In This Month's Issue:
1 Dedication: this issue is dedicated to Sean Dooley, Daniel Black, Tyler Siefert , Noah Fisher, Alex Cooper, Gregory Shearon and Their Families
2 FROM THE EDITOR: About the Guardian Society
3 GUARDIAN UPDATES: Picnic Time, Upcoming births,
4 RESEARCH BY ... RainDance: Gas and what you need to know about gas
5 STOP THE MADNESS: Help the Children
6 LOST ANGEL: A tribute to the lost ones of Guardian Society
7 WEB LINKS: Guardian Geo Pages, Guardian has a new web address, Listsever, and much more
8 GUARDIAN CHILD OF THE MONTH: Terran Robinstein
9 A CHILD'S VIEW: Jennifer's Q k A by our star Reporter Jennifer Sparks
10 A Special Tribute to Sean Dooley as told by Lynn Dooley
11 LETTERS FROM THE MAILBOX: Letters from our Online k Offline Members
12 A LITTLE THIS, A LITTLE THAT: toddler miricle diet, Discover a Pen Pal , Parenting humor
13 NETWORKING PARENTS: Parents Share Their Stories, Ask for Help, and More
14 QUESTION FROM A PARENT: Michelle long asks about dilations
15 THE POWER OF THE INTERNET EXPLODES AGAIN: A parent ask about motity testing and the internet provides her answers, via our web reseracher, angela bennett who finds information on Anorectal Manometry and Esophageal Manometry
16 QUESTIONS FROM OUR ONLINE PARENTS: A Questions from parents
17 A FATHER'S VIEW: Read About Tom and Matthew Beehler, Father and Son with Hirschsprung's Disease
18 A SPECIAL POEM Guardian Angel sumitted by Carolyn Rosado
19 Neuronal Intestinal Dysplasia; simple constipation many children are being found to actualy have this read why and who can be affected sumitted by debbie Aunt to Matthew and Andrew Beehler
20 C-Difficle: Has your child recived antibiotics lately, then you need to read this research summitted Debbie aunt to Matthew and Andrew Beehler
21 A NOTE FROM OUR Medical advisor : Will Loe Introduces Himself
22 ANADOTES; smile, and funny things these kids say by jennifer Beehler
23 NOTICE: Please Do NOT Forget to Read Our Disclaimer
]˜™˜™] Dedication ]˜™˜™]
This edition of The Guardain is dedicated to Sean Dooley, Tyler Siefert, Daniel Black, Noah Fisher, Alex Cooper, Gregory Shearon and Their s courageous families.
Please rember these children by wearing your Burgandy Ribbons and and distrubuiting the newsletter in your area you to can help Stop the Madness of needless death and suffering
]˜™˜™] FROM THE EDITOR ]˜™˜™]
Dear Members,
I understand its been a while since we have been able to provide you with a newsletter, due to my sons increasing illnesses I have not been able to devote the time needed to accomplish this goal, Due to my son's increasing problems I have decided to make the newsletter a by monthly one to help cut down on some of my work so I can devote more time to my son, I hope you all understand and we will go back to monthly for at least the online version soon. Also in the news our web pages have moved please see our new location thanks to Fortune city for providing us with enough space for our pages and Mark Vannan for moving them for me. Our membership has grown so much in the last few months on June 28 guardian society will celebrate our 1 year anniversary its been a wonderful year for all of us in the group, our online listsever has become extremely active and the list grows daily. We have been able to reach children and adults from all walks of life as well as the globe. Who reach out to each other in times of need and in times of happiness, I am very pleased that I have been blessed with . finding such a wonderful group of people to work with. But bear in mind we are all parents some of us are experiencing loads of difficulty's like my son who I guess has not been a text book child with Hirschsprungs so due to this fact sometimes the newsletter will be late or that sort I hope and pray you understand and continue to support our efforts. On the Fundraising front Paula Siefer (co chair of Fundraising) has been very successful in raising funds on our behalf and we thank her for her efforts its her efforts and efforts of others that the guardian society is able to continue.
Kimberly Robinstein, President
Guardian Support Group
]˜™˜™] GUARDIAN UPDATES ]˜™˜™]
Its picnic time again and we are planning a wonderful one this year the picnic will be held in Quincy Illinois at Moormon Park, There will be a batting cage, Swimming, fishing, miniature golf, and a bang up baroque compliments of my husband Dennis (the great cook) Hot dogs, Barbecue chicken, Potato salad, Cole slaw but most of all good friends and loads of fun, So come on out make a weekend of it if you can lets make this years picnic a great one call 217-222-3906 for more information or directions. The date of our picnic is June 6 1998 10:00 am - exhaustion.
For those not on the listsever our own Gerry mcgregor has been giving us some very good insights into what it feels like after pullthru as some know Gerry is and adult with Hirschsprungs and after years of suffering had a illestomy and a few weeks ago underwent a new pullthru its been very informative learning what our kids may or may not feel after pullthru. Thanks Gerry for allowing us to invade your privacy and giving us insight to something I had wondered myself how it feels,
Upcoming births
Jeannie cooper is due in June with her third child, Jeannie as you all know lost her beloved Alexandra to Hirschsprungs disease we hope and pray for a healthy baby and look forward to the announcement sometime in June
Our own Karen Higgs is due in November, Karen most of you know is mom to Mary Helen who has total colon Hirschsprungs Karen herself has long segment Hirschsprungs their unique family has been very insight full in the genetic aspects of Hirschsprungs , we wish her a happy and fun pregnancy, and look forward to hearing of her birth as well.
]˜™˜™] RESEARCH BY ... RainDance ]˜™˜™]
Note: This article was found at The National Institute of Diabetes and Digestive and Kidney Disease's Homepage.
]˜™]Gas ]˜™]
GAS
Everyone has gas and eliminates it by burping or passing it through the rectum. However, many people think they have too much gas when they really have normal amounts. Most people produce about 1 to 3 pints a day and pass gas about 14 times a day. Gas is made primarily of odorless vapors-carbon dioxide, oxygen, nitrogen, hydrogen, and sometimes methane. The unpleasant odor of flatulence comes from bacteria in the large intestine that release small amounts of gases that contain sulfur.
Although having gas is common, it can be uncomfortable and embarrassing. Understanding causes, ways to reduce symptoms, and treatment will help most people find relief.
WHAT CAUSES GAS?
Gas in the digestive tract (that is, the esophagus, stomach, small intestine, and large intestine) comes from two sources:
Swallowed air - Normal breakdown of certain undigested foods by harmless bacteria naturally present in the large intestine (colon).
Swallowed air - Air swallowing (aerophagia) is a common cause of gas in the stomach. Everyone swallows small amounts of air when eating and drinking. However, eating or drinking rapidly, chewing gum, smoking, or wearing loose dentures can cause some people to take in more air. Burping, or belching, is the way most swallowed air--which contains nitrogen, oxygen, and carbon dioxide--leaves the stomach. The remaining gas moves into the small intestine where it is partially absorbed. A small amount travels into the large intestine for release through the rectum. (The stomach also releases carbon dioxide when stomach acid and bicarbonate mix, but most of this gas is absorbed into the bloodstream and does not enter the large intestine.)
BREAKDOWN OF UNDIGESTED FOODS
The body does not digest and absorb some carbohydrates (the sugar, starches, and fiber found in many foods) in the small intestine because of a shortage or absence of certain enzymes. This undigested food then passes from the small intestine into the large intestine, where harmless and normal bacteria break down the food, producing hydrogen, carbon dioxide, and, in about one-third of all people, methane. Eventually these gases exit through the rectum.
People who make methane do not necessarily pass more gas or have unique symptoms. A person who produces methane will have stools that consistently float in water. Research has not shown why some people produce methane and others do not.
Foods that produce gas in one person may not cause gas in another. Some common bacteria in the large intestine can destroy the hydrogen that other bacteria produce. The balance of the two types of bacteria may explain why some people have more gas than others.
WHICH FOODS CAUSE GAS?
Most foods that contain carbohydrates can cause gas. By contrast, fats and proteins cause little gas.
*Sugars
The sugars that cause gas are: raffinose, lactose, fructose, and sorbitol.
*Raffinose
Beans contain large amounts of this complex sugar. Smaller amounts are found in cabbage, brussels sprouts, broccoli, asparagus, other vegetables, and whole grains.
*Lactose
Lactose is the atural sugar in milk. It is also found in milk products, such as cheese and ice cream, and processed foods, such as bread, cereal, and salad dressing. Many people, particularly those of African, Native American, or Asian background, have low levels of the enzyme lactase needed to digest lactose. Also, as people age, their enzyme levels decrease. As a result, over time people may experience increasing amounts of gas after eating food containing lactose.
*Fructose
Fructose is naturally present in onions, artichokes, pears, and wheat. It is also used as a sweetener in some soft drinks and fruit drinks.
*Sorbitol
Sorbitol is a sugar found naturally in fruits, including apples, pears, peaches, and prunes. It is also used as an artificial sweetener in many dietetic foods and sugarfree candies and gums.
*Starches
Most starches, including potatoes, corn, noodles, and wheat, produce gas as they are broken down in the large intestine. Rice is the only starch that does not cause gas.
*Fiber
Many foods contain soluble and insoluble fiber. Soluble fiber dissolves easily in water and takes on a soft, gel-like texture in the intestines. Found in oat bran, beans, peas, and most fruits, soluble fiber is not broken down until it reaches the large intestine where digestion causes gas.
Insoluble fiber, on the other hand, passes essentially unchanged through the intestines and produces little gas. Wheat bran and some vegetables contain this kind of fiber.
WHAT ARE SOME SYMPTOMS AND PROBLEMS OF GAS?
The most common symptoms of gas are belching, flatulence, abdominal bloating, and abdominal pain. However, not everyone experiences these symptoms. The determining factors probably are how much gas the body produces, how many fatty acids the body absorbs, and a person's sensitivity to gas in the large intestine. Chronic symptoms caused by too much gas or by a serious disease are rare.
