Wednesday, April 14, 2010

Update--Little Bean's JXG

You may remember several months back I shared about Little Bean's JXG. If not, you can read more about it here. The gist of the story is that my little man has a rare, benign, skin disease called Juvenile Xanthogranuloma. Because it is SO rare, our regular doctor, and even the pediatric dermatologist who sees Little Bean regularly know very little about this disorder (we see a derm because the first manifestations of this disease are shown on the skin). As a result of her little knowledge of JXG, Little Bean's derm told us very little about this disease, and as a result of that, I was under the (false) impression that JXG in general is relatively harmless.

I'm so thankful for those parents who also have children with JXG who commented on my last post about this and directed me to some different resources online where I could educate myself and my husband about JXG. We've since learned that while the first manifestations of JXG are papules (orange lesions on the skin), which Bean has lots of, are pretty harmless, the more the disease grows, the more danger the patient is in. Little Bean has had it for 3 years, and his lesions are still growing, but not rapidly. Most of the parents I've met so far (online, I mean) have infants who by age 1 are already leaving JXG behind.

We learned that in some cases the JXG can move into the eyes and cause blindness. Since the majority of Little Bean's JXG is very close to his eyes, and even on the eyelids, we became very concerned about this aspect. We learned that many people with JXG also have high cholesterol, something our doctors never tested for or mentioned to us. We learned that in severe cases, surgery or even radiation and chemotherapy can be necessary because JXG can become systemic and result in the deterioration of vital organs like the liver, the brain and the spine/nervous system. Sometimes, if treatment doesn't work, children with JXG even face death. If you have a child with JXG, a great resource is the Histiocytosis Foundation of America. They even have a message board where you can meet others with children dealing with this disease and others like it.

I have to admit, I felt like a really lame parent after realizing how serious JXG could be, and having done virtually nothing for him other than bi-annual check ups after his diagnosis. In general, I suppose I trust medical professionals too much, and when they told me it wasn't serious, I believed them. Little did I know, they just had no clue themselves whether it was serious or not.

After reading how serious it could become, I became filled with fear as well. I literally broke down crying reading some of the stories parents of kids with JXG had to tell, and reading the information that is out there on this disease. It was then that I really realized that our life is not our own, our children are a gift, and it is impossible to keep them totally safe from harm. I really had to put my concerns in the hands of God at that point or I'd not have made it through.

We have since been back to the dermatologist who Bean has been seeing since we noticed the papules. I usually have my husband take him to the appointments, but this time I came, and I came armed with new knowledge and questions for her. I asked that he be seen by an eye specialist to ensure his eyes were clear--she referred me to the surgeon who actually did his biopsy 3 years ago, and as it turns out, his eyes (at this time) are clear of JXG. I asked that his cholesterol be tested. All his blood work came back normal. I asked her more about the possibility of his case becoming systemic, and therefore more serious. Her opinion was that his case was not severe enough to be a concern (basically she said that the more skin lesions, the more likely it would become systemic as well).

So for now, we just watch and pray. He is a healthy, happy, little boy in every way right now. I only hope and pray that things stay that way until the JXG disappears.

On a side note, those of you who read my first post on this may remember I was asking for advice on how to handle the constant (rude) questions of others about this. Little Bean asked me the other day, "Mom, will my xantho ever go away?" to which I replied that the doctor said it would probably go away within 4 to 10 years. He replied, "I don't EVER want it to go away--it makes me handsome!". So I guess we are okay in that department! :)


Annette W. said...

It really is tough when you have to teach your doctor about something. (For us it was some of the signs of apraxia).

As you journey through these next days and months, I pray that you will not live in fear and will continue to trust in the Lord.

Jenny said...

He is so adorable and handsome!

I think we all put a lot of trust in doctors, that's what they are there for. They are supposed to know what is wrong. I will keep you all in my thought that the JXG does go away or at least never gets worse!

N said...

Wow, I'm so glad that you were able to find more information on it! It's great that you could do the follow up tests to put your mind at ease on those things. Information is power. :) I love his quote at the end!

C said...

I just came across your blog and it is so very familiar to me. Our 3 year old son was diagnosed with JXG when he was 1. We too were told "not to worry" and watched as the lesions continued to multiply over the last 2 years. We heard about, and subsequently met with, Dr. Ken McClain at TX Children's Hospital. He is an expert in Histiocytosis Diseases, and specifically JXG. We sent him an email and he quickly responded...we then met him in person. If you have questions or concerns, I would suggest reaching out to him. I hope all is well with you and your family!

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