Little things

Since having the MPS diagnosis I’ve noticed how the little things take on so much more significance.

Driving past our local hospital always gives me a twinge of  anxiety even though we hardly go there at the moment. I over-analyze people’s reactions. I watch out for any new symptoms intently; is this something that could be a problem?

So this morning he still had a cold – should we go to Manchester? We did. And part way through the ERT his temperature went up to 38.6 degrees, so the infusion had to be stopped. Not in itself a disaster, but it has repercussions – longer until we can start having treatments at home, more trips to hospital.

But it works the other way too. Yesterday, when Pudding said ‘Me!’ to let me know that it was his turn to sit on my knee I couldn’t have been prouder. When he is watching TV and reaches out to stroke his brother’s hair my smile could fill the whole room. And when my poorly boy still says thank you (‘Dyu dyu’) before starting to drink the milk he’s been demanding, I want to hug him till the world ends.

I’m learning to appreciate the little things more. Instead of getting frustrated about what we couldn’t do as a family or worrying about Pudding’s abilities, I’m letting go. I’m loving lazier mornings at the weekend, tumbling together on the bed, enjoying my family for what they are. Little things can be treasures as well as minefields.

Brave? Not me.

I’m shaving my hair off to raise money for the MPS Society. People tell me I’m brave, but I’m not.

I’m doing it because I feel like I have no other choice. I want to raise money to ensure future research into these horrible conditions, and shaving my hair, though unpleasant, is the easy option really. If I was brave I’d do something I hated, like running, or I’d jump out of an aeroplane.

When I started writing about our new life with Hunters, it was a private diary and I used it to blurt out all my innermost thoughts. As a blogger it’s easier to suppress the difficult emotions; to portray myself as a better person. But I made a promise to myself to be honest. So here goes.

I want to raise awareness of MPS, and shaving my hair will certainly have an impact and make people notice. But also I know that it will be me who is noticed. So am I being attention-seeking? Quite possibly. As a life-long introvert that is not something I normally crave, apart from when on stage. But right now, I feel desperation sometimes to reach out to people – to grab them by the arms and say ‘Can you hear me? Do you understand? This is what we’re living with.’ I want their care and concern. I want them to talk to me, to ask how we’re doing. Without this attention, I feel almost as if I would disappear from view. Not waving, but drowning.

People tell me that I’m brave for facing this condition the way I am. I can talk calmly about its causes and life expectancy, make light of it with, ‘It’s all pretty pants!’ But when I see pictures of children further along in their disease progression, I know that one day it may be Pudding in a wheelchair; when I think about the details, I know that there will come a time that he will no longer be able to swallow safely and will have to be fed through a tube; and when I see posts from mothers who are already mourning the death of their child, every part of me thrusts away the thought that one day that will be me. At those moments I am not at all brave. Just very, very scared.

I cling to the hope that he will get on the clinical trial, that the new treatment can halt brain involvement, that gene therapy will provide a longer-term answer. But on an entirely selfish note, I dread the continued trips to Manchester and further disruption to my normal life. So when people use the ‘b’ word, I feel even more of a fraud than usual.

But perhaps, just perhaps, I’ll feel a little braver when I hit ‘Publish’ for this post….

Reason to be grateful

Over the last few months I’ve been rehearsing for a play. Tonight is the opening night and I won’t be on stage.

That may seem like a strange reason to be grateful but let me explain.

I’ve always enjoyed acting – that chance to get up on a stage and be someone completely different, to move and entertain an audience. Since having children I’ve not been able to do much, so I was dead pleased to be cast in the ensemble for this play back in June. Then, before even the first rehearsal, we got the positive screening result for MPS. By the second cast workshop, we’d heard that it was MPS II and Pudding was started on enzyme replacement.  At that point I was hopeful that come the performance weeks we would no longer be travelling to Manchester; I knew that I would struggle to perform after one of those exhausting days, let alone having the worry of getting back on time.

Rehearsals were not always easy; sometimes it felt like the difficulties of the battle scenes were echoing my own struggles and more than once I had to go and find a quiet corner. But unlike my life at the moment, acting is generally much more predictable – you stand where you’re told to stand, say your lines and someone responds.  Sometimes things go a bit wrong, but we work together to cover a missed line or awkward entry. And for a few hours each week I was doing something for me. Something unrelated to development targets, treatments and worries about the future. So when it became clear that home infusions wouldn’t be able to start as early as I’d hoped, there seemed to be no other option but to pull out of the cast.

