Nothing New

No new developments in my medical life.

This Monday, May 3, the Fox show, "House," will be focusing on diagnosing a patient with Chiari Malformation. The episode is called "The Choice." Based on the promo, we know three things so far: 1. The patient is male. 2. He is accused of faking his symptoms. 3. He begins lactating. The second fact is very common of Chiari patients. And the lactating seems to be a sign of hormonal imbalance, probably caused by flattening of the pituitary gland, which is also common in Chiari patients. They just had to find a shocking presentation of the disease for the show. (At least this is my analysis.)

I'm pretty excited to see it. I hope a lot of people see it. We'll see if it can bring some awareness to the condition.


I found a compilation of symptoms of autonomic dysfunction that is really comprehensive. I'm including the link here. I'm also going to include a few that I found to be particularly specific to me that I hadn't seen before.

http://heavenleigh412-ivil.tripod.com/id23.html

-"Irritability due to decreased blood flow to the brain"

-"Inability to tolerate changes in temperature"

-"Abnormal deep tendon reflexes"

-"Intelligence is normal when receiving adequate cerebral perfusion."

-"Disruption of sleep/wake cycle usually consisting of increased energy late in the evening and lowest energy level in the morning regardless of amount or quality of sleep."

-"Need to sleep 12-14 hours in order to complete simple activities of daily living."

-"Distorted depth perception resulting in a feeling of unsteadiness. Often appears to be "clumsy" or excessively cautious when climbing stairs or reaching for an object."

-"Decreased awareness of what is in the peripheral visual field. This often causes the patient to startle because he/she did not perceive anyone or anything next to them.

-"Frequent "bumping into things." Attributed to a combination of visual and depth perception deficits."

-"Noise sensitivity. Loud or beating sounds can cause pre-syncopal episodes. Difficulty filtering out sounds. Easily distracted by sounds."

-"Sensitivity to odors. May cause extreme nausea, retching, vomiting, dizziness, and headache."

-"Sensitivity to touch. Mild pat on the arm or squeeze of the hand can cause excruciating pain, especially right after an episode or if the patient has not had enough sleep."

-"Taste and appetite changes. Fruits and other acidic foods may taste extremely acidic. Foods may taste differently if patient is tired, stressed, or post-syncopal episode."

-"Speech disturbances: Inability to finish a thought, loss of train of thought, especially if up and about for 2 hours or more without lying flat and resting."

-"Comprehension difficulties. Inability to follow a conversation. May hear words but be unable to understand their context in a sentence. Cannot focus on more than one activity at a time. May not realize they are being addressed. Looses conversation focus when topic is changed. Is easily distracted from the conversation focus by any environmental stimuli.

-"Memory recall deficits in long and short term memory. Improves with lying down and resting."

-"Abdominal migraines. Severe stomach pain triggered by large meal or sitting or standing too long. Usually resolves if patient lies quietly."

-"Drifting to the right or left when walking."


I just found these to be interesting, as they are all quite specific symptoms that may not have been noted elsewhere. I found them all at the website listed above.

More Doctors

Appointment with Endocrinologist 4/5/2010

I don't quite know how to explain what happened at this appointment. It didn't go well. It felt like an interrogation, actually. First, she asked if my surgeons were satisfied with my recent DEXA scans. I told her that I wasn't sure, but I thought that they wanted me to continue on the medication, since I am still in the range of Osteopenia. She said that this was ridiculous, and with any other patient, we would be celebrating right now. She said that she would not renew my prescription for Forteo any longer, because my bones are strong enough as they are, and no reputable surgeon would require them to be any denser. The interrogation began. She started asking me questions about the reasoning for each of my surgeries and what improvements I saw with each one. Then she questioned why I was having my current surgery. She told me she thought I should consider if I really needed the surgery, and getting a different surgeon. At the very least, she thought I should see a neurosurgeon that she recommends for a second opinion...a neurosurgeon that doesn't even specialize in Chiari, much less cranio-cervical instability and fusions. Before I could even ask her the rest of my questions, she walked out of the appointment, not to return. It was completely condescending and rude. I felt like I'd been slapped across the face. At the same time, I wasn't surprised. I've been through all of this before. It's just been a while.

I did learn one positive thing at this appointment: I asked her if my salt intake was affecting my bones, considering I take salt tablets regularly, so I get more than the average recommended amount. She said that all of my tests showed normal amounts of sodium in 24-hour urine samples, and I don't need to worry about my salt intake at all. YAY!!! My body simply metabolizes much more salt in a day than a normal person, so the salt doesn't leach calcium from my bones. So, as long as I don't take more than I feel I need, I'm fine. I know I was taking 5-6 tablets a day when I had those tests done. So that should be a safe amount for me.


