Category: Hypophosphatasia

It’s Ok to be Mad

It’s Ok to be Mad

Some days all I feel is anger.  I’m mad because I have this disease, mad that I have to do injections all the time, mad that my life revolves around appointments, drug delivery schedules, power outages, medical supplies, drugs, insurance.  I get mad that my disease has prevented me from working in the field I love, and I get mad that my husband works his body and soul to the ground to provide for us because I can’t, but yet we still need insurance because the drug I need would otherwise cost over 1.3 million dollars a year. 

It’s a funny thing to mad at what amounts to nothing.  I’m mad at my disease, but I can’t yell at it, or hit it, or confront it.  There is no physical object to aim my anger at unless I aim it inward toward myself.  I get mad that I’m one very small person with no influence in the world, and that I have no way to make people see what it’s like to be a person with an invisible disease.  I can’t make the ones around me understand how much I hurt both mentally and physically, and I can’t tell people why I’m mad because honestly there isn’t really a good reason.  I just am. 

Every time I think I have this disease under control, and like I can take back my life again, something happens, and it’s like I am losing my life all over again.  Not to mention how hard it is hear that others have it “worse” than me.  Because lets be honest, no one person has it worse than another, but rather each person suffers with their own illness in a unique way that is either manageable or not for them.  So that makes me mad to, when someone thinks that I should be grateful for what I have and what I have not been through, because I honestly think that’s a load of bullshit.  I have every right to be mad about what has happened to me, the same as the next person.  I may not have rods and plates and screws in my body, but still live my life around this disease, and in that way we all are the same. 

So be mad, it’s ok, scream at the world, curse whatever god you believe in for giving you this disease, or whatever disease you have.  Be mad that you can’t do the things you want, or that they are hard, or whatever it is that others make you feel like you should just except.  Pound your fists into the ground, throw something, break something, run until you collapse if you need to.  Tell your friends and family you don’t want to talk to them today, and that there isn’t a good reason other than your just mad.  Take a mental health day from work, and stay in bed, snuggle with your pets, eat an entire tub of ice cream.  Get really high and watch dumb cartoons.  Do whatever you need to to work through your anger and madness.  Don’t try to run from it or bottle it up. Keeping it inside you will only poison your soul and ruin all the things that truly are good in your life.  Sometimes you can’t do anything to make it better, sometimes you just have to be mad, until you’re not anymore.   You have to ride out the emotion like a roller coaster until you finally have the chance to get off. 

In the immortal words of “Big and Rich” if your going through hell, keep on moving.   I doubt you would willing stand in the flames and let the devil torture you if you had the chance. 

Remember that no matter what you have been through, or what you are going through, it doesn’t matter if it’s easier or harder than what someone else has gone through, you have a right to be mad about it. 

“Trains, Oooo I love trains” – Sheldon Cooper

“Trains, Oooo I love trains” – Sheldon Cooper

If you have never had the chance to ride the train then you should definitely add it to your list of things to do.  Getting from place to place is always a question when it comes to painful diseases because one way or another your bound to be stuck on a plane, train, or in a car for hours.  So out of all the options for how to get from one place to another I would have to say that train is one of the best options I have tried.  To start off there is more leg room! Way easier to stretch out and keep you body from cramping and locking up to bad.  Second of all you can get up and move around in an aisle actually big enough for two people, no awkward butt in face moments when trying to get past someone.  Plus the whole experience in general is more comfortable being that there is more space for everything and bonus there is even a place to plug in your charger for your phone, computer, or even camera battery. 

I recently had the chance to take the Amtrak from Milwaukee to Chicago, and honestly I would do it again in a heart beat and plan to investigate into planning all flights from Chicago by way of train from Milwaukee.  There was no pressure with traffic and it didn’t matter what time we left because it takes the same amount of time no matter what the time of day is.  So forget having to add an hour for rush hour traffic, just get on and go.  There was easy access to bathrooms, and special seats with extra extra room for disabled persons, or those traveling with crutches or walkers. 

