Category: Hypophosphatasia

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

Lidocaine Infusions: New Hope for Pain Management

Lidocaine Infusions: New Hope for Pain Management

As a person suffering from a chronic and rare disease I am always looking for the next thing that will help me deal with the daily pain and fatigue my illness causes me.  Recently I had the opportunity to try a relatively new therapy known as a Lidocaine Infusion as a way of treating my pain.  

To start let me tell you a little about what a Lidocaine Infusion is.  An infusion is the administration of medication through an I.V line directly into your bloodstream.  This route allows for medication to be administered quickly and systemically.  In other words the medicine quickly goes to all areas of your body, versus something local like an injection which tends to keep the medication in the specific area it was administered.   Lidocaine is a numbing agent that most people have heard of in the dental world or if you have ever had to have stitches or mole removal.  It is the drug commonly injected at the site of a procedure to numb the area.   Lidocaine comes in a lot of different forms in a lot of different strengths.  So what may be used by a dentist or an ER doc is not necessarily going to be the same thing you find in over the counter lidocaine patches.  

So when you put it all together you are essentially receiving a numbing medication systemically to control pain.  Now there is a slight catch with this type of procedure.  Lidocaine has a very short half life and what that means is that the average person will process all the medication in a very short period of time and no longer feel the effects.  Why can this work then you may ask?  Well when a person has been in pain for a long enough period of time it is possible for those nerves to essentially “learn” to be in pain.  Just like how a computer sometimes freezes up so do your nerves, and like a computer they need to be reset.  Lidocaine works on the nerve pathways to the brain and calms them down so they no longer transmit the painful feeling to your brain, or they transmit less of the feeling.  Chronic pain sufferers can also continue to have pain after the reason for their pain has been removed.  This is why Lidocaine Infusions can be a useful tool in pain management.  

So on to what I personally have experienced with the infusion.  Given that this works on your nervous system you are required while receiving the infusion to essentially meditate, clear your mind, and ignore all outside stimuli.  This is how it is supposed to work best, put yourself in the calmest place possible so the medication can do as much work as possible. Once you begin receiving the infusion whichever hand or arm you receive it in will begin to feel cold and kind of numb.  This is due in part to the lidocaine causing the numbing and saline solution it is administered in being colder than your internal body temperature.  So bring a comfy pillow and blanket.  I was told I may feel sick to my stomach during the infusion but to be honest I didn’t have an issue with this as I am used to the feeling and have worked on breathing exercises to suppress the icky pucky feeling.  Basically after that I just rested till the medication was done being administered.  At this time when I was starting to move around and get unhooked I did start to feel a bit lightheaded and heart started racing a bit.  Again I just took my time and a few deep breaths to calm myself and after a few minutes was good to go.  I could tell right away that my pain was better.  The achy feeling that usually resides in my joints was gone and I was really excited.  However I was told by the doctor that everyone feels that way right after and the real test would be seeing how I felt after a few days, by then which the medication would be completely out of my system.  I went home and had the expected feelings of being tired, but not so severe as to ruin my whole day, and not the drugged tired I was anticipating.  Needless to say I slept great that night. So generally if you are going to get any benefit from the medication you will know within a few days, then it’s just a matter of how long you will get that pain relief.  

For me it lasted about two and half weeks and made the almost 36 hours of traveling I did to get to Bali easily bearable.  Keep in mind that the last time I had traveled a month prior I was on a plane for only about three hours and suffered a major flare afterwords.  I found that not only did I have less pain in general but when I did need to take extra medication I could take half or less of what I would normally have taken and get the same results.  This was very exciting since I am all for anything that means I can take less pain medicine to function at the same level as others.  

The time spent getting the approval, the overall cost, and the time spent getting and recovering from the infusion were well worth it for me.  But remember that this type of infusion does not work everyone.  Some people receive no benefit at all, but in my opinion it is something worth trying if it can lead to a better life.  Try anything once they say.

For more information on Lidociane Infusions please visit the Therapies section of Articles on this website. 

