Lung Treatment

Introduction
Lung cysts and blebs
Pneumothorax Symptoms
Pneumothorax Diagnosis
Pneumothorax Treatment
Thoracostomy
Chest thoracostomy (Chest tube)
Pleurodesis
Chemical Pleurodesis
Mechanical Pleurodesis
Thoracotomy
Video assisted thoracoscopic surgery (VATS)
Lung resection
Pneumothorax Precautions

Introduction

It’s important to know that one of the manifestations of the Birt-Hogg-Dubé Syndrome are lung cysts and blebs that can lead to a collapsed lung, or pneumothorax. Being aware of the symptoms can help you be diagnosed correctly and seek the proper treatment. There are some precautions that may help you avoid having a pneumothorax. Recent analysis of cases of pneumothoraces related to the Birt-Hogg-Dubé syndrome shows that.
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Lung cysts and blebs

A bleb is a sac on the surface of the lung that is filled with air. Think of it as a blister or an air bubble. If it ruptures, it can cause a pneumothorax. Air collects in the chest area, causing the lung to collapse.

Blebs that are related to the Birt-Hogg-Dubé Syndrome may rupture with no apparent cause, but some activities, such as scuba diving, flying, or visiting places in high altitudes, may prompt the rupture.

Unless you have had a CT scan or other test that show that you have the lung blebs or cysts associated with BHD, you may not be aware that you have them. Even if you have blebs or cysts, you may never experience a collapsed lung. In many cases, the lung cysts and blebs associated with the BHD syndrome are not treated.
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Pneumothorax Symptoms

If you have acute chest pains, or suddenly feel short of breath, feel like your heart is beating rapidly, or your skin as a slightly blue tint, you should seek a doctor’s attention right away. If you can’t get a doctor’s appointment immediately, go to an emergency room. We recommend talking to your doctor ahead of time about what you should do if you think you are having a lung collapse so you are prepared to act appropriately.

Be prepared to explain that you have the Birt-Hogg-Dubé syndrome, and that it is very likely that once you have a spontaneous pneumothorax or lung collapse, you will have others. This information may affect the treatment option the doctor chooses.

People with a BHD diagnosis who have lung cysts are more prone to having a pneumothorax than those with no history of lung cysts. There is no way to predict when your lung might collapse. In the general population, tall, thin, athletic males are more likely to have pneumothoraces than other people. The statistics you can find about this do not necessarily apply to people with BHD.

Those who have prior pneumothoraces in their medical history may want to carry a brief record (link to the Tools Overview page) of the treatment received in the past, this could be particularly important if you are traveling.
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Pneumothorax Diagnosis

To determine if you are having a pneumothorax, it is likely that you will have a chest x-ray. You may have a CT scan, but this is not a standard diagnostic tool for lung collapses. It may be used to see if there is other lung disease present, or to confirm a pneumothorax diagnosis. Some institutions use ultrasound as well. Your doctor may check your lungs with a stethoscope.

You may have an arterial blood gas (ABG) analysis. For this test, a needle is inserted into an artery to collect a blood sample. This test may cause a little tingling or cramping just at the site, but is usually a feeling that passes very quickly. This test is sometimes used to evaluate respiratory problems.
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Pneumothorax Treatment

Some people like to take articles explaining the Birt-Hogg-Dubé syndrome with them when they have a doctor’s appointment. You may find that there is not too much published information on the treatment of BHD lung symptoms. It’s possible that some of the recommendations for treatment of collapsed lungs may seem contradictory, ranging from “Let’s observe for a while.” to “Have surgery!” Each case is different, so it is important that you have expert help to determine what treatment is best for you. If you do have more than one pneumothorax, the treatment will depend on each instance, so it may not be the same.

Treatment options for collapsed lungs (pneumothoraces) will vary from case to case. Some will not need treatment. For others, your doctor may propose one of the following:

Thoracostomy

A thoracostomy is a surgical procedure that is used to enable drainage form the chest cavity or pleural space; when you have a pneumothorax or collapsed lung, it is used to make an incision to create a hole so a tube can me inserted to drain air from this area.
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Chest thoracostomy (Chest tube)

If you have a collapsed lung, air from your lung can leak into the space around your lung (pleural space). If this air is removed via a chest tube, the lung can often re-expand on its own, and it may even be able to seal the leak without any other medical intervention. The doctor will determine if this is an option for your pneumothorax.

A local anesthetic may be used to make the process more comfortable for you. A hollow plastic tube is then inserted between your ribs, into the chest area so it can drain the air from the pleural space around the lungs. A suction device may be used to speed up the draining process. Sometimes this tube will be left in place for a few days until it has been determined that the pneumothorax has resolved and the lung is functioning properly again.

It is not unusual to combine a thoracostomy with another type of treatment such as pleurodesis.
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Pleurodesis

Pleurodesis is a procedure that prevents fluid build up in the pleural space, which is the area between the membranes that surround the lungs and the lungs themselves. This procedure causes the membranes to stick together so there is no pleural space. Some people who had pneumothoraces associated with BHD have been treated with chemical or mechanical pleurodesis in association with other treatments such as thoracostomies and throracotomies.
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Chemical Pleurodesis

Chemicals such as talc (perhaps the most common choice), silver nitrate, quinacrine or tetracycline are inserted into the pleural space through a chest tube. Pain medication will most likely be prescribed.
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Mechanical Pleurodesis

Either gauze abrasion ( which can be described as a scouring process) or electrocoagulation procedures are used in Mechanical Pleurodesis.
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Thoracotomy

A thoracotomy is a surgical procedure that opens the chest cavity by making an incision through the chest wall. This is a more extensive procedure than a thoracostomy and is used for lung surgery, to look more closely at the lungs, and to remove a lung or part of a lung.
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Video assisted thoracoscopic surgery (VATS)

This technique enables the surgeon to observe the lung area from within the body. Diagnostic procedures and surgeries such as lung resections can take place using this technology. It is possible to insert a video camera on a thin scope into the chest area using a smaller incision than a Thoracotomy. Studies suggest that VATS leads to shorter hospital stays, faster recovery and less pain than open surgeries.
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Lung resection

This is the surgical removal of a part of a lung or a whole lung. If your surgeon suggests a lung resection, make sure that he or she is aware that you have BHD and may be prone to recurrent pneumothoraces. It is important to review each case individually before making a decision to have a lung resection. If possible, get a second opinion so you can make an informed choice. In some cases, this is the best option.

It is possible to be treated with a combination of one or more of the above treatments.
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Pneumothorax Precautions

If you have a BHD diagnosis, consult a pulmonologist to determine a schedule of scans so you can determine if you have lung blebs. Have your own emergency plan in mind for getting to an ER or doctor as soon as possible. Take a medical history with you when you travel, along with a description of the pneumothorax as a manifestation of BHD.

Other precautions:

  • avoid flying in unpressurized airplanes
  • consult with your pulmonologist to determine if commercial flights are a safe option for you
  • avoid scuba diving
  • consult with your pulmonologist before undertaking sports and strenuous activity in high altitudes
  • don’t smoke

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Have you had these treatments and can you offer any advice on them, or would you like to know more? Why not post your advice or questions on our forum?

Sources

We have included sources here that are easily accessed through the internet for your convenience: