Maria Rose

Maria really loves the walker and so do the rest of us. Even Manolis has put her in it a couple of times and I don’t think he’s ever used any of her therapeutic equipment. She does a little better in it each time. I’m sure it’s not adjusted correctly but the PT comes today so she may be able to set it up better.

On Saturday we went to a meeting of the Phoenix chapter of the Hydrocephalus Association. In the past the local support group had been just for people who have normal pressure hydrocephalus, which affects people aged 50+ and is different than what Maria has. This is the first meeting where they added support for other forms of hydrocephalus. Cathy met another mom with a kid a little older than Maria.

Then on Sunday Shriners had their annual Cornfest. Cathy took Maria just so they could meet the beautiful girl they are helping. Maria goes in for her follow-up with Shriners a week from Friday (the 5th), the same day as her first visit with the new gastroenterologist we met a year ago.

When I picked her up from school yesterday she seemed the least stressed out she has so far. She does fine in class but is pretty overwhelmed by the end of the day and usually takes a long afternoon nap. Yesterday she was calm and didn’t sleep when we got home. We are hoping that means she’s getting used to school.

Her stander is supposed to arrive today so there’s another new toy to play with. Since she can both sit and stand in it, it might be useful as a feeding chair. She barely fits in her high chair and it’s falling to pieces anyhow.

Oh, yeah. I guess we never posted the link to the followup article that appeared three days ago.

I’ve added some links in the column to the right to other special needs blogs and equipment we like.

Maria’s still feeling poorly. The antibiotics have given her a yeast infection but since we know what one looks like now we are on top of it. She didn’t have any seizures when I was there yesterday, but then she slept most of the day.

The neurologist got her blood tests back. Her Depakane level is find but her Trileptal is a bit low so he nudged that up. We hope it helps soon since we are leaving town Friday!

He reminded us that this is not unusual. She is going to have stretches of no seizures, but they are going to pop up again periodically. When she’s tired or sick or stressed she’s likely to have problems. When things calm down she’ll do better.

I just uploaded a bunch of new pictures. So now I’m only two months behind on pictures and a year behind on videos.

Maria’s seizures are getting worse. She’s regularly having about a dozen a day and one was almost a minute, which is MUCH longer than they usually are. She’s been cranky and I’m pretty sure a lot of that is because of the seizure activity.

They weighed her at the doctors - and she’s still 30 pounds! I just don’t believe it. She is noticeably chunkier and the weight gain would explain the increase seizure activity, so we are baffled.

The are doing blood tests to check her drug levels before increasing her regular medication. In the meantime she’s getting her emergency Clonazepam about every other day. We (especially her daddy) just hate that stuff since it turns her into a zombie, but it definitely shuts the seizures down fast.

Unfortunately, the ONE person in the city who can draw blood from Maria has left her pediatrician’s office. Since most people just torture her for 5-10 minutes, digging around with the needle hoping to get lucky, and because blood draws are a big seizure trigger because of this, we were very unhappy. However the new guy took his time and got it the first time so maybe he’ll work out.

And because we don’t have enough problems, Maria got a bad bite from her kitty this week. We know how patient Kijana is with her so Maria must have been severely torturing her to have triggered that. As most of you know, Cathy and Manolis have an uneasy truce over the cat. He hates cats, convinced they are all vicious and disease ridden like the feral cats on Crete. He barely tolerates Kijana out of respect for Cathy. This has not helped the situation, especially since the bite is getting infected.

So as long as they were at the doctor, they got someone to look at the bite. They gave her a tetanus shot and antibiotics and it should be OK.

Good news though! Someone from Shriners called after reading the article and has offered to pay for the stander. Although we certainly feel the DDD should pay for it, I think we’ll take the bird in the hand rather than continue to fight.

There will be a follow up article on all of this. We’ve had lots of people offer to donate money for the stander, including one guy who offered to pay the first $1000! We are going to ask the columnist to mention that their money would be better off going to Shriners or some organization like that to help families more in need than we are.

Maria has been featured in another newspaper article, the second in her young life. This one is in the Arizona Republic.

I don’t plan to read the comments since the internet turns most people into idiots but I did read the first couple. It is amazing how Arizona residents can turn anything into a debate on illegal immigration.

UPDATE: The article is also posted here. It’s the same article but there are different comments (no immigration nutjobs yet).

