I am new to these forums. My son is 6 and was diagnosed at 2 and 1/2 and his doctor is recommending a right upper lobe removal and possible resection of the middle right as well. I am beside myself and any opinions, advice or feedback is really appreciated. Thanks in advance
6 y/o son and lung surgery?
- Thread starter lildenny
- Start date
AmalynRose
New member
I can't really give any advice on this matter, but I can say that there are a lot of people on these forums who are knowledgeable and will be able to help you. Stay strong!
TreasureGoddess
Member
Absolutely ask about getting a 2nd opinion! Can you travel to another clinic to meet with a CF team? It sounds like a major issue so find out all you can and talk to more than one CF specialist. Good luck and we're all thinking about you & your son!!
S
stephen
Guest
“Lildenny”
PLEASE - get more than just a second opinion before your son has surgery!
In 2005, before being diagnosed with CF, several pulmonologists and noted surgeons recommended removal of my right upper lobe. I had bronchiectasis, constant lung infections and fevers, and increased frequency of bleeding.
It was at that same time that CF was diagnosed and I began TOBI, Pulmozyme, and hypertonic saline.
In preparation for the scheduled surgery, I began IV antibiotics. My FEV1 improved and the fevers and bleeding subsided. The pulmonogolist, and an infectious disease doctor, both of whom I had been seeing for years advised me not “to rush into the operating room”.
I took their advice and postponed the surgery. Seven and a half years have passed, and thank G-d the surgery was not necessary. Rigorously following the CF treatment protocol and occasional IV antibiotics has almost eliminated the fevers, bleeding, and life long cough.
It took 63 years and numerous”second” opinions before I was diagnosed with CF - and then multiple opinions before I decided not to have lung surgery.
Wishing your son all the best - and Please get multiple opinions.
PLEASE - get more than just a second opinion before your son has surgery!
In 2005, before being diagnosed with CF, several pulmonologists and noted surgeons recommended removal of my right upper lobe. I had bronchiectasis, constant lung infections and fevers, and increased frequency of bleeding.
It was at that same time that CF was diagnosed and I began TOBI, Pulmozyme, and hypertonic saline.
In preparation for the scheduled surgery, I began IV antibiotics. My FEV1 improved and the fevers and bleeding subsided. The pulmonogolist, and an infectious disease doctor, both of whom I had been seeing for years advised me not “to rush into the operating room”.
I took their advice and postponed the surgery. Seven and a half years have passed, and thank G-d the surgery was not necessary. Rigorously following the CF treatment protocol and occasional IV antibiotics has almost eliminated the fevers, bleeding, and life long cough.
It took 63 years and numerous”second” opinions before I was diagnosed with CF - and then multiple opinions before I decided not to have lung surgery.
Wishing your son all the best - and Please get multiple opinions.
My 13 year old daughter with CF also required a lobectomy at the age of 6.
She ended up having her right middle and lower lobes removed due to a chronic infection concentrated in those lobes.
After a few bronchoscopes and frequent IV antibiotics over a course of year, it was recommended by a panel of CF specialists that she undergo this surgery. She could not improve even with frequent antibiotics and aggressive physiotherapy. It was such a difficult decision to agree to this surgery. I can definitely identify with your situation. After her surgery, her quality of life improved for five or six years. She could breathe better without these lobes causing all the problems. She could run, play, and ride her bike.
We are now facing some new issues. Danielle now needs new lungs and has been on the transplant list now for two months. Her CF seems to be very aggressive in spite of her being very active and completing her daily physiotherapy and inhalations. We hope for her quality of life to improve again.
She is currently 13 years old.
It is important to have many people look at your son's case. Then you can make an informed decision, which will be the right one for your son.
Diane
Mom of 3 wonderful children (one of whom has CF)
I am 47 years old. I had my right middle lobe removed when I was 6. I remember it well. Lots of attention. My mom was with me constantly, and if she ever left, a nurse came and played with me. I don't remember any pain, just the good stuff 
Granted, I had not been officially diagnosed, yet, and in 1972 they thought removing the diseased area would remove the disease. Of course it didn't, but I had many years of being a lot more well than before surgery. The other 2 lobes filled in and I have done pretty well ever since. I usually have IV's at least once a year, but a couple of times have gone 2 or more years between. No IV's for 13 months currently!
Good Luck with your decision.
Granted, I had not been officially diagnosed, yet, and in 1972 they thought removing the diseased area would remove the disease. Of course it didn't, but I had many years of being a lot more well than before surgery. The other 2 lobes filled in and I have done pretty well ever since. I usually have IV's at least once a year, but a couple of times have gone 2 or more years between. No IV's for 13 months currently!
Good Luck with your decision.
I would get a second opinion from a CF transplant doctor at a different clinic, even if that means traveling. Different clinics often approach CF with with different protocols. Transplant specialists approach CF different than general pulmonologists. That's a major surgery, be as informed as you can.
