Update on Deacon

At one point Hirschsprungs was so visual in our lives. We thought about it every day, sometimes multiple times. When he had his iliostomy, whenever he was sick in the hospital, through his 6 surgeries, everyday all day whenever we would change his bum, and in his struggle with fine motor skills. Now as I sit here and watch him playing with his 2 siblings out of 3, completely potty trained (except for at night) and growing like a weed. Its a few weeks past his 4th birthday.
Whenever I think about this blog, my stomache clenches. I feel so helpless. I know there are huuge amounts of people wanting to know more about him and Hirschsprungs in general. But I dont know how to reach them. To tell people about how good hes doing and what Ive learnt about the desease and how to fight it.

The endless waiting game.

So, now Deacon has had 6 surgeries. Dont worry, no more surgery since last post. BUT, We got home last time on the Wed and on the Friday we all (15 of us) took the Rocky Mountineer train up to Whistler. It was a family gift from my parents! We loved it! The boys had a blast, and it was a much needed rest. Little did we know how soon I would need more rest.

We went up there and that night we went out to eat at a very nice fancy restaurant with all 6 babies under 3! LOL! They were very well behaved. Everythign was going well, until 3/4 of the way through, Deacon started to whimper again. I thought, OH NO! Are we going to have to get an ambulance up to Whistler?? So I sat him on my lap and just held him. About 5 min later, I heard a plopping noise, I looked down and poo was POURING out the back of his diaper OVERFLOWING all over the floor, his pants, my pants... So Joe and I took him and gingerly ran him to the bathroom and cleaned him up. After that he was fine. I just thought maybe it was gas or something. The next morning, he was running around our hotel room and tripped and put his 2 front teeth through his bottom lip.

Gesh I know.

So we got home fine and everything continued as normal. Until that next friday. In the middle of the night I woke up to him screaming, I ran in there and there was throw up everywhere! So I called 911, and we got raced to Ridge Meadows. We got there, xrays done, and the DR there told me he was constipated. That on the xray was a huge lump in his bowels. I was like... Ya, no. He said its fine, he will pass it fine. We went home. He didnt poo all day and he started the whimpering that night, so I sent my hubby Joe (who has never taken Deacon to the hospital) to get the DR to give him an enima. He went there and the dr there said nope, nothing on the xray from last night, just go home. Or you can DRIVE him to CHILDRENS!

I WAS SOOOO ANGRY!!!

So Joe drove him home, and I drove him into Vancouver. Turns out it WAS entercolitis this time. ARG! So bowel rest for 4 days and then slowly giving him food again. Its sooo heart breaking, refusing your child food. All while you have to eat in the bathroom. :)

Im just so glad that my mom was there to help me.

So now the GI says I need to keep him on the Gentamiacin and he gave me stronger steriods for his bum to clear. Hopefully this will be it for awhile.

After a month Deacon has gained 8 lbs!! He is now 2 years old and 28lbs!! He has begun to speak alot more and hes getting better and better. :)

Holy CRAP!

So, I sat down a week and a half ago to write about Deacons latest adventure and I never finished it because I didnt know it wasnt finished...

Last month, almost exactly a year after he was hospitalized for the flu, he started to get sick again and we had to take him in again.
Within 24hrs he was very sick. He was lethargic and not pooing properly. We rushed him to Abbotsford hospital because thats where his Pedi Dr. Hewes works. As we were sitting in the waiting room he started to vomit. He vomited about 16 times in 4 hrs. They put him on bowel rest and gave him an IV. They also took and gave him an Xray. After that Xray, they said they were unsure but they thought he had a blockage. After 3 days, he was feeling better and the Xrays look good again. They said not a big deal, he just had the flu!

