Prevalence of Neutropenia in the U.S. Population: Age, Sex, Smoking Status, and Ethnic Differences

Prevalence of Neutropenia in the U.S. Population: Age, Sex, Smoking Status, and Ethnic Differences

Interesting article on Neutropenia! Includes several interesting charts.

Neutrophil Definition

Neutrophil Definition

Neutrophil
Neutrophils are Leukocytes (white blood cells) of the Polymorphonuclear Leukocyte subgroup.

Neutrophils form a primary defense against bacterial infection. Like all the cells of the immune system , neutrophils are produced in the bone marrow and circulate in the bloodstream. However, neutrophils move out of blood vessels into infected tissue in order to attack the foreign substance ( allergen , bacteria, etc.). Normally a serious bacterial infection causes the body to produce an increased number of neutrophils, resulting in a higher than normal WBC count . Neutrophils perform their function partially through phagocytosis, a process by which they "eat" other cells and foreign substances. The pus in a boil (abscess) is made up mostly of neutrophils.

Neutropenia: A Patient's Guide

Neutropenia: A Patient's Guide

This is an 8 page handout that is wonderful for Teachers,Schools, Patients and Parents!

Neutrophil Function Tests

Normal neutrophils--the body's primary defense against bacterial invasion--engulf and destroy bacteria and foreign particles by a process known as phagocytosis. In patients who have repeated bacterial infections, neutrophil function tests may reveal the inability of neutrophils to kill a target bacteria or to migrate to the bacterial site (chemotaxis).

Neutrophil killing ability can be evaluated by the nitroblue tetrazolium (NBT) test, which relies on neutrophil generation of bactericidal enzymes and toxins during killing. This action results in increased oxygen consumption and glucose metabolism, which reduces colorless NBT to blue formazan. The reduced dye is then extracted with pyridine and measured photometrically; the level of reduction indicates phagocytic activity.

Neutrophil killing activity can also be evaluated by noting the neutrophils' chemiluminescence--ability to emit light. After a neutrophil phagocytizes a microorganism, oxygen-containing substances form within the phagocytic vacuoles. As the cell is stimulated, it emits light in proportion to the amount of oxygen-containing substances that are formed, providing an indirect measurement of phagocytosis.

Chemotaxis can be assessed in vitro by placing bacteria in the lower half of a two-part chamber and phagocytic neutrophils in the upper half. After incubation, migrating cells are counted microscopically and compared to standard values."

Cellulitis

It seems like many SDS folks get cellulitis. We've dealt with it 9 or 10 times now and have had to be hospitalized for it. My boys have a chemotaxis defect as well as neutropenia. J developed cellulitis even with G-CSF and GM-CSF. Only one time did we have a fever with cellulitis...and with SDS, my boys did nothave a huge white blood cell count increase.

We have learned a lot over the years. We've had cellulitis on the toes, knees, surgery sites, injection sites and private areas. If possible, we circle the area with ink (pen) and watch to see if the redness is spreading. We've only had pus develop once and had to have it drained. People with neutropenia don't always have a fever response or develop pus because of lack of neutrophils. In my boys' case, they mounted a response, not a huge WBC, but bcause of neutrophil chemotaxis problems, there was no pus. Doctors tried to culture the wounds, but we've never had much to culture because of lack of pus!

These are a few good articles on cellulitis that also include pictures.

MedicineNet Cellulitis article

Mayo Clinic Cellulitis article

eMedicine Cellulitis article
Cellulitis on Healthline
U of M Cellulitis article

Expression of the SBDS protein in human pancreatic cancer and chronic pancreatitis

You can access the full-text article here:
http://www.shwachmandiamondamerica.org/SDSarticles.html

Expression of the Shwachman-Bodian-Diamond syndrome (SBDS) protein in human pancreatic cancer and chronic pancreatitis.Kayed H, Bekasi S, Keleg S, Welsch T, Esposito I, Shimamura A, Michalski CW, Friess H, Kleeff J.
Department of General Surgery, University of Heidelberg, Heidelberg, Germany.

