The following diseases and conditions of the lung will be considered for a lung transplant.
Obstructive Lung Disease
Emphysema is a chronic lung disease where the air sacs at the end of the lungs are damaged. Owing to this, the air sacs carry less oxygen into the blood. This causes shortness of breath, and difficulty in breathing which worsens over a while. This is a form of chronic obstructive pulmonary disease (COPD). Emphysema is treated by a combination of medication, and therapies to improve breathing. Sometimes surgery is also recommended to remove damaged lung tissue.
• Alpha-1 Antitrypsin (AAT) deficiency
Alpha-1 antitrypsin is a protein in the lungs and bloodstream. A deficiency of this protein increases the likelihood of developing emphysema or COPD (chronic obstructive pulmonary disease) at an early age (between 30 and 40 years of age). This deficiency usually is linked to genetic factors than environmental factors. Your doctor may diagnose you with AAT deficiency if you develop emphysema before 45 years of age, without any accompanying lifestyle factors such as smoking, and/or have unexplained liver disease. Often the treatment for AAT deficiency is medications, along with several lifestyle changes and incorporation of breathing exercises. Sometimes, the doctor may also recommend artificial AAT injections made from human plasma.
• Obliterative Bronchiolitis
Obliterative Bronchiolitis, or constrictive bronchiolitis, is a disease marked by inflammation in the smallest airways of the lungs. The cause of this condition is an infection, or connective tissue disorder following lung, heart, or bone marrow transplant. Often this condition is treated with medication such as corticosteroids, or immune suppressants.
These patients can be candidates sometimes for this therapy.
Interstitial Lung Disease
• Idiopathic Pulmonary Fibrosis
Idiopathic pulmonary fibrosis is a condition where there is progressive scarring of the lungs. This affects the regular function of the lung and causes difficulty in breathing. There is no known cause for this condition. This condition causes shortness of breath, dry cough, and fatigue. As the disease progresses, there is difficulty in breathing even at rest.
Sarcoidosis causes scarring and inflammation in several organs, especially the lungs, liver, skin, lymph nodes, and eyes. The cause of sarcoidosis is not known, and it causes symptoms such as high fever, body aches, breathlessness, and numbness. Sarcoidosis in the lungs is marked by shortness of breath. For severe symptoms, medicines to suppress the immune system, and/or corticosteroids are prescribed. Physical therapy is also recommended to improve muscle strength and reduce fatigue.
• Eosinophilic Granulomatosis
This condition is marked by the inflammation of blood vessels. The inflammation reduces blood flow to organs sometimes causing permanent damage to the organs. This is a very rare condition with no known cure. The symptoms of Eosinophilic Granulomatosis in the early stages are often asthma and sinusitis. Since the symptoms of this condition could easily be mistaken for several other conditions, there are six steps to diagnose the condition:
• Higher than 10% of eosinophils, a type of white blood cells
• Damage to 1 or more nerve groups
• Lesions on a chest X-ray
• Sinus problems
• White blood cells outside the blood vessels.
The treatment for this condition is medications such as corticosteroids, immunosuppressive drugs, and sometimes a monthly injection of immune globulin.
• Occupational Lung Diseases
Repeated and regular exposure to certain irritants can lead to chronic lung diseases. Often people face these irritants owing to the nature of their job. Those working in car garages, textile factories, are often exposed to harmful irritants. Occupational lung diseases are marked by dry cough, shortness of breath, chest pain, and abnormal breathing pattern. These diseases are treated by a combination of medication, and therapies, and lifestyle changes to avoid exposure to the irritants.
• Hypersensitivity Pneumonitis
Pneumonitis refers to non-infectious inflammation of the lung tissue. This inflammation is mostly spread throughout the lungs. This condition is marked by difficulty in breathing and dry cough. Hypersensitivity pneumonitis refers to the kind of pneumonitis which occurs due to exposure to a certain allergen or chemical irritant. This is often diagnosed with blood tests, chest X-rays, CT scans, bronchoscopy, and sometimes requires a surgical lung biopsy. Treatment includes reducing or eliminating exposure to the chemical irritant, and other lifestyle changes apart from medications such as corticosteroids. Some people also require oxygen therapy.
• Drug Toxicity
Lymphangioleiomyomatosis (LAM) is a rare lung disease where abnormal muscle-like cells grow in certain organs such as the lungs, lymph nodes, and kidneys. Over time this destroys the healthy lung tissue and prevents delivery of oxygen to the rest of the body. Signs of LAM include shortness of breath, cough, and fatigue. Lung transplant is the final cure for LAM. However, before that doctors prescribe medication to prevent further deterioration, and to address symptoms of the condition.
