My Poor Neglected Blog (Nearly 6 Months Post Transplant)

I'm sorry.  I'm sorry for neglecting you. I keep meaning to update the blog.  I really do.  But then life happens (as it does), and this poor blog gets pushed to the back burner.

So here I am, better late than never, updating you fine people on Miya.

Miya is FANTASTIC!! She is SO AWESOME!! And I'm not just saying that becasuse she's my kid, ya know.  I really mean it.

Last Thursday, Miya underwent a small surgery to remove her Hickman line!! This is a huge step in the post-BMT world.  It says "Hey!! I don't need loads of blood taken anymore!! I  don't need IV drugs anymore, or blood products!!"  This removal means so much to Miya and to our family.  This means normal baths! It means swimming when she is released from semi-isolation.  It means no more weekly dressing changes (Miya HATED them) and no more tucking lumens into clothing and making sure they were hidden well in pajamas, so little hands couldn't reach them in the middle of the night.

Watching Pingu on her iPad after coming back from surgery to remove her Hickman line

Although the hair produced from taking Ciclosporin is slowly coming out, Miya still has a lot of hair on her back, bum, shoulders, arms, and the tops of her legs.  The hair on her face and lower legs is slowly starting to thin/fall out.  

Miya is becoming more and more independent.  She doesn't want to crawl up the stairs anymore.  She wants us to hold her hands while she walks up.  She chooses her footwear and which jacket she wants to wear.  She also speaks!!  Off the top of my head, here is a list of Miya's vocabulary:

No, Yes, Yay!, Mum, Dad, Cat, Blue Car, Car (I'd like to point out that "car" is often used independently of "blue" but "blue" is never said on its own), Uh-oh, up, down, corn, I don't know, where?, airplane, nose, ding-dong, cow, shoes, two (she thinks every number after one is "two"), shhhhh (while putting a finger to her lips to indicate quiet), and Comebo (it's how she says "Columbo". So what? I like to watch Columbo!!).  That's all I can think of for now, but I know as soon as I post this, I'll think of more.

She can do the actions to "Head & Shoulders, Knees & Toes" and the actions to various other songs I've taught her.  She also likes to dance and play music. 

We have a clinic appointment at GOSH in two weeks.  At that appointment, a CD4 marker blood test will be done to determine Miya's T-Cell production.  The magic number is 300.  If Miya's T-Cell count is 300 or above, she can come out of isolation.  It generally takes 2-3 days for the results of this test to come in.  

The following is a video I made of Miya while driving in our blue car.

4 1/2 Months Post Transplant And All’s Well

 

Yesterday was our monthly trip to Great Ormond Street Hospital in London for Miya’s clinic appointment. 

Miya was hardly recognisable to some of the staff, who were astonished at how much she had grown since January, and how good she looked.  The usual blood tests were run, along with an engraftment test and a CD4 Marker test (to gauge the production of T-Cells, a part of the immune system). Her bloods came back today (apart from the engraftment and CD4 Marker tests) and they were very good.  She is doing so well, that the doctors don’t need to see her until April, at her 6 month post-transplant checks. 

Miya has started saying some new words: up, bye, yay! (while clapping her hands) and she can climb stairs REALLY quickly.  She tries to climb them one foot at a time, and holds on to our hands or the wall to accomplish this. She understands almost everything we say to her, and is very good at putting things back in their correct location (for example, she’ll put her chalk and crayons in the correct trays on her easel, and she’ll put her books and dolls back on her toy shelf).  Her appetite has increased and she no longer requires feeds through her NG tube.  We have learned to place the NG tube ourselves and the community nurse only comes to take Miya’s blood pressure, take bloods and do Hickman line maintenance. 

At April’s appointment, our termination of  semi-isolation will be determined as will the removal of the Hickman line.

A New Year...2012, Here We Come!!! T+77

Since my last entry, nothing much has happened, prooving the adage "no news is good news" is also true for my blog.  Miya was last seen in clinic on the 7th of December 2011 and a new appointment was made for the 11th of January, 2012. 

On the 20th of December, a small hole was discovered in Miya's hickman line during her weekly blood tests, done at home by our community nurse (whose name is Dave). We immediately aborted the blood tests and set off for Royal United Hospital in Bath, which is our local shared care facility.  While we were on our way, Dave called us and told us we should go to Bristol Children's Hospital because Bath couldn't repair the line. 

We waited in a small side room of the Bristol Accident & Emergency department (a HUGE no-no for post-BMT patients...sitting in an emergency room with no immune system is NEVER a good idea. Luckily, she didn't become sick as a result.)  For several hours, Bristol consulted with Great Ormond Street and everyone decided we'd better go to GOSH the next day for a repair. 

