Category: Hypophosphatasia

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.

Exercise

Exercise

Something I have struggled with is exercising and staying in shape for as long as I can remember.  It wasn’t that I couldn’t get in shape, but rather it was much more difficult, and I always had to fight the pain and fatigue because I just did what I was told which turns out was not the right type of training for someone with HPP.  Over the years prior to diagnosis I started to learn what things I could do more of and what would cause the most problems.  Another key thing I learned is to have access to a hot tub or sauna to jump into after a workout to loosen and relax the muscles preventing excessive stiffness and soreness.  Also knowing to ice problematic joints after each workout is critical for a successful exercise routine.  One of the ways to do this I had found to be not only time efficient, but quickly beneficial is to go for a cryotherapy session after a workout.  With this I am able to essentially super cool my skin temperature and let the cold seek into my deeper tissues more gradually.  It also releases a blast of serotonin, the hormone that makes you happy, so you feel extra good for several hours.  Also knowing when the appropriate time to take NSAIDS is important to maintaining good physical health.  You may have to get creative with your exercises if you can not travel to a gym but efficiency and accessibility are the name of the game.

The list below is of some key activities one can do along with notes and definitions

Weight Bearing: Feet and legs support your weight

Weight Lifting: each muscle group twice a week, low impact, machines not free weights less chance uncontrolled fall or join failure, therabands in varying elasticities.

Dancing: can be a fun controlled way to obtain cardio but also utilizing weight bearing

Swimming: the ultimate low weight bearing activity that conditions heart and lungs and also allows for a cardio and semi cross fit workout building good muscle mass

Balance Improvement: Tia Chi is a way to improve your balance in a controlled setting, fragile bones mean you have to be more careful, and means you need to have better balance to prevent falls

Simple calisthenics: repetition of a troubling activity such as sitting and standing, repeat motion until tired as many times a day as tolerable

  • Need to maintain a good balance between strong muscles to keep the body strong and weight bearing exercises to keep bones strong

Below is a website that provides a list of workouts for person with fragile and soft bones, or for anyone needing a low impact workout.

http://www.nmbreakthroughs.org/fitness/safe-workouts-for-fragile-bones

Marathon vs. Sprint (Activity level with HPP)

Marathon vs. Sprint (Activity level with HPP)

Many people with HPP know the concept of completing work over time slowly due to pain and fatigue, a common side effect of the disease.  The other end of the spectrum is when we finally get energy and no pain we are able to accomplish more in a few hours then others are able to complete in a day.  You learn ways to do things more efficiently, safely, and with minimal consequence on the body.  The concept of work smarter not harder is a hard won but well worth it lesson if one can do it.

For example, I had a bad spell the night before last and was basically unable to sleep from 930pm till 0400am. I finally managed a few hours and after waking up feeling drugged I got up and tried to have some breakfast and good cup of coffee.  But that would just not be enough so back to bed I went for a few hours and finally managing to get out around 1245 in the afternoon.  But magically upon waking up I had no pain and my energy, though low, was no longer drugged feeling and manageable.  So I got up an started on house work, because I don’t know how long I’ll  be up or how many good hours I’ll have before another crash so I pack all the work I can into the few precious hours I have.  People who do not suffer from a chronic painful illness will never understand what it is like to easily accomplish the work though small, needs to be done on a fairly regular bases.  They take for granted the amount of energy both physical and mental that it takes to complete simple tasks such as laundry and dishes.

I do not live a normal schedule, I sleep when I can, work when I can, run errands when I can, travel when I can.  Though my life is run by my disease I do not let it limit what I can or want to do.  I just don’t always accomplish things in the same fashion or at the same time as others.  I live my day in thirds usually.  Kind of like living three mini days in one, which allows me to sprint with my energy and accomplish what I need.

Travel in the Air

Travel in the Air

Traveling with HPP can be a bit of a trick.  The average person makes sure they have the basics, medication, clothes, snacks, tickets if needed.  For me it takes extra planning.  For example when I fly I always show my Softbones Identification cards when getting my seats.  Usually this allows me to get a seat in a better location such as the very front or back of economy or will allow me to be seated in a row by myself if possible as well as having the aisle seat.  These things are important because I know I cannot sit on an uncomfortable plane for more then an hour or two, so it important for me to be able to get up stand, stretch and move around as much as possible without disturbing the others in my row.  Another useful tip I learned recently when traveling is that I can make the plane seat more comfortable by utilizing three of the small on board pillows, two to sit on for extra cushion, and one behind my low back for extra support.  By utilizing these few tricks I was able to make the near 16 hour flight to Bali Indonesia.

Traveling with the life changing drug Strensiq is a whole other ball game.  Depending on the airport it can take an extra 30-45 minutes just to make it through security.  Because there are no studies on whether or not the airport x-ray machines affect the medication it is advised that all patients have their drug hand inspected.  Although there is some discussion over this I do not personally wish to take any chances with a medication costing around $1.7 million a year.  Because I have asked for my medication cooler to be hand inspected I have had varying degrees of difficulty.  Flying through Minneapolis International Airport I was sent through the metal detector twice and then the body scanner, then a brief pat down, then a detailed pat down, and all my belongings from both my bags were removed, ion swabbed and placed in bins for all to see.  So at this point I basically felt like a criminal and did not understand why all my belongings needed to be hand inspected since they all went through the x-ray machine except for the medication cooler.  After discussing this with some fellow HPP’rs I learned this was not an isolated instance and that others in the young adult age range had experienced similar and worse events.  One young woman had all her belongings and all her husbands belongings gone through.  I ask why? I must look like a drug smuggler being that I am a young, not unattractive white female so obviously I am hiding something.  Now on the other hand I have also had the complete opposite experience, and in particular when going through international airports.  I have gone through the same process with the security team only hand inspecting the cooler and waving me on through.  I was shocked in these cases as I was traveling with my husband, and as such had planned ahead for him to take my extra bag and go in a separate line from me so we both didn’t get harassed.

The last little bit of information I have is to make sure you bring good quality plastic bags.  No matter how good a cooler you use to transport your medication anything can go wrong, but you can always get your hands on some ice which will keep the medication at about the right temperature in a pinch.

So the long story short when flying with medication be prepared for delays, don’t be afraid to ask for accommodations it can only help you, and make sure your prepared for airport delays, cancellations, and other obstacles.