The Journey

2007 - May 11

This morning at 8:46am mom lost her battle with this tragic disease.

I want to thank all of you for your prayers and words of encouragement during this challenging time.

2007 - May 10

Mom's appearance was changing. She couldn't move her body at all. Again she awoke unhappy because she was still alive. She could barely talk, but she could still lift her arms to embrace her loved ones. She was happy to see her grandson from Salt Lake City. She told him she was praying that he would find a nice wife. We all smiled. When she was through talking, she reached up to remove her glasses. I took them from her and asked if she was 'going to sleep'. She whispered, 'yes' and tried to remove her oxygen. I knew it wasn't doing her much good, but told her she could sleep with it on.

The crowd had gone home. She talked to one other grandson, then the preacher and then she was ready to sleep through the rest of this process. My sister, Janice, and I gave her medication every 2 hours through the night, so she would know that her body was becoming paralyzed. We realized in the night that she didn't seem conscious, but we didn't want to stop the pain medication just in case she could feel anything.

2007 - May 9

That next morning she awoke very unhappy that she hadn't passed in the night. Her neck was so stiff that she couldn't move her head. I know she didn't want to experience this, but she had a few more to talk to. The day was a little quieter as we tried to keep her as sedated as possible. She still kept waking up everytime anyone came in her room and kept asking when she would die.

2007 - May 8

Vicki told us that mom was in the 'death process'. There were barely any bowel sounds. She was put on a liquid diet. Her voice had become very quiet and we began using liquid anxiety and pain medication to keep her calm.

She asked us to call family and friends so she could say good-bye. came from everywhere. Everyone went in to talk to mom. She hugged each one and offered her love and ocassionally her final instruction or advice. She laughed as best she could muster and had her own funny comments. She said, 'I bet nobody's dying was ever like this.' She thoroughly enjoyed the attention after months of near isolation with the disease.

She had asked for stronger pain medication as her neck and head had become very painful. She decided to wait for her grandson in Salt Lake City to come, before taking stronger pain medication, because she thought she might sleep from then on.

That night we had a houseful of company (her daughters, 2 sisters, 2 nieces) and dad sleeping all over the house.

2007 - May 4

Today mom contacted her Hospice Nurse (Vicki Coppel) from New Frontier Hospice. She told her that she was ready to stay in bed and was giving up the fight. Breathing had become increasingly difficult and going to the bathroom was impossible. Being mobile had become a danger to her and her care-takers. She was giving up the fight.

Christmas 2006

Vicki told us that mom didn't have long to live. She was oxygen-dependent and was breathing with only the top portion of her lungs. She was suffering from respiratory failure and wouldn't last long. At that time we began round the clock duty. She used a bed pan or was lifted out of the bed to use the bedside comode when necessary. Mom's appetite was still good and we cut her food into small pieces as swallowing was becoming increasingly difficult.

2007 - April 27 thru May 3

Mom seems to be losing her strength very quickly. During this week, she found it impossible to stand from a sitting position. If she used her lift-chair, her knees buckled and sent her to the floor. Although we caught her fall a couple of times, she fell to the floor about 5-6 times during this week. It was so hard to get her to give up the hand crutches. She injured her face and found herself hurting in different areas after such a difficult week.

2007 – April 5
MDA/ALS Center at Integris Southwest Medical Center (OK City, OK) - Dr. Beson

I need to tell you about my mother’s greatest partner in this ride on the ALS bus, the ‘MDA/ALS Center at Integris Southwest Medical Center’ - more specifically the wonderful team of angels that walk into this building everyday and deal with the diagnosis and progression of this incurable, depressing disease. I give them my eternal gratitude. They give honest answers and feedback, accompanied by support, hugs, and tears to patients dealing with every stage of ALS. They even have a sense of humor and bring smiles to very sad faces. (You should have seen Teresa today, demonstrating that you can drive a power chair, with your head almost touching the ground and your feet in the air.) Additionally, they ease the journey by providing resources and equipment that is needed for every patient that comes through their door. I believe Wendy said they’ve treated about 185 ALS patients in the past 3 years.

