In 2007 I was diagnosed with Alpha Antitrypsin Deficiency phenotype MZ. It is quite rare, one in two thousand and the doctor had never had a patient diagnosed with it. My other family members didn't believe it but upon testing we found out my sibling and one of my parents has the same disease. This is a hereditary disease causing a protein deficiency and in my case affects my liver. I carry only one defect so I am considered a carrier. I let my son know as soon as possible. If he has the disease and marries someone with the disease there is a one in four chance a child would be born with two defects (SS, SZ, ZS or ZZ) and would likely need a liver transplant before the age of five. Needless to say, most of these children die.

The doctor read up on the disease and blessed one or two drinks a day as "Perfect" but I should avoid smoke of any kind. I never liked to be responsible for a campfire anyway.

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APNEA

2010 May 27 Steve was prescribed CPAP to treat sleep APNEA. If you have recently been prescribed CPAP or another treatment for snoring or other forms of APNEA start here:

Equipment:
Resmed S9 AutoSet CPAP 4-20 (since May 2010)
Not using S9 Humidaire H5i Heated Humidifier w/Climate Control
I use both of these alternating to let my face and nose heal:
ResMed Swift LT Med. Nasal Pillows W/Headgear
Respironics ComfortFusion Sm. Nasal Mask W/Headgear
Not using ComfortClassic Sm. Nasal CPAP Mask W/Headgear
ResScan 3.11
Respironics EverFlo O2 Concentrator
CMS50E Oximeter & Software


The story (so far):
Patient’s Perspective:

In 2010 Feb. an allergist diagnosed me with Obstructive Sleep Apnea and scheduled me for a sleep study. Based on confirmation of the diagnosis he prescribed CPAP. I reviewed the study report and some things did not add up. I didn’t think the test was very scientific and I didn’t feel like I was getting a “normal” sleep. Prior to applying CPAP I had an:

AHI/hr: 73.8
Avg. SaO2: 88%
Min. SaO2: 79%


The tech. Applied 5.0-16.0cmH2O and I had:

AHI/hr: 81.4
Avg. SaO2: 92%
Min. SaO2: 80%


When I showed up to take possession of the CPAP machine I told the representative that I did not want to take the machine until I got another opinion. My minimum O2 was still below the warning limit (89-91 depending on your insurance), did not improve significantly and my AHI (events) got worse with CPAP therapy! The representative said it probably indicated central apnea and said, “Good catch!” We marched downstairs to the doctor’s office and he said that my O2 levels were low because I was so obese that I can’t breath properly lying down. I am 6’ 220lbs and used to bodybuild (bodybuilding invalidates traditional BMI charts), that makes me only about 20lbs overweight. The doctor also runs a weight loss clinic, what a scam. I officially fired the doctor with a letter for; incompetence on this, incompetent allergy testing and rude, unprofessional comments. My General Practice physician referred me to a Pulmonologist.

Armed with a copy of the sleep study report I explained the situation to the Pulmonologist and my issue with the report versus the diagnosis. He diagnosed it as CPAP induced central apnea due to the pressures they took me up to, central apnea events started at pressures above 10cmH2O. Taking me up to 16cmH2O, “They tried to turn you into the Michelin Man,” he laughed. I liked this new doctor. He prescribed CPAP and I got the S9 AutoSet through the original sleep lab representative, as it seemed the right thing to do. Meanwhile, this sleep lab representative to whom I had pointed out the discrepancy asked an equipment dealer friend of his to do a free overnight oximetry. I did the study at home and based on the results he talked my General Practice Physician into signing a prescription for O2 titration. I received a machine, delivered to my home and started using it. On my next visit I shared this information and the O2 study with the Pulmonologist and he did not think that I needed O2 and said to send it back. So, the equipment dealer had me do another overnight oximetry and ran a report to take to the Pulmonologist. The difference was obvious to me, on CPAP alone my:

Basal SpO2: 90.1
Min. SpO2: 77.0


On CPAP with 2LPM O2 my:

