When the diagnosis comes... part one

With the diagnosis I've been given for my "Mounier-Kuhn Syndrome", I've become very aware that I may be absolutely living on borrowed time until the judge and jury sounds the gavel on the desktop (lung transplant or death). but for pure optimal mental health, it remains of utmost importance to press on with the most positive attitude I can muster, even on the most difficult days.

When I was first diagnosed, my wife and I both felt helpless given that the doctors did not even recognize what it was I was presenting to them, let alone the fact that there was nothing at all available on the world wide web for me to perform any sort of timeless personal research, in the hope of preparing myself for the future.

Exposing awareness to MKS for me, has become ultimately of huge importance, if only to offer another MKS patient camaraderie and support. 

The question becomes, not what I can do for myself having already lived for this long with my diagnosis, but how can we in turn can help another struggling with this difficult still mostly unknown diagnosis? 

Awareness. 

We believe that is the key for living with this rare diagnosis.

Yes! We've been working away, planning on bringing plenty of awareness to MKS.

To make others aware of the disease, to assist those who are diagnosed with it, and to support anyone in the medical field who might be searching high and low for answers to their medical questions. If we can help them in any possible way, we are willing to step up to the plate, as are others we've met throughout the world currently also living with MKS.

Awareness is most definitely key to coping, to understanding, to moving on, to taking even the smallest step along the long medical journey ahead!

Though I am NOT a professional in the perfect sense of the word by way of respiralogy or medical personnel, by having already lived with and through this disease, it has allowed me to become an true expert on my own health journey at the very least. I have lived through double the years all the doctors thought I would, surprising a whole host of specialists by being alive still today.

My own diagnosis was very difficult to come by, a medical puzzle for an entire team of doctors, internists, respiralogists, nephirologists, and even a world renowned pathologist. I was prepared for and bracing myself for a diagnosis similar to the intensity of a confirmed lung cancer, so not sure what was ailing me, I would have been satisfied with that, knowing what we were at least dealing with. A few specialists wondered about COPD, but none were ready for the breakthrough ahead for realizing such a rare diagnosis, penned on my hospital patient chart.

Fast forward a decade, and sadly "Mounier-Kuhn Syndrome" continues to be a very rare and often unacknowledged lung disease even in the best "respiratory lung" circles.

For instance, my wife made contact with the Canadian Lung Foundation to make an inquiry as to whether or not they could include MKS in their data base system on their website, creating a link to something offering more information on it. As usual to most of our inquiries of the past decade, this is the reply we got in return;

Hello,

 

Thank you very much for your comments to the Canadian Lung Association on Tracheobronchomegaly.

 

I am sorry to hear your husband has this disease, and I commend you for your efforts in explaining it and providing support to others.

 

I can understand your disappointment at not being able to find information on tracheobronchomegaly on the national Lung Association website, www.lung.ca.  The truth is, it takes a lot of resources to research, write, translate, verify and continuously update health information. The sheer number of lung diseases is staggering- there are major long-term lung diseases like asthma and COPD (emphysema and chronic bronchitis), infectious lung diseases, work-related lung diseases, lung cancers… it’s a huge range.

 

Sadly we do not have the staff and resources to provide information on every lung disease. This is regretful; I really wish we could do more. The reality is that will small staffs and budgets we just can’t get to everything we’d like.

 

I will note in our files that there has been a request for information on tracheobronchomegaly, and will share this with my supervisor.

 

You may also want to call the respiratory health educators at the Ontario Lung Association. You can reach them toll-free at 1-888-566-LUNG (5864)

or by e-mail at info@on.lung.ca .

 

I wish you and your husband all the best.

 

 

Regards,

 

Mary McNeill

Information Specialist / Documentaliste spécialisée

National Lung Health Framework / Cadre de travail national sur la santé pulmonaire

The Lung Association – National Office / L'Association pulmonaire - Bureau national

1750 Courtwood Crescent, Suite 300

Ottawa ON K2C 2B5

** For all newly diagnosed patients, stay tuned as I'll be offering some practical suggestions in the part two of this article, coming up soon. 

Rare Disease Day - February 28th, 2011

Get involved! 

Find you more about this great designated day by clicking over to their website, located HERE. 

Still working hard behind the scenes. 

