ALS Advocacy Day

IMG_0270As my husband and I prepare for a trip to Washington, DC, for the annual ALS Advocacy Conference, I sit here and contemplate all the things that had to be planned in advance to make the trip comfortable and enjoyable for both of us. The things that used to be taken for granted are much more complex.

The conference itself is pretty expensive. Last year it cost us more than $3,000. This time we decided to forego staying at the hotel where the conference is being held. We also rejected two other nearby hotels that are accommodating conference guests. We opted for a hotel nearby that was less expensive and listed as one of the best ADA rated hotels in DC. It included a hot cooked made-to-order breakfast; my husband will appreciate not having to run around the city in the mornings. There was an evening cocktail reception which was nice.

The hotel change resulted from the reluctance of the three recommended hotels to accommodate my request for a hospital bed. Oh, my mistake, the conference hotel would allow the bed as long as my caregiver, that would be my husband, had his own room. Makes sense for a semi-paralyzed PALS (Persons Living with ALS) to be alone in their room all night, right? Maybe that was because the rooms cost $399 a night – the reduced rate!

So now that my chosen hotel was four blocks away, I knew I would have a problem when it was time to use the bathroom. So I asked the National ALS Association if they could ensure the availability of a Hoyer lift for me and other PALS. That did not happen for a variety of reasons, none of which I fully understand. So, for the comfort of my caregiver and myself, I decided to go ahead and get a Foley catheter. I am not really thrilled with another tube coming out of me. The ALS Association graciously provided an aide for the duration of my stay.

We left Friday, May 11th for Washington, DC with the van packed like the cargo bay on an aircraft. We packed the van with all of my machinery (cough assist, suction, trilogy, and Hoyer lift) and my custom shower chair. My husband also took along the pile of pillows I sleep with every night. The drive started off with me feeling uncomfortable seated in my power wheelchair (PWC) which required a quick stop to shove a pillow under my right side and remove my leg braces and shoes. We made it a little over halfway when I needed to escape the van. I took a break to tilt my PWC back and shift weight off my back side and raise my legs. A very necessary break for comfort and to keep any more blood clots from forming.

Soon we arrived at our hotel and it was time to unpack the gypsy van. The hotel staff were super friendly. Our handicap room had double beds. Unfortunately, the beds were on platforms and the Hoyer would not be able to get beneath the bed. The floor was carpeted, too, which makes moving the Hoyer very difficult. (The Americans with Disabilities Act really needs updating.) My bed solution was the pull out sofa in the other room. Hubby’s addition to the solution was to drag the mattress from the bedroom and put it on top of the sofa bed. I felt like a princess in search of a pea. The next day we rented a hospital bed for the rest of the stay. Minimum rental is a month; the cost is not pro-rated.

Saturday, two of our besties traveled from New Jersey via Greyhound to visit for almost three hours before turning around for another four and a half hour ride home. Part of our hearts went with them but it was so worth it! Just before they arrived we were surprised by a call from my cousin Mike. He drove up from Virginia for a visit just like the year before. We are blessed by friends and family.

Sunday we got down to business – the National ALS Convention. We had a meet and greet all day up until the welcoming dinner. The following day we received a lot of information relating to current research. There was a panel with a researcher, doctor, and clinician. It was exciting to hear researchers say that they feel they are close to solving the riddle of familial ALS, maybe even by 2019! Ten percent of people diagnosed with ALS have the familial form. The rest, myself included, have the sporadic form of the disease.

I spent a lot of time during the lunch break with the convention exhibitors, two in particular. They were Biohaven and Tobii Dynavox. My husband and I had traveled to DC in March to take part in a patient advisory panel for Biohaven. They were writing a protocol for FDA approval of a new sublingual form of riluzole. I am happy to tell you that I wrote a letter to the FDA in support, and it has been approved for expanded access prior to clinical trials. This means that it is available to PALS now, before final FDA approval, but PALS must ask their doctor to complete the necessary paperwork.

Later in the afternoon, we met with representatives of our local ALS chapters for a strategy session. We went over what we would be discussing Tuesday on Capitol Hill with our senators and representatives. I had a script prepared in advance that I would read with my Tobii.

