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Friday, June 27, 2008

Help out a girl with a rare disease called chILD

We have a greater mission today, and that is to help out the cute little girl in the picture to the left.

Bob Sherman, my fellow RT blogger over at Respiratory Report, has written a couple posts about Julia Roberson, who has been stricken with a rare disease called Children's Interstitial Lung Disease (chILD).

She is involved in a fundraiser in an effort to raise money. Click here to learn more.

I've taken care of patients with interstitial lung diseases (ILD) before, but all of them were adult with idiopathic pulmonary fibrosis. This would be a disease that causes scarring in the distal air passages that causes the lungs to stiffen and decreases the lung's ability to exchange gases. Interstitial pneumonia is also considered a type of ILD.

One of my favorite all time patients was a 40-year-old lady with pulmonary fibrosis. Her and I used to have some really cool discussions, but only while she had her BiPAP on. She told me she hated that BiPAP, but that's what was needed to maintain an SpO2 level even in the mid 80s. That and lots of supplemental oxygen. And even with it on she remained dyspneic.

She told me one night, shortly before she died, that she so much wished that she would get better so she could help other people who had a disease that made it difficult to breath. She was thinking of other people even while she knew she was dying, and for that I respected and admired her greatly.

But there was nothing she could do to stop the disease process, as so little was known about it. Likewise, very little is known about chILD.

So what exactly is chILD? The cause is idiopathic, or of unknown origin. But there are some theories.

child Foundation reports that adult ILD is different than child ILD. It involves a series of diseases "that vary in their severity and in their long term outcomes. In simplest terms, all types of chILD decrease a child’s ability to supply oxygen to their body. If you suspect your child has chILD a pediatric pulmonologist with expertise in chILD should be consulted."

According to emedicine, and per the most recent research, the process is believed to be the result of "tissue injury with aberrant wound healing resulting in collagenous fibrosis." This ultimately leads to "structural remodeling (scarring) of the distal airspaces, leading to impaired gas exchange."

While the exact cause is questioned, it is theorized that the disease process is the result of "some type of lung injury to the distal air spaces caused by the adenovirus (RSV), or exposure to organic dust. This results in damage to the epithelial or endothelial layers and the associated basement membrane."

It is very rare for such a disease to inflict a child, but when it does the mean life expectancy has been just 47 months.

According to the chILD Foundation, after ruling out other diseases, a child would be considered to have this disease if he or she has three of the following:
  • Fast breathing
  • Use of accessory muscles
  • Abnormal chest x-ray
  • Need for supplemental oxygen
  • Failure to gain weight
  • Persistent crackles, wheezing or other abnormal sounds heard during auscultation
  • Recurrent pneumonia
  • Recurrent bronchiolitis
  • Recurrent cough

A more definitive diagnosis would come from a lung biopsy.

So, very little is known about this disease, and there is no cure. Which is why more money is needed to research it.

The respiratory report writes that "Julia is going to participate in the Respiratory Health Association of Metropolitan Chicago’s 2008 Hike for Lung Health. She has set a fundraising goal and I want to help her reach that goal. If each person who reads this article would donate $10.00 to Julia’s effort along with $10.00 to chILD Foundation, you would be doing so much more than that for her and her family’s spirit. Continue reading →"

So, we here at the RT Cave urge you to open up your hearts and your wallets, and click on this link for more information about Julia Roberson and her rare illness. Or, click on one of the links above to help her out.


Amy said...

Nice post--I'm sure the family's grateful for all this coverage. I'm going to go check it out right now.

Bob Sherman said...

We got Julia over her goal. Thanks so much for your help. About $1200 additional dollars will post later this week.
Don't stop giving though!
Thanks again Rick,
Bob Sherman

Mary said...


This is the assistant editor for which is a medical publication offering hospital news, information and reviews. We also cover a wide variety of medical issues, one of which being Children's Health and the importance of choosing the right hospital. You will notice articles on this topic under Children's tab. If possible I would like to be included within your blog roll, offering our information as a resource to your readers. Please let me know if this addition can be made.

Please email me back with your URL in subject line to take a step ahead and to avoid spam.

Thank you
Mary Miller,