antibiotics

so this was the solution, I make it up daily! masses of equipment for what I thought would be a simple course of antibiotics! Feel like a proper nurse now!

end of EEN

We have had a busy end of term and start of the holidays! When he remained symptomatic they decided he needed blood tests that day, but by the time we got to our local hospital it was 6pm, out of hours and it took 4 hours all in! This also set back his finishing the exclusive formula diet which he was very brave about.

At the end of June they called to ask if we could bring Aaron to Great Ormond Street the following week, not sure whether for 1 or 2 weeks, so they could assess him, introduce foods (after 9 weeks), see how his infections were doing etc as he now also had inactive c-diff along with the adenovirus and it was getting hard to work put what was what. It was a quick turn around from getting the call Friday pm to going in on the Monday after putting them off from admitting us over the weekend so we could attend a CICRA crohns family day, when they have a bed things move fast! The admission sadly and happily came just as he started to improve, isnt it always the way, hence food reintroduction went fine, if a little fast for me upsetting his bowel a bit. He also had to be in quarantine whilst there due to the other patients, which meant things were pretty dull, but luckily his favourite activities transfer well to a hospital bed Lego, DVDs, art! Once home we had Aaron's long awaited celebration day at the beach!

 He also managed to go to his eagerly anticipated Beaver-Scout camp day accompanied by Tom

HE remained up and down symptoms wise, his doctors unclear whether it was one infection or the other or the crohns not yet settled. The first day of the holidays saw another infusion day, not quite what he had in mind, so we had a short walk by the South Bank and an ice lolly treat to improve the day, however by the train him he started to feel unwell, and then spent the rest of the evening being sick. Seeing as none of the rest of us caught anything for his next infusion the plan is to pre-dose him with antihistamine and steroids and keep him in for 4 hours for observation in case this was a medication reaction. We hope this is not the case as this is currently one of the last treatment options, we anxiously await the next date at the beginning of term...

A week after that and unrelated as far as anyone can tell he had a fainting type thing - vasovagal episode. One minute he was about to start lunch the next he said he felt sick, then went white as a sheet even his lips and ears and could barely hold his head up, before sort of falling in and out of 'sleep', this lasted 20-30 minutes, an ambulance took him to our local hospital but they could not find an obvious cause although it does have some links to crohns and his blood pressure was a bit low.

Next problem is they have decided they would like to treat his c-diff in case it is causing his bowel to still be unsettled, but the antibiotic they would like to use they cant get hold of as a liquid, they assumed he cant swallow tablet and he has had a real problem with them since he took the methotrexate that made him so nauseous, in fact he cant even watch us take tabs still. The other option is he takes the iv solution orally but this would have to be made fresh daily, so a daily pharmacy trip and we go away on the 11th!!!

Josh on his appointment day with Minion glasses on! He is seeing the benefits of all his hard work with daily physio with lots of improvement seen although not up to average yet for his age

Ethan has had a great first year at Secondary school

7 weeks EEN

So Aaron had the scopes before Easter, they were bad so was admitted for iv pred(steroid) and to start the new medicine vedolizumab

He got better very fast on iv pred, but dipped a bit energy wise when he came off that. He was pretty unsymptomatic for 2 weeks, but then he had increasing dia and blood so he had to started EEN and settled after a week except then approx every 3 days he had a little dia and maybe some blood, slowly increasing in his typical pattern. We mentioned this to his Dr at infusion #3 and she said we might need to do TPN and we should also give in a stool sample, unfortunately the nurse sent this for the wrong test! 

infusion

We were relieved a couple days post infusion as his stomach really settled to formed once a day stools.
He finished the steroids this weekend after a delay in reducing these and the plan had been to wean back to solids but he has started dia again. Its hard to know if this is due to a crohns flare up or a positive stool test to adenovirus (he was retested a few weeks after the error)
He's desperate to start eating after the long 6 weeks but is being so patient considering its really hard to explain all this to a 6year old when we don't know what we're doing ourselves! 
We are waiting on a plan from our Dr but she is away until next week, he is ok but not as well as he was, despite still being EEN. He has done amazingly well and completed 7 weeks no foods or drinks except formula with no tube either!

frozen formula 

smart at a wedding

Back on 'feeds'

So we came home on the steroids, and noticed quite soon he felt a bit more tired than we were expecting, usually on steroids, and initially this time too he is pretty 'bouncy' and a bit moody, but not really this time.