*Belching
An occasional belch during or after meals is normal and releases gas when the stomach is full of food. However, people who belch frequently may be swallowing too much air and releasing it before the air enters the stomach. Sometimes a person with chronic belching may have an upper GI disorder, such as peptic ulcer disease, gastroesophageal reflux disease (GERD), or gastritis. Believing that swallowing air and releasing it will relieve the discomfort of these disorders, this person may unintentionally develop a habitual cycle of belching and discomfort. Frequently, the pain continues or worsens, leading the person to believe he or she has a serious disorder. Two rare chronic gas syndromes are associated with belching Meganblase syndrome and gas-bloat syndrome. The Meganblase syndrome, which causes chronic belching, is characterized by severe air swallowing and an enlarged bubble of gas in the stomach following heavy meals. The resulting fullness and shortness of breath may mimic a heart attack. Gas-bloat syndrome may occur after surgery to correct GERD. The surgery creates a one-way valve between the esophagus and stomach that allows food and gas to enter the stomach but often prevents normal belching and the ability to vomit.
*Flatulence
Another common complaint is passage of too much gas through the rectum (flatulence). However, most people do not realize that passing gas 14 to 23 times a day is normal. Although rare, too much gas may be the result of severe carbohydrate malabsorption or overactive bacteria in the colon.
*Abdominal bloating
Many people believe that too much gas causes abdominal bloating. However, people who complain of bloating from gas often have normal amounts and distribution of gas. They actually may be unusually aware of gas in the digestive tract. Doctors believe that bloating is usually the result of an intestinal motility disorder, such as IBS. Motility disorders are characterized by abnormal movements and contractions of intestinal muscles. These disorders may give a false sensation of bloating because of increased sensitivity to gas. Splenic-flexure syndrome is a chronic disorder that seems to be caused by trapped gas at bends (flexures) in the colon. Symptoms include bloating, muscle spasms, and upper abdominal discomfort. Splenic-flexure syndrome often accompanies IBS.
Any disease that causes intestinal obstruction, such as Crohn's disease or colon cancer, may also cause abdominal bloating. In addition, people who have had many operations, adhesions (scar tissue), or internal hernias may experience bloating or pain. Finally, eating a lot of fatty food can delay stomach emptying and cause bloating and discomfort, but not necessarily too much gas.
*Abdominal pain and discomfort
Some people have pain when gas is present in the intestine. When gas collects on the left side of the colon, the pain can be confused with heart disease. When it collects on the right side of the colon, the pain may feel like the pain associated with gallstones or appendicitis.
WHAT DIAGNOSTIC TESTS ARE USED?
Because gas symptoms may be caused by a serious disorder, those causes should be ruled out. The doctor usually begins with a review of dietary habits and symptoms. The doctor may ask the patient to keep a diary of foods and beverages consumed for a specific time period. If lactase deficiency is the suspected cause of gas, the doctor may suggest avoiding milk products for a period of time. A blood or breath test may be used to diagnose lactose intolerance.
In addition, to determine if someone produces too much gas in the colon or is unusually sensitive to the passage of normal gas volumes, the doctor may ask patients to count the number of times they pass gas during the day and include this information in a diary.
Careful review of diet and the amount of gas passed may help relate specific foods to symptoms and determine the severity of the problem. If a patient complains of bloating, the doctor may examine the abdomen for the sound of fluid movement to rule out ascites (build up of fluid in the abdomen) and for signs of inflammation to rule out diseases of the colon.
The possibility of colon cancer is usually considered in people 50 years of age and older and in those with a family history of colorectal cancer, particularly if they have never had a colon examination (sigmoidoscopy or colonoscopy). These tests may also be appropriate for someone with unexplained weight loss, diarrhea, or blood not visible in the stool. For those with chronic belching, the doctor will look for signs or causes of excessive air swallowing. If needed, an upper GI series (x-ray to view the esophagus, stomach, and upper small intestine) may be performed to rule out disease.
HOW IS GAS TREATED?
The most common ways to reduce the discomfort of gas are changing diet, taking medicines, and reducing the amount of air swallowed.
*Diet
Doctors may tell people to eat fewer foods that cause gas. However, for some people this may mean cutting out healthy foods, such as f ruits and vegetables, whole grains, and milk products.
Doctors may also suggest limiting high-fat foods to reduce bloating and discomfort. This helps the stomach empty faster, allowing gases to move into the small intestine.
Unfortunately, the amount of gas caused by certain foods varies from person to person. Effective dietary changes depend on learning through trial and error how much of the offending foods one can handle.
*Nonprescription medicines
Many nonprescription, over-the-counter medicines are available to help reduce symptoms, including antacids with simethicone and activated charcoal. Digestive enzymes, such as lactase supplements, actually help digest carbohydrates and may allow people to eat foods that normally cause gas. Antacids, such as Mylanta II, Maalox II and Di-Gel, contain simethicone, a foaming agent that joins gas bubbles in the stomach so that gas is more easily belched away. However, these medicines have no effect on intestinal gas. The recommended dose is 2 to 4 tablespoons of the simethicone preparation taken 1/2 to 2 hours after meals.
Activated charcoal tablets (Charcocaps) may provide relief from gas in the colon. Studies have shown that when taken before and after a meal, intestinal gas is greatly reduced. The usual dose is 2 to 4 tablets taken just before eating and 1 hour after meals. The enzyme lactase, which aids with lactose digestion, is available in liquid and tablet form without a prescription (Lactaid, Lactrase, and Dairy Ease). Adding a few drops of liquid lactase to milk before drinking it or chewing lactase tablets just before eating helps digest foods that contain lactose. Also, lactose-reduced milk and other products are available at many grocery stores (Lactaid and Dairy Ease).
Beano, a newer over-the-counter digestive aid, contains the sugar-digesting enzyme that the body lacks to digest the sugar in beans and many vegetables. The enzyme comes in liquid form. Three to 10 drops are added per serving just before eating to break down the gas-producing sugars. Beano has no effect on gas caused by lactose or fiber.
*Prescription medicines
Doctors may prescribe medicines to help reduce symptoms, especially for people with a motility disorder, such as IBS. Promotility or prokinetic drugs, such as metoclopramide (Reglan) and cisapride (Propulsid), may move gas through the digestive tract quickly.
*Reducing swallowed air
For those who have chronic belching, doctors may suggest ways to reduce the amount of air swallowed. Recommendations are to avoid chewing gum and to avoid eating hard candy. Eating at a slow pace and checking with a dentist to make sure dentures fit properly should also help.
CONCULSION
Although gas may be uncomfortable and embarrassing, it is not life- threatening. Understanding causes, ways to reduce symptoms, and treatment will help most people find some relief.
Points to Remember
1.Everyone has gas in the digestive tract.
2.People often believe normal passage of gas to be excessive.
3.Gas comes from two main sources: swallowed air and normal breakdown of certain foods by harmless bacteria naturally present in the large intestine.
4.Many foods with carbohydrates can cause gas. Fats and proteins cause little gas.
5.Foods that may cause gas include: Beans Vegetables, such as broccoli, cabbage, brussels sprouts, onions, artichokes, and asparagus Fruits, such as pears, apples, and peaches Whole grains, such as whole wheat and bran Soft drinks and fruit drinks Milk and milk products, such as cheese and ice cream, and packaged foods prepared with lactose, such as bread, cereal, and salad dressing Foods containing sorbitol, such as dietetic foods and sugarfree candies and gums.
6.The most common symptoms of gas are belching, flatulence, bloating, and abdominal pain. However, some of these symptoms are often caused by an intestinal motility disorder, such as irritable bowel syndrome, rather than too much gas.
7.The most common ways to reduce the discomfort of gas are changing diet, taking nonprescription or prescription medicines, and reducing the amount of air swallowed.
8.Digestive enzymes, such as lactase supplements, actually help digest carbohydrates and may allow people to eat foods that normally cause gas.
ADDITIONAL READINGS
Larson, D. E. (Ed.). (1990). Intestinal gas. In Mayo
Clinic family health book (p. 634). New York:
William Morrow and Company, Inc. General medical
guide with section on intestinal gas.
National Digestive Diseases Information
Clearinghouse. (1994). Lactose intolerance (NIH
Publication No. 94-2751) [Fact Sheet]. Washington,
DC: U.S. Government Printing Office. General patient
Information fact sheet.
National Digestive Diseases
Information Clearinghouse
2 Information Way
Bethesda, MD 20892-3570
E-mail: nddic@info.niddk.nih.gov
The National Digestive Diseases Information Clearinghouse (NDDIC) is a service of the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK). The NIDDK is part of the National Institutes of Health under the U.S. Public Health Service. Established in 1980, the clearinghouse provides information about digestive diseases to people with digestive disorders and to their families, health care professionals, and the public. NDDIC answers inquiries; develops, reviews, and distributes publications; and works closely with professional and patient organizations and Government agencies to coordinate resources about digestive diseases.
Publications produced by the clearinghouse are reviewed carefully for scientific accuracy, content, and readability.
This e-text is not copyrighted. The clearinghouse encourages users of this e-pub to duplicate and distribute as many copies as desired.
NIH Publication No. 95-883
May 1995
Angela May Bennett (aka RainDance) is the official Web Researcher for the Guardian
RainDance (Angela May, 24, who has the tummy troubles, who got proudly through the tests on July 2, and still waiting for answers, and now has to have another test done)
Email RainDance at lazlo@best.com
http://www.geocities.com/SouthBeach/6606 (Find out all about me there)
http://www.geocities.com/Heartland/Meadows/7948 (My Tummy Trouble page got a whole new look!)
]˜™˜™] STOP THE MADNESS ]˜™˜™]
Please help the children.
"If only for someone to hold my hand in my time of need or to sit and chat and let me cry that's my prayer tonight."
Stop the Madness.
What is "Stop the Madness"?
