Search

Finding Freedom

Living Forgiven and Free

An Open Letter to the ALS Community

I’ve lived past my expiration date and I’ve still got questions.

This month marks five years since my ALS diagnosis with a 2-5 year prognosis. I’ve crammed a lifetime of living, loving, learning, and advocating into those few years and I’ve still got questions.

I’ve met hundreds of patients and their families, caregivers, advocates, medical practitioners, researchers, drug developers, regulators, and legislators and I’ve still got questions.

Last week, I was reminded at a meeting of people with ALS and FDA regulators that we are all one ALS community. I would agree that we all have a common goal of finding an effective treatment or cure. However, I would argue that we look at the problem though distinct lenses. We champion our own point of view and often dismiss, distrust, or disparage community members who view it through a different lens. We disagree on how to reach the goal at best and cannibalize at worst. I’ve got some questions about that.

This problem is not new nor unique to the ALS community. But for now, my life and the lives of tens of thousands of other people currently living with ALS depend upon an immediate solution.

The National ALS Association led an effort to create an FDA guidance document to facilitate the clinical trial and drug approval process. It was presented as a collaborative effort, inclusive of all parts of the community. The voice of the conservative neurological science community dominated the conversation and document. Looking through their lens, it was a big win. Looking through my lens, it tragically meant we support the 12+ year, $2.5 billion, status quo drug approval process. That means the research community keeps their version of perfect science while patients like me are guaranteed to die waiting. I understand their position, they are desperately searching for an effective treatment and are unwilling to change because they want a scientifically proven treatment. They truly believe that is what’s best for patients. Drug developers are looking for an effective treatment as well and their lens includes dollar signs and a highly regulatory environment. The FDA would be thrilled to approve a treatment that has been proven effective by passing the gold standard of trial design. I want access to potential treatments now. My lens is urgency and access, period.

Can someone please draw a cartoon of this conundrum?

Here’s the deal. I, along with many others, have tried to gain traction on pressing for bold changes to this process. Today, I was asked by Sandy, a newly diagnosed woman, “Why isn’t something being done? What can I do to create change now?” My heart sank. If I knew what to tell her, I’d be doing it myself. She is one of the lucky ones — participating in a stem cell clinical trial. But, dear God, the study protocols are nothing short of barbaric. Sandy is voluntarily being denied access to currently approved medication for three months to see if she declines rapidly enough to be included in the study. If she progresses enough, she still has a 50% chance of a placebo being surgically injected into her spine. Here’s the real kicker: If Sandy gets the stem cells and shows improvement, she will not have access to future treatments for years. Barbaric.

They’ve got their conservative science that looks promising. Sandy is a desperate, dying woman. She feels it’s her responsibility to endure this trial to benefit patients in the future. Can someone please explain to Sandy’s family how this is okay? A promising treatment exists but your wife and mother is a lab rat and can’t have access to the treatment outside of a barbaric clinical trial.

Nothing is going to change until the patient community demands it. We have to find a solution that creates a win for those of us living with ALS. Let’s take an honest look through each other’s lenses and find a compelling solution. We need a Manhattan project, funding the brain power and advocacy to make it a reality.

Here are my questions:

Who has the leadership, resources, and influence to create a win-win for the entire community?

What barriers are preventing this from happening now?

I’m sitting in my hospital bed, typing with my eyes. Yet, I believe I can make a difference. What if those with resources and influence acted as though their lives depended upon finding a solution now? The answer is out there. Please help me find it.   

The Surreal Gift

My bed gently rolls me from my side to my back. The familiar hum of the bed rouses me. You know that place, you’re half awake and half asleep? I open one eye, just a sliver, to see if the sun is up. Yes! Time to get up. In my mind, I sit up and swing my legs off the bed. “Steve, you won’t believe the dream I had. I was paralyzed and completely dependent on you for everything. It was crazy!”

My attempt to sit up met with resistance. I’m reminded of the reality, this is not a dream. I feel perfectly fine, not sick, like a dying person is supposed to feel. Yet, I can’t move because ALS is killing off my motor neurons. Surreal.