All in all the atmosphere of the train was much more relaxed than a plane or trying to drive in a car.  It really allowed me to just sit back and relax, type or sketch, and enjoy looking at the countryside going by.  The trip was only about an hour and a half which honestly was not that much more than if you were to drive, especially going into downtown Chicago.  What a blast, I can’t wait to see more and share it with all of you!

 

Always look for new ways to do things, that way nothing is out of reach!

Playing on the Water

Playing on the Water

For any person with a chronic disease, or rare disease, or anything causing ongoing pain, normal summer activities can seem like the most challenging types of fun.  You have to plan and plan and plan for something as simple as a boat ride.  Do I have the meds I need, did I bring the right equipment “just in case”, do I have to do anything the next day if I over do it, is there medical attention near by if something goes wrong, if there is will they be able to properly handle my condition?  This is just the tip of the question iceberg.  But the nice thing is, the fun summer activities can be done, especially with a little advice from someone who has already done it.  

I had a chance to go visit family on a lake up north for a few days.  This also afforded the opportunity to try out some new water sports.  I was always a fish, so being in the water is nothing new to me, and I had loved going tubing as a kid.  But in the last few years those things have happened less and less to the point of non existence.  So I was naturally apprehensive at the thought of getting back on the water to try the traditional summer sports, i.e water sking, jet sking, knee boarding, tubing and so on.  

There was all the normal preparation involved, make sure to pre-medicate with a good anti-inflammatory, have pain medication on had, assess current body statues for pain and points of tenderness to make sure everything is good to go.  I had been doing pretty good, nothing flaring at the moment so I just made sure I had meds ready for later and ice packs just in case.  

My first experience was with water skies, of which I have tried before and was successful at getting up and skiing. But to say the least it was really hard on my body back then and I suffered afterward.  This time I was prepared to be careful and not push myself if there was even a hint of over doing it.  Luckily I had calm water, so no worries about dealing with wakes from other boats, plus it was less hard on my knees to keep balance.  I got up right away, which of course I was super proud of, and was able to make it once around the small lake, another victory.  By the time I was done my back was a little sore from trying to hold every muscle tight so I wouldn’t fall, but otherwise okay.  After a break, some stretching and some water I had my first chance to try knee boarding.  This made me really nervous since I have osgood-schlaters in my knees, so kinda like tiny bone hooks where the doctor always hits your knee to check reflexes.  So naturally kneeling is painful for everyday activities but bouncing up and down on a board on the water seemed like an even worse idea.  But I wanted to give it a chance because you never know what you can do until you try.  I talked it over with the person driving the boat so they knew I might bail right away if it felt like it was going to be problematic.  Luckily and surprisingly it wasn’t!  Because of the high density foam on the board and more anatomical position I was sitting in, I didn’t put any pressure on the portion of my knee that I normally do and worry about.  Plus it was less stressful on my body overall and more comfortable being that I was closer to the water so If I had to bail it would be easy and less painful when hitting the water.  Plus I actually took a minute to try kneeling on the knee board on land before giving it a shot in the water which was smart since it gave me more confidence to start. 

In the end after two days and five rides I was doing pretty good.  I took a hot bath to sooth sore muscles, and dosed with my anti-inflammatory for a few days.  Not to much pain beyond what you would expect from a physical activity.   And I had a lidocaine infusion coming up so that was just good planning in case I did cause a flare the lidocaine would help prevent it from getting bad.  

In conclusion don’t limit your summer fun to the beach and sidelines.  Take a bit and figure out a way to do those summer activities that make summer worth having.  All you have to do is take a little bit of time and plan out how your going to try those activities.  There is nothing you can’t do with a little bit of planning and creative thinking. 