It's a campaign to help raise awareness for our children who suffer from rare disorders. The campaign was started in memory of Alex Cooper, July 5, 1996 - November 22, 1996. Alex died from undiagnosed Hirschsprung's Disease and because of her death, our members decided to start wearing burgundy ribbons in memory of our children who suffer and sometimes die from being misdiagnosed or undiagnosed and/or rare diseases.
Please help us by sending for copies of our brochures and wearing a ribbon.
For our children please help.
]˜™˜™] Web Links ]˜™˜™]
here's the url for the Guardian site on geocities.......
http://www.geocities.com/Heartland/Prairie/7780/index.html
This site is maintained by our Web Research Angela Bennett
Guardian Members ....
I want to announce a new addition to the Geocities Guardian Socity Site ...
The Guardian Socity Boardroom. I found a great free site that lets people have their own message boards, and thought this would be a great way for
people stoping by the web page to leave messages, and for non-memebers to find and ask questions and find answers.
The Guardian Socity BoardRoom Guidelines .......
This is a message board for people with all kinds of digestive disorders such as: Hirschsprungs Disease, motility disorders,
Crohn's Disease, Pseudo-Obstruction, and any and all other forms of tummy troubles. Please only post in this subject manor.
The Geocity's Guardian Socity WebPage ......This site is maintained and updated by Angela Bennett
http://www.geocities.com/Heartland/Prairie/7780/index.html
Guardians new web page site is up and running
http://www.fortnuecity.com/milleum/ratty/136/guardian.htm this page is updated by Kimberly Robinstein
Guardian Societys Chat room is open
our chat room is open 24/7 for our online members who wish to meet and chat we have had some goofy chat seeions and some real fun.
http://wg.rnet.com/maniac/discuss.htm
Our listsever is one of the most popular in the internet. email us to day to find our how you to can join us in the online world
or send a messge addressed to
majordomo@avenza.com
subscribe guardian-l maniac@rnet.com
(replace my email addres with your own)
thanks nick and denise ulman from avenza who donates our listserver
]˜™˜™] LOST ANGELS]˜™˜™]
Recently guardian society has lost some special children Paula Siefert lost her son Tyler and Also The blacks lost their son Daniel so its with a sad heart that I write this months section, as Paula has been so active its been a sad time here for us too and I wanted to write about how I feel with Tyler's death, as well as all our lost angels, I have grown so fond of all the children in guardian Tyler's death shocked me, during all the stress of terrans illness I was so torn I wanted to jump in my car and rush up and hug her so tight, and tell her its all right I wanted to give her all twelve and support ties given me all these months, I have known this little boy for months before all of you met him Paula has been one of our longest members but mostly was shy at first, talking with me via icq and such and lurking our list when she first shared her sons story I was so touched to this day his picture and story is on my wall (made it hard to talk to Paula I looked at his picture as she told me the sad news) Paula always felt she did not belong as Tyler's problems to her were so minor, but tried to always raise my sprits when I was down, Tyler's had a tough time lately so has Paula she had a hard time but always seems to be so up and happy in speaking to her on the phone I always felt better. I looked forward to speaking to her and Virginia who always calls me in the am
and gets me up (heheh) So its with great shock and dismay at his sudden death, I had always hoped that with time Tyler would get better and the stress Paula and her family had would stop, she always spoke so highly of her Dr. Dufort made me jealous how her doctor always made time for Tyler and was so nice to her family, she was such and advocate for Tyler I never doubled she would do whatever she could to help her son, I felt her frustration in trying to figure out what was his problem and each time he would have a bad time she called me I got to know this little guy very well,
Paula your son was a light that will never go out his life was full of ups and downs but you always seem to bring him though, he was most loved I felt this love you had for your son in your words and heart when you spoke about him, it seemed with each problem you faltered but always landed on your feet on the day of Tyler's funeral I wished I was there to hold you and let you cry and talk I wish I was a millionaire and could afford to hop a plane and come to your rescue as you have so many times for me, I hope and pray you still come to our picnic and will continue with us as you are still welcomed,
Tyler will never be forgotten his life is forever embedded in our hearts and minds, and we will be here for you. He takes a very special place on guardian now with parents that can understand you loss together you all will laugh and cry together I am glad that if Tyler had to leave this earth it was while you were in our care and not alone, I wish they find a reason for his death, to put closure to your grief I know this will take time and loads of love and compassion. Your a special family your husband and daughter must be beside themselves with grief also I pray for them as well that they too find comfort. Tyler is with Jesus now I know we want him with us we want to have him safe and sound in his crib with his parents that love him and sister that needs him but god short angels so he took some of ours , We will miss seeing them grow and laugh but know they will have fun in heaven with our savior,.
Daniel I did not have the blessing of getting to know this little man very well, he was with us so shortly and left us so suddenly but I know his light is shining in heaven as from reading his story I feel I got to know him and his parents somewhat what a wonderful family whose pain right now is so deep, words can not express my feelings. Noah Fisher, Alex Cooper and Gregory Shearon, Sean Dooley These children's deaths were so avoidable in this day and age to have children die from Hirschsprungs. Death to me was never a reality till I met Jeannie and her daughter Alex, their story shocked me as it could have been my son, then we met lorrie and her son Gregory, and now Noah, and Sean Death of a child is so hard but when it is so avoidable it makes it so much harder.
Our guardian society now has Alex Cooper, Greggory Shearon, Daniel Black , Noah Fisher, and Tyler Siefert, and Sean Dooley I pray for all our lost angels, and their parents. It saddens me deeply that this can happen even today we still lose our angels I hold my son tighter and appreciate him more because you have shared your stories with us. and shared your pain and grief with us. I made posters of each child with their stories they are always on my wall and my heart and we take them with us to events like the picnic, each time I put on my burgundy ribbon and guardian pin each day I think of these children when asked I recite their names and stories, and hand them a brochure and newsletter and each night I pray for them as well as their families. In hopes that I may be able to help in some small way to elievate some of our children's and parents pain.
May you never grieve alone, may you never be forgotten, May you never cry alone, May you forever be loved.
The Lord bless you and keep you, the lord make his face shine upon you and be gracious to you; the lord turn his face toward you and give you peace.
numbers 6:;24-26
Kimberly Robinstein
President Guardian
]˜™˜™] GUARDIAN CHILD OF THE MONTH ]˜™˜™]
Terran Xavyer Zacharya Tymothy Edward Robinstein
Our son made his debut on August 16 1996 after 25 hours of labor he came into this world with a whisper, we took him home at 14 hours old. We were old hands at taking care of new borns terran was our 6 th child he was the quietest one of our bunch and we had to pinch ourselves that we had been blessed with such a sweet and quiet baby, He preferred to sleep than eat, never making a fuss, after a few days of this I became worried at his lack of eating our town provides a nurse to come check on the baby at a few days old even she could not coax our son to take any nourishment, on his 4 th day of life we finally got some food into him only to have him vomit it back out at us we were amazed that it was green, and grew very scared, upon taking him to his doctor he was admitted to our local hospital to find out what was wrong, we knew he had not had a bowel movement yet tests were started to rule out this or that our doctor told my husband he felt Terran had Hirschsprungs disease. He was airlifted out of our small town in the middle of the night to St. Louis Children's hospital and put under the care of Michael Skinner who performed a Full thickness biopsy to rull out Hirschsprungs but it came back positive fo it in stead, terran received his colostomy at 5 days old, and we were thown int o a whole new world, I did not do well with terrans ostomy and looked forward to hs pullthru in the hopes of having a normal child,
On December 6 1996 terran underwent a Soave Pullthru again performed by Michael Skinner, it was a momentous occasion when my son first pooped out his bottom, the whole ward cheered we took pictures to celebrate the occasion. He went home a few days later and so our new life began, He developed the worst rash I had ever seen in my life, to me and my husband it looked as though his bottom was burned, we tried nurmous remedies finally finding Ilex, worked for us he also developed a stricture that required our surgeon to dilate him this was horrible for both us and him, later on we had to dilate him several times a day at home, I think the worst part was after his pullhthru he cried and vomited constantly, never was he happy or that sweet baby we once knew, they blamed it on colic we changed formulas the whole nine yards, We took him to a GI here in Quincy who diagnosed terran with GERD and started him on Zantac this helped some later on they added in Propusid, this brought him some relief but not as much as we had hoped, I read about irrigation's and with our doctors permission we started these in hopes of helping terran. these too helped some for a few months, but we struggled to figure out what was wrong with our son. Soon we were sent to see a pediatric GI again in St. Louis once terran was maxed out on zantac she decided to start terran on prilosec for his reflux this made a tremendous difference in his vomiting but his stool pattern was still erratic, several times he was thought to have enterocoltis and was placed on flagyl, this too helped some but nothing seem to work long term, We could not get terran to have bowel movements for any length of time on his own, we tried irrigation's, senkot, lactlose, Milk of magnisia all worked only for a short time,
In December of 1997 terran was again admitted with distention and dehydration it was this admission that prompted my husband and I too seek new answers to our sons problems, In running guardian I had heard of some of our children undergoing a Cecosotmy tube placement, and decided to contact the doctor performing this procedure it seemed to me that this may help terran as the irrigation's were becoming quite a battle. I was amazed when Dr. Fonkalsrud called me at home in response to my emailing him my sons story, he felt terran would benefit from having the cecostmy tube so we decided to bring terran to see him at UCLA medical center, I can not say enough good about Dr. Fonkalsrud, he showed me and terran in his office as soon as we arrived and looked over terrans records a few days later terran underwent exploratory surgery Dr. Fonkalsud felt terran had Enterocoltis and a stricture and this accounted fo terrans continued problems, even attributed to terrans reflux, While there I had the pleasure of staying with Michele cairns and her family , and having Connie Thompson come dawn to sit with me during terrans surgery, Terri Buccate came down as well to meet terran and I and show us her beautiful son Billy, and her daughter and husband also visited us. this was by far my most happiest hospital stay ever. we left a few days later with terrans new cecosmty tube and started a new adventure.
