#RussellsWish - Children's Wish

2 more sleeps until we are off on Russell's Wish Trip!

This was the subject first thing this morning when Russell got up.  In fact for the past week...sleep has not been one of his highest priorities.  Wednesday morning he got us up at 5:00AM because "he couldn't sleep."  If you think Russell is excited; Nicole is just as excited times 10.

This is a very small price to pay to see the excitement on our little guy's face.

When we were first introduced to the idea that Russell could be granted a wish by Children's Wish, Russell was still very little.  Too little to really understand what was going on.  Our other complication was Susan's health.  We've had setback after setback that put Russell's "wish" on the back burner.  Partly out of necessity and partly out of design.  Because Russell was so little, at the time, we wanted to delay as long as we could because we wanted him to be able to remember his wish and to fully participate and understand what was going on.  Very tough for an infant/toddler to do.  So we dragged our feet filling out the paperwork and getting all our documentation in order.  We really only got serious about this a year ago.  I hope the folks at Children's Wish weren't getting impatient with us as we weren't exactly springing into action.  Sorry about that.

It's been an emotional journey getting to this place.  We've had a lot to overcome physically and mentally.  Getting ready for this trip was something that we had to make a priority.  We had to put aside all of our excuses and just make this happen.  In other words it's time to "get a life."

Honestly, we could make a very good argument for not going.  Susan's health at this point is precarious, and in many ways she probably has no business going...BUT WE"RE GOING!  There have been some very strange phone calls to Susan's Cardiac clinic verifying that Susan is OK to fly and that they are OK with us doing this trip.  So we are taking reasonable precautions and will have to be careful.

We're going to relax and have fun!  This is about our kids having a once in a lifetime experience.  For many years they have been short changed because of our circumstances and that is not happening next week.

For the better part of 8 years we have tried to get to some semblance of "normal."  In some instances with disastrous results.  Does that make me nervous...you bet.  Is that going to stop us ... No Way.

Stay tuned for many fun Twitter, Instagram, and Facebook posts from Florida.  Hope to have time for a blog post...but not making any promises.

For any one interested in where we are staying. Here is a link to Give Kids the World.

https://www.gktw.org/

This should be fun!

Missing the Point on Hospital Parking

I've been involved in patient/caregiver advocacy for about 7 years now.  I get to hear a lot of patient stories and patient experiences.  As a hospital board member I hear a lot about what patients complain about in health care. I've noticed something interesting.

Complaints about things like cost of parking and the quality of food are very common.  Are these real issues?  Most definitely.  These are real issues but are they the most pressing issues in health care.  Not by a long shot.

Are complaints about non-medical issues like parking and food quality a function of growing frustration with health care?

This seems like a fairly innocuous observation but it highlights a bigger problem.  Health care is extremely intimidating for most non-medical people.  If you have a concern about a medical issue, it can be a very difficult process.  You are dealing with a significant power imbalance as it is you against a very technical bureaucracy.  When a subject like the high cost of parking comes up patients and caregivers feel a lot more comfortable complaining about that issue than discussing hospital protocols or treatment strategies.

Many people are frustrated with the state of health care.  Health care is often cited as one of the most important issues facing Canadians.  Although I pay an exorbitant amount on hospital parking, it is not in my top ten of health care concerns.  Wait times have become common place and almost accepted in some jurisdictions.  Lack of equipment, poor communication, and shortages of Doctors and Nurses are far more pressing issues than parking.

Parking is one of those issues that is usually the "straw that breaks the camel's back."  Imagine yourself being called for a follow up appointment following a biopsy.  You are waiting for a result from a test, and deep down you know the test result may not be good news.  Your mind is jumping to all kinds of conclusions and you are on an emotional roller coaster.  All before you even arrive at the appointment.  When you make your way to the appointment you attempt to find a parking spot.  You drive in circles and hunt and hunt.  You can't find one.  Your emotional state has now jumped off the chart.  In desperation you find a meter to park at.  You throw whatever change you have at the meter and hurry to your dreaded appointment, hoping to be back before the meter expires.  You head off to your appointment where you hear the dreaded news.  You have cancer.  The rest of the appointment is a blur as very caring and competent staff explain next steps.  You hear the words but only a few register.  Many of the words you don't even understand.  All you recognize are words like "surgery" and "radiation".  You are dazed.  Because of the diagnosis and the conversations that happen because of the diagnosis.  You end up staying much longer than you had expected.  When you return to your car, dazed from the medical bombardment you have just endured, there it is.  The parking ticket.  It is at this point, alone and confused, with a clenched parking ticket in your fist where all the emotion comes together.  This is the breaking point.  Although the parking ticket is what may set you off...it is everything that lead up to that point that is the "real" issue.

