Love is All There Is

“Clinic”

The gold standard of care for an ALS Patient is an ALS Center of Excellence. There are currently 132 Centers of Excellence in the United States. This is a group of medical providers who have been recognized by the ALS Association as providing the most appropriate, caring and up-to-date care as possible, as well as being a center that participates in clinical trials as appropriate. We were fortunate to live an hour away from just such an ALS Center of Excellence: the Providence ALS Center in Portland, led by neurologist Kim Goslin, MD, PhD, an amazingly caring and supportive person. The Providence ALS Center is a collaboration between the Providence Brain and Spine Institute and the ALS Association of Oregon and Southwest Washington.
 
Attending an ALS Clinic is a marathon appointment that typically happens every three months. It is a four-hour appointment, with no breaks. You see up to 14 providers on the care team in that four-hour period, depending on your level of progression:
 
Neurologist
Pulmonologist
Speech Language Pathologist
Augmentative Communication Specialist
Respiratory Therapist
Occupational Therapist
Physical Therapist
Social Worker
Registered Nurse
Gastroenterologist
Dietitian
Psychologist
Clinical Research Coordinator
Hospice Coordinator
 
It’s a “one-stop shop” where the patient is placed in a room, and the providers come to the patient. It is overwhelming and intense, with some provider visits lasting only 15 minutes and others 30 minutes.
 
After the Clinic appointment, the entire care team meets to review every patient they have seen that day, and they develop an updated care plan. They write prescriptions for medications, equipment and services. They follow up on clinical trials and evaluate who might qualify for which trials.
 
This team approach is the gold standard because it treats the whole person, addressing physical, emotional and medical needs, and has the ability to address spiritual needs as well. This model has repeatedly shown improved quality of life and length of life for ALS patients.
By bansagart, ago

When “progression” is a bad word

ALS progresses differently for each individual, but there is always progression – disease progression. I remember someone asking me once how Mary was doing. I told them, “She’s progressing”. Their response was, “Oh, that’s good news!” Um, no, it’s not.

 

The tool used to measure ALS progression and “outcomes” (aka, give a rough estimate of life expectancy) is called the ALS Functional Rating Scale – Revised, or ALSFRS-R for short. Each time the person with ALS (PALS) goes to the ALS Clinic, the clinic staff develop the score; in my experience, it was always mailed/emailed several days after the clinic visit, after all of the providers had met in a case conference to discuss what changes they had noted and what supports are needed. (I will share more about “Clinic” in another post.)

 

Functioning ability is measured in the following areas on a scale of 0 to 4, with 4 being “normal”:

 

Speech

Salivation

Swallowing

Handwriting

Cutting food and handling utensils

Dressing and Hygiene

Turning in bed and adjusting bed clothes

Walking

Climbing Stairs

Dyspnea (shortness of breath when walking)

Orthopnea (shortness of breath while sleeping)

Respiratory Insufficiency (often measured by FVC, or Forced Vital Capacity, which I will also address in another post)

 

The ALSFRS-R score is the total of the above categories’ scores.

 

Someone with no impact from ALS would have a score of 40.

 

Nine months into diagnosis, Mary’s ALSFRS-R score had dropped 19. At 11 months, it was 14. Roughly translated, the 14 meant that she had a 20% chance of surviving another nine months unless she decided to get a tracheostomy and go on an invasive ventilator. She had no spontaneous breathing ability, minimal use of her right foot and a couple of fingers in her right hand, and was still able to speak, although her voice was getting weaker and she was starting to get fasciculations (muscle tremors due to dying nerves) in her face. I wrote then that I could see her getting “locked in” within a couple of months, unable to move any muscles or communicate in any way, completely paralyzed. About three months later, she opted for the trach and ventilator.

 

She never wanted to know what the ALSFRS-R score was or what it meant. She would never look at a report. When I asked her if she wanted me to read it to her, she would just say “skip the bad parts” – which was pretty much the whole thing. But understanding the score and what it meant helped me to be prepared for what might come next, to understand the disease progression and what would likely happen next.

 

By bansagart, ago

Grasping at Straws

I mentioned in a previous post that Mary put off telling people about her diagnosis. And the ones she did tell, she kept in the dark about how much and how quickly the disease had progressed. People would call that she hadn’t Image may contain: 1 person, sunglasses and hatspoken to in a while, and hear her “nasally” voice and ask if she was ok. “Oh, it’s just a cold. I’m fine.” Those were the ones she didn’t tell about the ALS. Others would call and ask how she was doing. “I’m doing GREAT!” she would tell them, not telling them she could no longer hold the phone and that the caregiver had just placed a headset on her, plugged it into the phone, set it on her lap and pressed the answer key for her.

Her reason was that she couldn’t allow herself to get caught up in the negative, that she needed to live in the moment and not think about the future. I would argue that she did need to think about the future so we could make plans for her care. It wasn’t until later that I realized she truly believed she was going to beat this disease. She was researching unconventional treatments, pouring through articles about how certain strains of marijuana cured cancer and ALS, so she started growing a marijuana plant to make into tinctures to take. Not that she actually used any of the marijuana, despite arguing with doctors about getting a medical marijuana card. She read about someone in Israel being cured of ALS by stem cell therapy, and swore she would live long enough to receive that treatment. When I read the article, I wasn’t convinced the single test subject had actually had ALS.

This is not uncommon for ALS patients. There are all kinds of ads and offers of “we’ve cured ALS” with bee venom therapy, paleo diets, crystal therapy, essential oils, marijuana, secret herbs of the Amazon or Africa. They all come with a very high price tag. It is heartbreaking to watch people recently diagnosed as they grasp at any potential treatment or cure, as if no one else has ever tried it, as if it had ever truly worked. Because if it truly did work, we would be telling everyone about it! It creates false hope, drains bank accounts, and causes rifts in families. And the predators walk away with thousands and thousands of dollars.

What we need are real treatment options, real medications that have been tested, real diagnostic tests, a real cure. It is often said that ALS is not incurable – it is underfunded. Even with promising research being done around the world, the funding is not there to support ongoing research, or new research projects. Please consider donating to support the work, through sponsoring an ALS Walk or other fundraising event, large or small. You can make a donation through purchasing a product, such as with my daughter’s “Jams for ALS” fundraiser . You can make a direct donation to the ALS Association. Help spread awareness about ALS, what it is and what it does, and what you can do to help those living with ALS.

By bansagart, ago

When your life depends on power

A week after the third time Mary almost died, early in November 2014, a Trilogy non-invasive ventilator was delivered as a replacement for the BiPap/AVAPS. The Respiratory Therapist spent over three hours making sure the settings were just right for her, and was very patient with Mary’s intense anxiety. In addition to the internal and external batteries in the Trilogy, Mary had requested and gotten insurance approval for a second external battery. It took some time for her to adjust to the differences between the Trilogy and the AVAPS; the biggest challenge she had was adjusting to talking with the increased power of the breath support and air volume (which she needed).

The first night she was on the Trilogy, I was up most the night adjusting and re-adjusting settings and alarms that kept going off every time she fell asleep.

People had always suggested contacting the electric company and requesting priority power restoration. I had contacted the electric company shortly after the second time Mary died, trying to be prepared for winter storms. It turns out not every area/community/company offers that option. In our rural area, when I called about getting a medical alert set for the home address due to life-sustaining medical equipment, the power company told me that their only suggestion was that I get a generator, or call an ambulance for a ride to a hospital that would have power. If we had a power outage, we were on our own. Restoring power was based on the number of customers impacted. Fortunately, we already had a portable generator that I had purchased for doing work around the property, and I did keep gas on hand, but only a couple of gallons.

Less than a week later, I was at work when I got a call from the caregiver that a big storm had knocked out the power at the house. The Trilogy seamlessly switched to battery mode. There was no panicked scrambling for the backup battery. Another life-threatening crisis had been averted because of the previous week’s incident. I had the caregiver hook up the land line phone in case she needed to call 911, and then I called the neighbor to come down and fire up the generator. The power company had no ETA for when our power would be restored. It was cold The caregiver spent the next four hours alternating the generator power between the Trilogy, the Kangaroo feeding pump, the lights, an electric blanket and a portable heater. I checked the forecast before I left work; it called for high winds, snow and ice moving in over the next 24 hours, so on the way home, I picked up a 5 gallon can of gas for the generator in case we lost power again.

Literally as I pulled up to the house, the power came back on. Mary was bundled up under multiple layers, but was joking with the caregiver. Despite the unexpected and early winter storm, we did not lose power again that time.

When someone is dependent on equipment to breathe, when they have no ability to breathe on their own, backup power is crucial. Having the Trilogy with its rechargeable internal power supply kept Mary alive. Having a generator ready to go with gas on hand helped keep her alive. Having a neighbor who happened to be home and willing to help out was priceless. All of this meant there was no “fourth time Mary almost died.”

By bansagart, ago

Broken and Shattered

I had very little knowledge of ALS before Mary was diagnosed.

I did know it was 100% fatal but I had no idea how much damage it could do before killing you.

Now that I know more than anyone should, every time I see or hear of someone being diagnosed, it breaks my heart. When it is someone I already know and count as a friend, it is even more heartbreaking.

My broken heart shatters each time I hear that a friend has died. In the last 10 days, two people in my circle, my ALS community, have died. Despite living with an ALS diagnosis for 4 and 10 years, both took sudden turns that claimed them quickly. They have left behind people who were my rocks while Mary was sick, while she was in the hospital, when she died and since that day.

Each loss tears the scab off of my own ALS battle wounds. Each loss brings me back to Mary’s journey, her final days, her last day, and the intense depth of grief that knocked me flat. And I know what my friends are feeling: the shock, the anger, the disbelief, the surreal and intense roller coaster of loss and grief.

Knowing the diagnosis does not make it any easier. Knowing that this round of pneumonia, this urinary tract infection could kill them does not make it easier. Platitudes do not help. “They’re in a better place now” doesn’t help. “They aren’t suffering anymore” doesn’t help. All you know is that the ALS bullet train has just derailed, that life as you have known it over the last 6 months, year, five years, ten years, has changed in a second. Who you are has changed in a moment. Your life will never be the same, the scars will always be there, the loss will always sting.

Someday, there will be an effective treatment. Someday, there will be a cure. Someday is not soon enough. Someday is too late for too many people.

So today, my heart is shattered again, and I reach down to gingerly pick up the pieces and try to put my Heart, my Self, back together again, knowing that once again it is different than it was a moment ago.

By bansagart, ago

Equipment

Soon after diagnosis, the equipment starts flooding into the home and it just never ends. Here is a partial list of likely equipment that will float through the home during the disease process, in no particular order. Insurance, if it covers anything, won’t cover all of it.

– Bipap/AVAPS
– Nebulizer
– Trilogy
– Hoses and face masks and filters
– Tracheostomy supplies
– Oxygen tanks
– Backup batteries
– Battery chargers
– Generator
– Suction machine and supplies
– Formula by the case
– Wound care supplies
– Feeding tube supplies
– Feeding tube pump (aka Kangaroo pump)
– Cough Assist
– Breath Stacker
– Adaptive utensils
– Neck braces
– Wrist braces
– Compression socks
– Contracture boots
– Leg braces and canes
– Walker
– Manual wheelchair
– Power wheelchair
– Adaptive clothing
– Hospital bed
– Over the bed trays
– Supply carts
– Alternating pressure overlay or mattress
– Pain and anxiety medications
– Medications for a bowel regimen
– Urinals and catheter supplies
– Bidet
– Chux and baby wipes
– Hoyer lift and slings
– Sit to Stand lift
– Lift recliner
– Bath/shower chair
– Inflatable shampoo basin
– Speech Generating Device with tablet or computer
– Letter board
– Eye Gaze Device
– Personal Alert System
– “Nurse” call bells ranging from a hand held bell to a small muscle triggered alarm
– Baby monitor

