Friday, 3 February 2012

4 weeks in

So today Reagan has her 30th day on steroids. Yesterday we went to see Josu for a review and to make a plan about the weaning process.

As I said she has had weekly blood tests to monitor her progress. FYI reagans bone marrow doesn't make enough red blood cells, when blood cells start out they are called precursors and the name for the amount of precursors is called the reticulocytes (retic). So usually hers is below normal, normal being between 25-85. HB should be between 12.14. Here's how her blood test have gone over the last month
Start. Wk1. Wk2. Wk3. Wk4.

HB 12.3. 11.3. 10.5. 11.7. 11.6
Retic 17. 94. 86. 390. 167

So it looks good so far as we would hope. Now the weaning process begins. Her current dose is 22.5mg per day and now we have to spend 1 wk giving 19mg every other day, then the following week it's still 22.5mg one day and alternate days 16mg. This continues to go down by 3mg per day until we see him in 4 wks time. Again she will have wkly blood tests to monitor her. So this is the real test!!!!!

So I bought a wkly pill box organiser to help me!!! He also prescribed her some sporanox, the steroids have given her oral thrush. It has a lovely list of nasty possible side effects!! Most of which I have no clue as to how on earth we'd know if she was suffering from them. So an even closer eye on her now.

And then this morning she woke up with a cough, cold and temp of 39.1!! So we took her to frimley hospital and she has pneumonia again!!, so more meds. 2 sets of antibiotics.

One set made her gag till she was sick, so I didn't give her the other set yet, then when I gave her her sopranos! She spat it all back it's going well so far.

Sunday, 8 January 2012

Steroid trial

Reagan had what we hope to be her last transfusion on 16th December 2011. She has had her flu, and a repeat of her MMR immunisations (her bloods showed she didn't have antibodies against measles, despite have the jab!)

Peyton also has had the chicken pox vaccine, even though Reagan has too it could still be dangerous for her to be around anyone who has it just incase. This is only the case while she is on the trial, a high dose.

She is on 2mg/kg which amounts to 22mg per day for 4 wks, after for wks the amount will be weaned down over another 4 wk period until she is down to no higher than 2mg per day. On the highest dose, around 80% respond, so we should see it working at first at least. By the end of the trial around half of those who respond will no longer be responding and it is deemed a fail! So she has just under a 50/50 chance of it working.

If she responds then it will mean no more transfusions, although at tough times such as bad illness, puberty and pregnancy she will most likely need to have a transfusion.

Today is day 4 of the trial and so far so good. The main side effects at this dose are mood swings and insomnia. Reagan still doesn't sleep through the night, although she only wakes for milk and feeds herself but still......however, we have only had one bad night so far, so fingers crossed it won't get any worse! As for the mood swings I thing yes maybe she is suffering from this a little but nothing to major.

She's having wkly blood tests for the first 4wks, the day before she started her HB was 12.3 so we would hope to see that rise a little by next wk.

Tuesday, 18 October 2011

Post Josu update!

Following on from our appt wi Dr Salma in July, when I asked about Reagans says for babies up to 18mths/18kg, and she's neither of those! Dr Salma has made and appt for Reagan to see the dietician again which is 3/10/11.

Reagan had her 12th transfusion on 30/7/11 , her HB was 8.3 and she received 130ml.

At the beginning of Aug Reagan saw Anouska, the physiotherapist, again and she has now fully caught up with her age and was discharged, yay!

Aug 24th saw Reagan receive her 13th transfusion, HB was 7.8 this time she received 150ml, her weight gain has been amazing she is on the 25% line now! 22lb

14th Sept was her 14th transfusion, she now weighed 22.5lb and received another 150ml.her HB was 8.5.

And so that brings us to the appt with Dr De la fuente on 15th sept. He didn't tell us a great deal that we didn't already know but it was reassuring to be seeing him.