I can’t speak highly enough in support of the tremendous help we’ve received from these folks…sigh, smile. They are a gift from God.

Brent Beson – M.D., Director
Wendy Beson – Nurse
Teresa Tisdell / Randy White – Occupational Therapist
Andrew Heuser – Physical Therapy
Mary Mathew– Appointments/Questions

. . . . . . . . . . . . . . . . . . .

I made this additional appointment for mom, after we had a serious breathing episode about 2 weeks ago. When we were leaving the appointment today, Mary came to mom and gave her a big hug. Bless you Mary.

Now that mom’s breathing is affected, she is having difficulty with the equipment that has been provided. While she has no problem chewing or swallowing food, she says she can’t drink her apple juice without stopping 4 or 5 times.

NO weakness in talking.

Recommendations from Respiratory Therapist:
Mom deals with congestion in her throat and has trouble breathing when she lays down. A couple of weeks ago, she was provided a ventilator (PLV-100), which she has tremendous trouble with. They’re going to try and find her something that is quieter, with a more comfortable mask. She was also provided a ‘Cough Assist’, which she hasn’t been using much, as it is uncomfortable and she can’t feel any benefit. The importance was explained in that failure to use it will result in pneumonia, as secretions will creep into her lungs. She told the doctor that she was sleeping with oxygen that was provided by Hospice last week. It has helped her to sleep. She was told that oxygen was not good for ALS patients because while your brain appreciates the oxygen, your lungs will just give up and shut down. Many ALS patients go to sleep and never wake up. She needs to sleep with the ventilator, which she can’t seem to tolerate.

Recommendations from Physical Therapist:
Last week dad bought a handicapped-equipped van. It made the trip to Oklahoma City so much easier. This would give mom much more freedom, but what about when she needs to go to the bathroom? She can’t get off the stool by herself and occasionally, her knees buckle. Teresa told us about a ‘Spill-proof Urinal’ that could be ordered online. She also said that many of her patients wear long skirts, to ease the process. Teresa also took us to the restroom and demonstrated how to use the Power Chair to transfer to and from the stool in a public restroom.

Recommendations from Doctor:
When asked about depression, mom stated she has never been depressed in her life. In reality, I believe it is becoming a real concern. Dad is struggling greatly with this issue and I don’t think my sisters and I are without some effects. It was brought to her attention that she needs to be concerned about the kind of memories that her family will have of her in these last days. If medication is in order to ease this process, it should be a welcome solution. I couldn’t have said it better. What a bumpy road this is.

2007 - Mar 15
Walgreens Homecare (Tulsa, OK) – Respiratory Therapist

The therapist came and tested mom’s breathing. For the past couple of weeks, she has been having difficulty at night. She isn’t sleeping well, because she can’t breathe adequately. In addition her covers are too heavy and she can’t roll from side to side or sit up to get out of her bed. The following notes were recorded:

NIF Readings (Sitting) – 24, 40, 44
NIF Readings (Laying) – 40, 44, 32….reclined in her Lift Chair – doesn’t fully recline

Blood Pressure 132 / 80 Pulse 76

Weight – 145 (down from 158)
Height – 5’ 5 ½ “ (down from 5’ 6 1/2 “)

The therapist left the following equipment with mom:

- Cough Assist
6 sets of 5 breaths or 5 sets of 6 breaths (pressure should be 30 – be aware of leaks)
Place mask on face when dial is straight up
Cough with machine when it makes a noise
Replace mask every 6 months

- Ventilator – PLV 100 (LTV is a smaller unit which may be available next time)
It was advised that a Ventilator was a better choice, rather than a Bi-Pap Machine. This would give her more volume, which she needed.

Once the settings were all adjusted, we were told to report any adjustments to those settings. They would coordinate their information with the MDA Clinic.

