Equipment

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

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

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

When you need to go to the hospital

I live in a small town – population 600. We have a fabulous volunteer fire department with volunteer paramedics who work closely with the local ambulance company. I know because while Mary was alive, they were frequent visitors at our home.

The closest hospital is 12 miles away. They know nothing of ALS; they didn’t even properly treat Mary’s bloody nose well before her diagnosis. Long story. Suffice it to say there is no way in hell she would go there (neither will the ambulance drivers). The next closest hospital is 19 miles away. We went there several times, when it was “unplanned/urgent”. The hospital next to the ALS Clinic is 29 miles away; that’s where we went for “planned” stays.

Some tips to share from my experiences:

1. Before an emergency: Contact your local Fire Department. Let them know someone at the address has ALS; educate them in advance if you need to.

2. Before an emergency: Contact the ambulance company. Have them flag your address with someone who has ALS, who may be unable to communicate, and what equipment they use.

3. ALWAYS go to the ER by ambulance. You do not want your pALS hanging out in the waiting area, exposed to who knows what, with a compromised ability to shake off even a cold. “Walk-ins” are automatically lower on the triage scale; if you come in via ambulance you will be in a treatment room immediately.

4. Arrange ahead of time with your ALS clinic; if your pALS can wait, have ambulance personnel take them to a larger hospital. Tell them it is “Physician Request”.

5. If your pALS uses a bipap/avaps/vent, send it on the ambulance with them. Have them plug it in to the ambulance outlet so they are actively using it when they arrive. It will make it harder for the ER personnel to think “oh they don’t need it, it’s just a cpap” if they are already on it. They may, for liability reasons, want to switch your pALS to the hospital’s equipment. That’s fine; they can get the settings right much more quickly with the home equipment there.

6. If there is any chance your pALS could be admitted, take your Cough Assist with you. Small town hospitals may not have one; larger hospitals may take days to track theirs down. Same goes for feeding tube supplies and formula.

7. Always have an updated medication list; while waiting for the ambulance, note the date, time and dosage of the last time they received each meds. Send it with them on the ambulance, along with a list of any allergies. Don’t trust that the hospital will have them on file just because you’ve sent them before. Take copies of these with YOU: POLST, Five Wishes, Living Will, Medical Power of Attorney, DNR, etc. I always had the originals and copies so the originals never left my hands.

8. If your pALS has trouble communicating, take any communication equipment with you to the hospital.

9. Don’t be afraid to challenge the ER personnel about what your pALS needs. Ask if they are familiar with ALS. Ask if they have a neurologist, pulmonologist, respiratory therapist available. Give them the numbers of your pALS’ doctors and suggest they call for a consult.

10. If you don’t get what you need, if you are being brushed off, ask to speak to the Patient Advocate or Quality Management team. Often this is enough to get them moving.

Call on your ALS Community – other CALS, widows, family members. Let them know what support you need. There are a lot of experienced folks around. But also remember every pALS is different, every pALS has a different medical history; what is right for one person is not the best choice for another. Trust your gut.

By bansagart, ago

Questions

An ALS diagnosis brings a myriad of questions. Here are just a few.
– What is the prognosis? It is 100% terminal – there is no cure.
– How long do I have? On average, 2 to 5 years from diagnosis. Some last 20 years or more. Some die within one month after diagnosis. People make guesses, or estimates, but there really is know way to know.
– What happens when my tongue and mouth muscles stop working? You will start experiencing “slurred” speech, and eventually lose the ability to speak. You will have options of low-tech and high-tech communication devices, depending on how much use of your eyes, hands, and feet you still have. Mary was able to speak until she had the trach surgery. By that time, she had minimal use of her hands or feet, and the eye gaze devices wouldn’t work for her. We did our best to read her lips to find out what she was saying.
– What happens when my throat muscles stop working and I can’t safely swallow any more? You can keep trying to eat and risk aspiration pneumonia or choking asphyxiation. You can choose to stop eating and eventually not be able to swallow thick liquids either, which will lead to death by starvation. You can choose to get a feeding tube placed through your intestinal wall and into your stomach and receive all your nutrition in liquid form. If you choose to do this, you should do the surgery sooner rather than later, before your breathing is too compromised. Mary had her feeding tube placed three months after diagnosis because her breathing was so compromised that they were afraid she would not be able to come off ventilation after the surgery.
– What about the breathing? In most cases, the diaphragm nerve (the phrenic nerve) is the last to go. In some people, it starts earlier, or even first, like with Mary. this leads to diaphragm paralysis, and the inability to inhale or exhale. The lungs can still exchange gases, but the diaphragm can no longer make the lungs inhale and exhale. Some people have had a DPS, or diaphragm pacing system, implanted. This has had mixed results, and is not usually recommended any more.
– What can I do about the breathing? You can choose to do nothing; you will experience carbon dioxide buildup which will cause loss of appetite, confusion, personality changes, lethargy, coma, death. As the air hunger gets worse toward the end, there are medications that can help make you more comfortable. You can choose to use non-invasive ventilation via a bipap/avaps (similar to a cpap) that will force room air into your lungs with positive pressure and assist with the exhale using negative pressure. You can have a full-face mask, a mask that only fits over your nose, or nasal pillows. Mary used the nasal pillows, since it allowed her to talk and eat and drink while having her breathing supported. Eventually, the bipap/avaps will not be enough, and you will have to decide whether or not to have a tracheostomy and go on invasive ventilation. Mary chose to do that 14 months after diagnosis.

Each of these questions leads to more questions, more decisions, more loss. ALS takes every ounce of strength out of the PALS (person with ALS) and CALS (non-professional caregivers of patients with ALS). It is important to have a support team of medical professionals, therapists and social workers, and spiritual leaders if desired. That will be the topic of another post.

By bansagart, ago

The first time

The first time Mary almost died was January 2014, only a month after diagnosis. We hadn’t told anyone yet about her diagnosis. She had started using a bipap to help support her breathing but wasn’t completely dependent on it. We did not have caregivers in place – we didn’t realize she needed them yet – and I was at work. At about 3pm, she left the bipap in the living room and went to the master bathroom. Getting up from the toilet, she fell. She didn’t injure herself, but she couldn’t get back up off the floor. An hour later, she had managed to slowly butt-scoot her way to the dresser (about 15 feet away) while struggling to breathe, and used her cane to hook her fanny pack off the dresser. Inside the fanny pack was a deactivated cell phone. On the verge of passing out from lack of oxygen, she managed to dial 911. When the paramedics and sheriff arrived, the sheriff had to kick in the door since she always kept it locked when she was home alone. The paramedics carried her to the living room and put the bipap on her, staying with her until her pulse ox was back up to normal range. A little after 5, I called to let her know I was on my way home (like I had done every single day for 10 years), she started with “I’m ok, but we need to get the door fixed”. That was when I realized how quickly things were going to change. I called a neighbor who came right over and fixed the door. The bipap made its new home on the seat of the walker, and I attached it to a 50-foot extension cord so it could move around the house with her. No more going to the bathroom without the bipap. Go to the nearest bathroom, not the one you like the best. Use the walker, not the cane. Always keep your cell phone on you. I began to wonder how to keep her safe while I kept working so that we had a place to live, and I realized whether she wanted to or not, it was time to tell them family.

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By bansagart, ago