Her bloods were repeated and it showed her HB to be 11.5. So having 150ml blood had only brought her up 3! In my opinion, it wasnt enough. Having an HB of only 11.5 is still considered to be low.
We decided that she would have her immune system checked, they didn't, however, do that test on the day we were there and so they had to sort it for us to have Reagans blood taken at Frimley and then couriered up to Great Ormand street. Basically they want to see of her immune system is suppressed. Because she has had so many chest infections it's an indication that it might be, Josu (dr de la fuente) also said that DBA patients with the gene mutation that Reagan has do quite often have problems with their immunity!
If her immune system is suppressed then she wont be able to go on the steroid trial at all because steroids lower the immunity anyway. So we'll find out about that at the next appt.

We also checked her ferritin, it was checked at transfusion #10 and was 367, it should be between 15-250. Chelation therapy usually starts when the levels get somewhere between 1-2000.
I had them check her ferritin before her 14th transfusion so I could take it along with me the following day to Josu. It was 1043, so quite a significant jump. Josu checked it again the following day and it measured 1257!!!!! After just one more transfusion?!?! Blimey.

Apparently you can get a false high if you have an infection or something but Reagan appeared well! Although she did get a chest infection less than a wk later.

Also he HB was 11.5, so that was what she had been taken up to with the previous days transfusion which isn't really high enough.

So when we see Josu on 3/11/11 he will have the results and we will finally know if Reagan can go into a steroid trial.

Her next transfusion was just 3 wks later on 7th oct, HB was 8.2. Because last time she only went to 11.5 and because she was only lasting 3 wks at a time I managed to convince them that she should have more blood. Usually they give 15ml/kg but it is possible to give up to 20ml/kg. Which is what she had in the end, so she had 215ml. So hopefully it should be 4 wks before she has to have another.

Her ferritin was checked again but this time it showed as being 714!! I just hope that the previous 2 were false highs and this one is correct. We were also told to have a test done to see if she has any infections or viruses, think it was called a CRP test but not certain. Any way that came back as normal range, less than 4!

On sat 8th oct Reagan came down with a really nasty bug and was violently sick for 17 hrs with terrible diarrhoea, which she then shared with Alex and Kaylum!! Lol and now 11 days on she's still got bad diarrhoea! It really hangs around for her!

But she's growing at an amazing rate and the dietician decided that Reagan didn't need so much milk! She would have a bottle mid morning to go to sleep, another mid afternoon, one to go to bed with then another 2 through the night, that's a total of 1litre over any 24hrs!! Basically she was completely relying on the milk to give her everything she needed and at some point she has to rely on sold food to do that eh!
So now she has cows milk through the day but still infitrini through the night but she seems to have dropped the night ones a little.
She still has one to go to bed with but most nights she still has a lot left in the bottle when I go up so it doesn't need re filling.
Most nights she only has 2 bottles now and sometimes only one.....don't be fooled into thinking that means she's sleeping better tho! It's the opposite, she crys more but doesn't want her milk and eventually she goes back off but she makes a big fuss about doing it....have no idea what it's all about but it's really bloody annoying, not to mention knackering!

Monday, 18 July 2011

Long time no post :-|

Sorry's been such a long time since I've posted and now I have to try and remember all that's gone on!!!

So Reagan went in on 26th April for transfusion #9 but her HB was 10.4 so they wouldn't do it and we had to go back the following week.

So we went back on 3rd may, we had an appt with Dr Pelidis any way. Tis week her HB was 7.4, there's no way it woud have. Dropped so much in a week so the previous weeks must have been a false result!!

As far as her appt goes, it was all good her weight gain was doing really great, I do t remember what it was tho!

It was decided that we would go back in just over 3 weeks so she could be pinked up for our holiday, also that dr Pelidis woukd write up the prescription for the steroids and leave them on the ward ready for us to collect at the next transfusion and then to start her on them upon our return from holiday.

Reagan also had pnueomonia again in mid April, she had a short stay in hospital again.

20-22nd April saw us at the annual conference for DBA......we met lots of other families with children who had DBA and adults too! It was so fab to meet them all and they we're all so welcoming. We learnt so so much it was amazing.

Most interesting was to learn that a little boy with a disorder very similar to DBA had been cured by seeing a nutritionist.....needless to say we are gnu explore this avenue!

