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Location: Wilkes Barre PA | What is VATER association?
VATER or VACTERL association is an acronym used to describe a series of characteristics which have been found to occur together. V stands for vertebrae, which are the bones of the spinal column. A stands for imperforate anus or anal atresia, or an anus that does not open to the outside of the body. TE stands for tracheoesophageal fistula, which is a persistent connection between the trachea (the windpipe) and the esophagus (the feeding tube). R stands for renal or kidney anomalies and radial or arm anomalies. C is added to the acronym to denote cardiac anomalies, and L is often added to stand for limb anomalies. Babies who have been diagnosed as having VATER association usually have at least three or more of these individual anomalies. There is a wide range of manifestation of VATER association so that the exact incidence within the population is not exactly known.
What are the heart problems seen with VATER association?
Up to three-quarters of patients with VATER association have been reported to have congenital heart disease. The most common heart defects seen with VATER association are ventricular septal defects, atrial septal defects and Tetralogy of Fallot. Less common defects are truncus arteriosus and transposition of the great arteries. Babies may have a murmur at birth, however absence of a murmur does not rule out congenital heart disease. If a baby is suspected of having features of VATER association, consultation with a pediatric cardiologist is recommended.
What other defects are seen with VATER association?
Vertebral anomalies, or defects of the spinal column, usually consist of small (hypoplastic) vertebrae or hemivertebra where only one half of the bone is formed. About 70 percent of patients with VATER association will have vertebral anomalies. In early life these rarely cause any difficulties, although the presence of these defects on a chest x-ray may alert the physician to other defects associated with VATER. Later in life these spinal column abnormalities may put the child at risk for developing scoliosis, or curvature of the spine.
Anal atresia or imperforate anus is seen in about 55 percent of patients with VATER association. These anomalies are usually noted at birth and often require surgery in the first days of life. Sometimes babies will require several surgeries to fully reconstruct the intestine and anal canal.
Esophageal atresia with tracheo-esophageal fistula (TE fistula) is seen in about 70 percent of patients with VATER association, although it can frequently occur as an isolated defect. Fifteen percent to 33 percent of patients with TE fistulas will also have congenital heart disease. However these babies usually have uncomplicated heart defects, like a VSD, which may not require any surgery.
Renal or kidney defects are seen in approximately 50 percent of patients with VATER association. In addition, up to 35 percent of patients with VATER association have a single umbilical artery (there are usually two) which can often be associated with kidney or urologic problems. These defects can be severe with incomplete formation of one or both kidneys or urologic abnormalities such as obstruction of outflow of urine from the kidneys or severe reflux (backflow) of urine into the kidneys from the bladder. These problems can cause kidney failure early in life and may require kidney transplant. Many of these problems can be corrected surgically before any damage can occur.
Limb defects occur in up to 70 percent of babies with VATER association and include absent or displaced thumbs, extra digits (polydactyly), fusion of digits (syndactyly) and forearm defects. Babies with limb defects on both sides tend to have kidney or urologic defects on both sides, while babies with limb defects on only one side of the body tend to have kidney problems on that same side.
Many babies with VATER are born small and have difficulty with gaining weight. Babies with VATER association, however, do tend to have normal development and normal intelligence.
What causes VATER association?
No specific genetic or chromosome problem has been identified with VATER association. VATER can be seen with some chromosomal defects such as Trisomy 18 and is more frequently seen in babies of diabetic mothers. VATER association, however, is most likely caused by multiple factors.
What if my baby is diagnosed with VATER association?
The important thing is to identify all of the possible associated defects and treat them accordingly. Unless there are several very severe defects, babies with VATER association do well and can lead normal productive lives.
What is VATERS??:
VATERS which is also referred to as VACTERAL association. It is a association characterized by the sporadic, nonrandom association of specific abnormalities. Each letter stands for a specific abnormality.
V: Vertebral dysgenesis
A: Anal atresia
C: Cardiac anomalies
T-E: Fistula +/- esophageal atresia
R: Renal or Radius anomalies
L: Limb anomalies A person with more than two problems in any combination may be recognized as fitting in the VATER Association. However, VATER Association is not a diagnosis. There is no one cause for the association of these problems in individuals.
Frequently asked questions
If VATER Association is not a diagnosis, why label my child with it?
The VATER acronym is a tool for physicians. These groupings of problems tend to occur together more often than can be explained by chance. If a physician sees at least two problems that fit within the VATER Association, he/she knows to look for other related problems.