The Gift

Psalm 46:10 says, “Be still and know that I am God.” I read that and think, be present, let go of results, don’t worry about what tomorrow will bring. Then, I remember Matthew 26, “Do not worry about your life, what you will eat or drink; or about your body, what you will wear. Is not life more than food, and the body more than clothes?” Each faith tradition speaks to being present and at peace. That’s the gift. Let’s practice gratitude and peace, regardless of circumstance.

What’s stopping you? Root it out, let it go. No buts, be still.

I’m Forgiven and Free and still 😉

Top Ten List

I’m not dying anymore. I’ve outlived my 2 to 5 year ALS prognosis and I’m done being sick. I’ll continue to advocate and irritate until we have an effective treatment, but until then, I’m choosing to LIVE without death hanging over my head. I know a band of heroes and sheroes who are showing me how it’s done.

You’ve read my laments; I anticipate mourning losses to come. My life is different than I expected, but it is what it is. I once said in an interview, “There aren’t many people I’d trade places with in this life. I’ve got it pretty good.”

Here’s the top ten list of the upsides of my less physical, more cerebral life. In other words, the benefits of holding still:

10 — Plenty of quiet time
9 — Rarely need an alarm clock
8 — No housework
7 — Preferred parking
6 — No expensive gym membership
5 — Manicure stays perfect
4 — Feet stay callous free
3 — Only need to tan my front side
2 — I’ll never need a knee or hip replacement
1 — Did I mention no housework?

There’s always a bright side. I have my top ten list, the most supportive family and friends, and my faith.

What’s on your top ten list?

Guest Post: Patient Voices Matter

I’m grateful the National ALS Association gave me a platform to be heard.

The Official Blog of The ALS Association

This guest post is from Shelly Hoover, EdD, a member of the Patient & Caregiver Advisory Committee, and does not necessarily reflect the opinions of the Association staff, its Board of Trustees, or its chapters.

By guest blogger Shelly Hoover

Patient voices matter, yet the collective ALS patient community has been too patient and too quiet for too long.

The ALS Association led an unprecedented effort to develop an FDA Guidance Document for Industry. Here’s a quote from the introduction:

“In February of 2015, The ALS Association launched this effort to develop the first-ever community-driven drug development guidance for ALS with funding from the ALS Ice Bucket Challenge, bringing together over 100 participants including people living with ALS, caregivers, researchers, clinicians, and industry experts from across the world to contribute their expertise and experience. In parallel with this effort, a committee was formed to update the ALS clinical trial…

View original post 526 more words

Blessed Are Those Who Mourn

I’ve never known such pain as that of my broken heart, a physical presentation of indescribable grief.

My ALS journey, a treatise on grief, started with the loss of cute shoes, then my career and mobility. Sometimes I threw things in frustration, followed by the snot-faced ugly cry. You know, the cry that mocks your Kleenex.

I adjusted, adapted, accepted, and returned to gratitude. I found a new normal with the help of family and friends.

My arms and hands betrayed me next. Goodbye driving, feeding myself, and giving hugs.

So, I adjust and adapt. Technology helps. Yet, I haven’t found my new normal.

I’m in the throes of grieving and the tears show up uninvited. I keep the grief hidden until it forces its way to the surface, like a foreign body that needs to come out. There’s no stopping it and it hurts like hell.

I mourn the everyday use of my hands, but I’ll adjust to someone else bathing and feeding me. I use my eyes to type and text, hands free. What I’m having difficulty losing, that no one and no technology can replace, are the simple pleasures of teaching my grandchildren. I’d dreamed of being like my grandmas. One taught me to crochet, and I picked cucumbers with the other. We baked and shopped, read and played games. Above all, I felt their love.

With my grandchildren, I want to play silly duets on the piano. I want to make sandcastles on the beach, cookies in my kitchen, and strings of popcorn and cranberries for the Christmas tree. I want to play hide and seek and tickle their tummies when I find them. I want to hug them and put Band-aids on their knees. I have to accept the fact that I never will.

What I fear the most is being a disengaged grandma that isn’t able to show my grandkids how much they are loved and adored. I fear missing out. I’m profoundly sad and know I have to grieve before I can move past this.

I share my pain and struggles in this public forum because I promised to be authentic and transparent on my journey. I know it’s hard for you to read this. Please don’t feel sorry for me. Compassion is welcome, pity is not.