Basal SpO2: 95.3
Min. SpO2: 89.0


I am no expert but the data doesn’t lie. I bought an oximeter, paid too much for it at a local Medicine Shoppe, and tried to confirm O2 levels each time I woke up in the night. This is almost impossible to do but I remember seeing some low numbers so I confirmed in my own mind that the equipment dealer wasn’t falsifying the O2 reports. I took these reports to the Pulmonologist on my next visit. He glanced at the reports and shrugged it off. I paraphrased some Buddha, “Never believe anything … unless it agrees with your own reason.” I respectfully disagreed, so he took another look at the reports. He said it didn’t make sense to him and that I had to do another sleep study in his lab. Before CPAP was applied:

AHI/hr: 33.6
Avg. SaO2: 89%
Min. SaO2: 80%


At 12cmH2O central apneas appeared. At 7cmH2O I went into Deep Sleep:

AHI/hr: 0.0
Avg. SaO2: 94%
Min. SaO2: 93%


The Pulmonologist diagnosed CPAP induced central apnea, continued use of the AutoSet CPAP and no oxygen needed. This makes sense, except, what about the O2 study I did at home (min. 77%) on AutoSet CPAP? His answer was that it takes a while for the body’s CO2 sensor to adapt and the oximetery tests were done too soon. Maybe, but I would need another oximetery test to verify.

My AutoSet pressure usually runs a median of 4.5cmH2O with a Max. of 7.3cmH2O, AHI 1.2 so the low O2 readings do not appear to be caused by obstructive or central apnea (the S9 AutoSet detects both). The doctor says I don’t need the O2 but the data seems to indicate that I do. Time is probably running out to return the O2 Concentrator. The sleep study is diagnostic in nature and I was only at the ideal pressure, 7cmH2O, for 35.4 minutes. This is why I think the sleep lab is not a good diagnostic tool. I am uncomfortable and am not sleeping normally, they are changing the parameters too often to catch significant events that may only happen a few times a night, and you can not take a measurement without affecting the measurement. At this point, if I wanted to figure out the mystery, I was on my own. I bought the CMS50E which is a data recording oximeter with software and proceeded to perform an all night oximetry test the first night after I got it. Without reading the instructions. Frustrated that there was no data recorded, the next day I read the instructions and set the device to record the next night. Event/hr is as reported by the oximeter.

Date _______________ 2010 Oct. 2-3 2010 Oct. 3-4 2010 Oct. 2010 Oct.

CPAP ___________ off on on off

O2 _________ off off on on

Adj. Events/hr _______________ 38.4 20.0 2.3 8.6

Basal SpO2 _______________ 89.0% 93.0 96.0 95.0

Min. SpO2 _______________ 68.0% 74.0 67.0 79.0

Time(min)<88% _______________ 146.2 4.7 0.6 1.8

Note: I didn’t sleep from approx. 1-3am Oct. 3-4, maybe I’ll redo this one.

I laid these reports out and asked my wife to pick one. The best is obviously both CPAP and O2 (the 67 min O2 was a single outlier in the data), if I had to give up one therapy it should be CPAP and keep the O2.

Conclusions:
CPAP is not a scam. It not only helps eliminate obstructive apnea, the open airway test on the S9 tends to wake me up like an alarm during a central apnea. I think that is probably an unintended/undocumented feature. After seeing scientific results I finally accepted the therapy mentally. That epiphany seemed to make a differance and how I felt and my health seemed to "turn a corner."

Every CPAP user should periodically check their treatment effectiveness with an overnight oximetry study.

If Apnea isn’t causing all of my O2 events, some unknown/undiagnosed condition is.

“Never believe anything you hear or read, no matter who said it, not even if I have said it, unless it agrees with your own reason.” – The Buddha

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Diabetes (II)

Still to do...

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PVCs

Steve has been having physiological (not emotional) anxiety attacks for years and was diagnosed with "harmless" PVCs (Premature Ventricular Contractions) during an EKG prior to the neck surgery.

Because of the controversy over oxygen with the Pulmonologist I bought a recording, overnight capable oximeter.

One night the oximeter (CMS50E) recorded an episode of the heart behavior and I showed it to my GP doctor. The Pulse Rate got over 175 and the average was 106.6 for the five hours it caught.




Steve did what he usually does when he can't sleep, got up and watched CNBC. He felt okay other than normal concern when your heart is acting weird. In the past we have verified what Steve was feeling with a stethoscope we keep around for that reason, so it is legit. Heart rate and strength is very erratic. Blood pressure this am was 113/72 (Steve is usually ~130/90 and on Diovan HCT 320mg/12.5mg for it).

Doc. said, "I don't know about that stuff and I don't want to know about that stuff." and referred Steve to his Pulmonologist.

The oximeter displays a wave form but doesn't record it. The computer can display a live waveform when the oximeter is connected via USB or bluetooth so during another attack I managed to take a couple of screen snapshots:




I asked my pain management doctor for his opinion. I realize that his education is in anesthesiology but all I wanted to know, is it an emergency or can it wait for my next appointment with the pulmonologist. He said it was not an emergency and he felt it could wait. I will update upon news or a referral from the pulmonologist in November.

http://oximetersonline.com/

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Separated Shoulder(s)

In 1997 I separated my right shoulder shooting prone position. I didn't know it at the time, I just thought it was a big bruise (from the top of my shoulder to below my right pec!) The surgeon said that I would need surgery eventually, but, to live with it as long as I could. So I live with it. The separation itself causes no pain to speak of but there is also a pinched nerve that inhibits my range of motion.

My left shoulder gives me more problems and more pain but has never been diagnosed. I think I have filled my quota for surgeries so a few things haven't been presented to a doctor.

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Spine (DDD)

One of my hobbies and my preferred health regime was Body Building. This included running and lifting weights. I didn't have the genetics to be a professional body builder, but I loved the healthy lifestyle. I started lifting weights in 1980 and got more serious about it in 1983.

I suffered a ruptured disk L4-L5 (lower back) in 1987 due to Degenerative Disk Disease. In 1989, when I could no longer sit in a car I finally had surgery to relieve the pain. After many months of recovery my doctor cleared me to continue working out so I faithfully continued what I considered a healthy lifestyle. My ex-father-in-law, Mike disagreed. Seeing one of my workouts at home he commented, "You are going to wind up in a wheel chair." Perhaps he had the gift of prophesy.

In 2001 I was diagnosed with another ruptured disk at C6-7 (neck) causing complete loss of strength in my right arm and numbness in my fingers. The two vertebrae were drilled and fused with a cadaver bone and a titanium plate was screwed in place to fix the problem in May 2002, called an "Anterior Discectomy and Fusion."


My head is screwed on right!



At the time my doctor recommended, and I still chose, to actively exercise, if I didn't - my accumulated injuries would have caused me to stiffen up pretty bad. Body building has never of itself caused me any injury that I know of and I could point out several times it has prevented injuries through increased strength and flexibility.

Sadly L4-L5 herniated again some time after the original surgery and has caused some long term problems. In 2006 I had to give up lifting weights and in 2008 the problem was finally diagnosed. In February I submitted to the necessary surgery.


The damage the doctor found was the result of a long term compression of the spinal chord.

Bummer. I felt great while I was in the hospital, logically with all that pressure removed, and I started getting hopeful that some of my long term numbness might miraculously heal. After being home for only a few days my right foot went dead. I mean cold and I couldn't move it at all. They call this "drop foot paralysis." The doctor took one look at me in the follow-up and said, "MRI, now! We'll get you in." They did, in less than 45 minutes! He called me the next day and said to report to the Emergency Room the next morning and have them page him. The disk had re-herniated again! This time there was no question but to fuse the vertebrae. Both the Orthopedic and a Neurosurgeon (the same one that worked on my neck) worked on my back this time. They found a couple pieces of vertebra growth plate in the disk material this time and he said that the vertebra was "falling apart."




Two rods and four more screws.

To add injury to insult, the wound developed an infection. I had to report to the ER again and the doctor on call, after encouragement over the phone from my surgeon (they don't like to mess around spinal cords), literally stabbed me in the back! He installed packing material to let it drain which we had to change at home for months and antibiotics. I then had to see a disease specialist who started treating me for Mersa (an antibiotic resistant strain of strep I think) just to be safe. Once the test came back as regular strep he admitted me to the hospital and they installed a PICC line so that I could give myself IV antibiotics for six weeks. One of our vendors saw me programming some equipment with my IV hanging from some shelves and commented, "Boy, if you don't get Employee-of-the-Year no one should!"