In the 22 months from diagnosis to death, and for almost a year after, most of these lived in my house. I’m sure there was much more than this. I became an expert on medical equipment that most people have never seen outside of a hospital, let alone had to learn to use, maintain, program and troubleshoot. Caregivers and I figured out how to make equipment work together that the doctors and Respiratory Therapists said couldn’t – then we took pictures and made instruction sheets and took them to the doctors and RTs. When the medical professionals were at a loss, I reached out to my Facebook groups for ideas, then I googled for hours, ordered supplies from all over the world, trying to find something that would work and fit Mary’s needs.Image may contain: people sitting

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By bansagart, ago

Round Three

The third time Mary almost died was only a few weeks after the first time.
 
I had taken a bad fall at work on Tuesday – luckily, I hadn’t broken anything, but rewarded myself with a “minor head injury”, a mound of paperwork, and a doctor’s order to stay home for at least a day. Luckily, the caregivers showed up the next day and I was able to rest. Then on Thursday morning, the caregiver and I were getting Mary ready for a trip to the eye doctor when we had another incident with the power and air flow on the bipap going out and NO ALARM! Neither of us were looking at Mary when it happened, and it took a few seconds for the caregiver to see what was going on and tell me, then what felt like forever to switch the power supply back from battery to the wall outlet. I had to call and cancel the eye doctor appointment because Mary absolutely refused to go back on the battery power or even to leave the house.
 
I then called the ALS clinic to let them know what had happened and ask about switching from the bipap to a Trilogy with a better alarm, and to ask for oxygen for recovery from situations like this. They called back with a message from the pulmonologist, who was concerned that she was decompensating so quickly (she had zero ability to breathe independently), and told Mary that if she still wanted a trach the time was NOW.
 
Mary said she thought she had more time. I told her the ALS doctor had suggested she do it back in August, but apparently she hadn’t registered or remembered that. I finally told her that if it was me and I had no mobility, I wouldn’t want a trach, but that I would honor her wishes. Now, I thought, she will face the decision. She began to look more seriously at the issue and doing some research to decide whether or not to get the trach, and I found some other people with ALS who were living on a trach and ventilator for her to email with about their experiences. I hoped that maybe she would actually finish telling her old friends what had been going on, the ones she hadn’t even told yet that she had ALS.
 
I posted in a Facebook support group, “How long can we do this? I am overdone, physically and emotionally, and the roller coaster never stops. I’m a wreck. How do I sleep not trusting the alarm to go off on the AVAPs if the power goes out? How do I keep doing my job with a smile on my face when I keep getting punched in the gut?”
 
To trach or not to trach…. a decision we struggled with for nearly a year. A decision we argued about, cried about. She stilll didn’t make the decision at this point. She got the Trilogy a few days later, and with the additional settings and alarms, and the internal backup battery power, she became more comfortable with it and yet again delayed making a decision and reaching out to old friends.
By bansagart, ago

Caregivers

Working full time, caregiving, and dealing with paid caregivers means you have three full-time jobs. At first, when Mary’s needs were minimal, it wasn’t too difficult to find caregivers. We did get rid of a couple early on – one who tried to convert Mary to her religion. Um, really? Another who sat on the couch and slept the whole day, not waking up when Mary called to her to ask for a drink of water. As the months went by, Mary needed more skilled care. We started going through caregivers more quickly. We typically had four caregivers scheduled in a week, since we needed 15 hours a day. I kept begging for them to hire and train back-ups, but that never happened. I wrote this in September 2015.

“Caregiver hell week. One caregiver decided Tuesday that she’s now in over her head and Friday would be her last day here. Another caregiver disappeared for a week then cancelled her shifts – the agency sent a replacement last Saturday. We thought we would give her a shot. Wednesday didn’t go well, then yesterday she called out for today. Buh-bye. Yesterday evening’s caregiver called out sick. The agency is sending yet another new one tomorrow to train with our Sunday CNA (who rocks). Please let this one work out, or I don’t have caregivers after 3pm during the week M/W/F and I have to work.

