Seattle Children's Evaluation

December  17 & 18, 2012

We are in Seattle to get B’Elanna evaluated at the Reconstructive Pelvic Medicine Clinic at Seattle Children’s Hospital.   We are staying at my Mom’s house, so B’Elanna had a bit of fun playing with Grammy’s dogs Rascal and Charlie… and playing with their Kennel.   Poor Posideon… who knows what he did to get put in the kennel… but he looks so guilty here.  Hahahaha.

We also took Posideon to get his checkup and shots at our Vet (yes... I take my dog to a vet 2 states away from where I live.... I have been seeing this lady since 1996 and LOVE how she is with my four-legged babies!)   OK, just to set up the video, I forgot to bring my lead for Posideon... so I just grabbed one of my bungy cords to use.  Yes... the is THAT cooperative of a dog!!

On Monday, B’Elanna had two procedures scheduled.  First a sedated Voiding Cystourethrogram (VCUG) to check her Bladder, urethras and possible reflux to the kidneys, followed immediately by a water-soluble contrast Enema to determine her rectum and colon anatomy and see if there are any fistulas.  They gave her Versed as the sedative… it didn’t knock her out… just made her a bit loopy AND she acted Drunk afterward.  Hilarious!!  

B’Elanna hasn’t started to “play” yet, so I didn’t think the Child Life Specialist would really work, but she was great with music toys and a portable video player.   We had a translator  when the Nurses were giving B’Elanna the Versed, I just had her explain to B’Elanna that the medicine would make her feel funny, and then she was going to have a procedure that might hurt, but that Mommy would be with her the entire time and she would feel better afterwards.   I didn’t feel it was necessary for her to be there for the actual procedures.

On Tuesday, we had to be at the hospital VERY early.  Today B’Elanna was anesthetized for an MRI of her spine to check for Tethered Spine, followed by a Vaginoscopy to examine her vagina and cervix and to see if they could see her uterus and ovaries.  The General Surgeon also went in to examine her external anatomy and to do an electrode stimulation test to see how her anal sphincter/muscles react.    Sitting out in the waiting room waiting was EXCRUCIATING!!   I knew she would be fine… but still!! 

Once she was in Recovery, the GYN came out to the waiting room to talk to me.  She had GOOD NEWS!!  From what she could determine (mostly from lack of scarring) B’Elanna had a LOW PERSISTANT CLOACA had her cervix looked GREAT.  B’Elanna is too small to determine if her Uterus has any deformities…. BUT she does have one and her ovaries!!  She said all of this bodes WELL for B’Elanna to have sensation, the ability to have non-painful intercourse and have children!!  Because of the Persistent Cloaca she will have to have a Cesarean Section if she has kids.   Once she hits puberty, she will need to have more Ultrasounds and MRI’s to evaluate her Uterus construction AND to determine if anything has changed once hormones kick in.

After the GYN came to talk with me, I had to wait for about another 30 minutes before I was allowed to go back to B’Elanna.   They had put an endotracheal tube down her throat, so she sounded rough and gruff and her throat hurt (of course).   

 

While we were waiting for B’Elanna to wake up more and drink some fluids… the nurse came to tell me the Doctors had ordered up a Renal Ultrasound for B’Elanna.  We had one done in Missoula, but the video never made it to Children’s.   So they released us from Surgery before B’Elanna was “completely” awake… but we were just going down the hall for the Ultrasound… so we were safe.  We got the Ultrasound done and then we had to be at the Clinic appointments.   Yeah… no lunch, but the receptionist gave us some crackers to tide B’Elanna over.  And then we started the Clinic appointments… we started at 1:30 and didn’t get out until 5:00pm and then had to wait for the Pharmacy to get the prescription together… so we didn’t leave Seattle Children’s until about 7:30pm!!

OK… so the Clinic Appts…

1.        We met with the Urologist.  He said her Kidneys, Bladder and 3 Ureters look good (yes 3… she has two coming out of her left kidney).  He said they don’t see any evidence of scarring… so she has either had None or very few kidney infections or UTI’s!!  No Reflux!!! YEAH!!   He said with the results of his tests B’Elanna has a VERY GOOD chance of attaining Urinary Continence.  He stressed to NOT push Potty Training until her ability to have Bowel Continence is determined.  He said that girl with Persistent Cloaca that do attain Urinary Continence but not Bowel Continence… often just give up and regress.   He said IF B’Elanna shows/voices interest in sitting on the Baby Potty to let her.  This is good since she is my constant shadow and copycat (even in the bathroom)… so she is already wanting to sit and go.   He said B’Elanna will need Annual Ultrasounds to watch her kidney function and to give her about 2 oz. of Cranberry juice every day.