I am not certain if I will return to this endocrinologist. I may need to find a new one to prescribe Forteo, which I know won't be easy, from experience. But I will wait for now.


Appointment with Dr. Grubb 4/14/2010

My appointment with Dr. Grubb was excellent.

He is unhappy for me that I need another surgery, but he is in support of the surgery, and expects that I will see some improvement in POTS symptoms from the surgery. It seems we have come to the understanding that my POTS symptoms will never be cured. But he says that if the surgery helps, then the symptoms will be easier to manage with medications.

Surgery recovery is expected to cause another large setback in POTS symptoms. There is simply nothing to do about that. But if I am struggling a lot, I should consider contacting him. Perhaps to consider IV fluids to get me through the rough time.

Exercise is still Dr. Grubb's number one concern. He suggested a recumbent bike for ongoing cardio exercise. We are looking into buying one used. They are quite expensive. But if we can find a good deal, it may be a possibility. I'm also supposed to walk around the house wearing ankle weights to build up leg strength. We'll see how that goes.

He's changing my dose of Midodrine a bit. We're going to try for higher doses with breakfast and lunch to see if I can be active earlier in the day. Usually, I cannot walk around until after dinner pills.

Finally, he wants me to add a couple of supplements. He wants me to be taking D3 in liquid form instead of solid form. Also, I'm supposed to look for a flavored liquid fish oil supplement. That sounds absolutely disgusting to me, but I guess I could try it. (I'm particularly picky, so I'm really not sure if it will work out...)


Upcoming Appointment with Dr. Bolognese 6/18/2010

I have an appointment set to see Dr. Bolognese in New York. Here is what I know about it:

The surgical committee did NOT deny my ICT (Invasive Cervical Traction) because of bone density. They did not believe there was enough evidence showing I was a good candidate for ICT.

So, protocol states that to qualify for ICT, I need to have a consultation at TCI (The Chiari Institute) with new imaging and new testing showing that I have symptoms of cranio-cervical instability/cranial settling.

After the appointment, the surgical committee will vote on ICT again.

If they determine me to be a candidate, I will have ICT.

If the ICT is positive, the surgical committee will vote on surgery.

If they determine me to be a candidate, I will have the cranio-cervical fusion revision.

It's a TON of red tape, but it's the only way that I will have this surgery. And it's the only way that they can be absolutely certain that I need this surgery, and that this surgery will help me.

So, this is the way it needs to be done.

It's a long, long, long road since that first surgery in 2002.

This time, I'm hoping to recover from surgery in time to have my wedding in the summer of 2011, whether or not I have hair!

Exercise and Doctor's visit

First, I have been exercising regularly (but not quite daily) since March 1st. It has been pure torture, to be honest. I wish I had a gym available to me, but I certainly can't drive to one right now, and I don't belong to a gym, much less one that's open at 10:00 at night, and I don't have a personal valet to drive me to one, anyway. So I'll just have to deal with that. That means that I don't have a recumbent bicycle for cardio (or the seated elliptical trainer from PT). So, I have two options, depending on the day: take a brisk walk around the neighborhood or climb up and down the stairs in my house. I hate the stairs, but it feels like good exercise. My best is up and down 40 flights in about 20 minutes. It hurts my joints and I have to monitor my heart rate carefully. Every time my heart rate surpasses 170 bpm, I sit down. It happens pretty frequently. I break out a good sweat. I can't describe how horrible it feels, but it's what I'm trying right now.

That's cardio. For strength, I do weight lifting with a 5 and a 10 lb. weight, and I do various core exercises. They feel terrible, as well, but what are you gonna do?

Easter week, I was feeling quite a bit better. But I think it was just a fluke. I'm definitely back to where I was, if not worse. I can barely move most of the time. It seems like I save up all of my energy for exercise, and then I suffer for it the rest of the time.

There's a study taking place right now by a research team in Texas on POTS and exercise. It has been accepted for publication in the American Journal of Cardiology. I will link to the findings when they become available. From what I've learned so far, I may be going about this exercise all wrong. This team advises only sitting exercise...no standing, or you will "overtax your system and setback your recovery." Maybe that's what I'm doing right now. They advise the use of a rowing machine or a recumbent bicycle for cardio every other day. No walks! Certainly no stairs. That does sound more manageable. I will have to consider this more. I look forward to the research being published. For more information, read here: http://www.potsrecovery.com/


I have an appointment Wednesday in Toledo with Dr. Grubb to discuss my POTS treatment. I plan to ask him for his advice on exercise, although I know he generally advises leg strengthening the most. I don't have a leg press machine at home, so stairs are probably the best thing I've got, along with ankle weights. (I wish I could still go to the gym!) I don't have any other specific questions, so I will just see if there is anything else the good doctor has to offer at this time.