Antibiotic Supplies	Wound Care Supplies	PICC Line

The full surgery and antibiotic epic can be read in the 2008 Weblog.

I was about six months into the recovery from this last (I hoped!) surgery but I was not feeling well. I still had very little strength in my right foot and constant pins & needles and sometimes it feels like someone is pouring ice water down my leg. The doctor diagnosed Arachnoiditis, an irritation of the tissue surrounding the spinal cord. The condition causes "clumping" of the nerves in turn causing neuropathy; loss of function, numbness, sympathetic pain and hyper-sensitivity. On another follow-up he said that Arachnoiditis can either heal after a few months or turn out to be permanent. He 'regretted' to tell me that since it had already been several months it put it in the permanent category.

On a good note, Arachnoiditis was a ticket to guaranteed qualification for both our Long Term Disability insurance at work and Social Security Disability. I tried desperately to continue working for several months but soon realized I couldn't do it any more. I don't want to be disabled but it is relieving to know I can still have a source of income and not starve! Well, of course most people know applying for disability is not simple. I worked my last day Saturday, 2008 November 15 (yes, a Saturday, I was pushing myself hard on a difficult vision project).

Applying and receiving the Long Term Disability insurance from Hartford was not too complicated but when I got my Social Security retroactive pay I would owe that back to Hartford. A friend of mine helped me apply for Social Security but we failed to get the information they needed to them and they rejected the application. My friend filed an appeal but under the wrong Social Security number. I decided to start over with a local disability attorney in California. He new someone at the SS office and could ram it through in a couple weeks. After several months of constant badgering he made the excuse that the personnel at SS had changed and started a new application online. The SS office tried to work with him but he failed to return their calls and they refused to work with him. I did finally get the award more than two full years after I had to stop working. It is not enough to live off of so I keep looking for investments with which to supplement my income.

Time will tell what I will be able to do to stay active. For now I try to walk around the RV park when I am feeling up to it. Thanksgiving 2009 we got Wii Fit with the balance board. There are a lot of exercises I can't do but it is safer than going out by myself to walk. Anyone in a similar situation might look into this option.




One of the requirements of disability is to be under the care of a physician. The attorney referred me to both a Neurologist and an Orthopaedic Surgeon. The consensus was that I was inoperable due to the Arachnoiditis and that my only option at this point was Pain Management. One of the doctors referred me to a good Pain Management doctor.

When he saw my medical issues and how little pain medication I use he called me his "model patient". He was the first doctor to diagnose my foot and leg being physically cold was Reflex Sympathetic Dystrophy (RSD) Besides pain medication the doctor felt I was a perfect candidate for Medtronic Neurostimulation Therapy. He first did a nerve block to simulate the expected result to confirm if I would benefit from the therapy.


Medtronic Neurostimulator Trial Unit Leads


No shower for a week!



The trial provided significant benefit. In order to get the implant the insurance required a psychological evaluation which I must have passed because it was eventually approved. The unit was implanted where the electrodes run along my sympathetic nervous system and the computer/amplifier/battery unit was implanted in my right butt cheek (which is mostly numb anyway). If you are interested in the details I recorded my experience in our 2010 Weblog.


For all of you that secretly wanted a glimpse of Steve's arse, here is some of the bruising. Tunnelers are brutal!


It is hard for people to understand that while drugs and the stimulator help deal with the pain, I am still in significant pain all the time and there are some things I just can't do. Even people I consider close friends say things like "suck it up" or argue when I tell them I can not sit on an airplane for any amount of time. In all fairness I do not share all of my problems with others so I guess I should expect them to treat me like I am some kind of wimp when I can't do something.

