Understanding Rare Wolman Disease in Newborns
Rare Disease is Fatal in Newborn Babies
Wolman Disorder is an inherited fat storage disorder that involves the breakdown and use of fats and cholesterol in the body. Large amounts of lipids accumulate in the liver, spleen, bone marrow, small intestine, lymph nodes, and adrenal glands. Infants born with Wolman's may appear healthy at birth, but symptoms of Wolman Disease begin to develop soon thereafter.
Symptoms of Wolman Disorder include:
1. Enlarged spleen
2. Poor weight gain
3. Low muscle tone
4. Jaundice (yellowing of the skin and whites of eyes)
5. Diarrhea
6. Vomiting
7. Delayed development
8. Anemia (low iron in the blood)
9. Inability to absorb nutrients from food, resulting in malnourishment and excessive amount of fat in the stool.
How is Wolman Disease Diagnosed?
In addition to the above symptoms and the presence of calcified adrenal glands, a blood and skin sample is tested for levels of lysosomal acid lipase that is present. Those infants born with Wolman Disease have levels that are ten percent lower than normal levels.
A CT Scan or MRI are used to check for enlargement of the spleen and liver, calcification of adrenal glands, and enlarged lymph nodes.
Is there a cure for Wolman Disease?
There is no known cure for Wolman Disease; however, bone marrow and cord blood transplantation are being tested as a possible treatment. Additionally, doctors are able to manage the symptoms of this disease and, depending on how the adrenal glands are functioning, may give medications to replace the hormones the glands make or give intravenous nutrition. Surgical removal of the spleen if it's enlarged and blood transfusions in instances of anemia are also other alternatives.
Unfortunately, since there are no known cures, newborns usually succumb by the time they are six-months old.
Little Dakota Dykes of Wills Point, Texas was born with Wolman Disease and lost his fight Friday at only 12-weeks old. This article is in dedication to Dakota so that perhaps we will educate ourselves about Wolman Disease and in hopes that physicians will find a cure so that others born with this condition may thrive into adulthood.
Sources:
Cedars Sinai
Hide & Seek Foundation
Published by Kenzy England - Featured Contributor in Arts & Entertainment
I'm a full-time freelance writer, A&E Featured Contributor, and recipient of the Yahoo! Contributor Network's 2010 Top 1000 award. I enjoy writing about my favorite celebrities, music, and television shows.... View profile
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- Wolman Disease is a rare disorder affecting approximately 1 out of every 350,000 newborns
- There is no known cure for Wolman Disease