Ahhh so I took the yellow brick road (otherwise known as Loop 101) to the Mayo Clinic and then the real fun began.
I got to meet OZ - The knower of all things Scleroderma.
The Mayo Clinic Scottsdale is actually a Center of Excellence for Scleroderma. Lucky me that I lived here in the Ol' AZ. My house may only be worth 100 bucks, but I lucked out in the rare disease department.
First I must say that Dr. G (OZ) is amazing. He is a wonderful caring man who has not been jaded by years of practice and who truly seems to care about the plight of his patients. He always holds my hands during or after our consults. He is so cute. Anyway...I digress
So as you can see 2009 was not my best year. From January to September I playing musical X-ray, and then October was when my world crashed.
I was officially diagnosed with the Big S and I lost my job. That's one good Bingo!
So The Mayo....ahhhh. Well, they make one hell of a tuna sandwich.
October, the fun of invasive testing. Memories...
So I saw OZ who ordered a bunch of tests. Basically I spent most of the month of October traveling the yellow brick road to the Mayo. I had a test every Monday and a consult and/or test every Wednesday. Good Times.
One of my first tests was the PFT - Pulmonary Function Test. Which is blowing through a straw in a closed glass coffin. It actually wasn't bad and it was necessary to get a baseline reading of my lungs. Scleroderma can cause fibrosis of the lungs and/or pulmonary hypertension...or PAH which leads me to my next test - The Echocardiogram.
The Echo is done to make sure there is no valve damage or pulm hypertension. Scleroderma can affect your heart too. For some added cardiac fun, I had an EKG too. Those were both painless and fear free. Peace of cake! (yes peace was used correctly..for me anyway)
The next test was called an Esophageal manometry. Ohhh lets just say, we were not in Kansas anymore. Oh my goodness gracious - If I never have to do that again I will be one happy Sclerie!
So the EM. Well, first you get a qtip with lidocaine shoved up your nose to numb it. It has to be pushed up there gradually because of course until the lidocaine is there, you can feel it - and well, it hurts. So once the q-tip is almost lost forever up my nose and can be shoved no more, then the real fun begins. A wire with a special tip that can measure pressure is then guided up your nose and curved to go down the back of your throat. If your not gagging now...you will be. Once it hits the back of your throat near your uvula the gagging begins. Its a reflex, you can really help it.. and once you start to gag, you cant really stop. The more you can, the more prone you are to throw up - which is what I did. When you throw up with a wire down your throat, you kind of think your suffocating because you can feel it, so I ripped the wire out of my nose.. which meant the fun began again. Up through the nose, down the throat and lets the gags begin. After three times (third time is a charm) I learned that if you pant like your in labor, you cant gag as much and the wire went past the uvula and my throat into my stomach and was then taped in place. Again..Good Times!
Once it is in place you get the added pleasure of getting to drink water and swallow while the machine reads the pressure within your esophagus. Swallowing anything with a thick wire down your throat - Not Fun - but nonetheless, I did it and as I swallowed my last bit of water the Chariots of Fire theme went off in my head because I was about to hit the finish line of wire free freedom. Once I was done swallowing, said wire was then pulled out fast from my nose. That didn't feel too bad actually. I guess the only other person who would know what that felt like who has not had an EM would be that weird kid in school who could swallow a spaghetti noodle and pull it out his nose? My husband and my new friend were waiting for me when I came out. I must have looked like I was put through the ringer because they were kind of giggling. Lets just say I should have worn a bib and apparently a face mask because there was schmootz everywhere.
After the EM was the Gastric Emptying study. Well.. anyone who knows me - KNOWS I do not do eggs. Do NOT do eggs. So for this study you come fasting (aka hungry) and you are asked to eat the most unappetizing looking egg thing cooked with radioactive material and milk (I also don't do milk) So for me, this was like my worst meal nightmare. You have to gag down every last bit before you are aloud to leave and they do check....Yuk. You then just hang out and come back every 30mins to an hour for an Xray of your tummy to see how fast/slow food moves through you.
The PH Study. Ahhh another "Fun with wires" day. So for this study you get the qtip up the nose again, although this time I am a pro. No Sweat - Bring it on. Then a smaller smoother wire is guided up your nose and down your throat into your tummy. This time because the wire was smaller or because my gag reflex went on vacation after the last wire fiasco, I did not gag and the wire went down relatively easy (which by the way is such an odd thing to say) The wire is then taped to your cheek and neck and you are given a portable walk man looking thing that will record any reflux - GERD events you have for 24hrs. Basically you walk out looking like MR. Roboto... You have a wire going up your nose for the world to see. I owned it though... I rocked that look like nobodies business and went power walking. After 24hrs I yanked that bad boy out myself and dropped off the walk man at the Mayo.
Other studies... My Manic Mondays. Lets see - I also had a holter monitor, CT Chest, EGD (No fun...forgot about the EGD, although Versed was my friend and once they knocked me out I don't remember anything). I also had an esophageal dilation during my EGD (they stretched my esophagus because of difficulty swallowing - Scleroderma can cause narrowing of the esophagus and diff with swallowing) I might be forgetting a few, but I am sure you'll forgive me as this post is a novel already. you get the drift....every Monday in October was spent at the Mayo ALL day doing tests. Enough said I guess.
So after all of the above fun - The final results came in and the winner was Scleroderma!