BACKGROUND: The Shwachman-Bodian-Diamond syndrome (SBDS) protein is a member of a highly conserved family which influences RNA activation and is associated with pancreatic, skeletal and bone marrow deficiencies, as well as hematological malignancies. METHODS: In this study, the expression and localization of SBDS were investigated in normal human pancreatic tissues, chronic pancreatitis (CP) tissues, primary and metastatic pancreatic ductal adenocarcinoma (PDAC) tissues, as well as in cultured pancreatic cancer cell lines by immunohistochemistry, immunoblotting and immunocytochemistry. RESULTS: In the normal pancreas, SBDS was localized in the cytoplasm of islet cells and ductal cells. In CP tissues, SBDS was found in the cytoplasm of ductal cells, tubular complexes, stromal fibroblasts and in PanIN1-2 lesions. In PDAC tissues, SBDS exhibited cytoplasmic and occasionally nuclear localization in tubular complexes, PanIN1-3 lesions, cancer cells, and stromal fibroblasts. Different levels of SBDS protein were detected in cultured pancreatic cancer cell lines. CONCLUSION: SBDS is expressed in normal, CP, and PDAC tissues, as well as in pancreatic cancer cell lines. The different expression and localization patterns suggest a role of SBDS in the pathogenesis of, or response to, inflammatory and neoplastic pancreatic diseases.

Liver Enzyme Elevation from the American Liver Society

Liver Enzyme Elevation (Incidental) Referral Guideline


Diagnosis/Definition
Elevation of serum ALT or AST in a patient for > 6 months. Cases with ALT > 150, or suspected autoimmune disease, should be sent sooner than 6 months (see below).
Initial Diagnosis and Management
Usually an incidental finding in a chemistry panel obtained for reasons other than suspected liver disease.
Review medication list (prescription, over-the-counter, and supplements) and discontinue, if possible, medications that are known to cause abnormal liver enzymes.
Have patient abstain from alcohol (suspect alcohol when AST > ALT and GGT elevated).
If patient is overweight, encourage weight loss.
Ongoing Management and Objectives
Repeat liver enzymes in one month, and if still abnormal, following lab tests are recommended:
HBsAg, HCV Ab, ANA, ASMA, Iron & TIBC (fasting), Ceruloplasmin (if < age 40), Alpha-1-antrypsin level, SPEP, Protime, and U/S RUQ (include AMA if alkaline phosphatase elevated). For ALT <150, above studies can be obtained gradually over 6 months while observing the enzymes for possible spontaneous resolution. Check common things first (like viral studies and fasting iron panel). If ALT > 150, check above studies and referral should be within 2 months if enzymes are not resolving (or refer immediately if autoimmune hepatitis is suspected - young female, other autoimmune features, elevated gamma globulin, or + ANA or ASMA).
For minor elevations (<1.5x normal ALT) if work-up is negative and ultrasound suggests fatty liver, referral may not be necessary and a trial of weight loss is reasonable. Indications for Specialty Care Referral If any of the above listed diagnostic tests are abnormal. If AST or ALT is above normal for 6 months or more and above work-up is complete (see also "minor elevations" above). If AST or ALT is > 150 and not coming down for 2 or more months and above work-up is complete (or pending).
Signs or symptoms suggestive of underlying liver disease (RUQ pain, tenderness, encephalopathy, ascites).
If autoimmune hepatitis is suspected (see above).
Criteria for Return to Primary Care
Completion of GI evaluation with assessment of potential etiology, severity, prognosis, treatment plan (if any), and recommendations for any periodic surveillance or evaluation.
Certain disease categories treatment plans may require ongoing close follow-up with the gastroenterologist (ie., interferon for viral hepatitis).


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

Liver Enzyme Information

Dr. Palmer's Article on Liver Enzymes

Four separate liver enzymes are included on most routine laboratory tests. They are- aspartate aminotransferase (AST or SGOT) and alanine aminotransferase (ALT or SGPT), which are known together as transaminases; and alkaline phosphatase (AP) and gamma-glutamyl transferase (GGT), which are known together as cholestatic liver enzymes. Elevations of these enzymes can indicate the presence of liver disease.

The link above takes you to an article that tells you about each of the tests and their values.



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

Jesica's Hope Chest

Jesica's Hope Chest

Jesica's Hope Chest, Inc. (a foundation for critically ill children) is dedicated to helping critically ill children up to the age of eighteen (18) years old (but not past the child's eighteenth birthday). Jesica's Hope Chest, will assist families with critically ill children and help cover costs of necessities which are not covered by private medical insurance and/or government medical assistance programs, such as Medic-aid and Medic-care. We will help cover the cost of transportation to and from medical facilities, and help fund housing costs when medical treatment is at a hospital far from home. We will also help families purchase specialized equipment and formulas. Below is a list of other activities that Jesica's Hope Chest, Inc. (A Foundation for Critically Ill Children) will be providing but shall not be limited to;

*Assist child life specialist with activities and interact with children and families in the children's medical centers and pediatric clinics.

*Assist patients, families, and staff members with locating appropriate health related information (books, journals, internet information, etc.)

*Serve as liaison between patients and staff members in the identification of potential patient needs, problems, or complaints.

