Mediastinal fibrosis

Name itself is dangerous. I am writing this blog because if you google this, you will find Wikipedia link wherein the definition of the disease is given in one sentence, and the description is in a single sentence :

Mediastinal fibrosis is a rare late complication of histoplasmosis. It is characterized by invasive, calcified fibrosis centered on lymph nodes that block major vessels and airways.[1]

There is not much known/written about this disease. It happens to only 2% percent of the total population. So its kinda very rare.

Before a few months, my father suddenly had a hand swelling which started from palm till elbow in an unusual way. As usual, he waited for a day or so thinking that it would be the result of  unusual sleeping posture etc. and later went to a normal physician. The doctor gave him allergy tablets. But the swelling continued for a week.

My uncle is general surgeon and in our family, if anyone has any health issues, we do consult him. My uncle sensed something unusual in this and asked us to go for X-ray and few other tests.

After seeing X-ray, he asked us to consult the chest physician and the chest physician took our case. My father is diabetic and suffered from left vocal cord paralysis few years back (just for background) but anyway, this is immaterial as the doctors say. He asked my father to undergo numerous tests. Now since medical language is not a layman’s language, we had to do all the tests. My father was admitted to Lilavati hospital for around 3-4  days for performing all the tests in one go. We were eagerly and curiously waiting for the results forgetting sleep as my father had to undergo PET- SCAN also. This test can be used to:

• Check brain function
• Diagnose cancer, heart problems, and brain disorders
• See how far cancer has spread
• Show areas in which there is poor blood flow to the heart

Then, we went to the doctor with reports in hand. Our chest physician along with the team of other doctors had to write a report for the results of the tests. He diagnosed it correctly as Mediastinal Fibrosis, along with Thrombosis and pulmonary fibrosis. Now till all these diagnostics, another week passed by. And my father also developed a very thin layer swelling on face. Even near to his left side of chest, a vein started protruding and distinctly visible. Even his lip color begun darkening on the blue side.

So actually the hand swelling was due to a blockage in vein of the hand. And they also had found the blockage in a vein which goes to brain. So it goes like this. Mediastinal fibrosis in layman’s language means that near to the major airway (respiratory tract in my father’s case), there was a covering of tissues formed on the outer side surrounding the airway/ heart etc. This lead to the vein blockages in my father’s case. It can also spread to other parts of body. This was creating pressure on his respiration due to which he used to have breathlessness while climbing stairs. This covering cannot be removed by any type of surgery, radiation etc. There are no medicines for curing the disease either. Even the root cause is unknown. The only possible option for the person to survive remains is Angioplasty.

My father underwent Angioplasty in two of the veins. It also led to some emergency conditions for my father even though the angioplasty was a success. But that does not happen to every individual. After all these hardships that we faced, my father is doing really well and has started to do daily work. But he has to still keep up with the artificial oxygen for a few days. It is actually a lethal disease if not diagnosed at an early stage.

Symptoms :

1. Breathlessness while climbing stairs
2. Swelling over face, leg. (more prominent in legs than hands)
3. Darkening of lips. Bluish color of lips/nails.
4. Distinctly visible protruding vein in chest area.
5. Blockages in veins.

Tests that are generally performed :

1. CT scan
2. Mediastinoscopy
3. Spirometry
4. chest X-ray
5. Bronchoscopy
6. PET-SCAN

Angioplasty is generally preferred and performed to tackle the situation.