So on Thursday May 20th, he ate Cheerios for breakfast and had a perfectly normal poop. After that things just went downhill. He started to act a little different, not playing with Riley. Wasnt really hungry. At about 12:30 he asked to go to bed. After his 4 hr nap. He started whimpering. Very lethargic and we decided he needed to go in again. I go to the local walk in clinic becasue then I can decide which hospital to go to. If his Pedi is in at Abbotsford, then we go there. If its an Emergency we go to Ridge Meadows. If shes not in and its not an emergency we go straight to Childrens in Vancouver. So I go there and the Doctor there tells me hes totally fine, just ride it out. Whatever. So I ask them to page Dr. Hewes at Abby and I find out shes not working the weekend. Fine. I take him into the parking lot, strap him into his carseat and he starts vomiting everywhere. Hmmmm... this time it was 12 hrs that he started throwing up. Weird. I wait for him to stop, and end up waiting 45 min. When I get to Ridge Meadows he has been puking constantly. It takes the Pedi there 1 1/2hrs to finally IV him. He wouldnt stop asking questions and LISTEN to me! At that point, Deacon was limp in my arms from all the vomiting. I had to hold his neck while he puked. He kept on until he had thrown up 60 times in 2 hrs. The Pedi finally started listening after the Xray. The Xray was horrid. It showed big loops of distended bowel. So they shipped us by ambulance right to Childrens. They at first thought it was Enterocolitis and tried to treat him with Antibiotics on bowel rest for 3 days. We thought it was working and started to feed him. Within 24hrs he was puking again, so they preformed emergency surgery on him on Thursday the 27th. They took all his bowels out and he had a twisted bowel. We finally came home yesterday the 1st of June. Wheph! Thats really all I have to say right now...

My miracle baby!

Soooo much has happened.
We had a little baby girl on Nov 30th by c-section. She pooped on the surgeon. For those of you that dont know... thats VERY good news!

A few of you asked how we knew that this baby had Hirschsprung's. Well I researched it ALOT and found a little bit of info on in utero ultrasound showing dialation in the intestines. That could mean one of 3 things:

1. Hirschsprung's
2. An impacted bowel (twisted bowel)
3. imperferated anus (No bum hole)

So I went and had an ultrasound done @ BC Childrens Hospital and they indeed saw dialation.

We were devestated.

For weeks and weeks we just cried. I thought it was better that we knew before hand that the baby had HD. I thought it would get better the further we went along. That the pain would get less and less or something. That I would "come to terms" with the facts. Not so. I just spent the last 2 months of my pregnancy, making plans, crying, feeling bad, making different plans, crying, trying not to think about it, and you guessed it more crying.

I had 2 more ultrasounds done and at each one, they said it was not as bad as the last one. I didnt think anything about it as dialation can get worse and better. I wanted to think the worse, so I wouldnt be devestaed again.

So she was born... and she pooped. I only got to hold her for 30 min before they took her to the nursery to be hooked up. 4 hours later she came into my room and she stayed! No hospital visits, no IV's .... nothing!

It was quite hard because family and friends wanted a concrete answer. Does she have it or no? The only way to find out is a biopsy. So we couldnt give them an answer. I didnt want everybody yelling out, "Shes a miracle!!!" before I knew for sure. I didnt want everybody to say that and then find out 2 weeks later that she did infact have it. Lots of people didnt understand what I was doing. Infact people assumed that I was uncaring about it. I showed little emotion infront of people about her. I put up a stone wall. I didnt break. I couldnt break. I was trying to cope. It was so unbelieveable to think that God had given ME this gift. A perfect baby. It wouldnt have been THAT big of a deal if we hadnt seen it on an ultrasound. I SAW it! ME! With my own eyes. It was very obvious. And now... I had to accept the fact that she was fine, better than fine... perfect! So I had people ask me why I wasnt happy about it. Why I wasnt crying all the time about it. I just needed time. Time for it to sink in and time for me to realize that she was fine. And now that I do... I can post!

Other news... Deacon is doing super awesome! Hes growing and speaking and ON THE CHART! Hes finally in the 10th percentile for his height! Hes 20months old and can say "Daddy!"
:)

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????