Thanks so much for all the responses. We are currently in Hawaii and getting a 2nd opinion here is next to impossible. We had the consultation with the surgeon today, and she recommended resection of the upper right lobe and the top portion of the lower lobe that shows focal bronchiectasis (sp). His PFTs are low 80s. I really want to do what is best for him and they are not saying that it is a "required" surgery. They believe that it could decrease hospitalizations and improve lung function. When he has been hospitalized in the past it has always been those areas that have been the "problem" and He has grown MRSA mainly. My husband is retiring from the military in March, and we will be relocating to South Carolina, so that is another factor in the decision making process. The CF hospital is there in Charleston, but I really don't have a lot of information concerning their "protocols" and treatments, and I know that every place is different to a degree. His pulmonologist said that those "focal areas of damage can contribute to greater decline in lung function because they are "pools" for infection and do not contribute to good lung function" I can understand the reasoning, but is that "fact" and is it worth putting him through such an invasive surgery? Sorry for my spelling. Thanks again for all the responses and for any thing further.
Aboveallislove
Super Moderator
Mom,
I can only imagine how stressful this is for you, wanting to do what is best and not knowing. I would think you could get a phone consult with other CF doctors and possible even discuss with SC now. With medical records so easily transferred/xrays, etc. scanned, email, etc. it would seem so doable.
Hugs and Prayers,
Love
I can only imagine how stressful this is for you, wanting to do what is best and not knowing. I would think you could get a phone consult with other CF doctors and possible even discuss with SC now. With medical records so easily transferred/xrays, etc. scanned, email, etc. it would seem so doable.
Hugs and Prayers,
Love
TreasureGoddess
Member
This is entirely your decision, but I have to say look at some of the posts here and see how many, many people are online with less than 80% FEV scores. Have you already had multiple hospitalizations for IV and respiratory therapy? Have you exhausted those treatments yet or is your pulmonologist being extrememly proactive to want to do this surgery? Please contact a CF center in SC where you will be relocating to. Explain the situation and ask for a phone consult. Your son's records can be sent by the current hospital/treatment center. If your move is coming soon, it could easily impact how the new CF center would expect to care for your son's health in the future. Is your pulmonologist part of a full CF treatment center? I know resources are tight in Hawaii so you may not be getting all the info you need or the experience you'd like to have before agreeing to this kind of a surgery.
If you decide this is truly best for your son, go for it. I just caution you to please seek more info from another CF team first, even if it's over the internet or phone. Best wishes and we're all thinking of your family!
If you decide this is truly best for your son, go for it. I just caution you to please seek more info from another CF team first, even if it's over the internet or phone. Best wishes and we're all thinking of your family!
I am dying to chime in here!! They wanted to remove part of Abby's lungs too, I think it was the right upper due to a lymph node was closing it off and causing it to pretty much "fester" as the infection could only seep out. We decided to wait to see if she would out grow the lymph node and so glad for that. But guys we have already got Kalydeco and the vx809 and vx661 hot on it's heals....the world as we know it hopefully is fixing to take a major turn!!! I mean shouldn't you ask someone about the new drugs to see if they could turn his lungs around? I know I don't have all the facts and maybe they are too damaged but what if they're not? What if all he needs are these meds? I would be asking if you had time to wait and I would for sure ask specifically about the new drugs, and if you don't feel that you get a good answer ask and ask and ask until you talk to someone who is truly interested in what you are saying. I would get a second opinion regardless. And I just want to add that my heart goes out to you and your son!! I pray that he gets well soon. Big Hugs!!!
Aboveallislove
Super Moderator
Totally agree with Jenny re the new drugs. Someone posted that doctors are now thinking of "permanent" damage differently for those on Kalydeco b/c they are now seeing it was old mucus plugs and that they were clearing up with Kalydeco. The combo could be to market in 2 years. And then a few more for even better. And maybe 10 years the perfect "combo."
First off wow...Thank you all for the support and encouragement that I feel from reading all these posts!!! I wish I would have logged on earlier, but forums and how to use them used to intimidate me. I was actually wondering if I could get a phone consult from another CF team, so that is confirmation to me. They do have a really great group of people here and I haven't always agreed with their plan of care and the team has never pushed anything on us that we were uncomfortable with. We are originally from Tennessee and Ryan was diagnosed at Vanderbilt Children's Hospital in Nashville. I have thought about contacting them concerning Ryan and hopefully get some other opinions. But to answer one question yes his pulmonologist is very proactive. She worked in Boston before here and lung resections were "treatment" for focal damage. It is my understanding that Bronchiectasis is permanent damage? Please correct me if that is untrue. I felt I knew a lot about CF but everyday I am learning something new and this forum is a Godsend. His latest CT in those 2 areas looked like "Swiss cheese" that is the best way I can describe it, and it was not what I was expecting to see. I guess part of the urgency is our moving, my husband retiring and starting a new job that is currently on the government job hiring freeze! This duty station is very liberal about giving him time off when Ryan goes to the hospital, and if we wait until we move that may not be the case. We are kinda stressed at the moment and I do want more information so ,as someone said, that we can be totally informed or more informed. When he was first diagnosed that right upper lobe section was the primary area of infection. Thanks for your prayers and encouragement. Bless you all!