Tuesday morning traffic was a nightmare on the Hill. It was law enforcement week and the President was addressing the police. There were police walking, biking, and riding motorcycles everywhere. It was a hot day and we had a long walk from our shuttle bus to the Senate office building. Every year walkers are advised to wear comfortable shoes.

We met with staffers for Senators Burr and Tillis for about fifteen minutes. Senator Tillis was tied up in committee meetings most of the day and could not meet with us. When Senator Burr arrived, we told him what our “asks” were and then the individual PALS told their stories. I was first. I spoke about my ALS journey so far and asked the Senator to support legislation eliminating the five-month waiting period for social security disability benefits. He assured us that he was in favor of the legislation and was a co-sponsor. Next we visited the House office building.

 

 

The House and Senate offices are connected by an underground tunnel. It is a bit of a walk between buildings. If you are lucky enough not to be in a wheelchair, you can ride a tram between buildings. We visited the offices of four Congressional Representatives and met with their staff people. In addition to presenting our four asks, we requested that the representatives and senators vote to approve a bill that would award the Congressional Gold Medal to Steve Gleason, a fierce advocate for ALS rights.

It was a long and exhausting day. And I violated one of my own ALS cardinal rules, not to do stupid stuff (thereby risking my own personal safety) by agreeing to walk, and not ride the shuttle, back to the hotel. Okay, I don’t really walk, but the trip back was the longest half mile for my PWC and me. When I entered the hotel lobby, my battery was at 3%. I prayed that I would make it from the lobby to my room on the 12th floor. As I entered the room, my battery read 0%. That was the same way I felt, drained. I was so tired that my husband got me undressed and put me to bed before the aide arrived at 6 pm. I slept straight through to the next morning.

ALS Advocacy is important. Next year there will be new PALS fighting for a whole community of people who are either dying or being diagnosed every 90 seconds. Please give us your support.

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Bitching & Complaining

bitchingYes, you read that right. This blog is about bitching and complaining. It is mostly about ALS, the bitch in my life, but a few other things may slip in. I am after all a born and bred New Yorker, so it’s a given that I am going to complain. If you don’t like complaining, don’t read any further. I am not about to change now. There are lots of things to complain about with ALS, and I’m taking the opportunity to put it all out there.

Disclaimer: These complaints are not directed toward any specific person, but even so, names will be changed to protect the guilty.

Communication
This is a big one. It may actually be the only real issue because not being able to express even the smallest desire is a huge frustration.

I live with my husband who has hearing loss and wears hearing aids, most of the time. So communication has always been an issue. I was always being reprimanded for talking to him from another room instead of face to face. That was a difficult thing for me to get used to since he was the first hearing impaired person I really knew. Heck, in my childhood home we would yell to each other from one floor to another. So, of course, it’s Murphy’s Law that I should lose the ability to speak and make our communication even more difficult. I spent about six weeks reading 1600 nonsensical phrases so I could have a synthetic voice that would sound like me. I now have a speech device that Medicare paid $16,000 for, and guess what? He can’t understand my synthetic voice on it. Murphy, again.

We used to say that as we got older, we could learn sign language to add to our ability to speak to one another. Damn you, Murphy. I can’t use my arms and hands very well.

I have my speech device which is a Tobii Dynavox. I just call it my Toby. I took it with me to the hospital where no one had the time to wait for me to type my responses. So I was essentially voiceless. I take the effort to practice so I can be faster at communicating and there is always something to annoy me. For example:

1) Toby quits in the middle of a sentence I have been painstakingly writing;
2) People are on to another discussion before I can fully respond;
3) As I type sometimes people will read the screen and try to anticipate what I am saying (sometimes this is OK, but it takes away privacy of prior conversations because of its predictivity function. I really don’t want people to see “I need the bed pan” when I was writing “I need some help.”)
4) I attempt to use the dwell-free keyboard which is faster and doesn’t appear on the screen until I want it to, but if I make an eyegaze mistake, it takes longer to correct and, therefore, longer to speak;
5) People don’t really understand what Toby said, but don’t bother to ask for clarification. To me this seems like they are not interested in communicating.

The worst times for me are when I am asked questions that require more than the nod of my head, which I can still do right now, and Toby is packed away or out of my sight. If I notice something important or need something, I am helpless. These instances occur frequently during bathing and dressing.