After the line was repaired (on the 21st of December), blood samples were taken to see if the line had become infected or contaminated during the time of the puncture.


We received a call late in the afternoon on the 22nd from GOSH, advising us that Miya had an infection, and to go immediately to RUH in Bath for IV antibiotic treatment.  Miya received a small amount of antibiotic in her line, and it was locked in for 24 hours.  The first three doses were done 12 hours apart, and then she received a dose every 24 hours after that, for a total of five days.  It wasn't a long treatment, and even though we had to go in on Christmas Eve and Christmas Day, it wasn't a huge ordeal.

Miya has started kissing everything (except her parents) and is a loving and lovable child.  She is very bright and although she isn't speaking, she understands almost everything we say.  She loves music and loves to dance.  Right now, she is covered in brown hair, as a result of the immunosuppresent Cyclosporin.  She has huge, fuzzy eyebrows, a furry forehead and fur around her eyes.  She had furry arms, a furry back, and furry legs.  This hair will fall out when the Cyclosporin stops.

Miya, Chris and I would like to wish all our readers a Very Happy New Year!!!

"A Long December And There's Reason To Believe Maybe This Year Will Be Better Than The Last" T+49

There's a song called A Long December by Counting Crows and a portion of the lyrics goes
"A long December and there's reason to believe maybe this year will be better than the last". I sing this song every year, but this year it really  has meaning. 


The year began with a routine tenotomy to release the tendon in her right foot, as a part of club foot correction and ended with a bone marrow transplant.  It seems like all we've done this year is spend time in hospitals.  Maybe in 2012, we'll spend more time out of hospitals. 

We've been home for three weeks tomorrow and it has gone by so quickly.  Our days are dictated by routine and if we deviate, it can be a struggle to get back on schedule.

Miya takes several medications via her NG tube four times a day.  She also requires 1100ml (1.1 litres) of fluid per day in order to flush her kidneys.  If we go anywhere, we have to make sure it is just after she's had her meds and a liquid bonus (through her NG tube).  She's still not eating properly so most of her nourishment is through Pediasure, given through her NG tube. 

Our first clinic appointment was two Wednesdays ago, and we go back tomorrow for our second appointment.  Usually, children attend clinic weekly for a while, and then move to bi-weekly, then every three weeks.  Miya went straight to bi-weekly.  The consultant said that in 10 years at GOSH, he has never had a better Hurler's patient. I was so pleased to hear that. 

Other than the daily liquids and medications. life has been relatively uneventful (thankfully).  I am soooo excited for Christmas (19 more sleeps!!) and have had fun searching for toys for Miya.  (I think I may have gone a little overboard. Oops.)  Miya has been so brave and so good this past year, I just want to over-compensate with...stuff.  (Yes, yes, I am aware that if I continue, she will turn into a spoiled brat.)

After 10 weeks off, Chris went back to work yesterday.  I must admit that when he came home, I collapsed on the sofa.  Looking after Miya isn't difficult it's just tiring. 

Sadly, there's not much to update you on.  Miya is well and Chris and I are well.  The dog is well, too.  I am excited to receive my mum's yearly Christmas box. It should be here next Tuesday or Wednesday.  Dad also sent a box too, with Ganong's chocolates in it!! Fabulous!!

Up And Down

Bath Time

This Video Was Taken In The Hospital.

I Feel Like Such A Grown-Up T+24

To follow the old adage "When in Rome", I decided to do just that (only replacing "Rome" with "London") and bought an Oyester Card (a plastic card you put credit on so you can use it to travel on busses, the tube, trains) and decided to travel to and from the flat on The Tube (the London underground system, to those who aren't savvy).  I had never travelled on the Tube alone before, and only a couple of times before that with Chris on a previous trip to London, so I was a little nervous. 

So I planned my first journey carefully, put on my best London face (a neutral expression that displays nothing) and I boarded the Tube.  I felt like such a grown-up!! (I must admit that although I am 32 chronologically, I am five in my head.) I was soo excited!! After my first journey (an epic fail, by the way.  I neglected to make sure all the lines I needed to complete my journey-Picadilly line to Central or District lines-were open and I ended up walking an hour and a half to the flat from South Kensington because the Central and District lines were closed for the weekend. My phone's GPS was confused by the tall buildings and I walked through Chelsea,Westminster, and down the FANTASTIC Victoria Street before arriving at my final destination *sigh*) I began to be a little envious of the people on the Tube, dressed in their suits and work clothes reading reports or going over projects, so I started to wear my nice brown velvet coat with the embroidery and I bought a nice new bag from River Island and I began to dress as if I were going to work too. 

