How would you feel if your worst fears are confirmed?

Whenever we go to Stanford for a medical appointment, we stop at the Disney Store so Queen Teen can indulge her obsession with all things Disney. Ice cream isn't enough any more, instead Queen Teen gets to buy a new doll or shirt or trinket, usually related to a Princess.

My Disney Store is called Godiva... as in Godiva chocolate. There is a Godiva store at the Stanford Mall so my treat after the long drive and worry is to blow thirty bucks on a box of delectable truffles. I would spend so much because we used to only go to Stanford three times a year. Now we'll be going more often, so I'd better cut back on my Godiva splurge before I gain 40 pounds and max out my credit card.

However, we won't be cutting back on Disney.

Queen Teen had a genetics appointment last week with Dr Enns, the leading expert on Mitochondrial Disease in the U.S. We've been working with him for 5 years and he's put her through every test he can think of to confirm whether or not she has Mito. All of the tests come back negative. The mitochondrial sequencing test showed an anomaly in 100% of her mitochondria, but this is more likely a familial trait, not the cause of her illness. This time, I was the one who had to get blood work (Queen Teen loved that) so they could determine if I too have the anomaly. This would confirm that it's just the way our mitochondria are made.

Dr. Enns was alarmed at how rapidly Queen Teen has lost ground, and it felt reassuring that the doctor is just as worried as we, her parents. Reassuring isn't the right word. You know that feeling when you go to the doctor convinced that something is terribly wrong with your child, only to have a condescending physician pat you on the head and tell you not to worry? It's infuriating. Then you go to a different doctor and that doctor takes your fears seriously and immediately gets to work trying to help your child. You feel calmer and reassured that the doctor cares about your child.

But how should I feel when the doctor confirms my worst fears about my daughter, tells me to trust my intuition without actually giving a prognosis, and talks about aggressive tests to find the right treatment to improve her quality of life? We're not discussing cures or longevity, we're discussing symptom care and slowing down progression.

Dr. Enns and her new neurologist want a muscle and skin biopsy, and we agree, even though this means anesthesia and minor surgery. While she's out, they will draw more blood and try to squeeze in an MRI. He has recommended a newer Mito-Cocktail and is recommending that she be part of a medication research trial.

I can't believe I'm going to put my kid on experimental medication.

It will take some time to get the appointments organized (she also needs to see a cardiologist and respiratory specialist, as well as the surgeon and several doctors on the medication committee) and I'm so thankful that Dr. Enns' assistant is dealing with that. Hopefully, we'll get this done before school starts August 20.

Queen Teen will be getting a lot of Disney swag this summer, and I'll probably be eating way too much chocolate. 

Second Easter in Hospital

Queen Teen has had two Easter baskets from Lucille Packard Children's Hospital, which is two baskets too many. The first Easter we spent there was after her feet surgery when she was 11. The second was this past Easter.

We finally heard from the neurologist who wanted her admitted to the hospital immediately for tests, so we dashed down to Stanford and spent three days there. Queen Teen was low on glucose and electrolytes, but a couple days of IV fluids brought the color back to her cheeks. Our new neurologist is excellent and I have a lot of faith that he'll be able to help us sort out the mystery of Queen Teen's disorder.

Disorder. Syndrome. Why can't I call it a disease?

We're home now, waiting for the results of all those tests, including an EEG to check for possible seizures. Her strength is poor and stamina worse, but she's slowly feeling better (I can tell she's feeling better because she's getting bossy again). Eating is still tricky and some days she has trouble swallowing juice. Other days she can eat Mac and Cheese with gusto. Just like everything about her neurology, her symptoms come and go. She's still not strong enough to go to school a full day, but maybe in another few weeks? Time will tell.

That's always the answer: time will tell. What will happen next? Time will tell. What does she have? Time will tell. What can we expect? Time will tell...

I found this great site that explains what Queen Teen has: What is Mitochondrial Disease? The United Mitochondrial Disease Foundations is an advocacy and resource organization which also raises funds for research. With a long list of possible Mito diseases, why is Queen Teen such a mystery? Why can't we discover her disease's label?

And why do I need to know so badly now?

I could lose my daughter; I know that now. Am I strong enough to go through this with her, to be there for her no matter what may come?

The possible syndrome roller-coaster.

Well, here it is: the possible syndrome roller-coaster. It's the exciting, heart-pounding, emotional ride in which you get to worry incessantly about your kid while doctors hunt for answers, and then after a fever pitch of panic, you get the 1000 foot drop straight down when all the tests come back "negative."

What's causing your child's illness? It could be this or that or this other really awful thing. But don't worry. We'll run all these tests which will give us answers. Just be patient.


Huh, that's odd. All the tests came back negative. Gee, I really thought it was going to be this really awful thing. She has all the signs, but the tests are negative. Hmmm... you know... it could be this other really awful thing. We should run some tests. 

We've been riding this coaster for fifteen years. We find some balance for a while, until either Queen Teen shows some new sign of a degenerative disorder, or a doctor says she "might" have this thing, and there's a new test he'd like to run. And so we climb back onto the roller-coaster, strap in, and hope for answers.