For anyone in a similar situation considering this kind of therapy I can tell you that the Medtronic clinician has set up several programs and one of them is quite effective at interrupting the pin & needles and many of the other symptoms, but only in my legs and lower back. They cannot use it for the pain in my upper back, shoulders and neck. Sometimes my pain is so significant that I have to turn up the stimulator amplitude. For me 4.00 out of 10 is high. I can turn it up (and Marissa has done so several times) high enough to go into body spasms like convulsions. If that can't interrupt the pain nothing can! Marissa (the Medtronic clinician or tech) has been changing the programs every three months to try and keep coverage of my lower back and the tips of my toes, but also so I don't get too used to the pleasant buzzing feeling such that it becomes ineffective.

Unfortunately, if you just want to 'try it out' they have to stick electrodes into your spinal area adjacent to your sympathetic nervous system. If you are a candidate for the therapy consider it might be worth the extra surgeries. It has been worth it for me so far and I would not want to give up that relief! Also keep in mind that the batteries will have to be replaced periodically so expect a surgery every nine years or so.

One major concern was the warning in the brochure about interference from electromagnetic energy sources. During the trial I submitted my self to the BART (electric mass transit system in San Francisco), escalators, elevators, HAM radio and any other source I could think of with no problems. I do take the precaution to let people around me know how to turn it off in an emergency. So far I have only set off one RFID anti-theft device at Blockbuster Video.

As mentioned before I have Reflex Sympathetic Dystrophy (RSD) in my right leg and the end-game of this issue is atrophy and the limb wasting away. I got an idea and presented it to the doctor. I have an Empi TENS unit for pain but have been also using it to stimulate my paralyzed muscles. That is not it's intended use and the manufacturer warns not to use it on numb areas (I think to prevent electrical burns). I was an Electrical Engineer and I understand the risks of introducing electrical impulses to the body.

So I asked Dr. Longton if he thought my leg would atrophy. He answered that he did not know. Because the RSD is caused by a problem in my spine and not my leg he wasn't sure. Logic says that if you can't exercise a muscle it will atrophy so I asked him if an Electro Muscular Stimulator (EMS) would help prevent possible atrophy. He said it was a great idea and couldn't hurt and wrote me a prescription.

I started using the TONE-A-MATIC® TDR-68 in May 2011. After using the EMS for a few weeks I have some thoughts to share. Whatever unit you choose call them an ask how many milliamps it can deliver. I had trouble getting the paralyzed calf to contract (quads worked fine). I went up to over 700mA on the right calf and got them to fire. My unit came with both self-sticking pads and the dry reusable pads and I use them both. The kit came with a sponge to wet the dry pads as they don't work unless slightly damp. I added a dash of salt to better conduct and not burn as easily. To use these they must be on a body part which you can wrap with a strap to hold the pads in place. You will need ace bandages or something if it doesn't come with straps. I use these on my legs and arms.

The self-sticking pads are the same ones that I use with my TENS unit and so I can get them from Empi and my insurance will pay for it. These are handy for areas that are difficult to wrap or if you are having an assistant place them. I use these on my stomach, chest and back.

I am keeping a body measurement log and will pay special attention to my calves. I update this log on or after the 15th of each month.

Last June, user Scott54321 in the Apperelysed Incomplete SCI forum pointed out the WalkAide® device for Foot-drop Syndrome. It sends a signal directly to the peroneal nerve to activate the muscle to lift the foot up and out during the users walking stride. During the first trial it worked immediately and effectively for me! Sadly, Medicare nor Blue Cross would cover the $5,000 device unless the "SCI is diagnosed above a certain level" (not lumbar). The clinician thought my drop-foot was more likely caused by my cervical injuries but that may not convince my doctor or the insurance company. I still decided to purchase the device at 0% interest for 18 months.

My pain management doctor agreed to write the prescription and mentioned that I was probably one of the few patients he would trust to review the product though he has a lot of patients with drop-foot. Below is my review so far:

" During the initial WalkAide® trial it worked immediately and effectively for me causing me to get very excited to take a unit home with me. To start they limit the user to one hour a day of use but ramp up that amount as the user regains some strength and balance. For the first two weeks I have noticed that if I walk slow I can walk without a limp and the clinician indicated they would speed up the program on follow-up appointments. Another major thing I noticed is that I no longer need to watch the terrain in front of my feet when I walk.

