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Thread: Severe Restrictive Lung Disease

  1. #1
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    Default Severe Restrictive Lung Disease

    Hi. Tried to figure out how to search but new to group and so decided to just post.

    Our son is 17, severe cerebral palsy. I realize that complicates things because the issue isn't just scoliosis. At 12 yrs. old he went into puberty. Spine was pretty much ok before that but he also had very little trunk control. The initial curve was 47 degrees. Six months later it was 82 degrees and a few months after that they did spinal surgery. He was 13. From xrays it "appeared" that he may have pretty much stopped growing. Around 15 years old or just before he did one last growth spurt. He developed crankshaft. The vertebrae rotated with the ribcage. The curve increased. He has a stable kyphosis, stable lordosis and somewhere between 76 to 85 degree broad "C" thoracolumbar curve to the right.

    The result is that the right lung is smaller than it should be and compressed between the spine and the ribcage. He has the elongated hump on the back right side. The right chest is flat and pushed back and the left lung is enlarged and ribcage very protruding. The sternum is more off to the right. He has decreased lung volumn. He has increased difficulty moving secretions. He had a 3-d CT of the ribcage and spine, which was pretty interesting because you can see the total deformity of the ribcage, and the rotation of the spine. With the rotation he ended up with the bar going between the two rods "poking" the skin from inside on one corner. That turned into infection and he had surgery to remove the bar and deal with the infection. Bacteria is on the rods but they cannot remove the rods safely, cannot do surgery without the high risk of total pulmonary collapse, plus the one rod is almost more to the side where the rotation occured. He is on lifetime antibiotics to confine the bacteria to the rods.

    The diagnosis is severe restrictive lung disease. He is on daily breathing treatments. We've noticed more issues during seasonal allergies. So far no pneumonia. Big thing is the secretions and all. We do suction for that. We now have an oxygen concentrator machine in the house...haven't had to use it to much so far. The big things we have been told is flu or any respiratory infection would be a major issue and that even with antibiotics he may not be able to fight it.

    I realize the odds of anyone here dealing with a similar situation is probably pretty slim. I have joined numerous groups related to CP/scoliosis and seems like the combo of both and restrictive lung disease is not that common? I don't know. My main question is this. We understand the whole aspect of respiratory infection/pneumonia being an issue. What I'm wondering is everything I have read on line says that when a lung is restricted then the volumn is decreased, the air flow is affected. When I listen to the right lung from the side I can hear just a short intake and out take. My understanding is that if there is not adequate air intake then that affects the oxygen/carbon dioxide exchange, shallow breathing becomes the norm and the air sacs can begin to close down (Atelectasis) causing the lung to become stiff. If the condition persists then the lung may not be able to re-expand.

    This sounds to me like even if our son avoids say pneumonia, the flu, bronchitis, etc. that the mere aspect of the situation can be progressive. That the lung can suffer long term damage to to the poor exchange. We were told that the breathing treatments were primarily to assist the left lung is being at it's optimum...keep the airway/bronchi clear of mucous/secretions and open but that it would not help the right lung.

    The thing is that for the past year we thought "ok..just watch out for him getting the flu this winter etc." Then we saw was allergies did when we went through a two week period of his heart rate high, sats in low 90's and watching him struggle to breathe...resulting in the oxygen in the house and having to use that. Now I'm thinking that yeah, those things are a concern but long term wise my gutt says this is progressive no matter what and that at some point we will start seeing more and more issues and possibly pneumonias.

    Sorry this is so long. I'm not even sure what I hope to get from this group. I think it's just a bit frustrating. There is so much out there for obstructive lung disease. Information and support groups etc. Restrictive lung disease is like "oh yeah...and there is that one". Add cerebral palsy and it's almost like "forget it". I guess I just want to know if anyone is dealing with restrictive lung disease and it can't be corrected by surgery and what impact it's having on them.

    Thank you for taking the time to read this.

    Karen

  2. #2
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    Default Re: Severe Restrictive Lung Disease

    Hi Karen,

    I'm afraid I don't know much about CP in connection with restrictive lung disease. I'm going to read up on it and get back to you. I'm sorry to hear of the situation you're in. It sounds very difficult and upsetting.

