This edition of The Guardian is dedicated to Sean Dooley, Tyler Siefert, Daniel Black, Noah Fisher, Alex Cooper, Gregory Shearon and Their courageous families.

Please remember these children by wearing your Burgundy Ribbons and distributing the newsletter in your area you to can help Stop the Madness of needless death and suffering

 

]˜™˜™] FROM THE EDITOR ]˜™˜™]

Dear Members,

I understand it's been a while since we have been able to provide you with a newsletter. I have not been able to devote the time needed to accomplish this goal, due to my son's increasing problems. So I have decided to make the newsletter a bi-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 Gerry McGregor for fixing, updating and maintaining the new pages. Our membership has grown so much in the last few months, and it has been a wonderful year for all of us in the group. Our online listserver 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 the times of need and in the times of happiness. I am very pleased that I have been blessed with finding such a wonderful group of people to work with. So please keep in mind that 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. I hope and pray you understand and continue to support our efforts.

Kimberly Robinstein, President

Guardian Support Group

 

 

 

]˜™˜™] GUARDIAN LISTSERVER NEWS]˜™˜™]

Wow the internet has been a buzz, Paula Siefert alerted us all to the problems that can be caused from Propusid (see attached article from Jannsen) and shared with us the delight and sorrows from the life of her son Tyler who died last year.

Jennifer Beehler and Gerry McGregor have done a stand up job running the list and web pages in my month long absence, many topics have been discussed, like pullthru, pullthru rash, chronic constipation, Hirschsprungs Enterocoltis (HEC), and my favourite is Jennifer describing how to make a happy face with her keyboard. (Sorry Jen I still can't do it myself.)

]˜™˜™] Important News ]˜™˜™]

Note: Sherri Jordan found This article at http://www.fda.gov/medwatch/safety/1998/propul.htm

JANSSEN

PHARMACEUTICA

RESEARCH FOUNDATION

IMPORTANT SAFETY AND EFFICACY INFORMATION

June 26, 1998

Dear Doctor,

Janssen Pharmaceutica would like to inform you of labelling changes concerning new

CONTRAINDICATIONS, WARNINGS, PRECAUTIONS, ADVERSE REACTIONS, and

DRUG INTERACTIONS with PROPULSID (cisapride). In addition, revisions have been made in

the PEDIATRIC USE, INDICATIONS AND USAGE, and DOSAGE AND

ADMINISTRATION sections.

We wish to draw your attention to the following sections of the revised PROPULSID labelling which

contain the essential changes.

Warning: Serious cardiac arrhythmias including ventricular tachycardia, ventricular

fibrillation, torsades de pointes, and QT prolongation have been reported in patients

taking PROPULSID. Many of these patients also took drugs expected to increase cisapride

blood levels by inhibiting the cytochrome P450 3A4 enzymes that metabolize cisapride. These

drugs include clarithromycin, erythromycin, troleandomycin, nefazodone, fluconazole,

itraconazole, ketoconazole, indinavir and ritonavir. Some of these events have been fatal.

PROPULSID is contraindicated in patients taking any of these drugs. (See

CONTRAINDICATIONS, WARNINGS, PRECAUTIONS, and DRUG

INTERACTIONS).

QT prolongation, torsades de pointes (sometimes with syncope), cardiac arrest and

sudden death have been reported in patients taking PROPULSID without the

above-mentioned contraindicated drugs. Most patients had disorders that may have

predisposed them to arrhythmias with cisapride. PROPULSID is contraindicated for those

patients with: history of prolonged electrocardiographic QT intervals; renal failure; history of

ventricular arrhythmias, ischemic heart disease, and congestive heart failure; uncorrected

electrolyte disorders (hypokalemia, hypomagnesemia); respiratory failure; and concomitant

medications known to prolong the QT interval and increase the risk of arrhythmia, such as

certain antiarrhythmics, including those of Class 1A (such as quinidine and procainamide) and

Class III (such as sotalol); tricyclic antidepressants (such as amitriptyline); certain tetracyclic

antidepressants (such as maprotiline); certain antipsychotic medications (such as certain

phenothiazines and sertindole), astemizole, bepridil, sparfloxacin and terodiline. (The

preceding lists of drugs are not comprehensive.)

Recommended doses of PROPULSID should not be exceeded.

 

INDICATIONS & USAGE

PROPULSID (cisapride) is indicated for the symptomatic treatment of adult patients with nocturnal

heartburn due to gastroesophageal reflux disease. Because of the risk of serious, and sometimes fatal,

ventricular arrhythmias (see Boxed Warning), PROPULSID should generally be reserved for patients

who do not respond adequately to lifestyle modifications (See PRECAUTIONS: Information for

Patients), antacids and gastric acid reducing agents.

CONTRAINDICATIONS

Serious cardiac arrhythmias including ventricular tachycardia, ventricular fibrillation,

torsades de pointes, and QT prolongation have been reported in patients taking

PROPULSID (cisapride) with other drugs that inhibit cytochrome P450 3A4. Some of these

events have been fatal.

Concomitant oral or intravenous administration of the following drugs with cisapride may

lead to elevated cisapride blood levels and is contraindicated (See WARNINGS,

PRECAUTIONS and DRUG INTERACTIONS):

Antibiotics:

Oral or i.v. erthromycin, clarithromycin

(BIAXIN), troleandomycin (TAO)

Antidepressants:

Nefazodone (SERZONE)

Antifungals:

Oral or i.v. fluconazole (DIFLUCAN),

itraconazole (SPORANOX), oral

ketoconazole (NIZORAL)

Protease inhibitors:

Indinavir (CRIXIVAN), ritonavir

(NORVIR)

 

PROPULSID is also contraindicated for patients with: history of prolonged electrocardiographic QT

intervals; renal failure; history of ventricular arrhythmias, ischemic heart disease, and congestive heart

failure; uncorrected electrolyte disorders (hypokalemia, hypomagnesemia); respiratory failure; and

concomitant medications known to prolong the QT interval and increase the risk of arrhythmia, such

as certain antiarrhythmics, certain antipsychotics, certain antidepressants , astemizole, bepridil,

sparfloxacin and terodiline. The preceding lists of drugs are not comprehensive.

PROPULSID should not be used in patients with uncorrected hypokalemia or hypomagnesemia or

who might experience rapid reduction of plasma potassium such as those administered

potassium-wasting diuretics and/or insulin in acute settings.

WARNINGS

ECG should be considered prior to initiation of cisapride. Cisapride should not be used in patients

with a prolonged QT interval at baseline, those with a history of torsades de pointes, or those with

long QT syndrome. Cisapride should also be avoided in patients with sinus node dysfunction, and in

those with second or third degree atrioventricular block.

Cisapride should not be used concomitantly with other drugs known to prolong the QT interval;

certain antiarrhythmics, including those of Class 1A (such as quinidine and procainamide) and Class

III (such as sotalol): tricyclic antidepressants (such as amitriptyline); certain tetracyclic

antidepressants (such as maprotiline); certain antipsychotic medications (such as certain

phenothiazines and sertindole); astemizole, bepridil, sparfloxacin and terodiline. (See

CONTRAINDICATIONS, PRECAUTIONS and DRUG INTERACTIONS.) The preceding lists

of drugs are not comprehensive.