Problems that are identified early can often be fixed with surgery or treated with medicines or other therapies before your child has major complications. Therefore, several different specialists may see your child and have several different tests done. Possible diagnoses to explain the associated problems in your child will be explored. However, a specific diagnosis is not identified in the majority of children who have two or more of the associated problems.
What can I expect for my child who has problems that fit within the VATER Association?
Children who have VATER associated problems may have delayed growth and development in the early years. The majority of children have normal intelligence. The specific needs for each child who has associated problems vary. One child may have heart, kidney and spine problems. Another child may have a missing or unusually formed thumb and a tracheal esophageal fistula. Comparisons between the two children cannot be made.
For this reason, you will need to talk to your child's specialists to know what to expect. Write down questions before you meet with each physician. Make sure he/she answers each question or concern. If you do not understand, say so and ask him/her to repeat what was said in simpler terms.
Can this happen in future children?
Sometimes VATER Association problems can be due to known syndromes. If this is the case with your child, a genetic professional can discuss the chances of the syndrome happening again.
In most cases, a cause cannot be found which explains the VATER Association problems. Such associated problems do not appear to run in families. No specific drug, chemical, radiation or other environmental factor has been shown to cause the associated problems. Parents can often be reassured that nothing they did or didn't do during pregnancy caused this to happen in their child.
Without a genetic diagnosis, the chance of VATER Association problems occuring in future pregnancies is low. This is also true for the offspring of the child who has VATER Association problems.
Can this be detected before the child is born?
Many of the VATER Association problems can be detected by a level-two ultrasound. It can be done as early as 18 weeks in pregnancy. The unborn child's bony structures and major organs are closely examined with the ultrasound. Talk with your obstetrician if you are interested in having this done.
Google Search on VACTERL/VATER:
http://www.google.com/search?hl=en&lr=&q=VACTERL+VATER+ASSOCIATION
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(Edited by Arlene "Zoe's Mom" on 5/26/2002 1:32:44 PM) |
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Expert
Posts: 3132
Location: Albany, NY | Hey, Arlene,
I actually downloaded this the other day for Julia's kindergarten teacher to put in her record next fall. Thanks ! |
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Elite Veteran
Posts: 1183
Location: Beverly, MA | Good idea Laura. I always give Matthew's teachers his official "resume" of problems, treatments, medications, etc. He can look pretty frightening on paper. I think I'll download the above and attach it so that they can get more of an overview of VACTERL. The more people we educate, the better off our children will be! |
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Expert
Posts: 2770
Location: Oceanside, CA | Hey Arlene,
This is so nice to have. I had my husband print it out for us, we're going to give it to the people at his work so they can understand more on Tims condition. |
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Elite Veteran
Posts: 831
Location: deep in the heart of texas | Hey everyone..i also downloaded this and sent it to my mom...she doesn't understand that Dylan is special..like at the dinner table,she thinks Dylan should be eating all table foods raight now..he still gags alot..hes 12 months gonna be 13 on the 7 of July..he is just now on whole milk..she says when she looks at him it so hard to understand that he is different that he looks " normal" to her..not on the inside outside excecpt the bagbut not on inside maybe this would show her...thanks for this ,,,i do appreciate it..Carla~Dylans mommy~ |
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Expert
Posts: 1904
Location: Australia | I printed it out and ended up giving copies of it to any new doctors I see, so I dont have to sit there for about 1/2 hour trying to explain what it all is! |
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New user
Posts: 1
Location: Tulsa OK | OK - sounds like you are pretty well versed on VATER. I'm impressed and cannot tell you how much help all of this would have been when my son was younger. He is 25 and I don't see many online patients in that age-range. I learned alot by reading from the local medical library...mostly because I felt I needed to make some very serious decisions and wanted to be as informed as possible.
I am hoping to find some that are possibly in that age bracket. And to seek information regarding problems later in their lives.
My son had several surgeries (from age 15-18) to correct his spinal anomolies..basically fused his entire spine and fused the pelvis to the spine. He was diagonsed about a year later with Epilepsy. He was just told, during an ER visit after a minor accident at work, that he has "clubbing" in all his fingers, which is indicative of pulmonary distress or even cystic fibrosis........we have an appt this week to see our family Dr.
Any information you may have would be greatly appreciated.
