|Leap of Faith|
|Hi Everyone, |
My 10 yr. old son Luke was born with a very rare bone disorder called Cleidocranial Dysplasia. His bones are "soft" and he has a barrel shaped chest with no collar bones. He also has large soft spots on his head that may never harden, is of VERY small stature, and has severe Kyphoscholiosis. His scholiosis curvature is at 55 degrees and his kyphosis is at 125 degrees, which is giving him a breathing capacity of 62%. He wil be having his first VEPTR surgery next month, performed by Dr. Campbell in Philadelphia. This surgery has been "in the works" for over 3 years now....It's been discussed in large forums and at conferences, etc... Dr. Campbell has said he's struggled with what to best do to help Luke, as his poor bone quality has presented many obstacles and challenges. It has been put off many times, however, his kyphosis has progressed significantly over the past year, as Luke has had a growth spurt as a result of his improved nutrition via an NJ tube. (He went from 20 to 30 lbs!!) Although Luke looks and feels the best he ever has, Dr. Campbell feels the surgery needs to happen now or his kyphosis will begin to stretch his spinal cord and cause paralysis. I am incredibly scared. I have been reading all the posted stories on this site and am just blown away at the courage and strength of you and your children. You are all just amazing... What I am struggling with the most is the decision to do this NOW, even though he is happy, healthy, and pain free. He can't run without becoming winded and walking for long distances is hard for him, otherwise, things are great. I keep telling myself it's for his future quality of life.- but it is a leap of faith.....What if it's not the right decision? I will say, I have become better educated just from reading your stories as communication with the doctors office has been a challenge... THANK YOU for sharing them all. I do have some questions after reading some of them.....
Do all kids have to have an IV in the heart for this procedure? why?
How long does the surgery last?
What's been the "criteria" for being released from the hospital?
How long is the hospital stay if there aren't any complications?
What pain medication is given in the hospital?
What have you done re: emergency aftercare issues when there are no Doctors within driving distance that have any VEPTR experience?
Sorry for firing so many questions at once! Would love to hear from everyone and especially those with Kyphosis experience! Thank you!
|Hi Luke's mom, |
My heart goes out to you with this difficult decision. It's hard to put into words the kind of things you feel as a mom when your children have to go through these things. My daughter Ava is a patient of Dr. Campbell's and we feel she is in very good hands. She is 2 years old and also has kyphoscoliosis. She had the VEPTR rib to pelvis put in on the left side when she was 10 months old and then had the right one put in when she was 14 months old. We saw a big improvement with the kyphosis after the right one was put in because that was the side where the "hump" was. Since then, she has had 3 more expansions and we have seen overall improvement since the beginning. The kyphosis is tricky and we do notice that it will get worse at times, and then expansions generally help. It has been a lifesaver. All the unknown's can definitely be scary though, and with your son's soft bones I can see the extra level of complication. Sometimes when there is no other option we do have to take that leap of faith. Know we are wishing you the best possible outcome .
I will do my best to answer your questions:
1. I don't think all kids have to have an IV in the heart, but I'm not totally sure. If you mean a central line, Ava did have to have a central line for the very first implant but not for any of the others, including the second one. I think the first one is the most crucial, and depending on the severity of the situation it may be necessary to have.
2. The surgery time varies, but it seems like they will schedule at least 6-8 hours for the first one. I know it can be a a lot shorter than that, but it depends on how long it takes to prep the child and get all the lines in and what the actual procedure is. Maybe some children even have to have it longer but I haven't heard of that.
3. The criteria for being released from the hospital, generally seems to be if the child tolerates their regular intake of food for a consistent amount of time, their oxygen and stats all remain in the healthy range, and their pain is under control and everything they need medication-wise can be given without an IV. Of course that is very general, and every child's experience is different.
4. The hospital stay varies just about like everything else, but I have heard 5-7 days without any complications. That is for the initial implant of course. Ava was in the hospital 12 days the first time because she had a few complications and some kids could be in longer.
5. There are alot of different possibilities as far as medications go, but our experience at first was morphine for pain and valium for muscle spasms. They didn't order the valium right away and when the morphine wasn't cutting it, a very helpful resident said she thought Ava's discomfort might be muscle spasms, and sure enough, the valium made a big difference. They also give Toradol sometimes through the IV which is like Ibuprofen. That was our experience only though. For the expansions, Ava doesn't even need morphine. Sometimes they will give her a few oxycodone doses right at the beginning but the pain is not nearly as rough as the first implant.