And also we met with the UKs leading DR who specialises in red blood cell production. He gave a few talks and something that struck me was that they have fairly recently discovered that many DBA patients have a weakened immune system.....I immediately thought of Reagan and her repeating chest infections. I asked him about it and he said that if it is the case that reagan should go on steroids as they will only weaken her immunity further and could be dangerous. He agreed that Reagan would be a candidate for the test to see if her immune system is weak.

So, we asked him he would be willing to take Reagan on as a patient. It was obvious to us that he had more up to date information on DBA and would benefit reagan alot. He agreed and said. Needed to get a referral from our GP.

So we decided that she wouldn't be going on the steroids yet!!

We went back to st Georges on 27th May for Reagans 10th transfusion and dr Pelidis hadn't left a prescription for Reagan so that suited us!

Reagan had had another chest infection earlier in may, this time they didn't even both with the usual Amoxicillin (which they have always prescribed before and it's never worked and then she's needed another antibiotic) I think because she developed pneumonia last time.

Anyway she had 130 mls on 27th may, i think her HB was about 9.5, so the highest its ever been when shes getting pinked! she weighed 18lb on the nose, so now she was half way between the 2nd and 9th percentile.......remember when she way down below the chart all together? She's done so well lately.

I managed to get some antibiotic to take on holiday with us because she had a bit of a cough and I was worried it might develop into an infection again. When we arrived on holiday (1/6/11) she still had a cough and her voice was husky and a bit snotty, but, she shook it off all on her own. She's never done that before I don't think, and then she was so healthy for the entire holiday!!

Her next transfusion was due 24th June. We decided to go to Frimley park because we were obviously waiting for her referral to go to the new DR, Dr Josu De la fuente, at st Marys. Plus it had been a while and she was bigger now so they might not have such a hard time getting the cannula in.

Her HB was 9.9 so they wouldn't transfuse, we were to go back again the following wk. It took 3 attempts tho and the still didn't manage to get a cannula in.

So we sent back the next wk (30/6/11) her hb was 8.4 but they said (at bloody 5.30 at nit) that they wernt going to do anything until they'd spoken to Pelidis in the morning! Gggrrr
Long story short.......if you're on steroids you shouldn't have transfusions- dr pelidis thought she was on steroids (despite not ever writing the prrescription) and said not to transfuse her!!!

Finally got it sorted and she had her 11th transfusion (130mls)

She had a clinic appt with DR Salma at frimley chic RENs centre on 12th July, her weight was 19lb13oz, which takes her to smack bang in the middle of the 9th and 25th percentile, so she's jumped up another percentile, so impressive. Her height was 74cm, which is half way between the 0.4th and 2nd percentile. So she's a bit shorter but still doing so great!

Dr Salma was very surprised with her, I told her that she was signed off from portage some time ago and signed off from physio just the wk before because she is physically doing everything an 18 mth old should be doing!!yay!

We've had an appt through for dr De la fuente, 15th sept, I'm currently trying to bring it forward.

Reagan is due another transfusion next wk.

Over and out!!

Saturday, 2 April 2011

transfusion #8

Tuesday 29th March

we were back to St Georges today for a blood transfusion, Dr Pelidis had called me the day before to check we were still coming and said she would order the blood so it would be ready for us and would just need to be cross matched and there fore should be quicker!

So the traffic was absolutly horrendous, as soon as we got onto the Hogs back we came to a stand still! So we didnt arrive at the hospital until 11am. Alex dropped me off outside and went to park the car.
I got to the ward and it was heaving, someone had been given Reagans bed so we were sent to the play room to wait. there wasnt any where to sit in the play room it was so busy! (although one family had 5 adults with just one child!!)
Then Alex calls to say that the car park is so full hes had to park out on the road and i had his wallet!
So I had to try and write down all the info (with a crayon!!) to call the parking company to pay for the parking. All the while Reagan is desperatly trying to get down and crawl and empty the contents of my purse while im trying to read my card number. Plus i got cut off twice because of back signal and im not even ment to be on the phone!!!!