Thank you in advance for anything you might have to offer. |
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Expert
Posts: 2365
Location: Thornville, OH | I'm going to print this out for all of hunters docs, plus give it to that nasty nutrionist. Thanks for all the info! |
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Location: Wilkes Barre PA | I am so glad this is helping so many people and that my research can come in handy |
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| i was wondering what you found out about your son at his doctor apt. My son has vacteral syndrome and has been clubbing his hands lately. |
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New user
Posts: 1
Location: Bryceville, Fl | Sandy, I am trying to support my daughter in taking care of my grandson, Jarrett. Right now she is so concerned about all the nurses who have taken care of him in his 7 weeks of life. She is worried he will not be bonded to her. She wants the nurses on one hand, but has so many bad feelings on the other. Jarrett has a twin brother Brendan who doesn't have Vaters. Jarrett has a tracheostomy and a colostomy. If you can relate please let us know if you had these feelings and how Mathew has done with his problems. |
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New user
Posts: 2
Location: Massachusetts | Hello! I just wanted to say that you have done an excellent job describing VATERS/VACTERL. I am 14, and i have VACTERS and i am doing well with it. I am a new user, and would love to talk to you, and answer any questions that even you may have!Looking foward to hearing from you!
Amy |
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Location: Wilkes Barre PA | Thanx Amy and welcome to the boards!!! |
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Regular
Posts: 88
Location: Amelia, Va | Hello,
My name is Haylie and I also have VACTERL. I'm 17 and i'm doing fine with it, i still need another surgery for my kidney and my scoliosis. I was wondering if epilepsy is common in VACTERL patients, i know there aren't many studies, especially because there aren't that many people who are older that have it, but do you think that since i have scoliosis, that i will develop epilepsy?? I would appreciate any info you can give me, and i think you gave an awesome definition of VACTERL, i just did a college essay on my condition and i couldn't think of all the things to say. You've helped me out.
Thank you, Haylie |
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Veteran
Posts: 270
Location: Ann Arbor, MI | I just found a great section on the National Institute of Health website, links to all kinds of research papers on VATER and associated things. http://www.nlm.nih.gov/mesh/jablonski/syndromes/syndrome644.html |
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| Shirley, I have a very close friend who has twin boys which are going to be 3 in June 2004. She too has experienced having one twin with Vater's and the other twin not having it. I am sure you are well aware of the hours spent in the hospital, but let your daughter know that my friends son has bonded with her without any trouble! :-) It is okay to have feelings like that, but that baby is always gonna be yours. Her son has bonded so much he is like a baby duckling that never leaves her side.
I jumped on this website for the first time to get a better understanding of what little Max, my friends son, has. I was reading through the posting and saw yours. I felt the need to respond to your post. It will work out. It just takes some adjusting to. |
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Veteran
Posts: 102
| TY TY TY for that and Ive downloaded it for my daughters school district nurse. Erin is her first Vater's kiddo and it will help her understand overall the condition.
Shirley...The concerns your daughter have were what I had when my daughter was born. I have three other children and one of them is a special needs kiddo who has Cerebal palsy. The bonding is there with her and always will be. Erin showed me that she knew who her mom was and to this day is my lil shadow. Just keep reasurring her that he knows, and when she least expects it..He'll show her too.  |
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| I am doing a research problem for my science fair on VATER syndrome in which my brother Tyler has. I wanted to ask everybody with a son or daughter with the syndrome do your kids have a hard time making friends? And i wanted to ask Toni, is your son as mature as a 25 year old adult should be. Does he still have a hard time adjusting to new people and places? Thanks for anything you can give me.
tyler's sister |
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Extreme Veteran
Posts: 352
Location: Plymouth, UK. | Thanks for the information, it is really good to pass to people who wish to know what VATER means. |
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| My daughter Miriam is five years old. She was in the NICU for five weeks after her initial surgery. Whe she was a baby we read all the books and they all spoke of bonding and I was very worried about it myself. As a parent you grow out of it. My daughter can't wait till Saturday when it's Mommy day all day long. Everyday you are a parent to both your children and it all adds up to an amazing bond. When they cuddle when you read to them or run up and hug you when they see you, or when she does the Mommy's home dance, just like Zoe's happy dance on Elmo's world you forget about the bonding stuff in books. You did the best you could when your child was in the hospital, its time to enjoy them now. When Miriam was in the hopital the nurses would unhook her from the monitors to put her on my lap but somehow as soon as they did it I had to go pump so it was easier to put her on her fahers lap. We worried about bonding but now there's no question she is connected to us both.