It is important to note that the device does not "push-off" and does not increase strength in the muscles in such a way that the user can catch themselves if they loose balance or trip on something. While I no longer feel I need a cane to walk it is still a good idea to prevent a fall. The device mainly eliminates the need for an AFO or foot brace.

The electrical signal to the peroneal nerve is transmitted through pads similar to TENS and EMS units. Some individuals will likely find the shock uncomfortable depending on what level they need to function properly. I feel the signal quite distinctly despite much of my calf being numb. In my particular case, I use my EMS unit regularly and am used to the sensation.







"

I will continue to add to this review as I gain experience with the device.

Both my knees are in bad shape and I occasionally need to wear knee braces when I go out or wrap a knee at home. I can see no way to do this with the WalkAide in place.



http://www.neurologychannel.com/rsd/symptoms.shtml
http://www.spine-health.com/conditions/back-pain/bone-spurs-osteophytes-and-back-pain
http://www.bonati.com/
http://sitincomfort.com/cetrnecopr.html
Steve's Body Measurement Log


    

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Exercise for the Disabled

No matter to what level you are injured or your long term prognosis a very important part of your health is to stay positive. And part of staying positive can include exercise. I have found it very difficult to get any meaningful exercise. Some of these ideas can be used despite total immobility with the help of a care giver. But please, please, please ask your doctor if you could benefit from any ideas before you try them!

Here are a couple ways I deal with my struggle. I decoupled how I am from how I feel. When someone asks how I am or how I am doing I nearly invariably reply "Great!" If they ask how I feel I don't lie but most people don't ask that. It really helps me and the people around me to stay positive.

Despite the prognosis that my disability is permanent people will ask if I am getting better. It helps to keep in mind that they mean well by asking. Another annoying question for me is "How did this injury happen?" and to help deal with it I imagine The Simpsons episode where the Doctor asks Marge if Homer had ever hit his head. She spends the next several minutes of the show remembering all the scenes in which Homer hits his head, doh! It makes me laugh. I led an active lifestyle and have fallen countless times.

Thanksgiving 2009 we got the Nintendo Wii Fit with the balance board. I can still walk but not well. With the balance board I can work out and improve my balance in the security of my home and within reach of support if I loose my balance. I especially like the bike riding and the obstacle course but sadly both my knees are also bad (undiagnosed) so I go for months without being able to use it. If you can stand even a little bit maybe ask your doctor if you could benefit from the exercise.

If your weight is a problem from lack of exercise your body does not need the energy found in carbohydrates so ask your doctor if you can benefit from a low-carb diet like Atkins or Paleo.

As mentioned before I have Reflex Sympathetic Dystrophy (RSD) in my right leg and the end-game of this issue is atrophy and the limb wasting away. I got an idea and presented it to the doctor to use an Electro Muscular Stimulator (EMS) unit to prevent the possible atrophy. He though it was a great idea, that it couldn't hurt and wrote me a prescription. I have just started using the TONE-A-MATIC® TDR-68 so it is too early to comment on its effectiveness but I noticed it doesn't fire the paralyzed calf muscles though it fires the quads with no problem. I will ask the doctor about that when I see him in July. Regardless of your immobility this might be an option but likewise you will need some help placing the pads in awkward locations.

    

As wierd as you may feel about them I am glad I purchased a pair of the Vibram Five Fingers. Despite offering no support I walk much better in them. I am not sure why, maybe because they are lighter and don't fatigue the few muscle fibers I can use due to drop-foot syndrome. I imagine they are good therapy for me at relearning balance without having the normal use of the muscles.



Another feeling we all must deal with is feeling worthless. Some of us held jobs before our injuries and now struggle just to live, let alone work. One of my hobbies was investing. I have found that I can still do that. I can invest in and trade equities in the stock market using an online broker. It helps me feel useful. My wife may bring home a paycheck but it is still up to me to make us rich! :) Also, if you have rental real estate, using a property manager means that you can have several income producing properties without extraordinary efforts. Don't give up, use any resources you have to acquire assets instead of frivolous liabilities.

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