2007 - Feb 28
MDA/ALS Center at Integris Southwest Medical Center (OK City, OK) - Dr. Beson

It was noted that her breathing is now affected. She retains congestion in her throat and has trouble sleeping as it feels like she is being smothered. Both feet have now dropped. There is noticeable increased weakness in her hands.

NO weakness in swallowing or talking. Appetite is fine.

Recommendations from Physical Therapist:
She was asked to use her Power Chair instead of crutches. Mom still struggles with the crutches, as she’s afraid she’ll lose the ability to walk at all. Walking is so hard for her and she risks falling every time she walks. She was told that if she falls and breaks a bone, her disease will progress much faster. Also, that she puts her Care-Givers at risk when they pick her up off the floor. Mom has fallen many times since her diagnosis.

Recommendations from Respiratory Therapist:
They will set up a visit from Walgreens Homecare to set mom up with a Bi-Pap Machine and a Cough Assist

• Breathing test - sitting up and laying down
• Prescription for Bedside Commode w/ folding arm (Suggested a Slider for access to the Bedside Commode when she is no longer walking)
• Prescription - Hospital Bed, so that her head can be elevated at night

2006 - Nov 02
MDA/ALS Center at Integris Southwest Medical Center (OK City, OK) - Dr. Beson

They discussed her visit to the doctor in San Diego on 10/30/06. They disagreed with his intention to do IGIV Therapy…it is not for ALS patients. I explained to them that the doctor in San Diego didn’t have her ‘lab results’ from their office, so he was unsure of her diagnosis. They also did not understand the prescriptions she was given (Mobic, Prevacid, & Prednisone…for muscle pain & stomach pain, which she doesn’t have). Dr. Beson was going to contact the doctor in San Diego to get more information. Naturally, they are concerned because the doctor in San Diego is not a specialist in ALS and I’m pretty certain once he sees her lab test results, he will conclude that she isn’t a candidate for the IGIV Therapy. I’m sure that the prescriptions were to combat the effects of the IGIV Therapy and he gave them to her before the therapy started, because she’d be going back to Oklahoma. In all, we felt pretty confident that we wasted our time and money in San Diego.

Dr. Beson checked her resistance by having her push with her arms and legs. Then, a breathing test was administered. We did not get the results.

Because of her inability to get up from a chair or walk by herself, Dr. Beson told her she was no longer safe on crutches. He told her that she needed to take responsibility for her own safety and that if she fell and broke another hip or her leg, she would be dead in less than a year. He recommended a wheelchair or power chair.

She says her other foot seems to be dropping. Also that everything twitches…legs, arms, hands, fingers, feet. She has become much, much weaker over the period of about 4 weeks. They explained that it is normal to have little change for a while, then change a lot, then have little change again for a while. Nothing she can do to change that. That’s how the disease progresses. She had a breathing test - sitting up and laying down.

Other foot is dropping. Everything twitches….legs, arms, hands, fingers, feet. She is much weaker.

NO weakness in swallowing, breathing or talking. Appetite is fine.

Recommendations from Physical Therapist:
The process was started to acquire a power chair. She picked out a model Permabill C300 Corpus power chair and they pulled together an order. Her insurance should cover the cost of the power chair. Mom told Dr. Beson that she needed more Physical Therapy. It was explained to her that she should NOT be doing anything with resistance to her muscles, because that tears down your muscles and hers will not come back. She should only be doing ‘Range of Motion’ movements. Mom has been involved in Physical Therapy ever since her hip replacement. ‘No resistance’ is a tough concept to grasp and mom continues her exercise routine.

She showed the Physical Therapist, her braces that were made from their prescription. He concluded that they were very poorly made from cheap material, not adjusted correctly, too big…and would harm her feet more than help. He asked for the name of the company that filled the prescription. He will talk to them and see if they can be remade…if not she was instructed to return the braces and buy another standard black one. She should call the insurance company and tell them the problem.

Recommendations from Respiratory Therapist:
Nothing recommended at this time to help with breathing.