We still don't have any answers. After years of hunting, we had made peace with that. Not knowing meant the future was still wide open. She could plateau, learn skills, gain strength, and eventually have a life of her own. Maybe she could live with a friend in an apartment with support and find a job she enjoys. She could fall in love. Not knowing what would happen meant we could pretend that everything would be fine for a while longer.

But now, we need to know.

Three weeks ago Queen Teen started gagging on food. It got so bad she couldn't eat anything solid or textured. She's been living on Ensure and yogurt. I took her to Stanford for an emergency gastroenterology appointment where they ran a bunch of tests. The good news is they didn't find anything structurally wrong, which means she's safe swallowing and won't aspirate on her food. The bad news is it means it's probably neurologic. There isn't a cure for that, or an easy answer. So now we're waiting for an MRI and neurology appointment, hopefully this week. In the mean time, we wait. And worry.

We knew her condition was degenerative, but without a real diagnosis it's impossible to predict what degenerative would look like. I figured it meant she would eventually be unable to walk or her hand tremors would get worse. We could manage those. But not be able to eat? How do you deal with that?

It's hard not to think the worst. This is my child, and I feel fucking helpless just watching her suffer while waiting for the phone to ring. No matter how busy I try to stay, or how much I practice mindfulness and "stay in the moment," the fear crawls up my spine like a hungry tarantula. This roller-coaster is a bitch.

I don't know what will happen now. Maybe her eating problem will disappear on its own. She could wake up tomorrow hungry and eat a scrambled eggs without any problem. The MRI might not show anything at all. This could be just a weird thing that will go away in a few weeks. It's happened before; her hand tremors were terrible for a while and just as suddenly, the tremors improved. Why? They still don't know.

But whatever happens next, this is a reminder of just how fragile Queen Teen really is.

 Strap yourselves in kiddies, it could be a long, bumpy ride. 

The Doctor wants to control my entire life!

I'm taking Queen Teen to see a therapist in Mill Valley tomorrow. This person is supposed to help us manage Queen Teen's anxiety issues, especially around doctor's appointments. But right now, this appointment is creating anxiety for her, not helping.

When I told her about the upcoming appointment two days ago, she yelled that she wasn't going. Luckily I told her in the morning right before the school bus came so I didn't have to listen to the yelling all day. When she got home, she seemed to have forgotten about the appointment. But today when I picked her up from school, she was really quiet. When we got home she told me she didn't want to go to the doctor's.

"I'm tired of going to doctor's. Why do they have to be so far away?"

"I'm tired of them too, Honey. I wish they were closer."

"Well I don't want to! I hate doctors!" Then she started crying. It got even better from there.

Her councilor came to the house for his usual appointment and the two of them talked in her room for a while. She told him how angry she is about having to go, how much she hates doctors, how they are boring, how the car ride is too long... and on and on and on. She started to cry and he told her everything would be okay. When it was time for him to go, she went into the hallway and hit her calendar with two fists. "I'm not going and you can't make me!"

Oh this is fun.

Sometimes I really hate being the mom. I hate having to drag my furious daughter to doctor's appointments, hate having to hold her down when they need to do blood work, hate bribing her to get in the car. I hate the long drives, the long hours, the endless paperwork. I hate my daughter's screams of rage and then the tears when she realizes there's nothing she can do to stop it.

At dinner she looked directly at me and said, "The doctor wants to control my entire life!"

That is probably exactly how it feels to her. She has very little say about what happens to her. All she can do is fight for the meager control she has.

Where is the balance in all of this? How do I help her stop feeling so helpless, while also providing the care she desperately needs? How can I help her understand doctors are trying to help, not torture her?

Maybe the councilor and the therapist tomorrow will help me find some answers. But for now, this really sucks.

A great big, epic gravity check.

Tuesday morning at 7:45 the phone rang. "Hi, this is Queen Teen's teacher. I wanted to let you know that she fell. She seems okay, just embarrassed. We didn't see it happen so I'm not sure how she fell, but the other students told us and when we got to her she was sitting on the floor. It looks like the brakes on her walker stopped working. Can you fix them?"

"She's okay?" I asked.

Her teacher said she looked fine, just shaken up, so I told her I would be there with tools to fix the walker after my hair appointment.

But when I got to school an hour later, Queen Teen was definitely NOT okay. She was sitting at her desk with an ice pack on her right hand and the school nurse by her side. QT's hand was swollen and black and blue. Did she break it? I felt like the worst mom on the planet because I'd chosen to get my hair done rather then rush to my daughter's aid. But they told me she was okay!

I took her to the ER where we sat for three hours, got some x-rays, and were told it was a bad sprain. They wrapped it up and told me to take her to her primary doctor the following day. Really, a bad sprain? Should it be so swollen if it's just a bad sprain?

Her primary doctor wasn't so sure. "Queen Teen may have broken a tiny bone in her wrist. It's hard to tell right now, but this area is very tender." She explained that there's a small bone in the wrist that is prone to breaking when people fall on their hand, which is probably what Queen Teen did. It can be hard to see at first in an x-ray unless you're really looking for it. She told us to get another x-ray next week and come back to see her after Thanksgiving. Then she put a wrist splint on Queen Teen's hand and told her to take it easy.