I love the anonymity of London.  I love that no one cares where I'm from, that I have an "accent" or that I'm foreign.  I love that no one cares what I'm doing because they're so wrapped up in their own little worlds.  I love taking the Picadilly Line on the Tube and going down, down, down those loooong escalators, that take me into the belly of the busy, busy city.  I love shuffling along with the masses and standing or sitting on the Tube, reading my Kindle and looking nonchalant. I love exiting the tube and being lifted up, up, up out of the belly of the beast and being spat out onto the busy sidewalks of London. I love being a part of the crowds who cross the crosswalks and who take the free newspapers from the vendors who stand outside the Tube stations.  I feel alive. I feel as if I am a part of things. 

Enough about me.  Let's talk about Miya, the miracle child. 

Miya is fantastic. Miya, I think, has defied the doctors' expectations. Miya's neutrophils are growing nicely, although she did have a dose of G-CSF last night.  G-CSF is a drug that encourages the bone marrow to make more neutrophils/white blood cells. This past week, we've been able to go outside for walks with the rain cover on Miya's stroller to prevent interaction with germs/infections.  We were also told that Miys is 100% ENGRAFTED!!!!  I have began preparing Miya's many oral medicaitons myself (in preparation for discharge, so I'll know what to do at home) and have been feeding her via her NG tube with an apparatus called a Gravity Feed Unit.  This has been touch and go, as it's difficult to get the flow at the correct speed and Miya has vomited a few times because I've had the feed unit going to fast.  (A gravity feed unit is a recepticle for holding milk or water, attached to a tube which fastens to the NG tube. You hold the fluid recepticle up in the air and gravity pulls the liquid into the NG tube. The higher you hold it, the faster the flow.) Today, we are going to try smaller amounts of liquid more often and see how that is tolerated. 
**I should mention that Miya is still eating and drinking very little. She has a fluid target of 1.1 litres a day, which is why we are still using the NG tube for milk feeds and water.**

The best news, however, is that on Wednesday the 16th if Miya remains well, WE CAN GO HOME!!!!! If that actually happens, we will have been in hospital for 37 days.  I think that this is a record time!! A 5 1/2 week transplant!!

We won't be out of the woods yet, though.  Miya will be in isolation at home for at least six months while her immune system continues to grow. (It can take about a year to reform.) We will have to travel to London every week, initially, for clinic visits. She will be given 4-5 medications four times a day for at least six months. These include anti-sickness, an immunosupprant, some antibiotics, an anti viral, and anti fungal medicines. These are to provide support to her immature immune system and to keep introducing the new bone marrow slowly so she doesn't reject it.  Miya will be on penicillin for life.  She may have fevers, vomiting, diarrhea, she may need red cells or platelets.

This experience has changed me.  I think it has changed our family.  The nurses say what a great team Chris and I make (Team Hulse, Woop Woop!!!) and I think it has brought all of us closer together.  Although I love London and have had fun playing "grown-up", I will be sooo glad to go home!!

Me, Holding A Gravity Feed Unit

WE HAVE NEUTROPHILS!!! T+11

Days run into each other in this place.  I hadn't realized until last Thursday that I hadn't left the hospital in four days.  Also, I now fully understand why sleep deprivation is used as a form of torture. 

On Thursday, T+8, Miya's platelets were decreasing and her neutrophils were at zero. Neutophils are produced in the bone marrow and circulate in the blood.  They make up about 50%-75% of white blood cells and are the first type of immune cells to respond to infection.  Also on Thursday, Miya spiked a fever (anything above 38°C is considered a spike). Miya was given standard treatment of fever reducer and antibiotics and she responded well.  Her pain relief was increased to oral Morphine due to mucositis developing.  Her mouth is sore and red, and bleeds when we do her mouth care routine.

On Friday, T+9, Miya's pain relief was increased to Morphine via her Hickman line. She now receives a small, steady dose called a "background" at all times and can have a "push" of Morphine when she becomes distressed (ie. when we do her mouth care). 

Saturday, T+10, is when we noticed Miya's hair beginning to fall out.  Although we shaved it prior to being admitted, it had begun to grow back over the nearly three weeks we've been here.  Miya had a bad morning, complete with lots of screaming. She had a push of Morphine and after we dressed her and completed her mouth care routine, she settled nicely and had the best day she's had in a while.  She played all day and chattered and laughed.  She even ate 1/4 of a chicken nugget and 8ml of Ribena blackcurrent juice.  She also began having some diarrhea yesterday (which I'll take any day over vomit.  I don't do vomit).