6. Fortunately we haven't ever had an emergency issue with the VEPTR and we have always been in driving distance, a few hours away. Hopefully you won't need to ever worry about that. I'm sure the local hospital could call Dr Campbell's office with any questions though if you ran into a situation like that.
If you have any more questions, feel free to ask. I understand the need to know what you are coming up against and don't ever feel like you are asking too many questions!
|Thank you, Lindsay, for all your kind words and for answering my questions.... I meant to ask about infections, have you had to deal with them? What advice is given to help keep these infections from forming? I've read alot about home I.V.'s, is that part of the aftercare or does that have something to do with treating infections? You can just barely "pinch that inch" Dr Campbell wants- I pray that doesn't increase his risk for an infection.... I hope your little Ava is doing well. She is beautiful.|
Location: nixa mo
|We are still unable to pinch that inch over Noah's veptr. Dr. Simmons is very innovative in grafts and flaps to help cover Noah's veptr. We are very fortunate to not have had a infection. The only time I was truly worried it would break though the skin is when the hook came loose from drifting through the bone. Noah is about 42 inches and 36-38lbs depending on the day .|
|Noah is so handsome! How did you know the hook had come loose? Did it show up in an xray or did he suddenly experience alot of pain in that area? i'm fearful that something like that will happen with Luke and the closest VEPTR dr. is over 4 hours away. How has he done with the expansions? How has his recovery been? Im gald you haven't had to deal with infections, I keep reading about them....Luke is 10 now, but only 40" tall and weighs 46 lbs. He stayed at 30 lbs for what seemed like an eternity, until we got an NJ tube for him. Dr. Campbell said he couldn't do surgery until he saw some meat on his bones. He put on 20 lbs in the 14 months he had it, lost a few after it was removed. Just trying to maintain now- he has reflux and never wants to eat.|
|We have been very fortunate to not get any infections. Believe me, I worry about it every surgery though. I wash my hands a lot and everything that comes in contact with Ava's back for at least a couple weeks after surgery. Since she is still little, I have her wear a onsie while her back is healing. I don't know if all my efforts really make a difference or not, but it's all I can do to feel like I have some control! I'm not sure if any one knows why infections happen sometimes and not others, but I do know they have started to give families these CHG wipes to wipe the child with the night before surgery as well as giving them a bath before and making sure that towels, clothes, and linens are clean after the bath. We can only do our best though. I think the issue of coming home with an IV or PICC line is generally related to infection control for the administration of antibiotics. I hope that helps.|
|It does help - Thank you. I'm also worried about traveling home afterwards. We were able to get a non stop flight....Has to be better than the 12 hour drive|
Location: Plymouth, Pennsylvania
|HI LUKES MOM, AND WELCOME, AND THANK YOU FOR SHARING YOUR SON WITH US.... |
I KNOW THE PANIC YOU AND ANXEITY TO FEEL...AND WANT YOU TO KNOW THAT OUR THOUGHTS AND PRAYERS WILL BE THERE WITH'
ZOE IMPLANT FOR OVER 11 YEARS AGO...BY DR. JOHN EMANS AND DR. CAMPBELL...AT THE BOSTON CHILDREN'S HOSPITAL...ZOE HAS SINCE HAD 30 PLUS SURGERIES, AND WITH THE BLESSING OF THE 'VEPTRS' SHE IS HERE WITH US TODAY, A HAPPY, AND VERY STABLE 13 YEAR OLD...
YOUR THOUGHTS AND CONCERNS ARE GOOD ONES...
ALTHOUGH ZOE DID NOT HAVE A CENTRAL LINE FOR THE IMPLANTS, SHE DID FOR A SURGERY 10 DAYS LATTER...THEY PROVIDE DIRECT ACCESS FOR MEDS, AND FOR BLOOD WORK, AND HELP WITH RECOVERY...
PAIN MANAGEMENT, ARE PEOPLE YOU WANT IN MY OPINION TO BE YOUR BEST FRIENDS...TALK WITH THE STAFF, BEFORE THE SURGERY, AND AFTER...THEY WILL TELL YOU WHAT THEY FEEL IS BEST, AND YOU CAN HELP THEM WITH THAT AS WELL...YOU KNOW YOUR CHILD THE BEST, AND YOU WILL NOW WHEN HE IS IN PAIN....AND YOU CAN KEEP THE NURSES IN-TONE...