PRECAUTIONS

General: Potential benefits should be weighed against risks prior administration of cisapride to

patients who have or may develop prolongation of cardiac conduction intervals, particularly QTc.

These include patients with conditions that could predispose them to the development of serious

arrhythmias, such as multiple organ failure, COPD, apnea and advanced cancer. (See

CONTRAINDICATIONS.)

PROPULSID (cisapride) should not be used in patients with uncorrected hypokalemia or

hypomagnesemia, such as those with severe dehydration, vomiting or malnutrition, or those taking

potassium-wasting diuretics. PROPULSID should not be used in patients who might experience

rapid reduction of plasma potassium, such as those administered potassium-wasting diuretics and/or

insulin in acute settings.

Paediatric Use: Safety and effectivenesss in paediatric patients have not been established. Although

causality has not been established, serious adverse events, including death, have been reported in

infants and children treated with cisapride. Several paediatric deaths were due to cardiovascular

events (third degree heart block and ventricular tachycardia).

Paediatric deaths have been associated with seizures and there has been at least one case of "sudden

unexplained death" in a 3-month-old infant. Other unlabeled potentially serious events which have

been reported in paediatric patients include: antinuclear antibody (ANA) positive, anemia, hemolytic

anemia, methemoglobinemia, hyperglycemia, hypoglycemia with acidosis, unexplained apneic

episodes, confusion, impaired concentration, depression, apathy, visual changes accompanied by

amnesia, and severe photosensitivity reaction.

A one-month-old male infant received 2 mg/kg of cisapride four times per day for 5 days. The

patient developed third degree heart block and subsequently died of right ventricular perforation

caused by pacemaker wire insertion.

ADVERSE REACTIONS

Postmarketing Reports: In addition to the cardiovascular adverse events, the following events

have been identified during post-approval use of cisapride in clinical practice. Because they are

reported voluntarily from a population of unknown size, estimates of frequency cannot be made.

These events have been chosen for inclusion in this insert due to a combination of their seriousness,

frequency of reporting, or potential causal connection to cisapride: allergic reactions, including

bronchospasm, urticaria, and angioedema; possible exacerbation of asthma; psychiatric events,

including confusion, depression, suicide attempt, and hallucinations; gynecomastia, female breast

enlargement, urinary incontinence, hyperprolactinemia and galactorrhea.

The following events were specifically reported in the paediatric population: antinuclear antibody

(ANA) positive, anemia, hemolytic anemia, methemoglobinemia, hyperglycemia, hypoglycemia with

acidosis, unexplained apneic episodes, confusion, impaired concentration, depression, apathy, visual

changes accompanied by amnesia, and severe photosensitivity reactions.

DOSAGE AND ADMINISTRATION

PROPULSID should be discontinued if relief of nocturnal heartburn does not occur. The minimum

effective dose should be used. Recommended doses of PROPULSID should not be exceeded.

It is recommended that the daily dose be halved in patients with hepatic insufficiency.

We at Janssen want you to be aware of this important information to ensure the proper use of

PROPULSID in your patients. Please refer to the enclosed revised package insert for full prescribing

information. The Medical Community can further our understanding of adverse events by reporting all

cases to Janssen at 1-800-Janssen (526-7736) or to the FDA MedWatch program by phone at

1-800-FDA-1088, by fax at 1-800-FDA-0178, or by mail to MedWatch, HF-2, FDA, 5600

Fishers Lane, Rockville, MD 20857. For additional medical information, please call 1-800-Janssen

from 8 AM to 8 PM Eastern Time, Monday through Friday.

Sincerely,

Mark A. Klausner, M.D.

Vice President, Medical Affairs

The following brand names are registered trademarks of the companies listed below.

Abbott Laboratories

BIAXINÒ (clarithromycin), NORVIRÒ (ritonavir)

Bristol-Myers Squibb

SERZONEÒ (nefazodone)

Janssen Pharmaceutica, Inc.

SPORANOXÒ (itraconazole), NIZORALÒ (ketoconazole)

Merck & Co. Inc.

CRIXIVANÒ (indinavir)

Pfizer, Inc.

DIFLUCANÒ (fluconazole), TAOÒ (troleandomycin)

 

Janssen At Washington Crossing

1125 Trenton-Harbourton Road

PO Box 200

Titusville, New Jersey 08560-0200

 

]˜™˜™] 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 ]˜™˜™]

Guardians new web page site is up and running http://www.bigfoot.com/~theguardians. Gerry McGregor updates this page

Stop by and check out the awesome job Gerry has done on the photo gallery and pages, put faces to the names and add your child or yourself to the most favourite page.

Guardian Societies 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 sessons and some real fun.

The Guardian backup site on geocities.......http://www.geocities.com/Heartland/Prairie/7780/index.html

Our Web Research Angela Bennett maintains this site

Guardian Members ....

I want to announce a new addition to the Geocities Guardian Society Site ...The Guardian Society 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 stooping by the web page to leave messages, and for non-members to find and ask questions and find answers.

The Guardian Society 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 Society WebPage ......This site is maintained and updated by Angela Bennett

Guardian also has a children's listserver for children and adolescents who would like to be connected with others its open to sibs, and sick kids, and is run by Angela Bennet, contact us to get your kids online, list is supervised for the children's protection.

Guardian Children's listserver guardian@lists.best.com

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 message addressed to

guardiansociety-subscribe@egroups.com. In the body of text say

subscribe guardian and then your email address example "subscribe guardian

maniac@rnet.com"

(replace my email address with your own)

 

]˜™˜™] ROTOVIRUS ]˜™˜™]

 

ROTAVIRUS

What you need to know

10.30.98

Rotavirus is the most common cause of serious diarrhoea in young children. This infectious virus can cause your child to lose fluids very quickly, and it's especially dangerous for children less than a year old. This infection of the intestinal tract is known as rotavirus gastroenteritis. Almost all children get rotavirus at least once by age 4. The risk is highest between 6 and 24 months of age. Every year, thousands of young children are hospitalised and, rarely, some even die due to complications from rotavirus infection. In the Northern Hemisphere, rotavirus is most common during winter.

Symptoms

Rotavirus gastro-enteritis usually starts with a fever, nausea and vomiting, followed by diarrhoea. The illness can range from mild to severe and last from 3 to 9 days. Diarrhoea and vomiting may result in dehydration. Billions of rotavirus particles are passed in the stool of someone who is infected. Even tiny amounts of rotavirus can lead to infection if a baby puts fingers or other objects contaminated with the virus into his or her mouth. Young children may pass it on to siblings and parents. Childcare centres are high-risk areas for spread of the disease. Careful washing of your infant's hands, as well as handwashing by those who come in contact with your child, is the best way to prevent rotavirus from spreading. Do you have what it takes to fight germs?

 

About the vaccine

A vaccine is now available to prevent the most serious effects of rotavirus in young children. This vaccine helps prevent serious dehydration. Even if your immunised child catches rotavirus, the illness may be milder. The vaccine does not prevent diarrhoea caused by other organisms. Who should get the rotavirus vaccine?