Saturday night prooved to be a horrible one. Miya didn't settle until nearly midnight and our nurses were noisy and kept forgetting to turn of the light when they left.  Machines were constantly dinging and clanging for some reason or another and I only got about two hours of sleep. 

This morning, I went to the nurses' station to get Miya's blood results and was delighted to find that she has Neutrophils!!! They are currently 0.06 (normal neutrophil counts are between 3000-6000 in each mililitre of blood). When Miya's neutrophils are  0.2 for three consecutive days, we will no longer be on "Green Precautions" (the strictest kind of precaution-wearing plastic aprons, crazy hand washing, etc.) and Miya may be allowed out into the ward corridor in the evenings when there are fewer people around.  I CAN'T WAIT FOR THAT!!!!

TRANSPLANT DAY!!! Wednesday, the 19th of October, 2011 - T- 0

It's Transplant Day!!! Miya's donor cells should be in the building, and the volume being reduced at the lab. The volume is reduced to allow for the fact that our donor weighs 60kg and Miya is now hovering around 11kg.

On Sunday night (T-3). Miya awoke during obs (or "observations"-temperature, blood pressure, pulse rate, etc.) and was difficult to settle. She ended up having codeine and I slept in her hobbit-sized bed with her.

Miya was experiencing some peripheral coldness and blood samples were taken to check for infection. So far, Miya's results have been good and there is no sign of infections.  We did, however, start to bundle her up and her umbilical hernia subsided. Her belly is back to normal size.  We feel that because she was tensing in the cold, it caused her umbilicus to emerge from the split in her immature abdominal muscles.

Miya's appetite and weight have decreased since Sunday.  She began to refuse food on Monday (T-2) and she had a nutrient-rich milk feed through her NG tube.

On Monday, (T-2) we began wearing plastic aprons when in the room with Miya and having to wash and gel our hands in a manic ritual to prevent infection.

On Monday night, Miya's NG tube came out while she was having an uncontrollable fit of rage, and the nurses had to put another one in.  At the time, she was having a milk feed through the tube and it slowly leaked all over her pyjamas. 

When an NG tube is inserted, a small sample of stomach contents are extracted into a syringe to test the PH. A PH level that is unlike the acidic stomach acid can indicate that the tube is not in place correctly.  Each time Miya receives something through her NG tube, a sample is taken and tested to insure Miya will be receiving the medication/milk into her stomach.  Because Miya had not been eating, a sample could not be  retrieved and tested and the milk feed was scraped.  She continued to receive IV fluids and I slept with her again as she was diffucult to settle and didn't want to be alone.

On Tuesday (T-1), the NG tube was tested in the morning after I got Miya to drink some water after having her teeth brushed. It was indeed successfully placed.

Miya started Aciclovir, an antiviral drug on Tuesday and started Ciclosporin on Sunday. Ciclosporin is an immunosupprescent given so that Miya's own body will not reject the donor's cells.  The doctors will control the dose of Ciclosporin so that the stem cells will be introduced to Miya's system gradually. 

Last night, (Tuesday night) Miya slept well and I got to sleep in my bed (which is not Hobbit-sized).

An interesting point: Miya has not vomited or had diarrhea yet. She has not had a fever since the first dose of Campath.

Miya continues to take a variety of medicines via her Hickman line and through her NG tube. She takes an antifungal, antisickness, some antibiotics, some pain medication when required, some antihistamine to reduce any reactions she might have.

***A NOTE ON TRANSPLANT***

A bone marrow tranplant, as some believe, is not an operation. Bones are not opened up, marrow placed, and then bones screwed shut. How painful!!

Remember when I explained the chemo drugs and their purpose? If you remember, the purpose of some of those drugs is to tunnel holes in Miya's own marrow. This is so when the donor's cells are infused via IV line into her Hickman line, they will migrate from Miya's bloodstream into the holes in her own marrow.

Bone marrow produces new cells, and contain stem cells. Stem cells (found in umbilical cord blood) can differentiate into a variety of cells. In a transplant, the donor's bone marrow is harvested and the stem cells are extracted. These stem cells are placed into an IV bag and infused over a period of time (4-8 hours) into the recipient. Sometimes, recipients can have a reaction, so the infusion is slow. 

Our nurse has just gone to phone the lab and we have found out that Miya's cells are here and being worked on right now!! They should be on the ward arounf 1400hrs-1500hrs!!

Sunday,16th of October (T-3)

 

I seem to have lost all track of time in this place. Days seem to run into each other, and time runs together.