I have more on which to update, but it will have to wait until after this trip to Ohio.

Surgical Review Board Says....

No to Invasive Cervical Traction at this time.

I'm not surprised, nor am I disappointed, as I did not expect anything at this time. It's too soon, and I know it.

Oh, the wonders of having very low expectations and very little hope (for now).

Dr B, however, wrote to me directly, telling me that I should schedule a follow-up visit with him, now. I really don't understand why I would need to see him right now. He's the neuro-surgeon. I don't qualify for invasive traction or re-fusion at this time. So, why would he want me to fly to New York to meet with him right now? Maybe he's changing my surgical plan? I'm just not sure why I need this appointment right now. This is the waiting time. Waiting for the bones. Then, when I have a better DEXA scan, wouldn't that be a logical time to have a follow-up visit? When my body is ready for surgery?

I'm going to ask my nurse about the details of the visit, to see if she has any answers. But I suppose, if THE man wants to see me, then I probably want to see him, too. I don't have much else to do right now, to be honest. Really quite bored.

Maybe...

I heard from my nurse at TCI, and she said that Dr. B is willing to see me for a follow-up appointment now, meaning that he thinks my bone density has shown enough improvement. However, we need to wait until the surgical review board votes to determine if I am approved for Invasive Cervical Traction. So, I was given the option of going to see Dr. B right away, or waiting until a decision is made on the traction. I decided that for financial reasons, we will wait until a decision is made on the traction, and hopefully do both things on the same trip to New York. Of course if it isn't approved, then the whole trip will be delayed anyway.

That's the update...not much of one. I'll write again when I have actual news, from the surgical committee, since they seem to decide everything now anyway.

Reassessing

As I discussed in the last post, I've needed to reassess my situation, based on the fact that my surgery will most likely not be this spring. (I haven't yet heard anything final from my nurse at TCI or Dr. B...I'll let you know when I do hear something final.) I am determined to not let this discouraging news paralyze me.

So today, I would like to write about what my new plan for the next months of my life will be. Without a plan, it's easy to lose hope. But with it all written out, I can know that I am on track for a determined future.

First, I need to hear from TCI, of course. I need to find out what bone density numbers I should be aiming for, so I can know how soon I should plan on having my next DEXA scan. Also, I'd like to ask them for a prescription for a new CTO (cervical-thoracic-orthosis). I haven't been able to wear mine, ever, because it doesn't fit right. I'd be interested in getting either an Aspen CTO or a custom CTO. This may allow me more upright time during the day until I can have surgery.

Next, I made an appointment to see my endocrinologist. I would like to discuss several things with her. First, I'd like to make sure that she is willing to extend my Forteo treatment until surgery (and for at least 3 months after surgery, to allow adequate bone growth after surgery). I'd also like to find out if she has other recommendations to raise my bone density. I'd love her advice on specific exercises I can do or any dietary changes I can make. I am also curious if there are any other supplements she can recommend or if she would recommend a limit on the amount of salt I take in each day.

Also, I have made an appointment to see my POTS specialist. I need to see him yearly. I would be interested in his ideas on how to manage exercise with the extreme heart rates endured by my body.

I also need to get a new local cardiologist to monitor my bicuspid aortic valve and valve leakage problems. I need a new echocardiogram as well.

Dr. F recommended that I try Myofasical Release. However, I have yet to find any doctor that practices this within the confines of evidence-based medicine, so it is not covered by insurance, and I am not really interested in it at this point in time. I know that physical therapists have other therapies to offer that may be of benefit (like laser therapy). Unfortunately, only myofascial release was recommended at this time.

I have gotten orthotics for my shoes. They are extremely uncomfortable, but supposedly, that means they're working! If they were all squishy and gel-based, they wouldn't be doing anything for my sagging arches. Mine are made of cork and leather, and they are serious business. I think I am gradually getting used to them.

Since I got my bone results at the beginning of March, I have started two things: I've begun nightly exercise, no matter how terrible I feel. I can tell that I am stronger already. I'm following my own exercise plan, based on the equipment I have available, and the ridiculous number of exercises I've learned in my year of private training and multiple stints of physical therapy. But I really have to base the plan on my body's abilities for that day.

