Sunday, February 22, 2015

Breathing easy, dairy free, and adrenal fatigue recovery

I don't wish dietary restrictions on anybody.  Especially if they present themselves in the form of a true allergy.  How excluded a child with a gluten allergy must feel when their classmate brings birthday cupcakes.  And these allergies and intolerances are on the rise.

I wan't born with an allergy OR an intolerance of dairy, but the choice to make a change has presented itself.  It began with severe fatigue.  You know that time of day when you realize there's no more 'umph' left?  Usually that time is the moment you know if the dishes haven't been done, they aren't getting done.  Well usually that should coincide with bedtime or at least at couch time.  My servere fatigue also presented itself with anxiety and a feeling of being too fragile to deal with normal frustrations.  The anxiety alone was bad, really bad, but when I started feeling physically drained routinely around 1 or 2 in the afternoon I knew it was time to see what was up.

I went to a homeopathic doctor - she didn't take my insurance, but I said to myself, 'if there's anything worth splurging for, your health is it!'.  I knew with a general doctor I'd end up on an anti-depressant, but I'd done that years before and wanted to see if there's another, safer alternative this time.  You guys this doctor spent a full hour with me, asking my family history, diet, caffeine intake, spiritual beliefs, and support at home.  I left feeling totally distressed.  All I walked out with was a a welt on my butt where she gave me a vitamin B complex injection.  That and a list of recommended probitics, fish oil, and multivitamins.  This doctor didn't give me false hope for a quick fix. She did tell me she would personalize my recovery, therefore needing more information before she could make further recommendations.  Off I went to the lab for blood work and a saliva test (to look at my adrenals and cortisol levels).

THREE WEEKS LATER:  Yes, that three weeks was awful but somehow I was at peace, knowing the waiting game was a necessary step and that I might have some definitive answers soon.  The doctor sat me down and affirmed my lack of energy and emotional strength - I was in adrenal fatigue, my cortisol levels were below normal from the moment I wake all the way through the day.  Cortisol is often called the stress hormone - it's not good to have too much or too little.  It's the hormone responsible for the flight or fight response in a sketchy situation.  When you have too little cortisol, you experience fatigue, sleep disruption, anxiety, depression, blood sugar imbalances, low thyroid function and inflammation.

At this visit we had a piece of the puzzle - something to work on.  Away I went with Isocort, an adrenal support supplement.  She also explained to me how important sleep is to normal adrenal function.  For ease of drifting off to sleep and quieting 'chatter' I was given two herbal supplements to try: Glycine and L-Theanine.  I was also instructed to exercise 4 times a week with at least two of those being outdoors.  We decided to do a food panel to see if an underlying reason my adrenals were being sapped could be a food sensitivity.  She also asked me to up my sleep and cut my sugar and caffeine - the artificial sources of energy that I felt like I really needed to get me through my day.  I had cut out coffee already, anticipating it might be part of the problem, and wanted to cry each morning.  I grieved my morning ritual and I just truly enjoy the taste of a creamy cup of strong coffee.   Her response to my sadness was, "How much stress has it added to your life by cutting out coffee?".  Did I tell you how much I love this doctor's advice??

THREE WEEKS LATER:   Food panel results were in.  I did not notice a relevant relief in my fatigue due to the Isocort, exercise and additional sleep.  On my results I saw some highlighter and red pen, oh crap.  I was excited too though, thinking that maybe there is something I can modify in my diet to get back to normal.  She laid it out for me straightforward:  I had the highest sensitivity to coffee she had seen in a long time.  I was also highly sensitive to dairy.  This was not going to be an easy road to recovery.  She had literally just given coffee back to me, and now it's slipping right through my fingers.

Surprisingly, determination was a stronger force that my addiction to coffee.  Dairy, I found to be much harder to kick - it's hidden everywhere!! Whey in Special K, milk fat in dark chocolate, and cheese,  how I miss cheese!!  I went through a 100% coffee and dairy elimination for 30 days.  To my surprise, I felt exhilarated.  I felt so much energy and such inner strength, I was invincible.  At this point my awesome homeopathic doctor released me, there was no more work to do, the proof is in the pudding.

The amazing side effect of being dairy free is how wonderfully I can breathe.  Dairy is a mucous thickener, due to the protein casein and if you have tracheal stenosis, you know how much more stridor you experience if you have thick mucous.  I haven't had a dilation in almost two years, and I'm feeling great!!

If you are interested in trying a dairy elimination, do at minimum 30 days to see if it helps you.  Check out the Go Dairy Free website as a resource for tips and recipes.

Saturday, July 14, 2012

To resect or not to resect?????

I have no reason why I made that appointment with Dr. Senders, my current doctor in April.  I honestly think that God's plan for me was to receive this information, after a decade suffering with ISS, it was time for good news.  The information Dr. Senders delivered to me had me blubbering with tears of joy - THERE IS A PERMANENT SOLUTION!!!!   He mentioned Tracheal Resection so nonchalantly - does he not understand this is an answer to my prayers?  He almost said it as a side note. "Ahem, excuse me???   Sign me up!"  I said.

I'm not exactly sure why I decided not to consider Dr. Senders himself for the tracheal resection, as he has given me very competent care - probably because his specialty is cleft lip, and becuase of all the horror stories I have read about ISS patients getting into the wrong hands for this surgery.  A rare disease calls for the most skilled surgeon in this exact condition.  So my friend Dr. Matt Forrester hooked me up with an appointment at Stanford.

I was left confused after my consult at Stanford Hospital......the news was devastating, yet not unexpected.  I am so very appreciative of Dr. Shrager's very honest advice, but upset nonetheless.   I kept it together until we returned to the waiting room.

"My advice is to continue with the balloon dilations, that's what I would do if I were you."  Shrager said.  My list of prepared questions is now rendered useless, he does not want to operate on me.  Why does the Chief of Thoracic Surgery turn me down? He fears the laser surgeries I was subjected to may have left 'the field' in a condition that I would have less satisfactory results.  He also fears that the exact location of my subglottic scar might be too close to my vocal cords.  "I have doubts that I am the surgeon who can do a flawless job considering you have had almost a dozen laser treatments." were his exact words.

Dr. Shrager did give me a glimmer of hope, in fact he highly recommended Dr. Mathesin of Mass General Hospital if I were to pursue the resection.  Dr. Mathesin trained Dr. Shrager, and he has complete faith that when it comes to this surgery, Dr. Mathesin doesn't fear proximity to vocal cords, OR previous surgeries.

Now the tough part - making a decision to continue with dilations or go to Boston.  I think the decision would be easy if I didn't have two beautiful children.  My place here in this world matters immensely in their lives.  I cannot even begin think about not being around for them - it's too painful.  But then again when I think about the relief I would feel post resection - as log as I am among the 80% of patients who have great results.   I would feel so much peace to think I wouldn't have to return every year for a dilation.

Friday, July 6, 2012

What to do if you suspect you may have Idiopathic Subglottic Stenosis

You suffer from difficulty moving air in and out.  You hear a wheezing sound on inhalation, its starting to get noticeably worse.  You have probably already googled many things: breathing problems, shortness of breath, windpipe, trachea, respiratory issues, etc......... You probably are on an Athsma inhaler after your ENT incorrectly assumed Athsma.

The first thing you need to do is see your general family practitioner as you'll need to rule out other respritory problems with some routine tests. 
  • airway CT scan
  • chest x-ray
  • respiratory function studies 
  • a blood test
If your family practitioner is stumped, you don't have asthma, allergies, or cancer, then the next step is to see your ENT for a direct laryngoscopy (a fiber optic instrument that goes through your nose to look more closely at your throat) This is very uncomfortable, but they will numb your nasal cavity to lessen the discomfort.  If your ENT suspects there may be something down there, he'll want to do a bronchoscopy next, which you will need to be put out for, and will get a much clearer look.

THE NEXT STEP IS VERY IMPORTANT:  Don't let any doctor touch you unless he or she is a genius. If your doctor has a hard time diagnosing you, or shows ruffled feathers or bafflement, he/she is NOT going to be the right choice for treatment.  It's a rare diagnosis, hence tough to find the right surgeon.  Since being diagnosed with IPSS I have read many many horror stories about laser surgeries leaving you with more scar tissue than you started out with, and many patients who have ended up in the wrong surgeon's hands and now breathe through a tracheostomy.  You want the best surgeon, or the result can be devastating.  I know many doctors who would love to claim they are an 'expert' when they really are not.  Ask quantitative questions such as, "How many IPSS patients do you currently treat?", and "How many of these surgeries have you performed to date?"  Also go online to trusted resources only.  

I have learned that some people suffering from Tracheal stenosis are cured after 1 - 3 laser surgeries of balloon dilations.  So my recommendation is most definitely to see a qualified ENT or thoracic surgeon to see if you are among this lucky group of people who are cured with a less invasive procedure.  Now myself, after 11 laser treatments, I have been told I may not be a good candidate for tracheal resection because of the damage lasers can cause.  Balloon dilations, on the other hand are a good non-invasive, although temporary treatment that an ENT can offer if resection sounds too frightening, or while you weigh the pros and cons.

I am still weighing the pros and cons.  I don't feel confident that resection is the answer for me because I can go a year to 18 months in between dilations.  I believe major surgery should be reserved for emergencies.  I know a mis-step during resection could be devastation at best, and this is not something I'm feeling good about at this point in my life.  Now if I get to a point where I am narrowing down and needing dilation every three months, I would certainly go for a resection.

Thursday, July 5, 2012

Tracheal resection

When I saw Dr. Senders in April he gave me some new information that I had not yet heard.  He said there is ANOTHER OPTION.  An alternative to maintenance surgeries every year.  My whole world stopped for a moment. I even like the name of the procedure, it has a ring to it..................Tracheal resection, an answer to my prayers.  Tears came to my eyes, and then rage:  Do you know how much money I have spent on these surgeries?  Well I don't either, it's not something I care to dote on, but why in the world hadn't he shared this very pertinent piece of information with me before??? Is this procedure new?  Why had both my ENT specialists Dr. Dedo and Dr. Senders opted to do a series of surgeries that wouldn't leave me with permanent results?  The answer lies in the anatomy of the scar itself:

  • Short areas of scar tissue may not require many procedures to fix them, or if they do, may only require 1-2 procedures.
  • Long areas of scar tissue tend to recur after procedures such as dilation and laser treatment, often around 6-9 months following surgery
I suppose surgeons who do laser and dilation surgeries are hopeful that you may be in the first category, only requiring a couple procedures.   I was baffled as to the reason they continue to do maintenance surgeries and withhold information about tracheal resection.  I think ENT's should do a series of three balloon dilations and if it doesn't hold up, refer to a surgeon for tracheal resection.  The progressive nature of ISS is that it will keep returning time after time.  So why the maintenance surgeries? 

I almost said, "Sign me up."  But then I knew I needed to call my mom.  She has been my biggest advocate through this whole ordeal - she would have an opinion here.  She did a ton of online research, and decided that this tracheal resection was a grand idea.  Seeking advice, I called Dr. Matthew Forrester, resident at Stanford.  He spoke with the head of Thoracic surgery at Stanford University..........AND I HAVE A CONSULT WITH THE MAN!!  I'm nervous but exited too.

Here's the anatomy of the tracheal resection:  A very, very, very, practiced Thoracic surgeon will actually remove the stenosed portion of your trachea, and sew it back together.  Tracheal resection is so far the best alternative to cure the stenosis completely. The narrowed part of the trachea will be cut off and the cut ends of the trachea sewn together with sutures.   Brace yourself, they go through your throat, and you will have what they call a collar scar. Totally a non-issue as far as I'm concerned, as long as it assures me I won't have to have another surgery. This will not be performed by your ENT, and it's a much bigger deal than laser or dilation is a final surgery with lasting results!

I can't wait to share more after my consult with Dr. Shrager.......until then!!

UPDATE: October 2012

Well it seems this dream cure might not be in the cards for me.  I now understand what a big deal this tracheal resection surgery is...... you have a four inch incision, a drain, and chin stitches to hold your head still for a week, and that is really really scary to me!  At this time I have decided not to go through with it, I am way too scared that I may not be one of the lucky people who receive flawless results and have a permanent solution.

What is Idiopathic Progressive Subglottic Stenosis?

 I am pleased to announce tracheal (subglottic) stenosis now has a facebook page!!!   No really, it does!  It's a closed group, so your work friends and family won't see what you post.  Go to facebook and search "Living with Idiopathic Subglottic Stenosis".  I'm sure you will feel so much relief that you now have online support and a forum for chat about this uncommon disorder.

You will see a couple different names for it:

Idiopathic Laryngotracheal Stenosis (ILTS)  
Idiopathic Subglottic Stenosis
Fibrosing Subglottic Stenosis

I call it Idiopathic Progressive Subglottic Stenosis because that's what my first diagnosis from Dr. Dedo at UCSF was called. IPSS

It's very rare, therefore hard to detect, hard to diagnose and especially difficult to treat.  Most, if not all people who suffer from IPSS will have been wrongly diagnosed as athsmatic.

Symptoms can include, but are not limited to, dyspnea (shortness of breath), stridor (wheezing) and coughing.  People report needing treatment anywhere from every 6 weeks to every 2 years, and are almost exclusively female. 

For me, yearly treatment is scary and expensive.  Scary because one day my children will have to take care of me and keep up on maintaining my airway.  The alternative is suffocation or tracheotomy. SCARY AND EXPENSIVE, VERY.  I am blessed to have family who helps out with insurance costs.  With this diagnosis, you will need guaranteed issue insurance, or you will go broke.  Not to mention travel costs to see a well-practiced specialist.

Two times when I went longer than a year between surgeries, (once with the pregnancy of my son) I got letters from Dr. Dedo's staff.  "If you don't come in for a yearly procedure you will end up with a tracheotomy." I really didn't care for this pressure, it's my body, right?  I should be the one who says when it's time.  I had a total of 11 laser lanyngoscopy surgeries, received two of those letters, and received countless lectures from Dr. Dedo as scare tactics warning me against seeking other procedures.  I felt compelled to seek a new surgeon. 

I found Dr. Senders through online research.  I immediately liked his no-pressure approach.  I liked the idea of less-invasive balloon dilation as opposed to actually being cut with the laser laryngoscopy Dr. Dedo swears by.  To my question of how often, he nonchalantly answers, " It's your body, and you can let me know how often you would like to see me."  I cannot tell you how much I loved that answer.

Laser laryngoscopy vs. balloon dialation

Think of them both as maintenance surgeries.  Neither will cure you.  Not after a series of three, not ever.  This diagnosis is PROGRESSIVE people!  If any doctor tells you that it might go away, call baloney on them!  Both of these surgeries work:  they keep the airway open, they are fairly non-invasive, and it is in-and-out surgery.  THEY ARE NOT A CURE.  (Unless you are very very lucky)

Laser Laryngoscopy  I can no longer count on both hands how many times I have had this done, I did 11 of these with Dr. Dedo at UCSF, under general anesthesia.  Dr. Dedo may request you stay the night if you are older ( I was only 22 when I first saw him).  Many doctors completely obliterate the scar with the laser, but Dr. Dedo has perfected a flap procedure, in which he flaps healthy tissue over the laser incision, so new scar doesn't form.Your throat is sore, but not in enough pain to require pain medication.  You are told not to cough, that you might 'ruin' the surgeon's handiwork, when the urge is so strong to do exactly that.  When you finally oblige that urge you will cough up a bit of mucousy blood.  Dr. Dedo put me on 5 days voice rest.  I did refrain from talking for five days the first time I had the surgery (I used a whiteboard to communicate with my husband), and I refrained from speaking for about five hours the other ten times (I just like to talk, OK?) And it didn't actually hurt to talk, so why not?  Your surgeon might spray some Mitomicen - C on the area in hopes to inhibit regrowth of the scar.  Or he might send you home with a steroid inhaler.  Dr. Dedo only did half of the scar's circumference each time he saw me, because he worried about it collapsing altogether.  The relief you feel post surgery is about a 7 1/2 out of ten.  In millimeters you will be given a whopping 2 -3 millimeters more space to move air through your trachea.  Which is a lot if your airway is narrowed down quite a bit by the scar tissue.

