T+365. Signing Off.

Well, one year on - almost to the day - and we have been signed off by the BMT unit.

When we started this process by Saffy being recommended for a transplant and then starting the search for a donor about 18 months ago, we had little idea what we were letting ourselves in for. We had only an outline idea of what we were letting our little girl in for.
We were convinced that a BMT was our only hope of giving Saff a fighting chance of something like a 'normal' life. The odds were good - around 90% of BMT kids survive the procedure and many of the 10% are those poor kids whose conditions (including cancers and auto-immune diseases) had weakened them already.
Over the weekshowever, it's fair to say that the 10% started to haunt us. Living with the prospect that the worst might actually happen had several effects on us. We prepared like mad - emotionally, practically - we thought about what Saffy would need, what her brother Reuben would need. Who could help with what, who would need what. In all of these preparations our ISMRD friends were immensely helpful and knowledgeable. We made arrangements about work and childcare and my mother agreed to be our 3rd carer. We noticed somewhere along the way that we had stopped being interested in some of the things we used to do that somehow just didn't seem very interesting any more. We probably drank too much.
And then we were in GOSH and life had its own momentum and intensity. We worked at everything we could, learning as much as we could from everyone - medical and other staff at the hospital, other parents, other kids. I won't forget some of the most profound lessons. In a sense, this was the easiest part - it was intense, there was always something that needed doing. We work in deadline-driven jobs so this part played to our strengths. It was all about Saffy (and Reuben) and we did not have too much time to sit and think. Doing the blog every night was therapeutic too.
But, above all, we were very, very lucky. We were well-prepared and expecting infections, rejection, extreme reactions to medication. In the event, none of these turned out to be as bad as we had feared and Saffy was an absolute delight. Her anxieties were there. We saw them for example in her developing a fear of clowns (called coulrophobia, if you're interested) whilst in hospital - but she found the good in every day and every situation.
And now, here we are. BMT officially over. It has been the single defining episode of our lives so far. For Saffy, of course, it is her chance to develop. Her alpha -mannosidase level is 77 on a scale that runs to 520, which puts her at the low end of carrier level. This could go up, but to be honest, our doctors have no idea. What it means to her development will unfold in the years to come. Her doctors, teachers, psychologists, occupational therapist, speech therapists, ophthalmologist, spinal consultant, audiologist, dentist and the rest of the team will monitor and help her.
As for the rest of us, Reuben carries on pretty much as before. We consider this a victory. I am not the same, largely through learning something about myself and my priorities in life. I won't bore you with all of that. Sonja having given up her job to care for our kids through Saffy's BMT is applying to become a teacher. The plan is that this gives her more time to spend with the kids and, although she has not said so, I suspect the opportunity to help and nurture children in some way has more of a magnetic pull now than maybe it would have done before. Getting onto a course will be a new challenge, so fingers crossed.
Before we sign off and complete this blog, which we really hope will be of some help to people starting on the same road in the future, we want to thank all our friends and family - both those we knew before and those we have discovered during the last year and a half. Because things have turned out well, what has happened is that many of our relationships have been strengthened and we have found others who have entered the Woolley Family Hall Of Fame. This isn't Oscar night and I am no Gwyneth Paltrow, so we'll leave it there, but because of the people around us, we believe we are luckier than most.
Thank you.
All our love,
Martin, Sonja, Reuben and Saffron Woolley.

T+208. A trip down memory lane...

I can't believe that it's been THAT long since we put an entry up. I mean, I know it was a long time, but....

This is for whoever's reading this in the future, as there's no one who can still be checking for an entry after 95 days of not seeing one.

It seems like a lifetime ago that those entries were coming thick and fast. Since then, it's been like an uneasy limbo. I'm working, Sonja can't think about getting out to work and Saffy can't think about playgroup as every last aspect of our near future is governed by those weekly lymphocyte readings. Saff is doing really well, but it seems that lymphocytes after alpha-mannosidosis BMTs just don't come back quickly. About the same speed as a glacier. We know how lucky we are - we really do - but it just isn't possible to feel lucky all day for weeks and weeks.

Among other things, this has been a voyage of self-discovery for us, and we now know that our patience has limits and that we are better suited temperamentally to the parts of the process that involve immediate action.

