"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

T + 17 Some Good News!!

 

This week, Miya has been fabulous! Her neutrophils are steadily increasing and this week, they will be doing some tests which determine how she is engrafting and if these are Miya’s neutrophils or the donor’s neutrophils.

On Monday, Miya ate a little piece of a Nutragrain Bar and on Tuesday she ate more Nutragrain Bar and drank some juice. On Wednesday, she came off the background of IV morphine and was eventually disconnected all together in the evening.  She ate one slice of toast with butter and jam and drank some juice. 

Despite having a little appetite back, Miya is still receiving two bonus Pediasure milk feeds and an overnight Pediasure feed through her NG tube.  Some people are a little confused about the difference between the NG tube and the Hickman line (which gives IV access).  The NG tube is a tube that goes up her nose and into her stomach.  It is used for milk feeds and giving oral medication.  The Hickman line is linked directly to a vein and gives venous access to IV medications. 

On Thursday, we we advised that her counts were definitely on the rise and if they continue, Miya will be taken off supportive medications at the beginning of the week (ie. anti sickness medicine). We were also told Miya is the only child on the ward NOT on / never been on TPN!! TPN, as I explained in a previous post, is Total Parenteral Nutrition and  is given through the Hickman line (intravenously). It is nutritional support for children who do not tolerate milk feeds and / or who are vomiting a lot. 

On Friday, Miya’s mouth seemed a bit better and her voice wasn’t as hoarse.  We were also advised that if she is doing this well next week, WE MIGHT BE ABLE TO GO OUT FOR A WALK OUTSIDE!!!!!!!!  Also, we will start converting IV drugs to oral medications!!!! This is a HUGE step in the recovery process. 

I had mentioned on Facebook that if Miya’s neutrophil counts were above 0.2 for three consecutive days, she may be allowed out into the corridor.  Miya will not be going into the corridor because of the possibility of catching viruses that may be going around the ward and because of the possibility that she may pass a bug called C Diff to other patients.  (Miya was admitted with a bug called C Diff in her poop.  Please see earlier posts regarding our admitting process if you don’t remember.) Fresh air will be good for Miya and it is better than walking around a hospital full of sick people. 

Fingers crossed, this week coming will allow us to convert IV drugs to orals and allow us to go outside!!

I have started thinking about Christmas and have even purchased a few items for Miya!! (Thanks, Lisa, for letting us use your house as a dumping ground for this stuff!!) My mum has sent me money to get Miya a Little Tykes Kitchen and we have bought her some BSL (British Sign Language-Baby Sign) books.  We also want to get her a toy Silver Cross stroller.  I am SO EXCITED for Christmas this year!!  I LOVE TOYS!!

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!!!!

Counts Nearly Zero T+7

Miya continues to have a decreased appetite.  She is nourished almost entirely via her NG tube with Pediasure milk and water.  On Friday and Saturday nights, Chris stayed with Miya and I stayed at the flat.  Miya continues to take anti-sickness, anti-rejection, anti-fundal, and anti-viral medicines. 

On Sunday evening, the phone rang and no one answered when I said "hello".  Miya woke up and was FURIOUS!! It tooke me forever to get her settled again and she managed to get her NG tube out.  She was on a milk feed at the time.  Milk was all over her and all over the bed.  The NG tube was reinserted on Monday morning. 

On Monday, Miya's red cell count was low and she was to receive red cells Monday evening.  The lab sent up the wrong blood type (Miya's pre-transplant type, rather than her post-transplant type) and Miya was given the transfusion on Tuesday. 

Miya also developed some mucositis on Tuesday and we have a special rinse we swab her mouth with 3-4 times a day.  She also developed a couple of small patches on her bum where the skin was peeling back.  A couple of nights ago, she had a student nurse looking after her and I don't think she knew she was meant to change Miya's bum in the night. I slathered her bum in the 50/50 cream they make up here (half Vaseline, half Metanium) and the nurse she had last night did the same, and this morning her bum looks much better.

Miya woke this morning at 11.00hrs and refused food and water. She was put on a boost Pediasure feed.  She is now sitting on Chris' knee.

The doctor told us this morning that her neutrophils are nearly zero. They will remain at zero until she begins to engraft. At this time, she is susceptible to fevers and infection.  This is normal and she will be treated with antibiotics as required.

Also, if Miya's weight begins to drop (so far it's holding steady at her admission weight) or if she develops mucositis in her gut and begins to vomit from the milk feeds, she will be put on something called TPN (Total Parenteral Nutrition).  This is given intravenously via her Hickman line and to begin, she will probably be on it 24 hours a day. 