To make a long story short, a few weeks upon returning home terrans cecostomy tube fell out, it was replaced by a ER doctor but had to be taken out again a few weeks later, due to an infection and other complications, a local surgeon replaced the tube surgically a week later but the infection and other complications required terran to be again airlifted from our small town but this time my husband and I decided to have terran taken to a new children's hospital, as we no longer felt comfortable with our current GI, who did not agree with our courses of treatments, thats when we met Dr. Thomas Weber, poor Dr. Weber he had to take quite a bit of anger on our part, we had struggled so much with terran and he felt the full rath of our anger but he never seemed to mind, and set to work to find out what was wrong with our son, after one contrast enema, he found terran had aa significant striker and underlying enteroclotis, and needed immediate surgery, he placed terran on tpn via a central line, and set to work, a few days later when terran was stronger terran underwent repeat biopsy that showed the ganglion cells in his pullthu section and about were not normal (nerural intestinal dysphasia) Dr. Weber said that was the reason for terrans continued problems, and placed a colostomy 11 days later we took our son home again, but he still was not right, he had been sick for too long, he refused to eat or drink much upon retrying for a follow up Dr. Weber took on look and admitted him again, and said he could not go home until he's eating or at least drinking some, after a few days it was evident terran would not eat well he was combative to all who tried to force him to eat even the most minute amounts, we consulted with Dr. Weber again about putting in a gtube and after some tests he aggeded terran needed a gtube and a few days alter terran again went under this time he was scoped and a peg tube was placed, A few days later terran received his first night of drip feedings and a few days later we finally got to bring our son home. This night mare began on Friday the 13th in march and ended Good Friday in April.
Now here a few weeks later its like I have a new baby, he's so full of energy, we no longer have to force feed him, as he gets most of his calories from his tube, meds etc. too, we are able to vent his stomach to prevent vomiting he's been off laxatives, irrigation's etc. for over a month, although he still has some enterocoltis Dr. Weber feels this will go away with terrans not holding stool in now that he has an ostomy, As far as pullthru goes Dr. Weber agrees with us that for terran now its not a priority, his heath is. Theres allot more to our story but it would be a newsletter itself, the reason I chose terran for this months newsletter is I hoped that by sharing terrans story some of our struggle parents would be able to understand and fight for their miracle ours came in terrans osotmy and gtube, With terrans new health we are not so angry as we were at first mainly I blame his GI for terrans problems, she listened but did not do much past that and med changes, even once telling me I needed counseling when we pleaded with her that we felt terran needed a repeat biopsy, that his illness was affecting all the family even down to our 3 year old son who began to have nightmares, had she listened and done something maybe our son would have been spared unneeded pain. But in the end we were able to help our son, our family doctor stood beside us and supported us and while its been a long long fight thankfully I have not been alone during all terrans struggles, I have been blessed with having a wonderful group of online friends to help me understand that what was happening to terran was not normal for a kid with Hirschsprungs and helped me seek answers. I will forever be grateful for all the prayers and loving support I received during our struggles, when our call went out for prayers I felt a 100 angels fly by our sides, I was renewed each time members called our hostile room to find out how my son was doing even from other countries Bea and Gerry called me and lifted my sprits, and when I needed them most they are always here ready to love me and my family I can never give back whats been given , to me but I will try. and at long last that nagging feeling in the pit of my stomach is gone.
Today terrain is beautiful normal healthy toddler, who just so happens to have an ostmy and gtube at night when he's not sleeping I simply put his formula and his pump in a back pack and off he goes like any normal child, he's so wonderful now the only bad thing is he no longer wants me to rock him for hours, he prefers to run with his big brother. And me I get down on my knees nightly and thank god he sent me terran but most of all that he sent me some of the most wonderful people to guide us in our struggles.
Kimberly Robinstein
President Founder
Guardian Society
819 College
Quincy Illinois 62301
217-222-3906
maniac@rnet.com
]˜™˜™] A CHILD'S VIEW ]˜™˜™]
By Jennifer Sparks
Jennifer is an Honorary Reporter for Guardian
Jennifer is 7 years old and was diagnosed with short bowel syndrome when she was 21 days old. She has always had a central line for TPN and has a button.
]˜™] QUESTIONS AND ANSWERS ]˜™]
Q: Does it hurt to have a c-line or the button?
A: The button hurts sometimes when it gets red. The line is a little sore right after they put it in. My leg hurts for a few days. It hurts to pull the tegaderm off. I hold my blanket and Daddy's finger.
Q: What don't you like about having the line and button
A: I don't like it when it shows through my clothes. I don't like being hooked up so much. I can't go places sometimes cause I am hooked up.
Q: Is there anything you like about having them?
A: I don't have to taste my yucky medicine
Q: What do you tell other kids when they ask what they are?
A: It's how I get my medicine. I say that the dressing is a Band-Aid.
Q: Does it embarrass you when the other kids notice it?
A: Yes, but some of my friends know about it, and they don't care.
Q: Do you feel hungry when you are on the pumps?
A: Sometimes.
Mom's P.S.:
I used to wonder how Jennifer felt about everything that she has gone through. To her, it is just part of her. She once asked why she had problems, but for the most part, she just accepts it. If any of the parents of babies who have lines or buttons have some questions about what it is like for their child, just send your questions to Jennifer. She thinks it would be neat to be a "reporter" for the newsletter.
Jennifer Sparks
C/o Marita Sparks
1060 Oak Street
Stephenville, TX 76401
Email: st_msparks@tarleton.edu
Thanks Jennifer for your honest answers and insight to our children's world. We look forward to more from you in the future.
]˜™˜™] A SPECIAL TRIBUTE TO SEAN ]˜™˜™]
a special story written by his mom Lyn Dooley
I will never forget the thrill of learning I was expecting a baby. Married 3 months, due just before our first wedding anniversary - how perfect. I thought of nothing else but that baby for the whole pregnancy - did everything 'healthy' and nurtured the baby as best I could. Now overdue. After a very healthy 'picture-perfect' pregnancy, a scan reveals baby is breech and large. Booked into hospital the next day for caesarean section. So nervous the night before. Stephen and I couldn't sleep and both felt sick (and we both were) just because of nervous excitement.
Then at exactly 4.00 p.m. on 23 June 1990, the day before our first wedding anniversary, our beautiful 8lb 11oz baby boy was born. He was absolutely beautiful. Welcome darling Sean. We both cried with joy and I couldn't believe how handsome and healthy he was.
Stephen went with Sean back to maternity while I had to stay in theatre to be closed etc. Finally went back to the ward and held my darling baby for the first time, I was so proud. Nurses helped me to try and breastfeed Sean for the first time - he was not at all keen. They suggested we leave the feeding until later as Sean seemed more tired that hungry. They left Stephen, Sean and I together. Suddenly Sean vomited over me. It was thick and black and awful. We felt frightened - it was obviously the meconium. Stephen got a nurse. She cleaned Sean up and said it was nothing. As it was late they would keep an eye on him in the nursery and that I should get some sleep - they would wake me when it was time to feed Sean again. About 9.00 p.m. Stephen went home, we were both exhausted, and I settled into sleep.
At 10.30 p.m. that night, someone was waking me. I woke to find five people in my little room. Two doctors and three nurses - the look on their faces made my heart race. Suddenly they were telling me all of this information about xrays and blockages and blood tests and operations - they had ordered an ambulance and were transferring Sean to Wellington Hospital (about two hours away) for emergency surgery - he was very ill. Why oh why hadn't they told me this was all happening. I was crying - I needed Stephen. I wanted to be with Sean - doctors were not agreeable as I had just had major surgery - I said I didn't care if I had to walk, but I was going with my son. My obstetrician was called in, he agreed to let me go - gave me a large dose of morphine to help with the trip and instructions for further doses to be given on the trip. Nurse brought me a phone to ring Stephen....ring, ring, ring....all I could get out was "Sean very sick" "Operation in Wellington" "Get here". So much was happening so fast. Nurses whispering in the corridor. My parents trying to be encouraging - kissing Seanie, praying for him. Stephen and I were crying. Darling Seanie was sleeping all through it!
Into the ambulance at midnight and off to Wellington - happy anniversary Stephen. I lay opposite Sean in the ambulance, staring at him all the way, willing him to be OK. Arrive at Wellington - Sean whisked away immediately. Doctor Kevin Pringle arrives. He is the paediatric surgeon. Thinks that Sean has a blocked or twisted bowel. Xrays appear OK, need to operate now. Sign consent forms. Absolutely blew me away when he asked if we wanted Sean baptised. It hadn't even crossed my mind as so much was happening. Priest waiting at the door. Yes. Priest baptised Sean in pre-op. We were not allowed to be there.
Wait, wait, wait. Finally Mr Pringle arrives back to tell us results. No obvious blockage or twisted bowel. We have to 'wait and see'. He mentioned that it could possibly be a disease called "Hirschsprungs" - may involve another operation or two, but everything will be OK. Sean will be drip fed in the meantime - I was asked to express my breastmilk, which was then frozen. He should be able to feed in a couple of days.
Went to see Seanie in Intensive Care Unit that morning. He had tubes and wires everywhere. Machines were beeping and alarming. It was so noisy - but all I could see was my beautiful little boy. He had been cut right across his tummy. He seemed to be sleeping so peacefully. What a terrible start to life. Why? Why? Why?
Sean still did not pass a bowel motion. Mr Pringle suggested a colostomy until his condition settled. Day 3 of his life, and off for his second operation. Colostomy went OK. Biopsies were taken of his bowel for testing. Mr Pringle suggested trying a feed of breastmilk - 5mls! Just a teaspoon, but it seemed a start. Seanie continually sleeping - very high morphine dosage, so he wasn't really aware of anything going on. Took feed via a syringe. Waiting, waiting, waiting, still no bowel motion. Things not looking good. Vomiting, vomiting, vomiting. Mr P. said he would now put in a permanent central line drip - feeds by drip only. A special food called TPN - it was a yellow colour and kept in a black bag to protect it from sunlight.