Take it easy? Easier said than done. This morning at 5 AM I was woken by my daughter yelling, "WHOA!" Leaping out of bed I dashed to her room and found her trying to walk across her room without her walker, in the dark, with only one hand to catch herself. She can't use her walker because she can't put any weight on her wrist, so Queen Teen figures she'll just walk on her own anyway.

Isn't this the same girl who hurt her hand and wrist because she was trying to get her backpack off her wheelchair on her own, but when it got stuck she jerked it and then lost her balance (she finally admitted that was how she fell)? Isn't she the same girl who spent THREE HOURS in the ER yesterday?

Yes, this is the same girl. My stubborn, tenacious, independent daughter who seems to think the laws of gravity do not apply to her.

This is why I have gray hair, bags under my eyes, permanent knots in my shoulders and pain in my stomach.

Maybe it was all those times she fell learning to walk and we'd just call it a "gravity check," which made her laugh and get back up again. When she was three she weighed 25 pounds and the ground was only three feet away from her head. At sixteen she weighs 95 pounds and the ground is five feet from her head. Gravity hurts a lot more now.

Thank goodness for "Sponge Bob" and Starbucks Vanilla Cream Frappuccinos; we might survive the next few days.

Playing chess with the audiologist

After the Berkeley Low Vision Clinic appointment, we spent several days with the Denizons of Chaos (I'll write more about that next time), then drove to Palo Alto for an audiology appointment. Queen Teen was resigned to it, and happily distracted by three energetic girls, one loud boy and a giant house to explore. Once we reached our hotel room in Mountain View, her mood quickly changed. The sparkle in her eyes vanished, replaced by a nervous scowl. The hotel was nice (thank you Hotels.com) and we had cable TV, something we don't get at home, but nothing could negate the misery she felt thinking about the next day: audiology.

Of all the appointments she has, from neurology to dentistry to genetics to orthopedics, audiology is the worst. She likes the doctor fine, but the tedium of the tests and the reality that she does indeed need hearing aids ("No I don't," she insists, even after the hearing exam clearly shows she can't hear a blessed thing.) depresses her more than her best friend moving away.

She did her best during the test, and I told her how proud I was of her. Dutifully she put the block in the box when she heard the tone, but she also put a block in after guessing the interval between testing tones. The doctor mixed up the timing to avoid that, so it was obvious Queen Teen barely heard anything. And during the vocabulary test she got frustrated when she couldn't identify what any of the words were. I made sure she could see the pictures, and she identified them by looking, but when asked to point to the "baseball" or "ice cream", she just looked at me and said, "I can't find it."

Queen Teen is a candidate for Cochlear Implants, but there's a lot to think about before we go that route, primarily, Queen Teen's mental health. She refuses to wear hearing aids and denies that she can't hear. She cries at appointments and has a panic attack before we leave the car. If I can't get her to a dentist to have her teeth cleaned without her freaking out, how the hell will she tolerate major surgery and implants? We're concerned that she's losing language processing ability because that part of her brain is no longer being activated; hearing aids can help with that.

Before we left, the doctor took an impression of Queen Teen's ears for new ear molds to go with her new hearing aids. That's when Queen Teen started to cry, and she even tried to hit the doctor. I soothed her the best I could, then the doctor quickly made the impressions and we were out of there. We had planned to stop and visit another friend, but we were both worn out so we decided to get a head start before rush hour traffic began. That's the only plus to Queen Teen's hearing decreasing so much: the hearing tests take a lot less time.

Optic-Nerve Hypoplasia

I've spent many months studying eye diseases and conditions for my exams, things like Cortical Vision Impairment, Retinopathy of Prematurity, Albinism and Optic Nerve Hypoplasia. And one day while studying all that information, I suddenly realized that Optic Nerve Hypoplasia had fallen off Queen Teen's list of diagnoses. I have no idea how or when that happened.

She was diagnosed with Optic Nerve Hypoplasia when she was 2 years old. Luckily, she didn't appear to have Septo-Optic Dysplasia, which meant she didn't have the syndrome with it's hormone and brain abnormalities, or so we were told.  She "just" had the vision impairment: 20/1500 vision, poor depth perception, nystagmus and intense light sensitivity. Plus, she had physical disabilities such as hypotonia and ataxia and later a hearing impairment. After hundreds of tests and consultations for the last 14 years, including genetic testing, the reason why she has those physical disabilities is still a mystery. No one can  figure out the underlying cause of her shrinking cerebellum. We all finally reached the point where we ran out of tests and theories and decided to just give it time. She's happy, appears stable, and is thriving in school. Let's just stop all the testing and let her be a kid for a while.

And then came the day when I was studying for my Certification exam and read newer information on Optic Nerve Hypoplasia. According to the research, a child can still have the hormone problems and developmental issues, even if not diagnosed with Septo-Optic Dysplasia. And if the child shows no hormone deficiencies as a child, they can develop later in life, especially around adolescence. Holy shit, that's not what we were told 14 years ago!

