30th May 2012 Day 27

Time for a wee update.  So far Chiara's BMT has went fantastic.  Last Tuesday we were allowed to take Chiara out of the hospital and over to the park since the weather was just beautiful.  This had come so much quicker than we had expected as she was less than 21 days post BMT.  We continued every day to go out walking and have day trips to the park and enjoy freedom from the hospital room.  We were told to prepare for heading home on Sunday which would make Chiara day 24 post BMT which was amazingly fast recovery.  Unfortunately by Friday Chiara was covered in rash, she had been getting a few spots here and there for a few days but nothing really specific.  She was receiving IV Piriton for an itch coming from an undissolved stitch in her neck from her central line therefore the Piriton should be enough to help with treatment if the spots were an allergic reaction. 

On Saturday my brother came and spent the day with Chiara which was great as it allowed me to have a girls day out shopping with my sister in law and niece.  Before we left the hospital my brother decided he would take Chiara out for a walk.  As we all left the hospital we had a small incident involving a garment of my underwear being attached to a wheel of Chiara's pram, a story best kept within the hospital I think, as it certainly made the amusement for the day in the Ward.

On Sunday Chiara's rash appeared to be spreading and she was becoming a lot more itchy and uncomfortable. She had received more Piriton but had vomited this the minute it was injected into her line.  Her anti-histamine was therefore changed to Cetirizine.  Unfortunately that was not enough to subside the itch and Chiara received another 3 doses of Piriton over night.  By morning Chiara's whole body was covered in an urticarial rash with puffiness of her face and eyes, very typical of an allergic reaction.  Chiara was fasted and prepared for theatre to have skin biopsies taken.  She was evaluated by a Dermatologist who felt this was more a reaction to a drug or a viral infection.  Her biopsy was therefore cancelled as was theatre.  She was prescribed Elecon cream to apply and continue with Piriton.  Chiara's next dose of Piriton was not due until later on that evening and she was starting to show signs of improvement.  Her Piriton dose was given at around 6.30pm and she vomited immediately and became flushed again.  It was starting to look as if her reaction was to Piriton which is the most unusual medicine to be allergic to.  She has been on Cetirizine and creams since Tuesday morning and her rash is showing great improvement.  Unfortunately as you will have realised this kept us in hospital and we never made it home on Sunday.

We are hopeful that we will be homeward bound within the next few days providing the rash settles.

Chiara's medicines have increased yet again and we were handed 13 morning medicines alone to give her orally, I decided then that it was unfair on her to manage these, given that she would need to take another 4 at lunch, 4 at dinner and around 10 at bedtime therefore I opted for an NG tube to be inserted for medicines only.  This will be removed once her dependency on medicines reduce. 

She is eating as healthy as ever with steak pie, potatoes and beans for dinner.  Her fluid intake remains fab with her consuming at least 1400ml per day. 

Her sleep study from last week revealed that she no longer requires her  BIPAP machine overnight which is yet another huge step forward.  Today Chiara had bloods taken for her White Cell Enzyme level to see if she is producing enough on her own to allow her weekly infusion of ERT to cease.  She also had a urine test to check and see if her GAGS have reduced also.  Her blood counts remain brilliant , with high Neutrafil counts and acceptable Haemoglobin and Platelets and to date has not received transfusion since transplant.

Last week my girl and I appeared in the Sun Newspaper as I had been nominated for a competition as an Ideal Mum.  I dont think of myself as anything more special than any other Parent that is in this ward. We all endure so much and watch our kids go through so much pain and treatment it is hard to single one person out.  There is not one person in here that hasnt cried as much as me or felt isolated as much as me, we are all in the same position but every day we all face the challenges that are set in front of us and our children.  Every one of the parents I have had the experience of befriending all deserve the title of ideal mum or ideal dad.  Watching your child fight for life every day is one of the hardest tasks a parent will probably every have to deal with. 

I hope that my next entry will be from home and I will have ALL my family around me, it is becoming lonely in this hospital room just me and my girl, I miss my boys and look forward heading home for good with my ever improving girl and her new engrafted marrow.

