Rare disease day

It's Rare Disease Day. Let's raise awareness!

In general, raising awareness days piss me off. I wish people would remember those affected the other 364 days a year. I wonder at any lasting legacy from these days. So many days making it almost impossible to support them all. 

Does the sheer number of awareness days actually minimize their overall effectiveness? 

How can raising awareness be of value and actually cultivate positive change? 

Let's have a think. How do we raise awareness? The simplest way is through social media. But is it through sharing stuff like this on Facebook? 

Click 'like' to send a prayer because if not you don't care! 

Share this horrific photo of a disfigured and neglected child to show you care! 

The bullies at school don't think I'm pretty click "like" if you disagree" ( usually child with an obvious additional need using a big sign). 

Look here's a photo of disabled child crying. Quick! Click "like" and "share" to show you care people. Raise awareness of disability. CLICK LIKE AND SHARE OR YOU ARE ALL GOING TO HELL! 

Here's a child on life support all tubey "share" to show you care, "like" to send an amen. 

Now please don't misunderstand me. I know when people click on these things and share them it's usually because they do care. Or want to look like they care. Or are scared of going to hell... Err one of those anyway. 

 

But I think what pisses me off about all of the above is that people think they are making a difference, that they are helping, and I'm not too sure that it does. But then I'm not a fan of shock tactics for awareness anyway. There are too many questions to be asked about how it maintains the dignity of those involved. Or if they could consent. Or is it actually a photo of what it claims to be? 

Then there's life stories. I suppose my blog fits into this category, and so do those belonging to many of my friends. However families opening up about their constant battles, heartache, or family life can end up somehow twisted into inspiration porn. People "hold their children closer", "appreciate what they have", assume we are "amazing" and "strong", and err "inspiring". They don't know how we do it. God only gives special children to special parents after all... 

Meh. 

But maybe these things do raise awareness? Even just briefly. Even just a little bit. Maybe that's good enough? For some people I don't doubt these likes and shares are a token gesture, for others it's a genuine concern and a recognition of need. 

I do think many people fail to grasp the difference between sympathy and empathy, and the significance of this difference. It's all very well balling your eyes out at Children in Need, hugging your kids then telling everyone on Facebook how upset and heartbroken you are for these families. But how does that help anyone? Can you put yourself in their shoes and think about how you would feel? What you would need or want for your family?  Yes your donations are nothing to be sniffed at but surely a societal shift towards helping and supporting vulnerable communities would actually be more practical use in the long term. 

Maybe stand up against hate crime.

Maybe support the NHS.

Maybe encourage your children to accept difference as nothing scary, and nurture their accepting nature to grow and develop. 

Maybe don't nod and agree as you hear people down the pub criticize ill and/ or disabled people with accusations of being cheats and work shy. 

Maybe object to cuts to community based care and services.

Maybe don't assume that if you haven't heard of a syndrome before, or if someone is still undiagnosed, that their issues aren't significant and life affecting.

Maybe start to appreciate that the rarer something is, the less information and support there is for everyone involved. That research into the rare stuff doesn't happen much. The money isn't there and quite often the motivation isn't there either. Rare can mean tricky. Rare can be shitty. Rare or undiagnosed can mean services, resources and equipment can be even harder to access. Disappointingly the little box you get to tick now you have a diagnosis doesn't help as much as you thought it would. The treatment or therapy options you thought you would receive don't even exist. 

In the ATRX group we often talk over the issues our boys have and there aren't any clear answers. So we struggle on. 

Ah that's why the awareness day is important. 

*toddles off to share some shit on facebook* 

School holidays

So it's half term and teachers all over the country shout "hurrah", rejoice, reach for their wine, and happily ignore that pile of marking in their boot until, umm Sunday? Parents may rejoice less so, particularly if they work and have to fight feelings of general shittyness as they miss out on the fun part of parenting and instead juggle childcare arrangements. Or maybe they are strung out trying to fill the week as expected by our competitive society with a mix of play and semi educational visits while trying not to end up completely exhausted. I'm worn out simply writing that. 

Supermarket sales of wine must go through the roof during school holidays. 

