9 year-old son born with omphalocele, cloacal exstrophy, spina bifida, hip dysplasia, has iliostomy, incontinent, but man...what a joy!!

Sara - posted on 02/18/2009 ( 7 moms have responded )

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Hello, I am a mother to 3 sons. Our middle son, William, was born in Jan. of 2000. He is in the 3rd grade at a public school. Ironically all of his special needs are physical. His IQ is higher than most kids his age, he reads at a 6th grade level and is very witty. His body just cannot keep up with his mind. He is a great kid who has a very strong will...sometimes that is good considering his situation....and other times it gets him into a lot of trouble with mom and dad!! lol



He has multiple birth defects, and ironically I know of only one other person with the similar complex, known as OEIS Complex. It is very rare to have all of these birth defects together. To explain: Omphalocele: His liver and most of his intestines were on the outside of his body when he was born. Exstrophy: He has cloacal exstrophy which includes the bladder and sexual organs. Spina Bifida: Lypomyelocystocele: A fatty cyst on the bottom of his spine, it was skin covered and the nerve damage effects the use of his shin on down. He uses crutches and a wheelchair, but prefers to crawl...much to our dismay....and his, because he gets in trouble with us when he crawls. He does not have a shunt because hydrocephalus is rare with his form of spinal bifida. He has severe hip dysplasia and has had surgeries to start correcting it....will need more. He has a iliostomy bag which will be there for life and is also missing most of his colon and he does not have a rectum or a sphincter muscle, nor does he have any control with his urine. He has a peg tube but we haven't used it in years...they are afraid to remove it because it was so hard to find a port....his insides aren't exactly located where they should be. He didn't start to eat orally until he was 3 and before that we used NG tubes. We went through a stent of malnutrition too....he then had the disorder of pica...and chewed most of his fingers off ( the tips). Because of this three fingers on his left hand are now clubbed, and even though we found out why he was malnutritioned chewing his fingers has become a habit...one that we still try to break! The doctors found that his pancreas does not breakdown fats and proteins, so now he is on an enzyme, along with an iron supplement.



He has had a half dozen surgeries and has many more to come. He is little for his age weighing around 30 pounds and is about the height of a 3-4 year-old. Our youngest son, who is 7, towers over him now! We spent the first few years of our life in the hospital and now it's not as often. I don't push to get surgeries done unless it's absolutely needed. There is a surgery out now that could make him dry....not needing to wear pull-ups anymore. There is no risk for him to stay as he is (being wet), however, there is always a risk, whether it be small or big, to doing the surgery. Right now it doesn't bother us that he has to wear pull-ups (except for the cost - we just lost medicaid so now we have to pay for them...I am working on finding another outlet) so we decided that when he is of an age to make a decision he can make it for himself.



Being a mom of a disable child and having a family with a disable member can be difficult at times. I have a great husband and he is a lot of support. Our goal has and always will be to raise all of our children the same. They all get loved on, disciplined, are involved with activities and friends.



I am out there for anyone who may just need someone to chat with....I am especially looking for parents out there that may have some or all of the same birth defects that my son has. It is very rare. If you are interested in learning more about Wil, you may visit my blog at www.perfectwil.blogspot.com it has detailed information about our/his story and pictures too! I haven't updated it in awhile...I think it goes until age 3-4....I guess I will have to get on that!! Thanks for reading and I hope to hear from anyone who may have questions or just want to chat!!



Sara

Fort Wayne, Indiana

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Jill - posted on 04/15/2011

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Hi Sara,
Your story you told of your son is very inspiring to me. Last week at my mid pregnancy ultrasound, my husband and I were told our son has severe spina bifida (mylemeningocele), an omphalocele with his liver, bilateral club feet, chiari malfomation, single artery umbilical cord and possibly other trouble with other internal organs like the bladder. They thought it was Trisomy 18, but after receiving FISH results from an amniocentisis, it is not that. We are still waiting on results from the full amnio and other genetic testing. They dont know what this is or what caused it. These past two weeks of uncertainty and waiting have been very hard. My husband is a great support and we are both hopeful for our little guy but we are so worried. It is so hard not to spend my day researching the internet for answers, but I know my 2 year old son needs me right now too. I am not sure what questions to even ask you, but your post and your blog were inspiring and gave me hope and insight on what may lie ahead for us. You seem like such an amazing mom and what a great tribute to your son your blog is. Thank you.
-Jill