 

Miya has not had a temperature since the first night of Campath. She doesn’t have diaper rash, and hasn’t had vomiting or diarrhea.  Her blood pressure and weight have also been good. Her appetite is lagging a bit and tonight, she will be having some IV fluids to top her up. She is also excreting more liquid than she is taking in. These IV fluids will help replenish the fluid she is losing.

 

She sleeps a lot, and is grumpy. Sometimes in the evening, she wants to walk around a bit and push the IV trolley. 

 

She is very cold, all the time. Her skin is cold to the touch. We keep her bundled up all the time now.  The doctors are checking her for infections. A Hurler’s child in the past developed an infection after being cold for several days. If she does have an infections, they will begin antibiotics.  We will know the results this week. 

 

Yesterday was the last day of Campath (a 6 hour infusion) and today is the last day of Busulfan (Two infusions of 3-4 hours each, one taking place through the night. Tomorrow she will have her last dose of Fludarabine (about an hour) and her regular ERT (which she will have weekly for 4 weeks. Hopefully by then, she will have engrafted and will be producing her own enzyme).

 

We have not seen any mouth ulcers yet, and her mouth was clean and clear of any redness when the nurse checked this morning. She has her teeth brushed twice a day, and has her mouth swabbed with mouthwash twice a day.

 

Chris and I take turns holding her on our laps all day. Sometimes she wants to look at books, but mostly she wants to hold her seahorse and stare or sleep.

Thursday, 13th October 2011 (T-6)

 

***A NOTE ABOUT THE TITLE***

From now on, I will be including the letter “T” followed by a –/+ number. This indicates the following:

T=Transplant

-/+ number=the days until and post transplant. In today’s case, –6 indicates 6 days until transplant (Miya’s transplant is next Wednesday). Transplant day is day 0 and each subsequent day will appear as a positive number. (For example,  the day after transplant will be T+1.)

Today was a very busy day. Miya began the day with a good breakfast, but steadily lost her appetite throughout the day. She will be fed special nutrient rich milk through her NG tube overnight, for 12 hours to make up the nutrients and fluids she did not eat/drink.

We were told today that up until Sunday evening, Miya would be free to go into the play room on the ward but that has now changed. She has a bug called C Diff (Clostridium Difficile) in her stool and we are now in isolation. C Diff is a bacteria naturally found in the gut of around 2/3 of children and 3% of adults. It doesn’t pose problems in healthy people however it can multiply and produce toxins causing diarrhoea and fever. Miya won’t be treated right now, the isolation is just a precaution.

Today, we continued Campath (the drug that causes massive breakdown of the T and B Cells-Types of cells that protect the body from infection) and started a chemo drug called Busulfan. Among other things, this drugs attacks the bone marrow, creating a tunnel for the new marrow to reside. 

A volunteer called Margaret (a lovely Irish woman) came today and sat with Miya for an hour while Chris and I went out to food shop and have coffee, etc.  Miya did well and was sleeping when we came back. 

Tomorrow, Miya will continue the Campath and Busulfan. She will also begin a chemo drug called Fludarabine. This drug also attacks the bone marrow.

Miya is not her usual self and likes to sit quietly on our knees most of the day. She likes to run around a play a little in the day but by the afternoon, she  seems to be exhausted.  She has also developed an umbilicus hernia, where her umbilicus is popping through her abdominal wall. This is quite normal, I have been assured.

 

Umbilical Hernia                                                            With Volunteer Margaret

A More Detailed Update

 

We arrived on Fox Ward at Great Ormond Street Hospital on the 10th of October and were admitted for BMT.  A small amount of fluid was suctioned from Miya’s nose and tested for cold & flu viruses. If any viruses were found, we would be sent home and the BMT rescheduled.  Since they are eradicating Miya’s immune system to make way for new bone marrow to be accepted, it is not a good idea to continue with a transplant if her body is fighting  off infections, etc.

I was reluctant to unpack until we got the results of Miya’s virus tests, but after several hours in a small room with everything in suitcases I broke down and unpacked. Thankfully, around 1800hrs, Miya’s tests came back negative and we were cleared to proceed.

Miya began receiving many prophylactic medications via syringe on Monday evening. She received medications to protect her kidneys, to protect her liver, an anti-nausea medication, and some antibiotics.

Since chemo can cause a breakdown of the mucous membranes – called Mucousitis (all the way from the mouth to the bum) we have to clean her mouth four times a day.  We brush her teeth twice a day, and swab her mouth twice a day with sponges soaked in a mouthwash solution.  This helps to prevent mouth ulcers and other nasty things that can happen when mucous membranes begin to break down.