My current stats are:
At this time I have the lung nodule. One doc says Sclero related, one does not. Regardless it is there and I have to have a CT every 6 months to watch.
I have some mild mild mild lung fibrosis, but my PFTs look good.
My Echo was good. I have NO evidence of PAH for that I am so grateful. The prognosis is not good with PAH, but I will have to have an Echo every 6 months. If my numbers change than I will have to get a Right Heart Catheterization
My Kidneys are great. I was given a BP machine to monitor my BP daily as us Scleros can get something called Sclero Renal Crisis - which is an immediate life threatening elevation of blood pressure without warning, so I just get a Urinalysis every month to check for protein in my urine and check my BP daily and I am golden.
My EM revealed I do have esophageal dysmotility - I failed 30% of my swallows, but I look at that like "Hell - that means 70% were good!"
That basically means that the Sclero has gotten to my esophagus and I just have to watch it. Some people do okay forever and some people lose function completely and need feeding tubes. I have faith that whatever is suppose to happen for me will be. Right now I can eat - so I do and I enjoy EVERY minute of it!
My gastric emptying study revealed I have some delayed gastric emptying. What that means is sometimes I can eat normal volumes and sometimes I eat small amounts and my tummy feels like it is the size of a walnut. Basically food does not move through me as efficiently as it is supposed to, so if it stays in my tummy - then there is no room for anymore food. This also causes bouts of constipation with bouts of diarrhea and gastroparesis. It is what it is. I feel lucky. I have a Sclero friend that lost all function of her GI tract and will have accidents and another that has GP (gastroparesis so bad she doesn't eat) God Bless you Shari and Janice!!
My PH study was fine.. No heavy duty reflux episodes but I have been prescribed a prophylactic PPI in case...for preservation more than symptoms.
My EKG and Holter were good. I was having bouts of palpitations. BAD ones. My EKG was like one big long PAC - PVC - but ironically, when that thyroid nodule was found, I was placed on Synthroid - Cytomel and the palpitations stopped. FATE maybe... I laugh about my Old McDonald Nodule song.. "With a nodule here Nodule there, here a nodule, there a nodule" but - in finding that nodule and getting on meds to shrink it, the palps were stopped. Again, I feel lucky!
Which brings me to "The thyroid nodule" - basically I am on the above meds with a surveillance ultrasound every 6 months to ensure that it does not grow. If it does, then I get surgery - But for now its just hanging out enjoying the view. The brain nodule is also just hanging out enjoying my brain matter... Depends on who you ask if the nodules are Sclero related? One says yes, one says No - but is open-minded to the fact that when you have a wacky immune system anything can happen, so I just know they are there and grateful they are benign at the moment.
Sclero is a rheumatological disease as well as a connective tissue disease so essentially ANYTHING can be affected. I guess the easiest way to think of it is like Lupus and rheumatoid arthritis but a tad different and a tad worse. I get tired like nobodies business at times, so much so it hurts. I know that may sound weird, but the fatigue is tangible in that it envelopes and encompasses my entire body into my soul and I am done. Out of spoons.
I also have joint pain, at times symmetrical like RA but it can also be asymmetrical too. Right now my Rt ankle, hips and rt shoulder hurt the most. But its not prejudice, any joint will do ... so each day is a new journey. When my hips are bothering me and I walk, my husband calls it my gangsta walk. Nice... Like it.
My hands hurt the worst. Sclero really affects the hands and will at times disable them. They can become curled in on themselves and become disfigured. Mine have not curled. They just hurt. It does feel like something is inside my hand pulling it in on itself, if that makes sense. Almost like they are trying to curl? I wonder if its that start of something, but right now I can still open and close them. Some days its harder than others. Today was a bad day. They hurt so bad I wanted to cry... but I didn't. I am woman, hear me roar..... just cant do everything with them sometimes. Like this post I have had to rest a few times and I know tomorrow I will feel it (But its worth it)
My best friend knows when I am having a bad day and will help me open things, or sign credit card slips or whatever. I love her for that. She doesn't make it obvious or cause a scene, or even ask... she will just discreetly take the card slip, sign and hand back to whomever without any issue. It makes my heart melt... I am so grateful for that too.
My hands will swell sometimes and sometimes a random finger or two (maybe a toe or two) will swell really badly and look like a sausage. I cant bend them when they get like that, but that just happens sometimes.
Wrists, tendons, any tendon will become inflamed so I get a chronic tendinitis if I do too much... basically - Anything than can become inflamed, will with an autoimmune disease, so ?
I do labs about every month or two months for now to check my kidneys, liver function, CBC (platelets and such), and my panel. Right now my ACA went down, one doc says it was because of immunosuppresion and one says its a fluke and its a moving target and means nothing, but I want to think its the meds!
Lastly, right now I am just taking my thyroid meds for the ol' nodule, steroids as needed if I get a bad flare up or having shortness of breath, B12 injections once a week (malabsorption), Vitamin D, and Methotrexate (low dose chemotherapy) injections once a week.
That's about it.....and you get stiff. Like I am now....from sitting here and typing my own personal War and Peace!
Which means I am going to sign off for now.
I will end by saying I did not have the best day today, but I still feel like one of the luckiest people in the world. I love my life, I love my kids, I love my husband and I love the beautiful friendships that I have made during this journey.
Goodbye for now and I hope this finds you all happy and well.
Peace and Love ~