Sunday Roast and A&E

Today we celebrate my wonderful Mum's Birthday, and my favourite Nephew, Ben's. I can never say it enough, but my Mum is my ultimate hero. As well as being the hardest working Mum, she is the most incredible Grandma, who along with her husband Joe, have looked after mine and my sisters kids in their early years while we worked, and even now they are all in school, having them in the holidays when we can't, picking them up from school, and coming to our rescue with hot meals and fresh veggies from the allotment all the time. She is the most selfless person I know, only ever wanting to help others. 

So today it was our turn to spoil her a little, but just a few hours before they and family arrived for a roast dinner, Isaac and I were having to head into A&E to get him checked out. His longline is hurting him now, which it shouldn't. Thankfully they agreed it was not blocked, but likely his vein is irritated. Not surprising really, having housed a tube for the last two and a half weeks and pushing through pretty toxic drugs 3 times a day. It would be great for it to last until Thursday..... but it's still not comfortable for him tonight, so I'll be calling clinic in the morning. The team in A&E were fantastic, and Isaac as brave as ever, we made it home in time to celebrate. 

I promised a summary about IV lines, so here is my very non-medically trained summary; 

A cannula is the most common IV line, typically inserted in the back of the hand. A needle is pushed into a vein and a short flexible tube is pushed up the vein. The needle is removed, but the line stays in. On the end of the line is a port, which syringes (or drips, depending on the amount of drug and how slow this needs to be delivered) can be attached to, to then deliver drugs directly into the blood stream. 

Essentially, all lines follow this same principle, just in different veins in the body, in different lengths, and to last different durations. 

A longline is the same, but as the name suggests, longer. This is usually inserted just below the elbow, but Isaac has also had them in his ankles, and some veins in the groin may be used. These typically last longer than a cannula, usually up to 3 weeks. 

A PICC line (peripherally inserted central catheter) are similar to a longline, and Isaac has had these in the past too. Only they are longer again, and usually inserted under general anaesthetic. The length is typically from his elbow right up to his chest cavity, or from his ankle to his abdomen. These can last many weeks, or months. 

Isaac has not yet had any other kind of line, but Central, Femoral and Hickman lines are often used for drug delivery over a longer period, often in oncology. 

In CF, a more permanent IV device can be surgically implanted, called a Portacath usually in the chest or upper arm. Isaac is likely to have one when he needs more regular IVs as this makes drug delivery much easier. It's like a rubber bung, sitting under the skin, which can be accessed easily when needed for IVs, but at other times, is fairly invisible. It's also often used in CF due to the number of courses of IV antibiotics which damage his veins, meaning they cannot be used again. 

Our IV station; 

Happy birthday Mama and lovely Ben x

Pudding honey?

Ceftaz, Tobi, flush, with a side of Hepsal perhaps?

Home blimin sweet home.

At last. Two weeks in hospital feels like 3 years 4 months and 29 days when you are there. I was kind of hoping to get out and find that the whole Brexit/Trump thing was a whole Dallas type dream, Bobby would wake up in the shower again and none of it would have happened.... no? Hell.

Isaac has gained 15% lung function compared to admission, which is good, no.... it's great, but not quite as high as we would all like it. I am so thankful for his excellent CF team who like us won't settle for less. If we can get him better, we will! Therefore we are planning a third week of IVs, which is unusual, but may help his LF further. But I'll do these at home, which is so much closer to a normal family life. So we loaded the car with our many many bags, meds and equipment, and escaped. If his longline fails sooner we will rethink (I will post soon to better explain cannulas, long lines, and PICC lines, all of which he has at times, as I realise it is very confusing)

The fresh night air was exhilarating,  played the music so loud and sped home to be a family again, smiling all the way. Talking to another CF parent earlier who is in the same situation right now, about how you get home, and it's amazing, and you celebrate...... but unlike many other people leaving hospital, it's not over, it's never over for us. And so it's often when you get home that you crumble, after holding it together inside so well. I am not yet crumble. I am still smiling, but soon....

Huge thank you's to the Grandparents as always, coming to our rescue. We can never thank you enough. The Cayley's for always being there, Bronté for always visiting, Lautaro, Ju and Jess (so great to see you guys), Lemon, Lizzie, Gem, Jin, Hamish, James, Bon, Sarah, Gavin and Jennifer and many others for the soup, the wifi, the help, the kind words.... it really does mean the world to me, and helps Isaac pass the time.

And to the amazing staff on D2, nurses, our unsung heroes x

Hospital life part 2.

It feels like we have been here for weeks already. It is such a mix of emotions. On one hand he is relatively well, we feel like the lucky ones. He bounces around, eats non stop, goes to the gym twice a day, and is more than happy to have needles as it means more laughing gas. We see so many poorly children here, but many of them come and go. Patch them up and get them home. Whereas we stay. He looks so well, but his lungs are not.

I am with my boy, and I miss my girls, my dog, the cosy life we have made for our family in our little cottage. Dan comes to my rescue, but then it feels wrong not being with Isaac. He is brave, and he is brilliant company, but he is also at times angry, bored and frustrated, and it's me he lashes out at. Because that's safe. Unconditional. And I know this, I understand. I understand him. But it's still hard sometimes.

