New important information!!

So much has gone on with Deacon since my last post. First of all, they found out that the reason he was 12lbs at 10m was that he was missing his valve between his colon and small intestines. I guess no body thought to mention that that valve keeps all the bacteria in the colon. If bacteria gets in the small intestines then they block the receptors that absorb protein, and EVERYTHING else! So from when he was 4 m til he was 10m, Deacon didn't gain anything! Plus his measurements stayed the same! His head didn't get bigger and he didn't get taller. He stopped advancing physically as well. We didn't catch it because he started crawling at 4m. So we all thought he was super advanced. But after he started crawling, he didn't advance. So they put him on some pretty strong antibiotics to kill all the bacteria. So to counteract that Ive been giving him a protein shake 2x a day. Its got probiotics, protein, and a banana in it. It gives him something good! So since then, he now weighs 21lbs!! Hes 16m! He just started walking as well! We have found though that that set him back quite far. :( He doesn't speak at all.

In other news:

I'm pregnant with another baby with Hirschsprung's! They just found out on an ultrasound 2 weeks ago. We are very upset, but everything happens for a reason, right? The baby will be born around the end of Nov. So that means that we will be in the hospital for most of Dec. Booooo! So we are marching on, and I'm going to keep everybody much more updated!

Talk to you all soon!

The dreaded bleeding bum...

As parents of HD babies we all know about the diaper rashes. I didn't think it would be that bad when Deacon had his bag. I thought, ya I've seen "bad" diaper rash. Not a big deal. Except that it is.

He had his stoma closure and 4 days later the horrid red blotches sprang up, like little angry men. The nurses and doctors kept telling me before his surgery that after I would have to keep applying the barrier cream. There was nothing there and they kept saying, "You MUST apply it EVERY time!!!" I thought ya I will thanks. But nothing prepared me for those angry men. No body told me what to do AFTER the rash appeared. I threw out the barrier creme and started a LLLOONNGG battle with the baby aisle in the grocery store. We spent alot of money trying out everything under the sun. Everybody who had HEARD of a thing called a baby had an opinion on what cream was the only one to buy. Weeks of trying anything that promised any sort of bum cream, came around to me taking him to the clinic. First visit - try Nyastain. Yeast cream. Try for 1 week. The rash will go away in 2 days. Riiiiight. I used it for 2 weeks and nothing. There was a gizzilion little angry men on my baby's bum and 1 SUPER angry baby on his face while I wiped the poop off his toosh. So the 2nd trip to the clinic, the doctor took one look at his bum and prescribed a cream that she had to call 2 other doctors to make sure it was okay to give him. Sorry I threw it out so I dont know what it is. But I used it and there was NO sign of any change good or bad. Then he got the flu and was addmitted to BC Children's Hospital. When we were in there a Dr. Rothstein told me that gastrointestinal patients had been given a special mixture cream. We tried it, and since he wasnt eating (therefore not pooping) his rash went away!! When we left the hospital we got a HUGE tub of this cream and we love it! Its Nyastatin cream, Zinc Oxide cream and 2% Hytrocortizon. We have been using it for a month now. The rash did come back but that was to be expected. It has been the only cream that has worked at all. The only thing is that I know Hytrocortizon is very bad. Im going to the doctors office on Friday and Im going to ask her about it. Weird white skin patches have started to appear on his rash. I've reaserched it abit and have found some interesting articles about it. One thing that Im going to try after Im finished tonite is putting Tea Tree Oil on it. Iv'e heard that it works amazing. But Iv'e heard that sooo many times...

Topical steroids
Topical steroids have revolutionized the practice of dermatology since they were introduced in the late 1950s. They are effective anti-inflammatory preparations used to control eczema/dermatitis and many other skin conditions.
Like all medications, topical (cortico)steroids are associated with potential adverse effects (side effects) especially if they are used incorrectly.
The topical steroids can be divided up into four groups according to their strength. As a general rule, use the weakest possible steroid that will do the job. However, sometimes it is appropriate to use a potent preparation for a short time to make sure the skin condition clears completely.

