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. 

And They Were Turned Away, For There Was No Room At The Inn

Firstly, I should tell you that Miya is not having a gastrostemy.  There was no availability until just before the scheduled admission date, and the BMT team don't want to take the risk of delaying the transplant just because of the procedure.  Miya will have an NG tube instead. 

Now to the main point of today's post...

Here we are, 10 days from scheduled admission and we still have no accommodation for the second parent.  It was Chris' and my plan to share care of our daughter and relieve the other parent when necessary. We were going to share night duties, with me being on for two nights, and him being on for two nights, etc.  WRONG!!

GOSH have NOT been helpful in the accommodation department. They have advised that unless the child is in ICU, they are not obligated to provide accommodation for the second parent. We even have a letter from the CNS on the BMT ward stating that due to personnel cuts in their department, the second parent is needed to assist in caring for the child.

I have made a complaint about the Family Accommodation Office at GOSH, and I don't think anything was ever done about it. (The Family Accommodation Office has been rude to both Chris and myself on several occasions.) The liaison office (where I made the complaint) were most unhelpful as well.  When I asked about accommodation, I was handed a sheet of paper with local area hotels and rates printed on it (ranging from £87.00-£123.00 PER NIGHT!!!!) and was told, in the politest of ways, to PFO (Please F**k Off).  Who has £4000-£5000 to spend on a hotel room?!?! Not I ! 

We have applied for funds through a services charity called SSAFA but nothing as come of it yet. I think it's pretty late trying to make hotel reservations at this late date, anyway! Good luck finding a hotel in central London that will be able to provide accommodation for at least 6-8 weeks!! 

Next Sunday, it looks as if we are headed to London on a hope and a prayer.  One of us may be sitting on a London street corner!!  Maybe we should bring a sleeping bag and our dog...also a huge cardboard sign that says "Will Perform Like A Circus Monkey For Accommodation".  I feel like a circus monkey...all this running around and trying to get everything organized and get everyone packed to go. Could someone please invent a cloning machine so I can get more done? Why is nothing more being done to help us? Surely not all families who have two parents staying with their child can afford the astronomical cost of a downtown London hotel, plus the good fortune to actually be able to obtain a continuous 6-8 week reservation.

Tomorrow, I am having Chris phone a charity we've been told about that provide housing for families who are being treated at GOSH.  I don't have much faith in being able to secure anything, but here's to hoping! Maybe Chris will have better luck. People don't seem to respond to me. Maybe it's because I'm foreign...

MPS: It Sounds Bad Because It Is Bad

This morning, when we arrived home from GOSH in the wee hours, I was irritated.  I was irritated when I woke up this morning. Before I tell you why, I'll give you an update on how Miya did yesterday with general anesthesia and her CT scan.

Miya is a Super Star!! I don't know how she does it, but she manages to deal with everything that's thrown at her with a smile on her face.  That poor child hadn't had anything to eat for NINE HOURS when it finally came for her to be anesthetized. She hadn't had a nap (except for a brief hour or so on the way to the hospital) and she didn't cry. She didn't complain. She happily played in the ward's play area, or in her bed.

Chris held her on his lap when it came for her to be gassed, and I knelt in front of her. She was crying and I got weak in the joints and felt a little sick. I didn't know if I was going to have to run from the room or not. Poor Miya was wiggling and crying and then went limp. I feel as though I can't describe it in words. We said our goodbyes as she laid motionless on the stretcher which broke Chris' and my heart. How do you leave your child so helpless and vulnerable in another's care?

We went to the hospital restaurant to grab a drink and something to eat and then headed back to the CT scan area and waited for her to wake up. We hadn't been there long when we could hear Miya's dramatic cries coming from the recovery room. The anaesthetist (or anesthesiologist, as they are called in North America) told us that Miya did really well and she had a good airway, considering she has Hurler's.  Great news!! 

 Sitting On Daddy's Knee After Coming Up To The Ward From Recovery. Still A Bit Grumpy.

Poor Miya drank 14oz of milk and ate a banana and some Cheerios when we got back to the ward. She played with her stuffed toys for a while before settling down for a nap.  The pre-meds for the ERT came around 1800hrs and the infusion was hooked up close to 1900hrs.  She slept through most of the infusion, but woke up for the flush portion of it. (When the bag is finished, there's about 15-20ml left in the line. The nurses flush this through with saline solution. Therefore called "the flush".) 

ERT No.18 (There's a sock on her left hand so she won't pick at the canula)

We don't know the results of the CT scan, but I'm sure we'll hear something today if there's somthing wrong.  I am also going to phone the BMT ward to tell them that the anesthetic procedure went well and to schedule the gastrostemy if that's what Miya's medical team feel is best.  


Now comes the part I'm a little peeved about. This happens all the time (unfortunately) and I had a really difficult time dealing with it. 