I sat at his bedside for 12 hours a day - wouldn't leave him.
Seanie now settled into drip-feeding - 24 hours per day, but would eventually reduce to 12. Still vomiting - Mr Pringle said we may just have to accept the fact that he will vomit several times a day until the problem is fixed. He is very optimistic. I can stay in the hospital ward with Sean and sleep in a small stretcher beside his bed. For the first time since he was born, I was with him for 24 hours a day - it finally felt as though he was my child, I felt so content, at long last I really felt like his Mummy.
Life settled. I became part of the ward. The nurses and I became friends. It was challenging learning to change the TPN every day. The nurses taught me everyday - I was keen to do everything for Sean. I eventually learnt and then taught Stephen - we were both apt at it. I then did everything for Sean - even having to put in nasogastric tubes in his nose to empty his tummy, when the vomiting became too bad - I hated doing that. The nurses did nothing for him. I entered all the information on his chart and discussed everything with the doctors. I felt a little bit in control. Colostomy bag changes I could have done with a blindfold. The only help I would have from the nurses was the odd 'chat' to keep me sane!
Diagnosis was eventually confirmed as Hirschsprungs - long-segment. Possibly his whole bowel was affected. Many surgeries followed. His colostomy resited several times. More and more bowel removed. Mr Pringle performed all his surgeries, biopsies were taken from several sites. They had a pathologist on hand during surgery who checked the biopsies for ganglion cells and advised yes/no immediately. Apparently the cells are very difficult to 'find'. Mr Pringle was told 'yes', but it was later confirmed as 'no'. So it was very 'hit and miss'. Sean's biopsies were even sent to a hospital in Australia for specialist checking. Mr Pringle had dealt with a case as bad as Sean's before when he worked in America - this little boy made it - I remember seeing a photo of him, fishing with his Dad - it gave me hope. He contacted Doctor's at Boston for their advice when nothing seemed to work for Sean. He really worked so hard for us. The largest operation Sean had went for 8 hours and it was hell. Sean nearly didn't make it after that one and was very ill - it was a side-to-side anastomosis (Kimura Procedure) which Mr Pringle learnt in The States. It basically involved the removal of sections of the bowel and then re-attaching them throughout the rest of the bowel (in the hope that some ganglion cells might be present in the re-attached sections and aid a further part of the bowel to work). Nothing seemed to work. There was one further op. to try. Mr Pringle said that if ganglion cells were found, the operation would take the entire day again. If no ganglion cells were found, he would be back in the ward within a couple of hours and that there was nothing further he could do and they would not be able to cure or save Sean. We were waiting, waiting. After nearly two hours Mr Pringle walked in the room. I will never forget it. I just stared at him and he had tears in his eyes. It was a pretty chilling experience.
16 November 1990. A date I will never forget. Finally bringing Seanie home - and Daddy's birthday as well. I was free of that place. Arrangements were made for all the medical equipment and supplies to be delivered to our house. After five months, Sean and I were finally coming home for the first time.
Sean thrived at home. He was like a different child. The vomiting settled. He was like any normal little baby boy during the day - other than his weakness, never able to sit, or crawl etc, he led a relatively normal life. No-one in the street would have guessed he never ate - was attached to a drip feeder at night for 12 hours. Sean and I went everywhere. After all of those months trapped in hospital, we never spent a day at home. We always had somewhere to go. I was so happy with life - I thought nothing would change. Those doctors could say what they liked about Sean's future and his chances of survival - all along they told us he would make it, now they tell us he wouldn't. They had been wrong all the way along, so why should I believe what they said now. The less we saw of doctors the better!
By the end of the day he would be low on energy and you could tell he needed his TPN. Within 1/2 an hour of attaching the TPN, he would go crazy, like he was on drugs - a real high. Laughing and playing. He loved lying on the floor with no pants on and kicking and kicking. Within an hour, he would usually be ready for bed. Vomiting continued. He was so brave, it was normal to him. He would probably vomit several times every night. He would just wake, vomit, then go straight back to sleep. No crying - Stephen or I would jump up to him, and he would be smiling away at us. Sean was an inspiration to us.
One day Sean and I were visiting his Nana (Grandma), he didn't seem that well. He vomited - but this time it was full of blood. I felt scared, but tried to put on a brave face - its no big deal, he has probably just irritated his tummy wall or his stoma. What was happening. Looking back now, I can see that Seanie was slowly deteriorating, slowly becoming more ill and not quite as 'bouncy' as he had been. I felt so isolated and alone. I knew no-one to talk too that even knew about this disease. No Internet back then.
Blood vomits became more and more regular. They distressed Sean at first - the taste, but after awhile he just accepted them like everything else. I never got used to them. I would hear him vomiting beside me at night. Race to his cot - I can't explain the feeling of seeing that. I was frightened. Really frightened. Yet also, I became used to it as well. There would occasionally be blood in his bag also - just got used to it.
Sean was deteriorating. He wouldn't have his 'high' after hooking his TPN on. He was very yellow, but otherwise life just kept on going - we did all the normal things and really enjoyed being together. We loved that boy so much, no words can express it.
Then it was the long weekend. Sean hadn't been overly well that week. Just a little quiet and always wanting to be cuddled and held. Loved being walked around by his Poppa (Grandad), who would sing in his ear. Sean started looking very sick. This was so different. I knew what was happening and I was shocked. Honestly and truly I was shocked - even though I had been told time and time again, I never believed it. I always believed he would get better - but for the first time, I could see he was dying.
Sean deteriorated very quickly. Lapsed into a coma. It was a long night. We had him at home and the three of us cuddled all night. I hated seeing Sean suffer. Sean seemed to wake from his coma. Began 'baby-talking' in a really urgent way - as though he had lots to say to us. We both knew somehow that he was saying goodbye. I remember saying to him that it was time for him to let go, to stop fighting it and to not be afraid, that he will be happy. I had my hand on his chest. I could feel his heart beating. I realised that the beats were slowing. I told Stephen and I felt afraid. Slowly the beats begun being very erratic. Sean stopped talking and peacefully stopped breathing. My hand felt his heart stop beating. It was 6.10 a.m. on Monday, 3 June 1991. Seanie's suffering was finally over. If only love could have saved him. It was two weeks before his first birthday. Officially Sean died of massive haemorrhaging due to liver failure as a complication of the long-segment Hirschsprungs Disease.
Everyone had to be told. Arrangements had to be made. We were absolutely devastated. Nothing like this could ever be imagined. You could not guess at the excruciating pain we were feeling. How we got through it I will never know. These feelings lasted for many years. Life was hard. Everyone expected us to go back to being the people we were before. That is never possible. If you go through something like this, and it doesn't change you, then I don't understand. It was so hard - for both of us.
Three months later I started a new job. It was devastating. It was like the final acceptance that Sean had gone. Back to work. No longer a baby to care for. But I had a mission. I had one last thing to do for Seanie - and that was to buy a marble headstone for his grave. We had little money. We paid for the funeral and all the bills we had acquired over the past year. We then saved and saved and worked and worked. After six months we could afford a headstone. I cried and cried and cried when I saw it. It was all so official - so final looking.
I still have dreams that feature Sean - it is always the same - I am cuddling him, nothing more but lovely big cuddles. I love them and am convinced Sean is sending them to me. I always feel good. No-one else understands that feeling but it is like Seanie is remembering me too and sending me these dreams. They are precious. Just like every time I see a rainbow - there was a rainbow everyday for a week after Sean died. I believe Sean sent them to us. People must think I am mad, but everytime I see a rainbow, I say "Hello Seanie".
Sean died nearly seven years ago. I visit his grave every week with fresh flowers. It is the only thing I can do for him, but I will never, never forget him.
It is only now that I can even write these few words dSown. I only ever remember the good times with Sean. The family times. The healthy and happy times. I hide the sad, troubled, painful times. I am glad that Seanie's story has now been told. I know that one day I will again hold him in my arms and I know that he is in heaven and that he is well. I feel so lucky and so blessed to have loved Sean. He taught us a great deal. He is our inspiration and even though he is not here physically, he is always with me - always.
Five years later we had a second son. It was a very scary 9 months. But we have been blessed with a beautifully healthy son. You have never seen two parents so excited over their baby's first bowel motion - if only people knew why....
Sean Dooley - Christmas time!
Lyn Dooley
lyn.dooley@natlib.govt.nz
Lyn thank you for telling us of your beautful son Sean, in reading his story I felt the great love you have for this special angel, I grieve with you and your family, you are a special person to have such courage to share his life with us, thank you we are forever richer thanks to his life and a very special family.
]˜™˜™] IN THE MAIL ]˜™˜™]
Please sign me up to receive your newsletter. I think your organization is great!! I have a 6 year old son (Jake) who was born with Hirschsprung's Disease short segment. He is still having problems even though he has been through his three surgeries. We have made pooping a habit because he does not have the "urge". He is still not potty trained at night. He is a terrible eater and gets constipation and fevers quite often. I would like to help anyone I can who is just going through the colostomy or surgeries and I welcome any advise from people with older Hirschsprung's children. Thank you!!
email: cmanders@prestolite.com
==================================================
Hi! I am a 31 year old female Hirschsprung's Disease survivor. I just found your website and am very grateful. I am very out of the loop on the subject, since I had my surgery back in 1970.I would like to chat with others about the disease, especially any adult survivors. I am stillexperiencing some physical ghosts because of Hirschsprung's. Please help! Thank you.
cate@peconic.net>
+++++++++++++++++++++++++++++++++++++++++++++++++++++++++
I am interested in joining and finding out more!I am mom to Evan who is 4 months old. He was born with a type IIIbjejunal atresia, with the christmas tree deformity. Following theinitial surgery to correct the atresia he retained approximately 80-100cm of bowel. He had a second surgery to correct adhesions. He had a gtube placed at 3 1/2 months of age. He has a broviac as well. Hecontinues to have severe motility disfunction, and has never been out ofthe hospital.I also have three other children, twin 4 year old and a 2 year old.Thanks in advance for the info.