Sensations
Constantly feeling itchy is an extreme annoyance.  I cannot reach very far so lots of these feelings must be ignored. Mind over matter, or in this case itches, does not always work. I can feel loose hairs on my skin like bugs crawling on me. My pleas to remove them bring snickers from Barney. A real annoyance.

Burning sensations from pressure occur at times. I feel the burning in my feet at least once again daily. I used to experience burning in the heels of my feet almost every night. Fortunately, this situation seems to be under control with the use of many, many pillows. In fact, our bedroom has become overrun by pillows.

PBA
PBA or pseudo-bulbar affect is the nemesis of my relationships. I know many people are put off by it. It is hard on me and hard on people who are witness to it. I was never an emotional person and now I start crying at the drop of a hat. Sometimes it’s because I’m upset or frustrated. At other times I’m just overwhelmed by the emotion of the moment. Thankfully, medication has helped control it a lot, but a little bit lingers behind. It makes it hard to breathe. I hate it!

Relationships
Where have some of those old relationships gone? I’m dying and you can’t say you’re sorry? Did I piss you off? Let me know if you want to reconnect. I can apologize too. I will be dead a long time and I won’t be back.

Hospice
I have now signed up for hospice. It should begin this week, right in time for my trip to Washington, DC, for ALS Advocacy Day. Hospice isn’t a prison. They actually expect me to live my life before it’s over. How long I will be on hospice will depend on my respiratory system. While on hospice (which is at home by the way) I will get a weekly visit from a hospice nurse and 2-3 CNA visits a week. I will also have a case manager. Medications will be monitored and provided through hospice as well. When I travel, I get information on a local hospice to contact, if necessary. If you have ALS, look into palliative care or hospice, and find out when it is appropriate and how it works with the insurance you have. It can give your caregiver some help.

Getting hospice on a predictable schedule seems like that will be my next complaint. Can’t things be planned in advance? Don’t call at 3 pm to come give me a shower. I like to plan things ahead. Most of my funeral plans are finalized. There’s just one date I can’t schedule. So get with the program, already! Please and dammit!

The lazy blogger

IMG_0214IMG_0194This is what I am calling my lazy blog.  It has taken me quite some time to get motivated to write, and I feel as if I have been working on it forever.  I am not sure why it has taken me so long to get serious about it, but it might have something to do with some of the subject matter and the fact that I have been busy working on the ALS Walk.

Saturday, April 21, 2018, was the ALS Walk in Raleigh. It was lots of fun and I was excited to see friends and family who went out of their way to be there to support me. It was a fun time and together we kicked ALS in the butt. We raised over $9,200 and won the award for the best t-shirt design. Being Team Captain and a PALS did not work well for me.  It is very tiring. So a BIG thank you is due to my Team, including Alston & Bird, NCCU and Alpha Phi Sigma Criminal Justice Honor Society, PatientsLikeMe, and Woodlake Cares 4 Kathryn. I had tons of help from friends like Alisa, Ericka, and Eileen, who kept me sane. A special shout out to Maria! Thank you one and all. If I forgot to mention anyone, I apologize.

I had my three month follow-up visit at the ALS clinic on Tuesday before the Walk. I was surprised to find out that I gained back 5 lbs. eating only liquid nutrition through a tube. I am a lot weaker in my arms and my breathing has dropped again, but I expected this news. You see, we have been wrestling with the possibility of transitioning from palliative care to hospice care since the previous clinic visit in January. Hospice was recommended by the doctor and the palliative care nurse.  It was not an easy thing to hear at first. My husband and I mulled it over for a while. At my last visit we were ready to discuss it again with the ALS doctor I have has from the start. It was, as my husband put it, “the elephant in the room.”  We are now hospice “shopping.”

Hospice offers more services but takes away life-prolonging medication like my ALS medication. Initially, I was surprised that they would do that, but I guess that is the point of hospice. Hospice supports you at the end of life so there should be no need for life sustaining medications. I don’t feel like I am about to die, but it is not something that anyone can predict.

