Jean Angells' Fascinating Story

Here's a link to the story behind the Angell family's battle with ALS.

EMBRACING THE NEW NORMAL:
Jean Angell Speaks from Her Eye-Response Computer

Packard Center board member Jean Angell has lived with ALS for 10 years, the last six on a vent. What follows is the complete interview on which the story about her on the back page of the Winter 2008 ALS Alert is based. Read more!

Retired General Plans Attack on Lou Gehrig's Disease

Hat tip to the Times and Democrat for this imspiring article.

CHARLESTON -- Four and a half years have passed since Tom Mikolajcik was diagnosed with Lou Gehrig's disease. Seventy percent of people with amyotrophic lateral sclerosis, or ALS, die within five years. Time is not on his side.

Now, Mikolajcik must make decisions about how he will face the last stages of the disease before he loses the ability to do so.

The degenerative disease, which is known for killing New York Yankees first baseman Gehrig, strikes about 15 Americans daily, shutting down nerve cells responsible for movement. Limbs weaken and atrophy before paralysis spreads to the trunk of the body. Eventually, speaking, breathing and eating are affected.

Patients must decide if they want to go on a ventilator and feeding tube to hold off the inevitable a little longer.

"Today, my decision is I will put in a feeding tube even before I need it," Mikolajcik said. "Today, my feeling is I want to go on a ventilator as long as I can communicate with family and friends."

The retired Air Force general and former commander of Charleston Air Force Base is taking charge of these critical decisions by participating in a medical study testing diaphragm-pacing stimulators in ALS patients. Located below the lungs, the diaphragm is the large muscle used for respiration.

The pacing device stimulates the diaphragm with surgically implanted electrodes to maintain muscle mass. The stimulator, already used in people with spinal cord injuries, might delay the need for a ventilator by more than a year.

During the surgery, a feeding tube will also be inserted, although Mikolajcik does not yet need one. "The sooner you have the procedure the better," he said.

Dr. Raymond Onders, director of minimally invasive surgery at the Medical University Hospital's Case Medical Center in Cleveland, pioneered the technology and the procedure. The late actor Christopher Reeve, who suffered from a spinal cord injury, was Onders' second patient to receive a stimulator.

ALS is a fatal disease, but theoretically, people could live indefinitely with a tracheotomy and ventilator. But most don't want to do that, Onders said.

Doctors can predict when ALS patients will die based on their rate of decline in respiratory function.

To measure the success of the stimulator, Onders looks for decreases in that rate. "It's not a cure," he said.

Mikolajcik was successfully fitted with a stimulator last week in Cleveland. He is part of a 100-person trial in six U.S. sites. Onders previously completed a safety trial implanting the device in 16 ALS patients whose breathing function decline slowed, delaying the need for a ventilator by more than a year.

"I want to be able to listen, watch and absorb my children and grandchildren as they grow and change," Mikolajcik said. The mind and senses remain unaffected by the disease. But as time passes and the body shuts down, the ability to communicate diminishes.

Toward the end of the disease, some people use their eyes, looking right or left to signal "yes" or "no." In preparation for the time he will become speechless, Mikolajcik recorded himself singing "A Bushel and A Peck" to be played when his grandchildren are placed in his lap.

In August 2003, the retired general went to the doctor with a minor complaint: He was feeling tired and not hitting his golf balls as far, he said. The doctor noticed a slight twitching in Mikolajcik's chest called fasciculation.

The doctor told him the best case scenario was a benign tic, and the worst case was ALS. Mikolajcik went home and Googled ALS.

"I almost fell out of my chair," he said. The muscles in his arms shut down first, then his legs. He can move his left thumb and index finger, and if he concentrates and is well rested, he can move his left wrist and ankles. Little is known about ALS, which was discovered in 1869.

"In 70 years, there's only one questionable drug that may extend life by three years," Mikolajcik said. "In 70 years. Give me a break."

For unknown reasons, veterans have a 60 percent higher chance of developing ALS. That high rate is why Mikolajcik said he feels strongly the government has a higher responsibility to advance ALS research.

Legislation to establish an ALS database that will warehouse information on the disease for scientists and patients has passed the House and is awaiting consideration by the Senate.

"I am blessed that I'm a Type A personality," Mikolajcik said. "What about those who take no for an answer?"

Read more!