Dark side of rare

Dark side of rare

Sometimes I’m not sure where to start.  So many thoughts run through my brain, like endless waves crashing against an ever shrinking wall.  I wonder sometimes why me?  How did I get so “lucky” to be so “special”, and if I really am then how?  I’ve been told that God does not give us anything we are incapable of handling, but it doesn’t always feel that way.  Like when I lay awake in the small hours of the morning with nothing to calm the crashing waves.  Sometimes I think my mind is worse than my disease.   I have been diagnosed with an “ultra-rare” disease.  Shit like that doesn’t happen.  Bad breakups, heartache, failure, success, those are the things that happen.  I break my arm, or get dumped, I even crash my car.  All these are so small and insignificant when someone tells you that you suffer from a disease that most professionals can only claim to have heard of maybe once in their medical education.  I curse modern technology and medical advancements sometimes because without them I would just have kept living my small endless circle of feeling like there was something wrong, being depressed, breaking down, then getting over it.  But nope, not anymore, now I know I have Hypophosphatasia.  To be honest, among a million others thoughts, I worry about the way my disease effects those around me.  What does my husband feel, how are my parents doing, do my friends and family take me seriously when I try to explain?  I can’t read minds, but sometimes I wish I could because then I would know for sure.  What do you do when the person you love is suffering?  If I’m honest with myself, and the roles were reversed, and my husband or mother or father told me they had this……I’d be devastated.  I’m not sure I would ever be able to treat them or look or think about them in the same way.  But somehow I expect for those around me to both act like nothing has changed, but also empathize with me.  Kind of a have your cake and eat it to type of thing.   I had goals once, and dreams, I was going to be someone and do great things.  Somehow that feels so hard now, and yeah I’m 28 years old with my whole life ahead of me.  But sometimes I want to skip to the end, like reading a book, and see what happens.  A part of me needs to know if things might have been different.  And what would have happened if I had known in my “younger” years that I had this, or if I had never had it at all.  Would school have been more tolerable, would people have been nicer to me.  I’m not sure if that would have been a good thing since sometimes kindness from pity is worse then cruelty.  There is a certain kind of loneliness that comes with being “rare”, and an even worse one when you look “normal”.  

When you have a rare progressive disease no one seems to think of the other effects besides how it affects your body.  You don’t think about how it affects the family, your future, the chance for children, travel, even whether or not you can work in the field of your choice.  No one wants to feel like the greek Atlas holding the world on your shoulders, but the reality is that is what we deal with.  Being rare is not limited to the effects of the disease on the individual, it expands to effect every part of that persons life and extended life.

So I encourage you if you know someone with a rare disease, especially if that disease is invisible to take a few minutes and consider that that person is not only carrying the burden of their own disease, but how their disease affects all the people around them.

Minneapolis Pride and HPP: Invisible Acceptance

Minneapolis Pride and HPP: Invisible Acceptance

I recently had the chance to visit an event known to many as the main celebration for the LGBTQ+ community, Pride.  The vacation was all planned, two whole days of concerts, clubs, shows, festivals and more.  For those with HPP this can be exhausting and painful just to think about.  And for the most part they would be right.  Take a second and think about the difficulties a 28 year old with an invisible illness goes through when trying to keep up with other youngsters their own age during a massive summer event.  Starting with the parking.  If you have ever gone to a festival or major concert of any kind you know that parking can be a real bitch.  You can expect to pay a ton and walk two plus miles to get from your car to the actual outskirts of the event.  Many places, though having handicap parking, are still wholly inadequate for those with more severe and obvious disabilities.  Given that I was going through a particular rough spell with pain in my SI joint I really was hoping for something semi close and easy to walk through so I could use all my energy to enjoy the festivities.  Having been to other events I was worried after we had circled several times only to find nothing available and people flocking from miles around.  So taking the chance I wouldn’t be looked at as the typical “faker” of a disabled tag I am used to I pulled over and asked a police officer if there was any designated handicap parking.  He very polity and with a smile replied that there was a whole street blocked off for that purpose and then explained how to get there. This was my first bit of excitement, designated parking nearby, so there was a shot!  After finding the street I pleased to find a spot open immediately, near a sidewalk, and near the festival restrooms, which also btw’s had a handicap port a potty.  Normally when I put up my placard I get a sense of worry, like people are judging me and that this might be the time I come back to a nasty sign on my windshield.  But something was different this time, no looked twice and I felt immediately at home and comfortable.  

After parking we began the adventure of Pride festival.  Being that anything can happen I tend to look around first thing and try to identify staff, emergency personnel, and first aid tents.  Again I was pleased to pick out staff easily as well as officers on bikes, on foot, and golf cart, riding and walking around ensuring everyone could thoroughly enjoy their time.  This always makes me feel better for the “just in case” scenario where I fall wrong or get run into and something terrible happens requiring immediate medical attention. 