Low White Count, Hemoglobin & Platelets Okay T+3

Wednesday, T-0, was uneventful in terms of transplant.  Miya sat quietly while the cells were being infused.  She smiled and laughed a lot and had a milk feed through her NG tube overnight.  The tube came out overnight and was reinserted the next day.  Miya was also difficult to settle.
On Thursday, T+1, Miya played well in the morning and ate a lot of custard.  She gagged and vomited a little when the NG tube was being inserted.  Her food intake was low, and steadily decreased throughout the day.  Miya slept well and had a milk feed thorough her NG tube during the night.

Friday, T+2 was my 32nd birthday. Lisa came from Odiham to spend the day with us and to look after Miya for a few hours. Miya had a boost feed of milk through her NG tube at supper time and her regular slow milk feed throughout the night. Chris stayed with her overnight in the hospital and I stayed at the flat. 

This morning, T+3, we were advised that her white cell count is now zero and her red cells and platelets are fine.  The white cell count will remain at zero until the engraftment process begins and the count will begin to rise.  During this time, Miya may become quite ill and have fevers.  This is all normal and to be expected.  This morning, Miya showed the first signs of being unwell when she didn’t wake until nearly 1100hrs. She has returned to sitting on our knees, rather than running around and playing as she has been doing over the past couple of days.  
She received the first of two doses of a drug called Methotrexate, which helps to prevent Graft vs Host Disease (GVHD).  GVHD is a complication that can occur after a stem cell or bone marrow transplant in which the newly transplanted cells attacks the recipient’s body.  Symptoms include:
Abdominal pain/cramps, diarrhea, fever, skin rash, vomiting, weight loss.  A rash seems to be the most common symptom and we are watching closely for this. 
Many people are unfamiliar with the importance of staying germ free during transplant, and don’t think twice about doing things that we constantly have to give thought to.  For example, our bedding has to be changed every day.  All of us must shower and wash our hair each day. Miya’s cups are sterilized and she can only drink cooled, boiled water.  Her teeth and mouth are cared for using sterile water and each day, a new toothbrush is used. Mouth swabs for mouthwash (to prevent mucositis) are one use only.  Each article of clothing we put on has to be clean and dried in a high heat tumble dryer to kill any bacteria.  We must all put on clean clothes in the morning, and all clothes we wear to bed also must be fresh and clean.   Hands must be scrupulously washed and hand gel applied when we enter the room and leave the room and when we enter or leave the ward. Hands must be sanitized upon entering and leaving the kitchen facilities.  We have to wear white plastic aprons over our clothes.
A trip to the kitchen looks like this:
Take off apron in room, wash and sanitize hands. Leave room. Wash and sanitize hands on the way out of the ward. Sanitize hands before entering the kitchen. Sanitize hands when leaving the kitchen. Wash and sanitize hands coming back on the ward. Put on plastic apron. Enter room. Wash and sanitize hands.  I must admit that my OCD is a little out of control right now. 

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. 

Miya Laughs

Time Keeps On Tickin'

I haven't written in a while, so I thought I'd write a little update.

Not much to report, really.  Miya started walking on her own on Sunday the 11th of September.  I was making brownies in the kitchen at the time. By the time Chris got over the shock and awe and called to me, she had made it all the way across the living room. I caught her walking into the TV stand.

Miya has also started saying "what's that?" and pointing at various things. She can point to her toes when you ask her to.

We got Miya's chemotherapy protocols on the 9th of September. This is basically a schedule of the different drugs she'll be taking and we also went over the side effects of said drugs. A couple of things which struck me are these:

1)  A donor and a recipient may not necessarily have the same blood group. Blood groups need not match in order for tissue types to match.  The thing that amazes me is that by the time the transplant is over and Miya is out of semi-isolation at home, Miya's blood group will have changed to match that of her donor's.  Miya is currently O+ (like Chris and myself) but will turn O- (like her donor). 

2)  The second thing (which is more interesting than it is amazing) is that since chemotherapy changes the skin, Miya will have to wear sunscreen for life, even in winter. The importance of this was stressed to me. Long sleeved shirts, big hats, LOTS of sunscreen. 

We also know some information about our donor. The donor is female and a German. She is 50 years old and weighs 60kg.  I also know that in order to be deemed fit to donate (from her failing medical back in July) she had to go through many, many, many kinds of tests and procedures. Apparently the work-up this woman had was phenomenal. She could have said "No, never mind" or "I can't go through with these tests, remove me from the donor registry" but she didn't. She gave up a lot of time and went through a lot of medical testing just to be able to be cleared for donation. To save Miya. How do I find the words to thank this woman for all she has done?

Want Some Chicken?

We've all been sick with a stomach bug and for the first time ever (even as a little baby) Miya threw up. In the car. All over her car seat. I can't get the smell out and I'm tired of trying every piece of advice someone gives me about how to get the smell out. It ain't comin' out.  Nan H and Grampy Green (my parents in Canada) have generously agreed to buy Miya a new car seat. 

