The boys' video won $100 for SDA!

We found out today that the boys' video won $100 for Shwachman-Diamond America
Click in the post below to see the winning video!

Boys' Video for Goodsearch Contest

Helping Children Cope with Medical Procedures

I wrote two articles on Helping Children cope with Medical procedures-- the techniques have been a tremendous help in helping our boys.

Helping Children Cope with Medical Procedures: Using Social Stories

Using Humor and Fun to help Children Cope with Medical Procedures

I thought these might be helpful to some families.

Gluten Free Cookbooks

The Celiac topic comes up frequently on the SDS email list. We even have a few SDS kiddos who have celiac and SDS. We have family friends who have gluten intolerance and I have actally learned to make a few GF things for their visits. My friend says that any of Bette Hagman’s cookbooks are great. Especially the GF Gourmet Bakes Bread!

Since I have been asked about this so many times and keep having to go back to my friend to ask the name..... I decided that this time I would add it here --just in case I get asked again or anyone else may need the info.

Updates in the Management of MDS

An update from the 2007 ASH meeting

Updates in the Management of Myelodysplastic Syndromes: A Report from the 2007 American Society of Hematology (ASH) Meeting

Tips: 72 Hour Fecal Fat for Boys

Having two boys with Shwachman-Diamond Syndrome I have had to do many 72 hour fecal fat collections. When they are still in diapers, it is difficult to keep urine from contaminating the stool sample, but it can be done! First...... note that this post is going to talk about private parts---- it is part of life with SDS!

The best way to collect the stool samples for boys still in diapers--

Buy one pack of disposable diapers one size too big. Use disposable diapers that fit for the bottom layer.

1. Place the first diaper on the child inside out (plastic side toward skin).
2. Draw a circle around the area on the diaper where the penis is located.
3. Remove diaper and cut the circle out.
4. Place diaper back on child (inside out-plastic touching skin) making sure the penis fits through the hole. This will keep the urine from contaminating the stool samples collected in the diaper.
5. Place one of the larger diapers on the child in the proper fashion over the first diaper with the hole that has already been placed on the child.
6. Once the child has a bowel movement, remove diapers and scrape the stool into the proper container.

Most labs have you refrigerate or freeze the stool samples until it is brought in for analysis. Be sure to keep a food diary for several days prior to collection and the 3 days (72 hrs) of collection. This helps the doctors figure out the grams of fat ingested. Most labs say to eat a minimum of 50 grams of fat per day for a child and 100 grams for an adult.

Why keep a diary for the several days prior to collection? Remember what goes in one day comes out a day or two later!

Fecal Fat Testing - 72 hour

Very often, the fecal fat test is one of the first tests done when starting the diagnostic process for a child with failure to thrive. Children with Shwachman-Diamond Syndrome have excess fat in their stools due to pancreatic insufficiency.


Fecal Fat Testing from Medline

Stool Fat Test

AtoZ Stool fat Test

Serum Isoamylase

Serum Isoamylase is often used to test pancreatic function in SDS patients.

http://www.labcorp.com/datasets/labcorp/html/chapter/mono/sc024000.htm

Serum Trypsinogen

Here are links to information on Serum Trypsinogen. This test is often used to help determine pancreatic function in SDS kids.

http://www.nlm.nih.gov/medlineplus/ency/article/003560.htm

http://www.umm.edu/ency/article/003560.htm

http://www.labtestsonline.org/understanding/analytes/trypsinogen/test.html

http://www.healthcentral.com/ency/408/003560.html

ALP

The NIH has great information on ALP that can be found at the link below:

http://www.nlm.nih.gov/medlineplus/ency/article/003470.htm

Lab Tests On-line has good info, too:

http://www.labtestsonline.org/understanding/analytes/alp/glance.html

WebMD:

http://www.webmd.com/digestive-disorders/alkaline-phosphatase



For information on Shwachman-Diamond Syndrome check out Shwachman-Diamond America

ALP in SDS (Alkaline Phosphatase)

http://www.emedicine.com/ped/topic2060.htm

The emedicine article says this about Alkaline phosphatase in the SDS article:

Liver function tests

• Transaminases (ie, alanine aminotransferase, aspartate aminotransferase) may be elevated in individuals with SDS.
• Alkaline phosphatase may be within the reference range or slightly increased.
• Findings on coagulation studies are normal, and the serum bilirubin level is within the reference range.
• Hypoalbuminemia may be present secondary to malabsorption. 