Miya had an NG tube (nasogastric tube) inserted yesterday. Through this, she received her oral medications. Some of them are particularly nasty tasting and having them down the NG tube avoids her having to taste them. Also, it is easier to insert it now, while her mouth is not affected by chemo, than have to do it while she has a sore mouth.

Miya’s weight is good (12.05kg / 26.5lbs) and her appetite remains healthy.  She had her fists dose of chemotherapy administered yesterday, infused very slowly over a long period of time (8-9 hours).  She also received a dose of pethidine (demerol).  She takes drugs prophylactically each day, several times a day.  The chemo she is on presently is called  Alemtuzumab, more commonly known as Campath. This is a chemotherapy which destroys Miya’s T-Cells and B-Cells (involved in white cells counts, and relate to immunity).  As the T-Cells break down, they generate a lot of heat. It is not uncommon to develop a fever, which Miya did. It was treated with paracetemol (similar to Tylenol) and steroid. The fever began to come down and was just about normal when Miya went to bed. 

Because the chemotherapy chemicals are excreted in waste products, Miya’s bum is beginning to be sore and I change her bum every two hours or more. I use a special cream to clean her bottom and a special barrier cream to protect against diaper rash, etc.  Also, as chemo can thin and dry the skin, I have to bathe her in a solution of water and a special oil, and then slather her in a special cream.

Miya has observations done throughout the night (every four hours or so) and although she wakes up, she is very quiet and doesn’t cry. She goes straight back to sleep.  The bed where I sleep is separated from the main room by a curtain, so Miya and I both have our own space at night.

Miya’s second dose of Campath begins today between 1200hrs-1400hrs and will run at a slightly higher rate than yesterday.

It’s Go! Go! Go!

 

This is going to be the quickest blog update ever.

 

Miya passed her snot test yesterday and we began chemo today. The chemo is still infusing slowly, and there’s about a half hour left to go.  Miya spiked a temp (38.2 C) which is normal as her T-Cells produce heat as they break up. This is being treated as per normal protocol with paracetemol (UK version of Tylenol) and  steroid. 

 

Miya continues to eat and drink normally. She does, however, have a red bum due to the chemo chemicals being excreted in her urine and faeces. We are treating this with special cleansing agents and a powerful barrier cream. 

 

Miya is examining Simba with a flashlight right now, and earlier, she tried to feed him a biscuit.  She is in high spirits, although earlier today when she was given Pethedine (aka Demerol) she was a zombie for a while, and then fell over several times when she tried to walk. 

 

Miya was given an NG tube today, which made me have to go to the other end of the room and look away.  It wasn’t cool.  She’s fine with it now, and isn’t really picking at it.  She’s more interested in what’s going on with her Hickman line.

 

Off to put Miya to bed now.  Will update as and when I can.

All Systems…Go?

Can it be true?? The donor passed her medical? We have a bed on Fox Ward? 
We received confirmation today via the lovely Helen who works in the BMT department that the donor passed her medical with flying colours.  We have a confirmed bed space on Fox Ward at GOSH and we are to be there for admitting no later than 0900hrs on Monday morning.  On Monday, Miya will have a sample of nasal fluid taken (the polite term for “snot”) and it will be tested for the cold and flu viruses.  The results should hopefully be in Monday night, but most likely Tuesday morning.  If Miya is carrying a cold or flu virus, we will be sent home and the transplant will be delayed yet again. (Picture me with my grumpy face on.)

We are taking our dog to the dog-sitter’s in the morning. He’ll stay with them until our discharge.  Poor Mr. V. 

Tomorrow will be ERT no. 30 and our final trip to Kingfisher ward (I hope).  Miya will continue to receive ERTs on Fox ward until her donor cells engraft and she begins to produce her own enzyme. 
Apparently, there have been small amendments to the protocol, and we will be advised of these changes on Monday during our admission process.  As far as I’m aware, chemo will begin on Tuesday the 11th of October and Miya will receive her donor cells on Wednesday, the 19th of October.  The donor’s marrow will be harvested in Germany on Tuesday the 18th of October and air lifted to London. Miya will receive her new cells on Wednesday, the 19th of October, two days before my 32nd birthday.  What a fantastic birthday present to receive…my daughter’s life. 

Please think of my family, faithful readers, as we travel to London early tomorrow morning, and again early Monday morning.  I WILL be updating the blog with new information and a progress report as and when I can.  I am going to try and blog every 3 days or so, depending on if I have a spare moment. 

