Dear Friends and Family,

Many of you have asked for an update since I sent a request for prayer regarding my father’s condition and a tentative diagnosis of ALS last November.  (Scroll down below to read a copy of that original email.)

Before I discuss more recent events, I wanted to inform you of what has been going on the last several months.  I had asked for prayer that my father would respond to the treatment for Myasthenia Gravis despite the fact the antibody tests came back negative.  As it turned out, Dad did not respond to the medication and he does not have MG.

Each negative diagnosis, in a sense, leads closer to a diagnosis of Lou Gehrig’s disease because there is no definitive test to confirm ALS.  A final diagnosis of ALS is confirmed only after eliminating all other possibilities.

Despite the elimination of MG as a possibility, my father’s has some symptoms that do not seem consistent with ALS.  For example, Dr. Washington in Atlanta noted that he had weakened motor response in some of his facial muscles that would not be consistent with ALS.  She suggested that Dad might have an extremely rare disease called Lambert-Eaton.  This was a good news/bad news scenario.

Good news first:

1. Pretty much anything is better than ALS
2. It can be definitively diagnosed with a blood test.

Bad news:

1. Lambert Eaton is exceedingly rare so there is very little data associated with treatment options.
2. LE is always associated with a number of usually fatal cancers such as small cell lung cancers.

So now Dad was confronted with the possibility of have a terminal cancer instead of ALS.  The Mayo in Rochester MN is the only place that can test for the Lambert-Eaton antibodies so, after much drama related to having the blood work drawn and waiting for over a month to get the results, the tests came back positive (supposedly).

As a family, we were grateful for a likely diagnosis but concerned that my father had an undiagnosed terminal cancer.  And the doctor’s remained puzzled for two primary reasons:

1. All of Dad’s cancer screens came back negative.
2. He still had symptoms that were not consistent with LE but were consistent with ALS. Most notably, muscle fasciculations (twitches).

One of Dad’s doctors suggested that he was perhaps the unluckiest man in the world and had both ALS and LE!

Through all of this, I think it is fair to say, that the most difficult thing for my father has been not knowing what is happening to him.  He had steeled himself and girded the loins of his mind that he had ALS, and then maybe MG, and then maybe LE but maybe still ALS.  The not knowing has been extremely difficult for him.  I think, too, that the idea of a terminal cancer has been very difficult for him.  He had seen his own grandfather die an agonizing death more than 50 years ago of small cell lung cancer and he feared the pain that he had observed those many years ago.  And against it all is the overwhelming fear of ALS which is so debilitating and can be such a burden on families and caregivers.  My father has worked hard to trust in the Lord and lean not on his own understanding.  But the anxiety and fear of not knowing and what might come with the knowing has been extremely trying at times.

Last week, Mom and Dad and I were able to see an MD at Duke in Durham NC, Dr. Vern Juel, who is one of the few specialists in the world on Lambert-Eaton.  Originally, Dad was scheduled to see Dr. Juel in mid-July which was a problem because my mother is having a knee replacement at the end of June and she would have been in therapy and unable to accompany my dad then.  In addition, if Dad has an undiagnosed malignancy, we thought there was some urgency to getting a firm diagnosis.  Through the persistence of Dad’s primary care physician, he was able to arrange an earlier consultation which happened last Thursday, June 18th.

Here are our thoughts about that visit:

• Dad was very thoroughly attended too at Duke.  He was there for seven hours and was seen the whole time by three physicians who are doing a fellowship in Neurology at Duke.
• He had what was, by far, the most thorough testing and examinations he has had to date.  They completed the same Nerve Conduction studies and EMG studies that had been done by Dad’s local neurologist and by Dr. Washington in Atlanta and in some cases they repeated the tests to be assured of accurate results.
• Near the end of the visit, we met with Dr. Juel to discuss the results of the testing and his review of Dad’s medical records.  WE WERE EXTREMELY IMPRESSED WITH DR. JUEL. He is extremely thorough.  He is extremely methodical.  He listens.  He has a wonderful bedside manner.  He knows what he is doing.  And he seems to be the guy that can give us answers.