Parents in wards tend to congregate.  They ask what each other's child is in the hospital for and listen politely while the other parent explains. At least, I listen politely. Two mums last night did not. First off, when these two mums asked me what Miya was in for, and I tried to answer, they kept cutting me off with information about their children.  Fine. If you want to talk about your kids, I'm down with that. Just wait until I've answered your question about my kid!! If you didn't want to know, why did you ask?? Secondly, and the reason why I'm so peeved,  I HATE HATE HATE trying to explain  MPS to people who have never heard of it before.  They usually don't understand how bad it is ("What's the big deal,? It's just a missing enzyme. What harm can that do?") and when I try to explain the severity of it, people think I'm trying to make my kid seem sicker than everyone else's kid!! Kind of a munchausen by proxy situation, if you will.  


MPS is bad, right? I don't mean to sound like Miya's situation is worse than everyone else's but sometimes, it is. Sometime's, other kids are worse off. When people ask me about MPS and Hurler's, I tell them what it is. I don't sugar-coat it. This whole situation makes me wonder what I could do to bring more awareness to the disease. 


That is all.

On The Eve Of General Anesthesia...Some Technical Stuff & Some Pet Peeves

Tomorrow is an important day. And a scary day. Tomorrow is Miya's first general anesthesic as a diagnosed Hurler's Syndrome patient.  Tomorrow, Miya will be put under (briefly) for a CT scan. Miya has been under general anesthetic before, with no complications, when she was six months old. (If you've been keeping up, you'll know that Miya was born with talipes and had a tenotomy to loosen the tendon in the back of her right foot...Remember? The surgeon is the one who thought she might have a chromosomal disorder....No? Alright, well now you're up to date.)

Hurler's patients are notoriously difficult to intubate. I have just read an article (http://www.anestesiarianimazione.com/2010/Hurler%20Syndrome.pdf) which indicates that "in Hurler’s syndrome airway problem has been described as the worst in pediatric anesthesia". Not exactly confidence-inspiring. "What makes Hurler's patients so difficult to put under?"  you might ask. I shall tell you...

The most difficult problem is "maintaing the ariway because of the anatomical changes in upper airway due to deposition of mucopolysaccharides (GAGs) in tongue, tonsils, adenoid, epiglottis, glottis and trachea".  Chest deformity can also cause issues. (Miya's ribs are flared on her left side. Hurler's kids tend to have oar shaped ribs. I don't know if this affects her airway or not. Nobody seems to be concerned about it, so I'm not either.)

How Miya reacts to this anesthetic will determine whether or not she has a gastrostemy or not. The preferred gastrostemy for the Bone Marrow Transplant team is called a PEG (Percutaneous Endoscopic Gastrostemy). Nikki, the Clinical Nurse Specialist for the BMT ward at GOSH says that with a PEG in, children are better nourished (as they don't have to fiddle around with NG Tubes) and tend to go home earlier that children who don't have them. I'm all for going home early.

On a completely unrelated note, in regards to our hospital stay, I have also advised Chris that I refuse to be sans internet so I have persuaded him to get a dongle (wireless broadband). We were without the internet for a little over a month at the last place we lived, and I almost died. I don't mean that figuratively. That is a completely literal statement.

As a parent, I am terrified of the anesthetic risk and I am on the fence on the PEG issue. I agree that it would probably be best for her to have one however, it would be in her best interest not to go under general anesthetic more times than necessary.

This is Miya in her Ponseti cast, after her tenotomy. She wore this particular cast for 5 weeks. After five weeks, she began to wear Markell boots and bar for 23 hours a day, 7 days a week. She currently wears them at night. This will continue until she's 3 or 4 years old.





And because this is my blog (and I can start a sentence with "and" if I want to), I am going to go off on a tangent  and write about some things that bother me..call them Pet Peeves, if you will..I'm sure I do.

(Hurler's) Pet Peeves and (Non-Hurler's) Pet Peeves

• Bad Grammar. There is no excuse for it. And bad punctuation. Sure, we all make mistakes. I'm sure this blog is full of them (I took the liberty to mention that I began a sentence with "and"...my bad) but we all took basic English in school. Were some of us not paying attention?

• "Alot" is not a word. A lot is two words. Allotment is a word, but it has a completely different meaning than the aforementioned two words.

• I hate when the neighbours leave their washing on the line overnight...or for two nights in a row. Or three.

• The term dysmorphic when talking about facial features of a Hurler's child. We're all aware of it, it's a part of the illness. I don't talk about it. Or think about it. It's a non-issue. A moot point. It's not a nice word and I don't like it. 

•  When people use the apostrophe to pluralise (pluralize, for my North American readers). The apostrophe shows ownership or to stand in for a missing letter (called a contraction, as in the case of "don't" or "can't"). Please don't misuse the apostrophe. I find it very confusing. (I know that this falls under the grammar and punctuation pet peeve, but I find the misuse of the apostrophe quite irritating.) Interested in using the apostrophe correctly? Click here.

• Another grammatical issue I have is the use of "their", "there", and "they're". They are three seperate words and not interchangable. 

 That is all I can think of for now. I'm sure there are more and I'm sure you will hear about them in future posts.

***In this blog, I won't always talk about Miya. I think you might gain some insight into me and my family if I write a little about myself, and us. Hurler's isn't just my child's disease, it has become our disease. We fight it together. Every. Day. Of. Our. Lives.***

I Love A Good Party...

