Shelbie, Spencer and Samuel's updates...

The first thing Dr. S said when she walked in our room was, "Boy, you guys aren't really the picture of health you were last year."

I was quite surprised but it's true.  This past year has not been kind to us. The kids have struggled more than usual and infections have been rampant.

Shelbie is still stuck with an immune system that isn't working.  That means more IVIG.  As I mentioned in my last post, we tried a new kind of plasma called SCIG.  It is administered just under the skin as opposed to an IV.  It still takes some time to administer and requires a pump but it is done at home once a week.  She isn't keen on the idea but we will see how things progress.

She has had increased pain, respiratory problems, joint issues and the adenovirus rages on.  While examining Shelbie, Dr. S noted that she is lacking saliva and tears and wonders about more issues that could lead us to another diagnosis of Dyskeratosis Congenita, a disease even more rare than SDS or Sjogren's which is an autoimmune disease.

I found it interesting that the kids have the hallmark features of so many different diseases. SDS, Mito and Dyskeratotis Congenita.  Even I have some of the DC symptoms.

Basically, Shelbie has gone down hill since we saw our doctor last and it will be interesting to see how her marrow looks. When we get home, she will need to see a Rheumatologist to figure out the joint pain and swelling.

     I really thought Spencer's issues were all GI and bone marrow failure, so I was really surprised when orders were written to see a Cardiologist.  He has clubbing in his fingers which is getting worse.  It's a sign of oxygen not moving correctly through his blood.  His extremities swell and change colors so that is an issue too.  It's been happening for a year at least and getting worse but no one back home has had anything to say about it.

He too has been complaining of joint pain so it's of to the Rheumatologist for him too.  The GI problems continue to be something that no one can seem to remedy.  Spencer, and Shelbie too had to give up 20 tubes of blood for testing.

     Of course, Sam's big stuff is infection.  Cellulitis, C-diff and Colitis. The hospital freaked out when they found out Sam was still being treated for C-diff.  Everyone had to gown up, mask up and glove up.  We were quarantined to one room which actually ended up being a good thing and he was not allowed to walk around the halls of the hospital. He was not happy about feeling like a haz mat problem but, that's the way it goes.

      Sam won't need any special follow up with other specialties but he is getting a decent work up for immune problems since it has taken so long for him to get rid of these infections.  He will have to get to the Podiatrist we have been putting off to see about taking off that toenail.

      I asked the doctor about his knee problems and abnormal ACL.  She believes that you treat the symptoms.  If his ACL is abnormal, we should look into reconstruction so that it is in proportion to his muscles and other ligaments. Of course, we would need to consider the pros and cons of surgery.  For now, he is managing okay so that will be a tough one to decide.

The next few weeks will be busy and tiring trying to get Spencer to all these doctors and get a clean bill of health before he leaves.  The timing of this to me, feels horrible but I have to continue in faith, knowing that everything is going according to God's plan.



  1. Praying for you, Kathy. Congratulation on the mission call!


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