But the wonderful director told me we could find a way around it. Other cast members are taking over my lines and actions on the nights I can’t be there, and once again I get to be part of something special. On stage I won’t be a Hunters Mum. I’ll be a noble, a soldier, an attendant, a munition worker. Not tonight, but certainly tomorrow, and for that I am grateful.

So thank you, Lasses, and all the crew. Hopefully you all know how much this means to me.


Nothing hurts forever

It’s been an odd few days. Mostly in a good way.

Most significantly, I’ve been feeling a bit more stable again. Although this pathway through the Hunters diagnosis is full of rocky places and stumbles, every so often we get to the top of a small hill and the view makes up for some of the hard bits.

Hubby came with me to Manchester on Wednesday so we could talk to the consultant together about Pudding’s future and the possibility of getting him on the trial of intrathecal enzyme. This introduces the enzyme directly to the spinal fluid in the hope of reducing deterioration of the brain and possibly even improving function. It would involve more trips to Manchester and, if he is randomised to the treatment arm of the trial rather than the control, more interventions including general anaesthetics, so it’s not a decision to be made lightly. The first phase of the trial gave very mixed results, so we can’t hope for too much at this stage. But if he’s eligible we will likely say yes. Even if it doesn’t benefit Pudding, it may benefit others in the future. And we know the inevitable outcome if we do nothing…

Next thing on the list was a visit to our nearest special school. Pudding is due to start school next September so we’ve begun to think about where is best. Special school? Mainstream? Mixture of both? Another difficult decision to think about.

Yet yesterday morning before the visit I found myself humming while sorting the washing out. I had made contact with another Hunters mum after seeing her blog online, and then happened across someone else’s too. By the afternoon I was added to a Facebook group, and had friend requests from 10 others. It meant reading some pretty difficult posts about boys further on in their disease progression, but somehow that mattered less than suddenly being part of this community who knew exactly what I was going through too.

Then this morning the first thing I saw online was a comment from a complete stranger on my fundraising page. She had seen our story in the local press and wanted to make a donation. That fills me with hope for the future of our boys.

And perhaps best of all, Pudding is doing really well with his language at the moment – playgroup report that he is echoing more words and using some new ones spontaneously. He has even said a verb (‘pull’ to ask for help with his banana, and when singing ‘Wind the bobbin up’) and a personal pronoun (‘me’ pointing to himself when we ask where he is). Huge milestones!

I know I’ll probably be hit by another body-blow soon, but it is becoming easier to deal with them. We will get through this.


Of course it’s a horrible cliche that love conquers all. I’m not sure I’d agree with that, but I certainly know that love has done so much for me in the last few months, and particularly this week.

Love comes in Makaton lessons that help a grandmother communicate with her youngest grandchild.

IMG_6521Love comes in a bunch of yellow roses, as beautiful and sunny as Pudding himself.

Love comes in an acre of chocolates sent to save this committed vegetarian from a bacon sandwich.

Love comes from another mother on the playground who held my hand while I struggled not to cry in front of the children.

Love comes in a list of DIY jobs done by a friend despite running a marathon the day before.

Love comes from friends who share my fundraising page with others, and their friends who donate despite never having met me.

Love comes from my dear husband who, despite being shell-shocked himself, can make the world seem simpler and better with just a few sentences.

Love comes from a big brother who wants Pudding to go to his school so he can still see him during the day.

And love comes in a little boy who takes such joy in dancing and singing round the living room trailing a streamer of loo paper he has just filched from the bathroom. A boy who can make us smile and laugh even now.

The worst (part 2)

I feel like over the last two days on an emotional level I’m right back where I was in June and July. Tears are never far away, and it is difficult to carry on with the everyday things such as getting dinner ready, doing the shopping or washing up.

I want to disappear under the quilt and say to hell with it all.  I want to swear loudly in public. I want the comfort of a bacon sandwich or acres of chocolate.

I want the most difficult decisions we make about Pudding’s future to be which subjects he chooses at GCSE, not the choice between mainstream or specialist schools. Or whether we enrol him on a clinical trial.

I want the sun to stay out and keep the gloom of winter at bay.  I want to have a conversation with my son. I want him to walk to secondary school with his mates.

I want to choose our next house on the basis of which street it’s in, rather than whether there is space for wheelchair access.

I want to go back to being able to enjoy a novel.

I want someone to tell me that they have found a cure and everything will be ok.

I want him to bring his girlfriend home from university to meet me, so I can embarrass him with tales I tell her about his childhood.

I want him to have a quick and easy death.

I want_

I want_

I want him to live.

The worst

Yesterday I was told the results of the DNA test.

Rather than a spelling test on the gene that is responsible for making that enzyme, in Pudding’s case the whole gene is missing. So progressive brain involvement is inevitable.

The drive home was surprisingly beautiful. Sunlight escaping through dark clouds highlighted a bridge in the distance. Cows walked home across a grassy hillside.

Life continues. However unfair that seems.

A typical ERT

We get to the multi-storey and park up at about 10am. (The first couple of months I set off really early to arrive at 8am in the hope that the traffic would be better. But with the mornings getting darker I’ve tried a later time and found the wall-to-wall lorries on the M62 to be not quite as bad.)

Pudding in his buggy, we walk to the Children’s Hospital entrance and say hello to the fish on the way in. Pudding knows where we have to press the button for the lifts and again for entry to the ward. Once we’re through those doors the lady on reception waves us through – she recognises us by now.

We head towards the short stay side where the Willink team do the infusions. I let Pudding out the buggy here and he runs to find which bay we are in that day and gives the nurses a big hug each.

Most important job of the day is to choose which DVD he wants to watch first; this time it’s Jungle Book 2. Once that is on, the nurses do a set of observations (obs): temperature from his ear, oxygen levels (a sticky probe attached to his finger) and blood pressure. He’s never liked the last two being done, but accepts them now unless he’s feeling very grumpy.

Next are the pre-medications – Calpol (because he really doesn’t like the taste of the hospital paracetamol) and an anti-histamine – which reduce the chances of him having a reaction to the infusion. It’s a two person job, as otherwise at least one of us will end up being covered in sticky medicine as he bats it away from his mouth.

Then comes the bit he really hates. At home we’ve put Emla cream on the portacath site to numb it; now that has to come off and the gripper needle is put in. I sit on the bed and wrap my arms and legs around him and hold him close, one of the nurses holds any limbs that I’ve missed, and the other works quickly to wash the site and get the needle in. Pudding has always objected to being ‘messed with’ – dressing and undressing can be a bit of a challenge as well as nappy changes – and I hate having to pin him down like this, but accessing the port would be impossible any other way. It is getting easier though as he gets more used to the process and he doesn’t seem to hold it against us once we’re done!

The line attached to the needle is then checked to make sure it’s drawing properly, and if a blood sample is needed that week it can be taken. Then Pudding is released for a bit while the nurses prepare the infusion; two vials of replacement enzyme in a bag of saline. He runs down the corridor to the play room which has got loads of toys and crafts available for children and siblings on the ward. He plays with Peppa Pig for a bit then sits down and colours in a picture before dashing back to show it to the nurses – ‘Ook!’ (Look!) ‘Ta-da!’

IMG_6488Soon it’s time to get him back on the bed. His line is connected to another which runs from the saline bag through a pump. It is started at a slow rate and gradually increased so that the body isn’t flooded with too much all at once. Pudding settles in to watch his film and ask for food every five minutes. I resist as much as possible before giving in. It’s not fun being stuck on a bed for three and a half hours, so we need to keep him happy. Some older children do walk around with their pump, but I’m not sure any of us could face racing down the corridor and stopping him from yanking the line out!

Niamh chivvies me away to get my own lunch and I sit in the Costa downstairs having a panini and reading the paper, very grateful for their wonderful care of us. By the time I get back, Pudding is having his own lunch. Egg sandwich as usual. He can say ‘egg’ really clearly now, and sometimes does the sign too. Flushed Away is on now, and he smiles when he sees the singing slugs.IMG_6497

Obs are still done at regular intervals – he often seems to get a slightly raised temperature towards the end. The infusion finishes at 2.10 but we have to stay until they’re sure that he isn’t reacting badly. Pudding loves this bit – he helps push the pump back to its place and fetches the basin for washing the beds.

His temperature is still higher than it should be, so he gets another dose of Calpol, and we wait some more. By 3.10 it’s back to normal and the gripper needle can come out – only two people needed for that.

No other appointments so we can pack up our stuff and wave goodbye. Until the next week.