Hi I havnt posted for a while; my son was dx. with having blebs in his lungs when he was 14yrs. old he is now almost 19. He has a form of congenital emphysema. (blebs in his lungs and many of them) I was just wondering if any one else has heard of this????? He has never smoked. The specialists have warned him about scuba diving (that's really all he can't due) and also informed him of the possibility of having a pneumothorax. He had surgery when he was 4hrs. old; he had a diaphragmatic hernia in which his organs went into his chest cavity and put pressure on his left lung. The doctors repaired the hernia but I am just wondering if this type of surgery at birth had something to do with it or my son also having asthma dx at age 5. Any input would be great. He refuses to use his preventive inhalers. He only uses his rescue inhaler as needed. This is not what the Doctors's wanted him to do. He said if I keep my airway open all the time and take too much medicine it will just cause all the pollution and bad things to get in my lungs more. I am just worried that he will have a pneumothorax when he is out with his friends. Any suggestion???????? He should even wear a medical id bracelet I was told because of his past surgeries. initial one and then when he was seven yrs. old. total bowel obsturction; he had emergency surgery with that. He has been fine sinse. Any input here would be great for mom in California.
Hi....I just saw your latest posting, then checked this one out. I don't visit this board often even though I had a sponeaneous pheumothorax (three times before I was hospitalised). Then eventually after 3 tubings while in the hospital, had the thoractomy. This happened in 1975 when I was 21.
Regarding your son's situation it's definitely complex! Keeping in mind his age and how long he's been dealing with this....the older he gets, the more likely he'll have a pheumothorax.
I don't think the emphysema is caused by blebs tho. They are totally unrelated since the blebs are on the chest wall and the emphysema is a disorder within the functioning of the inner lung.
He has asthma. He should be using a preventer medication (inhaled steroid). I wouldn't suggest it WITH (two in one)or in combination with the rescue inhaler with every dosaging. If he only uses the rescue inhaler when he needs, then maybe that for now is good enough. But, he has complex lung disorders, a doctor should help in giving the pros and cons of that process. I was told that the preventer will help keep the lung inflammation down. The rescue inhaler if taken in excess will actually help the lung become lazy. I still believe that philosophy.
I too have asthma, and it's worked well for me.
Should he wear a medic alert bracelet? Up to him....collapsed lung is fairly obvious when it happens....but I think it would be a good idea for now at least.
Would you consider or has a doctor considered your son to have a thoractomy as a preventative for at least one lung? The worst one. It will never collapse after that.
Each disorder should be looked at individually. Asthma, blebs, emphysema. It's good his hernia was taken care of. I think it's possible that the emphysema and asthma might be connected?
Do you have a good specialist...or a good team of specialists that are caring for your son? As well...I think your research will ease some of your anxiety of what might happen to him in the future.
Colds must be a fear for him I would think. If he does get them, making sure he starts his preventer will be of great help.
Scuba diving...I would think he wouldn't even want to chance it.
He might be in denial. My brother has had asthma since a kid...allergic asthma, and he has been in denial his whole life. He uses a rescue inhaler, exercises, but when he gets sick..he waits too long before seeking care. I don't think he ever uses preventers either.
I hope the best for your son....but information and being really smart about this will definitely help (for you...but especially for him to become proactive in his care). The pheumothorax will more likely happen than not given his situation. This is only from my perspective from experience and research.
Keep us posted.....remember, that some people don't frequent the boards often and that you should still post and ask questions. Saying that, it's understandable your need for answers quickly. We all can learn from your (and others') situations.
As well, I'm definitely interested in how he's doing.
Best to you,
It's all a matter of perspective!