Post Diagnosis First Job Interview

Post Diagnosis First Job Interview

It’s been a tough road post diagnosis with HPP.  I had to re-think all the plans I had made for my life starting with what in the hell I was going to do for work.  At 28yrs old and for the most part an active person most of my life, having to consider that I might not be able to handle a full time job, let alone get one in my field of education, was a hard pill to swallow.  I had to take the time and evaluate how much I could safely handle.  My field of choice and passion is EMS(emergency medical services), and there is nothing better to me then providing comfort and care to people in their darkest times.  I have worked as an AEMT for years.  Mostly on an on-call bases but hey it was something.  So never in a million years did I think I would get another chance to work in the field after diagnosis. 

RCecently I was offered a chance to work for my local EMS service.  I was over the moon excited and immediately started planning how I was going to do better, get in better shape, eat better, be better so I could foe fill my passion and help people.  Thankfully they offered the chance at part time, perfect for me, so I jumped at the chance, sent in my application, and within days was at the clinic for my pre-work screening.   

The pre-work screening is standard in any EMS position since you do have to be physically fit enough to carry the equipment and lift and work with patients.  I knew I would have no trouble there since I had been doing this stuff prior to diagnosis and treatment with Strensiq.  And of course there was the drug screen, which again I was prepared for knowing the medication I take would show up positive.  I had a doctors note saying they did not affect my mental faculties and I was able to function at a higher level when taking them given my chronic condition.  I had done this before with another EMT position and was met with no opposition.  The next part was a consultation with the doctor who would assess me to make sure my reflexes and medical history would not affect my ability to perform.  

So you can imagine my surprise when the doctor took one look at my medications, a standard question on the form, and started asking questions about what they were for and what I had.  Being that I had not shared with my potential employer my disease I was hesitant to discuss it, but I also know that lying could open me up to legal issues.  So I proceeded to give him the basics.  Shortly after my explanation of a disease this doctor knew nothing about he asked me, in a not so positive fashion, if I really thought this position was a good fit for me given my chronic condition.  I must admit that the moment following that question seemed to last longer than it did because never has anyone questioned my physical capabilities.  I was after all a life long athlete and strong despite my disease.  I’m not even sure how I stumble through my reply of something along the lines of “I was doing this prior to diagnosis and treatment so yes it’s the best fit for me plus it’s part time”.  I think the doctor was a little taken aback by my reply as well because he continued to press the issue with comments about my ability to recover compared to others.  Just to top it off he asked what I would do if my partner failed while we were trying to lift a 250lb patient into the ambulance.  Again my mental faculties stuttered since I’m pretty sure anyone on the job would struggle with that much dead weight on their own.  But I replied that since I had this condition I probably was better equipped to deal with this type of situation since I have already trained myself how to lift, move, fall, and catch myself in emergency situations.  He simply replied he was not here to judge that only to make sure I was making the correct decision and determine if my reflexes and muscle tone were good, which of course they were perfect. 

The rest of the screening was a breeze, not to say I completed it without pain, but no more than anyone else and I felt good, like I had proved I could still do this job I love.  But something started to eat at the back of mind, what the doctor had asked and how he talked to me.  Given that this had never happened before, and that it had taken a year for this job to come along, I wanted to be prepared for any outcome.  So I turned to my support group for of course support and advice. 

You can again imagine my shock when the comments starting coming in and they were not positive.  I was told to grow a thick skin because I might have to go through denial a dozen times before a company was willing to take the risk of hiring me.   I was told I was selfish for being upset about not getting the job (even though I hadn’t even heard from them yet), because other families had suffered more then me, lost children to the disease, and been told they had limitations their whole life.  I was told I complain to much and that I hide behind my #nolimits.  It took a dozen posts before someone stood up for me and anyone offered solid advise.  

Not only was I now worried about getting a job that I had previously thought would be no problem getting, but I was upset at the outpouring of negative and judgmental commentary coming from the one group of people I could turn to who would understand where I was coming from.  This is ridiculous I thought, what ever happened to good advice, sympathy, empathy, support and positivity.  Had I not suffered enough to deserve that from this group of people?  I started questioning myself, and I felt like I didn’t deserve this dream job, and my suffering was no longer valid, that my pain didn’t count, that my voice was only annoying rather than helpful.  Of course I cried, and round and round I went in my own head, trying to think my way out of the pit of despair I just been thrown into.  