And what about parents with disabled children? How does a week without school impact this group of already exhausted and stressed out people? The simple answer is, well, as you might expect really, a HUGE amount, the main issues being:

1- Childcare

2- What to do and where to go 

Issue 1-  Childcare 

There is an assumption that this ( my) group of parents don't work. Whether it's calling to chase things up, ordering prescriptions between certain hours, health professionals arranging visits and appointments, deliveries of feeding equipment and pads, or those large pieces of equipment your child really really needs however when you attempt to barter a time, or even an AM/PM slot  with them end up made to feel like you've asked them to walk through fire. We are expected to be available, and not whinge about it. But that's another blog post entirely. 

The "system" isn't set up for us to work, let alone care for our children during school holidays. There are huge gaps in childcare provision for a start. I've seen friends try to get back to work, or continue in their pre disabled child work and have to give up or take a break from it. Those who have succeeded tend to rely on family, friends or part time and flexible working. Not to go on and on about it but the benefits of working are huge to my group of parents and their families, even without the financial necessity for many. 

Issue 2- Where to go and what to do

There is a huge campaign currently around having accessible toilets. This is something I keep in my mind when planing longer days out or trips away. Finding somewhere suitable to change Nate can be tricky. Floors are disgusting and then there's the problem of actually lifting him. I don't expect everywhere to have suitable changing facilities or for everyone to understand initially the need for them, but if you are a shopping center/ "mall"/ theme park/equivalent large business or new build it would be nice if you would make an effort. It's just good business sense. If there's a choice between two places I'll stick my neck out and say "we" ( the royal "we") would head to the one where we can change our children or family members. My biggest shock in this respect was when visiting Disney World and coping with their lack of facilities. 

I have been pleasantly surprised at the smaller venues who really make an effort with changing facilities such as Alnwick Castle and Gardens, Seven Stories and the Discovery Museum. With changing places toilets available in Newcastle's Intu Eldon Square and City Library any activity in the city centre becomes easier and less stressy. Equally shopping or taking part in activities based in the Metrocentre or Galleries in Washington become options. Frequent options. I stick to what works. I can't even begin to describe how difficult changing a non mobile mixed tone 6yo can be. If I'm being totally honest I would actually choose to stay home than have to battle with this by myself. Having Michael around helps considerably, but usually during the holidays it's just me. 

The next thing to consider is how accomodating places are. 

Are they disability friendly?

To be honest I settle for disability aware or just plain " not being a dick", but what I don't want to do is focus on the negative experiences we have had. 

This week we have taken Nate bowling at the AMF in Washington, and to a relaxed screening of Moana at the Tyneside Cinema. Both of which were made hassle free by great staff and accessible buildings. This was the first time we had braved the cinema with Nate. *milestone klaxon* We couldn't take him to a standard show as he might suddenly transform into "captain whingey pants", "mc screamy" or simply shout away happily much to everyone's annoyance. Relaxed shows in general are great. Of notable mention is the Sunderland Empire Theatre who put on a variety of shows and we are currently eying up the relaxed orchestra performance on Mother's Day ( much to Michael's dismay). Many venues taking accessible shows and events seriously have named staff who's roles are to arrange relaxed/ accessible events and gather feedback. A friendly face goes a long way   

in a society where ignorance and selfishness can spoil simply leaving the house. It means a great deal to do "normal" things. 

I feel that it's important to highlight the places who do a good job providing for people who have disabilities and their families as much of what we see on social media and in the press are stories of nightmare visits and ignorance. 

I don't like being a moany fucker. Honest!

One store we are always made to feel welcome is Lush. I do sometimes wonder if there's a photo of our family begind the counter with the tag line " engage with this lot- they spend shit loads in here", but truthfully it's probably more a reflection of their diverse staff, disability awareness, or simply good recruitment practices that mean within minutes of entry Nate is either having a sniff of something fruity or his hands in some fizz ( with our permission of course) and no awkwardness at all. We shall be invading a curtain Lush store this Friday ( as long as it's not too busy) as rumour has it they will be making some of their lovely soap bars. It's always good to be able to wheel around a store with ease, unlike our experiences in a certain sports store I'll not name where the only comparison I can think of is to some sort of fucked up obstacle course where the wheelchair user ends up looking as if they are attempting to shop lift once ( if) you make it to the other side of the store. 