Reflections on a Job-esque Week

From Jim Jordan's website - November 4, 2007

I went to see one of my best friends in the hospital this week. He's struggling with Lou Gehrig's disease (ALS). Two weeks ago he passed out and fell in the same hospital while they were doing work on his wife's broken foot. Their travails have been horrible lately. On the way in I called his wife to see if they needed anything. It turns out that she needed someone to be there to receive my friend from his therapy session because her car had just been broad-sided by a delivery truck.

While I waited for him to be wheeled down I looked around for something to read and saw their Bible sitting by the window. It didn't take me but a second to decide what I wanted to review, I picked the book of Job. You know the story, Job was wealthy and happy, blessed in every way, until Satan made a deal with God that Job would be tested. Everything goes wrong in Job's life. His children are killed in a windstorm and he loses his livestock. Job's wife and friends give their two cents on what God was trying to tell Job by these terrible events.

There were two new things that stood out even though I'd read this story numerous times. The first thing I noticed was reading God's response to all the ponderings ("words without knowledge") of the humans in chapters 38 and 39. He uses nature to make His case. It becomes plainly clear that the idea that Job and friends would even try to "figure out" the Creator's purpose is absurd. In chapter 42, Job confesses his sin. This is the way The Message puts it:



Job answered God: "I'm convinced: You can do anything and everything.
Nothing and no one can upset your plans.
You asked, 'Who is this muddying the water,
ignorantly confusing the issue, second-guessing my purposes?'
I admit it. I was the one. I babbled on about things far beyond me,
made small talk about wonders way over my head.
You told me, 'Listen, and let me do the talking.
Let me ask the questions. You give the answers.'
I admit I once lived by rumors of you;
now I have it all firsthand—from my own eyes and ears!
I'm sorry—forgive me. I'll never do that again, I promise!
I'll never again live on crusts of hearsay, crumbs of rumor."



Job didn't know what he was talking about. And neither do we. There are times when God annoys me, honestly. But that is not a hindrance to my undying love for Him. Job was right to ask "Why me?" And many times I see the stupid things that happen to me and ponder the incredible turn of bad luck that my friend has had. Both my friend and I have grown closer to God in recent years and yet our fortunes have been ransacked by a superhuman assault during the same time. Thanks be to God? Do we blame Him? Do we accuse Him?

The second nugget I had overlooked in the book of Job was the name "Satan" in Hebrew. It means "accuser" or "adversary". It is a logical distinction for the prince of darkness but it speaks volumes to us. Do we desire to be the accuser, the adversary of God? Will our discontent, as rational as it is, become fertile ground from which we oppose our God? The answer is "no"!

A few days after my friend fell in the hallway at the hospital, passing out and falling dead weight onto the tile floor, his left eye was still bulging out of the socket, green, red, and black, I went to visit. I arrived just as the chaplain showed up to the room. Seeing he had a visitor she said she was praying for him and would come back later.

My friend sat crumpled in his wheelchair, swollen eye and broken shoulder, withered arms at his side, he leaned forward slightly and spoke softly. "God...is...good." He nodded his head for emphasis and then repeated the statement.

What a testimony! It's easy to blame God, to become His adversary because of the crappy hand you've been dealt. By all our human standards His timing is awful and His blessing of our fidelity is often nil. But there is nothing more rewarding than simply being at peace with your Creator. He is good. He is awesome. He is perfect. Did we not always know that?

Heavenly Father, You are almighty and all-knowing, therefore I abhor myself and repent in dust and ashes. Though you lead me to the cross, breaking down each of my muscles and limbs, even though you slay me, yet will I trust in you. For you are all there is, all there ever was, and all that will ever be. I stand in awe. I can do no other than worship you forever.

Give my friend strength in his journey and bless his wonderful and devoted wife with your peace that goes beyond understanding. Indeed "beyond understanding" is where you are taking us. In Jesus' name I pray, amen. Read more!

"She's Dying. His drug could save her"

From The Hook.

Mary Jane Gentry is going to die, and the UVA Health Sciences
Center, which has saved countless lives, has pulled away the experimental drug
that might save her.
"It was a ray of hope," she says, "and then they
stopped it."
When she was diagnosed 18 months ago with an aggressive case of
"Lou Gehrig's disease," Gentry knew it was a death sentence. Doctors told her
she had less than three years to live.
Desperate, Gentry-- herself a nurse
at UVA-- agreed to participate in a novel drug study. After eight weeks, she was
thrilled by a sign that the disease not only seemed to have slowed, but might
actually be reversing. She could suddenly move her left hand, which had been
useless for several months.
And her experience wasn't isolated: nearly half
of the study patients reported noticeable improvements in their condition while
none reported side effects.
So why did UVA halt the study?