I decided in the end that if I had to suffer more and go off my medications in order to do the thing I loved most, I would.  The physical pain I would potentially endure would be nothing to the prison I would otherwise live in inside my mind.  The depression of being told you can’t, you won’t, or you shouldn’t, to me is more debilitating then any physical pain I have been through.  So I would find a way, take the chances, make the changes.  I would endure.  But I also decided that for the first time social media, i.e facebook, was not a healthy place to get what I needed support wise.  It was because of social media that I had my meltdown and questioned myself so deeply, something I had not done since deciding I would not let this illness rule my life or limit what I was capable of doing.  This incident had gone on to blow out of proportion and not only affect me, but my relationship with my husband and my ties to the only community that could share in my struggles.

Long story short, after tears, depression, sleeplessness, questioning, doubting, and more, I decided to leave facebook.  I gathered my thoughts, renewed my inner strength with the help of some supporters, vowed to make changes in the way I deal with my personal relationships, and set my mind back in the right place which was that they had no reason not to hire me and to remain optimistic. 

People with HPP are doing amazing things they have been told they can not do every day.  Each person that accomplishes something brings hope to others for a life where limits do not apply.

Dad’s Point of View – Ch. 1, Fear and Pride – May 10, 2017

Dad’s Point of View – Ch. 1, Fear and Pride – May 10, 2017

This is the first in a short series of article written by my dad, the parent of child with HPP diagnosed after living what we all thought would turn out to be a perfectly normal life.

We always knew something was wrong.  We thought it was depression, or ADD, or maybe she just wasn’t naturally academic.  Annalise was just too fatigued to complete her studying, and the level of fatigue varied with stress.  She had occasional injuries, but what do you expect from a no-holds-barred soccer and volleyball player?

I can imagine nothing harder than for a father to raise an active daughter who wants to reach for the stars on everything, but sometimes cannot make her body do it.

Nor can I imagine anything more rewarding than to watch one’s daughter cross the stage to get her college diploma, or win the Badger State Games in shot put, or build an accurate six-foot-tall human heart, or become known as the best I-V needle stick in the business, or hold your arm as you give her away to the next man in her life.

Annalise works occasionally for a very wonderful rheumatologist (remember that “best I-V needle stick”?) whose brother is a pain management doctor.  That doctor just happened to have been talking to an endocrinologist who happened to have heard about hypophosphatasia.

A referral was arranged, and Annalise had her blood test to compare to two previous tests.  They found an ongoing pattern of low levels of alkaline phosphatase.  A diagnosis of hypophosphatasia (HPP) was recorded.  Annalise was – very briefly – overjoyed, and I was nearly as happy for her.  Finally, there was a name for the enemy robbing her strength and energy.  Finally, there was proof that she was not a hypochondriac or a malingerer. 

Her mother, a superb RN who had fought the medical profession (and anyone else who didn’t believe Annalise) brought us back down to earth as she studied all she could learn about HPP, and we began to realize the enormity of the task facing Annalise in fighting this enemy.  Nonetheless, fight it she has.  Read her blog entries to learn more about that fight.  And I will add more articles to tell you about the effect her HPP has had on me.          

                                    

The Villages: Florida (A different kind of vacation)

The Villages: Florida (A different kind of vacation)

Just because we suffer and have this crappy disease doesn’t mean we have to be home-bound.  Yes traveling can be a bit of task with having to carry extra medication and allow for extra time, not to mention access to medical care where we’re going if something happens.  But I have stumbled upon a rather unique type of vacation that I would not have even considered interesting if I didn’t have this disease. 

I’m young, 28 years old, so the typical Cancun, drink, dance, stay out and party all night vacation is just not realistic.  Spending hours in a car going from place to place taking chances wherever you go.  Sometimes being young on the outside and being young on the inside are two totally different things.  I frequently say “I’m 28 on the outside but I’m really 80 on the inside”. 