Then I went on to read some of the common behaviors and symptoms:

• trouble with body temperature regulation
• hormone deficiencies
• growth problems
• sleep problems
• hypoglycemia
• developmental delay
• trouble with eating, including appetite
• impulsivity; easily frustrated; highly distractible
• high fevers when ill; fevers when stressed
• cerebral atrophy

There are even more symptoms which all depend on what type of hormone and brain dysfunction the child has, and Queen Teen has about 80% of them. 

Later that day, I looked at her medical reports from the last few years and saw that there is no mention of Optic Nerve Hypoplasia on any of them.

What?!!!!!!!!!

Even her official diagnosis from CCS doesn't mention Optic Nerve Hypoplasia any more. When the hell did that change?

If the diagnosis of Optic Nerve Hypoplasia is missing, does this mean we've all been looking in the wrong place to uncover the mystery of what has caused Queen Teen's disabilities, as if we started a treasure hunt on the wrong desert island? Or was the mystery uncovered several years ago in all those tests, found to be inconsequential, then buried again and forgotten? But if Queen Teen does have hormone problems now that she's older, especially with her pituitary gland, how much damage could have been done which might have been avoided?

How did I miss this? How could I have let things get so disorganized that I allowed something this big to slip through the cracks? Yeah, I know, I've been busy, but this is my child! I must stay on top of things, especially medical care. And don't tell me it's the doctors job to keep up with her medical care, because we all know how often doctor's miss things (and not always through a fault of their own. They have a lot of patients with a lot of different medical reports to sift through). No, it is my job. 

But I can't think that way. Blame is pointless. I'm doing the best I can. At least I found the problem now and I can do something about it. I have to figure out when and how Optic Nerve Hypoplasia was excluded from her medical diagnosis.  It's not like the optic nerve damage just went away. 

I contacted her geneticist and he seemed surprised that she had Optic Nerve Hypoplasia; they never got a report from the Ophthalmologist (I know I told him!). He also agreed that she should be seen by an endocrinologist and he made a referral to one at Stanford. I should hear back from them in the next week or so. Now I'm going through her medical files again looking for mention of Optic Nerve Hypoplasia and will send copies to her geneticist, neurologist, and another for the endocrinologist. And I need to figure out the last time she was seen at the Low Vision Clinic in Berkeley and possibly make a follow up appointment. Or should I take her to an ophthalmologist at Stanford? 

One step at a time. We'll get to the bottom of this. 

School was the biggest reason I lost track of her medical care, but it may also be the biggest reason I'm able to investigate what kind of care she needs. Now that's ironic. 

A Phone Call From Audiology

Two days ago, we got a phone call from our audiologist at Stanford. She hadn't talked to us in a while and wanted to know how things were going with Queen Teen. I gave her the run-down: Queen Teen refused to wear her hearing aids; when she did wear them the inside of her ears turned pink and she complained they bothered her; on those rare occasions she did manage to wear them, they didn't seem to help her hear any better.

"Do you think her hearing has changed?" Dr. Audiologist asked.

"It's worse. She can't hear conversations at all, and even with her movie turned up full power, I don't think she's hearing it very well."

"I'd really like to see her. She should come back for a hearing test again, and I'd like to look at her hearing aids. They probably aren't the right kind for her and I'd like to try a different type. Plus, we should get her ear molds made with a different type of material."

"I thought we were done because she wouldn't wear the aids."

"No, not at all! We can't give up on her hearing. There might still be something that can help her."

I wanted to crawl through the phone and hug her. Just to hear those wonderful words: there might be something that can help her. Lately, we've been hearing a lot of talk that sounds an awful lot like giving up. There are no more tests to run. No more treatments to try. No leg braces or therapies that will help her hypotonia. The doctors look at her and seem to say, sorry, there's nothing we can do. Go home and wait and see. Maybe in the future.


But here was a doctor who called because she wanted to see how Queen Teen was doing and when I told her that Queen Teen's hearing was worse she expressed that she wanted to keep on trying. We can't give up.

The doctor asked if we had considered cochlear implants and I said I wasn't opposed to it but would need more info to decide if it was something that could really benefit Queen Teen. She agreed and told me the process of evaluation Queen Teen would need to undergo before any of us could make that decision. But it was an option. She told me there was a newer type of hearing aid that was more powerful and that worked better with FM systems than the model we have now. And she said there are actually other types of materials to make ear molds. The receptionist at the clinic would call me the next day to make an appointment.

Which she did. I know Queen Teen won't be thrilled about going to the audiologist in April, but I am. And I know there's no guarantee even our wonderful Dr. Audiologist will be able to help Queen Teen hear, but I know this woman won't quit until she's exhausted every single option. That gives me hope, something we've been lacking around here lately.

Thank you for calling, Dr. Audiologist.

MRI and Test Results

I got a call from the genetics researcher at Stanford who has been working hard to unravel the mystery called Queen Teen. A few months ago Queen Teen had blood drawn while under anasthesia for an MRI. The researcher had uncovered two new tests that might pin-point the cause of Queen Teen's shrinking cerebellum, hearing loss, and visual impairment. The hypothesis is that she has a Mitochondrial Disorder and there is some evidence to support that, but so far no one has been able to diagnose a specific disease.

