; but he knows what I am like, there was no way I would have refused information regardless of circumstances. We held each other and cried together. We lowered the side of the cot and leaned in to be close to Teddy who was still sleeping. The Consultant was called back to see us when Adam arrived. He answered our questions the best he could with it not being his speciality, and explained that the Metabolic Consultant would come to see us Wednesday and explain more about the condition (he was based in Manchester and visited our hospital once a week). 

Both of our parents were on holiday. Adam chose not to tell his until they came home but I couldn’t hold my own water…..I had to ring my Mum & Dad and tell them what was going on. I think I was surprisingly calm on the phone, I told them, very matter of fact, that it was the shit metabolic condition Adam had researched, that there had been no talk of prognosis and that under no circumstances are they to return early. They were away with my Sister, brother in law and kids and they didn’t need to know there was an issue. I’m all for life lessons and hopefully Martha and Josh can reflect on these last 2 years in the future and know all about resilience and how life, fortunately or unfortunately, goes on.

Adams sister came to visit, and my brother done the 4 hour round trip after work to come and offer some moral support…. In the form of KFC 😉

We never did get any type of prognosis. From what I know now I can understand why no one wants to commit or suggest a timeframe; the condition is so rare and these children write their own stories so it’s hard to compare.

The Metabolic team were keen for us to go home and ‘enjoy him’. But unfortunately, the Endocrine team had other plans. Teddy was also diagnosed with another condition- Congenital Hyperinsulinism (CHI). It’s basically the opposite of a diabetic, meaning he produced too much insulin which caused his blood sugars to drop dangerously low. This is the main thing that kept us in hospital for so long, while we tried milk supplements and slowly introduced medication which in theory would control the insulin production. 

Well Teddy wasn’t one to play by the rule book and so didn’t respond to the medication, he did however respond to the side effects of rapid hair growth as we watched him quickly turn into a small werewolf.  I hated that! I mean it’s a small price to pay if it fixed a problem, but it didn’t so I was keen to get him off it; plus, another side effect of this medication was water retention, so there was more medication came hand in hand to manage that. Once it was established that the highest dose wasn’t working, the weaning off process started and we looked at plan B….or plan C….I had lost count at this stage.

Now, not responding to medication would be fine if you could rely on him feeding well. Teddy was having low blood sugars about 3 times a day and we were relying on milk to correct them. Every bottle was stressful for me and for Teddy and we were practically force feeding him whatever we could, which would only temporarily correct his low blood sugar.

The doctors were keen to insert a nasogastric (NG) feeding tube. I dug my heals in and said no. My argument was that there had never been a time when he didn’t drink enough to correct his blood sugar, so therefore it wasn’t needed. The reality was I was in denial! It felt like another backward step, another kick in the teeth. It was more confirmation that I had a disabled, sick child. It wasn’t long before I realised I was making life harder for Teddy by not allowing an NG tube. For whatever reason, he hated feeding and would become so distressed as I held him so close to me, trying to get the bottle in his mouth. Once he had an NG tube, he could feed for pleasure as and when he wanted and we could put the required amounts of milk through the tube. Don’t get me wrong…..the NG tube brought a whole new host of stress, which I can laugh about now (ish), but I’ll save that for another day.

In September 2018, Teddy went to theatre to have a Percutaneous Endoscopic Gastrostomy (PEG) tube fitted- which is a more permanent feeding tube directly into the stomach. This meant that we could now work towards a feeding regime that managed his blood sugars and was also suitable for at home. We added a carbohydrate to his milk and he fed religiously, every three hours through the day and continuously over night for 11 hours. I absolutely hated living to such a regimented routine, but that was the one thing that we had any success with so that’s what we done. 

On Wednesday 3rd October 2018, we finally left hospital with an unprecedented amount of equipment. Our life had been changed more than we ever knew it was possible but not it was time to make some happy memories.