Edited by SUK 6/4/2004 2:55 PM
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| To Tyler's sister.......I'm sorry I haven't checked in sooner. Hope you did well on your VATER paper. I believe my son is a litle behind on emotional development due to his health problems, but he is doing better and better, as he gets older. He is 26 now, has his first real long-term girlfriend and seems to be immature from my perspective, but works out his problems in a postive way, communicates better ,especially with authority figures and supports himself completely. That is a lot to be proud of and I am. |
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Expert
Posts: 1709
| Dave,
I checked out the link you submitted and viewed the article. It says "mental retardation" is part of Vacterl. I didn't think that was true.
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Extreme Veteran
Posts: 337
Location: Odense, Denmark | When my MOM list the things im suffering from it sounds like i'm in a wheelchier or something but im deffentley NOT. |
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New user
Posts: 1
| Hello,
I am a 34 years old VATER child. I was born July 27, 1970. I recently found this website and finally feel like some one out there may understand me. After surfing this site I came to realize that I have not only found a place to feel accepted, by cicrcumstances, but I may be able to help those who are young and experiencing this confusing set of circumstances in life. I saw your son Dylan, and for some reason I stopped and saw myself in this boy.
I hope he is doing well. This a difficult path to walk, but with love and an open hand it can be grand as well.
Like I metioned earlier, I am 34, and I am having surgury again on both of my hands in about another month. I will not be able to type after that for some time. If you choose to communicate please do not misinterpret my lack of response through March.
If there is anything that I could do to help you or Dylan, please do not hessitate.
MR. G
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| Hello Mr. G,
Welcome to the board!
I do not frequent this board as much as I would like to, but when I read your post I wanted to ask you questions.
You mentioned that you will be having hand surgies in about a month.
I was wondering what kind?
Our son Aaron (will be 6 on Feb.4th along with his twin brother Evan), had bilateral politicizations on his Type 3b thumbs.
His right thumb came out much better than his left & unfortunately we believe he would have been left handed.
He lost some circulation in his left thumb during surgery & thus function decreased.
I was wondering where you are having your hand surgeries done as well?
We live in Wisconsin & traveled across the Midwest interveiwing doctors in regards to Aaron's pending surgeries.
We finally chose Dr.Cooney at the Mayo Clinic in Rochester, MN.
Have you had previous surgeries on your hands?
I have little time as Aaron is home ill with Strep Throat & Scarlet fever.
When it rains, it pours!
Take care & best of luck with your hand surgeries.
Sincerely,
Aaron's Mom R.J.
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New user
Posts: 1
| Toni,
Hi my daughter is 37, so she is a real old timer. Sure
wish this site was available when she was small, but at that time, not many hospital had equiptment to even do the xrays needed let alone have the thorasic surgeons availabel to the the surgeries. I am just grateful her will to live was so strong. She is developing new problems now and is believed to be manifesting from problems associated with VATER. I has in hopes her life threating problems had been resolved. Looks like we are in for another hard battle with pullminary complications.
The results are not in yet, but the symptoms are not good.
I was looking for information on long term effects of VATER, and have found nothig that is helpful thus far. |
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Veteran
Posts: 166
Location: Wisconsin | Even "looking normal" I am kinda disabled. Even riding the elavator can be hard.. going up one floor can be difficult.. when someone says something mean.. cause I don't look disabled.. you know. But I'm glad we're getting the word out. |
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Veteran
Posts: 166
Location: Wisconsin | Hi Haylie!
I'm 19 and a little older than you.. not by much though. Yeah it's really hard for people to understand the syndrome where I am, cause I look normal. I had sugery on my spine as a Freshman in HighSchool.. a spinal fusion. I thought I would say hello cause someone was actually not a parent! hehe  |
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Veteran
Posts: 166
Location: Wisconsin | Hi!
Another person from the Cheese State. I had surgery on my hand, a thumb removed which was premature and not working.. it has some long and weird name, but I don't know it! lol I do have radial hands? With tons of other stuff. When I had physical therepy which was so difficult I found video games the best! |
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Extreme Veteran
Posts: 337
Location: Odense, Denmark | Then let me introduce my self to all these youngsters i didn't meet at the conference:
Hi there
My name is Mathies Jensen i'm 17 and was born with VACTERL i have VACTER in more or lessend degree. My lef hand is smaller than the my right and im missing a finger and my thumb is not working proberly just bending inwords. I hope to meet you all one day and see that i'm no the only one with the whole arm affected.