To my great joy and surprise, I was able to keep up with my friends for the whole day as we walked around.  Normally I have a limited amount of time determined by how much and when I take pain and pre meds to prepare for an eventful day.  However being that I was post Lidocaine infusion I was doing particularly well and thought I would try going without. For the most part I did great.  I never had to be the one to stop and take a break, or sit down and rest, and we didn’t have to leave early for me because of my pain.  Last summer this never would have happened, and because of my disease I never would have noticed what a great event to go to if you have a disability or need a little more security when going to festivals.  I had a blast and people were friendly and accepting wherever we went.  There were hundreds of booths and rows dedicated entirely to single subjects.  

There was everything from pet adoption, to different religions, adoption and fostering, medical care, dental care, insurance, education, the list goes on and on.  The culture was extremely diverse and truly a pleasure to have the chance to experience.  Those working the booths were always more then happy to share their product/service or simply point someone in the right direction.  Not to mention the free swag they were giving away was a major plus in some cases.  

As we wandered around what quickly began to resemble a wonderland fit for Alice I noticed the number of service dogs and ESA’s around, as well as extra seating and places to rest.  I always love to take my service dog with me when I go places as he can carry things for me if I am having a bad day, or I just need the support and a friend to help tell me when I pushing myself to hard.  But sometimes it makes me very uncomfortable in public as my dog doesn’t look much like a service dog, and to be honest I don’t look disabled. But here I felt things would have been different.  The air itself was tangible with acceptance, regardless of whatever, or whomever may be accompanying you.  

The next thing I found surprising but also awesome was the food selection.  As many people with rare diseases know there are almost always some sort of dietary guidelines or restrictions that go along with the disease.  This can mean finding food when you go out can be difficult unless you wanna have a “cheat day” and pay for it later.  But at the Pride festival there were several choices that allowed for many different diets from vegan, vegetarian, paleo and more.  I was happy to not have to think to hard about what I was ordering.  There were also plenty of places to get a drink and dispose of the garbage afterwords, but there were also no restrictions on bringing stuff in.  Given that alcohol was the one exception since the festival did provide the traditional beer tent, you were allowed to bring bottles of water or Gatorade to stay hydrated on the semi warm day. 

Later that night I was able to attend a small show put on at LUSH , a traditionally gay bar playing host to some very entertaining acts.  While enjoying the atmosphere and people watching I was able to meet some to the Drag Queens whom were there to perform.  For me this is always fun and exciting as I consider Drag Queens to be literally walking pieces of art, and have followed RuPaul’s Drag Race since the beginning.  They were amazing and wonderful people.  Never have I felt so normal and comfortable with people as I did when talking to the queens of LUSH.  In particular I had the pleasure to meet the lovely and talented Shangela Laquifa Wadley.  She approached me with warmth and smile despite how tired she must have been.  She spoke softly and made me wish I had more of a chance to sit and talk than just a quick fan to fame moment.  But despite this I was able to get a few good photos together and give her my business card directing her to my website here!  As silly as this sounds that small gesture made me feel so good.  I had just had the opportunity to pass on some knowledge, and possibly educate someone with power about one of the zebra diseases.  Giving her my card was a huge leap of faith and to be honest at 2:30am I wouldn’t blame her if she lost it or never bothered to look.  The point was she was kind and took the card, and I had the chance to spread the word about HPP to someone who matters. 

The last step in my journey through Pride was to attend the cant miss event, the parade.  For miles the street was lined with all manner of people in all manner of dress.  We had been running a little late so naturally I was concerned I might have missed the best parts but whether it was fortunate or not a “Black Lives Matter” protest in the middle of the parade street had delayed the start of the the parade by nearly an hour.  I was somewhat baffled as to why the protesters choose that place and that time to express themselves since it was interfering in an event meant to promote acceptance and equality for all.  Despite this my friends and I were able to locate a decent position where I could comfortably stand or sit and watch or take photos of the parade.  It was such a great time to see so many people openly expressing their true inner selves.  I loved being able to capture the souls of some of the parade walkers who truly shown.  It is not often that people allow us to see that intimate part of themselves that at our core defines us.  To capture in on camera was a challenge and pleasure.  

