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.
Sunday, 31 July 2011
Wednesday, 27 July 2011
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!!
Saturday, 23 July 2011
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).
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).
Friday, 22 July 2011
`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?
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:
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?
Tuesday, 19 July 2011
"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.
* * * * * * * * * *
"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!!
What prompted me to actually look in the mirror (longer than what it takes to brush my teeth) was this picture:
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:
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.
Monday, 18 July 2011
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.
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.
Thursday, 14 July 2011
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...
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...
Tuesday, 12 July 2011
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!!
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".)
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.
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.
Sunday, 10 July 2011
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.
***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.***
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.
***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.***
Saturday, 9 July 2011
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
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.
Thursday, 7 July 2011
When One Tells A Story, One Starts From The Beginning...
On the 18th 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 7th 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 7th 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 28th 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.
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