So, when Saff developed her first line infection three days ago, we were able to get back to what we do best and react to an evolving situation. To get to 205 days past transplant without having a line infection may be a record - many of our contemporaries in Fox Ward needed all their fingers and toes to count theirs after much less time. Anyway, we hadn't seen one, so it came as something of a shock...

We were actually at the UK MPS Society conference, picking up hints on trigger finger (which Saff is just starting to get) and biomarkers (biochemical indicators of disease), when we came out of the last session, turned on the mobile phones and got a message from my dad that Saffy had a temperature of 39.5 and was on her way into hospital (GOSH organised everything so that she could go into our local hospital). By the time Son and I arrived (in about an hour Northampton - London, hope we didn't pass any speed cameras) Saff was over 40 degrees, fitting, frothing at the mouth and apparently awake, yet unconscious and unresponsive. Pretty scary, but bringing her temerature down brought her back - suggesting that this had been a febrile convulsion.

GOSH took no chances and ordered a head CT scan to check for abcesses on the brain, a lumbar puncture to check for meningitis and a chest x-ray. Saff's temperature was 'spiking' - coming down with paracetemol and ibuprofen, then shooting up as it wore off. She was put on antibiotics to cover all major potential problems - particularly meningitis. Her lymphocutes dropped to 0.15 from aroung 0.8 a few days before. She was clearly ill, but, as ever, cheerful as she could be. One by one, the really nasty things got ruled out, thank goodness.

Anyway, within a day the hospital had identified Staphylococcus and Saff had another antibiotic added to her regime. We already 'knew' it was from her line as she had gone downhill within minutes of her Hickman lines being flushed - a sign we'd been told to look out for as the bugs get flushed into her bloodstream from the line where they were sitting, causing the sudden, dramatic deterioration we had witnessed. Tests confirmed this later and Saff is doing much, much better now the bacterial infection is being accurately and aggressively treated. We expect her out in a few days - maybe sooner.

Being back in a hospital room and going through all of the old routines brought it all back (probably why I did this blog, too, I suppose). If the infection doesn't get cleared from her line, it may well be taken out a few weeks ahead of schedule and GOSH think her counts will bounce back once she is better.

The medical staff at both hospitals were really great - professional and caring and made us part of the team immediately and a great 'catch' by Saffy's grandparents who did exactly the right thing by calling them as soon as they got concerned.

If all this excitement keeps up, I'll be putting another blog up soon - great to hear from messages that it has found some people in similar positions already and been of some use.

All the best for now

Martin

Last lap? To T+113

Is there anybody out there? Couldn't blame you if you had given up on us after so long without an entry, but as several have mentioned, no news tends to be good news and that has been the case with us.

We are at the point now where Saffy's last immunosuppressant drug (Cyclosporin, aka Neoral) is being eliminated by stages. We are down to 0.3 ml twice a day, from a high of 0.55. This drug suppresses any GvHD, so we can expect to see some sign of it as the dose comes down. So far, all we have seen is the intermittent rash we saw in hospital, which appears and disappears after a few minutes. If we can get away with nothing more than that, it will be a miracle, but then, everything has been a bit miraculous so far.

The issues we have been contending with so far have been different to those we were expecting, as Saff has been so well. With me going back to work, Sonja has been pretty much stuck in the house as Saff is not able to be around groups of people. For someone who has been used to working full time, that is a hard transition to make and it has taken quite some getting used to. For my part, I have been working full time for quite a while and taking over on duty from Sonja at weekends. The practical result of all of this is that it takes quite a bit of planning to do anything outside the usual routines and time out of the house for Sonja and me together, or for us to take Reuben out. Don't get me wrong, we still thank our lucky stars each day, it's just that we are not living what you could call an everyday life yet. Between Reuben's diabetic care and Saff's medication there are around 8 entries in our daily medical diary, when they are both at home, so it is a bit of a stop-start existence.

It has been a great help to us all that friends and family are really putting themselves out for us and the messages we continue to get from friends through ISMRD and GOSH reminds us that we're all getting through with each other's support.

Have to go now - it's bathtime and I've got some Hickman lines to wrap in clingfilm....

All the best

Martin

More of the same............... to +84

Sorry for the big gap - but it's getting harder to find things to tell you about here in Camp Lucky. Saffy remains happy and healthy. As yet we have no solution to the continued problem with the red cell count but all medical personnel remain remarkably calm and we are doing our best to mimic that state. In fairness, it's not that hard because Saff looks and acts like a girl thats never been ill in her life.