They couldn't do it this time either. As usual, the tests came back "normal."

The researcher sounded apologetic about the results, but I wasn't surprised.

"It's what I figured would happen," I said.

"She's still part of the research study, and they can examine hundreds of strands at a time instead of just a few," the researcher said, trying hard to give me hope. "If they find anything, I'll let you know."

"Thanks. I know this isn't your field, but is there any news about the MRI."

She said she thought that I would ask her that question, so she was prepared. The MRI report stated that there was no apparent degeneration as compared to her last MRI, which means it looks like the damage to her cerebellum has slowed down. Yes! When we go to the neuro-genetics clinic in November we'll be able to find out more specifics, but for now the outcome looks good. Why has the degeneration slowed or stopped? Is it the CoQ10 and other amino-acids? Maturity? Has the disease reached a plateau?

So many questions and probably never any real answers. It's okay. We're used to it. Sometimes I think it would be nice to have a clear diagnosis, one that would fill in the blanks and tell me what we're dealing with and what will happen. It might make it easier to prepare. But on the other hand, I'm glad that everything is still up in the air. We just take it a day at a time.

Resignation

Yesterday was our bi-yearly CCS appointment, where the doctors and specialists go over Queen Teen's case and decide what she needs, and doesn't need (in other words, what CCS will agree to pay for, and not). CCS is a phenomenal program and without it, Queen Teen wouldn't have such great medical care, but the clinics are a bit... long, or as Queen Teen likes to say, "Booooorrrrrrriiiiinnnnnnng." The last few appointments have been focused on QT's knees and how much she needs braces on her legs to support them, or she "won't be walking in a few years." The doctor and specialists push leg braces, and Rick and I try to explain that sticking her back in full, hip to ankle braces will destroy her mental health.

It's a delicate balance: how do we protect her emotional well-being while still supporting her physical needs?

After the last clinic, we decided to try leg braces if they would indeed protect her knees and keep her walking longer. But we only made it as far as the leg casting. Queen Teen almost kicked the guy making the mold in the head before she started crying and screaming. If she couldn't tolerate getting a cast made of her leg, there was no way she would put up with wearing leg braces. So then we got a second opinion from Dr. Rinsky at Stanford who told us putting leg braces on an ataxic child with a progressive disease is counter productive. Even with braces she probably won't be walking on her own in a few years. That ended the leg brace debate for us.

Rick and I prepared ourselves for our bi-annual lecture as we walked into the CCS clinic with Queen Teen, and right away the doctor asked if she was wearing leg braces. 

"No. And here's why..." I explained what had happened during the fitting and the man who had been making the mold, who was at the CCS clinic with us, told the doctor how QT had reacted. Then Rick and I told the doctor about QT's worsening ataxia and our conversation with Dr. Rinsky.

I said, "You can buy the braces if you want, but she won't wear them. We'll have to tie her down every single day to forcibly strap them on. I can't do that to her."

The doctor actually agreed. Everyone in the room, the doctor and the specialists and the CCS case manager nodded and smiled sympathetically, agreeing forcing Queen Teen to wear braces would be bad for her. What a relief. They were finally listening!

And then the doctor began talking about the future, how if her ataxia is worsening there are things we need to be prepared for, like conservatorship and group homes and power chairs and the fact she'll probably need us to care for her for the rest of her life. "I know this isn't something parents want to hear, but you need to think about it."

"We have been," I said. "We're already starting to plan."

Rick brought up toileting and maybe getting a bidet and the doctor agreed it could be helpful. Then the wheelchair guy checked out QT's chair and walker and ordered the repairs they both needed. QT smiled and laughed at the doctor, played with Rick and me, and only declared "I'm booooorrrrreeedddd" five times. Afterward, we went out to dinner to celebrate a successful clinic. No fighting this time, no stress, no feeling like every single person in the room was thinking I was a lousy parent.

They'd given up.

It suddenly hit me that the reason they weren't fighting anymore was because they had all reached the understanding that there wasn't anything more they could do to help Queen Teen. Instead, they would provide what was needed to make life a little easier for her. No more braces or therapies or other types of treatments. There weren't any. Instead they were resigned to watching her lose ground more and more at every future CCS clinic, while making sure she had a comfy wheelchair and a bidet.

And I realized, I've given up too. I am resigned to this fate, and I hate it.

Perhaps someday I'll reach acceptance, rather than resignation.

Vaccinations, sunshine withdrawals, and Laurie Berkner

"Rain, rain, rain! Why is it always raining?" Queen Teen shouts. She has said this every morning and several times throughout the day for a week. I can't really blame her. I love the rain but even for me this weather is a bit excessive. 7.25" in only three days, and it's still pouring. Occasionally it hails and the wind blows so hard from the south there has been rain-water pushed under my front door, making the entry damp and slippery. Queen Teen is a sunshine girl;she needs sunlight to make her smile. When it's gray and cold for more than a couple of days her spirit matches the weather: she gets gloomy.

And then to make the week extra fun, she got vaccinated.