• Mounier-Kuhn Syndrome is now on TWITTER, using our handle of "MounierKuhnSynd" (**no hyphen all one word), and nicely attached to every lung association and foundation worldwide, as well as every respiratory type of doctor and professional we could find. Just getting the name circulating will assist in MKS awareness. 

• Mounier-Kuhn Syndrome Journey is now also set up as a community page on Facebook. Come on over and "LIKE" the page, also aiding in the goal to create even more awareness to this rare disease. 

 

We are hoping by this time next year, Mounier-Kuhn Syndrome will be a lung disease featured on the "Rare Disease Day - 2012". 

How are you bringing awareness and helping get the word out about MKS? 

We'd love to hear from you.

Bringing awareness to MKS

Apologies for the delay in posting here. 

May we extend a sincere new year greeting to you and your loved ones for a bright and healthy 2011. 

Here's to bringing even more exposure and awareness to MKS.

Happy New Year!

Please be assured in our absence of posting for a time, there has been much happening behind the scenes. We hope to reveal details on something new and exciting right around the corner, tweaking ideas and possibilities to create a more interactive type of website for those with "MKS".

Over the past while, it has become emotionally happy for us as we've come to the conclusion on the reality that this blog has created baby steps towards a sorely overdue platform for bringing awareness to the forefront on "Mounier-Kuhn Syndrome (MKS)". 

With that as our end goal, we will continue to post anything of informational interest to share with others with this same disease, or to assist the medical field with detailed reference items. 

We continue too to receive emails from those who either have been recently diagnosed with the disease, or who have been living with it for a while now, somehow searching the internet in their attempt to educate themselves more, finding us awaiting them here. And we will be here, available to assist (if possible) anyone who comes in contact with us.

In October, 2010 when the "Canadian Respiralogy Conference" convened in Vancouver, B.C., very obviously MKS was not noted on their agenda.

It seems for all intensive purposes, the mighty two lung diseases which seem to carry the most scientific study are; COPD and Pulmonary Fibrosis. In reality though, there may be more population diagnosed with these two diseases, however there are actually many dozen more lung diseases topping "The Canadian Lung Disease's" own lists, and it's mind boggling that still in 2010, MKS has not actually been documented on such worldwide lung diseases informational lists yet.

Interestingly, the same group of Canadian respiralogy professionals will meet up again next month in Niagara Falls, Ontario, so we can only hope a continued possible growing "awareness" of MKS will eventually be brought to their conference forum.

What little information currently rolling round our globe is from people like you and I who have either been diagnosed with MKS, or from helpful doctors of MKS patients trying to get what information they can into their fields of study by way of using medical journals to record them. Truthfully and sadly though, and often I've seen it as the norm, there are some who may be working on a different agenda, hoping to document their findings or patient diagnosis for the purpose of gaining only possible medical notoriety. I'd like to think these are few.

Always interesting; if worldwide medical journals were filled with actual patient findings to offer further diagnostic and practical advice for MKS patients, then one has to wonder why the only prognosis (still many years later), is to only suggest one things - a life saving operation of a double lung transplant for their futures.

While walking the journey of MKS the past eleven years, I can only suggest a helpful solution would be to continue bringing even more awareness to this disease for rendering further immediate understanding amongst every medical field out there from the paramedic to the hospital emergency wards, and permeating through every professional beyond that.

If two Internists, and an entire team of  Respiralogists were initially unable to even read a simple chest xray of my lungs in my early diagnostic stages, then it's obvious continuous education needs to ripple throughout every single department of all medical fields.

Very recently we received the note below, left as a comment to one of our blog posts.

Immediately our hearts went out to the author, because that was us eleven years ago now, grasping anything we could find to learn how to cope with the MKS diagnosis given to me.

 My name is W. and recently my husband was diagnosed with MKS. Unfortunately he was seen (and diagnosed) by the most incompetent medical staff you can imagine. I am desperate to find a doctor that not only treats this, BUT KNOWS WHAT IT IS!!! 

Please, if you have ANY information that may lead us to who we need, it would be greatly appreciated. Thank you so much for creating this page and; I wish nothing but the best for you and yours.

 Sincerely, W.

Our sole comfort for those who may be recently diagnosed like W's husband, is to please be assured - due to the abundance of lack of information available to medical personal, let alone the lay person, this is the sole reason why this MKS blog was born in the first place.