"Children under 6 months of age who were born full-term should begin the three-dose series of this vaccine," says Dr. Robert M. Jacobson, a paediatrician at Mayo Clinic, Rochester, Minn. "The vaccine is given by mouth at 2, 4, and 6 months of age. The vaccine may be given at the same time as other vaccines." There are some cautions. Dr. Jacobson says it's important to remind your child's doctor or nurse if your child lives with someone with an illness that affects the immune system, such as HIV, or who is on chemotherapy for cancer or receiving immunosuppressants for organ transplantation. People with weakened immune systems may develop rotavirus symptoms if exposed to someone who's received the vaccine. The risks of rotavirus vaccine "Some babies may develop a fever 5 days after vaccination and usually with the first dose," Dr. Jacobson says. "Others may develop a loss of appetite, irritability, or decreased activity, which usually lasts a short period of time." As with any medicine, there's a very small risk that complications may occur. However, rotavirus vaccine hasn't been shown to cause any serious problems. The risks from the vaccine are smaller than the risks from the disease. if you want to learn more about rotavirus vaccine, ask your clinician or nurse to suggest additional information.

Summited by Kelly Clark

˜™˜™] GUARDIAN CHILD OF THE MONTH ]˜™˜™]

My puppy Rocky and me

Well I really don't know where to begin. Let's see...I am nine years old and my name is Devin. I love a lot about my life and I love to watch football on TV. What I do not love is this. I have this problem called Hirschsprung Disease. I have had several surgeries, or operations. In fact, believe it or not, I lost count. Guess you could call me a 'pro', though I wish that I weren't. I've even have had several inches of my intestines taken out...inches that sure weren't helping me very much.

So here's what happened. I had 4 colostomies and 4 pull-through operations. These are different ways of arranging the intestines to allow them to rest and get better. I have been teased a lot because I have several BIG scars on my tummy. Lots of kids have said "boy that is really ugly on your tummy"........at first I cried a lot but them my parents just told me to look them in the eye and tell them that I am a very special little boy. That helped. Because of all my problems, I haven't grown like other kids my age. And my bottom gets very sore because I don't have much control with my bowel movements. If you think that is gross, imagine what it feels like to me. It's tough, but I can still have fun and still do pretty much everything that you can do.

 

 

Here I am with my little brother

The one thing I don't like about myself is that my tummy hurts a lot! I keep trying different medicines to make it feel better. And I go to the Doctor a lot, even though it's a very long ride from where I live in Iowa. I go to a very small school, and even though I have to miss a lot of days because I'm sick a lot, I still really like it. One time I missed over a month of school at one time.......it was scary because all the kids looked at me like I was different.....but I was the same person. I have to go to the nurse everyday to take meds and the kids alway ask why I have to leave and what I do at the nurse's station ......

It is scary and hard because I never know what I'm going to be teased about next. One time at school, I had a bad tummy ache and when it was time for recess, I had a friend who just walked around and sat with me because I just didn't feel good enough to play. Now that was nice

This is my brother Levi when he was really little

I have one brother who's 4 years old. He also has Hirschsprungs and he feels a lot like me. Except that he has to wear diapers because he can't feel when he has to have a bowel movement.

 

My mom and dad are thankful for having two little boys and are grateful for the happy and healthy days we do get to spend together. Well this is my story and I love this site because it makes me and my brother, and my mom and my dad not feel so alone with our medical problems. I hope that you're glad you came to visit and learn about my life.

Sincerely, Devin

Devin may be contacted through his parents Dave and Pam De Roon at pdderoon@mctcnet.net

Gerry McGregor brought this story to our attention when Devin's parents found our web pages and signed the guestbook. Text and pictures were from Devin's site All about Devin.....http://funrsc.fairfield.edu/~jfleitas/devin.html

credit Joan Fleitas, RN, EdD

Assistant Professor of Nursing, Fairfield University

Fairfield, Connecticut 06430

Joan Fleitas, RN, EdD

Assistant Professor of Nursing, Fairfield University

Fairfield, Connecticut 06430

 

 

 

 

 

 

 

 

 

]˜™˜™] 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 very special child Kyle Hall ]˜™˜™]

a special story written by his Kim Hall

I am a new member to the Guardian and would love to share my son's story with everyone.

Kyle's Story

Kyle was born on March 30, 1998, 5 weeks premature. He was a tiny little guy weighing in at 5lbs 4.8oz. I was so scared when I went into labour. I had been having some problems with my pregnancy and had fought really hard to keep Kyle in as long as I did. When he was born I was so scared that something would be wrong. When he came out he did not make a sound and I thought that he was not breathing. The doctor said that he was just fine. He was very content. He just came out looking all around. He appeared to be very healthy for being so early. I was so relieved. They brought him to me and I got to hold him for a few minutes before they took him away for observation. They said that this was the normal thing to do with premature babies. Kyle checked out fine except he needed a little help breathing. He only needed help for about 12 hours and then he was on his own. I was so excited because I was finally able to pick my baby up and hold him. The hard part was that it was time for me to leave the hospital and I had to leave my baby behind. I had never experienced pain like that before. My arms ached so bad for my baby. Kyle was my second child. My first son Austin was born healthy and came home with me. The comfort in all of this was that the hospital that Kyle was in was just a mile up the road so I could be with Kyle whenever I wanted and we hoped that he would only be there for about a week. We were told that all Kyle really needed to do was wake up, eat, and show that he could put on weight by himself. Kyle did not start off as the best eater. All he wanted to do was sleep. I was told that this was normal for a 35 weaker and he had jaundice, which made him even sleepier.

Finally, Kyle started eating. His patterns were very predictable. He would eat good in the morning, he was not as hungry in the afternoon, and by his evening feedings he did not want to eat at all. He appeared to be doing good though. He was finally starting to put back on some weight. We thought we might get to bring him home soon. On his 5th day in the neonatal unit he acted like his tummy was hurting him. I said something to the nurse thinking that maybe it was something that I ate that was coming through my breast milk and giving him gas. She said not to worry that he would adjust to my diet. I just thought okay my first baby had a lot of problems with gas so I guess my second one will be to. The next day Kyle really did not want to eat and when they would put food down his feeding tube he would vomit it back up. This bothered me but no one else really seemed concerned. This went on for a couple of days. I just thought maybe they were giving Kyle to much milk. My first baby did this and it was because I had enough milk to feed 4 babies. On Kyle's 8th day I was hoping to go for his 8:00am feeding and hear the good news that Kyle would be coming home instead it turned into one of the scariest days of my life. When I got their Kyle would not wake up and eat. This was not like Kyle. His 8:00am feeding was always his best. The nurse came over to check and see how he was doing and I told her that he would not wake up and eat. She checked him and his stomach was destined and when she checked his feeding tube yellow bile was coming out of it. She told me that she needed to call the NNP and have her examine Kyle. The NNP came over and looked at Kyle, ordered tests and called the doctor. I called my husband at work and told him that something was wrong with Kyle and that the doctor would be in at 9:00am to examine Kyle and that he needed to get to the hospital. When the doctor got there he told us that it would take about an hour for his tests and that he needed to talk to doctors at another hospital for us to go get breakfast and come back in an hour. That was one of the longest hours of my life. When we got back he told us that he thought from looking at Kyle's x-rays that he might have Narcotising Enterocolitis (NEC). They're looked like quite a bit of necrosis in his intestines and he needed to be moved to another hospital across town where a paediatric surgeon would be on hand if he needed immediate surgery.

