Switching Craniofacial Teams

I thought this may be interesting for anyone else that is in a transient stage of life like we are...

My husband and I are both from southern California. Our little boy was born with a bilateral cleft lip and palate a year before my husband graduated from BYU. Clefts run in my family, and my cousin's little boy has a unilateral cleft and is seen at the Loma Linda University Craniofacial Team. He had finished all of his surgeries by the time he was 13 months old, so we anticipated a similar timeline for our little fellow. After some consideration we chose Dr. Morales as Ethan's surgeon, but were surprised to find that the timeline was vastly different than what was done in California.

Dr. Morales did three surgeries during Ethan's first year: placing the prosthesis, the lip and nose repair, and a partial repair of the soft palate. We were pleased with the work that he did and Ethan's repair looked amazing. My husband graduated in April and was on the accepted wait list at a medical school, so we were kind of in limbo during the summer. We talked to Dr. Morales about perhaps speeding up the surgery schedule because we'd be moving away, since we knew we couldn't afford to come back to Utah for Ethan's surgeries once we left; we had been on BYU insurance, but as starving students we couldn't afford the co-pays for all of Ethan's medical care, so Ethan was on Utah Medical. At one appointment Dr. Morales seemed to entertain the idea of doing things differently, but when we went in in July to schedule Ethan's soft palate repair for the fall, I asked again about changing things, and he responded by saying that lots of people traveled from far away (as far as Germany!) to have him treat their children. Not exactly the response I was looking for.

By the end of July we knew that my husband wouldn't be starting school until the next year and we needed to let our landlords know whether we were going to renew our lease for another year. We ended up feeling strongly that it was best to move back to California - so much so that we packed up and left in about a 2-day period. We had to cancel Ethan's surgery, since we wouldn't be covered by Utah medicaid, and MediCal or California Children's Services wouldn't cover an out-of-state surgery that could be done by someone in-state.

As soon as we got settled again, I called up the craniofacial team at Loma Linda to get Ethan in for an appointment. The soonest they had was at the end of October - 2 months out. At the appointment I was given several "assignments," including meeting with Ethan's new surgeon, Dr. Martin, who also operated on my cousin's little boy. Dr. Martin felt it best to go ahead and close the whole palate and gumline, since that is how he usually does surgeries and he didn't feel comfortable varying from what he normally does if it could be avoided. We were fine with that, and scheduled the surgery for late February. We also met with audiology/ENT and the pediatric dentistry clinic before going back to the clinic, and we found out that Ethan had mild to moderate hearing loss and some severe tooth decay (in part from all of the food that was continually stuck in his prosthesis - Dr. Martin's resident commented on how gunky it was when they took it out).

Ethan had his surgery, but Dr. Martin didn't end up feeling comfortable closing the gumline because the prior repair to the soft palate had created scar tissue, making it more difficult to bridge the gap between the palate and the gumline. He felt that if he had closed the entire thing, there would have been too much pressure and there was a very high risk of a fistula. We were pretty disappointed, because it meant that Ethan would still have to have the bone graft/gumline repair sometime between age 5 and 8.

We are still following up on Ethan's surgery, but he's looking good. One big lesson we learned is that once you start a treatment plan, you can't really switch half-way through. The timeline used in Utah works well, but once you start you can't skip any steps. Now I understand why Dr. Morales was so hesitant to switch things up. Dr. Martin had nothing but good things to say about Dr. Morales (he pretty well-known in the field); he just said that they do things differently.

There are things I like and dislike about both teams. I feel like the Loma Linda craniofacial team clinic is better organized and the process of making clinic appointments and follow-up were more straightforward, but I felt like Primary Children's Medical Center was a much more organized hospital and made for a much more pleasant and peaceful stay for Ethan on the actual surgery day.

Study: Are Arm Restraints Worth It?

Way back when, LeAnn Roling gave me a copy of a presentation she saw at a conference on the use of arm restraints following cleft palate repairs. I'll copy here the content of the slides from the presentation, and I'd love to hear what people think about it.