About 12.30 they called us in to get her cannulated, they had called in a speciallist this time to use the ultra sound scanner to see the veins. So I had to hold her to me while they did it. they dint use the magic cream because it only makes it harder but the freeze spray they use is just that.....freezing and she screams and screams.
Then they inject a local anesthetic which is a stingy intections and so makes her cry even more.
The first attempt failed and so she had to do the whole thing all over again.
Poor Reagan was so upset, aswell as all that shes being held tightly to keep her still, surrounded by strange people!

By this time it was 1o'clock. They were just making her bed up for her and eve worse than last time it didnt even have sides on it!

All she wanted to do was get down and crawl about but she had to be content with being walked about and cruising around our chairs and the bed.
Then.......someone brings their child in with full blown chicken pox! an you believe it, they didnt even realise, so we were quarantined to our bed and couldnt go any where until it had all been cleaned.

They were so busy we just couldnt gt angry with them and they are all so lovely but again the blood didnt et started until 4.30pm, it takes 4 hrs and after that she had to have her Chicken pox vaccine! oh yes forgot to say that St Georges ordered it in the end for her!

Fortunatly the transfusion went through smoothly and we were moved up to a paed ward at about 7.45pm because the Jungle ward closes at 8pm. Then the transfusion began to play up but it wasnt too much of a set back. Once it had finished at about 8.30pm we just had to wait for them to come and give her her vaccine.

We eventually got home at 10.30pm! Another stupidly long day that was spent trying to keep her occupied, which is getting harder and harder each time.

We're really working hard at getting her walking so at least she can be a bit mobile!

Oh and Dr Pelidis came to see us and we asked her abuout the Phenergan, she said she hadnt really heard of it being given as a sedative (really!!!!) and it can have a bad side effect that is treated with Piriton and so to try piriton first.
She said give her 2.5mls, which is just a normal dose so I couldnt see that doing anything. any way we were to try it for 3 nights and if not then we could go back to Dr Salma and get the Phenergan. I had already bought Phenergan and given it to her an dit had worked but we gave the Piriton ago and it did absolutly nothing at all to help!
So i've been onto Dr Salma to get a prescription, we're still waiting for it but in the mean time im giving her Phenergan any way and she sleeping so much better and has even gone all through the night once!!!
Think i'll go a week and as long as its still working i'll start reducing the dose.

Shes going to have another transfusion on 26th Apr and the 2nd part of the vaccine. Shes then due to see Dr Pelidis the following wk and she'll write her prescription for the steroids then, however, that will only be a couple of wks before we go away, so unless shes prepared to transfuse her before we go any way (which sort of seem silly to start the steroids if she going to be transfused) then we wont start them until we get back!

Weight wise, she was 16lb 5oz and it was 3 wks since her last weigh and she was pretty much the same, so probably lost alittle from the bug she had which had lasted a full wk and she hadnt eaten really either, but she is still on the 2nd centile though!
her length is 69cms, so still right at the bottom centile for height!!!!

Dr Salma, paediatrician

Thurs 24th March

We made an appt with Dr Salma today to see if she could offer any help with Reagans sleep.
She checked her over as usual and asked about her development and who she was still seeing - dietician, SALT, portage, physio etc. she questioned about what had happened the night previous and was very happy with her.

she said she could prescribe her some Phenergan but wanted to check with Dr Pelidis first just make sure there was no blood related reason for her not to have it.
Obviously this was going to take weeks to get sorted..........great!

Poorly again! :0(

Wed 23rd March
Reagan woke up with Diarrhoea today, she wouldnt eat a thing and at lunch time she woke up with a temp.
I didnt have access to a themometor so i dont know what it was. i rang the childrens ward and they wanted to see her because it had mucus in it.
we arrived about 1/2 hr afte she had some calpol and her temp was 38.
they decided to do a blood test to be on the safe side.
she had been very sleepy all day and was still at this point, then about 20 mins later she really perked up!! Typical!
Any way her bloods were fine, they showed no infection so just a virus and her HB was 9. this was 3 wks post tranfusion (remember they had given her an extra 20% blood to last her 4 wks) and her retic count was 63!!!! I cant remember it ever being that high!

so anyway, we went  home after a couple of hrs because we had an appt with Dr Salma the following morning.