So we're on a countdown now. 20 days until admission for BMT. Three more regular ERTs on the ward.  In the words of the iconic Steve Miller Band, "Time keeps on tickin' tickin' tickin' into the future....Tick, tick, tick, tick....

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??

The Lump

There's a lump.

In my right breast.

I'd been having teeth-gritting pain in my right breast and finally (after much urging from Chris) went to the doctor. Because I was mid-cycle, she asked me to come back in a couple of weeks for a more accurate examination. I returned yesterday. 

The doctor found a lump in my breast and I've been referred to the Breast Clinic at the Royal United Hospital in Bath. The lump is painful and hard. It doesn't move. The doctor says not to worry. She's right. It's probably nothing. Better to be safe than sorry and all that, but I'm still shitting the bed.

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!!!!!

Gargoylism

gar·goyle  (gärgoil)

n.

1. A roof spout in the form of a grotesque or fantastic creature projecting from a gutter to carry rainwater clear of the wall.

2. A grotesque ornamental figure or projection.

3. A person of bizarre or grotesque appearance.

gargoylism (n.) - hereditary disease (autosomal recessive) consisting of an error is mucopolysaccharide metabolism; characterized by severe abnormalities in development of skeletal cartilage and bone and mental retardation

Gargoylism. Go ahead, Google it (or use the search engine of your choice). I'll wait...

You probably came up with around 87,000 hits.  87,000!!

Gargoylism is a term for Hurler's Syndrome (MPS 1H) and is still used today.

This is a Gargoyle:

 And this...

And this...

 

This is Caleb. He has MPS 1H

 This is Chiara. She has MPS 1H

 This is Torie. She has MPS 1H

This is Sophie. She has MPS 1H

This is Cody. She has MPS 1H

This is Jakob. He has MPS1H

  And of course, Miya. She has MPS 1H

Gargoyles? I think not.

I have been trying to think how best to describe the way I feel when I hear that awful term. Gargoylism. Yuk. The feeling I get in my heart is a terrible, sinking feeling. 

I came across what must have been a student's project for school or a biology class of some sort as I was posting videos of Miya on YouTube. (Click here for the link.) This was posted just last year!! Last year!! It brought to my attention that this horrible, degrading, archaic word is still being used today to describe beautiful children like the ones you see above. (By the way, I do have the parents' permission to use these photos, in case you were wondering.) 

It is my hope that when someone searches the term "Gargoylism", maybe they'll come up with this blog post. And click on it. And stay on it long enough to scroll down, and see that these people aren't gargoyles. They're kids. They're people. One of them is MY kid!!I admit, this once was a term used to describe MPS 1...

BEFORE THERE WAS A NAME FOR IT!!!

If you're reading this for a school project, or for research purposes, or whatever, please, please, please think twice before using this horrible, derogatory word in your essay or paper or presentation. These are people! They have families and friends who love them very much. They are loving and love to be loved. They are regular people who like to do regular things, and just happen to have a terrible illness. That illness has a name. It is Mucopolysaccharidosis Type 1H also know as Hurler's Syndrome.  Please use the correct term instead. We as parents of children with MPS 1H thank you. Our children thank you.

An iPad 2 For Miya

I do not consider my daughter to have a disability; she is a perfectly able individual. She is right along side her peers in all areas of development. I am, however, not blind to that fact that someday she may need some assistance in some area or another. I want her to be fully prepared and ready to take on anything.

My parents bought Miya an iPad. iPads are wonderful tools for children with and without special needs. The iPad will assist in her cognitive development (unfortunately, until Miya has her bone marrow transplant, mucopolysaccharides continue to build up in her brain), assist with Occupational Therapy, language skills, word and object recognition, as well as her fine motor skills. (People with MPS and related diseases have unique hands. The shape of the hands is very noticeable. They are short and broad with stubby fingers, which can become "clawed". Hands are the symbol of the MPS Society.) Already, Miya has got the hang of many apps, and loves anything to do with "peek-a-boo". She loves apps which allow her to create music and draw with her finger.

We have invested in the Gumdrop Drop Series Military Edition case for this iPad. I think drop protection for this valuable piece of equipment is essential and after viewing this on the blog of another MPS family (see Saving Case) I decided on this particular cover.

Many of you might think that an iPad for a one year old is a little excessive. I might have agreed with you a few weeks ago, before I had the opportunity to see Miya use one and before I did some research. Not only is it a fun tool, it is also an educational tool. It helps ease boredom and provides distraction for sometimes painful medical procedures. 

Miya would like to say a big "THANK YOU!!!" to Nan H and Grampy Green!!! Oh, and I would too.