So, what now? Have you lost someone or something important? Allow yourself to grieve, it’s part of the healing process. Do you have a friend going through a difficult time? Come alongside and sit with him or her through the silence and tears. Don’t say or do anything to fix it. Just be there.

My tears will stop. My family and I will find a different way for me to stay engaged. I have no doubt. I will adjust and adapt. Eventually, I will accept it.

I’m Forgiven and Free and on the road to gratitude.

Get On Board or Get Out of My Way

I’m done playing nice.
I’m supposed to be dead.

This is an urgent call to action. Keep reading.
I need your help! 

With an ALS diagnosis in 2013, I was given 2-5 years. I’m alive but being protected to death by the FDA, and I’m not alone. Every 90 minutes someone is diagnosed with ALS and every 90 minutes someone dies from ALS.

The 2014 Ice Bucket Challenge raised over 100 million dollars for research. Today, promising treatments are sitting in the FDA pipeline. It takes decades and billions of dollars to get an effective treatment approved.

We’ve played nice and we’ve been ignored.

Two years ago, the National ALS Association (ALSA) wrote an FDA Guidance Document that is being lauded as the gold standard of industry guidance. Half of the 14 patients on the guidance committee died waiting for public comments. The FDA just released Its version of ALS Industry Guidance. I, the remaining committee members, and thousands of others will die waiting because the guidance does very little to advance treatments through the lengthy and costly approval process.

The voice, concerns, and suggestions of patients and our allies were largely ignored in the original guidance and our Call to Action was buried in the very last pages of a 139 page document. We had high hopes that the efforts of so many would dramatically impact drug trial design and approval process. Our lives depend on it. Nope. The new and exciting guidance from the FDA Includes: patients with ALS are willing to take greater risks because the condition is terminal. Thank you, Captain Obvious.

I’m waiting to test the California Right to Try law or use the FDA’s Expanded Access Program when a new drug I’ve been following passes Phase 1 safety and tolerability studies. There is no requirement for the company to give me the drug, only a moral imperative. I gladly spent a total of six weeks in the hospital and dozens of appointments in San Francisco. I have great hope that this new molecule will dramatically slow my progression or stop it all together. The trial is still in Phase 1 two years later. Waiting those two years cost me the functional use of my hands and arms, it cost other people their lives.

We are not asking for a safe and effective treatment for erectile dysfunction. We are demanding access to potential life-saving treatments that are stuck in a 15 year, billion dollar approval process. Answering FAQs and minor tweaking don’t come close to the paradigm shift required to save lives today. Stem cell therapies and promising treatments exist but are not accessible.

ACT UP seized the FDA 30 years ago with the same obstacles facing people with HIV/AIDS. It’s time for us to do the same. Most with ALS can’t march or yell because many are paralyzed and can’t speak. But we have assistive technology and 400 pound wheelchairs. Get on board or get out of our way.

Urgent Call to Action

  1. Immediate access to promising treatments that have passed safety studies.
  2. No placebos in clinical trials, use historical controls instead.

Stop protecting us to death. The cost of a military parade could provide life saving treatments to every American living with ALS. US veterans like me are twice as likely to have ALS. It’s time for the US Government to cut through the regulatory red tape of the FDA and provide life-saving treatments now.

What’s Next?

The patient community has the right and responsibility to get loud and demand our voices be heard. We need 1,000 public comments to get the FDA’s attention. You can help by leaving a public comment of your own. Click here.

Tell your story and demand access to treatments and radical changes in how ALS drug trials are designed now! Status Quo = Death.

It’s easy to comment. Here’s how:

  1. Click here to go to the public comment website.
  2. Type your comment. Note: It’s best if you write your own comment, but here are some suggestions on what to include:
    1. Your relationship to ALS (patient, loved one, friend, etc)
    2. A statement about how this guidance document is unacceptable. For example: The proposed guidance document doesn’t adequately address the urgent needs of patients who have a terminal illness – especially an illness without an effective treatment. It is cruel to withhold access to treatments that have been proven safe and show promise, but are stuck in a 15 year, billion dollar approval process. It is time for the FDA to stop protecting ALS patients to death. ALS patients need immediate access to promising treatments that have passed safety studies. ALS clinical trials should NOT require placebos and use historical controls instead.
  3. DO NOT put your name and contact information in the comment as it will become part of the public record. There will be a place later in the process to put this information. It is optional and will not become part of the public record.
  4. Uncheck the checkbox that says: I am submitting on behalf of a third party.
  5. From the drop-down category menu, choose: Individual Consumer
  6. Your comments won’t post until they have been reviewed by the FDA, so don’t be surprised if you see zero comments posted.

