Dakota Update

I was too tired to blog last night after our long day at Primary Childrens. It was a very long day. The following is a detailed synopsis of our visit. It is quite lengthy and not necessarily intended for entertaining reading, but as my personal account for our own records and Dakota's medical history.

First we met with Dr. Ai Sakonju, Neurologist. She did a series of initial reflex and strength tests with Dakota yesterday. It was different watching the neurologist push and pull, bend and straighten, twist and turn Dakota's body and limbs in so many different directions. Dakota was luck Gumby and was not sure what to think of this doctor as she was being put into all different "poses" and stretches, but she made it through. We then moved into a different room for the nerve conduction studies and electromyogram (EMG). The doctor gave her a little portable DVD player to watch a movie of her choice, in hopes it would distract her from the test. No luck. Even "Barbie and the Magic of the Pegasus" was not enough to keep her from watching and feeling every discomfort from the test. The first part of the tests were nerve conduction studies which test the speed at which electrical signals (responses) are sent through the nerves. It is used to diagnose nerve damage or destruction. After having a couple sticky sticky pads put on various spots on Dakota's arm, the doctor would send electric shocks through specific nerves. The velocity (how long it took the nerve under the sticky pads to respond to the electrical shock given up the arm) of the nerve responses were then measured. These shocks would cause involuntary muscle contractions as they were sent through her arm. So, a finger or a thumb or even her whole lower arm would jump as her muscles reacted to the input. Dakota was not impressed. She became very uncomfortable and wanted to be done long before it was over. Her poor little eyelids would blink most every time she received a shock. Both arms, shoulders, hands were done and then the neurologists went back and did a few more on the left side in question. Luckily, the nerve responses were healthy. ("Yay"!) There were a couple response times that were "borderline normal", but they didn't seem to concern the doctor too much. They still provided good base-line information.

The next phase of the testing was the EMG. Electromyography checks the health of the muscles and the nerves that control the muscles. This test was fascinating to Darin and I, but not to Dakota. The neurologist stuck a very thin (but very long) needle through her skin and into the muscles being tested. (She was poked a total of 5 or 6 times in different muscles.) This needle was somewhat of a "microphone" that could hear Dakota's muscles. There is actually and electrode on the needle that picks up the electrical activity given off by the muscles. The "sound" given off by the muscle was audibly heard and displayed on a special monitor called an oscilloscope.

When her muscle was relaxed (at rest), it was quiet and produced no "peaks" on the monitor, for there was little muscle cell electrical activity to be heard. But as Dakota was asked to bend fingers, thumbs, hands, etc. the muscles were voluntarily being contracted and activity could be heard and seen on the monitor. As the contractions became stronger, more and more muscle fibers produced more action and thus more sound. Cool, eh? Poor Dakota was not amused. The needles hurt going in, especially the time the needle missed the muscle and hit a blood vessel. The needle popped right back out and a speck (yes, only a speck) of blood rose to the surface. But that was all it took. She wanted OUT! We were able to calm her down and convince her to not only let the Doctor stick the needle in other muscles, but to also re-do the muscle that we missed. Luckily, the neurologist was able to finish the test. The neurologist was pleased with the results. Her muscles are healthy and reactive. (Another "Yay"!) She was very impressed with Dakota's composure as she has had 7-year-old patients scream their heads off during the test. Dakota never once LOST IT. She whimpered a little, teared up a little, and had a few silent sobs, but pulled it together quickly. Her arms were quite sore after the test, especially the left one where all the needle pokes were. Her muscles were exhausted, and she was grumpy. But there was no time to stop. We should have had time in between doctors, but we didn't. The previous lasted more than 2 hours; we were actually a little late to the second doctor appointment. We left that appointment and walked right back into the clinic for the next appointment with the geneticist. WHEW!

We met with two people in the genetics clinic at PCMC, Janice Palumbos (geneticts counselor) and Dr. David Viskochil. We had met with Dr. V last January just after Dakota's diagnosis. He is the NF guy and we are very lucky to have him in Dakota's "network". Janice was responsible for getting us in to see Dr. V as quickly as we did last January. Through divine intervention (I don't know what else to call it), she found out about Dakota and did what she could to get Dakota in immediately. I had never had a chance to thank her personally until yesterday, as it was our first meeting.