By the 2nd week of being home he had more frequent and softer stools, he also went up to London for his 2nd dose of Vedolizumab and a clinic appointment, it was a long day we left 9 and got home at 9! His doctor was happy to keep weaning the steroids and said to keep an eye on the tiredness and not to worry unless he also developed bowel symptoms.
By the 3rd week he started on the Tuesday with very loose, quite bloody stools. They immediately wanted him in for blood tests to see if anything else was going on such as a virus, but he seemed well in himself. The results of the blood still showed an off kidney function plus his white cell count and specifics were lower than ever, even when he was taking meds to deliberately suppress them. All afternoon Wednesday he complained of stomach pains and nausea and by late Wednesday eve he started a cough, with the low white cells the GP wanted him on antibiotics and he had a day off on Thursday. We then made the decision with his nurses that whatever was going on he would benefit from a few days of gut rest and started back on his formula feeds, this time orally, and he managed a half day at school Friday.

By the end of the weekend we felt the bleeding had improved but the consistency was still very loose with some soiling so the dietician said she'd rather keep him on the feeds for a further week and review.

New treatment

So as planned we went into Great Ormond Street for Aaron's colonoscopy etc on Tuesday 11th. The plan for day #1 was first start bowel prep laxatives (yuck!) then his Humira injection followed by a dexa bone scan, basically an xray to see how his bone density is following some steroid use and planned use. That night we were due to stay in the patient hotel across the road but arrived to be told we had been moved down he road, after explaining to the accommodation officer that it wouldn't be possible to feed aaron on his clear fluids diet without catering facilities, they rearranged things and got us back in the hospital accommodation, lucky that i knew the ropes having spent 2 weeks in with Josh recently!

The next day he had the scopes, but on return the Dr took me to one side and showed me the pictures of his insides and that instead of meeting in 2 weeks time as planned to discuss treatment, we needed to admit him and start straight away. She initially said he'd need a week as an inpatient, so i suggested possibly moving him to our local hospital, however it later emerged this was not possible due to the new medication he was on not being one they were familiar with. He was on a drip, but i'm unclear what for as it was all a bit of a blur, i would guess fluids, whilst going to the toilet the alarms for this kept going off and in the meantime he had had quite significant bleeding in the toilet, all a bit stressful in a small space!

He was then transferred to a ward, the only available bed was in the private ward which was nice, in particular being served afternoon tea gave me a much needed 'pick-me-up'.

 There was then an issue with getting hold of the new medication he was being put on, due to the trust not holding it regularly, bank holidays etc. We were told at this point he would need to stay in for IV steroids until Tuesday, we were both quite upset by this as it would mean missing Easter and all the plans we had. Luckily for us they later found that another patient had not come in for their treatment of this new drug vedolizumab (how amazing seeing as its not widely used!) and so he was set to start this on the Friday as by then it was getting late to start the infusion and 6hours of post treatment observation he would need.

We also had to transfer wards Thursday or the bank holiday as they have reduced numbers of patients over the bank holidays. This involved wheeling all the patients on their drips down the coridoors, i think he quite enjoyed it! He was pretty weary still and had a long afternoon nap i was very jealous of!

That night he had the nurses worried as his heart rate kept a very steady low rate of half what they'd expect for his age, he does often have quite a low reading, and has since I was pregnant, this combined with being deeply asleep from a traumatic day and steroids having a lowering of the heart rate effect, was they eventually concluded, but it made for a long night of alarms going off and not much sleep for me.

In total he had 3 days of intravenous steroids, and the infusion and started on an oral stomach acid suppresser to combat steroid side effects.




We were relieved to be discharged on Saturday in time for a little bit of Easter before straight back to school (who have promised to keep a close eye on him), and after a difficult journey home due to engineering works and a bit of a detour, we made it home.

