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The respiratory system consists of the upper and lower airways, it's task is to take oxygen from air that is breathed in, and to expel carbon dioxide on exhalation. The upper airways consist of the nasal passage, the oral cavity, the pharynx and the beginning of the windpipe or trachea. The lower airways consist of the rest of the trachea and its branches to the lungs; the two left pulmonary lobes and the three on the right. The alveoli are also found here, they exchange the oxygen and carbon dioxide.

Which diseases does a lung doctor treat?

The following list gives some examples, click on underlined items to read more:


Asthma

What is asthma?

Asthma is an inflammation of the airways. Because of the inflammation the airways become narrower. The symptoms are being short of breath, squeaky breathing and coughing. It's not always easy to say precisely what is wrong when someone has these symptoms. Some people that are short of breath and cough think that they have chronic bronchitis, but they actually have asthma. On the other hand, others that suspect they have asthma are actually suffering from chronic bronchitis, being overweight, heart disease or lung emphysema. Whenever you get these sorts of symptoms you should consult your doctor.

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Asthma doesn't usually get better of its own accord. When nothing is done about the symptoms they can become worse, and in the longer term cause irreversible lung damage. Of course it is sensible as an asthma sufferer to avoid the conditions that trigger an attack. For example stay out of smoky environments, don't have pets, keep your home as dust free as possible and make sure that rooms are adequately ventilated. However these sorts of measures are often insufficient to prevent shortness of breath. Fortunately there are effective medicines against asthma, with which the shortness of breath can usually be relieved.

Treatment is by inhalation and has two aims:

  • Inhibit inflammation
  • Widen the airways

Today there are also medicines available in tablet form which inhibit the inflammation process. However these drugs are only intended for a certain group of asthma patients and may only be prescribed by a lung doctor.

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Allergy

What is allergy?

Allergic reactions are reactions of the body's immune system, which can damage normal, healthy body tissue. When we talk about allergic reactions we are referring to reactions within which antibodies (so called IgE antibodies) play a role. When the antibodies come across antigens (in this case allergens) chemicals are secreted which damage the surrounding body tissue.

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Allergens are molecules against which, normally spoken, such reactions shouldn't be triggered. Examples include pollen, dust mites, the exfoliated skin of animals, mould etc.

Sometimes the term Atopic diseases is used to describe bronchial asthma, hay fever (allergic rhinitis), itchy eyes (conjunctivitis), eczema and such like.

Allergic reactions can range from light to serious. The can vary from watery, itchy eyes, to life threatening difficulty with breathing.

A diagnosis can be made on the basis of the patient's symptoms, skin tests or blood tests. Treatment focuses principally on the removal or reduction of allergic stimuli rather than the blocking of allergic reactions. Besides that there is also treatment to relieve the symptoms.

Can allergies be prevented?

Allergies are prevalent in children, and specifically children from families in which a close relative already suffers from asthma or allergy. Such infants run a higher risk of developing a similar condition. It's possible that the general increase in allergies results from living in an environment that is too sterile, or put another way, nowadays we and our children live too clean lives.

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Bronchitis, COPD and lung emphysema

What are bronchitis, COPD and lung emphysema?

Bronchitis is inflammation of the airways. We recognize several distinct forms:

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  • Acute: usually occurs with or after a cold and is caused by a virus or bacteria. In general this passes by of its own accord, though sometimes antibiotics are needed; symptoms are coughing and tightness of the chest.
  • Chronic: this sort of inflammation doesn't pass, though there will be periods when the symptoms are not so bad followed by a flaring up of the condition and worse problems. Symptoms include coughing, sometimes with sputum and in some patients shortness of breath (during physical exercise and/or in rest). If the bronchitis is coupled with a permanent narrowing (obstruction) of the airways this is known as COPD (chronic obstructive pulmonary disease). If COPD is accompanied by loss of alveoli this is described as COPD with lung emphysema.

Asthma, lung emphysema and chronic bronchitis all lead to inflammation of the airways. But the inflammation resulting from asthma has a different cause, also the treatment is different. As a result we now speak about Asthma and COPD (Chronic Obstructive Pulmonary Diseases). COPD is the new name for chronic bronchitis and lung emphysema.

Smoking is the most important cause of COPD and chronic bronchitis only occurs in adults. When children have 'bronchitis' it is usually the result of a viral infection.

Someone with COPD can't become completely healthy again. It is a chronic disease and the damage caused cannot heal. However effective treatment can help to prevent further damage and to alleviate the symptoms.