Although the scenario I described above is hypothetical, I've had very similar experiences to this.

The one thing I have had to remind myself over and over is to prioritize.  I need to concentrate on the people I care for and how I support them.  I need to focus on issues that effect their health.  Parking fees are an irritant but they are not what I need to be focused on.  It's not that important.  Ironically, when I discuss issues in health care, the subject of parking usually comes up.  It's an issue people gravitate to again and again.  It's just something that resonates and that anyone can relate too.  We are missing the bigger health care picture.

Together We're Better

Many years ago when I started out in business school I was introduced to the concept of "synergy."  At that time business schools were starting to emphasize the value of effective teams.  We did many projects, case studies, and presentations as part of a team.  It seemed like a logical concept and with minimal life experience it was just something you readily accepted.  I was just there to learn, get course credit, and move on with the intent of applying this somehow in the workplace.  Did I really believe that the "whole was greater than the sum of its parts"?  I am not sure but the concept made sense and it wouldn't be until years later when I really began to understand the concept in an applied form.

Patient and family centred care has embraced the catch-phrase "together we're better."  This is just an easier and a little catchier way of conveying the concept of synergy.  How a group of people working together can achieve much more than if they were to embark on a task by themselves.  For patient families this is especially true, due to the fact that families are outsiders in the health care system.  Access to health care is one of the biggest barriers patients face.  Many of the things I have been able to provide feedback on or where I have been a part of changes has only been done by our medical team accepting us as equal partners.  In the end we have learned from each other and enriched each other.

The past few weeks have been rather rough on a few families we know.  Susan and I attended a funeral a couple weeks back of a young man who passed away recently due to cancer.  Like many who have followed my blog and our care page.  I followed their experience through their blog.  Reading someone else's experience gives you such a feeling of helplessness.

This family was very much on my mind as I spoke to nursing students at Red River College this week.  I was once again given the opportunity to share our story about Russell's transplant and Susan's heart condition.  The whole time I was thinking how through all that we had been through that the 4 of us are still intact.  We have not lost a member of our team.  We're a little battered and bruised, and we have many health challenges ahead but for today we are together.

When you hang out with some of the sickest kids in Western Canada you are taking a significant emotional risk.  Not all of the stories have happy outcomes.  These families have taught us so many lessons.  We are much different people than we were 8 years ago.  It has made the bond between the four of us much stronger.  No matter what the future holds we will go through it together because we know that we're better when we're together.

Hospital Parking: Is this a Big Issue for Patients/Caregivers?

A couple of years ago I was asked to participate in a consultation as part of the Patients First initiative conducted by Alberta Health Services.  I participated with a group of patient families.  Many familiar themes were heard.  Being involved in Family Centred Care for several years, you start hearing the same issues over and over.  Parking costs are a subject that usually comes up with people who have spent significant amount of time at a hospital.  I have some theories as to why parking is often raised as an issue by families, and I don't think all of it is financial (something I may address another time.)  The fact remains that for many patients and caregivers this is a real issue.  In our own situation it is not hard to rack up costs of over $100 for a 3 day hospital stay.  Two parents doing overlapping shifts at a hospital is a very costly proposition if you plan on parking.

So let's make parking at hospitals free.  Good idea?  Let's think about that for a second.

Given the location where most hospitals are located, parking is expensive.  Something that requires a great deal of infrastructure.  Free parking is just not realistic.  Many argue that patients and their families don't chose to be sick but pay exorbitant hospital parking costs out of necessity.  They feel like they are being taken advantage of.  The reason for this is not because people have a problem paying for parking, the problem is how the fees apply to patients.  The way the fee structure works is set-up for long term "parkers" like staff; not for infrequent uses like patients.  The fees are also set-up to maximize profit.  I have no problem with profit, but if it unfairly targets patients I do think that is a legit issue.  I don't believe parking was intentionally set-up to target families, I just don't think how families use parking is something anyone ever thought of.  Like many things in health care.

Patients feel ripped off because often times they go to the hospital and wait.  While they wait for whatever appointment or procedure is being done the parking fees are mounting.  A patient does not control how long they have to wait.  What they pay for parking is just one more thing in the health care system that they don't have control over.  Loss of control is a huge issue to patients, and their families, and parking represents another thing they have no control over.

The other issue related to parking is that hospitals generate a significant amount of revenue from parking which helps support hospital operations.  How significant are these revenues.  In Ontario, parking fees represent $100 Million dollars annually.  That is important revenue that pays to support parking infrastructure and other hospital services.  For government to subsidize parking just takes health care funding from one bucket to another.  It doesn't really accomplish anything.  I take issue with health care funding being used to support non-health related costs. There has to be better ways to address this issue.

So how can we help patients and their families?

This is where I have a confession to make.  I can afford to pay for parking.  It isn't going to compromise my lifestyle to pay for hospital parking.  When my son is in hospital, and my wife and I park two vehicles we pay a ridiculous amount.  Notice what I said..."we take two vehicles."  If we can afford to own, operate, and insure two vehicles we should be able to afford to park them.  I don't like it but we should be honest about this issue.  Also, we qualify for the Disability Tax Credit which saves us a huge amount on taxes.  The purpose of this credit is to acknowledge that people with disabilities incur significant costs related to how they manage their disability.  We have absolutely nothing to complain about. That is "our" situation, that is not true of all families.

My proposal to offset some of these costs would be to change how some parking costs are charged to recognize how families use parking at a hospital differently than staff or other people who go to a hospital for work/business.  Here are a couple of family friendly ideas; I am sure there are others:

In/Out Privileges

In/Out privileges would be a huge plus.  When you are staying with someone at the hospital you need to leave and come back to deal with other life issues that are going on. Sometimes it is just a good thing to get out of the hospital to get a dose of fresh air and a home cooked meal.  Perhaps, you are balancing work and being a caregiver.  You could be going to the hospital a couple of times a day.  Each time starting a new tab.  The first hour in the parkade will be your most expensive.  Which brings me to my next point.

Change Fee Structures

My issue with the way parking fees work is that they unintentionally target patient families.  Many times we go to hospital for clinic appointments or blood work.  Often times I am there just long enough to incur the half day rate; which kicks in after 60 minutes.  I end up paying for 3 hours of parking that I don't use.  Using meters aren't a great option either because patients have no control over how much time they are going to spend.  You may have to run out and plug your meter or risk a parking ticket.  Not easy to do if you are their by yourself with a toddler and waiting for a Doctor. You don't want to miss your turn.  Some suggest that having day/weekly passes may help.  One of the problems with this scenario is that our visits to the hospital are often unpredictable.  Length of stays can vary dramatically.  Again, something patients have very little control over.

Technology

My previous two suggestions do require a loss in parking revenue, albeit not a huge impact, but it is a loss.  That is an important consideration.  My third  suggestion involves using modern parking technology.  Hospitals do seem like they are still stuck in the technological back woods at times, an industry still dependent on fax machines.  My technological solution is to use pre-paid parking passes that could be pre loaded and used in any hospital parkade.  That is not a cost saving but it allows some interesting possibilities.

There are many friends, supporters, and charities that could help with parking costs for people who legitimately can't afford it.  Many charitable organizations are realizing the extensive non-medical costs related to health care.  The David Foster Foundation is an organization that was set-up exclusively to address non-medical costs incurred by families going through the organ transplant process.  An absolutely fabulous organization.  When we were in hospital for 157 days there were many times that we were approached by concerned friends wondering if they could help us out financially.  There are people willing to help but the way parking at hospitals are set-up it makes it difficult to address.  Having pre-paid passes could allow charitable organizations or concerned friends to purchase passes for a patient or their family.  It would be a meaningful and tangible way to help.  I am aware of at least one organization that has tried to help out families with parking costs but there was no effective way of doing it so the project was abandoned.  Pre-paid cards could make this a really easy process.  It would just require a change in parking technology.  The key benefit is that this would not effect health care funding.  The other benefit is that this is a direct benefit to families who need it.  It is time for all of us to realize that there are many costs in health care that are not covered by our government systems.  We need effective ways to offset these costs.  This is just one way of doing it.

