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.  

Picture credit:  Sonoworld.com

Endocrinology & stuff

This has been the quietest week, mostly because we scheduled it for recuperation. Emily can feel the stent in her ureter and it hurts, but I don't think it is horrible. I keep telling myself that it will be out in another week, & hopefully she will feel much better. She has been refusing pain meds, so that is a plus.  Prayers would be wonderful, specifically for the ureter to be opened enough to grab all of the stones out of her kidney this time, no reaction to anesthesia, & of course to guide the surgeon's hands. 

Biofeedback is seriously one of the best things that we have ever done for her. I think that every child with chronic pain needs to try it.  Not only is she learning techniques to help her to deflate the pain but she is also learning how to take control over it. I really wish we had done this when she was five and limping everywhere, when the anxieties were so high that she seemed to be obsessive-compulsive. This would have been so life-changing then. I will have to tell our pediatricians that. And like all of that isn't cool enough, she is giving us some common-sense nutritional advice that I would not have thought of. Instead of keeping our fruits in the produce keeper, I am now washing them & placing them in a bowl on a low shelf so that everyone can see them. As Ginny says, people are more likely to eat what is already prepared. So true!

Aquatic therapy is still a challenge for her, but last time she got through it with the help of Xanax.  We try hard not to use them, but when they are needed I am happy to give her a half of one. That is the only way she did well last week. She told us at biofeedback that she is afraid that the kids in the pool will laugh at her, & the old people will pinch her cheeks. We found a time with no kids & few older people. I know she will love it once she chills out a bit. 

The big news of the day is endocrinology.  You may recall that she was seen last month. The doctor, who was absolutely one of the sweetest people I have met, ordered a bunch of lab work to be done. We had it drawn when she went in for kidney procedure #1. She already received 6 of the 8 ordered tests. So far most of it looks great. However, she also asked for copies of her growth chart from our pediatrician. I don't think I ever realized but Emily has always been in the 2nd percentile for growth. Yes- second, and she really hasn't grown this year.  Granted, I am NOT the queen of height (4' 9.5" myself!) but I think even I was taller back when I was her age. I worry about several things: A) the theory that the body holds in more pain when the cells aren't regenerating as quickly, causing her to feel amplified pain, and B) I worry about her jaw. One of the things that I have always noticed about adults who had JA as kids, and especially those that were on prednisone for long periods is that their jaws never seem to finish growing. It's like the jaw only grows to a certain point even if the rest of the body isn't finished growing. I didn't want her to be the kid with the tiny jaw. Imagine the implications of that- not having enough room for your teeth to fit (already a reality here), the need for more medical procedures to correct it. As it stands she only has a few more good years of growing. Her bone age is delayed, but not by a tremendous amount. It may be possible to catch up now- if we hurry. 

The doctor (her name sounds like Tab-ew-wad-a) said that she feels that Emily would benefit from growth hormone. She asked me to talk it over with Emily. I took that as "give her a choice". So, we discussed it last night. Her shots will be every day but she is okay with that.  She wants to try it.   I think she is getting irritated by people always thinking she is so much younger.  When they know her age they express shock. That's probably not good. I really hope that this is the beginning of a new, happy road. We will be so thrilled if this works.  All in all, things are looking up!  Thank goodness because this has been one heck of a summer that we wouldn't care to repeat.  Would anyone like to share their stories of aquatic therapy, biofeedback or growth hormones?  Please let me know!