we got out to a pretty yucky start yesterday. we left for the airport at 5am and made it to chicago around 9am. when we arrived, we found out that our 11am flight to cleveland was cancelled. we then got on standby for the 1pm flight...they could get me on but not justin. so....we had to wait for the next available flight which was at 6pm. so...we basically got to enjoy the inside of the chicago airport for 9 hours. we made the best of it...ate a bunch of junk food and laughed at all the strange people passing by. we were so so tired when we finally got to the hotel at 10pm cleveland time. we were disappointed that justin was not able to get his blood patch yesterday, but he is now rescheduled for that procedure tomorrow morning at 8:45am.
so on to the serious stuff. justin saw the rheumatology team today at 12:45...we were there for 2 hours and this is what went down:
the muscle biopsy confirmed that he does indeed have overlap syndrome of polymyositis and scleroderma (systemic sclerosis).
it also found that he has degenerating and regenerating myopathy. what is that? basically his muscles die (because they are being attacked)...then when they try to regrow...they are attacked by his own body again and they reform all crazy like.
this also causes muscle inflammation...which is currently being somewhat controlled by the chemo.
there are other places in his body that are being harmed by this inflammation. one being his lungs...which is in turn causing dilation of the left ventricle of his heart. AND his kidneys which can lead to hypertension.
his lungs are in a whole other category of concern on their own. both the polymyositis AND the scleroderma are causing issues there including haziness and inflammation. he is not a candidate for transplant because the same issues would come back in a new lung.
his liver is still causing concern...possible wilsons disease (body does not break down copper...which is surprisingly found in many foods and water)...more tests on that later. they cannot deicide if he should be put back on the methotrexate or imuran. both are harmful to his liver...but at this point it has not yet been determined which will be more harmful...the side effects of the medications or the diagnosis. more can be determined on this when they can determine whether muscle enzymes are leaking into his liver OR he has liver disease.
there is also new concern with his esophagus...he will have to get a bronchial test or endoscopy when we get back home. when he breaths and swallows...his esophagus does not close properly. this is called patulous.
they will begin weaning him off of the steroids over the next year...but he will take atleast a small dose for the rest of his life.
they need to find an immune suppressant that will control all the inflammation in his body. unfortunately there is NO medication that is FDA approved for this. so...he will just start trying things. the first thing they want to try is called prograf. this medication was created for transplant patients to prevent rejection from their bodies to the new organs. since justins body is rejecting its own organs...they think this could be helpful for him.
SOOOOO...this may just seem like a lot more UNanswered questions...and it is...but its actually a ton of information and that is really what we came here to get. we still need clarification on so many things...but none of it is going to be an overnight process. he had another ct scan on his lungs today and more blood work. he will get the blood patch done tomorrow morning and we are praying for immediate relief from the painful spinal headaches. he did wake up again with a fever this morning also. we are praying that will subside when the headaches do.
As I am typing this now...we received a call from Dr. Parambil the pulmonologist who just looked at justins ct scan. His words were "I am so very sorry Justin...I saw your scan and am very worried" He wants justin to come in for a bronchoscopy and lung biopsy immediately. We are on the phone now scheduling that and it looks like it will be around 2pm. He said that scar tissue is rapidly building in justin lungs.
We just want to go home.