I'll Take A Large Double Double With Milk & A Snack Pack Of Timbits

Nothing much to report from the Hulse household.  I had my  lump checked out at the Breast Clinic in Bath and was told that the breast tissue felt normal (whew, big sigh of relief). An ultrasound is going to be performed at a later date, just for the sake of completion, but I'm confident everything will be fine.

Miya and I have had HORRIBLE colds. Miya is not a cuddly child, so I knew she was sick when she wanted to cuddle between us in bed last Thursday and Friday night until she fell asleep.

This Friday at our regular ERT, we are meeting with Helen, the BMT nurse, to go over Miya's chemotherapy protocols.  We start Itraconozole AGAIN this Saturday. (Itraconozole is an anti-fungal medicine that is given daily one calendar month prior to transplant to kill any fungal infections that Miya might have.) She'll get 5.5ml per day and it tastes like shit.

Miya took her first unassisted steps on Kingfisher Ward last Friday. She hasn't done it since; she just wobbles around like a drunk when I try to get her to walk unsupported. 

We also have some potential good news on the accommodation front. It is possible that Chris may be able to stay at Wellington Barracks in London for the duration of the BMT.  It would cost us very little (if anything) and it is only a 15 minute walk from the hospital, close to Buckingham Palace.  I'm hoping to find out more this week. 

As for me, I am missing Tim Horton's, Wal Mart, repeats of The Red Green Show, and the old music from Hockey Night In Canada. I also miss old reruns of Degrassi Junior High and Trailer Park Boys. If anyone should have a box of Canadian cultural classics, mixed with a friendly smile and some of those squee-gee things from gas stations (non-existant in this country...imagine wanting to clean your windshield at a gas station!!) please send them my way.  I'd also like a box of Ganong Cherry Chocolates, some Hershey Kisses, and a snack pack of Timbits. And a Large Double Double with milk. Oooo! And how about some Baxter's Chocolate Milk, eh?? Please?? Am I ever going to go home again??

A New BMT Date

I received an email this morning from the BMT team who advised that the BMT has been delayed by two weeks.  The proposed dates were rejected by the donor's centre (for whatever reason) so we will be admitted on the 10th of October, chemo to begin on the 11th and the transplant day to be the 17th of October.

I will be spending my 32nd birthday in the hospital and it is a very real possibility that we will be spending Christmas/New Year in the hospital as well. I have asked about Christmas presents and have been told as long as the things we get her are new and in the original packaging she will be allowed gifts.  The ward make a great effort for families spending the holidays in the hospital. On the bright side, I won't need to cook Christmas dinner or decorate the house!!

The hospital staff have been excellent, and have provided Chris with whatever documentation he needs for work in order to get the time off work.  

I don't mind the two week delay. Miya's so active and awesome right now, I really enjoy spending time at home with her.

Here We Go Again...

On Friday at our regular ERT, the lovely Helen told us that the BMT admission was now scheduled for the 26th of September. "WooHoo!" we thought! THEN we were told that it was our OLD DONOR that was donating. She is now well, been cleared for donation by the Bone Marrow Registry, and is able and willing to donate.

During the last three weeks they have been searching for a donor, and they have only found two 9/10 donors and a Cord Blood donor (who was a 5/6, I think). No 10/10 donor was found. IF something goes "tits up", and the 10/10 donor gets sick AGAIN, there will not be a long delay, and they will slot us in with the cord blood donor. Helen told us that in all her time working as a BMT nurse, this has NEVER happened to her.

That's all (for now), folks!!!!!

Hyper Mobility In Hurler's?? Really??

On Thursday, Miya was evaluated by the lovely Michelle, a Physio Therapist at Great Ormond Street Hospital.  We love going to see Michelle and often pop up to the Physio floor when we don't have physio appointments, just to say hello. 

When Miya was first evaluated back in April, her joints were hyper-mobile. She did, however, have some stiffness in her shoulder joints. This stiffness is common in Hurler patients as they lack the ability to break down mucopolysaccharides (GAGs) and these accumulate in the joints, among other places.  (For some technical terms and links to more information on them, see this post.)  Because, at the time of the first evaluation, she lacked the muscle tone required to control her hyper-mobile joints, she was not yet crawling. The physios were confident that with time and enzyme therapy, she would crawl fairly soon after the evaluation. Within 4-5 weeks, lo and behold, Miya was crawling!!