*Provide follow up services, such as helping families cover the cost of lifesaving medications for children up to the age of (18) eighteen.

*Help locate Universities, hospital and/or other child help organizations who may provide financial assistance for major surgical procedures, and other lifesaving medical needs.

Andre Sobel River of Life Foundation

Andre Sobel River of Life Foundation

The Andre Sobel River of Life Foundation enables single parents of children with life-threatening illnesses to care for their children full time by relieving financial burdens when other resources are exhausted or unavailable.

Pattie's Tips for Increasing Calories

When my boys were little (especially my middle son) we tried everything to get him to gain weight. In one year, even though he was eating large amounts of food, he only gained about 4 ounces!

These are some of hte things we did to increase calories in hopes he would gain weight. Once he started enzymes, he did a lot better!

• Melt butter on the stove or microwave and pour melted butter over pancakes, french toast or regular toast and other foods
• Use cream with a bit of water as a substitute for milk in recipes
• High calorie snacks like Cracklin Oat Bran give you more bang per bite! (You can make snack mixes with cereal, nuts and butter--bake in the oven for a great treat)
• Use Scandical in low calorie foods like applesauce to increase calories, it also works well in spaghetti sauces and other drinks.
• Scandishakes are great! I waited until after meals to offer these...and we were getting 2100 calories a day in scandishakes alone for my middle son.
• Use butter to cook vegetables (at one point we had a doctor tell us not to give our son vegetables because they lack calories. I thought that was crazy because I wanted him to like vegetables when he was older--so I opted to add butter)
• Flaxseed Oil can be added to canned peaches, etc to boost the calories. Scandical works a bit, but doesn't really mix with slices of peaches or fruit.
• Use Whole Milk Yogurts, cheeses and sour cream. These items can be hard to find (although with the recent news about whole milk being better for diabetics, things are turning around!) Stonyfield Farms makes excellent whole milk yogurts.
• We made a lot of high calorie fruit smoothies. You can add whole milk, cream, ice cream or whole milk yogurt to increase calories. We even made smoothies using Scandishake powder.
• Protein powder can be added to oatmeal and various other foods to increase calories and nutrition.
• As our children became older, we started using shakes like the Zone instead of Scandishakes because the enzymes were working and these shakes did not contain the sugar that carnation instant breakfast and Pediasure contain.
• Zone bars. My children still love Zone bars as snacks.
• Nuts- my children love peanuts, cashews and almonds.
• Wheat Germ- you can add toasted wheat germ to baked good or even sprinkle it on yogurt for added calories and nutrition.

Scandishake has recipes available on their website and a free cookbook offer. Scandishake recipes

Scandishake helps with weight gain

We used Scandishake before my boys ever started on enzymes and for a long tme after to help them gain weight. They come in several flavors and even have a lactose free version. My boys loved these shakes! There is a free recipe book that you can order from Axcan with recipes using scandishake to increase the calories in other areas.

Scandishake

SCANDISHAKE® calorie-rich shake mix is designed to assist patients who need to gain or maintain weight. Mixed with 8 ounces of whole milk, a single serving of SCANDISHAKE® contains approximately 600 calories!
SCANDISHAKE® is available in great-tasting Vanilla, Chocolate and Strawberry flavors and can be mixed with a variety of foods and beverages to help combat taste fatigue. Try SCANDISHAKE® mixed with different fruit juices, sodas and extracts, and in casseroles, sauces and desserts. Also try SCANDISHAKE® Lactose-Free and Sweetened with Aspartame formulations, both available in Chocolate and Vanilla flavors.
SCANDISHAKE® is gluten-free and certified as kosher.
For a FREE sample of SCANDISHAKE®, or to request additional information, please call (800) 4-SCANDI (800-472-2634).
You can also purchase SCANDISHAKE® directly from Axcan by calling the telephone number listed above or by visiting our Online Store.
SCANDISHAKE® is also available through your local pharmacy.
SCANDISHAKE® is not a meal replacement and should not be considered a complete nutritional supplement. Patients intending long-term use should consult a medical professional for additional dietary considerations.

Scandical by Axcan helps with weight gain

We used this product when my children were little. It contains MCT oil (medium chain triglycerides) which are the most easily absorbed fats there are.

Scandical

SCANDICAL® Calorie Booster is a taste-free powder that quickly and easily adds 35 calories per tablespoon to your meals, without changing the taste of the foods you love. SCANDICAL® comes in a portable, 8 ounce, shaker-top dispenser can, conveniently allowing you to add calories to individual servings and eliminating the need to prepare separate, high-calorie meals.
SCANDICAL® mixes easily with almost any food or beverage, hot or cold, right at the table. To help gain or maintain your weight, sprinkle SCANDICAL® Calorie Booster on all your favorite snacks and meals.
SCANDICAL® is gluten-free and certified as kosher.