“The agency keep sending new people that have the right backgrounds but have crappy attitudes. I won’t have it. Yes, I’ve been thinking about another agency or hiring direct, but that has its own headaches…. the one coming to train tomorrow is a CNA brand new to the agency but who was a shift lead at a “memory care” center. The agency manager plans to only send CNAs out now, with the level of care Mary needs, but sometimes their egos are bigger than their licenses.

“My favorite line (not from the manager, another office worker): “but the girls are learning so much from Mary!” Excuse me, she’s not here to be their frikkin teacher!”

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Mary and Grumpy Cat sharing a moment.

By bansagart, ago

10 Months In

I wrote the following in the summer of 2014 for the newsletter of the ALS Association of Oregon and SW Washington, a segment called “Caregiver’s Voice”.

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My partner Mary DeWitt and I met in the fall of 1996 in an AOL chat room, where we quickly became friends. We chatted off and on via email and the phone, then lost contact for several years. In 2003, Mary tracked me down, we started talking again, and finally realized that our paths were meant to be intertwined in a deeper way. She moved to Long Beach, California where I was living, and my three nearly-grown kids soon adopted her as “SuperMom”. In November of 2007 a job opportunity opened up for me in the Portland area and we made the move. Mary was thrilled to be back in the Pacific Northwest where she had been raised, and we both loved living close to a nature center, where we could walk together in the woods. In July 2012, we were able to find a rural home on two acres and started to work on our plans to turn it into our dream place when things came to a screeching halt.

Mary had been diagnosed with fibromyalgia in 1997, and with rheumatoid arthritis in 2005. When she started developing more fatigue, muscle weakness and joint pain, we attributed the symptoms to those diseases. Then other symptoms started to crop up that didn’t fit with either of those diagnoses: increased memory fog, shortness of breath, muscle spasms, and large numb patches on her back. Her brother had just been diagnosed with MS, and since she had always thought her mother had died from MS complications (in 1969), she was referred to a neurologist for testing. It was nearly a year before MS was ruled out, and by then her headaches, weight loss, shortness of breath and leg/arm weakness were much more pronounced. She was diagnosed with Motor Neuron Disease/ALS in December 2013. Even though we both had known that was a possibility, the diagnosis was devastating. We tried to keep a positive outlook, and admittedly Mary has done a much better job of that than I have. In the last ten months, Mary has gone from walking with a cane to being unable to bear any weight on her legs. She uses an AVAPS 24/7, spends her days in a power wheelchair, and gets the majority of her nutritional needs met via a feeding tube; now we are looking at communication options as she begins to lose strength in her voice.

Having no family or friends nearby has been challenging; everyone is in another state. We have been fortunate that family members and some friends have been able to come and visit over the last six months, helping out where they can and providing emotional support and comic relief.

For Mary, these last 10 months have meant an incredible amount of change, forcing her to develop trust in the midst of vulnerability, of literally putting her life in the hands of agency caregivers every day so that I can continue to work and keep our home. For me, it has been a constant fight to keep myself together while I go head to head with bureaucrats, ignorance and arrogance to get her the services and support she needs. We have been through quite a few caregivers and home health nurses who could not keep up with the changes or who thought they knew what was best without listening to Mary and observing her changing needs.

I have found the monthly Caregivers’ Support Group to be an incredible source of strength and knowledge for me. It was hard to walk in the first time, but I was immediately welcomed and learned so much that I attend as often as possible. All of our PALS progress at different rates and patterns, and have different needs; sometimes you are asking questions, and sometimes you find you are the one with the answers. If you are a caregiver, and you haven’t yet been able to attend a Caregivers’ Support Group meeting, I strongly encourage you to attend at least two or three times before deciding if it is the right place for you. On a more daily basis, I have found my support in a Facebook group for caregivers where we ask questions, vent frustrations, gain tools to advocate for our PALS and share the joys.

