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Idiopathic Pulmonary Fibrosis: Treatment, Procedure, Cost and Side Effects

Last Updated: Apr 10, 2024

What is the treatment?

The Idiopathic Pulmonary Fibrosis (IPF) is a sort of chronic lung disease which is generally characterized by a progressive and irreversible decline in the functions of lung. It is also called IPF.

How is the treatment done?

Anyone who has been diagnosed with the Idiopathic Pulmonary Fibrosis has an unique experience with the disease, and there is no “standard” or a expected clinical course to cure the disease. Some of the patients remain in the stable condition for an extended period of time, but generally the situations worsen. Most of the patients of Idiopathic Pulmonary Fibrosis experience a rapid progression of the symptoms like shortness of breath and difficulty in breathing, many experience a stepwise deterioration over the time which fluctuates between the periods of stability and the worsening symptoms.

To diagnose your condition, the doctor is going to review your medical and family history, and will discuss the different signs, occurrences and symptoms and also may review the exposure you have or had to dusts, gases and chemicals, and will conduct sorts of physical exams. During the physical exam, the doctor will use a stethoscope to listen carefully to your lungs and will check your breathing patterns. The doctor may also suggest one or more of the tests like Chest X-Ray, CT Scan or Echocardiogram. The doctor might also suggest lung function tests like Pulmonary function testing, Pulse Oximetry and Arterial blood gas test.

Who is eligible for the treatment? (When is the treatment done?)

The lung scarring which occurs in the idiopathic pulmonary fibrosis cannot be reversed, and at present or currently there is no treatment available or has proved to be effective in stopping the progression of the lung disease. Some treatments are there which may improve the symptoms temporarily or slow down the progression of idiopathic pulmonary fibrosis and others may help to improve quality of life. Doctors generally evaluates the severity of your state to determine the most appropriate treatment for your condition.

Accordingly the idiopathic pulmonary fibrosis treatment strategies are very much individualized, and varies from one person to another, generally based upon a individual’s medical history and other important conditions.

Different medications are used to treat Idiopathic Pulmonary Fibrosis, but will vary from one to another. Only your medical provider can determine whether you should take any medicine to treat your Idiopathic Pulmonary Fibrosis or not, and if any, it will depend on your conditions and medical history. Medications like Cytoxan, Imuran, Celicept and the like are sometimes used by doctors to treat Idiopathic Pulmonary Fibrosis.

As there is no proper treatment available with the medications, lung transplant can also be done to treat Idiopathic Pulmonary Fibrosis.

Who is not eligible for the treatment?

There are no specifications, but the diagnosis of the idiopathic pulmonary fibrosis primarily relies on the clinician to integrate and correlate the clinical, laboratory, radiologic, and/or pathologic data.

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Are there any side effects?

It depends on the diagnosis of the idiopathic pulmonary fibrosis. But someone with a very weak condition, no ideal body weight and bad physical condition besides your lung function cannot be treated.

What are the post-treatment guidelines?

The idiopathic pulmonary fibrosis is generally accompanied by shortness of breath, also called dyspnea. Due to attack on lung, it translates it to dry cough and fatigue weakening the condition of the individual. The individual will experience unexplained weight loss with deformation of health. This will cause acute fatigue and cause aching of muscles and joints in the body. Soon the body will start experiencing widening and rounding of the tips of the fingers or toes (also called clubbing).

The body fails to retain fluid and as a result individuals experience loss of appetite with Coughing, Chest pain and Fatigue (tiredness) with disturbed sleep.

How long does it take to recover?

Though the disease is not curable, but during the treatment or post medications, the patents are advised to stay away from receptors like Anticoagulation (warfarin), Imatinib, which is a selective tyrosine kinase inhibitor acting against platelet-derived growth factor (PDGF) receptors, combination prednisone, azathioprine and N-acetylcysteine. Idiopathic Pulmonary Fibrosis is generally sensitive to such recpetors.

Patients are also recommended to stay away from selective endothelin receptor antagonist (ambrisentan), Phosphodiesterase-5 inhibitor (sildenafil) and Dual endothelin receptor antagonists (macitentan, bosentan).

What is the price of the treatment in India?

There is no specific time period, as Idiopathic Pulmonary Fibrosis is not treatable. The patient has to undergo medications on a continuous basis, and has to maintain himself in order to stay in a better condition.

Are the results of the treatment permanent?

The cost of Idiopathic Pulmonary Fibrosis treatment in India can ranges from somewhere between 2 lakhs to 25 lakhs, but that varies. The cost will depends on certain criterias such as if the patient requires a lung transplant, or if any surgery is required. The medicines are very rare and costly at the same time.

What are the alternatives to the treatment?

The Idiopathic Pulmonary Fibrosis or the lung scarring that occurs in pulmonary fibrosis cannot be reversed, and there is no treatment which has proved effective in stopping progression of the disease. There are treatments which may improve symptoms temporarily or slow the disease's progression, but there are no permanent treatments available.

References

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Written ByDr. Mool Chand GuptaMD - Pulmonary,MD PULMONARY,DTCDPulmonology
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