    I have Spina Bifida and resultant NM scoliosis, not the same, but I can relate to some of the issues such as lack of trunk control and.

    Born 1981 with Spina Bifida Myelomeningocele. Surgery to close lesion at birth. Anterior fusion T11-L5 aged 12. Laminectomy June 06. Posterior fusion with instrumenation T8-T11. Degenerative Disc C4/C5

    Waiting for VP Shunt surgery. Under the care of Mr. Tucker who wants to fuse me T2-pelvis!

  3. #3
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    Default Re: Severe Restrictive Lung Disease

    Hi Karen

    Reading your post and what your son is going through makes me realise how incredibly lucky I am to just have scoliosis

    I feel totally inadequate as I don't know the answer to any of your questions. All I know for certain is that your son is extremely lucky to have such a devoted mum who is actively searching for answers to help his condition. I hope you can find the help he needs.
    Me, a Mod? Nah ... I'll always be a rocker

  4. #4
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    Default Re: Severe Restrictive Lung Disease

    Hi Karen, I just wanted to wish your incredibly brave son all the very best! Respect and best wishes .
    "Pursue your ambitions,face life's challenges and never give up"

    March 15 2010 was definitely the best decision I've ever made in my life!

  5. #5
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    Default Re: Severe Restrictive Lung Disease

    Hello and Welcome to SSO. What a full plate of problems you have. I consider myself lucky to only have Kyphoscoliosis and Restrictive Lung Disease. I have had no treatment for scoliosis.So my lung capacity is decreased and my ability to get rid of carbon dioxide actually socks. I complained for years and GP's were telling me that I was having migranes and just needed to take pain meds. My husband also told them that I stopped breathing several times in my sleep. No help! Finally changed doctors and discovered an underlying pneumonia, and horrible lung function. After testing and sleep study - Oxygen prescribed and Bipap machine ordered for nights. The migranes are gone and I feel so much better and sleep much better. The O2 machine etc. are a constant pain in the butt with cords everywhere and concentrator noise but I feel lucky that someone heard us finally.
    I am 61 years old and the resident SSO fossil. I live in Oklahoma,USA with my husband Allen. We have one daughter Jae and she has three kids.Our grandkids are: Aidan is8. He's the one pictured in my current avatar. Jenna Jean is 7 and Ryan Allen is just 4 It's full time chaos here! I was diagnosed in 1965 at 14 years with Kyphoscoliosis and 2 curves measuring 68 and 63 degrees. My last measurements were in 2004 at 155, 88 and 55+ degrees. I have never had surgery or bracing so I now am on full time oxygen and use a Non Invasive Ventilator at night. at night.

  6. #6
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    tonibunny is offline T Sr: Admin, Big Sister and Da Police!
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    Default Re: Severe Restrictive Lung Disease

    Hiya, welcome to SSO

    I'm so sorry your son is having these problems. You may like to send a private message to Di B (via this link) whose 20 year old son Daniel has CP and had surgery for scoliosis too.

    Toni xx

    PS I have 48% lung function due to having had severe scoliosis as a baby (62/40+ degree double curve) - some of my lung tissue was never able to develop properly and I had that tissue removed during surgery when I was 10. In my case, because I am otherwise healthy, I have always tolerated it quite well.
    37 years old, diagnosed with infantile idiopathic scoliosis at 6 months old with curves of 62(T) and 40(L) degrees. Casting and Milwaukee braces until surgery at 10 - ant release/pos fusion T1-T12, halo traction. Post op cast and then TLSO. Further surgery at 18 (ant release/pos fusion extended to L3 to include lumbar curve, costoplasty) and 25 (another costoplasty). Fusion extended to L4 at 33 (XLIF with 4 pedicle screws and two short rods). Pre-op curves: 76(T) and 70(L). Post-op curves: 45(T) and 35(L). Diagnosed with Ehlers-Danlos Syndrome aged 34; scoliosis almost certainly due to this rather than being idiopathic.

  7. #7
    Join Date
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    Default Re: Severe Restrictive Lung Disease

    I have over 130 degree curve. I am a 30 year old that sleeps on a vent at night since the age of 7. I am looking for others to talk to who are in similar situations. Please contact me by emailing me through my profile.