22nd May 2012 (Day 19)

Well tonight I write Chiara's update with a smile from ear to ear. 

I headed home on Sunday night to spend time with my boys.  I left comfortably in the knowledge that Chiara was recovering well. Her counts were continuing to rise and on Sunday they were 0.8 therefore for the first time in 3 weeks Joshua and Lindsay were allowed to visit their sister.  It was fantastic to have them altogether again and Chiara was so happy, screaming and scrambling over them. 

I arrived back to hospital on Monday to be told that Chiara's Cyclosporin (her anti-rejection) drug was going to be changed from IV to oral which meant that was another step closer to home.  How close, I didn't realise until Richard said that it would or could be very soon as long as Chiara remained in the healthy state she was and she had a good holding Platelet count and good Neutrafil count.  To go home soon would be fantastic, she was in 8 weeks for her first transplant and I came in with the view that's how long we would remain and anything sooner was a bonus but never imagined it could be as early as just a few weeks.  As hard as it is I am trying not to get my hopes up as something is usually lurking around the corner to burst my bubble despite my positive mental attitude.

The side effects of her anti-rejection drugs are starting to show, her Electrolyte blood tests are showing her to have slightly low Magnesium, Calcium and Potassium despite oral substitutes.  She has had several infusions to try and replace this but until she stops her Cyclosporin then I have a feeling this is going to cause a problem.  We always knew that the Cyclosporin would be toxic to her kidneys but its outweighed by stopping graft rejection.

Today the sun was beating down therefore Chiara and I were allowed to go out of the hospital to the park, loved loved loved it.  We ran into a slight sad hurdle on our way out, Chiara initially was happy to be toddling through the ward having left her room until we made our exit out the Ward doors, she then started screaming and cuddled herself against the wall, she look scared, I didnt realise how much she had become dependent on her room and the familiar, safe surrounding it provided for her.  Sad to see I have to say but before long she had overcome this and was enjoying freedom.    To be walking through a park with Chiara 19 days post transplant looking as good as she does was just a joy.  When I returned my day was crowned with the news that Chiara's bone marrow Chimerism has been reported as 100% donor.  She sits today with counts of Neutrafils of 1.6, Platelets 281 and Haemaglobin of 91.  I am so happy as is her Daddy who has now proven his marrow was definitely the "good stuff".  Hurlers patients only need to have 25% donor to replace the White Cell Enzyme they miss so to have 100% is just a bonus.  Chiara was 100% with her first transplant but this time round we are determined not to let this one slip away.

To date Chiara still remains NG tube free which is amazing since she is on over 20 oral medications per day.  Her appetite is good and remains on a normal diet.  She still has a way to go and we still have worry of her developing infections but we will take each day as it comes.

 

17th May 2012 (T + 14)

Day 14 today and Chiara is brilliant.  She has no problems with her mouth or any after effects of Chemo.  However she has become grumpy and generally fed up being stuck in isolation. 

Chiara's counts today were, Neutrafils of 0.2, White Cells of 1.4, Haemoglobin of 88 and Platelets of 276.  Chiara has never managed to increment platelets to this level before so its amazing. 

The best news today is that her HHV6 came back today negative therefore her rash and temperature that she spiked at weekend had nothing to do with the HHV6 virus that we feared it could be.

Now we wait for this engraftment to take control and just keeping pushing these blood cells to an acceptable level with minimal side affects, one of them being the possibility of GVHD.  (Graft versus host disease).

14th May 2012 (T + 11)

Well until yesterday Chiara was keeping amazingly well.  She awoke yesterday morning just not her usual self.  She felt warm and was grumpy.  As the morning progressed Chiara's temperature slowly began to increase, so it was just a matter of time before she actually spiked.  Considering she was day 10 and had not fallen unwell before now was actually quite good going.  It would have been more unusual for her not to have had at least one temperature. 

She had developed some spots on her side and on her back.  These resembled the spots that she acquired back in November last year, one month before she developed Pneumonia which lead to the diagnosis of HHV6 virus which was the cause of her new BMT failing last December.  This obviously has made us very anxious as there is a possibility that the HHV6 could have been reactivated which is common post transplant because of the immunosuppressive nature of Chiara. 

