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.