So, in the meantime, I am planning on attending ALS Advocacy Day in May. It is costly and all out-of-pocket. If you believe in fighting for all things ALS-related, perhaps you’ll consider making a contribution to the GoFundMe page we set up for the trip: Advocacy Day

This year at Advocacy Day there are four legislative asks. Three of these asks involve funding for existing programs. First, for the ALS Registry at the CDC; second, for the Department of Defense ALS Research Program; and third, for the National Institute of Health, particularly the National Institute of Neurological Disorders and Stroke.  The fourth ask has, to my knowledge, been requested more than once. The fourth seeks to end the five month wait period before ALS patients can receive social security disability benefits.  A diagnosis of ALS automatically qualifies a PALS (person with ALS)  for benefits, but for many the need can be immediate.

The five month wait is, in reality, six months before the first benefit check is received. Some people cannot get by for six months without benefits for a variety of reasons. Even though some PALS may have other money coming in, they could require funds to make their home accessible, among other things.  I have heard of PALS who do not even survive six months.

As for me, I stopped working three months after my diagnosis.  Very soon thereafter I was forced to move from a house I purchased less than a year before. I could no longer climb the staircase to my bedroom. The new home was one level but still  required accommodations.  I wasn’t even able to climb the five front steps without assistance.  Six months post-diagnosis we were already discussing the necessity for a power wheelchair.

Finding a cure for ALS would undoubtedly help people with other neurological diseases.  ALS is one of those rare diseases many people know little about, and many people have never heard of it and how it affects individuals and families. those people include congress people and senators.

So even with hospice ahead of me,  I will continue to advocate for myself and others who will come after me.  Help me by raising ALS awareness.

The Transformers

300px-PrimeBeeAs time goes by I feel more and more as if my daily existence is machine dependent.  I am not talking about the usual home appliances.  I am referring to the multitude of equipment that an ALS patient relies on to make their life comfortable and livable.

Since my last post two weeks ago, I have been inundated with appointments.  One of the first was with EMS who arrived to help get me off the floor when I slid out of my lift chair.  I know, not exactly a planned appointment.  I had a new aide filling in for the day who wasn’t familiar with my routine.  I wasn’t hurt, but it did take quite a while for EMS to arrive which was disconcerting.  I was very surprised to learn that the paramedics are not trained on how to use a Hoyer lift.  My aide, however, was able to instruct them and they all worked together.

This incident has made me even more impatient waiting for occupational therapy (OT) to arrive.  You see, it’s time to give the hospital bed another try.  We’ve already dismantled our guest room to accommodate some furniture that will need to be moved out of the master bedroom.  The electric hospital bed is coming back into my life because I have become weaker.  The electric Hoyer will help get me into bed and the occupational therapist will be our instructor.  Then the battle to get comfortable will begin anew.  It’s a real challenge keeping ahead of the constant changes that are the life of a person living with ALS (PALS).

So, in addition to the electric hospital bed and lift, I had a visit from my durable medical equipment supplier who brought me a Trilogy machine and a cough assist machine.  You may be familiar with the terms CPAP and BiPAP.  Trilogy is similar to a BiPAP machine.  It helps with respiratory issues.  In my case it will help inflate my lungs and perhaps increase my lung capacity, but it will also help me get rid of the carbon dioxide that I cannot expel on my own.  It is difficult to expel carbon dioxide when the respiratory system is compromised by ALS.  Too much carbon dioxide can kill me.  It is also one reason why I keep an ALS alert sheet hanging in my kitchen.  It alerts EMS not to give me 100% oxygen.  I found this information at the site www.alsemergency.info and ran it by my doctor before posting.  Now both my husband and I are masked sleepers.

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The end of the month brought the much-awaited speech device, a Tobii Dynavox.  The Tobii uses eye-tracking so I can type and surf the internet hands free.  There were a few hiccups with the delivery like missing mounting poles for my wheelchair and several attempts to retrieve via email the information to unlock Windows.  But all is good and the sales rep is affixing the mounting bracket as I type this.

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I will be spending a lot of time getting up to speed typing by eye and learning Windows again.  Ugh!  All I need now is an appointment-free day, week or month.

In the days that followed Tobii’s arrival, I saw a palliative care nurse practitioner and had a physical therapy (PT) intake visit.  I’m already scheduled for PT twice this week.  My life and home are in shambles.  I have boxes everywhere (empty device boxes, liquid formula boxes), an empty guest room, dust bunnies.  Well, you get the idea.  OT where are you?  I need you so that decisions can be made about where to put everything.  It was very difficult to move to a smaller home just to accommodate ALS.  Adding more stuff to the mix does not help.  Being a little bit of a neat freak (which I didn’t think I was, but have been accused of being), all of this drives me nuts.