We know there are others like us around the world who would greatly benefit from having an actual support system for our disease, so if for any reason, this is our hope, that we may offer a tangible way to bring something into cyberspace which may assist another with the whole topic of MKS.

With MKS awareness in mind and for this very reason, I accepted the invitation last summer to have a professional photographer follow me around during my regular respiratory physiotherapy session. Photos were taken throughout my workout while following my usual regime and directive from my physiotherapist during the afternoon.

I am told over 250 photos were taken of me, and I was perpetually teased about becoming the new poster boy for the program. If that's what it takes, to have my medical diagnosis made public, and to have my photo taken while I am trying to keep physically active for possibly being able to fend off any bit of continued digression of the disease, then of course I will participate.

To my fellow "MKS" patients out there, I want to issue you a 2011 new year challenge;

How are you bringing further awareness to our shared disease? 

Let's get our diagnosis more known shall we?

If there is something you're currently part of, or you have an idea to get something rolling, we'd love to hear from you. ,

In the near future, we hope to have an informational video posted on YouTube and we have a few other ideas rolling round our heads. Your further suggestions are very welcomed, please feel free to email us with them. er

Also, we will be hosting MKS stories behind a special tab at the top of this blog, stories by and from those who have been diagnosed with MKS, or from a medical personnel who may be currently treating one. We invite your submissions, which we ask to remain anonymous with the exception of sex, age of diagnosis, country and general geographic area. These will be collected and posted here on this blog for others to read for support, possibly finding similarities in cases to further assist the development research for better prognosis in the future. Entries should be to the point, so they aren't too lengthy.

Have an MKS story you want to share with others?

We invite you to bring it forward.

Spirometry - Pulmonary Lung Function testing (PFT)

Who should have a lung function test?

Spirometry machine used to perform a PFT

Spirometry is most common of the pulmonary function tests (PFT), measuring lung function, specifically measuring the amount of volume and/or speed flow of air that can be inhaled or exhaled.

Spirometry is an important tool to assist in accessing lung condition, lung health issues and lung function.

In the photos below;

One of the many pulmonary function tests I've endured to determine the complex lung health issues of my diagnosis of "Mounier-Kuhn syndrome". This time though, I was asked if a class of nurses could observe my testing. 

I hope they walked away with further information on the importance of this spirometer equipment, and never forget how they were enlightened about what in the world MKS is!

Who then should have a lung function test? 

(Hint; Watch the movie below, for you'll be surprised ....)

The importance of respiratory physiotherapy

We've been remiss for the past little while to post here, but assure you all will become much more active in the near future. We have much to share, and apologize if you've yet to hear back personally via emails, those too will be caught up very shortly. 

In the meantime, we'd like to share this brand new video clip produced by Dr. Roger Goldstein, the respiralogist in charge of the (cutting edge) respiratory physiotherapy program I attend twice weekly here in Toronto. Patients from all over Canada travel in the hope of being assisted here. 

Although this clip focuses on those with COPD, all those who attend the in or out patient program at Westpark Hospital actually represent many assorted and various lung diseases, and not just COPD. 

I spent seven weeks as an inpatient, living at this physiotherapy hospital almost two years ago now, the best thing to come my way since my diagnosis with "Mounier-Kuhn Syndrome" for sure! My wife jests with me often that it compares to the "Hotel California", because one can always get in but never leave. And I've never left, that's true. Even the staff there have informed me - I am theirs now (forever more), with at least one mandatory yearly inpatient refresher course to monitor my lung and overall health for continued care through both pre and/or post transplant.

My initial hospital exit pass from that seven week stay was only offered under the condition I promised to continue with three more months of twice weekly out patient sessions. These though have remained a constant necessity as the staff feel my case renders a longer and more continuous flow of monitored and charted health care. 

Here is where I admit, this hospital has steadily become a lifeline to me with care and support like no other, with staff (same as shown in the video who also care for me), and patients also suffering from lung disease who absolutely "get" what I'm also having to deal with. 

Thank you Dr. Goldstein for implementing this program, and for all other respiratory doctors across Canada who are bringing it to other cities in order to assist others with lung diseases. 

Now we are SIX

Two more people have made contact with us, two who have themselves also been diagnosed with Mounier-Kuhn Syndrome. 

