Bilateral Diaphragmatic Paralysis
What is Bilateral Diaphragmatic Paralysis?
Bilateral diaphragmatic paralysis (BDP) is a rare condition in which both sides of the diaphragm, the primary muscle responsible for breathing, become weak or completely paralyzed. The diaphragm muscle separates the chest and abdominal cavities, and impaired function impacts the lungs’ ability to fill. This leads to significant respiratory impairment, particularly during sleep (lying flat) and physical exertion, as the diaphragm fails to contract properly to facilitate normal breathing. Individuals with BDP often experience breathing difficulties, especially when lying down, due to the loss of diaphragmatic function. The loss of respiratory function is more severe than unilateral diaphragmatic paralysis, in which only one side of the diaphragm has impaired function. People with unilateral diaphragmatic paralysis may lose 50% of total lung capacity, while those with bilateral diaphragm paralysis lose 70-80% total lung capacity.
Synonyms
- Bilateral diaphragmatic dysfunction
- Bilateral phrenic nerve paralysis
Bilateral diaphragmatic paralysis (BDP) is a rare condition in which both sides of the diaphragm, the primary muscle responsible for breathing, become weak or completely paralyzed. The diaphragm muscle separates the chest and abdominal cavities, and impaired function impacts the lungs’ ability to fill. This leads to significant respiratory impairment, particularly during sleep (lying flat) and physical exertion, as the diaphragm fails to contract properly to facilitate normal breathing. Individuals with BDP often experience breathing difficulties, especially when lying down, due to the loss of diaphragmatic function. The loss of respiratory function is more severe than unilateral diaphragmatic paralysis, in which only one side of the diaphragm has impaired function. People with unilateral diaphragmatic paralysis may lose 50% of total lung capacity, while those with bilateral diaphragm paralysis lose 70-80% total lung capacity.
BDP is rare, with its exact prevalence unknown. It occurs less frequently than unilateral diaphragmatic paralysis, which affects only one side of the diaphragm. The condition can affect individuals of all ages but is most commonly seen in adults with underlying neuromuscular diseases or after certain medical procedures.
| Name | Abbreviation |
|---|---|
| Bilateral diaphragmatic dysfunction | |
| Bilateral phrenic nerve paralysis |
BDP can result from various underlying conditions that affect the phrenic nerves, spinal cord, or neuromuscular function. The phrenic nerve sends signals to the diaphragm that cause it to contract, becoming thicker and flatter to allow room for the lungs to fill with air. Less signaling from the phrenic nerve to the diaphragm causes the muscle to relax, the lungs to compress, and the body to exhale. Thus, damage to the signalling between the phrenic nerve and the diaphragm will reduce the lungs’ ability to fill with air. Common causes of phrenic nerve damage and/or diaphragm muscle damage include:
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Neuromuscular Diseases: Conditions such as amyotrophic lateral sclerosis (ALS), muscular dystrophy, or Guillain-Barré syndrome.
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Spinal Cord Injuries: Trauma affecting the cervical spine (C3-C5), where the phrenic nerves originate.
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Surgical or Medical Procedures: Cardiac surgery, cervical spine surgery, or radiation therapy that damages the phrenic nerves.
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Infectious or Inflammatory Conditions: Poliomyelitis, viral infections, or autoimmune diseases that cause nerve inflammation.
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Idiopathic Causes: In some cases, the exact cause is unknown.
The primary symptoms of BDP are related to respiratory dysfunction, particularly during sleep and physical activity. These include:
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Shortness of Breath (Dyspnea): Worsens when lying down (orthopnea) and improves when sitting or standing.
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Sleep-Related Breathing Disorders: Symptoms of sleep apnea or shallow breathing during sleep, leading to fatigue and morning headaches.
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Weak Cough: Reduced ability to clear mucus, increasing the risk of respiratory infections.
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Exercise Intolerance: Difficulty breathing during physical exertion.
Fatigue and Weakness: Due to chronic low oxygen levels and poor ventilation.
| Name | Description |
|---|---|
| Immersion in Water on Muscle Function and Breathing | doi: 10.1378/chest.125.6.2069 CHEST June 2004 vol. 125 no. 6 2069-2074 Dyspnea is a common symptom in patients with diaphragm weakness or paralysis. In particular, dyspnea may be aggravated by immersion. We hypothesized that immersion to the neck in water would decrease vital capacity and consequently increase the demand/capacity ratio of the respiratory muscles. |
BDP is diagnosed based on clinical symptoms, medical history, and specialized tests to assess diaphragmatic function. A thorough evaluation is necessary to determine the underlying cause and severity of the condition.
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Pulmonary Function Tests (PFTs): Measures lung capacity and how well the lungs function, showing a restrictive pattern.
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Diaphragmatic Ultrasound: Assesses diaphragm movement and confirms paralysis.
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Fluoroscopy ("Sniff Test"): A real-time X-ray that evaluates diaphragm motion when the patient takes a quick sniff. Lack of movement confirms paralysis.
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Arterial Blood Gas (ABG) Analysis: Measures oxygen and carbon dioxide levels in the blood, identifying respiratory insufficiency.
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Electromyography (EMG) of the Diaphragm: Assesses the electrical activity of the diaphragm to determine nerve or muscle dysfunction.
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Phrenic Nerve Conduction Studies: Evaluates phrenic nerve function, which controls the diaphragm.
Treatment for BDP depends on the underlying cause and severity of respiratory impairment. Children diagnosed with BDP must be treated right away to avoid neurological damage. Options include:
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Non-Invasive Ventilation (NIV): BiPAP (bilevel positive airway pressure) or CPAP (continuous positive airway pressure) to assist breathing, especially during sleep.