We then moved to our second hospital. Kyle was 8 days old. When we got Many doctors examined their Kyle. Everyone had a different opinion. It was quite overwhelming. The surgeon said that he did not see anything on Kyle's x-rays that required immediate surgery. The neonatologist told us that Kyle's condition was very critical and that they would be monitoring Kyle very closely. Leaving the hospital that night was so hard. I was so confused. One doctor made it sound like Kyle was okay and another doctor made it sound like Kyle would be lucky if he made it through the night not to mention that Kyle was way across town and if he needed me I did not know if I could get to him in time. Kyle was placed on NEC watch for 10 days, no food, and was put on a central line and TPN. All of the doctors would make comments on how if you looked at Kyle's x-rays you would think man this kid is really sick but if you went over and looked at Kyle he looked so content. They could not believe it was the same kid. I remember the second day that Kyle was at Medical City a doctor came over and asked if Kyle had had a bowel movement and he had. Kyle had been having regular bowel movements since the day he was born. I thought that this was very odd. Why wouldn't he have a bowel movement? Doesn't everyone? Finally, Kyle's 10 days were up and his x-rays looked good. The necrosis seemed to be pretty much gone. We slowly got to start Kyle back on food. He started back very strong. He took back to breast-feeding with no problem and things appeared great except Kyle was not having bowel movements. I thought they if you took food away from me for 10 days I wouldn't have one either. So the nurse gave Kyle a little help and he started having some bowel movements again.

Kyle progressed up to full feedings and appeared to be doing great. The doctor told us that if Kyle had another good night that I could roomin with him and take him home. That night Kyle got sick again. He did not want to eat and he started vomiting again. I knew that something was not right. I told the neonatal doctor that was on call and she said he might have a lactose intolerance problem and we needed to take him off of breast milk for a few days and try a different formula. I thought that that was one of the dumbest things I had ever heard. I thought that a mother's milk was made for her baby. I spoke with a lactation consultant and she agreed. She said that the baby could have problems with the lactose in my diet but not with my milk. A few days went by and Kyle would not eat the formula that they put him on. Who could blame him. If it tasted as bad as it smelled I wouldn't touch it either. I spoken with another doctor and he agreed that we could put Kyle back on breast milk. He did think that it was a lactose problem. Since Kyle continued to still spit up he decided to have the surgeon look at Kyle again. The surgeon looked at Kyle and decided that it was probably a GI problem and called the GI doctors to look at Kyle. The GI doctor came and decided to do a rectal biopsy and check for HD. The first attempt failed. All she could get was poop. She ordered an enema to help clean Kyle out and had another doctor come back the next day to try again. He got a good sample he said and we had to wait the weekend for the results. The results came back inconclusive, he could have HD but he could not. There were no ganglion on the surface but they thought there might be some a little bit deeper in the lining of the intestinal wall. The GI doctors ordered for the surgeon to do a strip biopsy the next day. When our surgeon got there he wanted to go another route. He said that he had consulted with all of the doctors, radiologist, pathologists, neonatologist etc. and they were about 99% sure that Kyle had HD. He could do the strip biopsy but if he did it could ruin Kyle's chances for a pullthrough. He wanted to perform surgery on Kyle instead of the biopsy. He said that they could go in laparascopically, test to see if they found ganglion and if they did they would close up shop and be done. If they did not find any then they would remove the part of the intestine that did not have ganglion and would perform a pullthrough on Kyle. We did not want to do anything that might hurt Kyle's chances for a pullthrough so we agreed to the surgery. The next day Kyle was scheduled to move to his third hospital and to have surgery.

Kyle was one day shy of being 1 month old. The surgeon thought that Kyle's surgery would take about 2 - 2 1/2 hours. He thought that since Kyle had been having bowel movements and had been on full feedings that the segment without ganglion would be very short since this was not the normal pattern of an HD baby. On Kyle's x-rays you could see a change in size of Kyle's large intestine close to his anus. His large intestine was all the same size except for a few inches from his anus where it got smaller. They thought for sure that that was the affected area but they would not know for sure until they got in there. The moment that the anastetheolgist carried my baby away was the hardest moment of my life. I watched him carry my baby away to surgery and I did not know if he would ever be in my arms again. An hour into surgery we get a call saying everything is going good. Two hours into surgery we get a call saying they are doing his pullthrough. We were so relieved. Another hour goes by and we are wondering why hasn't the doctor been up to talk to us. Finally the phone rings and they tell us that they are still biopsying. We were so confused, we thought that they were done. Another hour goes by and they call saying that they still have not found any ganglion. By this time I am climbing the walls. Another hour goes by and they call and say that they have gone as far as they can go laparascopically and that they are going to have to open him up. At this moment I started to think that my baby was not going to make it out of surgery. Another hour goes by and we get a call saying that they have found ganglion and they will be able to perform a pullthrough on Kyle. We were so relieved and so thankful to our Lord for saving our sweet little baby boy. Our church had gathered and prayed so hard for Kyle at this time and their prayers were answered. I saw at this moment in time just how powerful prayer can be. An hour later the doctor came up and spoke to us. He explained that they had gone in to where the intestines changed size and cut it out and started a pullthrough when pathology called down and said that there are no ganglion, keep going. The doctor was shocked. He thought for sure that that was the affected area. The doctor did tell us for a moment he began to fear that they were not going to find ganglion. Kyle ended up losing half of his large intestine.

Kyle spent the first 5 weeks of his life in the hospital. Now that I look back I am so glad that he did. I believe that if he had come home that he would not have been diagnosed in time since he did not present like a typical HD baby. Kyle is now 5 months old. He is in very good health. He is such a loving, happy, content little baby. His smile and his dimples just melt my heart. To this today his biggest problem is his diaper rash. It looks like his bottom has been burned. We can not get it to clear up. Every time we think it is getting better it starts to flare up again. Kyle's prognosis for the future at this time is good. He is not out of the woods by any means. Our doctor tells us that it will be a few years before we really know how Kyle will be affected. We are just so blessed to have him in our lives and now we feel blessed to have this group in our lives. Thank you for forming this group.

Kim Hall (mom to Austin 2.5 yr. and Kyle 5 months, HD) kdh@fastlane.net

Kyle's picture can be seen at http://www.fortunecity.com/millenium/ratty/136/gallery.htm

 

]˜™˜™] IN THE MAIL ]˜™˜™]

Please sign me up to receive your newsletter. I think your organisation 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 advice from people with older Hirschsprung's children.

thank you!!

Carey Manders

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 still experiencing some physical ghosts because of Hirschsprung's. Please help!

Thank you.catherine stang <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 IIIb jejunal atresia, with the christmas tree deformity. Following the initial surgery to correct the atresia he retained approximately 80-100 cm 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. He continues to have severe motility disfunction, and has never been out of the 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. I had the same thing when I was young. My oldest now 4 had short segment hirschsprungs and did not need the colostomy. We opted not to have the surgery 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 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 cows udders

I use everytime and it does help but he goes so much it's 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 it's 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 visits to a paediatric 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 advice on possible medical intervention.