I definitely plan to attend this event in the future and I hope you all enjoy the article and the photos.  Some have been edited some are just as they were taken.

Fight for a Job I Love

Fight for a Job I Love

Never in my life have I been told I didn’t get the job after I had been basically guaranteed it.  Never has my medical history or medications been an issue.  I work in EMS, and I’m good at it.  I love to help people and work with patients.  I know how to make someone feel better when they are experiencing one of the scariest times of their lives.  Never did I think that I would not be able to work in the field I was put on this earth to be a part of. 

But sometimes life has a way of kicking you when you’re down, and turning you in a different direction.  God knows it’s happened to me more then once. Every time I get kicked I somehow manage to pick myself up and start over.  I don’t know how that this point because if I was humpty dumpty there would just be to many tiny pieces to try and put back together again.  But there is also an ancient Chinese practice that says when something is broken you do not throw it away as though it no longer has value, you mend it with gold, and make it better.  So I guess I can also be seen to be made of mostly gold from the number of times I have been mended.  

Today after feeling like there was no way I could pick myself up again I had a few things happen that renewed my strength in the fight I have to live my life fully and help others to do the same.  

The first thing was having a Lidocaine infusion this morning and even if the effects last only a few hours, I got the chance to remember what it feels like to live without pain.  I was so happy I could have cried because I didn’t realize how much pain I had been carrying around with me for so long, and how heavy it had gotten.  I smiled and it felt good, I stretched and nothing hurt, I napped and didn’t wake up with something in pain.  So I am grateful for this day because it reminded me I still have some fight left in me.  

The second thing that happened was I came across a young woman, deaf since the age of 18 from a connective tissue disorder, competing in a singing competition of all things.  As I watched her video I could feel the hairs all over my body stand up.  She sang beautifully on stage barefoot to feel the vibrations from the bass to keep time, and took ques from her interpreter down by the judges.   This is someone who gave up on something she loved for many years and then somehow found the strength and determination to make it work and reclaim the love she had lost so long ago.  I decided right then that this would be the video I would watch the next time I got kicked, the next time someone told me I couldn’t or wasn’t able to.  Because this girl is living proof of living life without limits!

Watch this girl and know you can do anything!

Being a Victim of the “Opiate Crisis”

Being a Victim of the “Opiate Crisis”

I am a victim of the opiate crisis.  No I have not lost any friends or family to drugs, and no I am not, nor have I ever been an addict.  But the war on opiates has more than one type of causality.  I have spent the last year trying to get a job in my area (so within an hour drive) in the field that I have dedicated my life to, emergency medicine.  I have gotten a bachelors in health sciences, taken many classes pertaining to medicine, obtained my AEMT certification, and worked for multiple fire departments as an EMS provider.  But all of a sudden I find myself unable to become employed in the field I was put on this earth to be a part of because of the medications I have PRESCRIPTIONS for to help control the chronic pain I have from a rare disease.  I do not want to go on disability yet, I am just not ready to admit that is what my life has come to.  I am able to work, I want to work, but no one will hire me because I have prescriptions for and take on occasion opiate medications.  I have worked for several years in this field prior to this year with no issues, an exemplary record for care provided and letters of recommendation from employers due to my ethics, ability to function under stress, and provide excellent care to any patient in all situations.  However, now because of the war on opiates, pain medication and chronic pain have become an extremely taboo issue in the workplace.  

The FDA uses a lot of different adjectives to describe everything from A to Z when it comes to opiates.  What I find interesting are all the things that seem to be ignored such as:

This medicine may make you dizzy or drowsy. Do not drive or do anything that could be dangerous until you know how this medicine affects you. Sit or lie down if you feel dizzy. Stand up carefully.