I only have a comment about the medic alert bracelet. I had a condition called idiopathic subglottic stenosis (basically narrowing of the trachea under the vocal cords for no known reason). I had to wear the bracelet while having the condition but not after the surgery that "cured" it. Even though it was major life saving surgery, my doctor said I should not need the bracelet any longer. I do have it saved just in case the condition comes back since they don't know what caused it in the first place. If your son will consider it, there are some web sites out there that offer sport bands for the bracelets. I ordered mine online and it was much nicer than the ones you see in local pharmacies, etc. I did not want to wear one either with how tacky some of them look. Even though they serve a great purpose, I suppose I let vanity in.
Quincy, It has been a long time since I have checked out this forum; I have been busy at the rare diseases forum thanks alot for you'r reply and input. My son is now 19, and is hard headed for sure. I wish he would atleast stay on the preventive inhaler; I think it's called azmacort. I was told it keeps the inflamation down, and especially when he has gotten sick (flu or cold) he had a pulmonary Doctor tell him this when he was 17. He stayed on his meds. and did well, and then decided to just take himself off of everything. I have given him articles to read etc.. maybe he is in denial. For so many years since he was 5yrs. old he had been on medications, to the Doctor, x rays, etc.. often. Maybe too he is just tired of the commitment it involves to keep his lungs healthy. What I don't understand is that he really hasn't had any problems with using in rescue inhaler when he feels he needs it; which is not very often. I have reminded him what the specialist told him about the potential of having a pnuemothorax, and he just says "he feels fine" I hope he doesn't ever have one. I hope he goes in soon to get a complete physical. Even thou he feels great we don't know what is going on inside of his lungs. I guess to find out if there are any changes, if this bleb condition is progressive or what. He plays baseball and runs very fast; you would never know anything was going on inside. You mentioned a thoractomy?????? What is that exactly???? I hope you are doing ok since you have had you'r procedure. Does it involve glue??? Do you take daily medication for your condition???? If I may ask what led to your lung problem????? I have learned alot from the boards here. When you get a chance maybe you can share more of you'r story?????????????? Thoractomy is something I have not heard of. I hope you are feeling well.
I think it would be darned near impossible to keep your son from getting a sponeaneous pheumothorax. At least he's living life.....as well, maybe it could work in his favour to have the oxygen exchange working, as well as keeping his lungs in good working order.
As far as the corticosteroid...if he doesn't want to use it on an ongoing basis...he should consider (this is a strong suggestion) to start it IMMEDIATELY upon the very first hint of a cold. He should use it at max dosage (whatever was prescribed for colds) till he feels better -- meaning perfect with no troubles -- then start to taper off if that's what he wants.
It's true, the corticosteroid will at least help keep inflammation and scarring down.
I have asthma. I wasn't diagnosed with it till I was 28....but I did have symptoms during colds and exercise and cold weather. It does run strongly in my family. My asthma is more intrinsic rather than allergic. I use pulmicort daily, double it when I get a cold, and use my rescue inhaler (Airomir) when I need.
The pheumothorax I had when I was required to be hosiptalised was when I was 21. It was caused by blebs....I just bent over to pick up my purse and I guess that was what did it. I had 2 other collapses within 2 years previous .... but this one required the stay.
Thoractomy is surgery....incision under the breast, around and up to under the shoulder blade of the affected side. A rib is cut, they're spread, the bleb area either removed or stitched up. The inner/outer lung is abraded....I believe mine was manually abraded, not with the use of talc. I call it "crazy-glued"....but it wasn't glue. The inner and outer lungs were stitched together....it's never recollapsed. I had it done in 1975.
I did have blebs on the left lung...few....but it never collapsed. I wasnn't overstrenuous...meaning very sporty....I did do a lot of horseback riding during that time of my life..but never became pregnant ..so, it could be a factor. That's not to say it can never happen if I were to get a severe lung infection .... but I'm not concerned about it.
I wouldn't worry about your son too much...he may be in denial, but don't fool yourself into believing he doesn't think about it. If his lung does collapse..he won't be able to hide it for very long.
There are also varying degrees of collapse....some are mild, others extreme. All mine were different.