These are just a couple of ideas that can make a difficult time for families a little easier.  I am sure there are many other ways that can help, we just need to be a little bit more creative.

Learning from Judicial Inquests into Health Care Incidents

Today the recommendations of the Inquest into the death of Heather Brenan have been made public.
I take particular interest in this inquest as we were in the Emergency Room only a few days before Heather Brenan arrived at Seven Oaks General Hospital (SOGH).  We had front row seats to the circumstances that existed at SOGH during January of 2012.  A few weeks after our experience I wrote a letter to management at SOGH.  I received a very thoughtful and reasonable response from the Chief Medical Officer.  At the hospital's request, I consented to have my letter shared with the staff at SOGH.

Here is an except from that letter written in February of 2012.

Events of Jan 12-13, 2012

My wife was taken by ambulance to Seven Oaks General Hospital Emergency Room after she was in a car accident at about 5:30 PM, Jan 12.  The injuries she sustained were not life threatening and when she called me to let me know, she suspected she may have a broken foot.  She arrived at the ER at about 6:00 or shortly after.  I arrived at about 7:00 as I had to find child care for our two children and drive to Seven Oaks, as we live in the Lockport area.  During this time she was in extreme pain and probably had some symptoms of shock.  My wife (Susan) as previously mentioned has a Congenital Heart Condition, Hypertrophic Obstructive Cardiomyopathy (HOCM).  As part of the treatment for this she has an AICD.  This information was conveyed to emergency services personnel and to the triage nurse upon arrival at Seven Oaks.

Susan saw a physician assistant at approximately 8:30 PM.  I believe the attending physician was Dr Van Dyk although we never actually saw him/her.  X rays were taken and the diagnosis was one broken toe and one dislocated toe.  The dislocated toe was reset and the toes were taped and we were sent home with a prescription for pain medication.  We left the hospital approx 10:00 – 10:30PM.

The next morning, Jan 13^th, we received a phone call from the SOGH emergency room.  The caller stated that the Susan’s x-ray had been reviewed by a radiologist and that they wanted us back at the hospital for a CT scan and that Susan would likely be referred to an orthopaedic surgeon as the foot is broken.  We were told to come into the hospital and let the triage nurse know that Dr Abbott had requested her to come in and to let him know that she was there. We proceeded to the hospital and arrived at 11:00 AM.  We notified the triage nurse and explained why we were there.  We waited for nearly 3 ½ hours.  Once we were taken to a treatment room…we received an apology from Dr Abbott as there had been a communication error and that Susan was supposed to be brought right in and not have to wait for hours.  We were waiting so long that we nearly missed the CT scan as the staff were heading home for the day.  We did get the CT Scan, which confirmed a diagnosis of a lisfranc fracture , and after that there was significant amount of discussion about the best course of action.  We ended up leaving the hospital at about 9:00 PM.  In the end, Susan was referred to Dr Chris Graham at HSC, where she had surgery on her foot.  As I stated earlier, the outcome of our experience was a good outcome.

Concerns:

• I was very upset at the condition my wife was in when she was dropped off by emergency services at the emergency room.  She was placed into a wheelchair, and placed in a corner by herself, and ignored.  She was in so much pain that she was in tears when I arrived.  From what my wife told me she received first class treatment from the paramedics at the accident site, but when she arrived at the hospital it seemed she was forgotten about.
• On several occasions, we reminded the staff of Susan’s heart condition.  We got the reaction we usually get when we explain this condition…a blank stare.  I am convinced no one had any idea what we were talking about or had any clue what HOCM is.  For a patient such as Susan she should have been put on a monitor.  Not once did anyone inquire anything about the condition or acknowledge our concern.
• The fact that the fracture was missed in our first visit is a concern, but I view this as a medical issue that I am not qualified to comment on.  From my understanding, a Lisfranc fracture can be difficult to detect.  I was pleased that the error was caught the next day.  Obviously the procedure works; as the error was found upon review.  I would think that a typical incident report would be done when these types of issues come up.  I am confident this can be managed in house.
• The communication in the emergency room seems to have failed as the information about my wife coming back to the Emergency Room should have been conveyed to reception at Emergency and Dr Abbott notified that we were there.  We assumed this happened but it did not.  When you ask a question of Emergency Room staff you feel like you are imposing and monopolizing someone’s valuable time.  You are expected to patiently wait and not ask questions.  
• We were somewhat frustrated by our experience at Seven Oaks but in the end Susan had the surgery she required and Dr Abbott did a good job of advocating for us.  However, when Susan received her follow up at HSC, we conveyed some of our experience at Seven Oaks to the staff at HSC.  The quote from them was…”that is why they call it Seven Jokes.”  I did not really appreciate that comment.  This is an off-hand remark that really does not matter; but is this the perception that is out there?  As a leader in that facility…that would concern me.

Opportunities

• You have a dedicated and hard working staff; that is very capable.  The concerns I have stated above (if you investigate them) will likely determine a root cause of a failing of a policy or process.  People make mistakes and have errors in judgement.  It should never fall to one person alone to make a system or process work.  
• Own the Waiting Room!  The treatment process (in some form) should begin as soon as you walk in the doors of the hospital.  My feeling is that just about anything could happen in the waiting room and chances are no one would notice what was going on until it was too late.  Waiting and delays are part of the process, but people’s condition can change significantly in the hours they have to wait.  Those who are in the waiting room need to be monitored and checked occasionally.  Perhaps even talk to them.  The feeling we got was that until you actually make it into a treatment room you were not really in the hospital…you had not yet penetrated the “system.”  The way the Emergency Room is constructed you cannot see the vast majority of the waiting room from the triage desk.  Compare this to the Emergency Room at Children’s Hospital, where you can see the entire waiting room from the triage desk.  If anything happens in that waiting room…it can be seen.
• The general public does not know “hospital-speak.”  We, as patients, do not know how to access the system nor the terminology to do so.  Critical information can be misinterpreted and misunderstood.  For those of us who have spent extended periods of time in hospitals we have had to learn how to navigate the system, and it is challenging, even for those who know how the system works.  There needs to be a better way for communication to happen between patients and medical professionals.  I cannot emphasize how critical this is.  When we brought my son into emergency on Oct 2, 2008, if we had not chosen our words carefully and asked the right questions our son would have died in the waiting room.  It is because our cardiologist prepared us ahead of time, and educated us, that we were able to communicate effectively and ensure that our son was seen immediately as opposed to waiting.

My purpose in sharing this letter is this. When you consider all of the cost and time of a judicial inquest, is it not more effective to listen to patients and caregivers and learning from their feedback?  

Patients are capable of providing valuable feedback that can lead to meaningful change and do it far more quickly.  As a result of my feedback to SOGH I was asked to participate in an process review of the Emergency Department processes at SOGH.  I was able to provide candid feedback, and more importantly learn about many of the challenges faced by staff in city Emergency Rooms.  I gained a new respect for many who work in our hospital ERs but was very disheartened to learn of all the challenges and shortcomings of our health care system.

I think judicial inquests do provide valuable information and important recommendations.  My concern is that it seems like an immense amount of effort to get to information and improvement that should be readily available.  It is reasonable that with an effective incident investigation and input from all stakeholders (including patients) to improvements can be made in weeks; not the years it takes for the judicial process.  Changes need to be made quicker and more efficiently.

I was fortunate enough to be able to engage the hospital and provide feedback through my letter, and my continued relationship with the hospital.  However, I am curious what Heather Brenan would have shared with the hospital about her care at SOGH?  Unfortunately, even with a judicial inquest. that is something we will never know.

Merry Christmas 2015

Another Christmas Eve is upon us.  Many people are doing their last minute preparations for their annual celebrations.  This is a day that I will always be reminded of our Christmas at the Stollery Children's Hospital as Russell was being kept alive on a Ventricular Assist Device (Berlin Heart) and he was waiting for his heart transplant.