Click on the link for the full story. Read more!

Letter from Johnny and Debbie

January 27, 2008

To Whom It May Concern:

Hello, my name is Debbie Miller. The information below will describe ALS (commonly referred to as Lou Gehrig’s disease), and the importance of proper treatment to help manage the symptoms of ALS. This letter is written in hope to raise your awareness of ALS and to assist me in providing funding for my husband, John Miller’s care so that I can fulfill his wished that he be cared for at his home.

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. The progressive degeneration of the motor neurons in ALS eventually leads to their death. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively affected, patients in the later stages of the disease may become totally paralyzed.

As the weakening and paralysis continue to spread to the muscles of the trunk of the body the disease, eventually affects speech, swallowing, chewing and breathing. When the breathing muscles become affected, ultimately, the patient will need permanent respiratory support in order to survive.

John has reached a point where he requires the use of a ventilator which he has. John was diagnosed with ALS in February of 2005. He had an injury in October because of "drop-foot", causing him to fall and fracture an orbit, shoulder, wrist and hand and suffered a hematoma on the left side of the skull. He was recovering well after a 32-day stay in the hospital. He was home for a period of 10 days when he suffered a heart attack on November 26th. Since the heart attack, he has required the use of the ventilator and now we are trying to wean him off of that.

While there is no cure for amyotrophic lateral sclerosis (ALS), treatment and appropriate care can help maintain John’s quality of life, manage his symptoms, and avoid complications for as long as possible. ALS has placed extreme physical, emotional and financial burdens on me and my family. Six months after John's diagnosis, I was forced to give up my full time career to care for him myself 24/7. Being his only caregiver for this long I have helped with some expenses, and the last year's medical demands have consumed all of my savings and nearly all of his. My daughter Shannon ( John's Step Daughter 16yrs. ) is too young to help financially and my son Ryan ( John's Step son 26yrs ) is maxed also financially from doing his best to help and lives in Tampa with his wife, Lisa. They cannot leave their full time careers to come to Fort Lauderdale to help on a permanent basis. John's birth family lives out of state and has not availed themselves to help. Granting me this financial support will aid in relieving stress and caregiver burnout by providing relief and enabling proper care for my husband. This is crucial for maintaining a productive and healthy lifestyle.

John has been blessed to have the option to choose to utilize technology to assist him in living beyond respiratory failure on a ventilator. Along with this right we have the responsibility to plan for the cost of care because neither the federal and state government nor community resources cover the cost of the care of persons on invasive ventilation. Nursing homes in Florida seldom provide long term care to adults with tracheotomies on a ventilator. With Medicare covering very few days in the facility, I am required to pay the rest out of pocket, in which I simply do not have enough funds to do so. As I have promised my husband that I will bring him home, upon discharge I will still need to plan to have several family members trained to provide care at home or I will need to plan to finance the cost of nursing care at home, at least 8-12 hours a day. The cost of a vent nurse is estimated to be $35-60 an hour. The biggest obstacle that I am faced with is locating the funds to ensure that I am able to maintain John’s quality of life. For the next full year it will require an estimated $150,000 to maintain John’s quality of care at home. A donation of any amount would be of great value in giving my husband some quality of life.

As persons with ALS often struggle to find an adequate amount of funding for professional care; authorizing this donation to assist me in providing proper care, will relieve the financial burdens that I am faced with as the primary caregiver, a daily basis.

Thank you for your time. God bless you,


Debbie Miller

Psalm 103 - Praise the LORD, O my soul, and forget not all his benefits, who forgives all your sins and heals all your diseases, who redeems your life from the pit, and crowns you with love and compassion, who satisfies your desires with good things so that your youth is renewed like the eagle's. The LORD works righteousness and justice for all the oppressed. The LORD is compassionate and gracious, slow to anger, abounding in love.

100%of the funds collected go directly to John and Debbie Miller to help in their financial battle with ALS. To follow John’s progress visit http://johnmilleralsfund.blogspot.com.
Make Checks payable to John Lodge Miller ALS Medical Trust Account:

Wachovia Bank
John L. Miller ALS Medical Trust Fund Account
Galt Ocean Mile Financial Center
3600 North Ocean Blvd.
Fort Lauderdale, FL 33308
(954) 468-2840 Read more!

Lithium - Could It Be The Stopper?

A surprising result in the fight against ALS.