For information on Shwachman-Diamond Syndrome check out Shwachman-Diamond America

Do Antihistamines Relieve Drug-Induced (G-CSF) Bone Pain?

Medscape Article: Do Antihistamines Relieve Drug-Induced Bone Pain?

Zyrtec may help bone pain

Bone pain after granulocyte–colony-stimulating factors continues to be a problem and appears to be more pronounced with higher doses used in stem cell transplant settings. Beyond medicating for pain, some practices have found great success with cetirizine hydrochloride (Zyrtec® , Pfizer, Inc., New York, NY) 20 mg orally per day in relieving pain. The effect is believed to be achieved through the anti-inflammatory response. If shoulder pain occurs, a rare side effect of splenic rupture should be ruled out. This appears at the web site:

http://onsopcontent.ons.org/Publications/SIGNewsletters/chemo/chemo18.1.html

Excellent Pancreatic Enzyme Resource

Pacreatic Enzymes
This site goes into the types of enzymes, what they do, the various prescription enzymes, OTC enzymes adn much more. Great resource!

The Center for Courageous Kids

The Center for Corageous Kids

Their mission is to uplift children who have life-threatening illnesses by creating experiences year round that are memorable, exciting, fun, build self-esteem, are physically safe, and medically sound.

A world class medical camping facility in the rolling hills of Scottsville, Kentucky providing a cost-free, safe, and fun camping experience for seriously ill and disabled children and their families.

Exocrine Pancreatic Insufficiency Support List

There is a new support list for those affected by Exocrine Pancreatic Insufficiency.

Exocrine Pancreatic Insufficiency

Prevalence of Pancreatic Insufficiency in Inflammatory Bowel

I have this one in full-text.

Prevalence of Pancreatic Insufficiency in Inflammatory Bowel
Diseases. Assessment by Fecal Elastase-1


Abstract Pancreatic insufficiency (PI) may be an extraintestinal
manifestation of inflammatory bowel diseases
(IBD). We report the results of a cross-sectional study that
was carried out to investigate both the prevalence of PI in
IBD patients and its clinical course over a 6-month followup
period. In total, 100 Crohn’s disease (CD) patients, 100
ulcerative colitis (UC) patients, and 100 controls were
screened for PI by the fecal elastase-1 (FE-1) test. The
decision limits employed were: £ 200 lg/g stool for PI
and £ 100 lg/g for severe PI. Patients with abnormal FE-1
values were re-tested after 6 months. Odds ratios (OR) for
PI were estimated by unconditional logistic regression
analysis. PI was found in 22 UC and 14 CD patients. The
OR for the FE-1 test £ 200 lg/g was 10.5 [95% confidence
interval (CI): 2.5–44.8] for IBD patients compared to
the controls. The risk of PI was related to three or more
bowel movements per day (OR = 25.0), the passage of
loose stools (OR = 7.7), and previous surgery (OR = 3.7).
At the 6-month follow-up, FE-1 values became normal in
24 patients and showed persistently low concentrations in
12. These patients had a larger number of bowel movements
per day (OR = 5.4), previous surgery (OR = 5.7),
and a longer duration of the disease (OR = 4.2). PI is
frequently found in IBD patients, particularly in those with
loose stools, a larger number of bowel movements/day and
previous surgery. PI is reversible in most patients, and
persistent