The staff of the ALS Association of Oregon and Southwest Washington have been amazing to us as well. The emotional support and advocacy available by email and phone are incredibly helpful, and the equipment loan closet has been critical as Mary’s equipment requests make their way through weeks or months of bureaucracy. And we can’t say enough about the staff at the Providence ALS Center who are guiding us through this journey and supporting whatever needs arise. Our families and friends took on the Ice Bucket Challenge and made their donations to the Oregon/SW Washington chapter, knowing that they were helping us and others in our community even though they can’t be physically here to support us as we all wish they could.Image may contain: 2 people, outdoor

I am trying to walk this journey one day at a time, and although I am not always successful, I continue to strive for what balance I can find. I am trying to find ways to take care of myself in the midst of constant change. I am trying to trust that something bigger than myself is supporting me, and will continue to support me as we walk this journey together.

By bansagart, ago

The Second Time

The second time that Mary almost died was in the early hours of October 3, 2014 – 10 1/2 months after diagnosis.

She woke me up at midnight complaining the nose pillows on her bipap were leaking. By this time, she had been completely dependent on the bipap to breathe for several months. I spent about an hour adjusting straps, but it was still leaking, and she said, “this is getting scary” as she started to go into a panic attack. I went to the kitchen to find a new set of nose pillows. I managed to replace the first one and attach to the side strap, then I went to switch the tubing to the new nose pillow from the old one and COULD NOT get it to connect. I usually made all those changes when the bipap was off; with the air pressure on, the tubing would not connect.

By 10 months in, Mary had lost strength in her hands and arms, and she discovered she could no longer hold the nose pillow to her face while lying down. I grabbed the nearby ambu bag and started bagging her while trying to get the mask back on, only to discover that she no longer had the arm strength to push down hard enough on the ambu bag to get air – that had been her plan if her mask failed – and that the ambu bag was not really an ambu bag but one modified as a breath stacker, so it was not very effective. I was standing over her at 1 am, bagging her and trying to figure out how the hell this was going to turn out.

I had her activate the Lifeline alarm she wore on a chain around her neck. The monitor was in the living room, and I heard a voice call out, “Mary do you need help?” I yelled “Yes!” over and over, knowing that even if they didn’t hear me, they would send paramedics and an ambulance. A minute or so later, I hyperventilated her and ran to unlock the door (so the paramedics wouldn’t kick it in again), but I completely forgot to turn off the burglar alarm. I went back to bagging her before I realized that, and I couldn’t stop bagging her again. A few minutes later, four burly guys (2 from our volunteer fire department and 2 paramedics who had been here a few times before) arrived. They were trying to ask me what was wrong and I was yelling directions at them over the screaming house alarm, the bipap patient disconnect alarm, and the barking dog.

I told the fire captain to grab the other ambu bag from the kitchen and take over bagging her so I could turn off the alarm and fix her nose pillows. The paramedics got on it, saw her O2 saturation was 76 (definitely not good) and added a bit of oxygen while I quickly detached her feeding tube (oh goody another alarm going off) so they had room to work. With the extra help, I was finally able to attach the nose pillows the rest of the way and slide the bipap mask back on her face. At that point her O2 sats jumped back up to 100, I got all the alarms turned off, and made sure she was ok before finishing up paperwork with the EMTs. As I told them, I just needed another set of hands.

It was after 3 am when I crawled back under the covers. The new plan was for her to mention the nose pillows were leaking BEFORE the evening caregiver left at 10 pm, so that we would have two pairs of hands to take care of it. The paramedics suggested we keep O2 on hand so she could recover more quickly if something like that happened again. I also started to keep a complete back up mask and tubing, already assembled, with her at all times so that a switch out could happen much more quickly.

This type of thing happened frequently, although not with such close calls. People don’t realize why family caregivers are always so stressed and exhausted. I was too exhausted to tell anyone about it. I had no energy to call anyone. My days were sleep-deprived, food-deprived, stressed, worried, physically and emotionally exhausted. It is nearly impossible for ONE person to care for someone with this level of advanced ALS, even with (unreliable) paid caregiver help. “Call if you need anything” asks the impossible. Please check in with family and friends who are family caregivers – don’t wait for them to ask for help. They may not be able to.

By bansagart, ago