One thing I would suggest is to require him to at least start using the corticosteroid inhaler when he has to use his rescue inhaler more often. What he doesn't want to do is use the rescue inhaler a lot....that means there's something up and the underlying cause should be treated.
This attitude may pass as he gets to know his body better and his symptoms. Let him learn on his own and try not to express your fears too much (which I would have a hard time)....
Just do lots of research on pneumothorax and blebs...know what to expect and the different kinds of surgery, treatments..etc. He has to learn on his own through experience...
Best to you both,
It's all a matter of perspective!
Quincy, thanks for your reply and information. I wish I would have checked back on this board earlier. I have been dealing with some health issues and trying to get use to the medication.
I am so glad you'r surgical procedure worked out for you and you havn't had any more pneumothorax episodes. If I may ask.......you mentioned that you have blebs; when you were told this was there an explanation as to why or what caused them????? My son was 16 when he was told (after ct scan of chest) that he had many blebs. My son asked me the day after talking to the Doctor who had talked with the radiologist; why me mom???? I couldn't explain why he was dx with blebs. (The Pulmonary Doc thought it was due to his diaphragmatic hernia__congenital) his lungs had been compressed, and he had his surgery when he was three hours old to have the hole in his diaphram repaired. ?????????
I tried to assure him that if he did have a pneumothorax that the Doctors would be able to help him and that to just be aware, and at that time he was very willing to take his meds. I think now four years later it is denial and it worries me a little but like you said my son really has to go through this on his own. I know he is good about keeping his rescue inhaler in his truck, one by his bead, etc.... When he was younger the preventive azmacort inhaler etc.... kept him out of the hospital a few times for sure. I can just be there for him when he needs me. Being 19 now he is working, going to college, and playing baseball. So far so good.
The talc you mentioned is that still used?????? I hope you are feeling well; it's nice to know I can get some info here I will keep you posted.
You take it easy
Hi summerbreeze.....good to hear from you. I didn't realise you were dealing with a rare disease -- I hope the medication will get you feeling better and that the side effects will lessen with time.
The blebs I had were "congenital" .. I was born with the defect I guess. No others in my family have had a s. pneumothorax. Maybe during the formation of my lungs when I was nothing but cells moving about, there was some disruption...who knows. There seems to be a common body type tho....ectomorph (short body, long limbs, thin..etc). I've inherited that strongly from my dad's side. They're all built like that, dang it.
A severe asthma attack or coughing attack could possibly pop a bleb.....he'll know for sure if his lung has collapsed. But it sounds as though he's in good physical condition, and that will help with keeping his lungs in good working order.
Remember, only one of my lungs collapsed, the other one never did even though there were blebs on it. It may happen when I'm a lot older if I have severe flu or pneumonia or something...but I'm not concerned at this time.
Asthma inhaled preventative meds serve a purpose. They keep inflammation down and help prevent the lungs from scarring from constant inflammation. The only thing you can do is talk with your son and have him promise to use the corticosteroid if he's using the rescue inhaler more often than normal....especially during a cold.
I've found that with males (at least in my family as well as my husband), denial is the norm. It may not be denial, maybe it's just that they truly don't believe in the preventative aspects of dealing with disease/disorders. It has to be bad before taking action. Ticks me sometimes....grrrr! But, my husband's changing, so that's good.
Talc seems to still be used for pleurodesis, although there are many others as well. It does seem to be a stop-gap measure of treatment from my perspective of researching it. One's lung can continue to collapse unless it's properly abraded and inner and outer lung stitched together with the blebs either removed/stitched closed.
At least you have information available incase it ever happens to your son. The "why me" is a normal reaction...we all say that. It's such an inconvenience when our bodies require so much care. Eventually, the care becomes a way of life...accepted. Sometimes life is put on hold. Sometimes it's consistently ups and downs.
Take care of yourself....there is nothing you can do to prevent your son from having a collapsed lung...keep in mind, it may never even happen as well.
I wish you both the best.
It's all a matter of perspective!