By an amazing coincidence that I’m sure had nothing to with the article (yes that was sarcasm) DDD just called and said they have approved the walker. However they haven’t changed their mind on the stander, so we still have our hearing on Monday.

We are still on schedule for our court date July 28. We had hoped the DDD would cave but they are apparently going to go through with it. We got a letter from the Attorney General naming which of their lawyers is going to represent the DDD. We are going with self-representation. Cathy called a legal aid charity but they have far more requests than lawyers. We think it will be OK, but that’s what we thought about the original claim and the appeal!

We have enlisted the help of a local newspaper columnist. She usually writes about kids who have fallen through the cracks of Child Protection Services but she was appropriately outraged by Maria’s story. She is going to interview us on Monday. Our concern isn’t so much about Maria specifically. We wonder how many other people the DDD are screwing over who don’t know how to fight back.

Maria’s been having seizures lately, a couple a day pretty regularly. Then she had a bunch on Tuesday and ten today; both times she got a dose of Clonazepam (the emergency medicine). The PT suggested that since Maria is gaining weight (she’s up to 33.5 pounds by Cathy’s scale) that means her regular dose is too low. We realized this today (Friday) at 4:30 pm of course. Cathy’s going to try to get through before the weekend to see if he’ll up the dose over the phone but it may have to wait until Monday.

Maria continues to work hard toward walking. Today she took 57 steps in a row when working with the PT! She still needs support since she can’t manage to hold her own weight, but she initiates the steps. She gets bursts of 10-20 steps that are in a very regular tick-tock rhythm. Today was the first time I’d seen it and it’s really great! We figure she must have heard about the hearing and is hoping to walk for the judge.

I finally got a chance to make sense of my notes from the conference.

There was a lot of talk about ETV at this conference. At this point ETV has about the same reliability as a shunt but is more appropriate for certain patients. For example ETV isn’t effective on babies because you need pressure in the cerebrospinal fluid to keep the hole open, but babies and their soft skulls have lower pressure than older kids or adults. ETV is more risky up front but has fewer problems in the long run than a shunt.

They talked about different kinds of shunts. Basically all the manufacturers are about the same. They each have their little gee-whiz features but in the end show the same rate of success and the same rate of malfunction. Programmable shunts, like Maria’s, allow doctors to change the flow rate but realistically they don’t have any need to. It just gives one more thing to break down. However in Maria’s case a programmable was appropriate because her ventricles were very large. They had to drain slowly to give her brain’s structure time to adjust to the changing pressure, then increased flow as pressure approached a normal level.

There is a ton of research just being done or right on the brink of being done. They are looking at new ways to understand what causes hydrocephalus, what effect it has on the brain, and how they can treat it. The shunt was invented only about 55 years ago. Before that they couldn’t do much to treat it. They’ve made strides as they understand it better and there is likely to be a lot of change over the next few decades.

The last night of the conference they had a big party with a talent show. The keynote speaker, Sherman Alexie was a guy whose mother had been told to abort the pregnancy because he would be born dead or a vegetable (his mother’s reply, “What kind of vegetable?”). Even after birth they said he would die or be severely brain damaged. He is now a college graduate, a successful author, screenwriter and standup comic, and has won numerous awards.

I heard five other women say that their OBs had told them to abort their pregnancies for the same reason. Apparently obstetricians know absolutely nothing about neonatal disorders.

After the conference we took our free gondola ride to the top of the mountain and hiked around. We went into Salt Lake to drop Rosanne at the airport then Cathy and I went to a planetarium to kill time before our flight. All in all pretty fun and in hindsight we could have taken Maria after all. At any given time usually there was one of us free to take her and she certainly would have been the cutest little girl in the place as always.

Our little four year old has been named as the complainant in a legal action against the Arizona Department of Economic Security.

This is the latest in our long series of efforts to get Maria the walker and stander that she needs. We go before a judge and argue our case and DDD does the same. In other words it’s the big bad government agency/insurance company against the adorable little disabled girl. We are hoping that DDD will pull their head out and rule in our favor before the court date (July 28). The rules say we don’t technically need a lawyer but after reading the rules I suspect we will need one.

Because the universe loves to complicate things, this is the same date as Sue’s surgery for her weird ear/brain/whatever thing that continues to baffle the doctors.

I’m going to post more about the Hydrocephalus Conference but every time I sit down to do it (there’s a lot of information to organize) something comes up. I’ll do it probably over the weekend.