Class 1
Very potent (up to 600 times as potent as hydrocortisone)
Clobetasol propionate (Dermol™ Cream/Ointment
Betamethasone dipropionate (Diprosone™ OV Cream/Ointment)

Class 2
Potent (I50-100 times as potent as hydrocortisone)
Betamethasone valerate (Beta™ Cream/Ointment/Scalp Application, Betnovate™ Lotion/C Cream/C Ointment, Daivobet™ 50/500 Ointment, Fucicort™)
Betamethasone dipropionate (Diprosone™ Cream/Ointment)
Diflucortolone valerate (Nerisone™ C/Cream/Fatty Ointment/Ointment)
Hydrocortisone 17-butyrate (Locoid™ C/Cream/Crelo Topical Emulsion/Lipocream/Ointment/Scalp Lotion)
Mometasone furoate (Elocon™ Cream/Lotion/Ointment)
Methylprednisolone aceponate (Advantan™ Cream/Ointment)

Class 3
Moderate (2-25 times as potent as hydrocortisone)
Clobetasone butyrate (Eumovate™ Cream)
Triamcinolone acetonide (Aristocort™ Cream/Ointment, Viaderm KC™ Cream/Ointment, Kenacomb™ Ointment)

Class 4
Mild
Hydrocortisone 0.5-2.5% (DermAid Cream/Soft Cream, DP Lotion-HC 1%, Skincalm, Lemnis Fatty Cream HC, Pimafucort Cream/Ointment)

Topical steroids are also available in combination with salicylic acid to enhance penetration, and with antibacterial and antifungal agents.

Skin absorption of topical steroids
Steroids are absorbed at different rates from different parts of the body. A steroid that works on the face may not work on the palm. But a potent steroid may cause side effects on the face. For example:
Forearm absorbs 1%
Armpit absorbs 4%
Face absorbs 7%
Eyelids and genitals absorb 30%
Palm absorbs 0.1%
Sole absorbs 0.05%

Side effects of topical steroids
Internal side effects
If more than 50g of clobetasol propionate, or 500g of hydrocortisone is used per week, sufficient steroid may be absorbed through the skin to result in adrenal gland suppression and/or eventually Cushing's syndrome.
Adrenal Gland Suppression. Topical steroids can suppress the production of natural steroids, which are essential for healthy living. Stopping the steroids suddenly may then result in illness.
Cushing's Syndrome If large amounts of steroid are absorbed through the skin, fluid retention, raised blood pressure, diabetes etc. may result.

Skin side effects
Local side effects of topical steroids include:
Skin thinning (atrophy) and stretch marks (striae).
Easy bruising and tearing of the skin.
Perioral dermatitis (rash around the mouth).
Enlarged blood vessels (telangiectasia).
Susceptibility to skin infections.
Disguising infection e.g. tinea incognito.
Allergy to the steroid cream.

The risk of these side effects depends on the strength of the steroid, the length of application, the site treated, and the nature of the skin problem. If you use a potent steroid cream on your face as a moisturiser, you will develop the side effects within a few weeks. If you use 1% hydrocortisone cream on your hands for 25 years, you will have done no harm at all (except for having wasted a lot of money!)
Bruising
Skin thinning
Prominent capillaries
Stretch marks
Adverse effects of topical steroids

How to use topical steroids
Ask for specific instructions how to use your topical steroid(s). See DermNet's information about fingertip units. Which one, where, when, how often and for how long? Cream, ointment or lotion? This is particularly important if:
You are using strong steroids over large areas of your body.
You have been asked to use plastic to cover treated areas (occlusion).
Your skin condition persists for more than two or three weeks.
You are a child.
Topical steroids are very effective medications. They work by reducing inflammation, and when used correctly are very safe. They should not be used as bleaching creams.
Apply topical steroids only to the areas affected by the skin disease, and generally only once or twice daily. If your skin is dry, apply an emollient frequently.

This is a part of an article on Rxlist.com about topical steriods.

General
Systemic absorption of topical corticosteroids has produced reversible hypothalamic-pituitary-adrenal (HPA) axis suppression, manifestations of Cushing's syndrome, hyperglycemia, and glucosuria in some patients.
Conditions which augment systemic absorption include the application of the more potent steroids, use over large surface areas, prolonged use, and the addition of occlusive dressings.
Therefore, patients receiving a large dose of a potent topical steroid applied to a large surface area or under an occlusive dressing should be evaluated periodically for evidence of HPA axis suppression by using the urinary free cortisol and ACTH stimulation tests. If HPA axis suppression is noted, an attempt should be made to withdraw the drug, to reduce the frequency of application, or to substitute a less potent steroid.
Recovery of HPA axis function is generally prompt and complete upon discontinuation of the drug.
Infrequently, signs and symptoms of steroid withdrawal may occur, requiring supplemental systemic corticosteroids.