Essentials for Giving Pancreatic Enzymes to Children

Another PDF brochure from Solvay. This explains why CF kids need enzymes (can also be used for SDS kids), information on how to give a child who cannot swallow pills enzymes, what to do if baby spits up during feeding, missed dosaes, etc.

Essentials for Giving Pancreatic Enzymes to Children

Essential Facts for Managing Pancreatic Insufficiency

Solvay has another great PDF brochure that is wonderful! This is a great resource to bring to teachers and school nurses. It goes into the basics of managing pancreatic insufficiency(PI), explaining what PI is, how pancreatic enzyme supplements work, symptoms of PI and correct dosage of enzymes.

Essential facts for managing Pancreatic Insufficiency

Essential Care for Pancreatic Enzymes - Education

Solvay has a great educational PDF brochure that is perfect for teachers, parents and physicians alike. This particluar PDF goes into how long Enzymes may be used, how to store enzymes and well as the best way to transport them.

Essential Care for Pancreatic Enzymes Brochure

SolvayCares Program

SolvayCares is a program from Solvay Pharmaceuticals is a nutritional, education and financial support program. For more information, please click on the hyperlink above. Solvay also has a More Extra Helpings program that provides free vitamins and high calorie drinks for patients.

Axcan (makers of Ultrase) Assistance Programs

Axan, the makers of Ultrase have a handful of programs-- for more info than what is below, see : http://www.scandipharm.com/productsunitedstates.php?lang=1
They have programs for countries around the world

ASSIST ProgramSM
CareFirst for CFSM Program
CF Direct Connect ProgramSM
Comprehensive Care Program for CFSM
Rx Cost Reduction ProgramSM

The Assist program helps folks without insurance pay for meds like Carafate and Ultrase.

The Carefirst program is for SDS and CF patients and it does the following:

With a prescription for ULTRASE® Enteric-Coated Microspheres or Enteric-Coated Minitablets, what will the patient receive?

Free ULTRASE® Enteric-Coated Microspheres or ULTRASE® MT Enteric-Coated Minitablets
Free ADEKs® Pediatric Drops
Free copy of Cystic Fibrosis: A Guide for Patient and Family educational book
Free diaper bag with CareFirst for CFSM logo

Rx Cost Reducation Program SM:

Information for Patients:

As the providers of ULTRASE® MT, it is important to us that the cost of your health care remains as affordable as possible. For this reason, Axcan created the Rx Cost Reduction ProgramSM.

This program will allow you to receive a rebate for your out-of-pocket expense (up to $20 per month), for a prescription of ULTRASE® MT12, ULTRASE® MT18 or ULTRASE® MT20.

How the Program Works:

Have your prescription for ULTRASE® MT filled at your regular pharmacy. Note: Your prescription must be for ULTRASE® MT12, ULTRASE® MT18 or ULTRASE® MT20
(a minimum of 100 ULTRASE® MT capsules is required).
Complete a Rebate Claim Coupon (which you may obtain from any CF center or by calling 800-472-2634), and mail it with the original pharmacy receipt to the address on the coupon. Please note that the Rx Cost Reduction ProgramSM is not available to patients who are participating in a federally-subsidized pharmacy benefit program, or where otherwise prohibited by federal or state law. Be sure to sign the coupon, certifying that the patient is not a participant in a federally-subsidized pharmacy benefit program.
You will receive a check (up to $20) in about three weeks for the amount of your out-of-pocket expense. Included with your check will be another Rebate Claim Coupon for use the following month.
Note: You may submit only one Rebate Claim Coupon per month. The Rx Cost Reduction ProgramSM may not be combined with the CareFirst for CFSM or the Comprehensive Care Program for CFSM. Coupons must be fully completed to be processed.

Natural Treatment for GERD

We discuss GERD a lot on the SDS support list. This is an interesting article written by a doctor on a natural treatment for GERD:

http://www.associatedcontent.com/article/685357/doctors_analysis_a_natural_treatment.html

Tea Bags for Mouth Sore Pain

I had heard of using tea bags to help stop bleeding...but until recently did not know that they helped with mouth sores! Some doctors suggest putting wet black tea bags on sores. Black tea contains tannin, a substance that can relieve pain. You can also find tannin in some over-the-counter medicines.

SSI Benefits Information

Government SSI information page includes a benefits eligibility screening tool.

Supplemental Security Income (SSI) is a Federal income supplement program designed to help aged, blind, and disabled people, who have little or no income; and it provides cash to meet basic needs for food, clothing, and shelter.