The transformation to a machine-filled ALS life began with a power wheelchair.  Now it has expanded to include the Amazon Alexa to turn on and off lights and other cool stuff, a Ring doorbell, cough assist and Trilogy machines, a suction machine, an electric Hoyer lift, electric hospital bed, and my speech device, the Tobii Dynavox.  Half of these machines I am not able to operate alone or unaided.  I thank God every day for the assistance of able and caring people who transform my life into a comfortable existence.

Life with a feeding tube

imagesSome of you reading this post may have a feeding tube, but I’m betting that most of you do not.  Three weeks ago I went for surgery to have a G-tube inserted.  It required an endoscope down my throat to locate my stomach.  I thought that would be the worst of it. Turns out that was the painless part.

We arrived at 10 am for a noontime surgery.  This was only my second surgery.  The first was 1962 for a tonsillectomy.  The waiting room for surgery stretched out the length of a football field.  I was not optimistic about getting in on time.  Of course, I was mistaken. The whole process ran like a well-oiled machine.

First I met the nurses and importantly the hoyer lift expert who got me from wheelchair to hospital bed.  I met two doctors on my surgical team and then about five minutes before noon the surgeon came to greet me.  I was taken into the operating room (it’s big!) and they explained a few things to me, including that they were restraining my arms.  A bite block was inserted into my mouth for the endoscope and soon I was knocked out.  The doctor, true to his word, paged my husband 15 minutes later and told him the procedure was done.

I woke up to pain and a new body part — a flexible tube from my stomach which exited under my left breast.  This was definitely not the optimal location I was hoping for.  The pain was pretty bad and I asked for pain medication three times.  Someone told me it was comparable to be stabbed in the stomach.  Duh!  I was miserable for a week.  Now and then I took over-the-counter pain relievers.  At night I had stomach spasms which woke me up.  I constantly complained that I thought the tube was too tight.  I joined a group on Facebook for people with feeding tubes to learn about their experiences.  Finally, eight days later I returned for a follow-up visit with the surgeon.  I was ready to tell him this was the worst pain I have had to date with ALS.  I was not a happy camper and I had some questions for the doctor.  Fortunately, my husband and my inability to speak coherently prevented a verbal assault on the doctor.  Of course ALS is the culprit, it has ruined my ability to eat and swallow.

Once the stitches were out and the tube loosened a bit, I returned home.  The spasms abated to just one or two.  I was overjoyed because I thought that waking up in pain was going to be my new reality.  By the second night, the spasms were gone.  I spent the first few days trying to figure out what to do with this new dangling appendage.  It felt itchy and bothersome at the site.  Now, almost a month later it doesn’t really bother me.  It’s just there.  I don’t use it for feedings except an occasional Boost or Ensure when we are in a rush. The feedings work by gravity, no pumping needed.

So why did I get the tube if I don’t use it?  It was because with ALS my forced vital capacity (lung capacity and breathing level) is slowly decreasing.  If it fell below a certain level, the surgeon would not perform the surgery.  If I wanted nutrition, I needed to do it now.  So it was a proactive decision.

I tuck this new appendage into a feeding tube belt I wear over a man’s tank t-shirt.  The t-shirt helps my sensitive skin from being bothered.  I’m working up to putting on a bra again.  I don’t know if the tube placement will allow it.  So at present the surgery has made me a bra-less rebel.  I guess I can live with that.

Tomorrow

alg-annie-comic-jpgTomorrow will be another new beginning in my ALS journey.  I will be getting a feeding tube.  The PEG procedure is only supposed to last 15 minutes.  I’ve heard from people who have them and it doesn’t seem to be a big deal.  I think I am looking forward to it in some ways.

PEG stands for percutaneous endoscopic gastrostomy, a procedure in which a flexible feeding tube is placed through the abdominal wall and into the stomach. PEG allows nutrition, fluids and/or medications to be put directly into the stomach, bypassing the mouth and esophagus.  (ASGE, 2017)

Taking medicine should be easier.  I can put it right in the tube.  It’s definitely better than coughing and/or choking for 15 minutes trying to clear my throat.  I will be able to talk and eat at the same time.  Talking by other means than my mouth though.  I can even sleep and eat!  I want to see you non-PEG people do that!