Miya's first birthday party today: Great Success!!


Although she had no idea what was going on, she LOVED her cupcakes (she only got one...the frosting was SO sweet) and enjoyed looking at and holding the wrapped presents. Nan also made a visit from Canada via Skype!! 

Miya got some books, a feeding set (bowl, plate, etc) two stuffed Simba toys, some activity balls, an Early Learning Centre gift card, and a pink Mini Cooper (operated by a 6v battery!!). She liked sitting in the car, but won't be using it with the battery charged any time soon.

Miya received some birthday cards from the neighbours, and some boys from next door stood in a row in next door's garden and said "Happy Birthday, Miya!!" in unison.

We are off to GOSH on Monday for a CT scan and won't be home until Tuesday. She will be having the CT scan unger general anesthetic, which is always a risk for MPS kids. How she tolerates this will determine whether or not she will have a gastrostemy.

For more birthday pics, click here

When One Tells A Story, One Starts From The Beginning...

On the 18^th of November, 2009, I shouted from the bathroom  to my husband Chris who was still in bed “I'm pregnant...we're going to have a baby!!”. And that's how our story begins.

It was a nervous pregnancy, until week 17. At week 17, we had a Fetal ECHO done at Guys and St. Thomas Hospital in London to determine whether or not our baby's heart was okay. In May of 2000, I lost a baby girl to hypo-plastic left heart syndrome (unrelated to MPS) and there was a small risk of it happening again. Relieved, we left the hospital knowing our baby's heart was okay...we thought we were out of the woods...

At our 20 week scan, we discovered our baby was a girl!! After being convinced we were having a boy, we couldn't believe it when we were told they could see “girl parts”. We thought they were liars. We had both secretly wanted a girl, but when one of us asked the other if we had a preference for a boy or a girl, we each gave the politically correct answer “It doesn't matter as long as it's healthy”. Which of course, was true. We just both really wanted a little girl.

I waddled and complained and had nausea and generally had a typical pregnancy. Nothing suggested anything was wrong with our baby.

On Wednesday, the 7^th of July, 2010, I went into labour. Big time. It came all at once and there was no break in contractions. I asked the midwife to let me die. Miya Aeris (pronounced Mia Iris) was born on 2350hrs on the 7^th of July, 2010. Her name means “Increasingly Beautiful Rainbow” in Japanese, which is why we chose the Japanese spelling rather than the traditional spelling.

Miya was born with talipes in her right foot, and positional talipes in her left foot. We were told her left foot would straighten on her own but the right would need correction. Miya didn't feed well and we stayed an extra day in the hospital because of it. She didn't seem to want to suck and couldn't get her tongue in the right position for it. She was very floppy, and when I picked her up under her arms I was afraid her shoulders would come out of joint. Her face seemed very squished and her neck was very short. Her eyebrows were bushy and her eyes seemed puffy. Her breathing was noisy and Chris and I quickly stopped “rooming in” with her. We had to wear earplugs! I thought something was wrong, but I rationalized...after all, what kind of mother would I be if I took her to the doctor and said: “She doesn't look right. She's noisy when she breathes. She won't stop crying.”

For two weeks, Miya slept and ate (although it was a pain in the butt to try to feed her). At three weeks old, Miya woke up. She screamed and cried and developed silent reflux. She was put on Infant Gaviscon which made her constipated. She couldn't be in light of any kind. I was depressed. Chris and I took shifts to look after her...I'd take one night and he'd take the next. We were at our wit's end. We bought every gadget and gizmo to try to help her stop crying. Finally, we took her to a cranial osteopath who really seemed to help. Miya stopped crying so much!

In January 2011, Miya began the Ponseti Method of correction on her right foot. She was in a cast from her toes to her thigh for a week, then she had an operation called a tenotomy, to relase the tendon at the back of her ankle. It was the surgeon who asked me if anyone had ever approached me about the possibility that Miya may have a chomosomal disorder. I was shocked and a bit relieved. I wasn't crazy!! Someone else saw that something wasn't right.

On the 28^th of February 2011, Miya was diagnosed with MPS 1H. (I had Googled her symptoms and came up with MPS...eventually....it was a small link on a page I was viewing about conditions that feature a large liver). I lost the ability to speak and breathe and stand upright. My knees buckled and a fought for breath. For a week, I couldn't look at my daughter. I was certain she was going to die.

That day seems like eons ago. Miya currently receives Aldurazyme infustions weekly to give her the enzyme her body lacks. The enzyme can't cross the blood-brain barrier so although it helps her body break down mucopolysaccharides in her lower body, they are still building up in her brain causing neurological and cognitive defect. She will be having a bone marrow transplant in about 2 weeks. In theory, the bone marrow transplant will enable Miya to produce her own enzyme, and have it work to break down mucopolysaccharides throughout her body, including her brain. She will still have problems with her skeleton, vision, and hearing. With the bone marrow transplant, we hope she will have "normal to near normal" intelligence. 

Miya amazes me every day. She's smiley and charismatic. She's everything I admire and love. She's my increasingly beautiful rainbow.