My personal feelings on restraints are mixed; I felt that Ethan didn't need them a lot of the time, but then heard a couple of horror stories about children causing severe damage to their recent repairs (particularly external repairs of the lip and nose). I just couldn't risk it when it came to the lip repair, but was a lot more loose with the arm restraint use when it came to the soft palate repair.

Anyhow, here is the study:

A Comparison of Palatal Morbidity With and Without Arm Restraints Following Cleft Palate Repair
DeAnn Wilson, BSN, RN
St. John's Mercy Medical Center
St. Louis, Missouri

Literature Review

Rate of Cleft Palate Fistula (CPF) increses:

• With children older than 18 months of age at time of repair
• A cleft that is more extensive (bilateral or unilateral cleft lip and palate)
• With a surgeon who has notably less experience

Type of repair had fewer implications and gender had none.
(Cohen, et al., 1991; Muzaffar, 2001; Rohrich, et al., 1996; Schultz, 1986; Wilhelmi, et al., 2001)

Only 1 study addressed the merits of using arm splints.
This Randomized Control Trial determined there was no difference in CPF rate when arm splints were not used:
28% CPF with arm splints
20% CPF without arm splints

None of the CPF were symptomatic or required treatment
(Jiginni, et al., 1993)

Problem and Purpose
Problem:

• Recovery from a Cleft Palate Repair (CPR) is stressful
• Arm splints:
• Frustrate infants and toddlers who do not like to be restrained
• Hinder parents' ability to hold and comfort their child
• Cause sore spots in the axilla, arm stifness
• Interfere with sleep by inhibiting self repositioning without waking

Purpose: To determine if the rate of CPF was higher without the use of arm splints as compared to those children that used arm splints following a CPR.

Methods

•  A single Craniofacial Surgeon's cleft palate repairs (CPR) over a 5+ year period:
• 2.5 years with arm splints (n=22)
• 3 years without arm splints (n=43)
• Retrospective chart review
• All patients underwent a standardized two-flap CPR with "radical" intravelar veloplasty

Extent of Cleft Palate (CP) and Age at Time of Repair

• With arm splints:
• Complete Cleft Palate:     n=7
• Incomplete Cleft Palate:   n=15
• Age Range: 8-38 months; average = 15 months
• Without arm splints:
• Complete Cleft Palate:     n=24
• Incomplete Cleft Palate:   n=21
• Age Range: 6-32 months; average = 13 months
• All children > 19 months: recently adopted, n=11; other illness, n=3

Results
With arm splints (n=22):

• 1 slit fistula - incomplete CP, 13 months (age at repair)
• 4.5% CPF rate

Without arm splints (n=45):

• 2 slit fistulae
• 1 complete CP (wide); 10 months; antibiotics for prolonged fever and sinus infection
• 1 complete CP (wide); 12 months; IV antibiotics for tracheitis
• 1 partial dehiscence - complete CP, 28 months, developed strep throat 3 days after surgery
• 1 complete dehiscence - incomplete CP, 13 months, screamed for 14 hours day of surgery
• 8.8% CPF rate

Discussion

• For each child that developed a fistula/dehiscence, the parent was asked if they observed the child putting fingers or toys in their mouth
• Each of these parents reported their child did not put anything in their mouths
• The child that developed tracheitis 2 days after surgery was also fed solely via G-Button.
• Most parents reported their child did not put anything in their mouths after surgery
• In the immediate post-op period, those children that used their fingers or pacifiers for comfort would try, but most quickly took them out of their mouths
• Those few children that did continue to use their fingers or pacifiers for comfort woudl adjust the position to their cheek pocket, or simply held their pacifier next to their cheek
• Very few children used bottles after surgery, even though we allowed them

Conclusion
Discontinuation of the use of post-palatoplasty arm splints does not significantly compromise palatal integrity.