CF affects all of us, Anouk especially gets very sad when we have to stay in, and she's beginning to question why the poorly looking kids get to go home, but Isaac doesn't ('Is Isaac more sick than them?' She asks).

Midweek it looked possible that we would get to come home early to finish the course at home. I've retrained, so I am all set to do his IVs myself, but his test results just weren't good enough. As much as we tried not to get our hopes up, we did, and it was a big disappointment. Then we were told he might even need a third week of IVs, which he has not needed in years (two weeks is the standard length for IV antibiotics). We agree with the plan, which is focussed on getting his lung function up as much as we can, but it's tough.

This weekend he has been allowed home for the day inbetween IVs. Tomorrow we are back on the ward full time to resume the extra physio sessions too. Last night he was up until 4.30am doing his IVs which had been due at 7pm. His longline had blocked, and he has another's cannula in his hand now. They will try and rescue the longline again tonight, otherwise he will have a new one tomorrow morning. He is looking increasingly pin cushioned, and the cannula (in his hand) limits him much more than the longline (in his arm).

On the plus side, we escaped for a few hours the other day and we had some fun, and the Christmas Spirit came to the ward early this year, when every child was given a book on Astronomy, a piece of Meteorite, and a Telescope. Amazing.

He is getting better, just not quite better enough yet. Merryness will resume shortly x

All is well.

It's been a good weekend. Isaac's IVs are well timed, we get a few hours free in the afternoon, and today he came home for that short time and went out to play with friends. His chest sounds worse, but this is likely good news; The gunk is on the move. Tonight he has returned to the ward with Dan, and I'll take over in the morning for the next 4 nights. I'm enjoying time with Anouk and Rosa, we sleep in a soft heap of girlieness. But while I know he is in safe hands, it is still very hard for me to not be with him. We sleep in his bed to feel close to him.

Monday means the return to proper doctors rounds, and a whole lot more tests to see if these antibiotics are having a positive affect. We know that we are in the right place, and fighting against CF aggressively like this is right; we welcome the battle as it will keep him well longer. But it's not easy to explain to a 4 and 7 year old who just want their family to be together.

Thank you for all the lovely messages. People have said in the past that they haven't contacted us because they didn't want to say the wrong thing, or ask what might be intrusive questions. But in my experience, I would always rather people did ask, however silly they might think their questions are, that you are thinking of him enough to ask shows that you care.

Hoping to have good news for you soon. If his lung function increases enough, we may get to finish off the course at home, once I've had my refresher training as 'nurse Lizzy'. Keep everything crossed! Good night all x

Hospital life...

You know when you have settled in to hospital life again when you're in M&S buying sushi for breakfast, in your tea stained pyjamas at 11am, and you don't care. It's freezing outside, but you're in flip flops because the ward is a sweaty 35 degrees all year round. You play chess, Xbox, cards and watch a film, hoping the hours are passing, only to find it's still only 10am. You go to bed at 8pm and stay in bed until 6am, to have 4 whole hours sleep. You have lovely cuddles, pranks, laughter, and then strops and shouting, all within an hour.

That said..... It's all going well. Cannula in (uncomfortable, and in his favoured hand) the first day, long line the next. Went in first time perfectly, on gas and air (which I had to wrestle off him in the end!). This is so much much better for him, he has great movement, and should last the whole 2 weeks. He was so brave. He has a full on routine; 4 lots of IVs. 5 nebulisers. 4 lots of physio. We have been in the gym twice today. On one hand it seems like an endless cycle of treatments, but on the other, time passes so very slowly. He was very happy to see two aunties today, and for us to all eat together as a family tonight. His cousin Ben is visiting tomorrow.

All going well so far, and I couldn't be prouder.

Hoping for good results from the lab and better spirometry results (early next week) when we will know more. Good night all x

Hospital stay.

We are heading into hospital tomorrow for 2 weeks of IV antibiotics. No huge surprise. He feels OK, but his cough and lung function are worse. We are confident this will kick some serious CF butt, and we'll have him fighting fit again for Crimble season. Don't you worry! 

More soon x 

Compliance.

I've been meaning to write for a while about CF and compliance to all the treatments CF entails. To illustrate this better for those less familiar with CF, this is Isaac's typical day (when he is well);

Morning; 

Anti-sickness medication when he wakes up

Inhalers (to open up his airways), nasal sprays

*Nebuliser 1 (DNAse, which is an inhaled enzyme that helps thin the mucous in his lungs)

Breakfast (with Creon, his enzymes which allow him to digest food properly)

Morning tablets (antihistamines, salt tablets, plus three medications for his tummy issues)

**Physiotherapy to clear his mucous

Nebuliser 2 (Antibiotics, Colomycin or Tobramycin, to fight his infections)

Afternoon; 

Lunch with Creon again

Evening; 

More inhalers 

Nebuliser 3 (Hypertonic saline, to draw more water into his lungs, to again loosen the mucous)

Physiotherapy again

Nebuliser 4 (Antibiotics again, to fight his infections)

Evening tablets (vitamins, oral antibiotics, many more tummy related medications)

Night;

Possibly more Physio, depending on how he feels

*A nebuliser is an electronic device which breaks down particles of medication into smaller blobs (sooo scientific, me) which are then better delivered to and absorbed in the lungs. Each nebuliser takes him between 10-30 minutes, and he has two different machines, because of the different drugs he nebulises. We call these 'nebs', and anyone walking past our home at any given time would have a pretty good chance is hearing us yell 'neb Ise!' as his concentration on these is lacking. 