So this week Thea is hugely chuffed that we've finally visited the cinema, all of us. TOGETHER. It's another small slice of normality pie for our family made possible by sense, awareness, and a bit of luck ( Nate's health). 

But that's not to say I won't be hitting the wine tonight with you all. 

What a difference a dog makes

Last September my mam's doggy Daisy gave birth to 7 mewling mouse like things. Our daughter Thea took great delight in pretty much living at her Grandma's house while helping feed Daisy, keeping the puppies clean ( she is an excellent poo picker upper) and socializing them. She thrived in this position of responsibility and formed a particular attachment to a little pup then named Sully ( green collar). 

Yes ok so I spent a large amount of time with the puppies too. But seriously PUPPIES!!  Although the one pup I didn't get to cuddle much was little green collared Sully due to the inseparability of Thea from him. 

As we approached the time the puppies would be going to their forever homes we started to consider keeping one. Not that we would have much of a choice as Sully would have had to be surgically removed from Thea to make room for a different pup. We started to weigh up the pros and cons of having a dog. It is a huge responsibility after all, and I was more than a tad reluctant. I worried we would struggle with him when Nate's health was poor, about house training, and about how he would "be" with Nate and all of his noisy equipment. A yappy or nervous dog wouldn't cope with our usual chaos and the variety of people who come to the house in their profession capacities. But here was a little girl who bloody loved this puppy, already adept at caring for him, who has quite a tricky life at times. And me, didn't I deserve something fluffy and positive? I was convinced Nate would benefit from the sensory experience of fur and a good lick. Michael was doing a decent impression of not being overly fussed about the puppies in general ( liar liar pants on fire) but came round to the idea fairly quickly, and before I did. We knew what we were taking on having both grown up with dogs and that made us pause, think, and then make a carefully considered decision. Many, many people think that we are quite mad taking on a dog. But what is one more piece of madness in our lives? 

We had made the decision a long time ago not to have any more children, even before we found out the fun fact that I am a carrier of a faulty ATRX gene which causes PMLD and very serious complex health issues in boys. I couldn't have coped with another baby, healthy or with ATRX. Not alongside all that Nate deals with on a daily basis, our constant lack of sleep, and already struggling to spend quality time with his sister. This dog could complete our family in the way only a dog can, forcing us out and perking us up when things get bad and we are feeling low. 

Sully met Nate several times at his most manic where he is hot, sweaty and bit thrashy aroundy ( very typical of ATRX boys). 

The little puppy just curled up on his lap and went to sleep. I think this was the moment I thought "yes".

Sully became Freddie and as soon as he was old enough we brought him home. 

All puppies need a great deal of socialization but Freddie needed the additional experience of our sats monitor alarming, suction machine ( he doesn't like it when the catheter goes in Nate's nose), oxygen concentrator and ventilator noises, feed pump, and being around a shit load of tubes. He has dealt with everything extremely well with the exception of a fascination with drainage bags and a few opportunistic licks of the feeding tubing. 

Thea's relationship/ obsession with Freddie was never in any doubt but I had wondered about Nate and Freddie, however the boys get on well. A bit too well. Freddie likes to give Nate a good lick hello and check how he is. Urgh. No objections from Nate in that respect. 

The real positive is in how Nate reacts to Freddie. He loves lying on his mat and feeling Freddie race over it, him, and around the room. Freddie will snuggle up to Nate giving him some comfort, and most recently Nate has started to interact with Freddie; holding toys hostage and grinning as he pushes Freddie away. Heart melting. 

US? Well we get the fluffy cuddles, enjoy amazing greetings, get to walk Freddie and play with him in the garden. I'm not going to lie the early morning wake ups after a dodgy night with Nate are quite hard, as are early mornings when we have care in and could have stolen a few extra hours of sleep. House training has me demented. One day everything is done outside or on a puppy pad, the next day there's a huge poo on the floor and I'm  convinced this is in direct retaliation for me leaving him. Then there's the weird shit Freddie finds and eats. Seriously where does it come from? "What the hell is that?", is a frequent expression when picking up poo. We've already had one vet trip due to eating plastic. There does seem to be part of Nate's mat missing so that probably explains that. 