Thank you. Jocelyn and Steven Weiss Weiss@eisa.net.au

]˜™˜™] A QUESTION FROM A PARENT ]˜™˜™]

This was just sent to me - thought you all would like to see this.

love,

Sharon

From: Kajimery@aol.com

IF YOUR CHILD IS ON PROPULSID PLEASE PAY CLOSE ATTENTION TO THIS!!!

Not trying to scare the pants off of any one here but I learned something at a G.I. visit today that I am not sure any of us know. Apparently last week the FDA released a major warning (A NEW ONE) pertaining to Propulsid. Apparently the number of deaths connected to the use of this drug has grown tremendously and they are suggesting that children not be placed on it any more and those who are on it should be taken off a.s.a.p. If the child must be on thisdrug they NEED to have a E.C.G., regardless of how long they have already been on it without any problems. My Paediatric G.I. said that at Connecticut Children's Medical Centre they are no longer subscribing this medication to any of there patients.....ESPECIALLY PREMIES!!! These deaths have occurred, apparently connected only to propulsid. The victims were not on any other medications that would cause interference and had no history of heart problems whatsoever!!!

My doctor said that he is removing all of his patients from this med and that if it were his own child there would be no way he would take the risk...it is too great! Kind of freaked me out and I thought you all should hear this.

PLEASE TALK TO YOUR DOCTORS ABOUT THIS A.S.A.P. THIS STUDY WAS RELEASED LAST WEEK AND THESE ARE BRAND NEW STATISTICS. My ped said that by next year he does not think they will even be selling this drug anymore because the FDA will probably be pulling it from distribution.

My peds office will be posting a warning on there web site in the near future and as soon as it appears I will send you the link so you can read for yourself.

Very Scary Stuff!

-----------------------------------------------------------------------------------------------------------

Just thought you all would want this information.

^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^

Mom to Tyler, almost 4, and Dustin, 9 mons, severe reflux

and Reflux-induced Reactive Airway Disease.

sjordan@mail.sgc.peachnet.edu

^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^

 

 

]˜™˜™] A LITTLE THIS ]˜™˜™]

HEAVEN'S VERY SPECIAL CHILD

 

A MEETING WAS HELD QUITE FAR FROM EARTH! IT'S TIME AGAIN FOR ANOTHER BIRTH. SAID THE ANGLES TO THE LORD ABOVE,

"THIS CHILD WILL NEED MUCH LOVE.

"HIS PROGRESS MAY BE VERY SLOW.

"ACCOMPLISHMENT HE MAY NOT SHOW.

"AND HE'LL REQUIRE EXTRA CARE

"FROM THE FOLKS HE MEETS DOWN THERE.

"HE MAY NOT RUN OR LAUGH OR PLAY,

"HIS THOUGHTS MAY SEEM QUITE FAR AWAY.

"IN MANY WAYS HE WON'T ADAPT

"AND HE'LL BE KNOWN AS HANDICAPPED.

"SO LET'S BE CAREFUL WHERE HE'S SENT.

"WE WANT HIS LIFE TO BE CONTENT.

"PLEASE, LORD, FIND THE PARENTS WHO

"WILL DO A SPECIAL JOB FOR YOU.

"THEY WILL NOT REALIZE RIGHT AWAY

"THE LEADING ROLE THEY'RE ASKED TO PLAY.

"BUT WITH THIS CHILD SENT FROM ABOVE

"COMES STRONGER FAITH AND RICHER LOVE.

"AND SOON THEY'LL KNOW THE PRIVILEGE GIVEN

"IN CARING FOR THEIR GIFT FROM HEAVEN.

"THEIR PRECIOUS CHARGE SO MEEK AND MILD

"IS HEAVEN'S VERY SPECIAL CHILD."

 

by EDNA MASSIMILLA

 

]˜™] A LITTLE THAT ]˜™]

A boy's Christmas Present to His Mother

"It is better to give than to receive."

Bobby was getting cold sitting out in his back yard in the snow. Bobby didn't wear boots; he didn't like them and anyway he didn't own any. The thin sneakers he wore had a few holes in them and they did a poor job of keeping out the cold. Bobby had been in his backyard for about an hour already. And, try as he might, he could not come up with an idea for his mother's Christmas gift. He shook his head as he thought, "This is useless, even if I do come up with an idea, I don't have any money to spend." Ever since his father had passed away three years ago, the family of five had struggled. It wasn't because his mother didn't care, or try, there just never seemed to be enough. She worked nights at the hospital, but the small wage that she was earning could only be stretched so far. What the family lacked in money and material things, they more than made up for in love and family unity.

Bobby had two older and one younger sister, who ran the household in their mother's absence. All three of his sisters had already made beautiful gifts for there mother. Somehow it just wasn't fair. Here it was Christmas Eve already, and he had nothing. Wiping a tear from his eye, Bobby kicked the snow and started to walk down to the street where the shops and stores were. It wasn't easy being six without a father, especially when he needed a man to talk to. Bobby walked from shop to shop, looking into each decorated window. Everything seemed so beautiful and so out of reach. It was starting to get dark and Bobby reluctantly turned to walk home when suddenly his eyes caught the glimmer of the setting sun's rays reflecting off of something along the curb. He reached down and discovered a shiny dime. Never before has anyone felt so wealthy as Bobby felt at that moment. As he held his new found treasure, a warmth spread throughout his entire body and he walked into the first store he saw. His excitement quickly turned cold when the salesperson told him that he could not buy anything with only a dime. He saw a flower shop and went inside to wait in line. When the shop owner asked if he could help him, Bobby presented the dime and asked if he could buy one flower for his mother's Christmas gift. The shop owner looked at Bobby and his ten cent offering. Then he put his hand on Bobby's shoulder and said to him, "You just wait here and I'll see what I can do for you." As Bobby waited, he looked at the beautiful flowers and even though he was a boy, he could see why mothers and girls liked flowers. The sound of the door closing as the last customer left, jolted Bobby back to reality.

All alone in the shop, Bobby began to feel alone and afraid. Suddenly the shop owner came out and moved to the counter. There, before Bobby's eyes, lay twelve long stem, red roses, with leaves of green and tiny white flowers all tied together with a big silver bow. Bobby's heart sank as the owner picked them up and placed them gently into a long white box. "That will be ten cents young man," the shop owner said reaching out his hand for the dime. Slowly, Bobby moved his hand to give the man his dime. Could this be true? No one else would give him a thing for his dime! Sensing the boy's reluctance, the shop owner added, "I just happened to have some roses on sale for ten cents a dozen. Would you like them? This time Bobby did not hesitate, and when the man placed the long box into his hands, he knew it was true. Walking out the door that the owner was holding for Bobby, he heard the shop keeper say, "Merry Christmas, son. As he returned inside, the shopkeeper's wife walked out. "Who were you talking to back there and where are the roses you were fixing? Staring out the window, and blinking the tears from his own eyes, he replied, "A strange thing happened to me this morning. While I was setting up things to open the shop, I thought I heard a voice telling me to set aside a dozen of my best roses for a special gift. I wasn't sure at the time whether I had lost my mind or what, but I set them aside anyway. Then just a few minutes ago, a little boy came into the shop and wanted to buy a flower for his mother with one small dime. When I looked at him, I saw myself, many years ago. I too was a poor boy with nothing to buy my mother a Christmas gift. A bearded man, whom I never knew, stopped me on the street and told me that he wanted to give me ten dollars. When I saw that little boy tonight, I knew who that voice was, and I put together a dozen of my very best roses. The shop owner and his wife hugged each other tightly, and as they stepped out into the bitter cold air, they somehow didn't feel cold at all.

