Instead of beginning this article with a list of questions and daunting answers, the first thing I want to include is our most frequent ANSWER…
These little girls are beyond precious! Our cloaca child is the light of our lives! They need you to come for them and you will reap a million times more than you invest.
Do not be afraid. Be informed. Be prepared. That is what you are doing by reading these FAQs.
And be comforted that you will not be isolated! There are incredible Facebook and Yahoo support groups. The medical teams who will help your child are amazing and accessible. And this past June the First Annual ARM Mommas Conference was held at Nationwide Children’s Hospital in Ohio, hopefully the first of many, where adoptive moms of children with anorectal malformations had a wonderful time of fellowship and instruction.
Q: What is complex cloaca?
A: A persistent cloaca is a complex anorectal congenital birth defect in which the rectum, vagina, and urinary tract meet and fuse into a single common channel.
Q: What might my child’s Chinese file say that might indicate cloaca?
A: Diagnosis of a child with cloaca should be suspected in a female born with an imperforate anus and small looking genitalia. In our daughter’s case the file read “ambiguous genitalia”. Her file indicated that she was female and included a picture of what seemed to be normal exterior genitalia. We now know that this description can mean many things.
Q: What medical treatment will my child receive in China?
A: Most likely your child will have had a colostomy immediately and then possibly a reconstruction of the anus and a colostomy reversal at about one year of age. She may also have had daily dilations to prevent her anus from shrinking.
Q: What medical testing will my child need upon arrival in the United States?
A: In addition to the regular exam for all adopted children, because children with cloaca have a high incidence of associated anomalies and the defect may occur as part of a syndrome such as VACTERL (vertebral, anorectal, cardiac, trachea-esophageal, renal and limb) she will need an experienced team of specialists to assess her situation. This team should be a part of a complete colorectal program that includes gastroenterology, gynecology, urology, nephrology, neurology and surgery in each specialty.
(note: our biggest mistake was thinking that our daughter’s situation was purely a urological issue and that a local specialist could handle our daughter’s condition. Even if your local urologist has treated such a condition in the past, your daughter needs the experience only a full team can provide.)
This team of specialists will likely require:
• a full urological evaluation as soon as possible as 90% of girls with cloaca have urinary issues which will include a renal ultrasound and a pressure flow test (urinary issues may be serious in nature, our daughter was refluxing urine back into her kidneys)
• x-rays and a MRI to determine is she has spina bifida and/or a tethered cord
• an ultrasound of her ovaries and uterus
• a sedated scoping of the channels by a colorectal surgeon, a pediatric gynecologist, and a pediatric urologist to determine function
• an x-ray of her colon to see how her colon is processing stool
Q: What surgeries should we anticipate?
A: She will need a surgery called a PSARVUP (posterior anorectal vaginal urethral plasty) or “pull-through” surgery which properly positions the affected structures. If you child has had surgeries in China there is still a good possibility she will need this surgery once home. Hospital stay will be around 10 days and pain levels during recovery are surprisingly low. The hardest part can be the days your child cannot have food or drink. Other surgeries will be indicated if your child has a tethered cord, renal issues, or issues with continence.
Q: Will my child have bowel continence?
A: Cloaca children usually need to enter a bowel management program. Your child will most likely have experienced constipation in her orphanage. She may not have adequate feeling or a properly working sphincter. It is essential that she have a bowel movement every 24 hours as constipation stretches out the colon. This can be as simple as learning to use the proper doses of laxative. Some children will need a daily rectal enema to stay free of stool. Some will need surgery so that the daily colon cleanse is done via a stoma in their belly button which is called a Malone/ MACE and often times with surgery is combined with other necessary surgery or surgeries. New technologies for bowel management include the Peristeen and the Sacral Nerve Stimulator. You may need to participate in a weeklong bowel management trial which will include training, a daily x-ray to see that a complete BM has happened, and tweaking of the formula so it is specific for your child. The goal is a “socially continent” child with a daily clean colon no later than school age.
Q: Will my child be continent for urine?
A: Urinary continence without surgical intervention is rare in cloaca patients, but “social” urinary continence is attainable! Some children need only take medicine to regulate the pressures in their bladder while others can catheterize themselves through the urethra. A few may need a procedure called a Mitrofanoff, which creates a stoma in the abdomen through which patients catheterize themselves for urine. Recovery for this surgery was more difficult than the PSARVUP. Our daughter’s surgery included reimplantation of her ureters, Malone, Mitrofanoff, bladder neck division, and bladder augmentation. Pain management was a bigger issue. Your child will leave the hospital wearing urine bags on her legs for several weeks. You will later return to have the tubes removed and to learn to cath via the newly created stoma.
Q: Will she have a normal sex life?
A: After surgery, your daughter can expect a normal and fulfilling sex life, and many cloaca patients are now adults who conceived children but underwent a C-section for delivery.
Q: What is day-to-day life like once the major surgeries are out of the way?
A: It is a challenge at first, but you and your child will soon develop a routine that becomes second nature. Our 8 year old has been cathing herself for two years now. She takes attends church and school, swims, rides her bike, climbs trees, and dances. The nightly colon flush takes an hour. There have been a few hiccups along the way. Your local pediatrician, nurse, pediatric general surgeon, and emergency room are not likely to be completely at ease with medical intervention, and you will likely need to call your surgical and follow up teams and talk with them regarding your child’s needs.
What is a normal day like? Laughter, amazement, joy, and admiration are in abundance, and if you read our family story on No Hands But Ours you will learn that we went back to China for a second daughter with a similar diagnosis (pictured above).
– guest post by Stephanie