We were finally able to get Queen Teen the H1N1 shot. She's been on the waiting list at the pediatrician's office for two months, and although there have been open clinics via the health department in our area, there was no way Queen Teen was going to stand in line for two hours waiting for a shot. Going to the doctor to get one and waiting 35 minutes was bad enough. She screamed and cried and hit. Luckily her dad came with us to help hold her down while I shoved up her sleeve, held her arm out to the frightened nurse, and said, "Now!"

I hate doing this but it's the only way to get her vaccinated, or her blood drawn. But I know every time we do this it just adds to her trauma and PTSD. I'm surprised she doesn't break into hives just at the word "doctor." By the time she's grown-up and able to make her own medical decisions, she's going to need daily therapy just to walk into a doctor's office for a check-up. That's why I only force this on her when it's really, really, really, really, really, really, really important. The H1N1 virus made this year's flu shot extra important.

Queen Teen was so upset she didn't even want to go to Starbucks. She wanted home: now! Then she hid in her room and rubbed her arm where she was injected. I keep trying to tell her that if she relaxes it won't hurt so much, but in the throes of panic she can't hear me. I gave her tylenol and juice and left her alone to sulk.

After a few minutes she came to find me. I supposed I was forgiven. She came into my room where I was surfing the internet and I suddenly had a brilliant idea.

"Come here, doll." I pulled her toward me and sat her on my lap, then clicked on Laurie Berkner's website. Together, Queen Teen and I watched a few of Laurie's webcasts. It was like the sun had broken through the rain clouds. Queen Teen was laughing and singing along to the songs. She grinned and then hugged me tight, and then we sang, "The Goodnight Song."

"I'm a little frog and my daddy loves me.
I'm a little frog and my mommy loves me.
And when they tuck me in to sleep at night,
they say Ribbet Ribbet Ribbet, good night.

Goodnight. Goodnight. Goodnight little froggy goodnight..."

No matter how bad things might get in Queen Teen's world, Laurie Berkner always makes her feel better.

The Audiology Marathon

Queen Teen had an audiology appointment Wed. morning at Stanford. I decided to make it a fun trip and leave on Tuesday, giving us an extra day to play, plus a stay in a motel that night. On our way to Palo Alto that day, we stopped in Santa Rosa to have lunch with Rick, then we drove to San Francisco where we took a break at my school, San Francisco State University. I gave her a tour of the campus and showed her where my class is.

"Now you know where I am every Saturday," I said.

"Cool," she replied, looking around the classroom with a satisfied grin. "I think it's funny all of us are going to school."

But the highlight of the day was when I introduced her to one of my teachers. Queen Teen was shy at first, but warmed up quickly, especially when she discovered that my teacher ALSO wears hearing aids.

"See," my teacher said, pulling her hair back from her ears. "I have them too."

Queen Teen's eyes widened. "I didn't know other people had hearing aids!"

"I told you there were other people in the world," I said.

She looked at me and then pointed to herself. Her eyes sparkled and she sat up taller in her chair. "There are other people different like me!" Her declaration sounded like the words of a scientist who'd just discovered intelligent life on Mars.

My teacher and Queen Teen talked about sign language and hearing aids, and as I listened to Queen Teen's animated chatter I realized what a relief this discovery must be for her. Not only are there people "different" like her, but there are adults who have jobs and are capable. This woman has hearing aids, but she's my TEACHER. What a mind-blower that must be to a teen girl who thinks she's the only "weird" girl on the planet.

We continued our trip to Palo Alto and landed at the Stanford Mall. I love this mall; it's so beautiful and polite. The shoppers and workers smile. Sculptural fountains cascade into streams of clear water which run through the heart of the shopping center, framed by ferns and exotic plants. The shops are filled with beautiful clothing and jewelry I can never hope to afford, but we both love to explore. Queen Teen fell in love with a Betsy Johnson handbag (shiny purple and decorated with sparkling red and gold flowers. $300!). We discovered a tea shop and sampled the most delicious Jasmine tea I've ever smelled. The Stanford Mall is so far from my own reality, visiting it is like taking a trip to Monaco.

Tuesday was fun and relaxing, which was an excellent balance to Wednesday. NOT fun and extremely boring.

Her appointment was at 10:00 am and we were seen right away by her audiologist, Anne. Queen Teen really likes her, but she doesn't like the tedium of having her hearing checked. It took an hour and a half to test Queen Teen's hearing. She dropped blocks in a box when she heard the tone, and pointed to a picture when she heard Anne say the word. After that came programming the hearing aids and then trouble shooting the FM system. The device has had intermittent problems and Anne worked hard to figure out what was causing the device to stop connecting to the hearing aids. Of course the FM worked perfectly, just like when you take your car to the shop because it keeps making a horrible sound when you idle at a sign stop, but the car works flawlessly for the mechanic. Anne say FM devices are notorious for intermittent problems, so she wants me to keep track of when and where we are when the device stops working. What's in the environment can effect the signal, especially motion detector lights and wireless networks (seeing as we have five computers hooked up to a wireless network in the house, I'm thinking that might be a big problem).