It is important to take the symptoms seriously. Medical treatment from a lung doctor is one part of tackling the problem, besides that you can do a lot yourself.

If you smoke we can already give you important advice: STOP. If you don't, chronic bronchitis will become still worse and can lead to more serious consequences.

Do I have COPD?

If you smoke and cough a lot this is a sign that there's something wrong, especially when you have so called "smokers cough". Sometimes such a cough leads to the generation of phlegm. When you are short of breath or wheezing it's time to take the symptoms seriously and consult a doctor.

Many people think that shortness of breath is normal when you become older. This is certainly not the case. The effectiveness of breathing does decline, but breathing even in old age should be without problems. If you become short of breath when you climb the stairs or when you walk a short distance, that is sufficient reason to talk to your doctor.

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Lung cancer

We refer to lung cancer when the primary tumor originates in the lung. Two main forms can be distinguished:

  • Non-small cell lung cancer
  • Small cell lung cancer

Metastases from other sorts of cancer can also appear in the lungs for example in patients with breast cancer. Cancer of the visceral pleura consists of 2 forms:

  1. originating from the pleura itself (mesothelioma)
  2. metastases from other sorts of cancer (pleuritis carcinomatosa)

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Non-small cell lung cancer

What is it?

The distinction between small cell and non-small cell lung cancer reflects the characteristics of the cells, but also says something about the speed of growth of the tumor, and the speed with which it disseminates to other parts of the body. Naturally these characteristics influence the treatment that is chosen.

The non-small cell form is characterised by relatively large cells. These cells also have a certain arrangement in the tissue. On the basis of a number of characteristics of the cell arrangement we distinguish between:

  • squamous (scaly) cell tumor;
  • adeno carcinoma;
  • and large cell carcinoma.

The rate of growth of these different forms varies: the squamous cell tumor grows the slowest and a large-cell tumor the fastest. Moreover these cell types spread relatively slowly (via the lymph vessels to the lymph nodes and via blood vessels to various organs) through the body. In which stage of tumor growth this actually happens is not clear. This uncertainty results from the fact that the tumor will typically have been in the body for a long time before it is discovered. There may be many years between the initial formation of the tumor and the time when it is discovered. During this time the tumor may already have spread through the body.

What causes lung cancer?

The most important cause is cigarette smoking. Also cigar and pipe smoking increase the chances of getting lung cancer, as do certain professions. Triggered by protracted exposure to dangerous materials, changes in the cell's genetic material occur, these lead ultimately to uncontrolled growth. The cell is no longer influenced by normal controls and grows unhampered at the cost of healthy tissue in the area. These cells also tend to nestle elsewhere in the body where they grow into metastases.

What are the symptoms of lung cancer?

The symptoms are very varied, and are dependent on the location and growth of the tumor and of any metastases. At first, when the tumor is relatively small, there may be no symptoms and the tumor is often found by chance. Subsequently it turns out that vague symptoms existed, such as not feeling totally fit, a reduction in appetite and sometimes becoming thinner. Complaints that tend to be associated with lung cancer include: a changing pattern of coughing, coughing up blood, a gradually worsening shortness of breath, pain in the chest, and pain elsewhere in the body from possible metastases.

What investigations are done when lung cancer is suspected?

Medical investigations aim to establish if it really is lung cancer, and if so, the extent of any metastases. It is also important to discover if a person has any other medical condition which may rule out certain forms of treatment.

A doctor will ask a patient questions to establish possible causes of the symptoms that the patient is suffering. They will also want to know about the general health of the patient. Specific attention is paid to heart complaints and any pre-existing lung complaints. The doctor will examine the patient noting any swollen glands or swelling of the liver. Blood tests don't help with the immediate diagnosis of a tumor but can indicate if there are metastases in certain parts of the body, for example the liver or the bones.

Important investigations include: a chest x-ray, in which a possible tumor can be seen and a CT-scan of the chest and upper abdomen, in which the precise location of the tumor can be determined and its relationship with the surrounding tissue. The CT-scan also reveals any swollen lymph nodes or liver metastases. A bronchoscopy (looking in the airway with a flexible observation instrument) also forms part of the investigation, during which it is possible to extract a tissue sample for further research. Depending on the symptoms and results of completed investigations (the physical examination and blood tests) further research may include investigation of the bones (body scintigraphy) or a CT-scan of the brain, which may or may not be followed by further tests by a neurologist.

Some large hospitals may be able to do a PET scan, which gives a better insight into whether secondary tumors exist in other parts of the body. In order to be certain that no metastases exist in the lymph nodes in the chest the surgeon conducts a mediastinoscopy (a keyhole operation behind the breast bone in order to remove samples of lymph node).