Mathies Jensen |
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New user
Posts: 1
| We too have twins - one with VATER and one without. They are 11 (12 on sunday) now, and Nate was just diagnosed. He was born with an imperferate anus, had the colostomy and reversal.. and now is dealing with scoliosis. A dr finally put it all togther for us and diagnosed him. We go for a kidney ultrasound next week.
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New user
Posts: 1
| Hi,
I just joined this website. When I saw your article, I just had to reply. I have a daughter with Vater Syndrome. Christine is 15yrs old and she is very mature for her age due to spending most of her lifetime around doctors and nurses. She is going through a tough time right now with her peers. She wants her medical condition to stay a secret although she has some obvious limb abnormalities. I realize part of her attitude is her age but she still wants her conditon kept a secret. Her reasoning is that she thinks people will make fun of her or tease her. |
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New user
Posts: 1
| Thank you for the information.
I just found out that my godson (only one week old!) has been diagnosed with VATER. It's nice to read all the personal stories as well as the more "medical" information you have placed on this site. I'll give the URL of this great site to his parents, because I think it can be a help for them understanding the condition of their son. |
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Member
Posts: 6
Location: Portland, Maine | Thank you so much. It is nice to have the information in one place like this. I registered in November but it has been such a busy time . My son is now 9 months old and has had his 3rd surgery wich was the colostomy reversal. I would love to be able to talk to some one, but the last time I posted no one replied. Is there a good way to be able to chat? I have so many questions. |
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Member
Posts: 7
| I read that your daughter had a finger pollicization. Can you tell me how this worked out? Are you happy with the surgery? I have a two-year old foster child who has just had wrist centralization surgery to a club hand. He has no thumb and his first three fingers a stiff with little mobility. The doctor is recommending a finger to thumb transfer. Max has to go for so many surgeries. I would like to hear from people who had this surgery and if they're happy they had it. PLEASE respond. |
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Member
Posts: 7
| Aaron's Mom R.J.,
I am new to this site and don't know if you even come on it any more. I have some questions about the finger politization (sp?) surgery. Are you happy you did it? I have a two-year old foster child who has just had wrist centralization surgery to a club hand. He has no thumb and his first three fingers a stiff with little mobility. The doctor is recommending a finger to thumb transfer. Max has to go for so many surgeries. I would like to hear from people who had this surgery and if they're happy they had it. PLEASE respond. |
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New user
Posts: 3
| I just logged on to this site tonight. I am 45 and have VATER. I have never before even looked for help in handling my condition. I googled this in an attempt to explain things to my kids. I'm a little overwhelmed with all the information on here. I'm grateful for the summary - I'll give it to any new Dr I might encounter. My current Dr. has educated herself to the max on VATER. I'm happy to answer any questions about adult issues with VATER. Peace to All! |
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New user
Posts: 3
| I just logged on to this site tonight. I am 45 and have VATER. I have never before even looked for help in handling my condition. I googled this in an attempt to explain things to my kids. I'm a little overwhelmed with all the information on here. I'm grateful for the summary - I'll give it to any new Dr I might encounter. My current Dr. has educated herself to the max on VATER. I'm happy to answer any questions about adult issues with VATER. Peace to All! |
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New user
Posts: 3
| I am 45 years old. Have no left thumb, left arm shortness, esophageal atresia, blah, blah, blah. Please tell you beautiful daugher that the "arm thing" shouldn't get it her way. I played piano (without my thumb) and played volleyball on a state team. College was a breeze, I earned a "corner office" in a big corporation,married the man of my dreams (his comment -- everybody has something wrong- so what?) and am now raising 4 beautiful children (no vater in the kids)...
Life has way too much to give her to worry about being "teased" -- Remind her it is a short period in her life (yes, I was called 4 fingers, etc.)-- and nothing can stop her from her goals!! Peace and Love to Her (and Your family!)!! God Bless! I'm expecting great things from her!! |
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Expert
Posts: 3132
Location: Albany, NY | Welcome to the site!!! Thank you for your post |
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For newbies VACTERL/VATER 411 informational post