Ellen A. Whelan <eawhelan@earthlink.net>
+++++++++++++++++++++++++++++++++++++++++++++++++++++++++++++++
Hi. My name is Lori . I have two children with Hirschsprungs disease. Ihad the same thing when I was young. My oldest now 4 had short segment hirschsprungsand did not need the colostomy. We opted not to have the surgury when he was first born cause we did not know anything about short segment hirschsprungs. Then when my youngest was born 2 years ago he was diagnosed at birth at a children's hospital and we had my 4 year old looked at and everything the other hospitals did to him was wrong they tried to stretch my 4 year olds bowel muscle when he was 6 months old but that does not work. Nobody ever said that. My 2 year old needed the the colostomy bag he had it for about 4 months . They both are doing well now. I am just having problems with my 4 year old not knowing when he has to bm for potty training. People say he will do it on his own but I know I had alot of problems when I was in grade school. I need some suggestions. My 2 year old has had his diaper rash since three days after having the pull through done. He gets used to alot of the diaper rash stuff out on the market. A former nurse told me to try that Bag Balm stuff it is the stuff for cow's udders I use everytime and it does help but he goes so much its hard to control. I been using this for about 3 months now and he has not had a really bad rash that has bled. I would like to here from parents with children with hirschsprungs.
Lori Stough ZL-1@prodigy.net
˜™˜™] QUESTION ]˜™˜™]
Hi Kimberly ,
My name is Jennifer and I just wanted to tell you that I like your son Terran 's WebPages its really cute . I just had a baby on Feb 3rd and he has been diagnosed with HD . We are currently irrigating him 3 times a day and weekly vists to a pediatric surgeon . He is not suppose to have the pull through until he is 1 year . I just wanted to know if you would be able to answer some questions about HD for me . This is all new to me and very confusing . I would greatly appreciate it .
e-mail lavinge@ifx.net
Thanks Jennifer
We have a 9 week old child who was diagnosed with hypoganglionosis of the small intestine after a laparotomy (multiple biopsies of the ileum,jejunum,transverse and ascending colon) 7 weeks ago. She passed meconium 55 hrs after birth, with bile stained vomits, and is currently on long term TPN (broviac catheter in the right jugular vein). She is,however, opening her bowel now but her gastric aspirates are still plenty. Please offer advise on possible medical intervention. Thank you. Jocelyn and Steven Weiss Weiss@eisa.net.au
]˜™˜™] A QUESTION FROM A PARENT ]˜™˜™]
Hi everyone
I have a question about the latest treatment that the surgeon is putting TJ. through. First, a little background:TJ is now 8 months old. He had his pullthru surgery on 10-20-97 and we have been doing daily dilitations at home since three weeks after that. We started with sizes 5/6, 7/8 and the 7/8 was tight. In January we were able to move up to the 9/10 and it seemed like it took so long to get those sizes even the slightest bit "comfortable" for him - never without crying. So, on 3-23-98 we had him in for a stricture revision. The surgeon came out of the OR and said that he was able to dilate with a 15/16 when he was done. The recovery from this surgery was HORRIBLE. TJ cried and screamed with every bm for a good 2 weeks and was going about 12+ times a day again. It has gotten better and then we were dilating 1x day with sizes 5-10. TJ has been so uncomfortable and still bleeding some with those dilitations. But, when we went to the surgeon on Thurs., he stepped him up to an 11/12. I was almost in tears - there was so much blood and he was screaming. I was so devastated. It seems like since this last revision we haven't made any progress - even maybe stepped backward in an attempt to get forward. I keep asking why the blood? and when can I get these "torture instruments" out of my house? I had hoped we would be further along than this by now, maybe even doing them on an as needed basis. TJ is anything but cooperative at this point. He is older, stronger, wiser, etc. The surgeon says that we are not there yet - so I asked where are we going? He wants to be able to dilate with a 13/14 (probably with no blood and no resistance) before we are done with daily dilitations. He said that the blood just shows him how deep the scar tissue is, but he thinks we are making progress.
Now the questions: Does anyone else do daily dilitations now or in the past? How long did you do them? What do you see as the purpose for them? (I thought it was to help him open up enough that he could have regular bms but he seems to be doing that already and we still are stuck with them) Do you think that the blood indicates an irritation of the scar tissue? How do you get past scar tissue - will we end up with more surgery? How can the surgeon say he wants to be at a 13/14? Is that what a typical infant would be? Basically, I am getting very frustrated. TJ seems to be doing very well - having some trouble getting started on babyfood, but is still breastfeeding well and is a BIG boy. He is having good bms at least 2-3 times a day and some small bms and smears too. I guess I maybe don't know what the surgeon's viison is for TJ. He tends to only tell me what he has to, unless I ask the right questions. Can anyone help me ask the right questions? I'm wondering if maybe I should take TJ for a second opinion, although I hate to do that since Dr. Morton has seen us through so much. Also, a little question: Has anyone had their children on an infant multivitamins with flouride and iron? Did it cause constipation for them? My ped has said the iron is physiologic iron and won't cause constipation, but I frankly don't believe him - I remember my prenatal vitamins! TJ hasn't even been tested for an iron deficiency - and I'm not sure he needs it.
Well, thanks for letting me vent. I hope maybe someone has had some experiences with dilitations and can help me get some perspective on this.
Thanks alot!!
Michele Long, mom to TJ, 8 mos., HD ( and crawling, but BACKWARDS!!)
]˜™˜™] A LITTLE THIS ]˜™˜™]
TODDLER MIRACLE DIET
People are always on the lookout for a new diet. The trouble with most diets is that you don't get enough to eat (the starvation diet), you don't get enough variation (the liquid diet) or you go broke (the all-meat diet). Consequently, people tend to cheat on their diets, or quit after 3 days. Well, now there's the new Toddler Miracle Diet! Over the years you may have noticed that most two years olds are trim. Now the formula to their success is available to all in this new diet. You may want to consult you doctor before embarking on this diet, otherwise, you may be seeing him afterwards. Good luck!!!
DAY ONE----
Breakfast: One scrambled egg, one piece of toast with grape jelly. Eat 2 bites of egg, using your fingers; dump the rest on the floor. Take 1 bite of toast, then smear the jelly over your face and clothes.
Lunch: Four crayons (any color), a handfull of potato chips, and a glass of milk (3 sips only, then spill the rest)
Dinner: A dry stick, two pennies and a nickel, 4 sips of flat Pepsi..
Bedtime snack: Throw a piece of toast on the kitchen floor...........
DAY TWO-----
Breakfast: Pick up stale toast from kitchen floor and eat it. Drink half bottle of vanilla extract or one vial of vegetable dye.
Lunch: Half a tube of "Pulsating Pink" lipstick and a handfull of Purina< Dog Chow (any flavor). One ice cube, if desired.
Afternoon Snack: Lick an all-day sucker untill sticky, take outside, drop in dirt. Retrieve and continue slurping until it is clean again. Then bring inside and drop on the rug.
Dinner: A rock or an uncooked bean, which should be thrust up your left nostril. Pour grape Kool-Aid over mashed potatoes; eat with a spoon.
DAY THREE-----
Breakfast: Two pancakes with plenty of syrup, eat one with fingers, rubin hair. Glass of milk; drink half, stuff other pancake in glass. After breakfast, pick up yesterdays sucker from rug, lick off fuzz, put it on the cushion of your best chair.
Lunch: Three matches, peanut butter and jelly sandwich. Spit several bites onto the floor. Pour glass of milk on table and slurp up.
Dinner: Dish of ice cream, handfull of potato chips, some red punch. Try to laugh some punch through nose, if possible.
FINAL DAY----
Breakfast: A quarter tube of toothpaste (any flavor), bit of soap, an olive. Pour a glass of milk over bowl of cornflakes, add half a cup of sugar..Once cereal is soggy, drink milk and feed cereal to dog.
Lunch: Eat bread crumbs off kitchen floor and dining room carpet. Find that sucker and finish eating it.
Dinner: A glass of spaghetti and chocolate milk. Leave meatball on plate. Stick of mascara for dessert.
Denise Camp <harleytra@hotmail.com>
]˜™] A LITTLE THAT ]˜™]
Please come and visit Discover A Pal.
Discover Technology's Directory lists those with handicaps who want to expand their world by communication with other person either with or without handicaps.
http://discovertechnology.com/Pals/PenPals.htm
Summitted by Amantha Cole <anna@electrotex.com>
]˜™] PARENTING HUMOR ]˜™]
Dear Parents and Lovers of Children,
Here is some Parenting humor.
DUMBWAITER: one who asks if the kids would care to order
dessert.
FEEDBACK: the inevitable result when the baby doesn't appreciate the strained carrots.
FULL NAME: what you call your child when you're mad at him.
GRANDPARENTS: the people who think your children are wonderful even though they're sure you're not raising them right.
INDEPENDENT: how we want our children to be as long as they do everything we say.
OW: the first word spoken by children with older siblings.
PRENATAL: when your life was still somewhat your own.
PUDDLE: a small body of water that draws other small bodies wearing dry shoes into it.
SHOW OFF: a child who is more talented than yours.
STERILIZE: what you do to your first baby's pacifier by boiling it and to your last baby's pacifier by blowing on it.
TOP BUNK: where you should never put a child wearing Superman jammies.
VERBAL: able to whine in words
WHODUNIT: none of the kids that live in your house
. From: Patty & Darrell Woods <woodscln@inland.net>
]˜™˜™] GOT STORIES FOR US? ]˜™˜™]
Send to Kimberly Robinstein,
- or -
email the Editor: maniac@rnet.com
]˜™˜™] NETWORKING PARENTS ]˜™˜™]
My son Andrew (18 months old) was born with Hirschsprung's (entire colon was removed). We have met another family in Bakersfield, CA who also has a child with Hirschsprung's. She referred us to you.