This morning Chiara was screened for the HHV6 and had her Acyclovir changed from oral to IV and dose increased.  If the HHV6 test returns positive then Chiara will need this treatment changed but the adverse affects of the new medication would be suppression of the marrow therefore her engraftment would be longer and put her at further risk of infections.

Today she still had zero Neutrophils but her White Blood Count had increased to 0.4 which is still incredibly low but at least its on the increase.  Also she had an increment in her Platelets which is good as this hopefully means the new marrow is starting to en-graft. 

There is a possibility that the spots and temperature could be related to engraftment but we cant rule out the chance that it is the HHV6 and if it is then we just have to keep fingers crossed and think positive that this graft will keep as long as prompt intervention with correct treatment is started.

I returned home last night to enjoy a well longed for night with my boys.  Unfortunately while I was at home Chiara's temperature increased over 38 and she was commenced on IV antibiotics as standard protocol and Paracetamol.

I arrived at the hospital today and was faced with a poorly looking girl with an almost bald head and a bed full of her hair.  Upsetting as it was her Daddy shaved the remainder of the hair off.  I watched as every last curl fell to the floor.  I remember going through this feeling last year but she had less hair and albeit hard to watch it did not compare to today.  The only good thing was she could now eat and drink without having hair in her mouth, on her bottle and on anything she came into contact with.

She became brighter later on in the afternoon, no more temperatures and with a fresh hair free bed, pyjamas and bath she looked great again.  She has remained well the rest of the afternoon.  

Her results for the HHV6 are still outstanding so we have another anxious night of awaiting the result.

9th May 2012 T + 6

Just a quick update.  Chiara remains extremely well.  She has started taking better quantities of food and continues to drink normally taking in over 1 litre of fluid by herself.  She doesn't appear to have any broken down skin or Mucositis yet due to the after affects of Chemotherapy. 

She has a low Platelet count of 42 which is to be expected post transplant.  Unfortunately this low Platelet count comes with huge drawbacks, one of which is bad bruising therefore it is advisable to avoid falling at all cost.  Obviously no one thought to tell Chiara this, as yesterday she took a big nose dive into the bed and instantly the damage started to appear.  She  was examined by the Doctor and treated with an ice-pack.  Unfortunately this morning she woke with a black eye.  Still cute but looks even more wild with a bruise on her forehead and a black eye. 

Today her Platelets are only 24 so tomorrow she will receive a transfusion. 

We still have  zero Neutrafils or White Cells so every day her number obsessed mum is annoying nurses for her counts in the hope that one day I will see even a slight increase.

Chiara herself, is unaware of everything she is enduring, the biggest trauma for her through all this is being not allowed out of a door.

Yesterday one of Chiara's samples came back as showing she was Norovirus negative.  She has had this virus since February and ironically now when going through a BMT she loses the Noro eventually.  Good thing with this is that when she eventually comes out of strict isolation she will be able to have a bit more freedom outside of her room.

Whilst in strict sadly for Chiara she only has the company of myself and her dad when he is able to come through.  She misses her brothers terribly and is constantly looking at the camera and giving them a kiss when she sees their picture.  I showed her a picture of them on my phone and she looked behind the phone thinking they were there.  Hopefully soon she will see them.

7th May 2012 Day T + 4

Well so far so good.  Chiara's counts are now at 0 for Neutrafils and White Cell Count.  She required a blood transfusion on Friday so today she has a Haemaglobin of over 100.

Clinically she remains well, she has developed a redness almost sunburn like.  This is the result of the Treosulphan Chemotherapy that she received prior to Transplant.  This will be treated with an 50/50 emollient and should hopefully improve.

At the moment her mouth appears fine, no sign of mucoscitis which is extremely common post Chemo.  She continues to have many loose stools and therefore her little bottom is a bit on the red side but so far it has not become broken down.  Basically every time she required a nappy change I plaster on Orobase in Vaseline in the hope this will prevent any problems in that area.