So what employers and their legal teams seem to ignore is the part where it states these meds MAY have certain affects, as in they MAY also not affect you at all.  Or how you should not perform certain activities UNTIL YOU KNOW how it will affect you, as in you are good to go if you already know how it will affect you.  This is what applies to my case.  I do not process most medications the way the average person does and as a result it means the combination of medications I take for various things is a bit odd.  However I also know after years of being on these medications exactly how they will affect me, for how long, in what manner, and even how long they will affect me based on what I have eaten or drank recently.  The websites basically give all the worst case scenario information which is what companies and legal teams grab onto.  

I find it very hard to be told that I should find another field to work in, or to let it go and apply somewhere else and just don’t put down my medication list.  I want to get a job in the field I love and I want to do it honestly.  Why should I have to hide my medication list, or give up the thing I love the most after already doing it for years?  I understand that rules and regulations are put in place for a reason to protect the patients.  Personally I would never endanger a patient by taking something that affected me so severely it could cause them harm.  But when advising physicians are making recommendations based on one aspect of the medication it can be very hard to for those few who have no issues to make it through.  There is a “black box” warning on almost all medications state that they can impair you in some way, and for good reason.  But again that is there because a few people reacted that way and now it must be put on all prescriptions regardless of the person taking them.  I have met people who respond so poorly to caffeine they are unable to function on even the smallest doses present in things like chocolate.  Just an example of how something benign can have a profound affect on someone the same way it can have none. 

I will not give up, I work every day to find a way to do what I love.  I am always looking into new research and new methods of pain control that do not involve medication or at the very least opiate medication.  There are people out there every day showing that you can do what you love despite all the odds, you just have to believe and work hard enough.

The Good and Bad of Steroid Therapy

The Good and Bad of Steroid Therapy

The use of steroids such as predinisone is a slippery slope in the world of chronic illness.  Many who have been on predinsone for any period of time typically report a significant reduction in symptoms, an increase in energy, and an overall improvement in daily life.  However, when they are used continuously they can have some pretty negative long term effects on the body.   

For someone in the HPP community some of these side effects can only add to the problems caused by the disease.  Most pronounced and serious is the thinning of bones caused by long term corticosteroid use.   This side effect, though not typically common in occasional and or part time use, can be very problematic in a disease already causing bone thinning or softening.  But being able to utilize coritocsteroids in the right way and only when needed can bring about a very positive effect.  

Though I can not speak for others in the community I know personally that I tend to experience occasional flares of tendonitis in random joints, with no provocation, and with no warning.  When this happens I do my very best to get the issue under control with all the regular therapies.  I utilize resting, icing, muscle rubs, NSAIDS, water and cryotherapy.  If I am unable to resolve the issue myself within a few weeks, or make no progress in recovery there is one thing I usually turn to that I have found to work well, is easy to use, and non-harmful.  

Methylprednisolone in a one week dose pack is my go to for issues like this.  It is a derivative of the very popular prednisone but without the hassle of a bottle and tapering which usually has to happen.  It comes prepackaged with a set number of pills to take each day starting with the first day being the highest dose, then tapering down to almost nothing.  I can usually tell after the first two days of dosing that there is a significant decrease in pain and restriction of movement in whatever joint is most problematic.   Even after the first day I notice a reduction in pain which typically comes as a huge relief since dealing with daily pain is only compounded when you have tendonitis to further restrict your movements.  

I really like this type of medication because for me it not only works well on the area that is specifically bothering me, but tends to also help other areas of swelling, such as nerve inflammation or bad muscle spasms.  This allows me to take a minimal amount of medication and fix the maximum amount of problems all in one setting.  Plus it’s one less time I have to go for an injection.  Beyond those other factors I am also able to communicate with doctor using the Aurora portal to request the dose packs avoiding the excess doctor visit and energy wasted on going in.  Not all medical professionals may be comfortable prescribing this medication in this manner and may ask for you to come in for an appointment.  This is also fine and still well worth the visit.  I have also found that getting this prescription is much cheaper then going in and paying for an injection of cortisone in the afflicted joint, and honestly when you spend as much time, money, and energy on going to the doctor and taking care of yourself as I do, it’s worth the savings.  

So if you any further questions please contact your local doctor or pain management specialist and ask about Methylprednisolone the next time you have a flare. 

 

For more information on the side effect of Corticosteroid use please visit the following website:

http://www.mayoclinic.org/steroids/art-20045692