I've also reduced my salt intake by 66%. I'm now taking only 2 salt pills a day (instead of 6). It's a challenge. I've added Gatorade, and I think I'll need to add more. I just may chug Gatorade all day. I'm wondering if I can cut the salt pills in half, so I don't get such a megadose of salt. Just a little kick to keep me going. Then, maybe I could take them more frequently and not zone out so much of the day.

Besides that, I also plan to take a Spanish class at the local community college this summer. They offer a blended course (half classroom, half online). That way, you spend half as much time in the classroom. And classes are offered in the evenings. So, it would be a real possibility for me. It might be overdoing it, but I'd really like to do this.

So, that's the plan. I just need to hear from TCI for confirmation.

Dealing with Disappointment

I've been forced to learn a lot about dealing with disappointment in my years of being sick. The most constant disappointment has always been the failings of my body. I had to learn in high school that for some reason, my body couldn't do the things that it should be able to do. I missed out on opportunities that I could have enjoyed. Every day was more challenging than it should be. But I learned to deal. I had no choice but to get through high school, excel in my courses, and move on to college. So that's what I did.

My next major disappointment came with the first surgery, in July 2002. It was an initial success. I felt better than I'd ever felt. So, when I began a quick decline after six weeks, having to eventually drop out of college, that was a major disappointment. But I learned to deal. I needed to remain a student to keep my health insurance. So I enrolled in community college and adjusted my expectations for myself. I also made an appointment at The Chiari Institute (TCI). I had to wait 9 months to go there. In that time, I became quite underweight, and lost most of my ability to function. But I kept pushing on, with a new goal in mind.

At TCI, we made plans for another surgery, a cranio-cervical fusion. I went through hard times. I missed out on a lot of life. But I believed that if I got through this, I would have my life back. So, I kept pushing.

My next major disappointment came after this surgery. Despite intense physical therapy, massage therapy, and a lot of patience, I wasn't getting any better. There was nothing more that could be done for me at the time. And my body was more broken than it had ever been. I was very lost for a while. In the end, I had no choice but to form a new plan and start moving forward again. So, I kept working and found a way to return to my college, in an electric wheelchair, 9 months after surgery. It was a long road, but I worked hard, with a goal in mind, and graduated school as planned. I even began working, part time, after graduation. I started my own piano studio, teaching 10 hours of lessons a week.

Unfortunately, the next disappointment was never far behind. As hard as I tried to pretend that I was fine, it was clear that I was not. My symptoms were unbearable. I hadn't contacted TCI in a while. When I did, they ordered some tests. They decided it was time for my next surgery. Life would need to be put on hold again. In June 2008, I had my spinal cord untethered. During the same trip, I had a life-changing experience: I had invasive cervical traction repeated. This test came back positive. This means that even though I already have my head fused to my spine, I still need more surgery to correct a problem of functional cranial settling in the area. So, I had a new reason to hope. The surgery on my spinal cord didn't seem to help much. I have actually been on a decline since then. I would have begged for them to perform the other surgery that day, but they wouldn't. They won't perform the surgery until my bone density improves.

That has been a new source of disappointment for me: my frustrating bone density. When I was first tested, my bones were practically average; just a little soft. But not strong enough to qualify for surgery. Unfortunately, the first endocrinologist I saw refused to treat me. There were two reasons: he refused to treat a pre-menopausal woman for osteopenia. And, he refused to treat osteopenia that was so mild. When I brought him a note from my neurosurgeon, he refused to see me anymore. He did refer me to another doctor.

The next doctor had similar concerns. The medications used to treat osteoporosis and osteopenia are untested in women my age. So you need to have really bad bones to get approved for them. It was decided that we would start with non-drug treatment. Unfortunately, my next DEXA scan, of course, came back worse than the last. So surgery was looking further and further away. Then, in October 2009, we finally started with a drug treatment (Forteo). Since it is such a strong drug, there was hope that it would show significant improvement after 4 months of treatment, which is when I had my next DEXA scan. Unfortunately, this showed very little improvement. So, we are back to waiting. This may mean postponing my wedding this time. We had actually planned the date based on having surgery sometime around this April. So, we may have to re-think that.

If I've learned anything from all this, it's that there are ways of coping with major life disappointments. For me, I need to stop and grieve over the loss. Then, I need to re-assess the situation. Then, I need to set new goals and time-frames. Then, I will be able to accept my situation once more, and keep moving forward. Of course, I know that disappointment comes in cycles, and acceptance doesn't last. So I don't expect to stay in this part of the cycle.