After two hours, Queen Teen slumped in her chair, looking dazed and declaring, "I'm so bored!" An hour after that Queen Teen was just dazed. She'd stopped hearing anything, her system shutting down from fatigue and stress. At 1:00 we stumbled out of the basement where Audiology is tucked away, hungry for food and fresh air. We had a nice lunch and I grabbed one more cup of tea, then we hopped on the freeway heading home, hoping to stay ahead of Bay Area rush hour traffic. We made it to Novato before finding the stop and go traffic which we sat in all the way to Santa Rosa.

I decided she needed to have fun on Wednesday too, so we stopped at Border's books. She bought two new books and two new coloring books, then we had a snack at the cafe. There was another hour of driving before we got home at 6:30.

The hearing test showed that Queen Teen's hearing is reduced in her right ear, but her hearing fluctuates so much it isn't evidence that her hearing overall is worsening. Anne programmed the hearing aids to allow for volume control to give us an option for those days when Queen Teen seems to be having a harder time than usual understanding speech. We go back to audiology in six months.

I am impressed with how hard Queen Teen worked during the hearing test and her patience overall. Three hours would have been a strain for anyone, let alone a fourteen year old girl with a neurologic disorder. When I told her how proud I was of her, she smiled and said, "Yeah, but it was too boring."

Two Doctor's Appointments in One Day is Too Much

On Monday Queen Teen had a genetics appointment AND an audiology appointment at Stanford. Great for saving on gasoline and travel, lousy for stress reduction. To make it more fun, I booked a motel room the night before, just ten minutes from Stanford, which was a good idea, because it took FOUR HOURS for us to get to Palo Alto (it usually takes 2.5) Gotta love that Summer-time traffic.

Queen Teen likes the novelty of motels, especially the fact they have TV (we don't). In the morning we watched too many hours of Nickelodian and were almost late for our 10:00 am appointment. Another way I tried to reduce the stress was by inviting her dad to the appointment. She loves spending time with her dad and doesn't get to see him more than every couple of months (he lives in Davis).

Unfortunately, despite arriving the night before and staying in the fun motel room with cartoons, plus her dad meeting us, it wasn't enough to mitigate the stress of a marathon day of doctors, especially because there was lab work.

The genetics appointment went well, even though we didn't see the doctor we had made the appointment with. Our doctor was no longer seeing patients at that clinic, but the new doctor was very kind to Queen Teen and answered our questions the best he could. He reordered the lab tests the previous doctor had wanted but had never been done (loooong story) and he wrote the prescription for the amino-acids which MAY help decrease the degeneration of whatever condition Queen Teen has. Hopefully the blood tests will reveal a clue to explain what is happening and if anything can be done.

Then it was time for blood work. Queen Teen HATES blood work. I'm afraid she's been too traumatized over all these years of tests and doctors and hospital stays, so I try to limit blood work to only very important and/or necessary procedures. Queen Teen decided she didn't care if these tests were important, she wasn't getting stuck with no needle.

I hate putting her through blood work and I know I'm setting her up for a lifetime of PTSD every time she sees a needle. But sometimes it must be done. I'm used to the drama, but I knew her dad wasn't. While we were waiting our turn for the a blood draw, I said him, "Don't be surprised by how hard she fights. Just wrap your arms around her tightly, but keep them away from her face. She may bite."

"Oh, I don't think it will be that bad," he said.

I looked straight into his eyes. "Yes, it will. Also, wrap your legs around hers so she can't kick the Tech."

He nodded and started to look nervous.

"It will be alright. She'll get mad and scream, but the tech's here are used to screaming children and are quick. Just hold on tight so she can't jerk her arm when they draw her blood."

When the tech called Queen Teen's name, Queen Teen gripped the arms of her wheelchair tightly and said, "No."

I took a deep breath. "I'm sorry, sweety, but this is important. Let's go."

Her dad and I wheeled her in to the lab and then her dad lifted her out of the chair. Queen Teen immediately began to fight, kicking and screaming and headbutting. Luckily he remembered to keep his arm away from her face so she couldn't bite him.

The techs at Stanford are highly trained, but it took an extra tech to draw her blood. In less than a minute it was done. Queen Teen's dad put her back in her chair and wheeled her away, back through the waiting room where unfortunately five young children had just listened to her blood curdling screams. Sorry.

Queen Teen was cheerful again the moment we left the building, and by the time we had lunch she was laughing with her dad. We strolled around the Stanford mall and looked at the flowers and window displays, then her dad said goodbye. Queen Teen and I went back to Stanford, luckily to a different building far away from the lab.

Our audiologist is wonderful; knowledgeable, dedicated, and great with kids. Queen Teen smiled when she saw our doctor. We were there to pick up the FM system for Queen Teen's hearing aids, which should be a big help in noisy, crowded places, especially when I'm behind Queen Teen pushing her in the wheelchair. At first, Queen Teen didn't respond to the direct input of my voice through the FM system. She seemed confused by it. The doctor played with the FM for a bit to make sure it was working, but even though the device worked perfectly, Queen Teen still didn't seem to hear or understand what I was saying. The doctor believed that Queen Teen needed to practice hearing with the device so that her brain could process the new sounds. And she was right. I kept the FM on while we were in the car and after a few minutes Queen Teen was able to hear me very well. We had a real conversation in the car, something we haven't been able to do in three years.