    -Tanya

  8. #8
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    Default Re: Severe Restrictive Lung Disease

    Hi Tanya

    I don't know what my degrees are now but I have an appointment on February 11th and will find out then. The measurements below were 2 years ago. My breathing is very shallow though. Fortunately the other day my sats were 99%.

    Anyway, I can partly relate to your situation. I hope you like it here.
    Born 1981 with Spina Bifida Myelomeningocele. Surgery to close lesion at birth. Anterior fusion T11-L5 aged 12. Laminectomy June 06. Posterior fusion with instrumenation T8-T11. Degenerative Disc C4/C5

    Waiting for VP Shunt surgery. Under the care of Mr. Tucker who wants to fuse me T2-pelvis!

  9. #9
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    Default Re: Severe Restrictive Lung Disease

    Hiya,

    I just wanted to welcome you, I'm Lyssie xxx
    "Your time is limited, so don't waste it living someone else's life. Don't be trapped by dogma - which is living with the results of other people's thinking. Don't let the noise of other's opinions drown out your own inner voice. And most important, have the courage to follow your heart and intuition. They somehow already know what you truly want to become. Everything else is secondary..." - Steve Jobs

  10. #10
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    Home town: Seattle, WA. Currently living in Wakayama, Japan.
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    Default Re: Severe Restrictive Lung Disease

    Hi Karen,
    I wish I knew more about what your brave guy is dealing with, but I don't know that much about CP/Scoliosis....
    All the best! You can always come here for support!

    Hi, I'm Hannah! Diagnosed w/ AIS along with my twin sister at the age of 13. My curves advanced to 99(T) and 54(L). Needless to say my sister and I had/have a hump on our backs.
    Had the anterior release---halo traction---posterior fusion surgery in July-September 2010, in Japan--> curves down to 42(T) and 20(L)


  11. #11
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    Jun 2011
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    Lake Mary, FL
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    Default Re: Severe Restrictive Lung Disease

    Hi! I am new to the site. I am pnly occaisionally suffering from symptoms of restrictive lung disease, but have been diagnosed with it. It seems to me that the symptoms are worsened by stress. Has anyone else found this to be true? I am currently in the middle of a several day run of fairly severe symptoms and it is freaking me out a bit. i am trying to get in to see someone who can advise me what to do but in the meantime i am afraid to be alone and feel terrible all the time. Would appreciate anyone's comments or advice. I am 56 and had surgery with harrington rods when I was 13.

  12. #12
    Join Date
    Jan 1970
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    Edmond,OK USA
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    Default Re: Severe Restrictive Lung Disease

    I am rapidly approaching 60 and my lungs are just getting more restricted every year. I am sorry to say the general outlook isn't the best but I keep in pretty close touch with my Pulmonologist and he reads me like a book. Recently he did an all night study of oxygenenation and wasn't pleased. So my oxygen has been bumped up to 3.5 litres and my Bipap machine is up to 17/6. I am a really shallow breather so I don't get rid of the CO2 that I need to hence the Bipap to help. I am starting to have headaches again so that isn't a great sign. One thing I might throw out though concerns helping yourself. It is natural for everyone to automatically open your mouth when you need more oxygen. So walking or doing anything I would a nasty headache because I was opening my mouth to get more air. Except when you breathe through your mouth you aren't taking in your normal amount of O2 through the nasal canula. Headache!!! I have to consciously think... CLOSE YOUR MOUTH and let the O2 work. It does help.
    It goes with out saying that we need to avoid sick people and going out and about where there are lots of germs. I am no longer allowed to go to schools, churches etc. wherever there are crowds of people. I wish you the best luck and PM me or email me anytime.
    I am 61 years old and the resident SSO fossil. I live in Oklahoma,USA with my husband Allen. We have one daughter Jae and she has three kids.Our grandkids are: Aidan is8. He's the one pictured in my current avatar. Jenna Jean is 7 and Ryan Allen is just 4 It's full time chaos here! I was diagnosed in 1965 at 14 years with Kyphoscoliosis and 2 curves measuring 68 and 63 degrees. My last measurements were in 2004 at 155, 88 and 55+ degrees. I have never had surgery or bracing so I now am on full time oxygen and use a Non Invasive Ventilator at night. at night.

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