nov97

ANNOUNCEMENTS, A LETTER FROM OUR PRESIDENT
Responses to the Survey in THE GUARDIAN MAILBOX , BIRTHDAYS, and calls for PRAYER
This Month's INTERNET LINKS and SPECIAL EVENTS
A HIGH PROTEIN - HIGH CALORIE DIET - Tips for gaining weight from Angela Bennett
Four special stories, including this month's featured story by Karen Higgs.
This Month's Feature - KAREN AND MARY HELEN HIGG'S SPECIAL STORY - mom and daughter with HD
GREGORY'S STORY - A very touching story about a special little boy and his battle with HD
GERRY McGREGOR'S STORY - Hear from an adult with HD
THE HARLOW STORY - Hear about the Harlow Family's experiences with GERD
LOOKING FOR A PENPAL - Is your child looking for someone to correspond with?
CREATIVE SOLUTIONS - questions answered, new questions arise
ASK JENNIFER - A new set of questions for our star reporter
SOMETHING FUNNY - A little humor for those of us online ...
RECALL NOTICE - Simethicone Drops recalled.
CHILDREN'S SSI UPDATE - What's happening to children's benefits?
An updated REGIONAL SUPPORT PARENT CONTACT LIST
Please don't forget to read our DISCLAIMER...

This edition is lovingly dedicated to Gregory Shearon 2/20/97- 7/4/97.

Announcements

The Guardian was recently introduced on a Support Topic Board for Rare Genetic Diseases in Children. This board is supported by The Rare Genetic Diseases In Children Website which is hosted by Eric Szczerbinski (EricS10332@aol.com )
Guardian was announced as a Motility Disorders Support Group and a letter by our President to this Board and to St. John's was included to give parents information and background about our group.

A Letter From Our President

On Monday, November 2, we had two special visitors in Quincy. Tim and Jennifer Beehler regional support parents for Ohio made the journey from Ohio to see us. With them were their two tow headed waifs, Andrew, age 3 and Matt (total colon Hirschsprung's). We were able to spend a few hours with the Beehlers and were so glad they made thejourney. They have some adorable children and it does my heart good to see a child who in my mind was so sick look so incredible. Guardian members met Jennifer and Tim last month in our section "A Father's View" by Tim Beehler. As you all know Tim hasgraciously volunteered to become one of our parent advisors. Jen has accepted the position of Parent Liaison to guardian and is now responsible to help me keep track of new members and send packages to them. While here they presented Guardian society with a check for $175.00. Jennifer said the Guardian members raised money to help repair the Guardian computer. I was totally aghast and grateful. Lorrie Sheardon made a donation in memory of her son, Gregory, who is in our newsletter this month.

The Guardian society has taken to the air waves. On November 4, our first ever Internet Vigil was held with the main Vigil being held here in Quincy, Illinois, with a follow up chat session in the Guardian Chat room. It was a solemn moment for me as the namesof our children were spoken and the prayer said. Parents all over the world set aside this night to honor our children to help raise awareness of our children's struggles, and it was declared "Freedom Night." KHQA television here was kind enough to cover the vigil here in Quincy and an extensive broadcast was done at 6 p.m. As a result, I was able to find anther parent, not with Hirschsprung's, but Imperforate Anus. On November 4 I also received a special package from our web researcher, Angela Bennett. In this beautiful package were some well made hand crafted items, for us to sell. For those that received the ribbons, credit goes to Angela, as she made those herself. Angela has been undergoing tests for undiagnosed motility disorder and we are so proud of her courage. We thank her for her kind donation.

Some of our adult members have been having a tough time lately. Gerry McGregor has been having problems as of late and I personally wish him a speedy recovery as he was just recently re-diagnosed with megacolon. Gerry has suffered with Hirschsprung's since childhood and lives in England with his wife and children. See Gerry's article in this months newsletter.

A Special Note

The Guardian society is run by parents and not by professionals. Sometimes we make mistakes in our attempts to help. Mistakes are common for those learning so please have patience as we learn from those mistakes, some times due to limitations of being human we may forget a typo or that sort but our love shines in our attempts to raise awareness of children and adults with motility disorders. We share our hearts in hope of helping someone who is lost in the system to find a way to deal with life. And each day we grow and find people who can help us in our journey to become the best support group ever. Parents have been volunteering to be regional support parents every day distributing the newsletter, brochures and such. We are actively seeking medical advisors to help us understand some of the disorders our childrensuffer from. A pediatric GI nurse in Oklahoma has offered to help us with the newsletter and such. We are compiling a list of compassionate doctors for referrals.

On a special note, Sue Glen, a grandma in North Carolina contacted us about the same time as Jeannie did with a story about her grandson. Thanks to the loving support of our South Carolina parents and many more the child has since been diagnosed with Hirschsprung's, the information the parents and grandparents were given allowed them to pushfor tests that resulted in the diagnosis. This child was not missed and the result was the child underwent a one step procedure and while there have been some problems he is doing OK (via talking to grandmother). As we all know this story could have had a much different outcome. Thanks for all you guys do for the children. The prayers, and stories are overwhelming and wonderful thanks for sharing your lives with us and allowing us to become a part of your lives.

Got stories or comments?

Send to
Kimberly Robinstein
819 College
Quincy Illinois
62301
Email maniac@rnet.com
217-222-3906

Kimberly Robinstein
President Guardian society
A society dedicated to persons with motility disorders.