Italian researchers claim to have stopped ALS
dead in its tracks.
By stopped, they mean no progression.
By ALS, we
mean the incurable disease for which there has been no significant medical
breakthrough.
The Proceedings of the National Academy of Sciences, no slouch
as scientific journals go, has accepted the researchers' paper for publication.
We should see the details of their possibly landmark study in early February, a
journal spokeswoman said.
I want to avoid overstating the significance of
these findings from what is, after all, only a single, small clinical trial.
But on its face, it's significant.
Sixteen ALS patients were given two
drugs: Riluzole, the generic name for the only drug approved for treating ALS,
and lithium, a mood
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stabilizer
used for bipolar disorder and depression.
Another 32 patients took only the
Riluzole.
The patients - 16 on lithium and 32 not - were watched and
evaluated over a period of 15 months.
The results of the trial were
announced in early November at a Parkinson's disease conference in Italy. News
of the findings was carried in an Italian science journal.
At the end of the
15 months, 30 percent of the group not taking lithium had died. Those who
remained suffered on average 50 percent decline.
This is what one would
expect with ALS.
For those taking lithium, however, there were no deaths.
Additionally, the ALS patients taking lithium experienced no meaningful disease
progression, according to Francesco Fornai of the University of Pisa.
Eight
of the 16 were diagnosed with the more aggressive bulbar form of ALS that
attacks the motor nerves for talking, eating and breathing early on, Fornai
pointed out. They would be expected to die more quickly.
Two years after
starting on lithium, only two had died, one from a heart attack and the other,
who suffered from an advanced case of bulbar to begin with, from ALS-related
causes.
The rest remained stable with no progression.

Fullstory at http://www.sbsun.com/ci_8089973?source=most_emailed.

Read more!

Recalcitrant Lake - Explained

This is an explanation of yesterday's post, Recalcitrant Lake. First, the title. In this case the title was the first thing to come to mind as was the subject matter. The part of a poem that inspires the rest is like a butterfly that lands on your shoulder and whispers something like "recalcitrant lake" in your ear. And you start repeating it. I think this fell together quickly - one week - because of the urgency of it (hospital visits weigh heavy over time).

Once I had a butterfly whisper stuck in my mind for three years. It went "She can't get the flash to work". Every attempt at working that into a poem collapsed miserably but I still recognized that it was an anapest follow by four syllables forming an iambic meter. It had a good sound to it. Shortly before Mother's Day the rest of it hit me like a thunderbolt - just in time for a winsome Mothers' Day poem.

Now I hadn't used the word "recalcitrant" in some time and I wasn't sure if "Recalcitrant Lake" made any sense. I was surprised to find that the meaning was something like I'd remembered: resisting authority or control; not obedient or compliant, basically hard to deal with, manage, or operate. Combined with lake, a thing in which you could drown, it was an apposite description of what it must be like to have a disease raging through your body. It's almost too accurate a metaphor.

Sometimes something unpleasant is necessary, especially if its true. But this recalcitrant lake experience also is a nightmare for the significant other or others whose lives are rocked by the tidal waves of the disease, in this case ALS - the dreaded Lou Gehrig's. Indeed these are the two personalities of the poem, the patient and his wife, my friends John and Debbie. My ignorance of the paid caregivers is not meant with any disrespect to them. We have met many wonderful people who care deeply about Johnny's well-being. It's simply that their roles didn't find their way into this particular poem.

It's not good, not at all

not at all, consuming, gnawing

away at my vitality;

Amyotrophic Lateral Sclerosis causes the muscle to wither and fail. It began in his shoulders then worked its way to his arms. When he had this terrible fall in October at the hospital(the beginning of this rapid decline), it became apparent that his feet were also coming under the spell of the corrosive muscular curse. His tongue and throat muscles were shrinking as well and now he is fed through a plug in his stomach.

IVs invade, filling an oasis

under my skin with numbing

concoctions making even

my bones tremble and grow soft.

A shaky hold I have, my lifedraining its putrid effluentover my bedpan, soaking the linens.The nausea of the IVs and the frustration of being incontinent.

It's not good, not at all,

not at all, as bauds of Pharma

make me sicker, make me dazed

and asinine alarms make me crazy.

"Bauds" is a little literary swipe from Wallace Stevens' classic "13 Ways of Looking at a Blackbird". There the bauds of Euphony, here the bauds of Pharma - the drugs that never work as good as they are explained they will. The reference to "asinine alarms" is that stupid nasally sounding "boop-oop" alarm of the respirator whenever the patient coughs or has trouble breathing.

I fight vile feelings of pique

My secret hope is that the world

will end tomorrow and take me with it.

