Wow! It's been awhile since I updated

So what do we know? NOTHING!!!!

The Neurosurgeon at Mayo thinks there are two possibilities. 1) she's having some type of seizure activity that we just didn't catch on her 24 hour EEG. This would make sense considering when we notice problems with speech and motor skills she's also very irritable and tired. BUT...these can last from 15 minutes to a couple of weeks, which is NOT typical of seizure activity. Or 2) that she has a generalized right-brain dysfunction. This would also make sense given the duration of the events. The problem with this posibility is we'll probably never know the why/where/how/ of it, because, in the words of the neurosurgeon, "We know a lot about the brain, but we don't know it all yet. There are lots of things we still don't understand."

Angela is supposed to be going in for a 3 day in-patient EEG at the end of the month, but I think I'm going to have to reschedule it. Shouldn't be too much of a problem.

Angela was approved for Make A Wish! Yep, all the paperwork went through, and the wish grantors assigned to her are coming to the house on the 27th to talk to her about her wish. Her answers vary depending upon the moment that you ask. If we ask "What do you wish for?" She might say, "to be an astronaut!" or "Go to Hawaii!" or "Go fishing with Uncle Jeff!" or "A cherry ice cream cone!" or, "Give Zach and Cody a hug!" (these are characters on a TV show she watches.)

The Zach and Cody theme has been around a long time. They are from the show "The Suite Life of Zach and Cody" on the Disney Channel. On the show, the twins live in an upscale hotel and get into all kinds of trouble as most tweenagers do. Last year when Angela and I went to Atlanta for the national DS conference, as we walked into the hotel she got all excited and could barely contain herself. "Yes!!!! Zach and Cody are HERE!!!!" It took a lot of convincining for her to understand that Zach and Cody were not going to appear around any corner in the hotel. Actually, I don't know that she was every convinced.

Over the past year Zach and Cody have been an intregal part of her pretend play. They party here every afternoon, they eat dinner with us, ride with us in the car. When we go shopping I have to say, "Now Zach and Cody...I'm sorry but you can't come into Target with us, because there are no invisible people allowed in the store. You'll have to wait in the car, ok?" I then remind Angela that talking to invisible people in public places probably isn't the best idea, but that I'm sure they'll wait patiently for her in the car while we get our things done. She then spends our time shopping talking to herself, "Zach and Cody are in the car. Yep....waiting for me. Yep....Zach and Cody....be patient...wait patient." (I wonder if she's telling THEM be patient, or herself?)

So last week when the Make a Wish person called me, she asked if I had any idea what Angela might like to do for her wish. I told her that meeting Zach and Cody would be a pretty safe bet! Oh, and by the way, they film at Disneyland in CA. Looks like that's probably where we'll be going if she gets a travel wish granted.

Waiting...

Well, I never heard from the neurosurgeon over the weekend. I tried really really hard to be patient, but by Sunday my patience had run thin. As much as I tried to tell myself "no news is good news" I just couldn't hold out any longer. So I called the neurosurgeon on call, who pulled up Angela's chart and read me the notes from Friday's angiogram. It said, "No evidence of vascularopathy, and no evidence of Moyamoya."

That means that Angela does NOT have any vascular disease, nor does she has MMD! That was music to my ears. What we still don't know is why she's having right-brain disfunction, and if she'll continue to have it. I have lots of questions for the neurosurgeon, but until I hear from him I'm in the dark!

Things that make you go "hmmm"

Ok, very first words out of the NS's mouth were, "I don't think she has Moyamoya, and I don't think she needs surgery...yet."

He doesn't think her MRI is remarkable for MMD, (is sharing information with Stanford and Boston though) but her MRI isn't completely normal either. There is NO evidence of stroke at this point. The vessels he sees on MRI appear stong and healthy. HOWEVER, within the circle of Willis (the main vasular system in the brain) she is missing one vessel on the right side. BUT, the same vessel on the left side has crossed over to the right and seems to be doing what it's supposed to be doing, just over there instead.

He has no explanation for the 3-4 weeks that we saw dramatic right-brain decline (and I brought along letters from the ST about their observations as well.) And said that....sometimes....there can be right-brain malfunction for absolutly no reason at all that we can see on any scan. That sometimes it's just too deep for anyone to actually find. And that it will likely happen again.

That said, we're still doing the angiogram tomorrow. He said if we were looking at MMD, the SPECT scan wouldn't be that important, but since we're now looking at something different, he'd really like to have that done. It can show us brain function problems that won't show up on an MRI. But THAT test was cancelled for tomorrow because they can't do them at the same time/on the same day. We'll probably schedule it a couple weeks out.