2006 – Oct 30
Physician in San Diego, CA

My mother’s physical therapist told her about a doctor in San Diego, who had some favorable results with a former patient of theirs. Mom and dad wanted to go see this doctor. I was able to get the name of the patient that he helped and I called her. She didn’t have the same disease mom has. I called the office in San Diego and they told me they were working with 3 ALS patients at this time. This doctor was not covered by mom’s insurance, so it would be an expensive trip. Mom and dad had hope that this doctor could help.

So, on this day we headed to San Diego. Mom is barely transportable and my dad’s condition is questionable due to several mini-strokes.

I was surprised, with the difficulty and expense involved in our trip, the doctor knew nothing of mom’s case. Although I had sent information ahead and talked with several in your office, you were unaware of and unprepared for my mother’s condition upon our arrival. She was given samples and a prescription for Mobic, Prevacid, & Prednisone…(for muscle pain & stomach pain, which she doesn’t have). He also drew blood and said he would talk to Dr. Beson (her ALS Specialist) regarding her lab results. If her test results had a particular result, she would be eligible for IGIV Therapy.

We returned to Oklahoma with some hope.

2006 - Sep 22
Primary Care Physician (Tulsa, OK)

Mom needed a prescription renewed in case of a bladder infection. She has been troubled with these since her diagnosis.

2006 - May 25
MDA/ALS Center at Integris Southwest Medical Center (OK City, OK) - Dr. Beson

Mom came to Dr. Beson for a confirmation of her ALS Diagnosis. They certainly know what they’re doing here. As a family (my mom, dad, 2 sisters and I), we stay in one room while they send in many members of their team (doctor, physical therapist, respiratory therapist) one at a time, to come up with a diagnosis. Dr. Beson reviewed Dr. Johnson’s test results, then did some testing of his own. His best probable diagnosis – PMA (Primary Muscular Atrophy) – a variant or mutation of ALS. Typical ALS begins in the throat.

Noticeable Symptoms:
No reflexes, head is dropping slightly, muscle weakness (including feet), drop foot in one foot.

NO weakness in swallowing, talking, breathing, and NO cramping/twitching or hyper-reflexes.

Recommendations from Physical Therapist:
Replace cane with Walking Stick, as her posture is being affected
Replace current inadequate leg brace, with 2 improved braces
Purchase better shoes (SAS, New Balance)

• Review of history
• Physical examination
• Blood sample for further testing
• Discussion of genetics testing and implications
• Prescription for (2) leg braces to replace current inadequate one

2006 - May 12
Neurologist - Dr. Johnson (Tulsa, OK)

Dr. Johnson administered an EMG (Electromyography) Evaluation. The EMG evaluation is a standardized series of tests designed to assess the patient's pelvic muscle condition and neurological control, in order to pin-point deficiencies and provide a sound basis for a treatment plan. He also had the results of blood tests and an MRI.

He scratched his head and read his results over and over. He said he wished he could come to another conclusion, but he diagnosed her with ALS. He said he hoped he was wrong and wanted her to see Dr. Brent Beson, Director of the MDA/ALS Center at Integris Southwest Medical Center.

I had accompanied mom and dad to Dr. Johnson’s office. I had done enough investigation, that I was not surprised by the diagnosis. Mom and dad were surprised at this devastating news. Mom went into a state of denial, saying, ‘he diagnosed me with this because my mother had it’. Her denial continued for many, many months.

2005 – Oct 24
Tulsa Bone & Joint Associates (Tulsa, OK) - Dr. John F. Josephson

Today mom had Hip Replacement Surgery. Her recovery was very slow, as she had noticeable weakness in her abdominal

2005 – Oct 22
Mom fell and broke her hip.

She was helping dad nail planks on the floor of his pontoon boat. When she stood up, she lost her balance and toppled off the boat. Her balance had become a problem over the past few months and the arches in her feet had both fallen.

This begins mom's journey with ALS..