Sooooooo……why not vacation in a retirement community?  Sounds dull right? Wrong! I recently had an opportunity to visit The Villages in Florida.  What a great place for someone who wants all the fun of being young and all the convenience of being older.  The entire community is laced with golf cart paths allowing for a very pleasant and comfortable ride wherever I went.  Even better, there is always a place to easily pull over, get out and stretch, or readjust.  The entire community is packed with beautiful golf courses not only teaming with interesting characters, but the wildlife is unreal, and tame enough to get some good pictures because they are used to the hustle and bustle of the regular residents. 

I spoke with residents of the community and learned about the great healthcare system in place.  There are AED’s located all around every neighborhood in case of emergency, and EMS is never more then a few minutes away.  Not to mention you the patient are never more than a few minutes away from a more then capable hospital.  Should something more catastrophic happen and you need to be flown out, there are always places for the helicopter to land due to the large number of cul de sacs, flat golf courses, open spans of road and so on.  For someone who can potentially step off the curb and cause major damage to themselves I found this to be of great comfort, because lets be honest you never know what’s going to happen and when.   And something that must not be left out is how handicap accessable everything is.  They have designed this place for an older generation but it also works great for those who try to avoid things like stairs.

There are several area hotels to stay at not to mention the great number of seasonal rentals should you wish to have your own home for a few weeks!  Another major comfort since it allows one to store their medication in more reliable refrigerator and freeze cold packs for the ride home. Plus there are some top quality grocery stores so you can easily stick to whatever type of diet you may be on.  Each person is able to obtain a visitor pass that lets them into the various sub-communities and their wonderful amenities.  There is everything from nightly dancing in several of the town squares, to Polo match’s, bike races, shuffle board, bocci ball, horseshoes, dog parks, golf lessons, concerts, lavish movie theaters, weekly events, farmers markets, the list goes on and on.  You can even download The Villages app and get all the calendar event times and locations, GPS for your golf cart, and more.  This place is busy, and not, all at the same time.  I loved eating at the unique country clubs and restaurants  and all were priced SUPER WELL!  Not to mention that majority of the places had menus with meals spelled out so you’re not guessing what might be in something.  It was great because no one seemed in a hurry but everyone seemed active, it was like my insides fit right in even if my outside didn’t.  The people here despite being mostly on the older side, were as full of life as any younger community and more then happy to socialize. 

So why not try a change of pace and enter the “bubble” that is The Villages.  Even if you are traveling with little ones there are activities for them since it is not uncommon for kids to visit their grandparents.  Not to mention you’re only an hour from Disney Land.  The days are warm the nights are cool and there is always something fun and new to experience.  Get lost on your golf cart and go find an alligator chilling on the golf green.  Remember you don’t have to be limited to what you think you can do.  Every person with or without this disease has #nolimits, and it’s nice to get away someplace where people understand pain, but also the pleasure of living. 

For more information on The Villages visit:

https://www.thevillages.com/

If you’re young take a chance and live like the older ones do, and if you’re older, see how you could be living!

My Friend Pain

My Friend Pain

Two am and still awake. Yeah you all know that feeling, the feeling of frustration because you need sleep but every fiber of your being is vibrating. It’s like every cell is doing its best to escape your body and run away from the pain inside.
Pain, my old friend who never really leaves me, no matter how many times I think I have gotten far enough away from it that maybe just maybe I can take a chance at a more normal life. Sadly no this is not that time, tonight I lay here frustrated and anxious. I had done so well almost 48hrs no pain meds, good for me. But my will was just not strong enough this time and after more then four hours of tossing and turning and attempts at a more benign solution, I caved and took some pain medicine. But why is it whenever I do that I feel guilty? Its almost like giving in or giving up. I know that society has made it “acceptable” to take medication for well known, non-painful diseases, but why is there such a stigma around people who need pain meds to live normal productive lives. My pain and anxiety are made worse by my mental loop of trying so hard not to take anything, suffering, then finally giving in and taking something, then feeling guilty because I did. Sometimes the worst and most painful part of a disease that causes chronic pain is the struggle to control it mentally. I guess it’s not as bad when you know early in life and don’t know any other way of being, but when you’re a full blown adult with life experience and then find your world turned up side down it’s so hard to adjust to thinking of yourself as “different”. I keep telling myself I have no limits, I can do anything I just have to do them differently, but even that can be exhausting to the point where every once in a while I just have to have a meltdown.