Feelings of ire or pique (including wounded pride)are common in terminal patients.

For now I am speechless and rigid

I am the emergency in 372, hysterical,

I am the commotion, the Code Blue!

Perhaps a little poetic license here. John has had panic attacks but not a "Code Blue" - I think that's when something really bad happens (when my brother-in-law was rushed to ICU, they called it a "Code Blue" over the PA system). I wanted to bring to life that moment when the experts take over and send the family member(s) to the hallway. Commotion reigns.

You're still here, your still here

I can see you across the way

It's just you and me, surrounded

by the blurry, dry-erase names

that come and go like IVs.

In the commotion, the patient makes eye contact with their loved one. The hospital where John has been uses a dry erase board to list the nurses' names. He is surrounded by the nurses.

I have my one good eye fixed on you -

deny me not, not once nor thrice.

Don't let me die here, floating

like a starved jellyfish drowning,

depraved in this recalcitrant lake.

A plea to the loved one. "Not once nor thrice" is from the gospels. In the clip from "Passion of the Christ" that they used for the "Just As I Am" video, Jesus, wounded and bloody, looks out at Peter who is receding into the crowd trying to get away.

You're still there, you're still there;

I can see you between the scrubs,

The loved one has not abandoned him.

stop crying - I am mute but not dead,

I am dying but I am far from sad

Since John hasn't talked since November 26th, we really don't know what he is thinking. He has not made any expression of sadness though. In fact, he has given signs of bemusement many times.

I know that there is debt but my soul

tells me it will be paid in full.

In times like these the sick one has nothing to offer their loved one who stays by their side. This is a spiritual debt, which is why the soul tells him it will be paid in full. It truly is agape love - the highest form - that I see with Debbie and John. He has nothing to offer her at this point except things like bowel movements and hard work. Agape love is in control when someone commits in this way to another, in bold decision that there is no other way. It is then, when agape love is ushered in, that God is intimately involved.

I am better, come back and stay,

hold my hand while I sleep and dream

of contours green by still waters where

we lay unoccupied, unhurried, being.

Where else would agape love lead you but to this kind of peace and tranquility, beside contours green and still waters (a paraphrase of Psalm 23). That is the Valley of Death, you might say, does that mean I think Johnny will pass on soon? I don't know. ALS patients can last months and years past the time that all bets have been called off. But I end the poem with a present participle, and not just any present participle. "I am that I am" is the name the Lord tells us to call Him. And we are told that God is love (1 John 4:16), agape love. Through Debbie's total commitment to love Johnny, the dream comes true. The lake tends to be recalcitrant in this life but through love the waters are stilled, even now, and forever.

So there it is. A butterfly landed on my shoulder and whispered "Recalcitrant Lake" in my ear and this is what became of it. May it bring some loved one peace as they spend the night in the uncomfortable rocking chair next to the bed in the cold, contagious hospital, listening to the periodic shuffling of the nurses' feet and that damned "boop-oop" sound. May the Lord give you rest. Read more!

Recalcitrant Lake - a poem

[To John and Debbie, with love. It has been an inspiration to me to see Debbie stand by my her husband day in and day out. Suffering from the complications of Lou Gehrig's disease on top of a heart attack in November, Johnny has not been able to speak. The agape love she has shown has made all the difference.]

It's not good, not at all

not at all, consuming, gnawing

away at my vitality;

IVs invade, filling an oasis

under my skin with numbing

concoctions making even

my bones tremble and grow soft.

A shaky hold I have, my life

draining its putrid effluent

over my bedpan, soaking the linens

It's not good, not at all,

not at all, as bauds of Pharma

make me sicker, make me dazed

and asinine alarms make me crazy.

I fight vile feelings of pique

My secret hope is that the world

will end tomorrow and take me with it.

For now I am speechless and rigid

I am the emergency in 372, hysterical,

I am the Code Blue!

You're still here, you're still here

I can see you across the way

It's just you and me, surrounded

by the blurry, dry-erase names

that come and go like IVs.

I have my one good eye fixed on you -

deny me not, not once nor thrice.

Don't let me die here, floating

like a starved jellyfish drowning,

depraved in this recalcitrant lake.

You're still there, you're still there;

I can see you between the scrubs,

stop crying - I am mute but not dead,

I am dying but I am far from sad

I know that there is debt but my soul

tells me it will be paid in full.

I am better, come back and stay,

hold my hand while I sleep and dream

of contours green by still waters where

we lay unoccupied, unhurried, being.

- Jim Jordan 1/26/08

Read more!