At this pre-BMT evaluation, Miya's joints are still hyper-mobile and the stiffness in her shoulders was gone!! The enzyme infusion she has been receiving is obviously working very well, and doing its job of breaking down the GAGs. Michelle said that Miya is doing all the age-appropriate things she should be doing, and doing them very well. She had no advice for us or exercises for Miya. She did give us a tip on giving her confidence in walking unassisted, which involves holding onto a rubber ring.  (She currently cruises around, holding onto furniture, people, toys or the dog. Sometimes it seems as if she teleports, getting from one place to another in a blink of an eye.)

Hyper-mobility is not often seen in Hurler children, and is often due to some hereditary component. We can only hope that she won't regress too much during BMT (although some degree of regression is common) and that she will continue to flourish under the ERTs that she is receiving. We also hope that the BMT is successful, and that the marker that creates the enzyme she lacks becomes 100% donor, therefore delivering enzyme to not only her lower body, but also to her brain (a place that the enzyme infusion cannot reach. GAGs continue to build up in Miya's brain, despite receiving weekly enzyme replacement therapy). 

`Twas brillig, and the slithy toves...

For those of you who aren't familiar, the title of this blog post is from Lewis Carroll's Jabberwocky (from Through the Looking-Glass and What Alice Found There, 1872.)  When I was young, this was one of my favourite poems (along with The Walrus And The Carpenter, which also featured in Through the Looking-Glass and What Alice Found There, 1872).  In 1985, Alice In Wonderland was made into a TV movie and in 1988, it was shown on the local TV station where I lived at the time. It was on too late at night for me to stay up and watch, so my mother stayed up to tape it for me. (Yes, I said tape. This was back in the day of VCRs. We all remember VCRs, right? Incidentally,  I also remember records and 8-tracks, too.)  As I recall, she also paused the recording at all the appropriate times so as to eliminate the commercials. Anyway, it was televised over two nights and I remember eagerly awaiting the second part so I could watch the whole film.

I was captivated. I loved it. Until the Jabberwocky came on. I was terrified. Fear gripped me like nothing else had before and put a sinking, dreadful feeling in the pit of my stomach. I think I cried. I certainly looked away in parts. I remember the Jabberwocky being the worst thing to happen in life. Ever. I searched online and found a picture of this fantastic, fear-inspiring creature. Here it is:

Not so terrifying as an (almost) 32 year old woman, I must admit.

You may ask yourself why I'm telling you this. I am telling you this because I wanted to give you some idea of how I felt when the phone rang late Wednesday afternoon, and I saw that it was a call from Great Ormond Street Hospital.  Worst.Feeling.Ever.

Before the CNS could even utter the words "Hi, this is Helen from BMT", I knew. I knew. And my stomach fell out. And I wanted to hide my head under the covers. And I immediately thought of my childhood nemesis, the Jabberwocky. It wasn't the fear that got me, it was the dread. The stomach-sinking feeling. The panic. The knowledge that I had to face this "monster" in order to get to the end, and I couldn't fast forward past the horrible parts.

If you haven't been keeping up with Facebook, or haven't guessed yet, Miya's BMT was postponed. The donor failed her medical and was not able to donate.  Devastated. We have, however, been given a time frame. They have one month to find another 10/10 donor, and if they don't, they have a 9/10 donor that they will use.  There is an increased risk of GVHD with a 9/10 donor but if the doctor's a happy to proceed, then so am I.  After all, I didn't go to medical school for years and years. I'm no specialist.  

I think I went through the five stages of grief in about 24 hours.  I accepted it and moved on. Chris and I accepted it and moved on. Miya doesn't care.  She doesn't even know. She's bald and she's cool with that.

So now we wait. Again. That's OK. I'm down with that. I can dig it.  After all, it's not as if it's the actual Jabberwocky, is it?

The Haircut

I cut Miya's hair today.  It was/is a big deal for me.  The haircut makes things real, you know? I dreaded the haircut because I knew it would make things real for me.  I had a little panic attack afterwards.  I bought her a beanie from Baby Gap so she wouldn't have to be bald in public.  I hate it when people stare at bald babies and think "Look at the poor cancer baby".  She can't help it that she needs chemo for her bone marrow transplant. She doesn't mind the hat, but she does tug at it from time to time.  We were out for a bit today and I had taken her hat off her.  People kept staring and I was wondering what they were staring at.  Until I remembered the haircut. 

Each time I look at her, I get a little jolt and think "What happened to your hair?!".  Then I remember...

On a brighter note, we received some funding from SSAFA and have found rooms available at the Union Jack Club, a hotel for service personnel and their dependents. It's about a half hour walk away from the hospital. If it's raining, we can take the tube but we have to be mindful about touching our hands to our faces, mouth, etc. to minimize the risk of infection. If one of us gets sick, we can't go in with Miya. If Miya gets sick, it could be disastrous.