On our most recent trip to GOSH - last week - the BMT consultant said all that worried him about Saff's tumbling Hb levels was his inability to pinpoint the precise cause. He thought her residual A antibodies attacking the donor AB marrow was the most likely explanation. He felt the immature marrow theory would also lead to reduced white cell counts and this hasn't been the case. What is an undisputed fact is that a significant number of post-transplant children suffer from this problem, and it almost invariably corrects itself, regardless of why it exists in the first place! She hasn't had a transfusion for over two weeks now and although her Hb level is low it's taken longer to fall than usual - so fingers crossed.

I have to confess that all this staying at home has given me an acute attack of cabin fever - I find myself looking forward to shopping trips - and I don't even mean for clothes! Still, all in a good cause.

Reuben, Pokemon master supreme, top Hogwart's wizard and family Top Trumps champion, continues life unconcerned by the endless medical interventions needed for him and his little sister - thank goodness.

Thanks to all of you still following our fortunate progress - I'll try and update you a little sooner next time.

Take care

Sonja

Music to our ears..........to + 58

I'm writing this entry from our local hospital as Saff has a blood "top-up". Everything continues to go really well.

We were at GOSH yesterday and saw Paul Veys - he's the "grand fromage" of the BMT unit and was really delighted with Saff's progress to date. He confirmed his belief that Saff's falling Hb levels are caused by immature marrow saying the red blood cells are typically the last to stabilise post transplant. He did however test another couple of possibilities to exclude them. The first is caused by the ciclosporin attacking the cells, they're left looking like they've had a huge bite taken from them. Treatment is simply to withdraw the ciclosporin. The second could be Saffy's original B cells attacking the donor's AB cells - this can occur because B blood cells contain A antibodies. If this is occuring they simply wait until all Saffy's residual B cells disappear and she takes on the donor's AB blood group.

Today's transfusion is to boost her levels closer to normal and then watch to see how long it takes to fall. Paul Veys says even if this process (i.e. transfusion followed by falling Hb) continues for a number of months he will remain unconcerned.

We left GOSH and travelled to the local hospital for the transfusion (obviously!). As Saffy had had blood taken 7 times in the last 10 days it stands to reason more would need to be taken to establish what blood should be ordered!! We arrived at 2.30pm, had blood taken at 3pm, and were told at 6.45pm that although the blood had arrived it was the wrong type. Words failed me - well actually they didn't but I think the nurse who had to tell me this wished they had.

So here we are this morning - I'm considerably calmer now the process is almost over. We're going home so Saffy and her nursery teacher Natalie can have fun. Tuesday's "lesson" involved a fantastic shaving foam fight. It's a pleasure to hear them at work!

More soon

Sonja

P.S. All blood results for the tests above were negative - brilliant

Testing Times......to +53

Following a hectic week, Saffy is back to feeling well and all the blood tests were negative - the good luck's still with us.

The slump in her Hb level had caused nasty pressure headaches if she bent over (troublesome if you still need your bottom wiped for you) if she jumped (Saffy's favourite way to move around) or if she moved at speed (although it should be said that Saff speed isn't very speedy!). The headaches disappeared a few hours after the transfusion and haven't plagued her since. At least we now have an early warning signal should this happen again.

The local hospital were very thorough in searching for a reason for Saffy's falling iron level, and although it lead to a nervous 24 hours, we've learnt about haemolysing and that it can be treated should Saffy succomb. It occurs in up to 50% of post bone marrow patients and is caused by the compromised immune system. It is treated with steroids. GOSH believe Saffy's level fell simply because her marrow is immature. Her Hb reading only went up to just over 7 after the transfusion - it should be between 11.5 and 14.5 - and although it seems to have temporarily stabilised we're expecting further transfusions.

Saffy's very mild rash seems to have disappeared, the hospital have lowered her cyclosporin levels so perhaps it will make another appearance but we're seeing no other signs of lurking GvHD (said with everything crossed whilst touching wood).

Although still happy to be home, Saffy misses the routine of her old life, she talks constantly of playgroup and the other children at her childminders. The playgroup have been fantastic and are now sending a teacher round for 2 hours every Tuesday and Thursday, to give Saffy a chance to make and colour things with someone who can actually draw. We're continuing to encourage all our friends with children to drop in, but with the good health proviso in the middle of winter our cancellation levels are expectedly high.