Rosanne, Cathy and I are here in Park City at the Hydrocephalus Conference. I had thought today was just registration but they had a session on cognitive and scholastic performance of kids with hydrocephalus so we had to go to that.

A study compared kids with shunted hydrocephalus to kids without hydrocephalus, often their siblings to minimize the number of variables. These were high-functioning hydrocephalics, meaning they were on the same level as their age group in school.

It showed that through middle school the hydrocephalus kids perform as well as their peers in school but average about 15 points lower in IQ. When the look at the individual cognitive tests they see that kids with hydrocephalus have specific weaknesses, specifically verbal memory (remembering what people say to them) and executive function (planning and organization). However they do as well as their peers in visual memory (remembering what they see). So, for example, they have trouble remembering a class lecture unless they take notes and review the notes later.

As they get older, they are expected to be more independent (executive actions) and more of their education is lecture only (verbal memory) so they start to fall behind. They can still do well in high school and college but require particular strategies to succeed. The doctor gave an example of a woman with hydrocephalus who had a PhD yet did poorly on IQ tests.

They looked at many of the conditions that occur alongside hydrocephlus such as seizures and prematurity and found that they weren’t skewing the outcome. So it is something to do with either hydrocephalus itself or the shunt procedure.

They did another study that compared people with shunts to people with ETV. A shunt is a physical tube and valve and has complications like any medical device. When doctors perform ETV (Endoscopic Third Ventriculostomy) they cut a little hole in the brain so the fluid can drain out in a more natural fashion. Each procedure has plusses and minusses although my guess is that ETV will eventually become the standard treatment once they perfect it.

The study found that there was a difference between the patients with ETV and those with shunts. In most areas the ETV people had higher function although in some areas (I can’t remember which) the shunt people did better. This was a very limited study - they were able to find only 6 ETV people to participate - so they aren’t drawing any strong conclusions to it.

I don’t know if I’ll do daily updates while we are here. It just depends on how busy we get.

As for Maria herself, she has been doing great lately. She’s growing more interactive and aware. Her therapists have noticed it and are very excited about it. I don’t know if it’s the Jin Shin Jyutsu but it seems to have started about the time the treatment did.

Maria had her semi-annual evaluation this morning.

The doctor was really impressed with how much progress Maria has made since the last one, which was right before we went to Shriners in November. She admitted that in past evaluations she sometimes had to struggle and fool herself (her words) to see much progress. She was happy to see so many examples of how much better Maria interacts with the world. I suspect that getting her seizures under control is a big part of why she has developed so much.

She also said Maria’s muscles were much looser and she thought that might have been the seizures as well. Maria was probably having many mini-seizures (dozens or even hundreds per day) that kept her muscles tight.

Maria’s scoliosis is getting more pronunced and the doctor said that her ribs are starting to move out of alignment as a result. At this point it’s nothing serious but it’s one more thing that could get worse. She said that scoliosis doesn’t get better, which surprised me because I had been assuming that we could reverse some of the damage.

And we continue to fight with the DDD to get the equipment that Maria needs to keep the scoliosis from getting worse.

She recommended sending Maria to a nutritionist, which we all agree is a good idea. Her swallowing and eating are still a problem and the concern is both the calories and the actual nutrients. It would likely be a matter of adding some powders to her milk so it would be easy to keep up on.

Her height is 36″ which baffles us. That is what it has been for months at least and maybe over a year and yet she is clearly longer. She is outgrowing her current stander, her high chair, and a few other things. We watched the doctor measure her twice and agree that she did it right, but she got 36″. Weird.

Later in the day we took her to a Jin Shin Jyutsu session, which was my first chance to observe it. Debbie, the practitioner, touches Maria very lightly at two points, say on her wrist and her back. The idea is to unblock the energy flow through the body - or something like that.

I’m both open minded and skeptical toward alternative therapies. Some, like the Qi Gong, definitely did Maria some good. I may not understand how it helped, but it clearly helped. Others, like the faith healer, didn’t seem to do anything.

Since she started the Jin Shin Jyutsu all of us (Manolis, Cathy, Sue and I) have noticed numerous examples of Maria acting more in tune with the world. It seems related to vision and hearing, although I’m not sure if it’s the sensory input or the cognitive interpretation of the input that has improved. It might be coincidence, but it sure seems to be linked to her therapy sessions.

Maria starts with a new speech therapist on Tuesday. Then Cathy, Rosanne and I are going to the Hydrocephalus Conference on Thursday and get back just in time for Maria’s 4th birthday!