**CF Physiotherapy can be of different kinds. He has small devices which he can breathe through against resistance, which help shake his lungs. Some of these he can do hooked up to a nebuliser at the same time, which help him clear his chest on exhalation, and then deliver the medication on inhalation. He also has chest percussion when unwell, which is the classic 'pat him on the back' physio that most people remember about CF, we call this patacakes. 

His current prescription list is 22 medications long, which we need to ensure we have in stock at all times. And due to crazy NHS budget rules, only some of these can come from our local pharmacy, the others we need to get from the hospital pharmacy. In addition to this, he needs to take enzymes (Creon) with everything he eats that contains fats or proteins (pretty much everything aside from plain fruit or veg) because of a rubbishy pancreas he produces none of his own. This varies according to what and how much he eats, usually around 30-50 capsules a day. 

There is a strict order to his treatments; for example, we want to loosen his mucous first, then help it come up, and finally throw antibiotics in to make war on the bacteria, when they have clear access. Also, many of his drugs have interdependencies, side effects, or just... effects, which mean you need to take them at a certain times of day, with or without food, or avoiding something else. This can be mighty hard to get right all the time (try giving babies antibiotics four times a day which can't be taken one hour either side of milk...!). 

Reader, I can't imagine how that reads to you..... Is it a lot? Less than you thought? 

To us, this is our norm. This is our good day. Our minimum. It can be much more when he is not so well; for example, he has occasional courses of IV's (Intravenous Antibiotics) - this might be because he is more symptomatic than usual, or because his lab results reveal something new (they culture samples from his mucous to see what bacteria are growing in his lungs). This means two weeks+ with an IV line in; antibiotics and physio galore. 

But we're lucky, IV's are an occasional occurrence for us still; many other kids and adults with CF have regular IV's - two weeks every three months (at least) simply to keep the infections at bay. There are also other complications of CF which we don't have to deal with yet - diabetes, liver issues, problems in maintaining weight (resulting in PEG feeding directly into the tummy to cram in the calories overnight).... The list goes on, and each of these would add significantly to the treatment burden. 

There is not a day that goes by where I don't think how fortunate we are to live in a time of such great medical advances, to have such a wonderful NHS, and such a supportive CF team. I even pass by our ever expanding meds cupboard sometimes and admire the neat pharmaceutical packages, so pristine and white, the syringes, the wipes, the nebuliser (retail cost £3K), all lined up and ready to join us in battle against those pesky bugs. We don't moan about these treatments, we are thankful to have them. 

But it is so hard for Isaac. On a good day, the above will probably take him one to two hours. On a bad day, three to four. On IV's? It's pretty much like a full time job. Just to stay well. He is 11 years old. He wants to be out playing with his friends..... he wants to be like everyone else. He doesn't talk about CF with his friends, and even taking Creon in front of people is an issue for him. Anyone who remembers Secondary School can understand that. When I was 11 I too wanted to be like everyone else (when I was 21 I wanted to be like no-one else). Right? I am ever mindful of how he must feel, but also need to get him to do all this, because the alternative is him being less well, and likely resulting in long term lung damage. That is not an option to me. He has an understanding of how important his treatments are, I tell him, his Dad tells him, his CF team tells him, and yet he still cuts corners wherever humanly possible unless we watch him the whole time (which with two other smaller children is difficult to say the least)...... The nebuliser which he insists he finished properly while I was in the bathroom, the pathetic blows of a mouse doing his physio, the 'forgotten' Creon, which then gives him a rotten tummy, and makes doing effective physio the next morning even less appealing..,.. 

I get it, I really do. I get him. I know him better than anyone. And if it were me? I'd have probably done the same at 11. But it doesn't make it any easier to watch, and getting him doesn't always make me any better at reasoning with him. We have this closeness in hospital where we can talk calmly about it, and I do anything to not sound like I am nagging him, I've drawn blood biting my tongue so much. But  when he's frustrated, and angry, and feeling like his life is so unfair, what to do? So I agree - CF does suck; or I tell him how many more people are worse off; I bribe, I bargain; I think up wacky new ideas to put the fun back in treatments; but sometimes.... I have nothing. I'm just mad too, not at him, but at CF, and how does that help the situation? Not one iota. 

Some days there is no issue at all, he just gets on with it. 

Other days I know we could have done better. 

On the worst days, I'll walk the dog for hours, just procrastinating, knowing the next round awaits me when I get home. 

Some nights this keeps me awake, thinking, have we done our best by him today? 

To support the Cystic Fibrosis Trust who fund research into CF please click here. 

I'm sat in front of my fire, with the dog on my lap, listening to this beauty, put it up loud, it's a grower, I promise. Goodnight all x