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Diaphragmatic Pacing: Electrical stimulation of the phrenic nerve to improve diaphragm function in select patients.
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Surgical Intervention: Procedures such as diaphragm plication (surgical tightening of the diaphragm) may help in certain cases.
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Oxygen Therapy: Used in cases of severe respiratory distress.
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Physical Therapy and Pulmonary Rehabilitation: Helps improve breathing techniques and overall lung function.
The prognosis for BDP varies depending on the cause and response to treatment. Some individuals experience gradual improvement, especially if the underlying condition is treatable. Others may require long-term ventilatory support. Without appropriate management, BDP can lead to chronic respiratory failure, significantly impacting quality of life. Early diagnosis and intervention are essential for improving outcomes and preventing complications such as pneumonia or respiratory failure.
| Name | Description |
|---|---|
| swimming | Watch out when you swim that you always can reach the ground. With swimming there is no ability to take air in when prone. If you stand the gravity works for you to allo0w taking air in. Short dips are going to be the limit. DO NOT DIVE IN AND TRY TO SWIM WITHOUT KNOWING HOW DEEP YOU ARE!! |
Last updated 24 Jun 2014, 10:47 PM
24 Jun 2014, 10:47 PM
I had a physical Jan 2014 in which everything seemeed ‘normal’ except for the breathing problem. Blood work was ‘perfect’. Over the years I have learned to work with the physical limits I have. I often wonder if the people that die from heart attacks are really sometimes caught with this breathing problem but were unable to get air before passing out and dying. I mentioned this to doctors who tell me that I don’t know what I am talking about. The interesting thing is when I first came to the hospital years ago- all the medical energy was spent taking care of my heart since it was racing, blood pressure was up, headache, etc. Just a note --Chronic Inflammatory Demyelinating Polyneuropathy had also been mentioned at the start of the problem. Doing -OK
28 Feb 2011, 09:41 PM
I had an exam last week and found out that I am about the same as I was last year as far as the different mechanical test are concerned. I am now used to the condition and have made life style changes. My love of water activities has been curtailed to a large extent even thought I still get wet but watch out depth and the area. When I was working thru disability rulings I thought an old injury that hit my diaphgram area back in 1997 was the primary cause. All the doctors said there was too much time between that accident and my problem. That accident had caused a trip to emergency and was documented with Workers Compensation. Sleep apnea developed after the accident. This developed after the sleep apnea. Oh, by the way I don't smoke and enjoyed an active life with sports,swimming, hiking, worked in construction,etc. I think the doctors could not explain the cause and therefore would not entertain any possibilities
14 Mar 2010, 05:20 AM
So your diaphragm never came back? There is some hope that it will/may - At least the L side, or more likely the L - I received IVIG when diagnosed on that side - with the R didn't really know what was going on - thought it was due to this brace I was wearing post hamstring repaing and extremely rapid deconditioning (wrong on both counts!) The headaches I had but only at first - though my pulmonolgist did get me a bipap machine thinking I was underventilating so much I was retaining CO2. But I think my body is adjusting - I can sleep lying down, flat even! - and am finding I can go a bit further in Yoga, riding bikes.....I have no underlying pulm disease, so I feel incredibly fortunate that it is truly only (?) a mechanical issue.....Did both sides go at once for you? did they ever figure out "what" was the cause? Autoimmune? That's what they think mine is.....trigger by 1) surgery then 2) a virus. Guess my hope to be a deep sea skin diver are out! (just kidding....never wanted to be!)
Last updated 4 Jan 2010, 02:14 PM
4 Jan 2010, 02:14 PM
At this point I am looking to get a medical ID bracelet but these are limited to the amount of information they can carry. Due to the condition even the doctors don't know what is most critical to have available. Any suggestions would be helpful.
Last updated 20 Jul 2009, 01:38 PM
20 Jul 2009, 01:38 PM
I have always loved swimming and have in the water since I was little. This year when the pools opened after the hoilday I took my daughter & a friend for swimming. I found that with this diagonsis I prevents me from going under like I used to do. The water bouancy affects the lungs. Without a diaphgram and with the water supporting the body there is no way to get air into the lungs. If I am in the water my lung capacity is related directly to the amount of body (chest area) out of the water. Full breath results when I I am standing in the pool waist deep. The breathing capacity decreases as I lover myself. Just a word. You can dive in as long as you can get right back out!!.
Last updated 1 Mar 2009, 12:29 AM
1 Mar 2009, 12:29 AM
I am currently in Pulmonary Rehab which I think is helping. The orginal testing in Oct '08 for the phrenic nerve was not able to find anything. Since no one has found a cause it is labeled idiopathic-NO KNOWN CAUSE. I went from fairly active one day to emergency room and breathing problem the next. What the difference a day makes. There seems to be no known cure but, possibe idiopathic cure. . I am located in the Washington D.C. metro area where there are doctors who are also learning as I go thru this process. Neurogolist at George Washington are using IVIG treating this as CIDP (Chronic Infammatory Demylenation Polyneuropathy) --eyesight seems to be getting better --not sure of breathing.
28 Feb 2009, 04:05 AM
I was orginally diagnosised Aug 2008. Ogrinal lung function was 45% but that has increased. I have been involved with IVIG treatments 5 days a week for the past 3 months. Current lung function is about 70%. Improvement is also helped by Pulmonary Rehab which is strenghtening chest muscles since the diaphrgam doesn't work.
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