It truly is better to give than receive! It also is true that if one has received a great deal in life, as most of us have, that we also ought to give something back. Just as the shop owner gave because he had at one time received, we as Christians need to give in the same way. We have received the greatest gift imaginable!!! If that is true in our lives we also will want to share the gift of God's love and grace with others. Go share and rejoice!

 

Dear Lord,

I have been given so much and you have been so good to me. Give me

opportunities this day to give back to someone in need. Amen.

>˜™] PARENTING HUMOR ]˜™]

 

Dear Parents and Lovers of Children,

Here is some Parenting humour.

 

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, 819 College, Quincy, Illinois 62301

- 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 diarrhoea. 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 Centre 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 esophageal manometry, which are motility studies. I have had both of these myself. They don't hurt to have done, but they are uncomfortable.

AngelaMay (RainDance) 25, Chronic Intestinal Pseudo-Obstruction. Whos

currently busy working on writing out her whole tummy story.

lazlo@best.com

>ICQ# 1385068

* * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * *

* * * * * * * * *

 

 

Hirschsprung's Disease: Colostomy ?

Hirschsprung's disease (HD) or the absence of ganglion cells in the distal bowel has traditionally been managed as a three-stage procedure: diagnostic rectal biopsy, levelling colostomy where ganglion cells are present followed by a pull-through (PT) procedure later in life (six months to one year). Historic arguments considered in avoiding a primary neonatal PT were: increased mortality, the limited pelvic size, fragility of neonatal bowel, risk of pelvic nerve damage and injury to muscular sphincters. With the advent of better intensive care support, pathological expertise, adequate instrumentation and technical experience surgeons are managing HD as a single procedure early in life with identical results to the traditional approach. This entails early diagnosis with imaging and suction rectal biopsy, the use of rectal irrigation washout for decompression, and a PT procedure during the same hospitalisation. Overall, the open (and recently the laparoscopic) primary PT procedure has shortened the hospital stay, decreased morbidity (that associated with a colostomy) and produce earlier intestinal continuity. This can be accomplished during the first week of life when the weight of the infant is above the four kilograms. Colostomy in the setting of HD will then be needed for cases with: perforation, toxic megacolon, severe enterocolitis, questionable pathology, unavailable frozen section and in the premature infant.

PEDIATRIC SURGERY UPDATE *

VOL 11 NO 02 AUGUST 1998

References

1- Teitelbaum DH, Coran AG: Primary Pull-Through in the Newborn. Sem Pediatr

Surg 7(2): 103-107, 1998

1- Hackam DJ, Pearl RH, Superina RA: Singlestage repair of Hirschsprung's

disease: a comparison of 109 patients over 5 years. J Pediatr Surg

32(7):102831, 1997

2- Pierro A, Spitz L, Drake D, Kiely EM, Fasoli L: Staged pullthrough for

rectosigmoid Hirschsprung's disease is not safer than primary pullthrough. J

Pediatr Surg 32(3):5059, 1997

3- Wilcox DT, Bianchi A, Bowen J, Bruce J: Onestage neonatal pullthrough to

treat Hirschsprung's disease. J Pediatr Surg 32(2):2435, 1997

4- Nour S, Stringer MD, Beck J: Colostomy complications in infants and

children. Ann R Coll Surg Engl 78(6):52630, 1996

5- Langer JC, Lau GY, Ternberg JL, Skinner MA, Foglia RP, Srinathan SK,

Winthrop AL, Fitzgerald PG: Onestage versus twostage Soave pullthrough for

Hirschsprung's disease in the first year of life. J Pediatr Surg 31(1):336,

1996

6- Cilley RE, Coran AG, Hirschl RB, Statter MB: Definitive treatment of

Hirschsprung's disease in the newborn with a onestage procedure. Surgery

115(5):5516, 1994

7- Rescorla FJ, Grosfeld JL, West KW, Engles D, Morrison AM: Hirschsprung's

disease. Evaluation of mortality and longterm function in 260 cases. Arch Surg

127(8):93441, 1992

]˜™] QUESTIONS FROM OUR ONLINE PARENTS ]˜™]

 

Pullthru Questions

My son Austin has short segement HD. When they do his pull thorough surgery will they close his colostomy at the same time or is that a separate procedure? I had assumed that it was all done at the same time, but an article by Dr. Pena made me wonder if I was wrong. I was not sure if pull throughs for HD were different than other disorders. Also, where are the incisions most commonly made. I had thought that it would be through his abdomen, but the readings also made me realise that I might be wrong. You can see that I wasn't sure exactly what to ask my surgeon last time we talked. After doing more research, I will have tons of questions for him

now! I appreciate being able to get some advice from all of you experts out

there!!

Christa, mom of Samantha and Austin (HD) rgreene@ipass.net

This question is commonly posed to our group and as usual parents were filled with answers, and filled christa in on what may or may not happen. here are some of the replies

Christa,

They can do it either way. I suppose it depends on the surgeon. When Matthew went in for his pull through his surgeon was doubtful that he would be able to do the pull through and reversal at the same time mainly because he was doing the procedure by lap and didn't want to leave Matthew under any longer than he had to. He said that he would rather do the pull through, give Matthew time to heal, and then go back and do the reversal. When he came to talk to us after the surgery was over he told us that things went so well he decided to go ahead and do the reversal.

Matthew's scaring was very minimal. He has a 3 inch scar on his lower abdomen where his stoma was and 3 very small "pox mark" scars where they placed the scopes for the surgery.

Hope this helps,

Jen

pooh@glasscity.net our parent Liason

I believe it will be a separate operation, about 2 weeks later. This gives the pull thru site a chance to heal before stool comes thru. At least this was how it was with my son and his ileostomy. I didn't know about this and assumed it would be reversed at the same time as the pull thru. Boy was I surprised when I looked after the surgery and the darned thing was still there! I didn't have any more ostomy bags left so I had to run out and buy more! Yikes!

Martha FLetcher, Randallstown, Md

mrfletcher@home.com

Christa

As has been said by others it can be done in two stages or one. When I had my pullthrough in January it was done in two stages. I had my rectum and part of my colon removed and a Ilestomsty put in place. If you have not heard of that then it is like a colostomy but higher up the colon. A colostomy general comes through the lower part of the body. I had it for 3 months while my colon healed and then it was reversed and my bowls hesitantly started to work again. You may of heard me speak of John. He is a good friend of mine and has HD also. He had a colostomy and then had the pullthorugh and stoma removed at the same time. His scars like Matthew's are very small and is now a very healthy one year old.

Please ask us as many questions as you want.

Gerry McGregor

Adult 46 with HD

ICQ 2889223

http://www.bigfoot.com/~gerry.mcgregor

]˜™˜™] A FATHER'S VIEW OF HIRSCHSPRUNG'S DISEASE ]˜™˜™]

Hi, my name is Tim Beehler and I'm a 25 year old male with HD. I am married to a wonderful woman by the name of Jen that is my better 3/4 (better half would be an understatement) and I have two terrific sons, Andrew will be three in November and Matthew who also has HD will be two in January. I am going to try and give you a father's view from a different perspective. Almost all of the cases I have heard about deal with a parent or a child that have HD, not both. So I am going to try my best to give you a quick rundown of my life (in a nutshell) with HD and the thoughts and fears I have having a child with HD. Be patient if I get long winded.

I was born in Toledo Hospital on January 3,1972, 6 pounds and never passed stool. I was released between day 6 and day 9 (not too sure which, as I was kind of small then) and still had not passed stool and would not eat. My mom told me all I would do was act very lazy, sleep a lot and I would throw up stool. The doctors told my mom "Mrs. Beehler, your son can not be throwing up stool." which is just what they told Jen the day after Matthew was born. My mom took me to the emergency room when I was 10 days old and they told her I was "failure to thrive" and I needed to go to surgery right away. I had a colostomy put on and needed it changed 15 to 20 times a day because I had very bad gas. Compared to now where I just have bad gas.

This went on for 10 months until I had my pullthrough. After that there were no more bags, but I was not able to stay regular. Between 1 year and 5 years I don't remember a lot except going to the doctor and knowing what the rubber glove meant and being able to describe what a fleet enema looks like with my eyes closed. I think that the main reason for most of my problems was procedures done at that time were just being tested and equipment was not modern enough. I have a scar about 12 inches long just below my waist line and when they did the pull threw they used staples to form and shape the intestines the way that they needed to be. My dad told me that it looked just like a large zipper. I have had all of my large intestines removed and I'm not sure about the rest.

Growing up through school years was by far the worst thing in my life so far. If the rest of you or your children have bad gas this is what caused me more trouble than HD itself did. First off, I'm not a very big guy. I don't know if this has to do with HD but I'm sure it played a part. Second, as a child your control of your body functions are not as great as you would like them so it is very easy to be in the middle of a room and squeak out even a little fart that could clear the room for 4 or 5 minutes easy. So as you can guess kids can be mean and boy were they. I began skipping school because I did not want to deal with it and as a result dropped out in the middle of the 9th grade. Now don't get me wrong, this was not all HD related and there is a lot of years I'm not writing about, but HD is what started the habits so I put it down. I am not a stupid person as a result of dropping out. I did go and get my GED for MYSELF and no one else. I am currently an executive for Target (discount retail store) and make a good living and have a wonderful family. So, although it sounds like I had a terrible childhood you can't judge a book by it cover and don't think from reading this that your kids are going to drop out of school and be nothing. I'm telling you my experiences so you know what you may be in for and can be proactive in positively impacting your child's life.

OK, enough about me now on to Matthew. Matthew was born on January 28, 1996, 6 pounds, 9 ounces and never passed his first stool. On the second day Jen noticed that they had not put down on the chart that he had any dirty diapers and asked if they forgot to put it down in the nursery. They told her that he had not gone yet and that that was normal not to worry. Yea right. He was not eating well at all and anything that he would get down would come right back up. This is where Jen told them that she thought that it was stool and that I had HD. Now at this time the nurse has a concerned look on her face, because she don't know what it is and tells us, "Mrs. Beehler, your son can not be throwing up stool." Well he was and when they finally did a rectal biopsy they found out it was HD. Imagine that! HD! What would give them that idea?

OK so here is what they did to Matt:

• Day 3 to 7 - Blood work, rectal biopsy, abdominal x-rays, put him in NICU and put him on feeding tube, IVs, pump to keep him empty, catheter, and some other stuff my wife would know.

• Day 7 - They sent him home and in about 4 hours we brought him right back in and readmitted him to the NICU (we were supposed to do irrigations at home to keep him empty. We were doing them at the hospital but they still were feeding him with IV's and fat.)

• Day 8 - Prep for surgery

• Day 9 - Surgery - Had colostomy put on above the damaged intestines - he was in NICU for 28 days until they release him. (We, I mean Jen did most of the bags in the hospital) He was released with his bag and for the next 6 months we did the yellow and blue makes green thing with his stoma and bags (aren't blowouts fun:-)).

• 6 Months - Did his pullthrough via scope, reversed colostomy, make new storage pouch for stool out of large HD intestines, remove appendix. Since then we got him home on 4 irrigations a day at 500 cc a time (50cc x 10 washouts). It was moved down to 3 a day same way and was just moved to 2 times a day same way but he just got sick and they made us go back to 2 times a day in the same way. We continue to have some problems with him but in my opinion nothing bad.

Any questions about Matthew e-mail my wife Jen at Pooh@glasscity.net and she can help you more. The first thing I did when we were waiting for results on the rectal biopsy was go down alone to the chapel in the hospital and cry. I blamed myself because I had it. And what did he do to deserve the hell and torture that was ahead of him. My biggest concern when I found out that Matthew had HD was that everything I went thought was in store for him. THAT'S JUST NOT SO. The abilities of the doctors are getting better everyday and with support groups like this one our kids could not have picked a better time to be born. I hope this helps someone understand something, I'm not too sure what but you can figure it out for yourself. Just remember the biggest part of our kids is their heart and no doctor can take that part of them away.

Tim Beehler 25/HD, Father of Matthew 2/HD

Guardian Society thanks Tim for this difficult article, and thanks him and his wife for being Parent Advisors, and Regional Support Parents for Ohio

 

 

]˜™] INTESTINAL MALROTATION ]˜™]

INTESTINAL MALROTATION

Intestinal malrotation is most often diagnosed in a neonate in whom the sudden onset of bilious emesis heralds the development of a midgut volvulus or duodenal obstruction from Ladd's bands; less commonly, the abnormality may be recognised in an older child or adult who presents with non-specific abdominal complaints.

Malrotation represents a defect in embryogenesis which occurs at the tenth week of gestation, as the intestines migrate back into the abdominal cavity following their extension into the umbilical cord. Under normal circumstances, the herniated intestinal loop rotates 90 degrees in a counterclockwise direction around the superior mesenteric artery so that the small intestinal segment lies to the right while the large intestinal segment lies to the left. Following migration back into the abdominal cavity, an additional 180 degree counterclockwise rotation occurs, with the small intestinal segment passing posterior to the superior mesentery artery. Elongation of what will form the ascending colon pushes the cecum, which is initially in the right upper quadrant, into the lower quadrant. Following fusion of the mesentery with the peritoneum, the ascending colon and most of the duodenum become retroperitoneal structures. The small intestines become attached to the posterior abdominal wall by a mesentery which passes from the ligament of Treitz to the ileocecal area.

In non- rotation, no additional rotation occurs following migration of the intestines into the abdominal cavity; the small bowel remains on the right side of the abdomen while the colon lies on the left. Although volvulus may rarely occur, many patients with non- rotation are asymptomatic. Malrotation also results from incomplete rotation of the bowel. The cecum remains in the right upper quadrant adjacent to the duodenum; its bands of attachment to the abdominal wall may overlie and obstruct the duodenum. In addition, the duodenal-jejunal loop remains in the right side of the abdomen instead of extending to the ligament of Treitz in the left upper quadrant; the mesenteric attachment of the small intestine is therefore very narrow and subject to torsion with obstruction of arterial flow and necrosis of the entire midgut.