2.       Next the GYN popped in to see if I had any new questions.  I didn’t.  She said she would check in with us when we come back to Seattle Children’s for the annual (poss. Biannual) visits.

3.       Then we saw the General Surgeon.  I had a whole SLEW of questions for him.  I have enough knowledge to know that what I was seeing on the Contrast Enema the day before was NOT NORMAL.   He walked in, introduced himself and then said “NO MORE MIRALAX!!”    I asked him if he could explain WHAT had been done to B’Elanna over the last 2 years?  Did she have a colostomy, because I can’t find a scar and how could they do the surgery without a colostomy??  Is her Anus too close??   I also had a few questions about B’Elanna’s constipation, diet and medications… he said the Bowel Management Nurse would go over that.  

Then he showed me the Contrast Enema and explained what I had seen as “NOT NORMAL” yesterday… basically her Rectum (not Sigmoid Colon) is 3-4x the size it should be.  He said this is from her being constipated for the last 2.5 years!  This means that IF she were able to achieve bowel continence she wouldn't KNOW/FEEL she needs to Go UNTIL she is already constipated.  The picture below isn't EXACTLY what B'Elanna has OR the surgical option... but it is the best picture I could find.

So how is this fixed?   The objective will be to reduce/shrink the size of her rectum.  This can be done two ways: 

1.)  Daily Enemas  - these are NOT fleet enemas… but more like the old fashioned kind with the old red water bottle… but more involved  OR

 2.) Create a diverting Colostomy.    B’Elanna would then be reevaluated after 6-12 months to determine if her rectum is responding appropriately. 

Next, he showed me a video he had recorded during the electrical stimulation of her anal muscles.   IT WAS GOOD!!   She has great floor muscles and her anal muscles contracted wonderfully.  BUT… it also showed that her current anal opening is in the incorrect spot and she has a Prolapse. 

So how is this fixed??  B’Elanna will need to have a corrective anoplasty.

So WHEN will all this happen??   First the Doctor is going to consult with the Cincinnati Colorectal Team for their opinion.  Second, we will either do the Enemas or Diverting Colostomy for 6-12 months.   Third, when she is evaluated after 6-12 months, they will decide if her rectum has reduced enough in size or do they need to surgically reduce it.  Fourth, is the “final” surgery.  During this surgery – if she has the colostomy – they will surgically reconnect the two ends of the colon, surgically reduced the size of her rectum (if needed) and do the corrective anoplasty at this time.  If she does not have the colostomy – they will surgically reduce the size of her rectum (if needed) and do the corrective anoplasty.

After he was done explaining the tests and her options, he said he believes B’Elanna had a LOW Cloaca or a Rectovestibular Fistula.   Since he had not seen her before she was repaired AND the China surgical notes are not very detailed.   He said the #1 question with Persistent Cloaca is:

“What is the Potential for Bowel Control.”

GOOD  POTENTIAL                                                      BAD  POTENTIAL

Low lying defect   +++                                          High Defect
No spine anomalies  +++                                       Spine anomalies
Good Surgery                                                          Bad Surgery  ---
Sensation                               ???                             No Sensation
Motility                                                                     No Motility  ---
Anal Muscles Good  +++                                         Anal Muscles Bad

“Incontinence??”

Pseudo incontinence  +++                                           True Incontinence (would be bad)

Treatment??

Bowel Management Constipation                             Enemas à Antegrade Continent

(This would be after the surgery)                                                   Enemas

4.       Lastly, we saw the Bowel Management Nurse.  She stated again NO MORE MIRALAX.  For now on B’Elanna will have a routine of Liquid Senna and Citrucel each evening.  Each day… I will need to evaluate if B’Elanna’s stools have been “good” or “bad”.   If “good” keep her on the dose.  If “bad”, give her a Pedialax Enema and increase her Senna dose.   Then the next day, repeat the above routine.   Email the BM RN to let her know how things are going and let her know if I have any questions.

We finally got done and then had to wait over an hour for the Pharmacy to fill the liquid Senna prescription.  B’Elanna got a little bored.