The doctor will also check heart and lung function through means of an ECG conducted whilst doing exercise. The condition of these organs has an important influence on treatment possibilities.

What are the treatments?

The treatment possibilities for lung cancer are:

  • surgical operation, in which the tumor and surrounding tissue are removed
  • radiation (radiotherapy), in which the tumor and bordering lymph nodes are radiated in an attempt either to heal the area or to reduce the symptoms
  • treatment with medicine that kills cells (chemotherapy), for when the disease has already produced metastases

Combinations of the above may be used including:

  • chemotherapy followed by surgery
  • chemotherapy followed by radiotherapy
  • surgery followed by radiotherapy
  • .

Which treatment is most appropriate depends on the results of the tests. If the disease is localized, and lung and heart functioning is good, then an operation will be appropriate. If there are metasteses another treatment will be chosen. If the metastases have only reached the local lymph nodes then chemotherapy followed by surgery or radiotherapy (a still experimental approach) may be appropriate. If there are remote metastases then chemotherapy will be prescribed.

Small cell lung cancer

What is small cell lung cancer?

About 20% of lung cancer cases are small cell. This type of cancer is characterized by the very small, delicate cells which divide especially quickly. They can spread rapidly through the body. Often small cell lung cancer has already spread by the time that symptoms are noticed. Its treatment is completely different from non-small cell lung cancer. Mostly it is not treated through an operation but using radiotherapy and chemotherapy.

The symptoms

The symptoms of lung cancer aren't only caused by the tumor, secondary tumors through the body which influence the functioning of other organs will also cause complaints.

Symptoms that result directly from the tumor include: coughing, production of blood with the phlegm, shortness of breath and sometimes squeaky breathing and repeated flaring up of inflammation in the airways.

A range of symptoms can appear as a result of the secondary tumors. Swollen lymph nodes can compress the upper vena cava (vena cava superior syndrome) characterized through swollen veins in the neck, a swollen face and arms and complaints of headache and blood rushing to the head when bending over. Hoarseness can result from damage to nerves that go to the vocal cord as a result of pressure from the swollen lymph nodes or the tumor itself. Pain in the chest can occur through inward growth of the chest wall. Swallowing problems are caused by swollen lymph nodes that can force the esophagus closed.

Because this type of tumor spreads quickly symptoms can exist in other parts of the body. Pain in the back, arms or legs can be caused by secondary bone tumors. Headache can be the result of metastases in the brain. Liver metastases give pain and can also result in jaundice.

The tumor cells can also secrete a chemical, or influence the endocrine glands (glands producing or expelling specific elements). This may cause the sodium level in the blood to drop or the calcium level to rise. Men may also become more 'feminine', possibly developing breasts and experiencing a change in hair growth pattern.

Investigating lung cancer

If there is a suspicion that someone has lung cancer many test must be done. It must be determined if there is indeed a tumor, how big it is and which treatments are appropriate.

The doctor begins by taking a case history (asking focused questions) in order to determine if the symptoms are really those associated with a malignant lung tumor. It is also necessary to establish if there are other health problems, specifically in the heart or lungs. A physical examination follows looking for swelling in certain parts of the body. A lung x-ray is made to identify any abnormalities.

A CT scan gives insight into the condition of the surrounding area and the presence of swollen lymph nodes. Simultaneously images of the liver and adrenal glands are made. A bronchoscopy (looking in the airway with a flexible tube) makes it possible to remove samples of tissue for further research.

After the diagnosis

If the tests indicate the presence of small cell lung cancer yet more investigation follows. Because small scale lung cancer spreads so fast it is assumed that the disease has also affected other parts of the body unless this is proved otherwise. In addition to the CT scan of the chest, a bone scan and brain scan are also likely. Through laboratory tests any metastases are traced and investigated to see if there are abnormalities in the sodium and calcium levels. Measuring the number of white blood cells and platelets is necessary because of the possibility of treatment with cytotoxic drugs.

The role of the PET scan in diagnosis of small cell lung cancer is still being developed.

Treatment

The tests should have determined whether the disease is local or if it has already spread.

If it's local then the tumor and surrounding tissue can be removed surgically, followed by chemotherapy. The chemotherapy is to wipe out any very small metastases that weren't evident in tests. Often the small cell diagnosis is only made after an operation.

