1 Dedication: This issue is dedicated to Sean Dooley, Daniel Black, Tyler Siefert , Noah Fisher, Alex Cooper, Gregory Shearon , Bailey Ralston, Amy Ebeling, Keegan James Kearns-Davidson, Alexander James Staudt, and Their Families
2 FROM THE EDITOR: all about the Robinstein family and updates on the Society
3 GUARDIAN LISTSERVER NEWS UPDATES: All about the Guardian Society
3 DIAGNOSTIC TESTING SERIES New 6 part series on GI Diagnostic testing this is part one of 6 "Sigmoidoscopy" by the National Digestive Diseases Information Clearinghouse
4 STOP THE MADNESS: Help the Children this months topic children who need transplants and those who have lost their lives waiting.
5 WEB LINKS: Guardian has a new web address, and new home address as well Listsever,news and much more
6 MEMBERS PAGES Meet our online members through their web pages
7 GUARDIAN CHILD OF THE MONTH: Alexis a success story
8 A CHILD'S VIEW: Jennifer's Q & A by our star Reporter Jennifer Sparks
9. A VERY SPECIAL FAMILY. Call for awareness of Organ Donation.
10. PARENT TO PARENT Parents ask and get responses from other parents who have been there
11. A LITTLE THIS, A LITTLE THAT: , Parenting humor
12 PICNIC TIME Join us for some special time at the third annual Guardian Society's Picnic.
13. HIRSCHSPRUNG'S DISEASE , A Special Article by our child of the month Alexis .
14. Poems For Chloe: Poems from a mothers heart to a special child
15 GOT STORIES calling all writers
16. NOTICE: Please Do NOT Forget to Read Our Disclaimer
]˜™˜™] Dedication ]˜™˜™]
This edition of The Guardian is dedicated to Sean Dooley, Tyler Siefert, Daniel Black, Noah Fisher, Alex Cooper, Gregory Shearon, Bailey Ralston, Amy Ebeling, Keegan James Kearns-Davidson, Alexander James Staudt, 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,
Wow its been a few months again I am sorry but its been very exciting for our family with many new changes to my family, as online parents know in march 1999 after many failures terran finally had an Illestomy placed, this has been a huge success in his life and has allowed him some peace in the Hirschsprungs front, as some know terran has suffered from apnea as well for all his life in march of this year as well he was taken off his CPAP and placed on oxygen (like I need more equipment to take around,) bit its made a huge difference in his apnea. We have moved, in may of this year my husband lost his job of 7 years so we decided to up and move from our small town to a bigger one in hopes of finding a better life for our family, we now live in the small community of Land O Lakes Florida and for the first time we own our own home, so its very exciting for us, I have completed updating terrans pages on the internet as promised the address of our families web pages is http://www.hometown.aol.om/trobinstei/terran.html
Kimberly Robinstein, President Guardian Support Group guardian@sundragon.net
]˜™˜™] GUARDIAN LIST-SERVER NEWS]˜™˜™]
Wow the internet has been a buzz, we now have 175 members on the list-server, many of whom are very active in helping others supporting each other or just having fun. look below to find out how to join in on the fun.
]˜™˜™] DIAGNOSTIC TESTING SERIES ]˜™˜™]
Sigmoidoscopy
Sigmoidoscopy (SIG-moy-DAH-skuh-pee) enables the
physician to look at the inside of the large intestine from the rectum
through the last part of the colon, 
called
the sigmoid colon. Physicians may use this procedure to find the cause of
diarrhea, abdominal pain, or constipation. They also use sigmoidoscopy to
look for early signs of cancer in the colon and rectum. With
sigmoidoscopy, the physician can see bleeding, inflammation, abnormal
growths, and ulcers.
For the procedure, you will lie on your left side on the examining table. The physician will insert a short, flexible, lighted tube into your rectum and slowly guide it into your colon. The tube is called a sigmoidoscope (sig-MOY-duh-skope). The scope transmits an image of the inside of the rectum and colon, so the physician can carefully examine the lining of these organs. The scope also blows air into these organs, which inflates them and helps the physician see better.