The dog is a huge pain in the arse at times but at least he gives us something to talk about.

Our fluffy pal.

Getting the care right

As time ticked on my stubbornness and what some may consider as self imposed semi martyrdom slowly gave way to a more open mind with regards to care.

 "No we don't need any help" changed to "oh fucking hell I am so tired can someone please take my son and care for him while I down a bottle of wine and pass out from tiredness".

When we began our journey with Nate over 6 years ago I couldn't have envisioned any possible situation where my son stayed out of the house to give us "a break", or that I would have strangers in our home caring for my son overnight ( not that they are strangers anymore). Part of this attitude was a direct result of his anticipated short life. We wanted to be with him as much as possible, he is our son after all. During Nate's stay in SCBU we took over all of his "cares", adopting the attitude of self reliance which was to continue at home. 

Fast forward a year or so of sleep deprivation and hospital stays and suddenly we were thinking of allowing someone else to take on some of our caring role. This decision can be a huge mental challenge. It involves trust in an as yet unknown quantity and a little bit of bravery. While liberating you to parent/ cook/clean/sleep/drink gin you kind of have to accept they will never do things quite like you would, or quite as well as you would. This is ok, as long as it's close. We have super high standards after all. That's not to say we should compromise our standards and accept sub level unsatisfactory care. Often as parent carers we are made to feel "grateful" by service providers and professionals for the care packages we have. I mean yes, clearly I'm feeling really fucking grateful I need help in looking after my son. Cheers. 

What i do "get" now is that what works well for one family who have a child or young person with a particular need won't necessarily work for another family with the same additional need. For example not everyone likes the idea of having carers in the home. Me, personally, I sleep like a log. Other parents use family members or friends and would never ever consider carers in the home or respite provision. Some things just aren't a good "fit". All of the available options need to be explained for parents to make informed decisions. 

Family dynamics are complicated, and mental health and coping capabilities play a large part too. Combine this with varied levels of education, experience and energy levels ( energy to rant and harass by phone or email), and also education, experience and energy levels of the professionals representing them what two families with similar young people come out with could look vastly different. Best case scenario is that this differing end product is the result of careful assessment based purely on need and not who has shouted loudest. 

Any given care package seems to depend on 3 things ( in my opinion) :

- what you think you would like ( i.e. what  you are aware is available) 

- your eligibility ( how many top secret boxes you tick) 

- what funds are available 

Actually getting a care package put in place involves detailed assessment(s) and basically your life "going to panel". Panel suggests a team of people pouring over the intimate details of your family life ( I think this is what is supposed to happen), but is often, in fact, someone sitting drinking coffee with a pile of paperwork at their feet. Or an evil genius stroking a white cat. I like to picture that version. In some areas there are separate social care and health panels due to pots of money ( anytime pots are mentioned I always imagine a gold pot like at the end of a rainbow, no idea why), but ours now has a joint panel ( I now have an image of two cats in a gold pot on my head, and they're fighting). In the last few weeks I've had two detailed conversations with health and then social care about Nate's health needs and why we are asking for more help. Every little change seems to require another in depth look at "How Shit Nate's Heath Has Been" or HSNHHB assessment. Catchy I think. 

I've rambled slightly but what I think I'm trying to say is that there aren't really many options for care ( and some are kept top secret), and that it can be tricky to access, especially as our children become young adults. Care in the family home with hospice short breaks or residential short breaks if you qualify, building extensions, living in their own home or in a residential care setting are pretty much the standard choices, and all of which depend on what funding is available. None of which are easy options for the young person or their family. This isn't something I think a great deal about for obvious reasons, although you never know. We have all seen articles in the press regarding independent vs residential living, inclusion vs the appearance of institutionalisation. Some headlines are about home scandals or where choice has been removed due to lack of funding. What must be remembered, however, before anyone turns into judgey mcjudgerson ( which seems to be the trend when talking about what we don't totally understand) is that residential care can be very different from what you are already imagining, as is hospice respite ( clue it's not depressing or all about death) and that above all whatever care is put in place it must be right for the young person. 

What works for one young person and their family doesn't work for all. 