Emergency Treatment Plan for Kids with Special Needs

Here is a great article for preparing for emergency situations for kids with special needs:

http://knol.google.com/k/emergency-treatment-plans-for-kids-with-special-or-chronic-health-issues#

Sleep Apnea Support Group

There is a Wasatch group, and you can find their information here:


http://www.sleepapnea.org/cgi-bin/databasenew.pl?UT&&State

You can also link from that page to find groups in other states.

Sleep Apnea

As parents of children with clefts, you may want to be on the lookout for signs of sleep apnea if your child has not already been diagnosed with the condition.

"Preschool children with cleft lip and/or palate have a risk of obstructive sleep apnea that is as much as five times that of children without cleft. Obstructive sleep apnea appears to be underrecognized in this group of children."

J.E. Maclean, K. Waters, D. Fitzsimons, P. Hayward, D. A. Fitzgerald (2009). Screening for Obstructive Sleep Apnea in Preschool Children with Cleft Palate. The Cleft Palate-Craniofacial Journal: March 2009, Vol. 46, No. 2, pp. 117-123.

Here are some of the night-time symptoms of children with sleep apnea:
Difficulty breathing while asleep

• Pauses in breathing
• Snoring
• Restless sleep
• Heavy sweating while asleep
• Nightmares/night terrors
• Bed wetting

Daytime behaviors may include:

• Sleepiness during the day
• Aggressive or hyperactive behavior
• Learning disabilities
• Morning headaches
• Discipline problems
• Failure to thrive

http://newtechpub.com/phantom/snore/osakids.htm

For kids with clefts, apnea may be resolved through surgery (such as a mandibular distraction for a child with Pierre Robin Sequence), or by using a CPAP (Continuous Positive Airway Pressure) machine for sleep.

Here are a couple more articles on childhood sleep apnea:

www.stanford.edu/~dement/childapnea.html
www.sleepapnea.org/info/children.html
www.entnet.org/HealthInformation/Could-Child-Have-Sleep-Apnea.cfm

There are myriad articles if you are interested in finding more information online!

Would any of you like to share your experiences with sleep apnea in your children? If so, please email Kidswithcleftsblog@gmail.com.

Surgeon Profile: Dr. Kjar

Contact Information:

Premier Plastic Surgery of Utah

1551 Renaissance Towne Dr #360

Bountiful, UT 84010

Get Directions

(801) 295-9105

drkjar.com‎

Parent Comments:

"Doctor Kjar does things differently that anyone in the state but does it the same as everyone in the country. This is our treatment plan:

"He had his lip repair on October 12th. It was a great success and he looks great! I saw someone in Walmart today that I haven't seen in years and when I told he had just had his lip repair surgery she told me she would have never guessed anything had even been wrong.

"He will have his palate repair three to six months from now. Dr. Kjar likes to repair earlier for a few reasons. The main being the palate muscles are repaired correctly if the whole palate is repaired in one surgery instead of two. This helps with speech among many other things. He will have to have a NAM (retainer from Dr. Yamashiro) until that point. But we are confident with his plan.

"Then follow basically the same timeline as everyone else. Bone graft around 7 years old, possbile mid-face surgery, etc. The mid-face surgery can be necessary no matter when the palate is repaired according to the research I have done. By mid-face I mean, the upper jaw doesn't grow forward like it should and has to be repaired.

"On top of the treatment plan working for our needs, our doctor is wonderful. We never have a problem getting in to see him, he is completely available any time of the day. He have his office number, cell number, and pager number. His staff is knowledgeable and very helpful."
 

My milk supply is dropping. What can I do?

Here are some recent tips from group members:

• Pumping is supply and demand. Try increasing the time you pump, even if you don't seem to be producing more milk immediately; you should see results in a couple of days. 
• Also try increasing how many times you pump per day. You may be able to cut down again once your supply is established.
• Make sure you have the right size horns for your pump. A lactation consultant can help you determine if yours are the right size.
• You may also try fenugreek supplements or lactation tea to help boost your supply.
• Drink lots of water!
• Try to relax as you pump.