Sorting out getting all of the medications, dressings, syringes etc. is a complete pain. It's not that anyone is being obstructive, there are just too many people to talk to in order to get things organised.

I have to say that yet again our daughter's ridiculously sunny outlook is making life much easier for us and herself. Long may she stay the happy little soul that she is.

Thanks to all of you continuing to follow our journey, particularly now it's getting less dramatic (and our reporting has slowed down!)

Take care

Sonja

Deja vu (all over again) Up to T+48

So, here I am at home again with Reuben whilst Son is in hospital with Saff. It's all strangely familiar. Don't be concerned, Saffy is in hospital for her flebogamma (immunoglobulin), which she needs intravenously every 3 weeks. She was also anaemic (Hb of 4.2), so she needed a blood transfusion. She is at our local hospital, who now share her care with GOSH, and not every paediatric ward works with Fox ward's speed, so it has turned into an overnight visit. Saff had been complaining of headaches and tummy aches, but had not developed a fever - we are hoping that she will get the boost from the red blood cell transfusion that she did before. No one at Chase Farm (our local) or GOSH is too concerned - sometimes new marrow just does not produce enough on its own at this stage and some extra is needed. To be on the safe side, they are going to run some tests overnight to see why the red blood cell count is dropping. The 3 usual reasons would be an infection (no sign of this), blood leaking somewhere (ditto) or haemolysing, which, as far as we understand, is the body breaking down red blood cells too readily. If it is this is happening, it leaves markers that can be traced and we will find results out tomorrow.

May I apologise that it has been such a long time since you have heard from us. No excuses, we should have let you know before now what has been happening and I hope that not everyone has given up looking out for updates.

I have gone back to work now, so the feeling of limbo between leaving hospital and getting back to a closer approximation of normal life is starting to disappear. We have set up our home and our routines and support networks pretty well. Medication sets the tempo for life. As suggested by experienced friends, we made spreadsheets of all of Saffy's medications, setting out times and doses. Even though Saffy is on the minimum medication for this stage, it is still remarkably easy to make a slight slip and having it all on paper makes a that a lot easier.

As I mentioned, her care is split between GOS and Chase Farm hospitals. Chase Farm send a community nurse every week to change dressings weekly and new NG tube monthly. They also take bloods on which they can do the more usual tests for cell counts and biochemistry. On our clinic visits to GOSH, they take blood for the more specialised tests, which includes cyclosporin levels. Cyclosporin is one of the key drugs at this stage. Its job is to mute any GvHD, which is important, but in doing this, it also suppresses the lymphocyte levels. This is partly why Saff's immune system is still not working at anything like full capacity. The ideal level is around 150 and, according to the blood test results, her dose is adjusted to try and keep it thereabouts. The theory is that she gets minimal GvHD and they will then gradually bring the dose down and tail it off. Fingers crossed, as usual. That approach has worked until now, so we're not changing.

Day-to-day, Saff is still delighted to be at home. She is starting to show signs of feeling the limitations of her life at the moment and of reacting to all the attention she has inevitably got more used to. This is manifesting in demanding more attention, thinking that she is going to be going to school with Reuben the next day and so on. This is really not much, and she is still far more likely to get excited about getting a visit from someone than worrying about what she's not getting. Her glass is always half-full.

Speaking of visits, we can have 3 or 4 people round to the house at one time, so long as everyone is healthy (no coughs or sneezes, no runny noses, no diarrhoea is the mantra). Other people we know have had more stringent rules on being discharged - we can only assume that this is down to how well Saff is, relatively speaking. This makes a lot of difference as it allows everyone - Sonja & Saff in particular - a break from being in each other's company exclusively, as well as giving friends and family the chance to see Saff for the first time in several weeks. Saffy is also allowed out of the house, but only where she can maintain some distance from other people. At this time of year, that means that if we wrap her up in about a dozen layers, we can get out to a park for a run around as they are very sparsely populated. Shops, restaurants, indoor play areas etc are all no-go areas.

I'll leave it there for now, but we will - we promise! - put another entry up in a day or two to let you know what happened with the blood tests. Radio Saffron is very definitely back on the air.

All the best

Martin