Dr. Viskochil compared Dakota's lump presently with the one he took pictures of and notations on in January at our last visit. He agrees with us. It is growing! There is no question about that. One can clearly see the visible changes of texture in the skin, puffiness, bulging, etc. He has not received any word back from his colleagues at NCI about the measured progressive growth between the December MRI and her March MRI. There didn't seem to be enough, if any, growth as noted by the radiologist and himself. But, it has been almost 7 months since her last scan and we need another. Being able to measure the growth on the outside is one thing, but now we have to see what's going on on the inside. How deep? How big? Where is it going? etc. He wants the scan done this month and wants us to see Dr. Lor Randall (surgeon) again. Back in February, Dr. Randall felt it was better to leave the plexiform neurofibroma alone for the time being, because of the risks involved. Surgery is always a last resort. We would just watch it and wait. If the tumor kept growing and if it was hindering movement (which it's not yet, luckily), we would have to make the judgement call as to whether or not they should go in and get it. This could possibly leave huge voids of nerves and muscles in that area (not a great alternative) and could possibly cause the tumor to grow back bigger and meatier than it was before. What would you do? We'll know more after we talk to the surgeon. We may not have to make that decision...yet.

There is the possibility of drug treatment. Dr. Viskochil talked to us about the drug, rapamycin again. As I posted before, it has not been successful in shrinking plexiform neurofibromas that are not growing, but is still showing promise for those that are still growing. Whether Dakota's tumor has grown enough will be the question after her next MRI findings. Dr. V will send the scans again to NCI for consideration. If Dakota gets accepted to the study, she might have an opportunity to try the drug treatment. If that doesn't happen, there are other drug treatment studies coming up in the near future that she might be considered for. Or, if the rapamycin alone doesn't work for her (if she gets a chance to try it), they are starting to couple it with other drugs to boost it and help direct it more specifically to the area needing to be treated. Regardless, something needs to happen.

As for the other lumps? They are neurofimbromas (three in her head and one on her back) that go along with the territory. They are not of the plexiform type; however, which is good news. They are the kind that account for 95% of the tumors people get with NF1 (unlike the 5% of the plexiform type that Dakota has by her neck). Those don't usually cause too many problems other than being painful. The ones under the skin are more painful than the ones that grow on top of the skin.) Some can be "popped out" pretty easily depending on where they are at, but will leave nerve damage where they were taken out. We agreed that it would better to just leave them alone for now, considering the more serious plexiform immediately. I need to be careful when brushing Dakota's hair, because I occasionally brush over the three on her head causing her pain. Poor thing. The one beside her left ear is not connected to the plexiform off to the left of her neck, thank goodness. It's a separate tumor. 60% of adults with NF get these more common-types of tumors and usually can live with them or get them out easily. The other 40% have more serious problems, like Dakota's tumor. And 20% of those have really, really serious cases involving deformations caused by tumors under or on the skin in the face, legs, arms, back, etc. (making them look somewhat like the elephant man or like someone with big warts all over their bodies). For now, Dakota's tumor is appearing only by and around her neck. None of us know what direction Dakota's disease will go. Generally, NF seems to go in spurts picking up intensely during high hormone phases of life (puberty, pregnancy, etc.) For some reason, as of late, Dr. Viskochil has seen more girls than boys with plexiform tumors on or around the neck during pre-pubescent (low hormone) ages. He doesn't know why there have been more girls than boys. The disease does not favor one gender over another. But, he does suspect that the high concentration of Schwann cells around the neck is responsible for the number of plexiform neurofibromas he's seeing in his patients.

As we were leaving the office, I thanked both of them for their patience and time. I had been a little "mother bear-ish" in trying to get a hold of them for some answers. I told them that I realize they are busy and know that Dakota's case may not be as pressing...Dr. V interrupted me and said, "Dakota just moved up a few notches." She is now on the watch list; her situation being a little more serious now. Not what you want to hear from your child's doctor, but comforting because you know that they finally know and understand what your concerns have been all along. The gravity of the situation has increased, but so has their vigilance.

Dakota has got her whole life ahead of her. It will be difficult and unpredictable. However, she is in good hands. Dr. Viskochil said as we were leaving that Dakota is so much better off than the NF patients 20 years ago. They know so much more about this disease than they did before, and though there is no cure, medical advances and knowledge are growing regularly. I don't know if a cure will be found during Dakota's life time; however, she is in the best place she can be in right now, with specialists providing the latest treatment and knowledge in the field and KNOWLEDGE IS POWER!