Maria Rose

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!

It’s been an interesting couple of weeks.

Maria developed some kind of respiratory thing, but this was different that ones she’s had before. It was bad at night and kept her parents awake, but during the day she was mostly fine. When it was bad she would wheeze and whine and do that “seal bark” cough that was so high-pitched sounded like a scream. When she slept I could see her struggling to breathe.

Cathy got her into the pediatrician the Friday before the long weekend, and it turned out to be a rough visit. 20+ minutes of Cathy holding Maria while the nurse tried to get her blood oxygen. Then 30+ minutes of Cathy holding Maria while I tried to give her a breathing treatment. Then 20+ minutes of Cathy and me holding Maria while the doctor tried to get a followup blood oxygen. By the end of it, Maria was a basket case. She was freaking out, her breathing was really weird, she was possibly seizuring, and so on. Needless to say, me and Cathy and the doctor were basket cases as well.

Her blood oxygen was around 91 and below 93 is when they put you in the hospital. The doctor said any other kid she would put in the hospital but she knows how close an eye we keep on Maria so agreed to let us take her home as long as we promised to head to the emergency room at the slightest hint of trouble.

The doctor had us give her breathing treatments every 2 hours (instead of the usual 4) and a round of antibiotics. Over the weekend we kept going back and forth about the emergency room but Maria got through.

She’s a little better but still wheezy. The doctor now has her on 5 days of steroids (prednisolone) and if that doesn’t clear it up we will go back in.

We have no idea what caused this. The symptoms are much like asthma, although the doctor pointed out you don’t call it “asthma” unless it happens over and over. For now it’s just reactive airway disorder and clinical pneumonia.

Maria hates the steroid and refused to take any of her medicine yesterday. When we have to fight with her to get the medicine in, there’s that much more of a chance of aspiration.

Meanwhile, we had a meeting with the insurance to evaluate Maria’s chance of walking, still trying to get the walker approved. There was a nurse from DDD and a representative from Cathy’s private insurance. They both seemed pretty supportive of our need but they aren’t the ones who make the decision. The PT was there and worked with Maria in the walker we want and we had lots of video of Maria walking with the PT that we gave them so they can show the medical director. We felt a bit more optimistic after their visit, but the decisions have been so nonsensical up to this point that we aren’t getting our hopes up.

In the meantime, Cathy’s been talking to her attorney about all this. She’s not an insurance lawyer but she is an advocate for special needs kids so wants to help if she can. If this latest attempt to the get this medically necessary equipment falls through we have to go to a fair hearing and we’re not sure what that involves.

June is going to be a very busy month. Lots of doctor visits and the Hydrocephalus Conference. It’s going to keep us old folks on our toes.

The big news with Maria is that the state has denied to appeal to pay for her walker and stander. Their reason is that they don’t believe she will ever walk independently.

Even if that were true, and none of us believe it is, that’s completely irrelevant. As her PT pointed out, does that mean they don’t pay for a cane if the person is unable to walk with a cane? The first issue is that she needs to do more weight bearing for the health of her hips and spine. The other benefit is that the ability to move around on her own in the walker will increase her cognitive development.

So we now get to have a hearing. We will certainly bring her PT as well as video evidence that Maria is showing interest in walking. The PT recently helped her walk back and forth over about a 30-foot stretch over a dozen times! We will also bring any other medical or therapeutic support we can to argue our case.

And every day that they jerk us around, Maria’s hips and spine get a little bit more deformed.

As someone who worked for health insurance for ten years, I generally side with the insurance companies. Most people have an utterly absurd idea of what medical insurance is then they get mad at the insurance for not living up to their fairy tale fantasies. However speaking as a medical insurance professional, this situation is just utter crap. This should have been a rubber stamp approval and they are dragging it out month after month.

Meanwhile, Sue has this thing in her ear and the doctors don’t know what it is. It’s some kind of fluid sac and they are utterly baffled by it but they are going to do some more tests then go in through her skull and cut it out. Sounds like fun. It also means she won’t be able to take care of Maria for at least a week.

This coming week is Maria’s last week of school. I think she’s going to miss it because she really seemed to love school, but it’s not long until it starts again in the fall.

Things have been pretty busy over the last couple of weeks. Cathy’s been traveling, Sue’s had some health problems, and Maria’s had a few issues too.

Maria had several Vitalstim treatments last week to get her in the best shape possible for her barium swallow on the 1st. Despite the treatments she still did quite poorly, aspirating several times during the test. Although they didn’t mention a feeding tube, they recommended that we give her honey-thick liquids only - no food. We are stubbornly continuing to give her food, but it’s still the mushy stuff. She has trouble with it and we have to feed her slowly but we still think it’s best. We go back to her regular feeding therapist this Thursday so we hope she has some good advice.

We closed out her current IEP this week and set up goals for next year’s. The meeting had her teacher and all her therapists so we could discuss progress and goals. Her teacher talked about how they were afraid to leave her alone for even an instant at the beginning of the school year but, as they’ve gotten to know here, they’ve become more confident and realized that Maria often makes the most progress when people back off and let her do it herself. That’s a lesson we are still learning after four years.

We talked about how Maria is becoming more communicative in her way. Cathy and I have notices how Maria lets us know when she’s in pain, even if we can’t always tell what hurts. In the past she might jump if something happened like getting a fold of skin caught in the belt buckle, but after that she’d just sit happily. Now she has long-term reactions to things and lets us know when she’s not happy.

The last day of school is coming up and it’s going to be a bit hard to go back to having her home all the time. The teacher tried to get her on the year-round school but it was decided that she’s not likely to lose her skills over the summer so she doesn’t qualify. They will see how she does in the fall. The teacher thinks (and I agree) that it will take Maria some time to get used to being back around so many kids again and that could be enough to qualify her for summer school next year.

She has a follow-up with her neurologist this week along with, as mentioned, her normal feeding therapist. She got a couple of blessings at a Greek Orthodox monastery yesterday, so we’re covering all the bases.