Make your public comment and share this far and wide. Thank you.

If you’re having trouble submitting a comment on the above link, try here and click the green button. Note that the 270 comments listed refer to a previous document from 2013.

If our voices are dismissed again, we will show up at their door demand action. Imagine 250 power wheelchairs at the FDA headquarters. Here’s how ACT UP did it 30 years ago.

***

Learn more about ALS

Let me know if there’s anything you need!

This is for people who are going through a difficult time and could use the help of your community or for people who know someone who is.

First, let me talk to the people who are going through a life changing crisis. I get it. I have ALS, a fatal neurodegenerative disease that has turned my life upside down. I’m a proud woman, my husband and I have done everything on our own. We don’t need help. Have you told yourself a similar story? I don’t want to bother anyone, we’re doing okay. I know that story too.

Three words: get over it. Three more words: you need help. Here’s another way to look at it. Remember how good it feels when you help someone else? Well, don’t be a jerk and deny your friends that experience.

Now for the friends who want to help but don’t know what to say or do. The worst thing you can say or do is nothing at all. I know it’s hard. It’s awkward. Here’s a start, call or text your friend and arrange a time to visit and say, “Good to see you!” That’s a better greeting than, “How are you?” Save that for later. If you don’t know what to say, be honest. “This is awkward, I don’t know what to say. But, you’re my friend and I’m here for you.” Love your friend enough to get over your uncomfortable self.

With all good intentions you may say, “Let me know if there’s anything you need.” I know you mean it. I’ve said it too. But the problem is, it puts the responsibility on the person to come up with something. And, it’s difficult to accept help so you probably won’t get the text asking for it.

The Practical Stuff
Everyone and every situation are different. A person recovering from chemotherapy has different needs than a person who has lost a loved one. Yet, some of of the social and household needs are universal.

Social Needs

  1. Text, send a card, or call. Let him or her know you are thinking about them. Feeling forgotten or isolated is awful.
  2. Arrange a visit. You don’t have to stay long. Watch for cues. If she’s falling asleep, take the hint. If he’s not feeling well, don’t take it personally. The important thing is that you showed up.
  3. Keep texting, stay in contact. Even if you don’t get a response, stay connected.

Household Needs

  1. When you stop by for a visit, take a look around. Is the yard a mess? Tell him you’ll be there Saturday morning with a yard crew. If it’s a prolonged issue, rotate with some buddies and take care of the lawn for a few months.
  2. Visiting for a few hours? Check the laundry basket. Tell him you’ve got time to wash, dry and fold a few loads.
  3. Bathrooms gross? Grab the cleaner and clean it. Bring a friend and clean the whole house.
  4. Food, everyone appreciates food. Be the point person and email coworkers and friends, share preferences and restrictions. Bring freezer-friendly meals with the appropriate number of servings in disposal containers. Label the lid with what’s inside and the date.
  5. Don’t forget the spouse, they are carrying a huge burden. Get them a gift card for their favorite activity and offer to stay while they go out. For example, my husband loves to golf, but feels guilty leaving me alone. So, you can come hang out with me while the guys go Top Golf. It’s a win-win.
  6. Are the kids going bonkers? Take them to ice cream or a movie. Dog getting ignored? Take her to the dog park. Notice the dog food is running low? Pick up a bag.  

Personal Care. I can only speak for myself on this one. Well, because it’s personal. You will know what your close friend needs.

  1. Arrange for a pedicure. If you’re friend can’t leave the house, bring the pedicure to her.
  2. Pluck her unibrow or Nair her legs.
  3. If you’re a natural caregiver, learn the morning and/or evening care routines and take over for a day.

I can’t speak for everyone in need. For me, it’s hard to ask for or accept help. But, I know I need it. I’m probably not going to ask, so just do it.