If surgical removal of the tumor isn't possible because there are too many metastases, treatment by chemotherapy is chosen. This consists mostly of 6 courses of various combinations of cell destroying medicines. If the disease is restricted to the chest and the response to chemotherapy is good, radiotherapy follows to tackle the tumor and glands in the chest and the head. The last part is necessary because the chemotherapy doesn't reach the brain cells effectively and thus microscopic metastases are not eliminated.

Survival chances

Estimation of survival chances inevitably relies on averages. That limits their value when considering any specific case.

In general the prognosis for non-small cell lung carcinoma is better than for small cell. Still, the treatment results for lung cancer with secondary tumors are disappointing. Chemotherapy delivers only a limited increase in life expectancy.

As a result, the emphasis in the treatment of lung cancer is moving more towards prevention and early diagnosis.

  • Prevention in the sense of stopping smoking and understanding genetic factors better.
  • Early diagnosis of patients is already possible with high risk patients through the means of a certain bronchoscopy technique. Using a special light source the inside of the airways can be investigated and suspect places, invisible to the naked eye can be recognized.

    In Barcelona there are hospitals (including CIMA), which are already committed to this form of preventive medicine.

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Sarcoidosis

What is Besnier Brock's syndrome / Sarcoidosis?

Sarcoidosis, or Besnier-Boeck-Schaumann disease, is a disease of unknown origin, in which many organs become inflamed. Sarcoidosis appears with varying frequency throughout the world, affecting especially those between 20 and 40 years old.

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The disease has various manifestation and the location and seriousness of inflammation determine the clinical picture. The condition can be acute or chronic. The duration of the (sub)acute condition is mostly less than 2 years, the chronic generally longer.

The symptoms vary significantly: temperature, weight loss, tiredness and pain in the joints can be the first signs. Sarcoidosis without symptoms may also be discovered by chance, for example a test may be done following the indication of abnormalities on a thorax x-ray. The lungs are the most affected part of the body. On an x-ray swollen lymph nodes can be seen, sometimes with abnormalities in the lungs themselves. This often leads to coughing and shortness of breath.

Sarcoidosis also occurs in the skin, in which case it begins with a painful red swelling, mostly on the shins (so called erythema nodosum), this is coupled with temperature and pain in the joints. There is also a (sub)acute form of sarcoidosis that manifests itself with a general sick feeling, temperature, erythema nodosum, joint pain and sometimes inflammation of the eyes (uveïtis) that together are known as Löfgren syndrome. This occurs mostly in young women.

Sarcoidosis can also occur in the liver, brain and bones.

The disease is classified in four stages:

  • Stage I: only gland swelling is visible on a thorax x-ray
  • Stage II: gland swelling and stripy or small round abnormalities are visible on the x-ray
  • Stage III: swelling of the glands is not visible on the x-ray, but there are extensive abnormalities in the lungs
  • Stage IV: lung fibrosis is seen, with serious changes in the thorax x-ray including collapse and destruction of lung tissue

The diagnosis is based mostly on the evidence of an x-ray; and in the case of skin abnormalities by means of a biopsy. If the problem only exists in the lungs testing will be supplemented with microscope research on tissue and/or fluid taken from the lungs. This is achieved through means of a bronchoscopy or a bronchoscopy lavage (lung wash). The latter reveals if there is a change in the number of white blood corpuscles in the rinse fluid.

The blood can be tested for angiotensin converting enzyme (ACE) and also the ratio of white blood cells (the CD4/CD8-ratio).

Treatment of the disease is only necessary in a minority of cases. To reduce serious complaints such as shortness of breath and pain in the joints corticosteroids (prednisolon) may be prescribed, but this treatment is reserved for patients with serious lung complaints. With the chronic condition sometimes long or short term treatment with corticosteroids or symptom inhibiting medicines (non steroid anti-inflammatory drug= NSAID) is appropriate.

The prognosis is variable. Most people with sarcoidosis recover spontaneously within 1 to 2 years, this occurs in 70-80% of cases. In 10-15% of cases the acute stage becomes chronic. With black patients the disease lasts longer and is more serious. Being older than 40 leads mostly to a delay in the spontaneous recovery.

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What are the consequences of smoking?

Pulmonologists work daily with, sick smokers and patients that have become ill from smoking or have problems from being in a smoky atmosphere.

Smoking causes two diseases that determine a large part of our work: lung cancer, which in the Netherlands strikes 1 in 10 men (and a growing number of women), and chronic obstructive pulmonary disease (COPD) which will disable one in 5 smokers in old age. Of the young who currently smoke, half will become chronically sick from smoking, their average age will be reduced by 8 years and they will spend 15 years of their old age in a chronically sick condition.