I myself went home on Sunday to spend a night with my boys, which was lovely.  We didn't do much but to spend some quality time just watching TV and talking made all the difference to my otherwise busy days with Chiara.  As much as Chiara requires attention it is also important for me to try and make my boys feel equally as special as her.  Again, like last year, I feel my boys are growing up before my very eyes and I am not there to give them my time and my sadness will come when one day they are all grown up and I realise how much I missed out on.


Chiara still remains off her food but continues to take milk and juice orally and some pureed fruit.  I am hopeful her appetite will return in the near future.  She still is NG tube free and takes all numerous amounts of medicines orally.  Her Potassium has been a bit on the low side therefore she has received 2 Potassium infusions.

As yet she still has a full head of unmanageable hair.  She also continues to misbehave having found a vent in the room under a desk and is able to stick her arm out this into the corridor and is shouting at anyone who can hear her. 

We now wait wait and wait on these counts to rise.

3rd May 2012 Transplant Day

Well today was yet another day that we will never forget.  At 3.58pm Chiara received her Dads stem cells in the hope that this will stop her Hurlers from progressing and hopefully give her the best chance of a normal life. 

This morning Richard left myself and Chiara to go to the Beatson to have his stem cells removed.  He had been on GCSF for the last few days to boost his counts and I have to say last night he looked like he was feeling the side affects from the injections.  These injections were given to him to fool his marrow into thinking he had an illness so that his blood cells that fight infection could increase.  Although he felt slightly sore it was a small price to pay for what could be the benefits and what greater feeling to have than knowing a few days of discomfort gave your daughter back the gift of life. 

Richard returned just after lunch, slightly weak but generally well.  They had managed to obtain a large cell dose from him, more than they expected therefore Chiara would receive her quantity and the rest would be stored in case she required this in the future.


We waited and then watched a tiny bag of cells getting transfused into Chiara while she sat on her cot unaware of what a huge moment this was for myself and her dad, a moment that on the 24th August 2011 we witnessed and hoped we would never witness again but sadly today we went through it a 2nd time but hopefully the last time.  Richard is always bragging that he has the good stuff so I am praying that he is right and it was delivered to Princess Chiara today.

All we can do now is wait, tomorrow I am expecting her counts to be at 0.  After that we wait and wait and hope that the cells will take and while we wait we pray she doesn't catch any infections as she has no immunity at all at this moment.

All in all another emotional day but a worthy one.

1st May 2012

Today Chiara received her final dose of Fludarabine Chemotherapy. 

She again 2nd time round has coped with the Chemo remarkably well.  She has experienced a slight sickness yesterday and today so much so that it has put her off her food.  By this I mean instead of sitting down to Chicken and Potatoes for lunch and Mince and Potatoes for dinner she is only managing a bowl of soup and bread followed by a yogurt, with snacks in between and about 4 bottles of milk in a day so really at the moment I am not too worried!!!!!!!

Her and I both are finding it very hard to be kept in isolation.  After escaping out her room the other night she has now worked out if she opens the door and pops her head out to check if anyone is there gives her a better chance of escape.  Last night I closed all blinds and tried to settle her but she stood in her cot and shouted "Hello" to anyone that was listening.  Frustrating as it is , it is also very funny

Her curly hair is day by day getting more out of control so I guess by the end of next week she will be starting to lose it.  I am making the most of bows and clasps in it the now but its a fight between me and Chiara to see who can keep it in her hair the longest.

Tomorrow Chiara will receive her Enzyme Replacement Therapy. 

Richard has been receiving his daily GCSF and today had a blood sample taken which will report whether he needs another dose tomorrow.  On Thursday he will be admitted to hospital in the morning and the donation will start. Chiara is due her transplant of the cells in the afternoon so as yet we are unsure if Richard will make it back in time from donating to actually witness Chiara receiving his cells.

Today Chiara's blood counts have started to decrease and she required a platelet transfusion so I presume by tomorrow they will be even  lower.  It is when the counts go so low and to 0 that Chiara is at increased risk of infection hence the need for strict isolation.