We ended up coming home tonight. They decided they didn't need to admit her, and I needed to let Ronald McDonald house know by 4:00. By the time I knew what we were doing it was 4:30. The hotels in the area who have RMH rates were already booked. So, it was pay regular rates of $45/night, or spend the same in gas driving home, then to the clinic and back AGAIN tomorrow morning. I was all ready to get a room because I was wiped but Angela announced, "Moooooooooom! No Pool????" and I realized that was going to be an issue. So we just came home.

We have to be back there at 6:30 tomorrow morning, which means get up at 4:30. Lovely.

She also had to see the pediatrician down there for a pre-op. It was a resident with no DS experience, but he tried very hard. LOL He went out to consult with the ped, then came back and asked when her last Thyroid and CBC work was done. Hmmm...I had no clue. He went back out, then came back and told Angela, "I want you to take this paper to the lab, OK? They're going to need to get a little blood sample."

Angela looked at him like he was an alien, crossed her arms and STUCK HER TONGUE OUT AT HIM!!!!! "I said NO POKES TODAY!!!!! I'm NOT having a POKE!!!" Then refused to look at him again. We left with her trying told bag gigantic crocodile tears. When he said goodbye she stuck her tongue out at him again. LOL

So tomorrow will be another long day. BUT...if all goes according to schedule, her dad is picking her up at my house afterwards and keeping her until Sunday evening.

That means I can take a nap. I'm really needing a nap.

MRI

The MRI went fine

We got to the Neuro's office, gave Angela her night-time meds, and she slept through the entire 90 minute procedure. It was a little tough getting her to walk out to the car in the downpour we were having, but other than that it was a piece of cake.

This morning we have her IEP, then head to Mayo. It's only an hour and a half away so not a big deal. If I can get online I'll post an update tonight about what the Neurosurgeon and Neurologist say.

So what are all these tests?

Here's an explanation of the tests Angela will be having next week:

Full-spine MRI: Lots of people have had MRI's done. A full-spine MRI takes a couple of hours, and sometimes involves having dye injected. Angela had a brain MRI done a couple weeks ago and without any sedation layed perfecty still for an hour and 20 minutes! This time we're doing late in the evening and she'll have her meds so she should sleep through the entire procedure. Here's a description of it if you're interested.

SPECT: Single-photon emission computed tomography (SPECT) studies use radioactive materials to get information about blood flow and activity in the brain.

Radioactive materials are inhaled or injected and then move through the blood to the brain, where the different regions of the brain can be studied for abnormal functioning.

Angiogram of the Head and Neck:

Angiogram of the head and neck is an X-ray test that uses fluoroscopy to take pictures of the blood flow within the blood vessels of the head and neck. A thin flexible tube called a catheter is placed into the femoral blood vessel (femoral artery) in the groin or just above the elbow (brachial artery) and guided to the head and neck area. Then a dye (contrast material) that contains iodine is injected into the vessel being studied to make it more visible on the X-ray pictures.

An angiogram of the neck (carotid angiogram) can be used to evaluate the large arteries in the neck that lead to the brain. An angiogram of the head (cerebral angiogram) can be used to evaluate veins or the four arteries (four-vessel study) supplying blood to the brain. An angiogram can detect a bulge in the wall of a blood vessel (aneurysm). It can also detect narrowing of a blood vessel or a blockage in a blood vessel that slows or prevents blood flow, an abnormal collection of vessels (arteriovenous malformation), or abnormal vessels supplying a tumor. The angiogram pictures can be produced on regular X-ray films or stored as digital images in a computer.

See an illustration of the blood supply to the brain.

Why It Is Done

An angiogram of the head or neck is done to:

• Detect blockage or narrowing of the arteries in the neck that supply blood to the brain (carotid angiogram). Blood flow to the brain that is slowed or stopped increases the risk of having a stroke or a "mini-stroke" (called a transient ischemic attack, or TIA). See an illustration of an angiogram of a TIA.
• Evaluate symptoms that might indicate problems with the arteries that supply the brain. Symptoms may include severe headaches, memory loss, slurred speech, dizziness, blurred or double vision, weakness or numbness, and loss of coordination or balance.
• Detect an aneurysm in the brain or in a blood vessel leading to the brain. See an illustration of an angiogram showing blood flow in the brain.
• Investigate the pattern of blood flow to a tumor. This can help determine the extent of the tumor and guide treatment.