Guardian Mail Box

Survey Results

90% of those whom returned the survey felt we were doing a good job. Most found the newsletter informative and fun, and looked forward to it each month and did not want this changed. Some areas we need to improve on were:
chat --- most members wanted a scheduled chat to meet
typos --- some members found typos we missed (sorry they have been corrected)
more updates --- some of our more active members wanted to be better informed on updates not to the society's events but regular updates on how thekids are doing, updated hospital list, birthday cards, hospital cards, so we have assigned this task to a member to update the address book to include birthdays, diagnoses.
To help us complete this task, members are asked to send a bio of their family with information they want to be included and if they would like birthday cards and such for their child, this can be done via internet or postal or you can call us with the information via internet: Gerry.McGregor.btinternet.com or maniac@rnet.com or via postal mail to Guardian Society at 819 College, Quincy, Il 62301 or call 217-222-3906
NOTE: All information is held in strictest confidence unless specified by the parents or member and is not distributed without permission

Internet Links and Special Events

Kimberly,

I thought you might want to know about a worldwide candlelighting time for all children who have died. This is being coordinated by The Compassionate Friends, an international support group for bereaved parents. I have been going to the local chapter meetings and found out about this yesterday. There is more information on their web page at: http://www.compassionatefriends.org

Briefly, on December 14, at 7:00 local time (whatever time zone you're in) they are asking people to light candles to remember all children who have died. This will begin overseas and the candles will be lit continuously for 24 hours around the world. I think it sounds wonderful. I just thought since you're working on a new newsletter and the home page, you might want to consider putting something in one of those about this effort. I know your group is not about bereaved parents, but you personally might be interested anyway.

Thanks!

Lorrie Shearon

I would like to invite all patients to visit the CenterWatch web site to complete a short questionnaire. The questionnaire is located at the bottom of the CenterWatch web site's home page (www.centerwatch.com), and can be completed on-line. The questionnaire has been posted to enable CenterWatch to gain a better understanding of how patients learn about clinical trial opportunities. We invite all patients to include feedback on how the CenterWatch web site can be improved as a clinical trials information resource. We thank you for taking the time to complete this questionnaire, and as always, we welcome your questions and comments.

Sincerely,
Ann Kennon
Web Manager
CenterWatch, Inc.
Cntrwatch@aol.com

Family Voices
A network for families and friends speaking on behalf of children with special healthcare needs.
National Office
P.O Box 769
Algodones, New Mexico 87001
Tel 505-867-2368
email fam01rw@wonder.em.cdc.gov
http://www.ichp.ufl.edu/MCH-NetLink/FamilyVoices

Special Days This Month

Happy Birthday to...

Kayla Rosado!!! Kayla turned 2 on November 10th.
Andrew Beehler!!! Andrew turned 3 on November 5th.
Alexandra Thompson!!! Alexandra will turn 8 on November 18th.

Prayers for our Sick Ones

Tyler Siefert
Alexandra Thompson
Gerry McGregor
Angela Bennett
Kimberly Sizemore
Sean Forney (Sean just had his pullthru recently)
Sue Glen and her grandson T.J.

Special prayers for strength

Shearon family
Cooper family
Thompson family
Forney family

A High Protein - High Calorie Diet

Contributed by Angela Bennett

I have noticed that a lot of our members have children who are in need of high calorie/protein diet, to gain more weight. Most people who suffer from stomach disorders are underweight, and that is not good for the body. Since I myself am currently going on this kind of diet. I will list some helpful ways to get more protein and calories into your diet.

1.) Increase the serving size of milk and milk products. According to the food pyramid guide you should have 2-3 servings a day of milk, yogurt, and/or cheese. Add 2 or more servings from this group, such as: cheese, cottage cheese, ice cream, milk, yogurt.

2.) Add powdered milk to food and drinks. Like in soups, casseroles, cook cereals, scrambled eggs, puddings, milk shakes, and into whole milk.

3.) When cooking foods, use heavy cream, evaporated or whole milk instead of water when making soups, hot cocoa, cooked cereals, and pudding.

4.) Add extra margarine, sour cream, salad dressing, cheeses, gravies to you food whenever possible.

5.) Add instant breakfast to mix drinks and foods.

6.) Add diced meat or cheese to sauces, veggies, soups and casseroles.

7.) Serve fruits canned in heavy syrup, sweetened cereals, toast with sugar, jelly, or honey.

Milkshakes are an easy way to get a boost of calories and protein. Here are a few recipes. Mix ingredients well in a blender.

Banana Nut
(provides 555 calories and 18 grams of protein)
1 cup vanilla ice cream
1 pkg. vanilla carnation instant breakfast
4 oz of half and half milk
1/2 capful of black walnut extract
1/2 of a ripe banana

Chocolate Mint
(provides 550 calories and 17 grams of protein)
1 cup vanilla ice cream
1 pkg. vanilla carnation instant breakfast
4 oz of half and half milk
1/2 a capful of mint/peppermint extract

Peach
(provides 550 calories and 15 grams of protein)
1/2 cup vanilla ice cream
1 pkg. vanilla carnation instant breakfast
4 oz of half and half milk
2 canned peach halves

The Higg's Special Story

I'd like to share my story with the members of the Guardian Society in hopes that it will be an encouragement to others facing similar situations.

In August 1966, only 17 days after I was born, I was diagnosed with Hirschsprung's Disease. Unfortunately, I don't know the details that led to this discovery. My mother passed away when I was 3 years old and my father doesn't remember much about that time except that I was a "very sick little baby." Luckily, I have a complete copy of my medical records beginning from my first surgery in San Antonio, TX at the base (USAF) hospital.

I underwent surgery to create a colostomy so that I could be stabilized. According to biopsy results, over two-thirds of my colon was aganglionic. After spending an extended length of time in the hospital due to chicken pox, I was dismissed. My mother and I moved to GA where we stayed with her parents while my father was overseas.

I have seen a few photographs of me during this time. From what I understand, I did not wear an appliance for my ostomy. They relied on gauze and ace wraps. My grandmother, now 83, says that keeping me clean was a major priority. She tells me that my skin did not break down too badly. I guess that it is because I still had some large bowel left and the stool is not quite as acidic as that coming from the ileum.

During this time, I was taken to Atlanta's Egleston Children's Hospital. Dr. Harlan Stone set forth the criteria for my pull-through. I had to be two years old or weigh at least 30 pounds. Well, I guess I was a healthy eater, because at 18 months, I underwent what was to be myfinal surgery, a Swenson pull-through. As luck would have it, my wound opened up and according to my grandmother, my "insides tumbled out." So back to OR I went. Once I finally started pooping, my grandmother said that I was very scared. I didn't seem to understand what was going on. Imagine that!

From then on I have led a fairly normal life. I do remember bouts of constipation and begging to go without the milk and molasses enemas or Senekot. My grandmother can only remember one time that the doctors seemed to be concerned about colitis. But I was never hospitalized.

The first of every school year, my grandmother would go talk to my new teacher and explain my bathroom habits. Fortunately, I went to a small private school so after the first few years, most of the staff knew of my situation.

The only regret I have about growing up with HD is that my grandmother was what some would call overprotective. Some of this I also attribute to her losing her only child and raising me, her only grandchild. I was never allowed to be active. My grandmother would always tell me that I was too frail to be running and jumping or playing outdoors with the other kids. This led me to be very sedentary and as a result, I am now an inactive adult battling the weight! Being raised by an overprotective caregiver made me determined that when I became a parent, I would encourage my children to be as active as they wanted.

In June of 1996, I became a parent and was ready to put this theory into practice. The minute they handed my little red-haired daughter to me, I was overwhelmed with emotion. Somewhere in the back of my mind I had stuffed the worries about passing on HD to my children. I was assured by my OB/GYN that HD was not hereditary. So for the first 24 hours of Mary Helen's life, I just enjoyed being a new mom and watching her sleep.

And sleep she did. The nurses had me waking her every 2 hours to try to nurse. She had absolutely no interest in eating! I agreed to try formula just to get something in her. She wouldn't take the bottle either. But she had pooped - 3 times that first day! So I still did not worry about HD.

I look back now and realize what a blessing I received when my doctor agreed that I could stay a second day...since my insurance would cover the stay. I had had a very easy labor and delivery and felt fine but chose to stay anyway. The second day, Mary Helen's color became very yellow. I started questioning the nurses. Several made off-handed remarks about me being a first time mom and not to worry, it was just a little jaundice.

Our pediatrician was out of town that second day and his replacement couldn't get comfortable with the lab results. Apparently her bilirubin was not high enough for her to be that yellow. She suggestedthat I bring Mary Helen in to the office that Sunday after we went home on Saturday for another check. This was as she came in to say that she had signed the discharge papers. As she turned to leave the room, my child made a horrible wretching noise and proceeded to vomit. What came up was very yellow, almost neon. The doc immediately sent her to X-ray.

After a few more tests, it was determined that Mary Helen had Necrotising Enterocolitis (NEC). This is an infection in the lining of the bowel. It causes the space between the interior lining and the exterior wall to be filled with gas. I had noticed that her belly seemed a little bloated, but again was shrugged off by the nurses. NEC is potentially life threatening in that the bowel wall could perforate and sepsis could set in. NEC is common in premature infants, but as Mary Helen was almost 2 weeks late and weighed in at 7 pounds, 14 oz., the doc seemed a little puzzled.

Our local hospital was not equipped to deal with such critical cases. We waited almost 3 hours to find a hospital with a NICU bed and air transport available. In that time, Mary Helen's belly became very distended and she looked swollen all over. She was life-flighted to Tallahassee Memorial Regional Medical Center.

During her first few days there, I repeatedly questioned anyone involved in her care about the possibility of her having HD. All seemed to agree that she didn't because she continued to stool. And besides, HD was not hereditary.

After 10 days on IV antibiotics, TPN and Hyperalimentation, we were ready to start oral feeds. I was so excited that morning. I knew that if she did well for a couple of days and put on a few ounces, we would make the three hour drive home with our baby.

She took her first few bottles well and we were pleased with her progress. The hospital had a policy of "nesting" before they would send you home with your NICU baby. We were looking forward to that day in a specially set up room. We were on our own for her care and feeds.

The first bottle she immediately spit up. Not a lot, but enough for me to call the nurse. She advised us to try again in about an hour and burp her more often. So, the hour passed and we tried again. This time she had what they call aprojectile vomit. I was scared to death. The nurse came in and suggested that we call the neonatologist.

Mary Helen was sent for some X-rays. All seemed normal, but we were told to expect to stay a few more days. In the meantime, our pediatrician hadcalled. We filled him in and he suggested that we not come home unless they did a rectal biopsy just to rule out HD. The docs did not think that necessary but agreed to do a lower GI study that involved a barium enema.

Thankfully the radiologist saw something he didn't like and ordered a few more films. From there they chose to do an upper GI to rule out GERD. But before they could give her the barium to swallow, the radiologist did a flat belly film as a"scout." Her gut was still full of barium. This led us to the rectal biopsy and HD diagnosis.

Mary Helen was scheduled for surgery the next morning. Her surgeon assured us that since she was still stooling, and there was no obvious transition zone (an area where the bowel becomes enlarged due toinability to continue to move stool out of the system above a line where the ganglion cells stop ) this looked like a textbook case of short-segment HD.

After 4 hours of waiting, our surgeon came out with tears in his eyes. He sat down and began to explain that Mary Helen's entire colon was affected. He had had to place an ileostomy instead of the colostomy we were prepared for. In terms of her care, this made little difference, an ostomy is still an ostomy. The long-term prognosis was a little grimmer.. If she had a conventional pull-through, one that involved a J-pouch, she would face a lifetime of chronic diarrhea and worries of dehydration. The J-pouch is where they use a section of the small intestine to create a new reservoir to hold stool, much like the rectum does normally. And since the small intestine had no water absorptive properties, the stool would be very liquidy.

To his credit, our surgeon picked up the phone and called someone more experienced in pediatric surgery. Our doc was not one and the hospital was not a true pediatric facility. We proceeded to go on-line in search of information too. The next day, when we met, we had all found out about the same procedure...a right-colon or Kimura Patch. This is where a section of the right colon is patched to the small bowel , much like you would patch a hole in a pants leg, and used to create the reservoir. The ET nurse joined us at this point to instruct us in the care of her ostomy. In the literature he gave us we noticed an American Hirschsprung's Association (now merged with another group to form the APHS). Through them, we were put in touch with a surgeon in St. Louis, MO, Jacob Langer.

Dr. Langer took a great deal of time to listen to me and then to talk. He recommended that we go to Atlanta and see his friend, Kurt Heiss. We couldn't be more pleased! Dr. Heiss has become much more than a doctor to our family.

Our first consult with him was in August of '96. Somehow, our records had not made it from Tallahassee to him yet, but he still spent over an hour answering our questions and explaining the different procedures. We set a time in September to meet again and decide what we wanted to do.

In October, Mary Helen underwent surgery to place the patch and to remove the rest of the colon and have her pull-through, a Soave procedure. Dr. Heiss also revised her stoma from a loop to an end ostomy. This was a result of a prolapse. Mary Helen did well with her surgery, but caught some sort of bug while hospitalized. So after 24 hours at home we returned to Atlanta for 5 days of antibiotics.

In January of this year, her ileostomy was closed. We have been very pleased with the results. So far she has been hospitalized once for colitis. This was 4 weeks post-op and we had not begun irrigations at that point. After returning home, we did irrigations daily. We have had continued support from Dr. Heiss and he has given us much liberty in deciding Mary Helen's course of care. We are now doing irrigations only as necessary. The last time was after a period of 3 weeks without one. We watch for several things before we decide to put everyone through the process. Our list is as follows:

-no poop in 24 hours
-a distended belly (more than usual...something you can gauge after a while)
-little or no appetite
-dark green, thick, crusty poop with a foul odor.

If any two or more of these occur, we irrigate. I have been keeping track and it seems that we have hadto irrigate more often when she is cutting a tooth. I can find no scientific evidence to back my theory, but I definitely see a pattern occurring after 8 teeth!

I know that having been down this road myself has allowed me to face Mary Helen's HD with a little more optimism than most parents. I continue to tell people that although HD is potentially life threatening, it is a fixable problem. Day to day life can be challenging for the parents and children, but life can and does go on. I am proof positive.

Gregory's Story

Gregory was special from the very beginning. He was born in the VIP suite of a hospital here in Nashville, the same room where many local celebrities (Garth Brooks, etc.) welcome their new babies. (We thoughtthis was only because all the other rooms were full, but maybe it was because he was so special.)

Gregory was born on February 20, 1997, following a full-term, uneventful pregnancy. He weighed 7 lbs., 6 1/2 ozs. (big for our families) and had Apgar scores of 9. My husband and I were surprised and so very pleased that he seemed so healthy that the nurses let him remain in the room with us for almost three hours following his birth. We cherished that time together, just the three of us bonding. We busily called friends and family members to announce the happy news and could not even tell people how much he weighed since he had not been taken to the nursery for a weigh-in yet.

At thirty-six hours of age, Gregory had not yet passed meconium. We were still in the hospital for unrelated reasons. The nurses and pediatrician seemed mildly concerned, but they used a rectal thermometer on him and he had a bowel movement. After that they dismissed their concern, and so did we. We brought home our"healthy", beautiful baby with great joy and anticipation.

But, Gregory liked to sleep instead of eat. This had happened with my older daughter, so I just figured that's how my babies were. For about the first two weeks, I had him at the pediatrician's office almost every day for weight checks, nursing tips, etc. After a great deal of effort, he began nursing well and gaining weight wonderfully. At his two-month check-up his weight and everything else seemed terrific. His bowel movements had slowed down to justevery few days, but they looked normal and he did not strain an unusual amount to pass them. The doctor's office assured me that this was normal for some breast-fed babies. All was well.

The weeks passed and Gregory's personality developed. He had a smile that lit up his whole face. He liked his rattles, sleeping with his mom, and watching his sister's cartoons. At about 3 1/2 months of age, I began to introduce formula so I could go back to work. Once I began giving him more than one bottle a day, hestarted to have problems. He had horrible gas and accompanying pains and began to spit up a lot. I thought he probably needed a different formula. After consulting with the doctor's office, we changed the formula a couple of times. One night, he vomited several times and seemed extremely uncomfortable. I went back to exclusively breastfeeding him and took him in the next day. By this time, the vomiting had stopped (the formula had also stopped). They took an x-ray of his abdomen to check for an obstruction and did not find one, so concluded it must be a virus. They also advised changing formula to Nutramigen.

Well, the vomiting stopped, but the gas pains got worse and so did the infrequency of bowel movements. I knew this was not right. I called the pediatrician once again and after discussion, she referred us to a pediatric gastroenterologist at Vanderbilt Children's Hospital. The gastroenterologist thought Greg's symptoms definitely sounded like Hirschsprung's Disease. (Hirsch-what?) That sameday, we had a barium enema done, which did not show signs of the disease. The radiologist diagnosed Greg with severe constipation. However, the gastroenterologist thought they should do a suction biopsy to be sure. Reassuring us that there was a 90% chance he did not have Hirschsprung's, he sent us home with instructions to come back the following week. Five days later (the first available appointment) we went back for a suction biopsy. We were pleased to tell the doctors that Gregory had had a large bowel movement only two days before; but we were concerned that it did not appear that any of the barium had been passed. Two days later, we were informed that the suction biopsy did not show ganglion cells and that we would be referred to a pediatric surgeon for a full-thickness biopsy.

Unfortunately, we had to wait two more weeks to see the surgeon. In the meantime, we became VERY concerned about Greg's distended abdomen, fussiness, painful gas, and failure to have a bowel movement. We took him to the pediatrician who also became quite concerned upon looking at him and doing an x-ray. His intestines were full of stool and still contained lots of barium from two weeks before. Two enemas were administered in the office and she advised us to begin putting Milk of Magnesia in his formula. In the next 24 hours, Gregory lost six ounces and had more than a dozen bowel movements. He also became very fussy and began refusing to eat more and more. A couple of days later, we returned to the pediatrician becauseI was worried he was becoming dehydrated. He was diagnosed with an ear infection. The doctor gave him an injection of antibiotics and did a blood test to check his white blood count. She said he was amazingly well hydrated considering how little he hadbeen eating.

The next day, we finally got to see the surgeon. After examining Gregory and listening to our history of his problems, he decided to do surgery the next day. Just prior to going in for surgery, Greg had several bowel movements. By this time, he was straining terribly to pass stool; it looked like his insides were coming out. The stools looked a little unusual, but not what we considered remarkably loose or hard (at least one nurse also observed this). Greg was in surgery much longer thananticipated, and when it was over the surgeon informed us that they had not found ganglion cells anywhere in his colon. Ganglion cells were found at the end of his ileum and he received an ileostomy. The surgeon told us how rare this was and also that he had put Gregory on two different antibiotics.

Gregory did terrific for about 24 hours. He rested comfortably and even began eating some formula. Then he began to vomit bile. He vomited about seven times and began crying. Gregory never stopped crying until he went into respiratory arrest about eight hours later. During that time, the nurses put a tube down his throat to absorb excess bile and they upped his pain medicine from Tylenol to Morphine. He ran only a low grade fever. His crying continued as my husband and I tried and tried to comfort him and get him to rest. We were assured that he was probably hungry and uncomfortable from the tube. His crying started to get weaker shortly before midnight. We thought he was getting sleepy finally. When the nursing student came to take his vital signs, she found no blood pressure. He went into respiratory arrest only a couple of minutes later. Our world fell apart in those couple of minutes; it could not have been more shocking and sudden to us. They eventually resuscitated him and moved him to the PICU, but they never really stabilized him. No one could really tell us what was going on or why this was happening. Eventually we were told that he had no brain activity and would die shortly. Our very precious, beautiful little son passed from this world the morning of July 4, 1997. He was four months and fourteen days old. The autopsy report showed evidence of enterocolitis and sepsis, caused by a very resistant strain of bacteria.

Gerry McGregor's Story

My history.

Well, some of it is difficult because I don't know a great deal about it. I have learned more about HD in the past few months than I learned in the rest of my time. I don't know if you saw one of my early e-mails but it is the importance of parents keeping there child medical history. I don't have mine and as all of the important things happen to us when we are young then our knowledge of it depends on you. So when your kids get older they may find it useful to have them to refer to and to pass on their doctor at that time. I just wish that I had mine. Here goes and forgive me if I ramble, you may have noticed it by now.

I was born in 1952 in the UK at a place called Sunderland in the NE of England, the youngest of 5 kids. From birth I had a problem with my bowels but in '52 HD was not well known. My mum struggled along as you are doing now, making me go by various awful means but not knowing what the problem was. After a while I was diagnosed as having HD. In the UK we only had 2 surgeons who couldperform the operation. Luckily I got one of them. I had to have 2 resections as the first time not enough was removed and before each I had a colostomy. What exactly was done is vague but in total I had 5 operations between the age of 1 year and 5 years. I basically spent the first 5 years in hospital. Those 5 years I remember very little. I have the odd remembrance like blood transfusions and being fed by drips into my arms and legs.

When I came out I had to have daily enemas and very frequent visits to outpatients. In those days the enemas were soap & water variety. I remember the big break through when other less violent types came in. The enemas were reduced and I was left on Agarol and Senekot tablets, the Senekot tablets I continued on until my early twenties. Early on I had did not have full control of my bowels at night especially but that did come except that I grew to enjoy the special attention. I was spoilt and I think to try to keep it going I used to be incontinent. It started off real but then I got used to it. As I got older I gained full control on by bowels and when in later life I had to have enemas. I would only pass them when I got bored of lying around.

Gradually I improved and except for my own purposes of manipulation I to allintent and purpose forgot that I had HD. At school I never had to do PE and at home I got my own way. At the ages of 11, 15 18 and 21 I did have to return to the hospital, as I was severely constipated but only for a few days. It was thought that at 11 I may have had to have another op to widen part of the bowel but they decided not to. For the greater part of my life so far, I have just got on with things and the HD has never really stood in my way.

Anyway, I passed my exams at school and then went to college got a diploma in computing. Now I am now a senior manager in a computer company providing a large software package to insurance brokers. I am married to June, with 4 kids/young adults. Kristian is 18, Emma is 16, and Michelle and Claire are 13.Mostly healthy except for Kris who at 9 fell down stairs and a few months later developed Epilepsy.

Currently things are not great that is why I found the group. For the past few years I have been having bowel problems and at first it was diagnosed as colitis as I was going frequently and had other symptoms. Recently my consultant had a change of mind and rediagnosed it as Megacolon. I have been referred to a consultant in London at a specialist hospital, St Marks. I saw him at the beginning of Octoberand he wants to give me a couple of tests, Anorectal Physiology and a Barium Enema. This will happen on 17th Nov and then I will see him in the afternoon when he will make a decision about what happens next. I think that it will be the removal but we have to wait and see. Over the past few months I have become run down with it all and that is why I am at home currently for awhile.

One thing that HD give kids is a great desire to get on. We have to. Everyone thought that because of all of my problems, missed school, etc. that I would not get on but here I am with a good job and great family. There is life with HD if you want it. To get on we have to learn how to battle and we do. Look at Kim Ulacco, she has just had a healthy baby girl and all of theother adults who are joining the group and how they have made a success of themselves.

I am sure there are other things I could have said. If you have any questions that you want to ask the email me at gerry.mcgregor@btinternet.com and I will try to answer your questions.

The Harlow Story

The tests our kids have gone through prior to their Nissens were pH probes, numerous upper GIs, esophageal manometry and repeated EGDs with biopsies. Each time a Nissen was recommended, I took the kids to another peds GI and another peds surgeon for second opinions.

Our first child's (Kyle) GERD was found while searching for the cause of his repeated respiratory symptoms, croup and pneumonias, asthma requiring constant steroids and nebulizer treatments. Evidently, he was aspirating all that time (it took three years before they found this). Following his Nissen age at 4, he went from an average of 1-2 hospitalizations a month to NONE for the next 10 years. Just in the last couple of years, he has had another pneumonia and some mild respiratory symptoms and unfortunately, recent biopsy shows, again esophagitis. For now, he is stable on Prilosec and Propulsid. His chest CT does show a good bit of scarring from aspiration. He is very self conscious of the large scar he has and it is very hard on him when he gets sick and cannot vomit. It is a high price to pay but obviously we had no choice but to do the procedure.

Our second son (Kenneth) did very well at birth, only had some difficulty swallowing, occasionally choking on food. At age 7 during his first EGD, he SAT UP during the procedure and perforated his esophagus. (That week in the ICU was the worse experience of our lives). He has a very tight upper esophageal stricture (very much like his mother's and believed to be congenital). He also has esophagitis and now is up to 60mg a day of Prilosec with finally, some response. We are hopeful he will not require surgery.

Our third son (Korey) is completely healthy. Our fourth son, Kody, did well until about age 10 months (with the exception of a lot of spitting up) when we tried to start table foods. He had difficulty swallowing the foods and began vomiting ateach meal and every night and even between meals. From age 12-30 months, he lost a pound and never gained any weight. Endoscopies showed worsening esophagitis on therapy and "felinization" -- thickening of the mucosa and ringed-liked characteristics, pH probe showed reflux. He began having severe episodes of chest pain and refused food. His Nissen was just done last week. He is on constant tube feedings now and still refuses food/drink. He will be 3 in January.

Son #5 (Killian) just turned one and is doing just fine.... and THERE ARE NO MORE. We cannot imagine life without any of our wonderful boys; however, we were repeatedly told that GERD is NOT hereditary. I find that very hard to believe at this point. As many parents of chronically ill children realize, the strain of caring for them, is felt throughout the entire family and is difficult for all involved. We strive to keep as normal an atmosphere as possible, keeping our expectations as one would of normal children and don't allow too much self pity. (An extra hug or two when needed, then on their way). We have our moments when it all seems too much to bear but for the most part, "one day at a time" works the best and lifestyle adjustments become a matter of habit without effort.

Kody's Nissen was done at Walter Reed by Dr. Dan Roby. Kyle's was done 10 years ago at Balboa Naval Med Center in San Diego. If the Prilosec had worked for Kody, we would not have done the Nissen. He was no better on it than off despite massive doses. My advice toyou is to NOT do the Nissen if you can control the symptoms on medical therapy. As long as there continue to be no serious side effects from long term Prilosec, it is easier to give meds each day, rather than deal with the problems from a Nissen. It istoo early to tell if Kody's was a complete success. My oldest had one 10 years ago and it was a perfect wrap (he can't vomit, but he CAN burp). Evidently, many are not this lucky, often it is too tight or not quite tight enough. There is also the problem of not being able to vomit. When Kyle has gotten a stomach flu, it is a terrible thing to watch since he cannot vomit.

If you do decide on having the procedure, make sure you get a peds surgeon who has done hundreds of Nissens. This is a skill that definitely needs experience.

S. J. Harlow
Co-Founder
THE HARLOW HOMESTEAD
"The Largest Herd of Harlows in the World"

Looking For A Penpal

Brenda writes:

I have a daughter 8 years old with Hirschsprung's and we are looking for a little girl her age or older that she could communicate with about her
daily issues. If you or anyone you know could contact me I would be very grateful. We live in Calgary Alberta Canada and have been unable to find someone her age or older for her to talk to in Western Canada. Any help would be gratefully appreciated!

Thank You.

Brenda Chute for my daughter Tristen. (bchute@rider.ca)

Creative Solutions

We never used Ilex for Matt. Here is what worked best for us. The Stomahesive powder and Desitin mixed along with 2 baking soda sitz baths a day. Hope this helps.

Jen Beehler

Alternative to Ilex --- Calmoseptine ointment, which is available over the counter or from the web
http://www.calmoseptineointment.com or by calling 1-800-800-3045.

I recently heard of this from some of our members and tried it on my son. It worked well when his bottom broke down due to a virus.

Thanks Bea & Whitney

Kimberly Robinstein

More bottom solutions needed contact. Email maniac@rnet.com to submit or call 217-222-3906

One solution for skin breakdown due to pullthru or diaper rash is Ilex. Ilex is available at most ostomy supply stores or can be ordered as well to use make sure the paste is smooth and cover with a layer of Petroleum jelly, and don't remove each diaper change.

Hello. We are new to this group and I wanted to introduce ourselves and see if there are any with similar situations.

We have five children, three of whom have severe gastroesophageal reflux. Our oldest had a Nissen with temporary G-tube placement 10 years ago. He is fine now. Our 10-year-old is soon to go to the OR for a Nissen (not sureabout the Gtube need yet) and our 2-year-old is four weeks post-op from a Nissen with Mic-Key G-tube placement.

We have had much difficulty getting the feedings up to speed with the 2-year-old. It is unclear exactly why he will not eat. But even tube feedings are a problem for him. Bolus feedings caused "dumping" (even as small as 60cc feedings). We have had much more success with constant infusion from an abdominal standpoint. However, he is feeling much better and we have found the "portable" pumps tonot be as portable as we would like. The alarm is constantly going off as he tries to go about his daily activities, to say nothing of the fact that he often reacts impulsively and will get up to run off or jump on a toy which either pulls him down or pulls the tubing apart (he hasn't pulled the G-tube out yet).

His stoma site also seems to have some trouble healing. It is still very sore and red around the site, four weeks postop. Anyone with experiences or advice in this area, your input would be much appreciated!

S. J. Harlow
Co-Founder
THE HARLOW HOMESTEAD
"The Largest Herd of Harlows in the World"

Use a baggage carrier for the pumps, (dolly type, two wheeled, small size) our portable pumps are the cad for iv TPN,
and the companion for enteral feeds. We put them in their carrier bags. Usually a shoulder strap bag for companion, and a backpack for her iv TPN. Set them upright the way each is supposed to work, put them on the baggage carrier, and take a bungy cord to rap it. The pumps we have stay fairly stable. Jennifer pulls it sometimes and pushes it other. Once in a while we will get an error, but not like we did before. As for the pulling on the stoma site we would make a loop in the line for slack, rap a piece of tapearound it (after the connection on pump side) this will help to keep it connected, (leave a tab of tape to pin through) take a safety pin and anchor the tube to their clothing. This will keep from yanking on the line as bad, and aggravating the site. Treat it with .5 % Triamcinolone Acetonide. It has helped our daughter's site heal. We heard about it on G-tube list. Everyone called it Kenalog I hope this helps.

From Melvin Sparks, dad to Jennifer Sparks

Remember in this section that we are not doctors, only parents who have some unique ideas which may or may not work for your individual situation. Please use with caution and/or contact you private physician for input.

Guardian Society

Do you have CREATIVE SOLUTIONS for these parents and children???

Paula Seifert writes,

I am really feeling frustrated with my son's problems. I know that they are not life threatening like some of our wonderful children's, but they are frustrating none the less.

I took Tyler in about two weeks ago for vomiting and continual fussiness. The doctor and I talked and saw no signs of illness so we assumed he was having reflux problems. So, we had an upper GI study done on Monday, which showed reflux. I was then referred to the pediatric surgeon, who asked us to contact the GI specialist to have a ph probe study done. Apparently with a Thal Fundoplication the barium can reflux even if you are not having reflux problems at other times. So fine, we contacted the pediatric GI specialist who wants to see Tyler on Friday. AAAAARG. I am sure you can all share my frustration when YOU KNOW exactly what the problem is and you are getting the run around. Or for some of you when you are unsure and you have to wait solong while your child is suffering.

Meanwhile Tyler has begun waking up at night crying in pain. So, since we have tried umpteen million medications before he had surgery, we are very hesitant to put him through all that again. He did not seem to agreewith most of them and ended up throwing up several.

The most troubling thing to me is, that he is not actually throwing up enough to interfere with his weight gain. Which we all know has nothing to do with being uncomfortable. Well my concern is that because the actual severity of his problem is minimal than before when he had only gained six ounces in his first three months of life that they will be very hesitant to do anything. My husband and I REALLY want him to have a repeat fundoplication, this time a Nissen, not a Thal. Evidently from what the surgeon told us the Nissen is a tighter closure than the Thal, which up until now was the most wonderful thing in the world for our family!!!!!

Thanks for letting me get that out. I feel much better now.

Paula can be reached via e-mail at: Angusgds@aol.com

EDITOR'S NOTE: UPDATE: Taylor had his surgery last week. Child and family are doing ok.

HELP

My grandson has been diagnosed with Cyclic Vomiting Syndrome secondary to Intestinal Neuronal Dysplasia. He is 26 months living on TPN via broviac line. My family is desperate in finding more information, for it seems his illness is getting far more complicated.

Jeannine Dufault jdufault@livingonline.com

Help Jordon, please

Jordon is a wonderful boy age 10 who is being teased at school when he has an accident, Jordon is in need of some creative solutions to help him deal with his problem.

Contact him at BTalon@century2.canada.ncr.com

Twila Foulds writes,

I am looking for some information about what surgery they do for reflux, my daughter's ped. is considering surgery for what he thinks might be a reflux problem. She has had a swallow study done, about 18 months ago, and it came back normal, but she keeps having these vomiting episodes, 7 out of the last 9 days she has vomitted at least once during the day, I can't seem to find a common with it, so it is difficult to avoid. We tried her on a trial of cisapride for one month, but it didn't appear to be helping so I took her off.

He isn't anxious to go into surgery, and I am really not anxious to do that, it seems so drastic. But she is really little for her age, 23.5lb at 3 3/4 yrs. She doesn't seem to be able to eat more than 2 oz of solid food at one time without being at risk for throwing up. I wouldn't mind putting up with the vomitting so much, but a lot of the time it happens when she is in bed, orhaving a quiet time, and it is interrupting her rest, which she really needs, since she isn't yet sleeping through the night, it is kind of scary too because she doesn't wretch when she vomits, she doesn't usually wake up if she happens to be asleep at thetime, so we check on her all the time, so many times we'll go in and she'll just be covered, so I have to wake her up, change her bedding, bathe her etc. I feel so bad for her, she doesn't understand what is going on. Anyway, now that I have rambled on like a mad woman, if anyone has any info about what kind of surgery they do, please e-mail, I am quite concerned for my sweetheart.

Contact Twila at Twila.Foulds@gems9.gov.bc.ca

Ask Jennifer

Dear Jennifer,

I am wondering if you help take care of your tube? Zachary is my son who is 5 1/2. He goes to school with his pump. At school he has to look at his dressing every time that he goes in to the bathroom. Then he has to tell his teacher if it is wet or dry. This is all he does by himself right now. That is fine with us. But if there was anything else that you could do all by yourself when you were 5 or 6 with your tube or your feedings we would like to know that. Thank you for offering to answer questions!

Anne Juhlmann

P.S. Zachary has something called pseudo-obstruction syndrome. That just means
that it is hard for him to keep food down unless we put it through his tube.

Dear Anne,

Thank you for writing to Jennifer. Here are some of her answers:

Q: I am wondering if you help take care of your tube?
A: Sometime. When I am in the bathroom, I check to see if it is leaking. I pick the crusty stuff off the button. If the flap opens up I close it because it can leak.

Q: Was there anything else that you could do all by yourself when you were 5 or 6 with your tube or your feedings?
A: I don't mix the formula, but I sometimes put the medicines in the formula. I push my medicines in the button. I know how to set my pump to run. I also have a central line for IV and I flush it after the IV.

Jennifer is 7 and can be reached at 254-965-2197 or st_msparks@tarleton.edu

Note: Articles are reviewed by Jennifer's Mom before publication.

Something Funny

You just awake... your eyes are still shut
Still cant quite focus.....still draggin' your butt
You know you need coffee......can taste that first sip
You wait for the maker.....and put the mug to your lip

The feeling is warm.... just what you need
But you know you need more....and its something to read
The paper you say??? no...dont think so.. not it...
Its much more exciting... you can't wait to "click"...

You boot up your 'puter.......you click that icon...
Can't keep from grinning.... you're really turned on!
When the voice says "Welcome"...your heart skips a beat!!
You know your addicted....all the friends that you'll meet.

And then you see it.......you wait with a stare....
The mail box lights up!! "you've got mail" waiting there!!
OH.. what a feeling!!.... you look with delight!
You hoped you'ld have mail.... and you knew you were right!!

So you go thru the mail..... knowing this is the "Best"..
Reading this reading that....as you go thru the rest.
Some you give the "delete" key....others get your first click
You know you must hurry......you gotta be quick!

It is then that you hear it.... You can't wait to see
Your heart gets a flutter... who's name will it be?
And then there it is..... covering part of the screen
The sweet little sound....Oh..you know what that means!!!

"Quick mail check" you promised....you said in your mind.
But you just got an IM.... and your pressing for time!
You know that you want to.... and respond you will
So you stop what your doing.. and go for the thrill!

You "LOL" and "BRB," give kisses and Hugs...
You type and send words... refilling your mug
You give your good friend your attention and time
So that quick little mail check... turns to hours online!

Recall Notice

Infant's Simethicone Drops, in 1 fluid ounce units, OTC, for relief of gas symptoms, antiflatulent, Made in the USA for Qualitest Products, Inc.
Recall #D-003-8. CODE: Lot No. 005B7A, EXP 2/99. MANUFACTURER: Vintage Pharmaceuticals, Inc., Huntsville, Alabama. RECALLED BY: Manufacturer, by letters dated August 4 and 21, 1997. Firm-initiated recall ongoing. DISTRIBUTION: Alabama. QUANTITY: 4,548 cartons were distributed. REASON: Product released without tamper evident neck band.

Note: if you are aware of a product recall of any goods or medications that you feel may be of use to the group, please share this information with Kimberly Robinstein (maniac@rnet.com) or Elizabeth Kohn (Kohns@prodigy.net)

Children's SSI Update

The Social Security Administration has made many drastic changes recently, discontinuing benefits for many children, and putting many children at risk for losing their benefits. The new childhood disability standar has effected primarily children withmental disorders and/or mental retardation. Reversal rates in terms of redetermination have skyrocketed from a standardd 10% to 58%, indicating that many families are losing and will continue to lose their appeals. Advocacy groups from around the nationhave met to discuss this disturbing trend. While the SSA has decided to review its appeals process, improper information and appeal denials are considered to be at fault for the loss of many children's benefits, there is still a serious threat to benefiteligibilities. Case summaries, which are a standard procedure for the SSA, consistently leave out important medical facts. Children lose benefits because parents are complying with treatments and are improving medically.

If your child's benefits have been terminated or denied, parents are advised to send a case narrative to Jonathan Stein, Community Legal Services of Philadelphia at (215) 981- 0436, fax or call (215) 981-3742. The case narrative should emphasize the seriousness of your child's condition and why you feel the decision was wrong. If someone is working with your family to appeal the SSA's decision, please direct them also to contact Jonathon Stein. Collect and forward any DDS decisions terminating or denying initial benefits and any administrative law judge (ALJ) decisions applying to the new rules to Tom Yates, SSI Coalition, 205 W. Monroe, Chicago IL 60606-5013, (312) 223-9518, fax or call (312) 460-8402. If possible please obtain and include the DDS form that shows the basis of denial.. If you would like to do state advocacy work with approaching state agencies that can notify families about their appeal rights when their children lose SSI benefits? Call Rhoda Schulzinger or Jonathan Stein.

Regional Support Parent Contact List

CJ Attion, Parent Advisor
cejay@pcpros.net

CJ is 28 and has Hirschsprung's is willing to help parents deal with the special issues of having Hirschsprung's.

Jennifer & Tim Beehler, Parent Advisor & Parent Liason
pooh@glasscity.net

Tim is an adult with long segment Hirschsprung's and they are raising a child with total colon Hirschsprung's and are interested in helping parents cope with raising a special child or older children with private issues.

Ramona and Hal Painter
Hal_Painter@prodigy.net

Hal and Ramona are raising a child with Hirschsprung's and are interested in helping parents with support and difficult parenting issues.

Leesa McDaniel

Leesa is raising a child with Hirschsprung's although she is offline she has been a tremendous help in raising awareness in her area and is willing to talk to parents about difficult parenting issues.

Melvin and Maritia Sparks
st_msparks@tarleton.edu

Melvin and Maritia are raising a child with short bowel syndrome, they are a wealth of information regarding parenting a child on tpn.

Ken and Kathy Sizemore
MKS2KSS@aol.com

Ken and Kathy are raising a child with total colon Hirschsprung's & GERD are interested in helping with whatever needs arise.

Disclaimer

Notice this newsletter is intended to report items of interest to persons that may be suffering from motility disorders, we neither promote nor recommend any treatment or therapy, etc. The relevance of anything printed in this newsletter to a particular person should be discussed by the family with their own physicians. Our hope is that this method of communication will promote communication between parents and foster support among families. All rights reserved. Copyrighted 1997.

Kimberly Robinstein for Guardian

The Guardian Newsletter online November 1997