A Double Lumen Hickman Line For Miya

Miya was admitted to Great Ormond Street Hospital on Sunday, the 24th of July, for the insertion of a Double Lumen Hickman Line on the following morning. This is a central line which links directly to Miya's blood supply. Blood can be taken from it, and medications injected into it.  It will also be used for Miya's weekly enzyme infusions. Venous access has been tricky as of late, and this will make our lives MUCH easier. 

Miya Munching A Banana After Coming Back From Surgery

Miya tolerated the procedure well. She was a little bit cranky after coming back from the recovery room (who wouldn't be). She didn't want anything to drink, but was happy to clutch a banana and shove it into her mouth at regular intervals. 

Miya did so well, she was discharged later the same day! She was difficult to contain in her bed after a couple of hours of being back from recovery, so she crawled up and down the halls of the ward.

It took us nearly two hours to get out of London and a further three once we were finally on the M3 Motorway.  

Miya doesn't seem to realize she has a new addition to her body. She hasn't pulled at it (yet) or even inspected it.  Her dressing was changed yesterday without incident and she also had her first bath with it in (with her chest wrapped in plastic wrap).

Miya And Mummy

 She hasn't really been herself since we came home, but she's no worse than I'd expected her to be. A little cranky from time to time, and a little clingy. She often pulls herself into my lap with a book or her Simba soft toy and sits for a while. It's nice. She's not usually a cuddly child. 

We're off to GOSH on Thursday night for our regular ERT on Friday morning.  I can't wait to see how the Hickman performs!! Looking forward to no canulas, no crying, no having to keep a 12 month old still or else the pressure in the canula sets off the machines.  

Hooray For Miya The Super Trooper!!

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?

"A Wrinkle In Time" or "When Did I Get So Old?"

NOTE: This post isn't about Miya.  It's about me. So if you come to this blog to read about the kid with the rare disease, don't bother reading this post...there should be a new Miya update on Friday or Saturday.

* * * * * * * * * *

My reflection does not resemble the image I have of myself in my head.  I realized this today as I took a really good look at myself in the mirror...for the first time in, well, a looong time.

In my head, I look 26 years old. (See photo above, so you can have the image of my 26 year old self in your head too, and at least play along with my little fantasy.) At 26, I drank more, smoked more, had poor judgement, and had less tattoos.  I don't want to be that person anymore, I just wish I looked more like her.


"Where," I wonder to myself "did those winkles around my eyes come from?" and "I can't believe I have so much grey hair!!"  Let's face it.  I have a one year old. She's busy. I'm busy. I don't have time to pluck my eyebrows and wax my upper lip. I barely have time to shave my armpits! Since Miya has been born, Chris and I have gone out by ourselves three times. Three.  I think.  It might only be twice.  So in the last year, I have paid attention to my reflection only as long as it took to put on enough makeup to render myself  "presentable". 

When did 32 happen? When did I get to be (nearly) 32 years old? When did I stop being a size zero and where did these hips come from? In my head, I don't look like that!!

In my head, I look like the image you see above this sentence.

What prompted me to actually look in the mirror (longer than what it takes to brush my teeth) was this picture:

This picture was taken a couple of weeks ago on one of our epic trips to London. What happened under my eye?? I had NO IDEA those wrinkles existed!!

The point is this:  Actually, I'm not entirely sure there is a point to this.  I'm just venting.  I am aware that people age. I wasn't entirely aware that I would age. (Ha Ha. A little humour there...Of course I knew I would age...I was just sure it wouldn't happen until at least 50...or 60.)

I don't recall aging this quickly pre-Miya.  Do children speed up the aging process? It is proportionate to how many children you have? Has anyone else noticed this? 

 Until  I am forced to look at myself in the mirror for many hours at a time, I am going to continue to think of myself  like this:

And less like this:

See my futile attempt to accessorize my "outfit" with a cheap necklace? Is that what I have been reduced to? (I use the term "outfit" loosely.  The image speaks for itself. This is not an "outfit". This is merely hiding one's nakedness with age appropriate clothes of the correct size.)

Please don't think I want to be someone else. I don't live in the past. I don't want to BE my old self. I am just wondering what happened to her.  She used to care. She knew how to put outfits together.  She knew what an outfit was!!

I suppose this is not the time to be thinking of such things. After all, I am going to be living in a hospital for weeks and weeks. I just don't want to lose what little of myself I have left. I don't want the hospital to suck the last bit of fashion sense I have left right out of me. (By "fashion sense" I mean I still have the ability to know which colours go together and which don't.) Is it wrong to want five minutes to myself to apply my newly purchased Olay day and night cream and eye gel?

I must urge you to remember, diligent reader and follower of my blog, that I DO NOT want to be someone else or look like someone else. I want to look like me. But with less wrinkles. And more hair that isn't grey. After all, I'd rather you hate me for who I am than love me for who I'm not.