If anything unusual is in your rectum or colon, like a polyp or inflamed tissue, the physician can remove a piece of it using instruments inserted into the scope. The physician will send that piece of tissue (biopsy) to the lab for testing.
Bleeding and puncture of the colon are possible complications of sigmoidoscopy. However, such complications are uncommon.
Sigmoidoscopy takes 10 to 20 minutes. During the procedure, you might feel pressure and slight cramping in your lower abdomen. You will feel better afterwards when the air leaves your colon.
Preparation
The colon and rectum must be completely empty for sigmoidoscopy to be thorough and safe, so the physician will probably tell you to drink only clear liquids for 12 to 24 hours beforehand. A liquid diet means fat-free bouillon or broth, Jell-O®, strained fruit juice, water, plain coffee, plain tea, or diet soda. The night before or right before the procedure, you may also be given an enema, which is a liquid solution that washes out the intestines. Your physician may give you other special instructions
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. Department of Health and Human Services. 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. 98-4332
June 1998
e-text posted: 7 July 1998
this is a part of a new series on GI testing this is part 1 of 5
| other diagnostic tests: to be featured in the future | |
]˜™˜™] STOP THE MADNESS ]˜™˜™]
Please help the children.
"If only for someone to hold my hand in my time of need to sit and chat and let me cry that's my prayer tonight."
What's the 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. You may notice some new additions to our dedications section as guardian grows so does this list. Its so sad in this day and age children still can succumb to the devastating effects of Hirschsprung's its for this reason we are helping to raise awareness of the devastating effects of not only Hirschsprung's but of many other motility disorders as well.
This month we expand the campaign to cover and urgent need some of our more severe hirshsprungs and short bowel children who are on TPN Total Parental Nutrition, and in need organ transplants Children who need our help before time runs out. see the Ebeling's story below for more information .
I often cruise the net in search of more parents who need help though their web pages I find some interesting stories but also get my heart broken such was the night of July 20 Th 1999 and I happened on the sad story of Keegan Kearns-Davidson http://www.hwcn.org/~keegan/ and once again my education began, a story of a mothers love and dedication to a beautiful child and her attempts at securing her child's much needed organs, I was so touched I immediately sent the web pages to our list hoping against hope that we may be able to help at least with support, much to my sadness time ran out For little keegan he died in his mothers arms April 1 1999 at 12:15 am, my attention then turned to why and what can we do to help others who are on TPN due to Hirschsprungs or other motility disorders like short bowel and such, I posted the sad news of keegans death to the group unbeknown to me one of our members Bea Vannan mom of one of our total colon children and who is also on TPN informed us that she had met the family of keegan and found them a most loving family despite such tragedy, Its my hope that some one out there reads this newsletter and maybe just maybe we can help a child who needs it. By wearing a burgundy ribbon. and telling of our children For our children please help.
Please get involved! Visit Multiple Organ Retrieval and Exchange Program of Ontario http://www.transplant-ontario.org/ WebPages or phone: 1-800-263-2833 for more information
to learn more about organ donation and transplantation and how you can help promote organ donation visit Visit TransWeb at http://www.transweb.org/
Please add Louis S Robert Ebeling kebeling@lindsaycomp.on.ca to your prayer list as his name is placed on the organ transplant List August 1999 he is in need of a multiple organ transplant
˜™˜™] Web Links ]˜™˜™]
Guardians new web page site is up and running http://www.theguardiansociety.org Gerry Mc Gregor updates this page this web page is updated regularly so check back and see what's new
Our Listsever is one of the most popular in the internet email us today 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)you can also read messages by us and join in on conversations by logging on at http://www.egroups.com/group/guardiansociety
]˜™˜™] Guardian Society New address]˜™˜™]
Guardian Society
]˜™˜™] Members Web Pages ]˜™˜™]
Meet the Bohlmanns Karl and Tangi parents to Kaily and Ryley they live in Tarpon Springs Florida http://home1.gte.net/bohlmann/thebohlmanns.html
Meet the Forney's Heidi and her husband and Kaj, Joseph, and Sean Patrick Forney. Heidi is very active in CHERUBS- The Association of CongenitalDiaphragmatic Hernia Research, Advocacy, and Support
http://www.fortunecity.com/millenium/lassie/717/
Meet Gerry Mc Gregor who lives in Milton Keynes in the United Kingdom. He has 4 teenage kids. Kris, my son is 20. Emma is 18, Michelle 15, Claire is also 15.
http://www.fortunecity.com/wembley/roker/134/index.html
More Member web pages in a future issue
]˜™˜™] GUARDIAN CHILD OF THE MONTH ]˜™˜™]
The Story of Alexis
Our daughter, Alexis (now 12 yr... old), was born with
Hirschsprung's 
Disease
in May 1986. It was missed at birth by our family doctor, and not
accurately diagnosed until she was 6 1/2 months. old.
I was extremely conscientious during my pregnancy in regards to diet and activity to ensure a healthy baby. I thoroughly enjoyed this planned pregnancy and everything seemed to be by the book, including natural delivery right on the due date. The baby weighed 5 lbs..., 14 oz. at birth, but I was only 120 lbs... at conception and my husband is not extremely large, so we didn't consider it low birth weight.
I wanted to breastfeed the baby, but she didn't seem too responsive. Being a first-time mother, I was nervous and unsure of my mothering capabilities, but figured that was normal and it would all come to me, as all my experienced friends encouraged. With things not going well, the doctor chalked it up to insecurity and allowed an extra day's stay at the hospital for me to adjust to my new baby with the maternity nurses' help.
Well, we came home and things got worse. I gave up breastfeeding within 2 days. I was so unsure and scared the baby was not getting enough nutrition since she did not seem interested in nursing, so I called the doctor and put her on the recommended formula and bottle. That way at least I knew how much she drank, if at all. She would throw up so much I couldn't imagine she kept any in her system. It would take an hour to get 1 oz. out of the bottle, and most of that was on her tee shirt from her pushing the nipple out of her mouth with her tongue!
I noticed the baby did not have a bowel movement at all the first week we were home, and reported it to the doctor. He thought that was okay. Then when she started passing stool, she eliminated continually for two days - kind of diarrhea consistency. I kept taking her into the doctor every few days for weight checks because I felt all along something was not quite right, even though the baby was very complaisant and did not fuss much at all. I felt there was a problem, but the doctor disagreed, was getting irritated with me, and thought I was just an over-anxious new mother. I remember him even making the sarcastic comment that he'll have a stronger bond with this baby than her father for all the times I bring her to see him.
At four weeks old, I noticed the baby would strain tremendously without producing a bowel movement. Luckily when I showed up at the doctor's office for a weight check, our doctor was not there and I told the nurse of the terrible straining and that the baby had what looked like hemorrhoids. She checked and immediately called in the partner doctor, who in turn put us in touch with specialists at Polyclinic Medical Center in Harrisburg, Penn. The next day the surgeons examined her and were 99% sure she had Hirschsprung's. We were devastated and didn't want to believe them. When the pathology report came back, it did not show Hirschsprung's and we were so relieved. However, the surgeon (Dr. Alvear) felt this was a Hirschsprung's case and asked us to return her in a few weeks for further testing. In the meantime, he was treating her for an anal stenosis. So we returned her for testing at 3 mos. of age, but it proved nothing.
I didn't understand the surgeon at the time - he was using medical jargon unfamiliar to me - but in hindsight, I know now that he wanted to do a minor rectal operation so he could more conclusively rule out or diagnose Hirschsprung's. At that point, I had to return to work from maternity leave and was willing to accept the advice of my local doctor who thought she just needed stool softeners until she grows more, and didn't allow the surgery.
Although improved by the anal stretching, we continued to experience bowel problems, alternate diarrhea and constipation. We'd help her complete elimination with gentle use of a rectal thermometer or suppositories. (I was going nuts -- carried hard marble-like samples of her waste around in a jar in my purse to show and ask my friends if their babies ever have similar bowel movements - I was so desperate for help!) One day I was in tears at my doctor's office - I told him I can reasonably handle things but I'm having a lot of difficulty with this baby. I had an overwhelming feeling either I wasn't capable of caring for her or something was still wrong with her. My husband was just as frustrated too, not to mention we were spending hundreds of dollars in doctor visits without much satisfaction.
At that time, there were only 2 pediatricians in our small town and I couldn't get in to see either of them. We asked our family doctor to refer us to a specialist in babies, but he wouldn't because he felt he was handling her satisfactorily. Finally one night when she was about 5 mos. old, we rushed her to the emergency room at the hospital with a temperature of 105. They treated her for an ear infection and wanted to call in our doctor. My husband caused a major scene and said we need someone else with an unbiased opinion to look at our child. At that point, this forced our way into a pediatrician’s practice. However, he was so booked up he wouldn't be able to see her for the initial visit for about 6 weeks. So I said, what do I do in the meantime because I know she'll be sick before then? Our family doctor reluctantly said he'd keep her on his roster until the transition, but he was disgruntled at us and commented to me he felt he got her through her problems and now another doctor will have the easy pleasurable moments in watching her grow. I was so confused and upset and didn't know where to turn or whom to trust. The doubt and uncertainty caused tension in our marriage - which was the last thing we needed to have happen in a crisis.
At 6 mos., I started solids. Inevitably, she became ill prior to the pediatrician’s initial visit and I had to take her to the family doctor's office on a Saturday. Another partner was on call. I can't say for sure, but I don't think our records were available since we were being transferred, but I did inform the doctor on call about suspected Hirschsprung's. Alexis was violently throwing up bile. This doctor was concerned and had us stop by the hospital lab for blood work, and sent us home. She got the lab results and said it may be just a stomach flu and recommended Pedialyte, but said to call the office first thing Monday if things didn't improve.
Alexis did not improve. On Monday morning, we noticed her stomach was swelled so large I could hardly close her sleeper. I called the doctor's office around 7 a.m. and was told to call for an appointment when the office opened. By this time, I suspect they were very tired of dealing with me and were rather rude. They commented they have other patients to see too and gave us a later appointment. So my mother and I took the baby directly in to the doctor's office to wait (we were an hour early for our appointment time). When they saw us come into the waiting room and that doctor saw our sickly baby, he knew there was big trouble and gave her immediate attention. He sent her directly to the ER where they worked on her for about two hours, and for the first time, the pediatrician saw her.
Upon my suggestion, she was transported by ambulance to Harrisburg's Polyclinic Medical Center for Dr. Alvear's attention. He unfortunately was out of town that afternoon, but the staff there gave her superb care until he could arrive. She was so unstable he had to wait until the next day to do emergency surgery. Everything but her heart had shut down, she had terrible convulsions and was hooked up to a respirator in intensive care for a week. She had the bowel blockage removed, received an ileostomy and had the Hirschsprung's disease confirmed. There were tubes in every opening of her little 12-lb.. body and monitors and IV's attached everywhere else. She looked as if she had been in a horrific auto accident. Days went by before we had much hope. She amazingly recuperated, and about 2 mos. later, had the area of intestines with no ganglion cells removed, and about 2 mos. after that had the ileostomy reversed and was surgically corrected. Thank God for Dr. Alvear saving her life!
Oddly
enough, we had friends whose son (born in 1983) had HD. Additionally, at a
family reunion, we discovered an unknown cousin with HD, and then in 1988
another local child was born with it. In doing genealogical research, I
was able to link all four children to common ancestors generations back
into the 1700 & 1800's. Univ.. of Pitts. Drew blood samples from these
4 kids and relatives to study similarities in their DNA, but to my
knowledge nothing was concluded from them.
I have tried to educate family members (and others) of this condition and possibly spare someone some of the turmoil we experienced with our child. Alexis is very fortunate and I think she has little repercussions from her terrible ordeal since she was so young when it all happened. Today she is a happy normal 12 year old girl.Thanks for reading our story.
Lori, mom to Alexis (12 yr.., HD) & Emily (7 yr..), wife to Daniel (17 yr..)
]˜™˜™] A CHILD'S VIEW ]˜™˜™]
By Jennifer Sparks
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.
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
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 family The Ebelings ]˜™˜™]
Please Give The Gift Of Life !!!
On Nov. 9/90 a beautiful little girl was born. I named her Amy Catherine. She was perfect, she had all her fingers and toes and was the most beautiful thing I had ever seen. I fell in love instantly. A few days later we found that Amy was not so perfect on the inside. We discovered three weeks later she had a very rare form of Hirschsprung's Disease, no nerves in the bowel. The Dr. wanted to give up but I wouldn't. Over the next few years, and many surgeries, I decided to have another child, Louis S Robert was born Mar. 23/96, again we thought he was perfect but he too had this horrible disease. Thanks to Amy and the Dr.'s at the Hospital for Sick Children we knew exactly what to do for my little boy. As time went on and many more surgeries Amy went downhill very fast. Amy had just turned seven and due to the type of nutrition she had received all her life, TPN, her liver was failing fast. TPN is Total Parental Nutrition, IV nutrition through a permanent IV in her chest. Louis also gets his nutrition this way. When we realized Amy's liver was failing we immediately put her on the transplant list. This is when we found she not only needed liver and bowel but also stomach and pancreas. We prayed everyday for a donor but unfortunately we did not get one and Amy passed away Apr. 9/98. Amy was a little girl who loved life and was very giving so when asked I donated her corneas because I know that's what she would have wanted. I pray everyday that when it is time for Louis to have transplant there will be someone somewhere who will give him the gift of life. Louis is due to go on the list in Aug./99. I know how hard it is to lose a child but I also know that if you lose a child and have the chance for that tragedy to turn into a miracle for another child it makes life just a little better.
Don't let time run out!!!
From the Ebeling's http://sites.netscape.net/kathyebeling/homepage with permission
]˜™˜™] Parent to Parent ]˜™˜™]
Our 6 month old son has Hirschsprung's, he was diagnosed at 3 days old and has had his pull-through surgery (at 8 weeks).We would be very grateful to hear and shear view and comments from parents in the same situation. Specifically those related to the use of formula milk and its effects on your baby. We wish to give our son the occasional bottle and wondered if this would cause constipation.
Regards
Karen and Neil Mattock, Buckinghamshire, England.n.mattock@virgin.net
Reply from Kimberly Robinstein President Guardian Society
Dear Karen
My son too had a horrible time with formula if he kept it down it made him constipated, we tried all kinds have you tried switching to soy as many of the kids with Hirschsprungs are lactose intolerant, it may help. allot of foods can cause constipation mainly dairy products you can try an elemental formula or breast milk thats easier to digest thereby maybe making it easier for him to pass.
Hi My name is Suzanne and my son, our first, is Conner. He is 7 months old. (sorry this may get really long) Conner was born 4 weeks early. He passed his meconium after approx. 30 hrs. Then he had BM's once per day. He was solely breastfed for one week and was losing weight. He went from 6.14 to 5lbs. So we started supplementing with Formula. At approx. 6 weeks old he quit having BM's altogether unless we rectally stimulated him, so we went to the Dr. He then was not going as well with stimulation so we started Suppositories and prune juice. All the juice did was give him diarrhea that he couldn't push out. So on we went to Lactulose, which was HORRIBLE!. In the mean time he had a rectal biopsy come back SHOWING ganglion cells. He also had a barium Enema show everything was normal with slight distention. I have had a VERY hard time explaining to the Drs that he is not constipated in the way they are thinking. His stools are sometimes formed, but NEVER hard. So now we are on 2 tsp. of mineral oil a day and appear to be doing well. He is having one BM a day. When we attempted to take him off he stopped completely. I don't know what is wrong, but I don't believe that this is right. I have read and read and read all that I can, but can only let them do so many tests to my baby. He is a very happy boy VERY, as long as he has had a bm. Sorry this got so long. Thanks! Suzanne and Conner 12/4/98 cns@sprintmail.com
Response by Ramona Painter regional support parent for Tampa Florida
Dear Susan, my name is Ramona Painter and your story is almost identical
to mine with my son. First my son did have a BM in the hospital and was
solely breastfed for 4 weeks. They took him off my breast because they
thought he was allergic to
milk but that was not it he had a reflux but was still gaining weight and
eating like a horse. When Brian went on formula he also stop having Bm's .
We also did the suppositories and prune juice. They also changed his
formula at least 10 times. Brian never got diarrhea with the juice
though. We were on lactulose and mineral oil also and it did help.
Our biopsy showed no ganglion cells and we were told that it was such a
small segment that he would outgrow it. We did not have the barium
enema until he was two but it did show how backed up he was and how big
his intestines were from all the waste. The doctor then sent us to
the surgeon and said he could not believe it because he looked so healthy.
They did not want to do the barium because it was so traumatic but I wish
we would of insisted on it. I know that everything I have read says
you can tell from the barium if a child has HD but I am sure there are a
number of things to consider. For 2 1/2 years I kept my son every green
veggie they made not junk food. I also baked with unprocessed bran I added
it to brownie and anything I baked. Also all of the P fruits and Veggies
will help, make sure if he eats something constipating at one meal then
give a P food at next meal. We also kept him on Mineral oil then he got
some in his lungs and we started using Kondumule(not sure of the spelling
check with Pharmacist) it is mineral oil but thick and fluffy, with
regular oil it can leak into their lungs while the baby is sleeping.
I would watch for coughing and give it when he is going to be up running
around. Don't ever worry about your letter being to long some time you
have allot to say and sometimes not. I wish you luck if you want to
talk please write me.Ramona rpaint@tampabay.rr.com
˜™˜™] Picnic Time]˜™˜™]
Its that time again this the third annual Guardian Society's Picnic this years picnic will be in Anderson Park in wonderful Tarpon Springs Florida on August 1 1999, if interested please RSVP to Tangi Bohlmann bohlmann@gte.net or call us at guardian society 813-996-3033
]˜™] Hirschsprung's Disease ]˜™
7th Grade Research Paper for Language Arts Class
By Alexis
May 21, 1999
HIRSCHSPRUNG'S DISEASE
In this report, Hirschsprung’s disease will be explored. What is Hirschsprung’s disease? Who is affected by this disease? What are the symptoms? Is it deadly? These questions will be answered in research.
Hirschsprung's disease needs to be defined. Hirschsprung's disease is a "genetic disorder", that is when there are no nerve cells in the large intestine the muscles can not work and can’t move feces along ("Hirschsprung’s Disease"1;World Book Rush-Presbyterian – St. Luke’s Med. Ctr. Med. Encyclopedia 425). Hirschsprung's disease was named after "Harald Hirschsprung's" who took care of two boys that couldn’t have proper "bowel movement" ("Hirschsprung's’s Disease"1).
Hirschsprung's disease originates before birth. The development of the disease starts very early in the embryo inside the womb (Fackelmann, Kathery A. 174). It happens within the fifth and the twelfth week of existence (Fackelmann, Kathy A. 174).
Hirschsprung's disease is caused genetically. This disease is caused when the bowel does not allow "stools" (feces) to pass through ("Hirschsprung's’s Disease"1). In some families, one single gene probably causes the disease, that gene is located on chromosome ten (Fackelmann, Kathy A. 174).
Who gets Hirschsprung's disease? Hirschsprung's disease’s odds boys to girls are four to one (Rush-Presbyterian 976). It is mostly found in babies (Rush-Presbyterian 976).
Chances of getting this disease are very slim. The chances of getting Hirschsprung’s disease are out of 5,000 births, one baby has the disease ("Hirschsprung's Disease"1).
There are many symptoms of Hirschsprung's disease. The infected infant has "severe, continuous constipation" and the abdomen gets very swollen because of the build up of feces in the intestine (Rush-Presbyterian 425). Severe vomiting, passing gas, slow growth, constipation from birth when the babies with this disease do not pass "dark green-brown" stools within twenty-four hours of birth are all symptoms of Hirschsprung's’s disease (Rush-Presbyterian 976). A bloated stomach is also a sign of this disease ("Unexpected Death from Enterocolitis after Surgery for Hirschsprung’s Disease"119). Babies "may have trouble passing meconium, the thick, sticky material that is a newborn’s first bowel movement" (Fackelmann, Kathy A. 174). In some cases babies can seem healthy until they eat solid foods that are harder to digest (Fackelmann, Kathy A. 174).
There are two ways to diagnose Hirschsprung’s disease. One is a "rectal suction biopsy" which is looking at a piece of tissue under a microscope to see if the nerve cells are missing (Lori , np). The other one is a "barium enema x-ray test", that is used to determine if the canals in the bowel have collapsed (Hirschsprung's’s Disease"1).
There is only one solution to correct or to treat Hirschsprung’s disease, but many different techniques to do it. After the diagnosis is made, if the baby is too young for surgery, the physician might suggest "saline enemas" to empty the intestine (Rush-Presbyterian 976). Surgery is needed to remove the affected bowel and join together the healthy segments of the bowel ("Hirschsprung's Disease"2). There are three different ways of doing the surgery, all mostly successful ("Hirschsprung's Disease"2). The three different techniques to do "pull through" surgery are Swenson, Duhamel, and Soave (Lori np).
The prognosis varies, but is important. Before "1948" almost every baby with Hirschsprung’s died, but today surgery lets them live (Fackelmann, Kathy A. 174). Usually a result of Hirschsprung's disease could be megacolon (Rush-Presbyterian 557). Episodes of diarrhea, acute illness, and fever might occur because of infection (Rush-Presbyterian 976). After successful surgery, there are usually no long term effects ("Hirschsprung's’s Disease"2). Some babies dies before anyone knows what’s wrong with them; chance of death from infection before surgery is 20 to 50 percent, even after surgery is up to 30 percent (Fackelmann, Kathy A.; "Unexpected death from Enterocolitis After Surgery for Hirschsprung's’s Disease"121).
This report explored all about Hirschsprung’s disease. It was learned that the absence of nerve cells in the intestine causes this disease. The chances of getting it are slim, and that it can be cured with surgery. Hirschsprung’s is a severe disease to be infected with.
WORKS CITED:
Fackelmann,Kathy A. ,"Gutsy Genetics", Science News, Sept. 11, 1993: 174 – 175.
"Hirschsprung's’s Disease"<http://www.tiac.net/users/aphs/hirsh2.htm> (2/9/99)
"Hirschsprung's’s Disease", World Book Rush-Presbyterian-St. Luke’s Med. Ctr. Med.
Encyclopedia 1998.
Lori, Personal Interview // http://www.theguardiansociety.org (5/2/99)
"Unexpected Death from Enterocolitis After Surgery for Hirschsprung's’s Disease",
Pediatrics July 1995: 118 – 121.
From the web pages at http://www.theguardiansociety.org
]˜™] Poems For Chloe]˜™
When you were inside me, I dreamt of and I longed, for cuddly baby days with you, for singing lullaby songs. But we never shared those pleasures, not a single peacefull day, My darling I'm so sorry, that it could not be that way. I have grieved for the loss, of having that time with you, but we do have something special, for all we've been through. An extra special something,that has come from tough times, a love beyond loves limits,will always be yours and mine.
It's been 5 years now, and you've battled on so well, just how you've struggled,
only I can really tell. For I am your mommy, and ofcourse I know best, from
the day you were born,there's been no real time of rest. Night after night,day
after day I've watched you battle, in your special coping way. I feel your
suffering in me, like we are somehow one, and I am so proud, of how well you
have done. My darling girl I love you so, will always be by your side, we'll
fight this battle together, no matter how many tears we cry.
--------------------------------------
Camea, mom to Chloe, a special girl.
]˜™˜™] GOT STORIES FOR US? ]˜™˜™]
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]˜™˜™] 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.
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