That night, we stayed with friends in South San Francisco. I had tickets for King Tut on wednesday, so we decided to stay in The City and make it a vacation. On tuesday we were visiting friends. That monday night, Queen Teen almost feel asleep while eating her dinner and went to bed early. It had been a long, exhausting day for both of us. We had the next two days for a little fun.

Does Everyone Go to The Doctor?

Yesterday, Queen Teen and I walked with Rick to his appointment at the eye doctor, enjoying the beautiful California spring time weather. We took our time and stopped at flower gardens where we smelled roses and touched their wide, soft petals. At the doctor's, Rick went inside and Queen Teen and I walked another block to the cafe to wait for him. We got Italian Sodas and chatted about how excited she is to be going to Disneyland (she's never been).

Then she asked, "Where did Rick go?"

"He's at the eye doctor's getting his eyes checked."

"Why?"

"Because the doctor is making sure his eyes are healthy and if he needs new glasses."

She thought about this for a moment, then asked, "But why does the doctor have to check his eyes?"

"Everyone goes to the eye doctor for an eye checkup. It's important to keep our eyes healthy." I looked at her carefully as she stared at me. "Did you know that everyone goes to the eye doctor?"

She shook her head. "No."

"Yes. Everyone goes. Even me. And everyone goes to the dentist for a check-up and a cleaning, AND we all go to the doctor for a check-up. Everyone even gets shots sometimes. And sometimes we have to get our blood drawn for tests. You're not the only person who has to go to the doctor, sweety."

"I'm not?" She seemed surprised.

"No! Not at all. Everybody has to go to the doctor sometimes, not just you."

She grinned. "Oh." Then she drank her soda, looking very pleased with the idea that EVERYONE has to be examined by a doctor now and then.

This was another one of those moments that showed me how much of the world Queen Teen misses. She honestly believed she was the only person in the entire world who has to go to the doctor, except for the few kids she sees at clinics. The ONLY person who has ever had to have her blood drawn, or her brain and eyes and ears examined.

And why shouldn't she think that? She doesn't really know any other people with disabilities and doesn't know anyone who goes to the doctor as often as she does.

How did she miss this fact of life? How did I not show her going to the doctor is normal for everyone. It's just one of those facts we don't think about, because it just IS.

For Queen Teen, nothing is as simple as just IS.

The Low Vision Clinic in Berkeley

Last friday, Rick and I took Queen Teen to the Low Vision Clinic at UC Berkeley for an assessment. It had been five years since her last evaluation and we wanted to see if there'd been any changes. Plus, Queen Teen is eager to read and has been picking out sight words like "the," "and," and "there." She can identify items from a list by looking for the first letter of that word (M for "milk") and keeps asking "What is that word?" If there's any chance she can learn to read, then I will do everything I can to help her.

Laura Fogg, her Orientation and Mobility instructor, met us at the clinic. I invited her because Queen Teen has been less than cooperative at appointments. Even something as simple as a quick doctor's visit for itchy skin can become a tearful battle. Between adolescence and the hundreds of doctor's appointments Queen Teen has had to go through since birth, I can understand her lack of enthusiasm about ANOTHER appointment. Laura seems able to draw Queen Teen from her shell, plus she works with her at school so would have more info than I about how Queen Teen uses her vision.

As expected, Queen Teen was NOT happy to be there. She went along with the visit for a while, but as soon as it got too hard, or went too fast, she shut down and refused to lift her head. Then when the doctor tried to put glasses on Queen Teen to test lenses, Queen Teen kept shoving them off her face, once knocking the lens completely out of the frame. The doctor went too fast for Queen Teen to keep up, quickly switching from one task to the other before Queen Teen fully understood what she was supposed to do. I understood why the doctor was in such a hurry, but I wish she had taken more time to show Queen Teen what each test was and how it worked. Queen Teen is a curious person and likes to examine and question objects before they're shoved in front of her face.

There were times when Queen Teen got into the games, laughing when she stuck her finger into the hole on the contrast cards and playfully snatching the light wand during the visual fields test. She played with Laura and answered questions clearly, saying "I can't see that." I was proud of how she spoke up for herself, telling us when she could or could not see something and asking questions about what was happening. This is not a passive Multiply-Disabled child; this is a girl who is capable of understanding what is happening to her and expects us to explain WHY we're doing it.

Happily I can report Queen Teen's vision hasn't changed much in the last five years. Unlike her hearing and ataxia, her vision is stable. The doctor changed her prescription just a little to accommodate nearsightedness and astigmatism, which are actually normal changes most children experience as they grow. She's seen an opthomologist every year to check her eye health and her doctor has said it didn't appear that Queen Teen's vision had changed (her doctor is a certified Low Vision expert, so I know this woman knows what she's doing). Now we have verification from two Low Vision experts that Queen Teen's eye condition is stable and we don't expect it to worsen any time. Thank goodness!

We also talked about ways to help Queen Teen read and discussed the use of a CCTV. I'll look into getting her one for home use. Because of how bad the budget is in California, I expect a battle finding someone to pay for a CCTV, but if that's what Queen Teen needs to successfully learn to read, then I'll find a way to get her one, even if I have to hold a bake sale!