Now we are SIX.

With each new contact, sharing information often becomes enlightening. For instance, take a peak at the comment introduction underneath of our "Hypoxia Altitude Simulation Testing" post.

From commentor "K" leaving a wonderful post of introduction, with that he/she has left two golden nuggets in the wake of posting. For instance, we had never heard of;

"Tracheobronchomalacia", a medical diagnosis strikingly similar to that of MKS. And then, secondly, we had never heard of ;

"Tracheobronchoplasty"; surgical central airway stabilization with posterior tracheobronchial splinting using a polypropylene mesh improves respiratory symptoms, health-related quality of life, and functional status in highly selected patients with severe symptomatic TBM.

Our thanks to "K" for posting these two tidbits of information from the personal story offered. This was great information to add to any MKS patient's portfolio, especially the latter when it comes to seeking a better quality of life when degeneration becomes of serious concern. 

For someone like myself, someone who is actively in the hands of an organ transplant surgeon, there are hopeful bits of information for alternative solutions which come our way definitely worth a personal research.

Speaking of appointments to continue tracking degeneration and future possibilities, we attended such an appointment this past week, once again reaffirming the fearful thought of being someone who must endure a double lung transplant. The prospect of such a life saving, yet life threatening operation and recovery, is overwhelming. 

 

Questions on the notepad for the next appointment continue to be jotted down, two at this time new for inquiry are;

- We cannot seem to find whether or not I was tested for a blood testing to check on my "CRP" levels,  which is invaluable for seeking levels of inflammation in the body. The normal level is "10" or under, and some folks with respiratory disease can show levels of over "200". When the body fills with additional stressors such as inflammation to extremes, it just becomes one more thing to contend with, and one more thing to create even more concerns over. 

- Tracheobronchoplasty is something we've now spent time researching, and though like all surgeries it does sound risky in many ways, the medical data around the internet offers information that four MKS patients have successfully sought this alternative surgery for better quality of life and the ability to have a greater pulmonary function. 

(So there are also three more out there somewhere sharing this disease...)

"K" our commentor did mention he took approximately six months for a total recovery and how it has assisted in greater wellness. Others can take from 6-9 months for full recovery from what we've read on this individualized medical surgery.

For further study, here are a few live links below you can click on concerning the topic of Tracheobronchoplasty, that is, if you care to read and research this topic on your own;

• What is, Tracheobronchomalacia?

• Tracheobronchoplasty, what is it? 

• Esophageal tracheobronchoplasty for diseases of the central airway.

• Tracheobronchoplasty for severe tracheobronchomalacia: a prospective outcome analysis.

Autumn; a time for colds and flu right around the corner now

Autumn...

In Autumn, there is beauty everywhere one looks. 

Mornings are crisp and cool, not so great for those suffering from lung disease. It hurts to suck in cold air, caution is necessary when breathing outdoors. 

This is the time of year when everyone seems to get a cold from the change of season, those very cold mornings and hot afternoons, when no one is really acclimatized just yet to the great temperature variations.

The flu shot clinics begin, and masks fast become the norm for people like us who have to protect ourselves from illnesses and the possibility of swift rolling lung infections that knock us over medically and can present very serious complications to our very lives. (And for those who have suppressed immune systems, or are extra sensitive to illness, please be very careful if you know someone who has recently received the flu mist, as it is still contagious for up to three weeks afterward.)

Simple protection in a home filled with children/teens becomes necessary, either for those who are well to avoid those who are ill, or to isolate the sickies from the rest of the family/friends. 

"Shoppers Drug Mart" in Canada is a large pharmacy store, one which stocks several types of surgical masks in their health section. 

At this time, we have two types in our home; super surgical masks with elastic side to wrap around the ears, or a lighter mask with double tie options. 

Everyone here seems to prefer the elastic around the ears, however the double ties are great too for those who prefer to avoid tension for any tenderness around the ears. 

The only draw back that we've seen so far is how easily the ties can rip off, or the feeling of clumsiness to get them tied up in the first place. I think when the tie backs are all used up, we'll resort to only have the elastic ones around here. 

If you are a mask user, which type would you prefer? Or can you recommend another type of surgical mask for those who require protection from sickies in their midst? Love to hear from you. :)