The plan is for him to stay on the steroids until we see his Dr next week (who was on annual leave for Easter) and then begin to taper them slowly. There is another lower dose steroid he may need to be on too, perhaps longer term, and of course the new infusions. Once off the steroids it remains to be seen if this maintenance med will finally be the one that works as its been 4 years of treat and watch him relapse so far, and options are getting fewer and fewer. This is sort of what we were expecting treatment wise, we knew he was flaring up again, but his symptoms were not the worst he has ever had yet his insides were the worst they have been, it all happened rather more instantly than anyone had thought. We are very glad to get him off the low dose chemo though so every cloud.....
For now he is eating as normal but tube feeding is another treatment option we will discuss next week. His next infusion is also due next week, so it will be lots of London again for us!
As previously he is quite bouncy on the steroids and can go up and down mood wise very easily, and is still suffering the side effects of the previous meds, mainly food associated nausea.

boys

So an update;

The second week of physio went well, the kids from the 1st week were all on the same floor of he patient accommodation and it was shrove tuesday so we had a pancake party. It really helped them with their PMA! The end of week assessment showed an increase of 12/30 to 28/30 where an average child his age would be 30. He has to continue the physio at home for 6 months but i can already see him struggle with controlling the exercises. The other interest of the week was we saw OT who looked in detail at his hands, trying 3 sorts of splints before deciding they would not work and he needs to gain some strength first. All his thumbs joints and finger joints are hypermobile and his thumb even dislocates under pressure. She took photos in the end of his very unusual hands for her teaching! Not great news for Josh though.

patient accom lounge

bedroom

by the gym

Aaron held on off steroids whilst I was away which is what we had prayed for but did have a little blood the day I returned! Since then his stools have shown the decline we are becoming used to and he has a little blood most days.

top of the bus in London

We saw his Dr last week to make a plan earlier than before assuming he will continue his downward spiral. She has taken tests and the plan is to re-scope before Easter and if he declines too much before then we can 1st add salofalk then 2nd budesonide (steroid) again but for longer. If he needs steroids he needs a Dexa scan to look at his bone density, she will hopefully arrange this for when he is in having scopes or at follow up. There is also the option of more tube feeds, but it is not one we prefer right now. The question or us is how to keep him stable after treatment.

A Beaver Scout

Physio residential

http://www.nhs.uk/Conditions/Joint-hypermobility/Pages/Treatment.aspx
Josh and I have been away this week and are back for the weekend before returning for another week of intensive physio for his hypermobility and muscle weakness. This takes place at Great Ormond Street hospital and we stay in their patient hotel, he gets 2.5hrs in the gym everyday! He's pretty tired but determined 😁

Treat at Pizza Express 

Topic visit to British Museum

2017

So since the last post;
My labyrinthitis cleared up surprisingly quickly although I still get the odd funny turn.
Josh's tummy took its time settling down after being cleared out, but he is back to having bouts of unexplained diarrhea again. The current theory is as all testing has come back ok, it is a functional bowel problem, probably caused by all the problems and interventions he has had over the years.

Aaron turned 6

Aaron continued to get worse despite the change to methotrexate (mtx), he also developed quite an aversion to the mtx due to the side effects he experienced, and consequently a real phobia of all tablets including the anti-nausea meds! We are doing well now with hiding these in other things and dissolving them and he is coping a bit better, but not before lots of tears and even actual vomiting. also the folic acid became problematic so we got that prescribed as a liquid. As it had not been a full 12 weeks of trying mtx we couldn't count it as a failure, despite worsening bleeding and diarrhea, anaemia and weight loss! So in early January he was put on budesonide, a steroid, which has settled things down finally, he is now weaning off this so it remains to be seen how it goes when it is stopped.
Aaron also had an eye injury involving 2 trips to the hospital to get it checked out.

 There was of course a nice Christmas break and Ethan turned 12!

GOS Christmas party

trip to see Santa at the Llama park

Christmas Day

smart boys

The new year saw a new batch of Humira, with the citric acid taken out, we can't tell you the difference this has made, after some numbing cream for the needle, he can hardly feel it! What a turn around for the crying and screaming.
Coming up after half term we have Josh's 2 week residential stay for physio for his hypermobility and weak muscles. This is meant to make a big difference, but will of course take a lot of planning for the rest of the family as I have to stay with him.

purple sale we held at school on their purple friday

purple friday