I am not usually surprised by much in life.  My husband has been trying to see excitement in me for a long time.  It’s just not me.  I’m sort of a matter-of-fact person.  When I was diagnosed, there were no options.  So why get overly upset or excited?  I had to accept it.  And even though the feeding tube is an option, it’s one that I will accept.  It’s my way of not giving into the monster that is ALS.  Believe it or not, there can be more to life than eating or talking.  I’ll let you know what more there is in future blog posts.

In the meantime, I will try to take in all in stride.  At this point in my journey I think all the hard work is falling on my caregiver, Joe.  I see his struggle as a labor of love, but labor just the same.  There is no road map to follow.  Everyday with ALS is a new adventure.  A new attempt to rip his heart out.

The sun will come out
Tomorrow
So ya gotta hang on
‘Til tomorrow
Come what may

(excerpt from Annie – Tomorrow lyrics)

 

References

ASGE, (2017), Understanding Percutaneous Endoscopic Gastrostomy (PEG), retrieved December 11, 2017 from https://www.asge.org/home/for-patients/patient-information/understanding-peg

Annie – Tomorrow lyrics: MetroLyrics, (n.d.), retrieved December 11, 2017 from http://www.metrolyrics.com/tomorrow-lyrics-annie.html#/ixzz50zYzYehQ

On the Road Again

imagesI’m on the road again, literally and figuratively. I’m on the road to vacation and stuck in traffic on I-95. Then last week we hit a roadblock when it was announced that two ALS drug trials had failed. One of them I had taken part in for more than a year. So after some time being stalled at the gate, I’ll hit the road again looking for another trial to take part in.

The past few weeks have been pretty well trafficked with people, appointments, and just things to do.

At my last clinic visit I was diagnosed with a probable case of thrush. Another bump in the road. Thrush is a problem usually found in infants who are bottle feeding. So it fits right in with my scenario of wearing bibs, eating toddler food as snacks, and requesting to be taken to the bathroom to tinkle. The instructions on the medication say to gargle and swish four times a day. Um, that’s not happening. Swishing, gargling, and even spitting have gone by the wayside.

On a Tuesday early in the month I had a massage. My massage therapist comes to my home. After moving the furniture around in the bedroom, my aide skillfully gets me into that hospital bed I never use. I get a full body massage even though I cannot roll over. It is a relaxing hour and one that my immobile muscles appreciate. I only wish it was a covered medical expense and could be twice as long and more frequent. If anyone out there has a foot fetish, I have two feet you can massage whenever you want.

Later that afternoon I had an appointment for a telephone conference with researchers wanting to learn more about a PALS’ point of view of their disease. Now, if you’ve been following my blog, you know that I have come to dislike talking on the phone. It takes up a lot of energy and the majority of people I talk to don’t understand what I’ve said anyway. It’s very frustrating for me.

I planned the call late in the afternoon so that my husband would be home to act as my interpreter. As it turned out, he was stuck in a meeting and would not be home in time. As the clock ticked past the arranged time, I realized that the researchers had called but my phone was still set on do not disturb for my massage earlier in the day. Another road bump. I called back and managed it with some repetition necessary on my part. The call was tiring but worthwhile. Anything that drives research along is, in my opinion, worth the time.

The next day I had a wonderful visit with two friends who flew down from New York for the day. We all worked together in Intellectual Property in New York City. Ironically, I worked in the Life Sciences end.

That week wore me out and then it started all over again with a mammogram on Monday where the biggest impression I made was with my power wheelchair. Did you know you can have a mammogram in a wheelchair? And it doesn’t have to be the Rolls Royce of wheelchairs, as the ladies dubbed mine.

Wednesday evening I had a follow-up visit by neighbors who are members of our community outreach, Woodlake Cares. They are researching ways to help Joe and me. Together with my neighbors and my church family collaborating it will all get figured out. Love abounds!

And now the time has finally arrived to get on the road to another new adventure — learning how to use an eye tracking system. The system will be my voice. It will replace my computer and if I get good enough at it, I may be back to talking on the phone.

ALS is sucky and makes life difficult. It plays with our bodies and tries to make us give up. I have many frustrations that don’t always make it into this blog. But it’s important that we find the right road and cram as much as we can handle into the pit stop of our lives.