What should you do to help a family who needs a hand?  Consider what you like to do — cook, pray, fix things, sit and visit? Do something you enjoy and it will be a natural extension of yourself. The most important things are to do something and find a way to stay connected.

I’m Forgiven and Free and grateful I have friends who show up and help me.

Life or Death

Living with ALS presents challenges and choices, ranging from how to safely get through a morning routine to deciding whether to live with mechanical ventilation or die. Shakespeare was right; To be or not to be, that is the question.

I’ve been weighing the pros and cons of getting trached, that is to get a tracheostomy and use invasive ventilation to breathe for me when my diaphragm and intercostal muscles grow too weak.  I haven’t decided what I’m going to do when that time comes, but I think about it more than I care to admit. 

Pros: stay alive, see my grandkids grow up, continue to enjoy my family and friends.

Cons: 24 hour carE, painful transition to a new normal, greater burden on my family.

People with ALS in the U.S., who are not veterans, also must contend with exorbitant medical costs. In countries with universal health care, prohibitive cost is not typically a factor. For example, In the U.S., five percent of people with ALS choose to extend life with a trach; In Japan, where they have universal health coverage, 80 to 90 percent of people get a trach. Let that sink in.

Yes, I have personal knowledge of friends with ALS who have chosen to die because their families could not afford the cost of keeping them alive. Others chose not to extend their lives because the are tired of suffering which is completely understandable. No judgment about that here. My point being, it is unconscionable that we, one of the richest countries, don’t adequately take care of our children and people who are elderly or disabled.  Downright shameful.  I didn’t realize the magnitude or consequence until I was one of them. 

To live or not to live. That begs the question, what defines being alive? Heart beating, breathing, communicating, walking, working, playing? It’s relative.

Let’s switch it up a little. What makes you feel alive? What’s your purpose? What brings you joy? My answers are changing, paring down, as I lose physical function. Peeling back the layers, I’m left with what’s truly important. Family and friends, loving, encouraging, supporting in good times and bad, that’s what makes my life worth living. I want to make a difference. 

Are you living? I  mean living, not existing. Get excited, set a big, hairy, audacious goal and make it happen. Volunteer. Bless one another. Get busy living the one life you have.

I’m Forgiven and Free and living the life given to me

Checking In

Unpublished blog posts sit abandoned in my draft folder. One is bitter;  Another begs victim hood. That is not what I want to put out into the universe. So here I sit, gazing at my keyboard, trying again. I’ll just give it to you straight.

Physically
The functional use of my arms is gone. That means I can still use my arms to knock things off the counter, but that’s about it. If we meet for lunch, you’ll have to feed me. Don’t worry about the mess. I’ll have a color coordinated bib, with a convenient Velcro closure,  neatly stowed in my pee bag.

The real bummer is that my progression has reached my mouth and throat. The changes aren’t noticeable unless you live with me. But the signs are unmistakable — excess saliva, choking on air, hoarse, reduced voice volume, muscle spasms in my throat and neck. And when I’m tired, I sound like #45 with dry mouth, “God bless the unitshed shates of amershith.” I have an appointment at the multidisciplinary ALS clinic next week and will meet with the speech the pulmonary therapists. I had been hoping for an effective treatment before I reached this point, but it is not meant to be.

Emotionally 
I’ve been struggling.  My emotional bandwidth isn’t there as I’ve switched from waterproof mascara to no mascara at all. Two local friends with ALS died this week. Tammy was the second woman I’d met with ALS. I was drawn to her gentle spirit, strength, and endless optimism. JT had unmatched charisma and charm, and a wicked sense of humor. Both were deeply loved by family and friends. Coping with my own loss and sadness for Tammy’s and JT’s families is overwhelming.

Spiritually 
I’m at peace with my maker yet miss being part of a faith community. I’ll add this to my New Year to do list.

Return to Gratitude
There’s a time to grieve and a time to live. I do both with profound gratitude and peace in my bones.

What are you grateful for today?

I’m Forgiven and Free and grateful I get to try a recently approved medication that may slow my progression!

radicavapic

Blog at WordPress.com.

Up ↑

%d bloggers like this: