Day 1 in Cincinatti for the clinical trial.

Well, today has been an extremely LONG, and very difficult day.  I spent my day doing all kinds of tests and getting all kinds of bad news.  I wish I could say something positive about the day, but there isn’t much positive to say about it.  So, HERE I GO.  BUT…first, I am going to remind everyone that I am laying out the facts here.  This is not the end of my world.  This is going to definitely throw a damper in life as I know it, and I will have to make some serious adjustments to life.  BUT I WILL DO IT.  No worries.  God will get me through my days, and I know that this will all be okay somehow.  Now, you can read the fact of the day…

I started my day off thinking to myself about how horribly I have felt in the past few months.  I expected very much to be put on SUPPLEMENTAL oxygen during extreme activities and exercising.  I was okay with that.  At my last doctor’s appointment in March, my oxygen saturations were about 94% when I was just sitting comfortably in a chair, and when I was active and walking around, they could get to about 88%.  An average person at rest would be anywhere from 97%-100%.  Obviously knowing all this time that I have a cystic lung disease, I knew that I was well below.  But, LAM is supposed to be a PROGRESSIVE lung disease.  Generally, very slowly…so I expected some change.  But not much.

Well, I am suffering from pretty serious hypoxia right now.  Hypoxia is when your body and organs are seriously deprived of oxygen for a long period of time.  When I am sitting in a chair comfortably without any exertion whatsoever, my oxygen saturations rested at 87% to 89%.  NOT GOOD at all!!  So, we can all imagine how much I have been damaging my body when I have been doing the steps in my house and all of the activities that I do with hardly any oxygen spreading through my body.  Then, realizing that I am in desperate need of oxygen even when just sitting down, they put me on 6 liters per minute of oxygen while just sitting.  Using the oxygen got me to 98% and was the lowest amount of lpm’s that could keep me going.  6liters per minute of oxygen is extremely high for just jumping in like I will be!!

Next, I did a 6 minute walk test.  This is when they have you walk slowly in a circle to see how much oxygen you need to keep over 90% oxygen saturations when exercising.  I could barely sustain 91% with 8 liters per minute.  Also…NOT GOOD AT ALL.  They had to put a mask on me because I needed more oxygen when exercising.

Now, in addition to the hypoxia, I was sent immediately for an echocardiogram.  An echo is basically an ultrasound of your heart.  My doctor is very concerned that because of the oxygen deprivation that my body has enduring, that I have developed pulmonary hypertension.  Below is a brief description:

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The right ventricle pumps blood returning from the body into the pulmonary arteries to the lungs to receive oxygen. The pressures in the lung arteries (pulmonary arteries) are normally significantly lower than the pressures in the systemic circulation. When pressure in the pulmonary circulation becomes abnormally elevated, it is referred to as pulmonary hypertension, pulmonary artery hypertension, or PAH.

Pulmonary hypertension generally results from constriction, or stiffening, of the pulmonary arteries that supply blood to the lungs. Consequently, it becomes more difficult for the heart to pump blood forward through the lungs. This stress on the heart leads to enlargement of the right heart and eventually fluid can build up in the liver and other tissues, such as the in the legs.

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Anyhow, all of my lab tests are showing elevated levels of lots of things in my body.  When your body is deprived of oxygen, the body makes high levels of red blood cells to compensate.  This causes a lot of problems in itself.  I will find out in the morning if I do have pulmonary hypertension.  If I do, I may not be eligible for the MILES Trial b/c I may need to be treated for my heart situation.  That is disappointing, but obviously a very serious situation that requires immediate attention.

So, here is what we are looking at:  Definitely FULL TIME OXYGEN for me every day.   And a high liter flow at that.  It is kind of sad knowing that for the rest of my conceivable future I will have an oxygen cannula in my nose.  And I will have to deal with oxygen tanks every day all the time.  But, the good thing is that I was able to function a lot better with that.   I walked for 6 minutes without being short of breath at all thanks to the oxygen.  Lately, I have not been able to walk across the room without wanting to pass out.  Now, I know why.  So, that will be a relief at least.  I need to be able to be a good mother to my children, and oxygen will help me with that.

Outside of that, I used a bronchial dialater (kind of like an inhaler) today that helped open my airways a little bit and gave me a little bit of relief.  So, I will be using those from now on every 4 hours.

And tomorrow I will find out if I have the pulmonary hypertension, and if not, if I qualify for the clinical trial.  I am going to pray that my heart is okay, and that I can qualify for this trial.  The doctors here are ABSOLUTELY SHOCKED at how I could possibly have gotten so much worse in such a short period of time.  They had no explanation for how this all could be.  The drug involved with this trial is not a cure, but should help stabilize the disease and maybe keep me where I am, and buy me some time.  I am so worried that I will have to be looking at lung transplant asap here and I don’t want to do that if I can help it.  Transplants are EXTREMELY RISKY, and a lot of people do not get them in time.  I was hoping to push that off at least 5 years, and now I don’t think I will have an option in the matter.

Anyhow, I am extremely exhausted, and need to go to sleep.  But, I will write tomorrow again after all of my appointments.  Thank you so much for all of the love and for all of the prayers.  I would not know what to do without them.

xoxoxoxoxo