Suppurative Lung Disease
• Cystic Fibrosis
Cystic Fibrosis is a condition where mucus becomes thick and builds up blocking passages in the lungs, pancreas, and other organs of the body. This is a chronic disease that is often diagnosed in childhood. Cystic fibrosis of the lungs causes blocks the passages in the lungs causing severe respiratory issues such as breathing problems, and increased lung infections. This disease cannot be cured but requires a lifetime of management with diet, exercise, and a combination of medication and therapies to avoid infections.
Bronchiectasis is a condition where the airways in the lungs become stretched. This happens due to damaged airways, which is often the result of another health problem such as cystic fibroids, or infections such as pneumonia. Sometimes the condition could also be congenital. The damaged airways are usually blocked with mucus and hence they start to stretch, causing swelling and more infections. This condition is treated with a combination of medications to relax the airways, avoid the buildup of mucus, and antibiotics, apart from lifestyle changes, and home therapies to drain the mucus.
Vascular Lung Disease
• Primary Pulmonary Hypertension
Primary pulmonary hypertension refers to high blood pressure in the lungs. The pulmonary arteries carry blood from the rest of the body to the lungs to get oxygen. The high pressure in the pulmonary arteries could eventually lead to heart failure if not treated. The symptoms of the disease include shortness of breath, extreme fatigue, dizziness, dry cough, chest pain, and swelling in the ankles or legs. PPH is diagnosed with a combination of tests such as blood tests, chest x-ray, ECG, Echocardiogram, pulmonary function tests, lung scan, and cardiac catheterization. This condition is treated with blood-thinning medications, diuretics – to decrease the amount of fluid in the body, and drugs to lower the blood pressure in the lungs, and to improve the heart’s ability to pump blood.
• Eisenmenger’s Pulmonary Hypertension
This is a specific condition that is caused by a congenital heart defect. This heart defect if it causes a hole in between the two chambers of the heart, it will cause the blood to flow abnormally between the heart and the lungs. The blood vessels in the lung arteries become stiff thereby causing high pressure in the lung arteries. When the pressure becomes very high, the direction of the blood flow reverses and oxygen-poor blood flows from the right side of the heart to the left side. So, the rest of the body does not receive sufficient oxygen. This condition is treated with blood thinners, antibiotics, medicines to relax blood vessels, and/ or medication to control arrhythmia.
• Chronic Pulmonary Emboli
Pulmonary Emboli is a blockage in one of the pulmonary arteries in the lungs. Mostly this is caused by a clot that travels from the deep veins of the legs to the lungs. These clots stop blood flow to the lungs and hence could be life-threatening. This condition is marked by shortness of breath, cough, and chest pain. This condition is diagnosed with a combination of tests including blood tests, chest X-ray, ultrasound, pulmonary angiogram, and MRI scan. This condition is medically treated with blood-thinning medication, and/ or clot dissolvers. Sometimes surgical procedures to remove the clot are also employed. When this happens repeatedly despite medication, this condition is referred to as Chronic Pulmonary Emboli.
CONTRAINDICATIONS TO LUNG TRANSPLANTATION
Some conditions indicate that the patient should not proceed with lung transplantation because it could cause more damage than benefit to the individual. This section will help you understand these contraindications better.
- Cancer in the last five years.
- Irreversible secondary organ failure unless considered for a combined transplant. However, severe right heart failure will be considered for heart/lung transplantation
- multi-drug-resistant airway colonizers
- HIV infection (though currently under reconsideration)
- Patients with hepatitis B/C- if antigen-positive and histological evidence of disease
- Active user of alcohol, tobacco, or other drugs
- Psychiatric history indicating persistent non-compliance to medical therapy.
- Coronary artery disease. The patient must amenable to surgically revascularize, if for lung transplant only
- If the patient is intubated and ventilator dependent, and unable to move
- Obesity: BMI >30
- Severe GERD – Gastroesophageal Reflux Disease
- Chronic renal impairment with GFR < 50ml/min, unless the patient is a candidate for combined renal transplant
- Diabetes with end-organ damage
- Severe osteoporosis (bone mineral density > 2 SD’s less than predicted for age)
- Active peptic ulcer or diverticulitis
- The patient is only able to walk less than 200 meters in 6 minutes