Jill - posted on 04/15/2011

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Hi Sara,
Your story you told of your son is very inspiring to me. Last week at my mid pregnancy ultrasound, my husband and I were told our son has severe spina bifida (mylemeningocele), an omphalocele with his liver, bilateral club feet, chiari malfomation, single artery umbilical cord and possibly other trouble with other internal organs like the bladder. They thought it was Trisomy 18, but after receiving FISH results from an amniocentisis, it is not that. We are still waiting on results from the full amnio and other genetic testing. They dont know what this is or what caused it. These past two weeks of uncertainty and waiting have been very hard. My husband is a great support and we are both hopeful for our little guy but we are so worried. It is so hard not to spend my day researching the internet for answers, but I know my 2 year old son needs me right now too. I am not sure what questions to even ask you, but your post and your blog were inspiring and gave me hope and insight on what may lie ahead for us. You seem like such an amazing mom and what a great tribute to your son your blog is. Thank you.
-Jill

Rhianna - posted on 12/28/2009

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my son was born with classic BE. I was actually going to get you in contact with miss Brandy, but she posted right above me!!! HEEHEE

Brandy - posted on 12/12/2009

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MY SON WAS BORN WITH OCEIS!!!!

Heather - posted on 09/09/2009

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Hi Sara, My son is 9 and was born in 2000 also with extrophy of the baldder and hip dysplasia. Hes actually above average in his weight and height he current weighs 130lbs.and is 5'1 at 9years old hes had 9 surgerys and many more to come he can be dry for long periods of time . He developed an anxiety disorder around the age of 4 and would throw tantrums when he had to go to doctors ..Which doctors say this is a normal thing.He conts. to have behavioral problems at school though, but makes straight A's His bladder and hip dyspiasia was repaired at 1 week old he was in a full body cast for 2 months but now he walks perfectly normal unless u would know he was born that way and has had deflux injected in the bladder neck several times it seems to help with the incontience.. Good luck to u and ur family in all u do :)

Heather
St. Louis, Missouri

Sarah - posted on 08/15/2009

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Hi Sara! My name too is Sarah, and I also have a son born with OEIS. It is very rare to find other children with this condition. You are the 4th we have found. We live in Michigan and my son is a "frequent flyer" to University of Michigan hospital. He just had his second hip surgery this summer for hip dysplasia and also a tendon release surgery while he was under. This was, I believe, his 9th surgery. He also has all of the same symptoms as your son, but also had bilateral club feet, and a non/low functioning right kidney. He has "stable hydrocephalus" so he doesn't have a shunt either, but goes in for an MRI every 6 months just to make sure the ventricle size isn't changing. The form of spina bifida he has was called a myelocystocele, where it looked like he had a basketball on his back. They did not do the closure surgery until he was 6 weeks old because he was healing from his hip/bladder/colon/kidney/colostomy surgery that was performed at 1 1/2 days old. He has AMAZING doctors at U of M...I can't say enough about them. My son too is above average for intelligence, and talks circles around kids his age. His fine motor skills are amazing...at 2 1/2 he can put his braces on his legs by himself! But his body too is lagging behind what he wants it to do. He is in therapy 3 days a week, and is learning to walk with a tiny 14" reverse walker. But he is a ways away from walking on his own. He is such an awesome kid and has the coolest personality--he charms everyone everywhere we go :) I've heard that about OEIS kids...our urologist specializes in cloacal exstrophy kids and he just loves them and says that they all have such great personalities...I tend to agree! I also have a daughter who is 5 and starting kindergarten in the fall. I'm so sorry to ramble on so much, but it's so rare to find someone else with the same condition! I have a website for my son that I started back when I was pregnant, if you want to check it out. It is www.caringbridge.org/visit/babybowman. Are you members of ABC? (Association for the Bladder Exstrophy Community) That has also been a great resource for us too! Well, have to get the kids dinner. We've been out at the Woodward Cruise (a big thing here in Michigan with classic cars) and my husband is still there. Time for dinner! Hope to hear from you!

Sarah
New Baltimore, Michigan

Abby - posted on 02/20/2009

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My daughter is alomost 6 months old. It was so crazy to me reading what you had written, because my daughter has a lot of the same disabilities as your son. Just like William, she was born with omphalocele which they fixed when she was 2 days old. She was born with a cloacal extrophe, her bladder and some intestine is still on the outside. We recently put in tissue expanders to try to prepare for getting the bladder back inside, but one was infected so we had to get it taken out. We are still in the hospital, and they are thinking of taking the other one out too, because it has started looking infected yesterday and today. She was also born with imperforate anus and tethered spinal chord. As far as we can tell, she has complete control of her lower limbs, and she has no mental disabilities. We have had 3 surgeries so far. She also has hip displasia. I have not heard of OEIS. Noone has mentioned that to me so far. What is it? It seems my daughter may have something very similar to that.