Aboveallislove
Super Moderator
Oh my, how hard this must be feeling the preasure to make not just the right decision but the right decision with insurance, leave, etc. etc. I think it might make the decision an "easy" or at least easier if you are able to get a couple phone consults because you could get a couple "that is a great approach;" "that is a risky approach" and that might guide you alot. Of course the "pros and cons" and no real guidance answer will just make it harder!!! But I'm hoping you'll get more guidance from other CF Centers which will make this not so intimidating.
I'm copying the exchange from another forum that I was thinking of re permanent/not permanent damage. I have no idea what this would be, but it is something to keep in mind:
"I chatted to my consultant about it today and she said the 770 results they are seeing are changing the way they think about cf lung damage. In that what seemed to be irreversible damage on x rays and cts was actually very old mucus shutting of parts of the lungs which do actually gain function back with 770. Just to explain a bit more. For example when you have had an lf of fev 40% for 5 years or so you do not expect to see it go up again as it is presumed this is now scarring rather than just an infection. This is why doctors have been surprised to see rises of sometimes 10% in patients who have had the same lung function for many years. (Vx 770 this is, i don't know much about vx 809 results but even so the theory is the same in that it seems some little bits may not necessarily be permanent damage). Is obviously not all your lungs but still nice to know not all is lost as some little bits may have the chance of coming back!"
Hugs and extra prayers tonight
Love
I'm copying the exchange from another forum that I was thinking of re permanent/not permanent damage. I have no idea what this would be, but it is something to keep in mind:
"I chatted to my consultant about it today and she said the 770 results they are seeing are changing the way they think about cf lung damage. In that what seemed to be irreversible damage on x rays and cts was actually very old mucus shutting of parts of the lungs which do actually gain function back with 770. Just to explain a bit more. For example when you have had an lf of fev 40% for 5 years or so you do not expect to see it go up again as it is presumed this is now scarring rather than just an infection. This is why doctors have been surprised to see rises of sometimes 10% in patients who have had the same lung function for many years. (Vx 770 this is, i don't know much about vx 809 results but even so the theory is the same in that it seems some little bits may not necessarily be permanent damage). Is obviously not all your lungs but still nice to know not all is lost as some little bits may have the chance of coming back!"
Hugs and extra prayers tonight
Love
I know very little about the new drugs that are out there or that are coming out. Who is eligible to take these medications? I was told that He had the delta 508 I think that's correct and another "rare" allele??? If you have anymore information on this please post it or links. I get overwhelmed trying to wade through "good" reliable information on the web. Researching for info can get mind-boggling! Thanks again
Aboveallislove
Super Moderator
Oh, mom, you need to find out ASAP what the other rare mutation is--it might well be your son is one of the "lucky ones" who can benefit. Kalydeco is only approved for one mutation but in studies for other similar ones, and you could already get off-label if his mutation is one known/believed to benefit. And your son might well have one of those mutations and benefit from Kalydecol since it is a "rarer" one. If you find out and post there are several on here who can let you know and then you can pursue that. Kalydeco won't work for those with 2 copies of df508 (but from your note, he has a second one that is different), but there are other drugs in the pipeline for that. I'd ask the doctors specifically: The EXACT name of the other mutation and what class mutation it is. I wouldn't let them just say "oh don't worry about that Kalydeco won't work" because many aren't as current as the moms!!!! AND maybe don't think of doing it off label until pushed.
I totally agree with Aboveallislove!!! Find out that mutation. It could be the answer to your prayers. It could be the thing that turns everything around for him! Kalydeco is nothing short of AMAZING!!! I kind of hesitate to say this out loud (don't want to jinx us)but my daughter is on Kalydeco and has been since last June and she does not have the right mutations.....she usually without fail has 2 hospital admissions a year. She has not been in since March 1, 2011!!! She fought of MRSA and RSV!! Unheard of for her, but with Kalydeco's help her lung function, BMI, and overall health is so much better. I'm so glad you found this forum
Aboveallislove
Super Moderator
Omg Jenny just got chills because it is almost two years since hospitalization!
Ok so First I have called Vanderbilt and Thank the Good Lord they still have our phone number all our info. I also called SC so waiting on phone calls. Now I am going to call his Doc here and find out that mutation information. I am trying to keep my cool intact! I mean when the docs wanted to do a G-tube b/c of weight I thought that was invasive and said well can we try stimulants first, which he takes but He's still thin. He takes a lot of Creon too which I wondered if that decreases Hunger? Or if the prevacid does too? I mean to the best of my knowledge any med that effects digestion could decline the appetite. Now my mind is going in all different directions. Right from the beginning Vandy put him on zantac with Creon and said it worked synergistically??? But later I had Docs here ask if he had reflux? So now I'm thinking have I been giving a med that is really not necessary? I mean I tend to think sometimes.... well a lot of meds are overprescribed. Ok I am rambling now~