They must be safe and happy, and their families must be satisfied they are safe and happy, regardless of who provides the care. 

Throwback post from 2 years ago- When did this become the new normal?

I had a bit of a moment today. Sat in Starbucks ( again) #dontjudge
I was feeding Nate his lunch ( a blended sandwich and fruit) and noticed people watching. Without any sense at all I thought to myself
" what! It's as if they haven't seen a child peg fed some blended food before?"
Well of course they hadn't, what a ridiculous comment (luckily not said out loud) . I mean why on earth would they? 

People are curious creatures. So they look. Especially children. I don't mind the curious looks anymore, but at one time they would make me sad. Sad that our lives were so unusual that people found it interesting. ( looking is not  to be confusing with gawping or staring ). As parent to a child with complex health need and PMLD you go through various stages of emotions. In the beginning it was shock and grief, that then moved onto injustice and and anger. I felt that everyone should understand and appreciate how difficult out lives had become and became frustrated when they didn't. ( note to past self Rachel- people can't read minds).  It's at the stage that you need the company of parents in a similar situation. You read SN blogs, stories and captions that tell you how amazing you are doing what you do and pftt society for not knowing or understanding. The problem with this is you can end up shutting yourself off from the rest of the world. And while feeling safer, long term it isn't helpful ( in my opinion) because the world is still out there waiting. The longer you leave it the harder it becomes to reintegrate. You can't want people to understand your life and your new normal unless you put yourself out there, difficult though it may be.

A boy once ran up to me in in our local park to ask about Nate's nasal cannula and oxygen. I was quite happily answering his questions as his mother dragging him away apologising profusely. There's that socially awkward area of what's acceptable or not around disability. It varies from person to person, whether the disability affects yourself or your relative, which of the "stages" you are in, and if the individual understands what people are discussing. For me personally unless I'm completely sleep deprived and needing a caffeine infusion, or having a general "I hate the world day" I'm ok with questions from children and curiosity in general. What I don't like is the ability of random adult strangers to offend or insult. As an adult you can't get away with asking " what's wrong with him then" or "can they not fix him". Just no. It isn't any of your business and quite frankly you should know better.

I feel lucky to live in a time when children like my son aren't hidden from view in a hospital  or institution, and talked about in hushed sentences. People with no experience of additional needs or disabilities need to become accustomed to seeing differences as.. well.. just that. Difference. Not bad or scary just different.
The last time we had sat in Starbucks as a family, a mother at the next table remarked to her child who was curious " the little boy is just having his lunch like you are but just down a tube instead" followed by a nod in my direction. Admirably dealt with I thought. Not that i spend a great deal of time drinking coffee or anything... or that when my friend went into said coffee shop without me they asked her where her friend was...

Anyway. My original point was " when did all of the kit get so normal?" So normal in fact that my subconscious expects the whole of society to be totally unfazed by my messy syringe feeding. In fairness they may have been watching in the hope they could  dodge anything sprayed in their direction.

I'm not sure when it happened though. This acceptance of the new normal. I went through shock, grief, anger, injustice. So when did I hit "acceptance" ? I wish I could remember when. I feel as if it must have been quite a turning point for me emotionally; heralding in a new age.

And I missed it.

Pants.

I wonder if there is some sort of equation for it? After x days and y hrs multiplied by number of days spent in tears you will finally come to terms with your new normal? And the equipment?  Equipment is funny. Actually it's not funny in the slightest. It's more funny how you accumulate it over months or years without really noticing it happening. It creeps up on you. One minute it's occasionally an ng tube, then it's a button, spare buttons and shed loads of syringes. It's talk of a special bed, and then it's hoists and slings. The mention of oxygen, and suddenly you are up to your eyes in cylinders, masks and cannulas. We are lucky in that this accumulation has happened so gradually. I remember being terrified of the ventilators and mask, and oxygen tubing in the beginning. Actually if I'm honest I was more than a bit scared of Nate. Babies are terrifying needy little things at the best of times but I was so scared of not protecting him and keeping him safe.








So yeah. It's all perfectly normal to me. This amazing amazing gorgeous little boy makes us very happy.  He may make odd noises and really bad smells, but show me the 4 year old boy that doesn't.