Christmas Day 2008
Stollery Children's Hospital  4C - ICE

Susan and I remember this Christmas very differently.  As strange as it may sound I enjoyed this time.  Russell was stable, for the most part, and all of the efforts the hospital made to make Christmas enjoyable for all of the families was enjoyed and appreciated.  Last year I saw a picture of the preparations that were made by the hospital for Christmas Day.  Child Life does a phenomenal job of putting on a very special Christmas morning.

Christmas Preparations at the Stollery
Santa's Workshop

Susan remembers this Christmas very differently.  As she describes it, she would be happy wiping this memory away forever.  At this point we were still waiting for Russell's donor heart and our patience was being tested.  The Berlin Heart had provided a capable bridge to get us to the transplant but even that was risky.  We were one clot away from a stroke or there was also the risk of bleeding due to the anti coagulation.  It was a tenuous balancing act we were performing and Susan felt every but of that pressure.

In the midst of all of that chaos, we did manage to celebrate Christmas with family.  For better or for worse it is a Christmas we will never forget.  We are also reminded how far we have come in 7 years.

Nicole and Russell - Christmas 2015

As Susan had her procedure earlier this week we were also reminded that our journey continues and is far from over.  We will enjoy every moment we have together as we think of all of the families who will be spending the holidays in the hospital.  We hope they can find joy in trying circumstances.  For all of our friends who are spending Christmas in one of our hospitals we wish you a very Merry Christmas and to all of the volunteers and hospital staff, who make a Christmas in hospital a little more enjoyable, a heart felt "Thank You"; your efforts are greatly appreciated.

The note below is for all of the fine people who help make Christmas in the hospital possible.  

'T'was the Night Before Christmas

'T'was the night before Christmas, 7 short years ago,

Where we found ourselves, far from our home. 

I remember the cold;that memorable night.

As our tiny boy, was far from all right. 

In the Stollery's care our boy stayed in good stead

His heart beat held steady with a device by his bed.

Our future uncertain, our outcome unknown

A new heart for Christmas; our impatience had grown.

Our journey had started three months before.

  His heart fell ill and could not take any more.

My 8 week old boy's heart was nearing the end.

His heart was sick and could not be mend.

ICU and hospital our new home became

A heart for a transplant became our new aim.

The days turned to weeks and our fate seemed so grim.

The watching and waiting was doing us in.

One offer, then another; not quite a right fit

Could Christmas bring that one special gift?

Christmas day came, our gift did not come

Another day gone; unanswered prayers to some.

 

One day passed and then another, an end not in sight

We doubted and fretted; would our boy win this fight?

But unknown to us the stage had been set.

In a city far away in the Pacific Northwest.

Another young boy was clinging to life.

A story filled with sorrow and unfortunate strife.

The stage was now set; a decision was made.

A call of compassion and plans were well laid

Early that morning just before the New Year

A heart had been found; joy replaced fear.

The waiting was over; a new chapter began

Our gift of new life was all part of the plan.

As I think of this time and memories so fond.

We stop and remember my son's kindred bond.

A selfless choice, by a father and mother.

A new beginning for us; an end for another.

At this time of year I remember our plight

And our special gift that ended our fight.

Our Christmas wish to one and all.

Is of joy, and peace, and thanks above all.

Merry Christmas from Donald, Susan, Nicole, and Russell

What is Hypertrophic Obstructive Cardiomyopathy (HOCM) ?

I assume some of my blog posts over the next little while may have something to do with Susan's Heart Condition.  I thought it would be a good idea to back up and explain exactly what she is dealing with.

Susan's condition is a congenital heart defect called Hypertrophic Obstructive Cardiomyopathy (HOCM).  Congenital Defects are common and effect approximately 1 in 100 babies that are born.  Congenital heart defects range greatly in severity and complexity.  Some defects may be as straight forward as a hole in the heart between the heart chambers.  Sometimes these "minor" issues resolve themselves as the child grows and require no intervention.  Other congenital defects are severe and require massive interventions.   This is why pediatric cardiology and surgical interventions can be so complicated.  The medical team can face an almost infinite amount of scenarios.  In some cases they literally have to invent new treatments or surgical procedures based on the patients need.  Nothing cookie cutter about these very complex kids.