Balloon Dilation  I had a total of 4 of these procedures with Dr. Senders at UC Davis.  Dr. Senders did not understand the need Dr. Dedo felt for voice rest, as it's got nothing to do with vocal cords.  But then again, it's a different surgeon, different procedure. The most painful part of balloon dilation of the trachea is the IV, seriously, the site of the dilation did not hurt at all. If it wasn't for the nausea I felt from general anesthesia, I could have gone to the gym afterwards.  The relief you feel post surgery is once again about a 7 1/2 out of ten as far as breathing goes.  In millimeters you will be given a whopping 2 -3 millimeters more space to move air through your trachea.  Which is a lot of you are narrowed down quite a bit. Because Dr. Senders left the scheduling of my treatments completely up to me, I got myself into a bit of a pickle almost every time.  I wait till it gets bad.  During the winter if I was congested, I would wake up in a panic because of shortness of breath, from a DEAD SLEEP!  You see, the anatomy of subglottic stenosis is a ring of fibrous scar tissue.  Laying prone for hours during sleep, any mucous in your sinuses will gather, rest there, and harden in your narrow airway.  It's gross, I know.  At the worst I would have to get up and steam in the shower for half an hour, just to get my breathing and heart rate under control.  Mucinex helps tremendously and I don't go to sleep without it if I have a cold.

Diagnosed at Last!

In 2002 customers started asking me if I had asthma.    "Do you have a puffer or something?" they would ask uncomfortably. For about a year, I too had been noticing it, a wheezing sound during exertion.......mostly with playing tennis, jogging, and climbing stairs. By 2003, however, I noticed the wheezing sound even at rest, accompanied by a constant urge to cough and clear my throat.

I started with my general doctor who incorrectly presumed asthma, and sent me home with a Pulmacort puffer. This was followed by an allergist, who tested me for everything under the sun.  She ran the computer simulated candle test on me.  "You're not doing it right."  she said, as I blew on the mouthpiece till blue in the face. "Try again.  I need you to fill your lungs fully and exhale completely until you blow all the candles out." She was baffled, as she couldn't even get a result on the test; I absolutely could not exhale fast enough to blow the last two to three candles out. "It's as if you're an eighty year old smoker." she concluded.

Next I saw an ENT doc.  He did a couple tests, saw nothing, and prescribed me Paxil and Prevacid, assuming it was panic attacks or acid reflux.  Crying uncontrollably on the way home, I resolved to just forget about it.  Maybe this was just stress?  Maybe it's just not bad enough to keep trying different doctors.  So I wheeze a bit, no biggie, I am still capable of running a normal life, right?

My resolve lasted about six more months. Constantly out of breath, panting al the time, I vowed to start seeking a diagnosis again.  Dr. Heithacher, a second ENT doctor was the first doctor who thought scoping down my throat was a bright idea.  Heithecher did a bronchoscope and CT scans and he said, "Heather, I don't know what you have, but you have something, and I'd like to help you find a diagnosis."  I felt so much relief knowing I now had an advocate.   After seeing the scans, he still didn't know what it was, but he did know who I should see next.

I was extremely nervous on my three hour drive to my referral in San Francisco.  After arriving at UCSF, Dr. Dedo's staff ushered me into a very small room with many many plaques and recognition in the ENT world, clearly this guy was 'the man'.   "It feels like you're breathing through a straw, right?". he says confidently. "Heather, I have an explanation for your shortness of breath, in fact I feel certain of it without even having examined you", he says.  He called it IPSS, an acronym for Ideopathic Progressive Subglottic Stenosis.  He loosely attributed the cause as female hormones, and asked me to stop using birth control. He said I would need a series of three surgeries to make it go away.

For a second opinion, I flew out to Rochester and saw a Mayo Clinic physician.  "You're seeing Dr. Dedo?" he asked.  "He invented the Dedoscope, which we use here at Mayo to keep the airway open while operating.  My recommendation would be to go back to UCSF and do the series with Dedo."   So that's what I did.

Dr. Dedo assured me that I would need a series of three laser laryngoscopy procedures, spaced out by three months each. He said my now 6mm trachea would be back to a normal 11mm.  Little did I know, I would continue to be treated by Dr. Dedo for 9 years.

After my third laser surgery, my airway narrowed once again 10 months later, as the scar tissue closed in.  Feelings of devastation crept up as the realization hit me:  My subglottic stenosis requires surgery yearly..........I would need treatment every year............FOR THE REST OF MY LIFE!!!!!!!!!!!!!!!!!!