Andrew seems to have chronic diarrhea. Nothing comes through but brown water. Last week he was getting dehydrated. He was admitted to the hospital. Tests show no bacteria, virus, etc in his stool. Is this common? What have others found out? Electrolyte level was low. This level would come back up and then go down.
He is on his way to UCLA Medical Center Today. Does anyone have any thoughts?
Thanks Babette, for sending them to the Guardian.
]˜™] MORE ]˜™]
My son is 71/2 and has Hirschsprung's Disease. I have been trying to start a group in my area and have not had much luck! Please write back if you receive my memo.
Thanks,
Ramona Painter
Hal_Painter@prodigy.com
Ramona has graciously volunteered to be our new Regional Support Parent for Florida. Welcome, Ramona!
]˜™˜™] THE POWER OF THE INTERNET EXPLODES AGAIN ]˜™˜™]
Sharon asks the group
What type of tests are done for motility testing. Any info would be great.
and a our ever ready web researcher comes up with some answers for her. heres what she found
Sharon .....
I have found info for you on anorectal manometry, and esophagealmanometry, which are motility studies. I have had both of these myself.They don't hurt have done, but they are uncomfortable.
AngelaMay (RainDance) 25, Chronic Intestinal Pseudo-Obstruction. Whos
currently busy working on writting out her whole tummy story.
lazlo@best.com
>ICQ# 1385068
* * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * *
* * * * * * * * *
Anorectal Manometry
Synonyms:
Anal Rectal Motility; ARM; Balloon Manometry for Fecal
Incontinence; Rectosphincteric Manometry
Procedure Commonly Includes:
Direct measurement of pressures in the anal canal, including the internal and external anal sphincters, along with assessment of rectal sensation and reflexes. This is performed by using either a manometry probe or a 3-balloon
apparatus with external pressure transducers. It is most useful in the evaluation of suspected Hirschsprung's disease and difficult cases of fecal incontinence.
Indications:
Only carefully selected patients should undergo anorectal manometry (ARM). Certainly, not every patient presenting with fecal incontinence warrants invasive testing. The procedure is indicated when: evaluating cases of suspected Hirschsprung's disease, in both children and adults; particularly useful in patients with megacolon of unknown etiology or adults with possible "short-segment"Hirschsprung's disease evaluating difficult cases of fecal incontinence especially if one of the following is suspected: surgical trauma to anal sphincters or nerve
structures; invasive perianal disease compromising regional nerve or muscle impaired motor function and/or sensory innervation involving one or both anal sphincters from systemic disease (eg, scleroderma, polymyositis,
etc); impaired sensation of rectal distention, as in diabetes mellitus assessing fecal continence mechanisms following surgery for defecation disorders (surgery for imperforate anus, Hirschsprung's disease, etc)
Contraindications:
Uncooperative patient, confused or comatose patient; ARM usually requires the patient to voluntarily squeeze and relax muscles controlling continence active lower GI bleeding history of allergy to rubber or latex products.
Patient Preparation:
The details of the procedure are discussed with the patient and consent is obtained. Patient should understand that the procedure involves inflation of a balloon in the rectum. In many centers cleansing of the rectal vault is performed
routinely, using saline or Fleet ® enemas, sometimes also cathartics. Some specialists do not request any bowel prep unless hard stool is expected. Sedatives should not be automatically given, but instead should be reserved for the very young (and extremely anxious)patient.
Aftercare:
No specific postprocedure restrictions are necessary. Patient may resume previous level of activity.
Complications:
ARM is a very safe procedure. To date, only one major complication has been reported, that of systemic anaphylaxis.2 This was presumed to be an IgE-mediated hypersensitivity reaction to the latex manometry balloon, as it came in contact with the rectal mucosa.
Equipment:
Two different systems for performing ARM are popular, and equipment for each is different. (The First International Symposium on ARM reported a wide variety of commercial devices in common use, the majority deemed acceptable3).In the first system, "perfusion manometry," a small diameter (0.7-2 mm) manometry probe is utilized, resembling an esophageal manometry catheter. In some models there is a large inflatable rectal alloon at the distal tip. This probe is a soft plastic catheter with radially arranged ports (sensing orifices), and uses a standard water perfusion system. Other acceptable devices include stiff, hollow metal catheters and dacron-woven catheters with microtip sensors. Attached to the manometry catheter is a pressure transducer for each sensing orifice. Results are recorded on a multichannel polygraph machine. The second system is a nonperfused or "balloon manometry" system. Here, three balloons are aligned in series on a hollow metal cylinder. The largest balloon (50 mL capacity) is the "rectal balloon," and is attached to the end of the cylinder. After prope placement, it will be located in the upper anal canal. The middle balloon is doughnut-shaped and will be positioned in the anal ampulla, surrounded by the internal (and part of the external) anal sphincter. The external balloon will lie at the anal verge, within the external anal sphincter. Each balloon is connected to its own pressure transducer, which in turn transmits to the multichannel recorder. The large rectal balloon may be inflated or deflated by means of an air-filled syringe.
Technique:
The procedure is performed only by an experienced GI specialist in a fully equipped procedure room. Perfusion manometry technique: With patient in supine position (or left lateral), a standard digital xamination is carried out and anal wink reflex tested. Manometry probe is advanced per rectum approximately 10 cm (in the adult), then slowly withdrawn using station pull-through technique.Pressures are continuously observed and the area of high pressure corresponding to the anal sphincters is located. (This may require several repetitions to ensure reproducibility of results.) This so-called "basal anal pressure" is recorded with the patient relaxed. The patient is then asked to perform a maximal sphincter squeeze, and again, highest pressures are recorded. This is termed the "maximal squeeze pressure". Following this the manometry catheter is reinserted as before and the 50 mL rectal balloon inflated slowly. Patient reports the first conscious sensation of rectal fullness and the volume of the rectal balloon is recorded at that time. In addition, response of the anal sphincters to rectal distention may be measured with some devices. Balloon manometry technique: If the three balloon apparatus is used, it is inserted per rectum following a digital rectal exam. This device is advanced approximately 8 cm or until the distal (external) balloon is just inside the anal verge and can barely be visualized. Both the middle and external balloons are then inflated and pressure tracings noted. The large internal rectal balloon is inflated with up to 50 mL air. Patient reports the first sensation ofrectal fullness. A "threshold value" of rectal sensation is obtained by deflating the balloon slowly and noting the smallest volume sensed. Following this, the large rectal balloon is reinflated and simultaneous pressures recorded from the middle balloon (reflecting internal and external sphincter in upper anal canal) and external balloon (reflecting external sphincter in anal verge). Additional pressure tracings reflecting the external sphincter may be obtained during cough, anal pinprick, and other maneuvers. Data Acquired:With perfusion manometry: basal pressure of sphincter zone (mm Hg)maximal squeeze pressure with maximum voluntary sphincter contraction squeeze increment (maximal squeeze pressure minus basal pressure) rectal sensation and threshold if rectal balloon is used. With balloon manometry: rectal sensation and rectal distention sensory threshold urge to defecate following rectal distention response of internal sphincter to rectal distention response of external sphincter to rectal distention estimation of rectal compliance.
Normal Findings:
Testing information is reported by an experienced GI specialist.
For basal and squeeze pressures, a wide range of normal values has been reported due to the variety of manometric devices in use. Thus, normal ranges for men and women must be defined by each individual laboratory. Examples are basal pressure roughly 60 mm Hg, squeeze pressure approximately 200 mm Hg in men. The normal response to rectal distention (simulated by the inflated rectal balloon) is reflex relaxation of the internal sphincter. This is the "rectoanal inhibitory reflex" under autonomic nervous system control. This occurs within seconds of rectal distention (often less than 20 mL in balloon) and a 30-40 mm Hg drop in sphincter pressure is seen. This is measured by the middle balloon of the 3-balloon system. Simultaneously there is contraction of the external anal sphincter, the "rectoanal contraction response".This response to rectal distention is felt to be a learned phenomenon and not a reflex per se. When the large rectal balloon is slowly inflated,most normal individuals can consciously sense distention with approximately 10 mL air. Rectal compliancecurves generated from pressure-volume measurements are compared against the norm for an individual laboratory.
Critical Values:
Test is considered positive (abnormal) if a measured variable or reflex response consistently falls outside the established normal range.
Additional Information
In certain disease states, ARM abnormalities may be pathognomonic. In Hirschsprung's disease, for example, rectal distention may lead to paradoxical internal sphincter contraction, rather than relaxation. Aganglionosis invariably involves the internal sphincter, and due to involvement of the intramural plexus, the rectoanal inhibitory reflex is abolished. Accuracy is high enough to obviate the need for deep muscle biopsy in some cases. In the adult with short segment Hirschsprung's disease, ARM may be the only practical means of establishing the diagnosis. Although ARM is rarely diagnostic of a specific disease in patients with fecal incontinence,important information regarding sphincter failure may be obtained. Abnormally low basal pressures (with normal squeeze pressures) indicate isolated internal anal sphincter dysfunction. Abnormally low squeeze pressures (with normal basal
pressures) a re characteristic of isolated external sphincter dysfunction. Systemic involvement of neuromuscular disease may at times be confirmed by ARM in patients with fecal incontinence. In scleroderma, for instance, incontinence may be due to selective involvement of the smooth muscle in the internal sphincter. Striated muscle characteristically is spared. There is loss of the rectoanal inhibitor reflex, but external sphincter contraction remains intact. In polydermatomyositis only striated muscle is involved. External sphincter contraction is impaired but the rectoanal inhibitory reflex is normal. It should be noted that a number of underlying systemic diseases may also cause fecal incontinence and isolated external sphincter dysfunction, including myotonic dystrophy, hyperthyroidism, myasthenia gravis, and perhaps diabetes mellitus. Abnormal sensory threshold for rectal distention suggests a lesion in sensory neural pathways. This has been demonstrated in some diabetics with fecal incontinence where the required distending volume of the rectal balloon is more than 10 mL greater than normal controls. Decreased rectal compliance is the etiology of fecal incontinence in a limited number of diseases. These include radiation proctitis, inflammatory bowel disease with rectal involvement, rectal ischemia, and (possibly) fecal impaction.
* * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * *
* * * * * * * * *
Esophageal Manometry
Synonyms:
Esophageal Manometric Study; Esophageal Manometry
Procedure Commonly Includes:
Passage of a multilumen manometry catheter into the esophagus and stomach. This device is connected to at least three separate pressure transducers and is capable of sensing squeeze pressures at different sites within the esophageal lumen. Quantitative data is obtained regarding the amplitude (mm Hg) and duration (seconds) of contractions of the upper esophageal sphincter (UES), lower esophageal sphincter (LES), and several sites within the esophageal body. A number of distinct esophageal motility disorders may be diagnosed. Provocative agents may also be employed to induce manometric abnormalities in patients with chest pain of obscure etiology. This procedure has become widely used and is considered the standard of diagnosis for most motor diseases of the esophagus.
Indications:
To evaluate the patient with suspected primary esophageal motor dysfunction. This includes (but is not limited to) the following: primary achalasia,diffuse esophageal spasm, chronic idiopathic intestinal pseudo-obstruction(manometry may aid in the initial diagnosis of this entity), nutcracker esophagus idiopathic LES incompetence.
To evaluate the patient with suspected secondary esophageal motility disorder due to systemic disease, including: scleroderma, polydermatomyositis, systemic lupus,erythematosus, amyloidosis, Chagas' disease, primary skeletal muscle disease (eg,myotonia dystrophica), multiple sclerosis, amyotrophic lateral sclerosis. To evaluate the patient with chest pain of unclear etiology, in order to determine whether symptoms are of an esophageal origin. This usually follows a negative cardiac work-up. To assess the competence of the LES, in difficult cases of reflux esophagitis, where the diagnosis is still in doubt or where standard medical therapy has failed. To evaluate the success of various surgical procedures on the esophagus, such as esophageal dilatation, LES myotomy in achalasia, and antireflux surgery. To ensure proper placement of pH probes (used to diagnose acid reflux) by accurately locating the LES.
Contraindications:
Few contraindications exist for this procedure. In general, the patient should be alert, able to swallow on command, and capable of verbalizing symptoms. This is particularly true if provocative studies to elicit chest pain are planned. Manometry should not be performed on a patient with tenuous cardiopulmonary status in whom vagal stimulation is hazardous.
Patient Preparation:
Technique and goals of the procedure are discussed with the patient and informed consent is obtained. No antacids, nitrates, calcium channel blockers, pain medications, or anticholinergic agents are permitted for 24 hours prior to procedure (if possible). If study is scheduled for the AM, the patient is made NPO after midnight. NPO for 8 hours beforehand if scheduled for PM. Patient is sent to procedure room with medical record. No premedications are given prior to manometry and sedatives are not permitted.
Aftercare:
There are no specific restrictions postprocedure. The previous level of activity may be resumed if no complications have arisen.
Special Instructions:
Requisition from ordering physician should state if additional studies such as pH probe testing, acid perfusion (Bernstein) testing, or pharmacologic provocation are desired. Otherwise, decision will be left to the discretion of the operator.
Complications:
Esophageal manometry is considered a safe procedure with little morbidity. Potential complications are those common to any nasogastric (NG) intubation, such as gagging, epistaxis, vasovagal reactions, etc. More serious adverse reactions may result from medications given for provocative testing. In particular, ergonovine is used in some centers to provoke esophageal contraction and spasm. This agent is also used to induce coronary artery spasm during cardiac catheterization in patients with possible variant (Prinz metal's) angina. Thus, there are potential cardiac complications if ergonovine is used. Other agents have come into favor recently, such as edrophonium, which have little cardiac effects. Abdominal cramps and nausea of a transient nature have been reported with edrophonium.
Equipment:
The manometry probe is a polyvinyl catheter with multiple lumens (from 3-8). Each lumen has a separate orifice. Probe diameter ranges from 1.1-5 mm, depending on the model. There are usually three or more side holes spaced approximately 5 cm apart which act as pressure sensors. Each catheter lumen is independently perfused with water from a multichannel pneumohydraulic infusion device (with low compliances). A separate pressure transducer is also attached to each lumen and pressures are recorded on a multichannel polygraph. A swallowing sensor is also used in some centers.
Technique:
The procedure is performed in a fully-equipped GI Laboratory. Patient assumes a supine position and a swallowing sensor is placed around patient's neck. The catheter assembly is calibrated and pressures rechecked with a sphygmomanometer. The device is introduced via the nares using standard nasogastric intubation technique (alternatively, per mouth) and advanced until all sensing orifices are recording gastric (cardia) pressures. The station pull-through technique is commonly used. With the recorder on, the catheter is slowly withdrawn until the proximal channel is within the LES. Resting LES tone is measured first. Patient is given water via a 50 mL syringe and is asked to swallow and the relaxation response of LES is noted. The catheter is then pulled out slowly at 0.5 cm increments until the most distal orifice is sensing the LES (all channels having passed through the LES). the catheter is taped into position and patient performs a series of wet swallows (5 mL H2O) over several minutes. Information is obtained regarding the LES, peristalsis, and esophageal body contractions. The catheter is then withdrawn approximately 3 cm and retaped, so that all sensing orifices are within the esophageal body. Wet swallows are repeated and contractions recorded. Next, pull-through is continued until the proximal orifice is surrounded by the UES; perfusion is stopped in this channel to prevent aspiration. Catheter is withdrawn until the proximal orifice is within the pharynx, the middle is in the UES, and the distal in the upper esophageal body. Ten dry swallows are performed. This concludes the standard procedure. When indicated, provocative testing is carried out once standard manometry is completed.
The catheter is repositioned and edrophonium is injected (10 mg I.V.usually) or a placebo. Again, patient performs wet swallows and esophageal contractions are recorded at different catheter positions. Subjective complaints of chest pain are carefully recorded and correlated temporally with any new manometric findings. The acid perfusion (Bernstein) test may also be performed at this time.
Normal Findings:
Preliminary written report on manometric findings and provocation testing is completed immediately by gastroenterology staff and placed in medical chart. This includes both selected numerical data and an overall diagnostic interpretation. Normal values are established for each individual testing center, and are dependent to some degree on the equipment system selected. An example of a normal motility and provocation study as reported by one laboratory is shown in the table.
Critical Values:
A wide variety of esophageal contraction abnormalities is possible.However, several "classic" patterns of manometric readings have been described, and may be diagnostic for a particular motor disorder.
Esophageal Motility Study Report:
As shown, a normal control subject experiences a short duration wave in the upper esophagus following a wet swallow. (In general, the upper esophagus is composed of striated muscle, and the middle and lower portions contain smooth muscle.) The wave progresses down the esophagus in a characteristic, timed fashion,that is, a peristaltic wave. The LES appropriately relaxes to accommodate the anticipated food bolus. In achalasia, however, the initial striated muscle contraction following a swallow may be normal but smooth muscle contractions (mid and lower esophagus) are of low amplitude and prolonged duration. Characteristically, the LES maintains a high basal pressure and fails to relax after a swallow. In a condition known as diffuse esophageal spasm, there is an increase in the baseline pressure in the mid and lower esophagus after a swallow. In addition to this, multiple repetitive high amplitude (short duration) contractions are seen in smooth muscle. These are "aperistaltic" contractions (ie, not coordinated with the initial skeletal muscle contraction). LES pressures may be normal or high and relaxation is variable. The "nutcracker esophagus" is characterized by smooth muscle contractions of very high amplitude and long duration. Peristalsis is maintained, although propagation may be slowed. LES tone and relaxation are relatively normal. In scleroderma, a unique pattern is seen. Upper esophageal contraction is normal but all smooth muscle contractions are markedly diminished in amplitude or even absent. Unlike achalasia,the LES pressure is low. In polymyositis (not shown) there is potential involvement of any striated muscle, including the pharynx and UES. Decreased amplitude contractions may be seen in the pharynx along with hypotension in the UES, but smooth muscle contractions remain normal. Milder variants of the idealized patterns in the figure may be seen. Also, mixtures of these contraction abnormalities are possible. In patients with chest pain of obscure etiology, esophageal manometry is often considered to rule out an unspecified motor disorder. If a patient experiences typical chest pain symptoms during the course of a standard examination and a classic motility disorder is recorded during the same time interval, an esophageal origin of symptoms is likely. Appropriate treatment of the motility disorder should be initiated. However, if typical chest discomfort occurs during standard manometry but pressure tracings are normal, then motility dysfunction is less likely as the cause of symptoms. It is not, however, entirely excluded. Conversely,if manometry is clearly abnormal but the patient denies any chest discomfort, the test is inconclusive. Some clinicians elect to treat the underlying motor disorder empirically. This is based on data which shows that the majority of patients who experience chest pain secondary to an esophageal motor disorder display continuous manometric abnormalities but only intermittent symptoms. In addition, 80% of patients with such continuous contraction abnormalities have reported significant chest discomfort. The proper management of this group is still evolving. Various pharmacologic agents have been used to induce abnormal contractions in selected patients with chest pain of obscure origin. This may be attempted in difficult cases where barium enema, upper endoscopy, and standard manometry are nondiagnostic. A cholinergic agonist, edrophonium chloride, is popular because of its specificity and favorable safety profile. If following injection of edrophonium there are symptoms of typical chest pain and manometric abnormalities, the test is considered positive. If either chest pain or contraction abnormalities are induced (but not both), test is inconclusive. Commonly, the acid perfusion test is included as a provocative test (see Bernstein Test). The finding of a hypotensive LES on manometry and a positive Bernstein test is strongly suggestive of symptomatic reflux esophagitis.
]˜™]