The true incidence of malrotation is difficult to estimate because some patients remain undiagnosed. The abnormality is most often recognised in infancy: 40-50% of patients present with symptoms within the first week of life, 64% by one month of age, and nearly 90% by one year of age.

Among those presenting in infancy with volvulus or duodenal obstruction, bilious emesis is the most common complaint. Abdominal distension, colicky abdominal pain, and bloody stools may also be noted at presentation. Sepsis and shock often ensue. Among older children, nonspecific symptoms such as chronic abdominal pain, intermittent vomiting, diarrhoea, constipation, and failure to thrive are presenting features.

Associated congenital anomalies are common, occurring in 70% of children with intestinal malrotation. Duodenal abnormalities, including atresia, stenosis, or web, occur in 11% of patients. Other associated anomalies include intestinal atresia, Meckel's diverticulum, intussusception, omphalocele, gastroschisis, diaphragmatic hernia, and Hirschsprung's disease.

Also associated with malrotation are non- gastrointestinal abnormalities. The polysplenia syndrome, which appears to follow a teratogenic insult at about five weeks gestation, consists of polysplenia, intestinal malrotation, a symmetric bilobed liver, biliary atresia, cardiac malformations, and several vascular abnormalities. The syndrome is felt to represent a defect in the development of "sidedness," with left-sided structures duplicated bilaterally and right-sided structures absent. A similar defect in laterality with development of bilateral right-sided structures, asplenia, and intestinal malrotation also exists.

In patients with malrotation, radiographic findings on plain abdominal films include the "double bubble" sign of duodenal obstruction, distension of the stomach with scant intraluminal gas distally, and generalised distension of small intestinal loops. Barium enema may demonstrate an abnormally high cecum; however, this alone is not diagnostic since the cecum may be mobile and in a relatively high position in a normal child.

Upper gastrointestinal series in the patient with malrotation will demonstrate failure of the proximal jejunum to extend to the left upper quadrant. If volvulus is present, additional findings may include abrupt termination of the dilated duodenum in a sharp beak-like shape; a "corkscrew" appearance of the duodenum; and thickening of intestinal folds because of venous and lymphatic obstruction.

In cases of suspected volvulus, emergent laparotomy is indicated with detorsion of the volvulus and resection of obviously necrotic areas. Questionably viable segments may be replaced in the abdomen only to be reexamined in 24 to 36 hours at a second laparotomy and resected if necessary. Correction of the malrotation begins with resection of Ladd's bands, which attach the colon to the abdominal wall and may cross over and obstruct the duodenum. A second set of bands attaching the right colon to the duodenum is then resected, permitting the cecum and ascending colon to move to the left of the midline while the small intestine remains on the right. The midgut now has a much broader base of attachment which is unlikely to undergo torsion. A Foley catheter is passed into the jejunum, the balloon inflated, the catheter withdrawn to detect intrinsic duodenal obstruction. Finally, an appendectomy is performed on all patients.

The management of the patient with incidentally discovered malrotation is less clearcut. However, because of the risk of volvulus and the likelihood that the symptoms leading to the diagnostic study may be related to the malrotation, many surgeons

favour elective repair of this population of patients with malrotation.

Among symptomatic neonates who undergo prompt surgical repair without resection, gastrointestinal symptoms generally resolve completely. The postoperative result may be less satisfactory among older children, in whom non-specific symptoms may persist even after surgical repair. The mortality rate for volvulus is less than 5%, a figure which has decreased as postoperative nutritional support has improved. However, mortality is much higher among patients who undergo resection of necrotic bowel. Long-term sequelae include short gut syndrome, feeding difficulties, sepsis from intravenous catheters, and subsequent small bowel obstruction from adhesions. Recurrent volvulus is rare.

REFERENCES

Coombs R. Intestinal malrotation: the role of small intestinal dysmotility in the cause of persistent symptoms. J Ped Surg 26:553, 1991.

Ford E. Malrotation of the intestine in children. Ann Surg 215:172, 1992.

Groff D. "Malrotation." In Ashcraft and Holder, Eds., Paediatric Surgery. Philadelphia: W.B. Saunders Company, 1993.

Karrer F. Biliary atresia and the polysplenia syndrome. J Ped Surg. 26:524, 1991.

Moore K. The Developing Human. Philadelphia: W.B. Saunders Company, 1982.

Powell D. Malrotation of the intestines in children: the effect of age on presentation and therapy. J Ped Surg 24:777, 1989.

Wesson D. "The Intestines: Congenital Anomalies." In Walker et. al., Eds., Paediatric Gastrointestinal Disease. Philadelphia: B.C. Decker Inc., 1991.

Yanez R. Intestinal malrotation presenting outside the neonatal period. Arch Disease in Childhood 61:682, 1986.

 

]˜™˜™] Anadotes ]˜™˜™]

Smile

She smiled at a sorrowful stranger. The smile seemed to make him feel better. He remembered past kindness of a friend And wrote him a thank you letter. The friend was so pleased with the thank you That he left a large tip after lunch. The waitress, surprised by the size of the tip, Bet the whole thing on a hunch. The next day she picked up her winnings, And gave part to a man on the street. The man on the street was grateful; For two days he'd had nothing to eat. After he finished his dinner, He left for his small dingy room. He didn't know at that moment that he might be facing his doom. On the way he picked up a shivering puppy And took him home to get warm. The puppy was very grateful To be in out of the storm. That night the house caught on fire. The puppy barked the alarm. He barked till he woke the whole household And saved everybody from harm. One of the boys that he rescued Grew up to be President. All this because of a simple smile That hadn't cost a cent

Out of the mouth of Babes

Jennifer Beehler

 

On a lighter note, I have a funny story for everyone! But first, a brief background for those of you that are new. I have a 2 year old son that has total colonic HD. After his pull through at 6 months he suffered from a few bouts with enterocolitis. His doctors decided that putting him on a rectal irrigation schedule would be the best way to keep this to a minimal. I am happy to say that we are finally almost done with them as Matthew is potty training and hopefully off of all irrigations soon! Anyway, as I was doing an irrigation on Matt tonight ( first one in 3 days!!!) my 3 year old was running around and playing as usual. When I was half way through the irrigation he came up and said to me: " Mommy, you have to put the water in and make Matt grow just like me and Daddy do my plants!!" Kids seem to come up with the funniest things..LOL

Love to all,

Jen Beehler

The Beehlers,

Tim/26-HD, Jen/25, Andrew/3, Matthew/2-Total HD

Pooh@glasscity.net

 

]˜™˜™] NOTICE ]˜™˜™]

This newsletter of the Guardian society is intended to report items of interest with regard to Hirschsprung's disease, and other motility disorders. We do NOT promote or recommend any therapy, treatment, etc. The family should discuss anything printed in this newsletter and its relevance to a particular person with their own physicians. Our hope is that this method of communication will promote interaction and the exchange of information between parents and foster support among families.

All contributions to The Guardian become the property of The Guardian Society and may be edited or condensed at the discretion of the Editorial Staff (with the exception of published works).

All rights reserved.