IF YOU CHILD HAS TROUBLE WITH CONTIPATION, AS MANY OF OUR KIDDOS DO, I WOULD SUGGEST, YOU MAKE SURE BEFORE SURGERY, THAT HIS SYSTEM IS MOVING GOOD...WE USE MIRALAX, AND MILK OF MAG...USING GREAT CAUTION FOR A GOOD BALANCE...
'TRAVEL'...THE TRIP TO BOSTON FOR US, IS IN CLEAR TRAFFIC 6 HOURS, BUT IN TRAFFIC CAN BE AS LONG AS 9....WE TRAVEL BY CAR, AND HAVE THE ROUTE DOWN PAT, AS TO JUST WHERE TO STOP, IF ZOE CAN GO NO FURTHER...AND KEEP TRACK OF WHERE THE HOSPITAL ARE ALONG A ROUTE...
'TIME IN HOSPITAL'...FOR HER VEPTR WE ANTISIPATED 10 DAYS, AND END UP THERE FOR 30...THE EXTRA TIME NOT FOR THE VEPTRS, BUT FOR A GI SURGERY...SO THIS IS SOMETHING YOU CAN NEVER TRULY KNOW...
AS FOR BRINGING LUKE 'HOME'...I KNOW THE FEAR OF THAT AS WELL....YOU ARE COMING FROM A FAR DISTANCE AND YOU NEED FOR YOUR PRIMARY CARE DOC, AND CHOP TO BE IN TUNE WITH ONE ANOTHER...REGARDING EMERGENCY TRANSFERS....ZOE HAS BEEN MED-JETTED TO BOSTON 3 TIMES...(NOT VEPTR RELATED) JET VS. HELICOPTER BECAUSE OF DISTANCE...IT IS DO-ABLE...MANY TIMES THRU THESE 11 PLUS YEARS, WHEN EVER WE HAVE A QUESTION REGARDING THE VEPTRS, MOST TIMES IT IS A SIMPLE AS GETTING A XRAY, AND SENDING IT UP TO THE HOSPITAL...AND ZOES MOM COPIES THE XRAYS ONTO THE COMPUTER AND WE HAVE ANSWERS VERY QUICKLY...
PLEASE JOIN US ON THE 'VEPTR KIDS' FACEBOOK PAGE....LOTS OF FAMILES, JUST STARTING THE PROCESS, AND ALOT OF US PIONEERS THEY AS WELL...LOOK FORWARD TO HEARING MORE FROM YOU...GOD BLESS...AND KEEP US POSTED...
|Joanna, THANK YOU, your advice is priceless to me. Luke has only had a few outpatient surgeries, non serious, just ear tubes and an ilingual hernia repair.... This will be our first major one. We've been blessed thus far. I hope Zoe continues to do well, she is just a cutie!! I'm not on Facebook, but will join now to get on the 'VEPTR kids' site, for sure.|
Location: Plymouth, Pennsylvania
Location: Phoenix, AZ
|Hi Luke's Mom, |
I just wrote a pretty long response to you that got deleted, so I just wanted to give you my website that we have for Carter. If you look back far enough, it gives a day by day update when we had Carter's surgery at CHOP with Dr. Campbell. It is at carepages.com search for littlen8tive. You have to register, but they will not send any spam or any emails at all. You only have to register so that the parents can see who is looking at their children.
Wishing you peace in your journey,
|Hi Kristy, |
Sorry I am just now replying! I haven't been on this site since the 2nd of November! Believe it or not, I am sitting in a hospital Room at CHOP right now- my son had his VEPTR implantion last week on Nov 30th and is here recovering! Dr Campbell performed the surgery. Luke has a VEPTR on each side of his spine, from his pelvis to rib. So far, he is recovering beautifully. Dr Campbell is VERY pleased!!! If things continue to go this well, he says, then we will be out of here much sooner than the 3 weeks he had envisioned! He has visited Luke everyday so far, but has a conference this week, so we won't see him until Monday. I am trying to put together a list of questions for that visit now!!! What can you tell me about expansions? I will try to get to Carters site in the morning if time allows- would love to see it. Take care