Thursday, May 22, 2014

The GI appointment

Before I begin my post with our news, may I ask you to pray for our friend Parker and his family?  Parker is having a very, very tough time right now.  He needs as many prayers, positive thoughts, or anything else you can throw his way, as he can get.  His family needs some peace and strength, too.  You can follow his story here.

Okay, so when Emily had her swallow study a couple of weeks ago, I knew it wasn't right.  For a minute there I thought they were playing with the camera.  I realized that more comes up than goes down when she drinks.  I actually cried watching the camera, thankful that she couldn't see me.  She was standing up, swallowing hard, and still nothing would clear until she had some water.  Not what I wanted to see.  

Because our rheumy group is amazing, they set up an "emergency" appointment with a new GI doc.  The nurse practitioner emailed me about that Wednesday, and by Thursday we had an appointment set for the following Monday.  I worried a bit about how the doctor would be, but when I pulled up his profile I saw that he was the head of the department for feeding disorders, and he writes the curriculum for the school that his office is affiliated with.  

The first thing that the doctor asked me after he introduced himself was, "how can I help you?"  For just a minute there, I was worried.  I explained that I just want her to be able to eat.  He said that he had already looked through her file.  He asked me if anyone had mentioned a problem artery to me.  Ummmm, no.  Apparently, she has what they call an "aberrant subclavian artery".  This means that there is an extra artery that snakes around behind the esophagus.  (Below is the exact picture the doctor showed us.)  This by itself can cause all sorts of really awesome problems like chronic respiratory infections and problems swallowing.  The NIH Rare Diseases page  says this about it: 

 Aberrant subclavian artery is a rare vascular anomaly that is present from birth. It usually causes no symptoms and is often discovered as an incidental finding (such as through a barium swallow or echocardiogram). Occasionally the anomaly causes swallowing difficulty (dysphagia lusoria).[1][2] Swallowing symptoms in children may present as feeding difficulty and/or recurrent respiratory tract infection.[2] When aberrant subclavian artery causes no symptoms, treatment is not needed. If the anomaly is causing significant symptoms, treatment may involve surgery.[1][2] Children with symptomatic aberrant subclavian artery should be carefully evaluated for additional vascular and heart anomalies.[2]

So, because that's never quite enough with us, he also points out that just because she has that doesn't mean it is the culprit.  It is likely from the scleroderma side of the Mixed Connective Tissue Disease, but it could also be gastroparesis, which is where the stomach doesn't empty properly.  For more info on this, I went to my trusty friends at NIH again.  

How can we distinguish which problem may be causing this?  More tests!  This was one of the two things that we somewhat expected.  Em & I had talked about how she would probably need another upper endoscopy so they could really figure it all out.  (We assumed it was from the esophogeal separation, and would need to be stretched with a balloon.) Not only does he want that done, (minus the balloon) but he also wants a different type of swallow study done.  Instead of drinking barium, they have you eat something like eggs with radiation.  (UGH!!!)  If we didn't really need her to eat I would be against this, but we have to figure out what needs to be done.  Hopefully she will have these tests with her next hospital overnight.  We go back on June 2nd & 3rd.  On June 4th we have her endocrinology follow-up; it will be the first one since she started the hormones.  

She is still having some wicked headaches, but we think that they may be from the sinus issues.  Tomorrow she will be heading to our ENT's office for a sinus culture and suction under anesthesia.  We are hoping he may have some answers for this.  I feel like she may need a prophylactic antibiotic.  She is sick more than she is well.  Perhaps that won't be possible with the artery issue.  Maybe she is just going to have to deal with being sick constantly forever because of it.  I don't know.  I discovered that if she has gastroparesis, sometimes low doses of erythromycin help.  I hope she does not have gastroparesis, but if she does maybe we can kill two birds with one stone there.  Hopefully by having her appointment now, her IgG levels will be high enough to not knock her back, and there will be enough time between this procedure and her endo appointment to really be able to tell if the headaches are sinus or hormone.  We are also hoping that her levels are staying high enough consistently for them to stop her IViG (platelets via infusion at the hospital).  That would eliminate the overnight hospital visits, and really make our lives much easier.  We shall see, I guess.  We are staying hopeful that we are on the right path, just with our usual bumps in the road.

Zach is doing well.  :)  For that we are very fortunate.  I will update more when we know more, or to point you towards Parker's page for his next update.  Please pray for all of their family and ours?  Thank you.  

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