Children may absorb proportionally larger amounts of topical corticosteroids and thus be more susceptible to systemic toxicity (See PRECAUTIONS-Pediatric Use).
If irritation develops, topical corticosteroids should be discontinued and appropriate therapy instituted.

In the presence of dermatological infections, the use of an appropriate antifungal or antibacterial agent should be instituted. If a favorable response does not occur promptly, the corticosteroid should be discontinued until the infection has been adequately controlled.

Laboratory tests
The following tests may be helpful in evaluating HPA axis suppression: Urinary free cortisol test; ACTH stimulation test.
Carcinogenesis, Mutagenesis and Impairment of Fertility
Long-term animal studies have not been performed to evaluate the carcinogenic potential or the effect on fertility of topical corticosteroids.
Studies to determine mutagenicity with prednisolone and hydrocortisone have revealed negative results.

Pregnancy
Teratogenic Effects-Pregnancy Category C. Corticosteroids are generally teratogenic in laboratory animals when administered systemically at relatively low dosage levels. The more potent corticosteroids have been shown to be teratogenic after dermal application in laboratory animals. There are no adequate and well-controlled studies in pregnant women on teratogenic effects from topically applied corticosteroids. Therefore, topical corticosteroids should be used during pregnancy only if the potential benefit justifies the potential risk to the fetus. Drugs of this class should not be used extensively on pregnant patients, in large amounts, or for prolonged periods of time.

Nursing Mothers
It is not known whether topical administration of corticosteroids could result in sufficient systemic absorption to produce detectable quantities in breast milk. Systemically administered corticosteroids are secreted into breast milk in quantities not likely to have a deleterious effect on the infant. Nevertheless, caution should be exercised when topical corticosteroids are administered to a nursing woman.

Pediatric Use
Pediatric patients may demonstrate greater susceptibility to topical corticosteroid-induced hypothalamic-pituitary-adrenal (HPA) axis suppression and Cushing's syndrome than mature patients because of a larger skin surface area to body weight ratio.
Hypothalamic-pituitary-adrenal (HPA) axis suppression, Cushing's syndrome, and intracranial hypertension have been reported in pediatric patients receiving topical corticosteroids. Manifestations of adrenal suppression in pediatric patients include linear growth retardation, delayed weight gain, low plasma cortisol levels, and absence of response to ACTH stimulation. Manifestations of intracranial hypertension include bulging fontanelles, headaches, and bilateral papilledema.
Administration of topical corticosteroids to pediatric patients should be limited to the least amount compatible with an effective therapeutic regimen. Chronic corticosteroid therapy may interfere with the growth and development of pediatric patients.

Weight and how it haunts me.

So, to continue this story and help others learn more (as well as myself). I have asked several people to share their stories as well. I can only tell what I know about my son. They have agreed which Im SOOO excited about! I think I would like them to email me posts they want to post and I will post them with their names on it. Their comments to me are the REASON I wanted to do this. If you are reading this and you have a story to share PLEASE email me so I can post it!! Im going to try posting alot of different topics and things I have tried, if you have a comment please share!



Weight Gain/Losses



When Deacon was born, he weighed 7lbs 13ozs. When he left the hospital a month later, he weighed the same. My pediatrician took his blood several times and found out that he was very low on salt. You can't gain weight without salt. So she put him on saline 4x a day. That went on for 1 month. He gained 3 lbs. It was crazy fast! Then they took him off of it because it was going too fast and they were hoping he would just continue gaining. Well he did for a while. At 4 months old, he weighed 12lbs. It was great! It was like he was a normal child. Then we went to his 5 month check up and found out he hadn't gained and she told me to start feeding him food. He was so not ready. He would scream when I tried to feed him. It was awful. But whenever I would go back to the surgeon or the ped they would weigh him and he would be 12lbs. I refused to feed him when he was 6 months. He was getting a complex. lol. We just amped up him formula to 24 calorie and mixed it with baby cereal. We did that because the formula was going right through him. So acidic. He was 12lbs even when they gave him his stoma closure surgery. That was on Jan 16th, 2009. He was 8 months. After that surgery he gained 2 lbs in 2 months. We were so happy! We had started feeding him again solids right after his surgery. And he loved them, finally. He actually ate real meals. We were told to feed him high fat foods, like potatoes with salt and butter. But I have found that if it has fats and or oils in it that it goes through faster. Therefore hes not getting anything from it. I have found that breads are great! They actually slow down his food and make it thicker. So for now, we are feeding him everything that we eat. I have been thinking and looking for something more for him. Something that will hopefully replace all the nutrients in his formula. I would like to take him off formula at a year. But with all the articles I have been reading about lactose intolerance in HD babies, Im thinking about putting him on almond milk. I went and saw a natural path the other day and she gave me a protein shake for children. Its called Ultra Care for Kids. Its got lots of great stuff for kids trying to gain weight. The only thing is that because he has no colon (which absorbs protein) Im not sure how much if any of it he absorbs. The reason I say that is because whenever he takes it his poos go very liquidity. Im not sure on what to do now. Do I give him more than I have been hoping that he absorbs more because he's eating more? Or do I give up because Im throwing my money away and he's not getting enough to warrant giving it to him? It is also making him very full. He dosent eat as much as usual. Im not sure what to do.
Plus because he is missing the valve that holds the bacteria in his colon, he has alot of bacteria. So the GI prescribed antibiotics. I go and see him at the end of this month. Im not sure I want to continue with them. Im pretty sure he wants to have him on them for a long time. So instead I've been giving him probiotics. I give him the antibiotics in the morning and night and I give him the probiotics at noon. Im trying to keep a well watchful eye on him to see what they do. Any thoughts????

My little Panda Bear...

My son Deacon was born on May 23rd 2008 at 7:15pm. He was a beautiful blue eyed baby and he weighed 7lbs 12ozs. I BF him once right after he was born and again at around 11pm. His last feeding was at 4am that next morning. He was very lethargic and kept throwing up a little bit now and again. That day I kept telling the nurses that something was wrong. My husband Joe has Hirschsprung's as well and we knew that our children could get it as well. Finally at 11pm that night our doctor sent us to the Children's Hospital in Vancouver. She had taken an Xray and seen the big loops of his intestines. He was then diagnosed 3 days later with Hirschsprung's. They had done a biopsy to find it. So they scheduled his surgery to be for June 4th. They told us, the surgery might be a Pull-through or he might have to have a colostomy bag. It all depended on the biopsies they would preform during the surgery to see how extensive it was. 5 hours later they came out and told us that he had long segment and that he was so extensive that he needed an iliostomy bag because he had no colon left. That was really hard to take. We spent the next 2 weeks at the hospital waiting to be released. They finally released us a few days before his 1 month birthday!
He had his iliostomy bag for 5 months before his surgery was scheduled to be in Nov. They went in and it was another 5 hour surgery. When they came out this time they told us they had preformed reconstructive surgery on his small intestines. They had connected them lengthwise together with a piece of his old colon. They did this so that he could better absorb water, salt and protein. But it was so extensive that they couldn't remove his stoma. So they had put in another one on the other side. Sucky!! So he had that one until his Jan 16th surgery. His stoma closure!! Yaa!! That surgery was only 1 hour long! But it was hard for him to adjust so we ended up spending an extra 5 days in the hospital. He was screaming from the gas pain! He had never had gas before! So after that surgery I started looking at him like he was a normal boy. I didn't want to think about potty training, or anything else. I just wanted to focus on my two sons.
On March 17th, I had to rush him to the ER at Children's because he had the flu and he had lost 2lbs in 2 days! They told me later that his kidney's had almost failed! He had been very close to dying.
So that's why I wanted to start this. I was hoping that other parents could give me advise and I could tell them what has been working for me. Right now Deacon is 10 months old and he weighs 15lbs. From the age of 4 months to 10, he weighed the same, 12 lbs. That's scary! So right now I'm trying different things to get him to gain weight.
I also know that 80% of Hirschsprung's is short segment. I know that Deacon is in 5% of Hirschsprung's. I'm looking for other blogs with other types of Hirschsprung's so that if you have a question, you can find it here! We need to stick together and talk about it! If Ive gone through it and it hasnt worked, maybe you need that information!
Anyways... comments are always loved!!

The facts

Hi! My name is Megan and I have a 10 month old boy with long segment Hirschsprung's. I have decided to start this blog to give people somewhere to learn more about it. When he was first diagnosed I looked around on the web and I couldn't really find something that answered my questions. So I read medical research papers. Now I need other parents who have been through what I have (or something like it) to post their stories. My hope is that parents will come here to figure out how to help their child through Hirschsprung's and debate what to do or not to do. I would LOVE your thoughts and input! How is YOUR child doing?

First I would like to explain more about the small intestines and colon.


1. The esophagus is a long muscular tube, which moves food from the mouth to the stomach.

2. The abdomen contains all of the digestive organs.

3. The stomach, situated at the top of the abdomen, normally holds just over 3 pints (about 1500 ml) of food from a single meal. Here the food is mixed with an acid that is produced to assist in digestion. In the stomach, acid and other digestive juices are added to the ingested food to facilitate breakdown of complex proteins, fats and carbohydrates into small, more absorb able units.

4. A valve at the entrance to the stomach from the esophagus allows the food to enter while keeping the acid-laden food from "refluxing" back into the esophagus, causing damage and pain.

5. The pylorus is a small round muscle located at the outlet of the stomach and the entrance to the duodenum (the first section of the small intestine). It closes the stomach outlet while food is being digested into a smaller, more easily absorbed form. When food is properly digested, the pylorus opens and allows the contents of the stomach into the duodenum.

6.The small intestine is about 15 to 20 feet long (4.5 to 6 meters) and is where the majority of the absorption of the nutrients from food takes place. The small intestine is made up of three sections: the duodenum, the jejunum and the ileum.

7. The duodenum is the first section of the small intestine and is where the food is mixed with bile produced by the liver and with other juices from the pancreas. This is where much of the iron and calcium is absorbed.

8. The jejunum is the middle part of the small intestine extending from the duodenum to the ileum; it is responsible for digestion.

9. The last segment of the intestine, the ileum, is where the absorption of fat-soluble vitamins A, D, E and K and other nutrients are absorbed.

10. Another valve separates the small and large intestines to keep bacteria-laden colon contents from coming back into the small intestine.

11. In the large intestines, excess fluids are absorbed and a firm stool is formed. The colon may absorb protein, when necessary.


Deacon has no colon. He is missing #11. "Can I get a number 11?!!"

He is also missing # 10, and a part of #9. He also has no appendix. So in medical terms he has total colonic aganglionosis. Which means, total loss of colon. Or something like that...



Just for some facts Ive grabbed a research article following Deacon's type of Hirschsprung's:



A 32-year retrospective review from 1972 to 2004 analyzed complications and long-term outcomes in children with total colonic aganglionosis (TCA) as they relate to the procedure performed.






Results
Thirty-six patients (27 boys, 9 girls) had TCA. The level of aganglionosis was distal ileum(the point of connection between the small and large intestines.) (26), mid–small bowel (8), midjejunum (1), and entire bowel (1).

Enterostomy was performed in 35 of 36. Eight developed short bowel syndrome. Twenty-nine (81%) had a pull-through at 15 ± 6 months (modified Duhamel 20, Martin long Duhamel 4, and Soave 5). Six had a Kimura patch.

Postoperative complications (including enterocolitis) were more common after long Duhamel and Soave procedures.

Seven (19%; 2 with Down's syndrome) died (3 early, 4 late) from pulmonary emboli (1), sepsis (1), fluid overload (1), viral illness (1), liver failure (1), arrhythmia (1), and total bowel aganglionosis (1).

Mean follow-up was 11 ± 9 years (range, 6 months-29 years). Twenty-four (83%) of 29 patients exhibited growth by weight of 25% or more, 21 (91%) of 23 older than toddler age had 4 to 6 bowel movements per day, and 17 (81%) of 21 were continent.

In 5 of 6, the Kimura patch provided functional benefit with proximal disease.

Conclusion
Long-term survival was 81%. The highest morbidity occurred with long Duhamel or Soave procedures. The modified Duhamel is our procedure of choice in TCA. Bowel transplantation is an option for TCA with unadapted short bowel syndrome.

I know that's alot of information. But it has helped me KNOW what is going on inside my son. I wanted to start with some facts, so now I will tell you a little about Deacon. :)