This is a summary of his findings:

• Dad does not have Lambert-Eaton syndrome.  (I was amused when Dr. Juel expressed his disappointment at this.  It is such a rare disorder I think he relishes every patient he can find!)  In fact, despite everything we had heard previously, his tests apparently came back negative from the Mayo but were somehow misread along the way.
• ALS is also sometimes called Motor-Neuron Disease (MND). Dad clearly has compromised motor neuron function which would cause the symptoms that he has experienced such as loss of muscle strength, muscle wasting and fasciculations.
• The testing, examinations and review of his medical record indicate that he also has compromised sensory neuron function leading to a loss of sensation in his extremities, especially his feet.
• ALS/MND cannot explain the loss of his sensory neuron function.  ALS only and always affects the Motor Neuron function.
• Dr. Juel seems to think that Dad may have ALS and another disease causing his sensory neuron loss (the unluckiest man in the world theory).  This seems unlikely to Dr. Juel but he can’t rule it out.
• Dr. Juel believes that the more “elegant” and likely answer is that Dad has a disease that is causing both his motor neuron and sensory neuron problems.
• Specifically, (and for the technically minded only) he is looking for a multi-focal, asymetric, demyelinating neuropathy as the likely diagnosis.
• Though rare, such diseases exist and they usually respond well to treatment.  They are also usually not terminal.
• The treatments are not “benign” which I assume means that they can cause significant side effects.
• One example of such a disorder is Lewis Sumner Syndrome.

At the end of his visit at Duke last week, they took spinal fluid and blood samples, in order to run some lab tests that will help them hone in on a diagnosis.  A follow-up visit was scheduled for this Friday, June 26th at 4:00 PM.

Please pray for the following:

• That his medical team would be able to hone in on a final diagnosis soon.
• That Dad would not be anxious and that he would trust in the goodness, mercy and sovereignty of the Lord.
• That his conduct would be Christ-like and a testimony and example of to all those that the Lord brings into his path.
• Please pray for mom’s upcoming knee replacement surgery and for a speedy recovery.
• Dad, above all, still longs to make Christ look beautiful and to see him glorified in his life or in his death.

Previous Update (Originally sent out on November 13, 2008

Dear Praying Friends,

As many of you are aware a couple of months ago, my father, Norville received a tentative diagnosis from a neurologist of Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig’s Disease.  ALS is a degenerative neurosmuscular disorder that results in a gradual loss of muscle use throughout the body and is usually fatal within 3 to 5 years.

Several weeks ago, my dad visited a neurologist in Atlanta who specializes in the diagnosis of Neuromuscular disorders and was given hope that he may have another disorder called Myasthenia Gravis which is treatable and not life threatening.  Myasthenia Gravis can usually be diagnosed with a blood test.  This past Tuesday, mom and dad and I returned to the specialist in Atlanta and were told that the blood tests came back normal which means that the anti-bodies we were hoping to find in the blood were not present which makes a diagnosis of ALS more likely.  The doctor noted though that he still has a couple of symptoms that are more consistent with Myasthenia Gravis. In about 10% of the cases, Myasthenia can be caused by anti-bodies that are not detected in available blood tests.  This gives the neurologist and us some hope that he does have Myasthenia rather than ALS.  However, the most likely diagnosis at this point is ALS.

Under the circumstances, my father is doing remarkably well.  Of course, we are saddened but we are trusting.  My father firmly believes that God is good, that he is sovereign, that He orders our days and that He loves us with an incomprehensible, everlasting love.  Dad wants to see Jesus Christ glorified in whatever God has before him.  Of course, we are praying that God would choose to glorify Himself through some other means such as healing.  We believe that God can do this, so we pray.

What you can specifically pray for now:

• That over the next few weeks that Dad would respond well to the medication he is now on.  This would be an indication that he has Myasthenia.
• Pray specifically that Dad has Myasthenia Gravis.
• Pray for healing.
• That God’s strength would be manifested in Dad’s weakness.
• Pray for the joy that surpasses understanding.
• Pray that the body of Christ would respond with compassion, love and service providing a testimony to the reality of the gospel and our faith.
• Pray, above all else, that God would be supremely glorified.

Thank you for your love and support. We covet your prayers.

Steve

Seth