Thursday

Wow...Looooooooooong day here today! First, I had a meeting with a public health nurse and new social worker about the TBI waiver. (traumatic brain injury) I am in LOVE with this man. Well, maybe not HIM, but that he is here to represent ANGELA, and HER NEEDS, and not the needs of the county. So they have all the medical documentation, along with everything from the psychiatrists, etc. He is recommending her for THE HIGHEST level of the waiver, called a TBI-NB waiver. Meaning Traumatic Brain Injury -Neuro Behavioral. This will about TRIPLE her funding! A certain percentage can be used to pay me, a certain percentage for weekend respite care, a percentage for 'stuff' (we can fence that *^&$# pond in our back yard...or fill it in!) A certain percentage for training (Like the conference PLUS a PCA to go with us!!!) Oh, and ANY needs that the social worker deems immediate necessities. He wants all our doors to be hardwire alarmed with a panel that talks and tells you WHICH door opened...like "basement walk out" or "Garage service door". We could do things like put in taps that CANNOT scald her, handrails in the shower, a certain type of shower head, etc. (he wasn't saying she needed those things, just listing them as examples.) OMG, that FOUR HOUR meeting MADE MY WEEK!!!!!!

Then there were the phone calls. HOLY COW I've heard from everyone today! Without repeating it all, here's the plan....

Tuesday 8:30 pm to the hospital for a full-spine MRI. We'll not only be ruling out AAI, but checking for an other anomolies along the way. Ths is to rule out her problems being orthopedic vs. neurological. This is a 2 hour scan. We're doing it late so I can give her nightime meds and she sleeps through the whole thing instead of having to sedate her with an IV.

Weds at 12:30 arrive at Mayo Clinic for check-in. 1:20 meet with the neurosurgeon and neurologist. When that's done she'll be admitted to the hospital, stay overnight then Thursday morning will have an SPECT and Angiogram. (and a couple other tests that I've now forgotten the name of.) THESE are THE TESTS that we need done. When these are done we will know if we're going to Boston, Standford....or worst case scenario....nowhere but to sit back and wait for her to have a stroke.

So that's what I know. I'm having a good day today because I have a new love in this new social worker. He said things to me, or asked questions that I'd been feeling for months, but never been able to put words to. Things like, "So...when was the last time you were able to let your guard down and just be you?" Ummm HELLO! 11 years ago when she had her first stroke!

So, that's our day. We'll see how next week goes.

A grave diagnosis

I guess it's time for me to start blogging on a daily basis. I need to be able to keep my thoughts together, and to keep people updated without spending double the time to do it.

Some of you are from my forum family and already know. Others are just stumbling upon my blog. So, here's a post I made to my forum family on Friday, April 27, 2007.
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Early this morning we had Angela's appointment with the neurologist. Since her MRI was done a couple weeks ago and I hadn't heard anything, I'd decided that "no news is good news". And then a few days ago I realized...hmm...when her EEG was done they called me in a couple days to say it was normal. When her EKG was done they called me in a couple days to say it was normal. I haven't heard boo about the MRI....and now I know why.

I really didn't think it was possible, but what Angela has is worse that MoyaMoya disease.

In Moyamoya disease, there is a narrowing (sometimes blockage) on the internal carotid artery in the brain. As the narrowing progresses the brain, being in short supply of blood and oxygen, will start to create new blood vessels that kind of search out new blood supply. When this happens they can do a procedure, (very basic explanation here) that gives the brain a new blood supply to leach onto to. It's major neurosurgery.

So, as an infant Angela was found to have a narrowing on her Right carotid artery. But there was no revascularization, however her SYMPTOMS followed those of MMD. We've watched her all these years. About 3 or 4 years ago she started having TIA's (transient inschematic attacks, which are basically like mini-strokes.) but her MRI showed now changes in either the narrowing or revascularization, leaving everyone puzzled.

About 4 weeks ago she started having what are called "Tripping spells". Apparently these spells are indicative of a new blockage, so we repeated the MRI.

The MRI results are devestating.

Angela now has a significant blockage on her LEFT carotid artery that was not there before. It is WORSE than the original blockage on the right. There is NO revascularization going on. THAT is the worst part of all. If there were revascularization starting, we'd have an operable situation. Without the revascularization, the problem blood vessels that are located in the brain stem are not operable.

In the 4 weeks since the Neuro last saw her, Angela's gait has changed significantly, and it's because the right side of her body is now affected as well. Her speech is somewhat more difficult to understand. Her pupils are uneven. She is very tired. He said the behavioral issues are most likely because she ALWAYS has some level of a headache, and the escalations are problably when she's at more of a migrain level.

He is contacting the Neurosugreons in Boston and Mayo who specialize in MMD and have more experience with the disease in kids with DS to see if they have any thoughts. All of her records are being sent to each of them. All the rest of her symptoms fit MMD, the narrowing on both the left and right Carotid arteries fit MMD, but the lack of revascularization has him puzzled.

Angela's biggest risk right now is a massive stroke. A massive stroke on the left side of the brain at the level of the brain stem will not be survivable.

I am in shock and numb. I have known for years there was something more happening. In the last month my stomach has been in knots knowing that there is something more. But I expected him to say the MMD had progressed to the point where it's time to do the surgery. I didn't expect to be told it's likely there is nothing they can do.