What is HOCM?

Hypertrophic Obstructive Cardiomyopathy combines three issues with the heart.  Hypertrophy, an obstruction, and cardiomyopathy.  Here is an explanation of each issue that comprises HOCM.

Hypertrophy

TOP: Normal Heart

BOTTOM: Hypertrophic

Hypertrophy simply meaning thick.  The wall of the heart is thick and inelastic.  What you need to remember is that the heart is a pump.  The walls of the heart should be very elastic in order to function.  With HOCM the myocardium becomes thick and inelastic (as shown in the picture to the left).  Imagine a tire tube.  A tire tube is easy to form and as you inflate it with air it changes shape very easily.  That would be similar to a normal heart; very pliable and elastic.  As you picture a tire tube, contrast that with the tire the tire tube might go into.  The rubber of the tire is thick and firm.  Does it change shape as you fill it with air?  Yes...but it changes shape slowly and requires a great deal more air pressure to change the shape.  This is very similar to the HOCM heart.  Very thick walls that takes a great deal of effort and time to expand and contract.





Cardiomyopathy

A cardiomyopathy is very simply a disease that effects the muscle of the heart. In HOCM, the normally congruent alignment of the muscle cells are malformed.  The cells are out of alignment and enlarged.  This is what causes the thickening of the walls of the heart. In the diagram below you can easily see the issue when you compare normal heart muscle cells in contrast to the disarray of the cells with someone who has HOCM,  In addition to the thickness of the myocardium that this causes it also can significantly weaken the muscle.  So you are left dealing with two issues with the heart.  The thickness of the heart and also a weak heart.

The important thing to keep in mind when you consider the thickness of the wall of the heart and the inherent weakness in the muscle is that there is no treatment to correct this.  The issue is at a cellular level and no medication or surgery is going to alleviate the root cause  It is what it is.  Of course this all depends on the severity of the condition.  Many HOCM patients will utilize drug therapy and can live relatively normal lives with some restrictions.  Others are not so fortunate.

Obstruction

The third challenge that we have to contend with is the possibility of this "thick" heart creating a physical obstruction of  blood flow.  If the muscle grows in such a way that it obstructs flow or interferes with the operation of a valve this can cause other issues and symptoms.  It is safe to say this is not a good thing.  There are some treatments for an obstruction.  One treatment is the septal myectomy (shown left) that physically removes the obstruction.  The diagram shows and example of what an obstruction may look like and how a septal myectomy would be performed.  There is no pretty way to describe this.  You crack the chest, go in, and remove the obstruction with a scalpel.

This is the surgery that Susan had in 2009.  The effect she experienced was immediate.  However, Since that time, the relief that she felt has unfortunately been short lived.  She has been steadily degrading ever since.


Next Steps:

As I have described HOCM, it becomes apparent that many of the treatments are somewhat limited.  Drug therapies, surgeries, and other procedures do provide some relief.  Our referral to the Mazankowski Heart Institute in Edmonton will provide another set of eyes to look at Susan's condition and if there is any treatment that may give some relief.  There is some discussion about another septal myectomy or an ablation procedure.  At this point it would be premature to assume anything.  What we are doing right now is diagnostic and about gathering information.  Decision making will come later.

The Elephant in the Room

Many people who know our story, are aware that Russell has a heart transplant and they immediately ask the question.  Is "transplant" an option for Susan?  At this point...No.  This is part of the harsh reality of the transplant option.  Because supply of donors is so limited transplants are reserved for those with the greatest need.  In essence, you have to be extremely ill to get listed for transplant.  Not a road that anyone would choose.  We also are well aware of this journey having been through it with Russell.  There would be many hurdles to cross and different (more radical) treatments that would be considered first.  If you asked Susan if she would want a transplant, she would likely say "No." The reason being is that she knows what it takes to get there.  Not something she would want to endure.  In discussions with our Doctors, transplant has come up in the conversation but at this point it is not being considered.  This conversation would change quickly if Susan's condition were to suddenly worsen.  Then all bets are off.  As it stands right now we are trying to get information and investigate what is going on.  Possible treatments will be discussed later.

For people who have control issues; this is likely a process that they would not do well with.