Cystic Fibrosis: Thoracic Endometriosis And What It Means For CF Women

Thoracic endometriosis is a rare phenomenon but it does occur in a small percentage of women causing monthly bouts of hemoptysis (coughing up blood). It is caused by rare form of endometriosis where endometrial-like tissue is found in the respiratory system (lung) creating plaque like legions. Driven by hormones, the excessive endometrial tissue eventually sheds just the same way as in the womb, creating a purge of blood, basically creating a “lung period”. Yes, you read that right! Your lung CAN have a period.

The most common symptoms of thoracic endometriosis are:

• Cough
• Shortness of breath
• Chest pain and tightness (usually before a period)
• Fatigue

But, I Already Have All These Symptoms With CF!

I hear you, having Cystic Fibrosis (CF) I was no stranger to coughing up blood or the associated symptoms outlined above. My largest bleed was about 300ml and I’ve had several bronchial emoblizations without much luck.

Bronchial Embolization, huh?

For those who have no idea what I am talking about… this procedure is just a really fancy way of saying I am having the leaky blood vessels in my lungs patched up with glue, a bit like repairing a bike tyre! Snazzy.

If this procedure is required, a chest CT scan is completed to identify the areas of weakness. If the CT scan shows little evidence of leaking vessels, or you’ve had a fair few lung repairs without success then I urge you to continue reading and, start learning a thing or two about the “lung period”.

How It All happened For Me

After 4 embolizations, I was still having regular small bleeds but my CT scans were showing no obvious signs of ruptures. It was a scratch your head kinda moment.

I decided to keep a diary for 6 months to see if I could identify triggers, and it coincided that my lung bleeds happened every time I was on my monthly menstrual cycle. Day 2, like clockwork. Great.

Next clinic, I mentioned it to my consultant, thinking she is going to think I really have lost the plot because I’m always coming up with something new my weirdly wonderful body is doing! But, I was surprised to learn, it is a thing. A very uncommon thing but, it WAS a thing.

It turns out the cells that line the female reproductive organs are the same as the cells that line the lungs. Mind blown.

Diagnosis & Treatment

There is no known cause or cure for endometriosis. To develop thoracic endometriosis, you already need to have endometriosis of the female reproductive organs. In my case, I always had heavy, difficult periods but I thought that was normal so didn’t get an endometriosis diagnosis until we started to put the whole picture together with my lung bleeds.

Symptoms Of Endometriosis In The Female Reproductive Organs

• Pain in your lower tummy or back (pelvic pain)
• Heavy periods (needing to use extra pads and tampons or leaking though)
• Extreme bloating (I always thought this was bowel blockages because of CF, turns out it wasn’t)
• Acute period pain, causing you to stop managing your daily routines (if you’re curled up in fetal position, get help)
• Pain during or after sex
• Pain when urinating or opening your bowels (again masked by CF for me)
• Difficulty getting pregnant (CF problem too, I’m noticing a pattern here)

1 in 10 women suffer with endometriosis and on average it takes 7.5 years from onset of symptoms to get a diagnosis.

Diagnosing Endometriosis Is Tricky Business

The only way to give a definite diagnosis is to have a laparoscopy under general anesthetic, mine confirmed I had endometrial tissue on my ovaries, bowel and bladder. This was removed whilst I was under and an IUD device was fitted in place of the contraceptive pill.

Thoracic endometriosis can be identified using different methods such as; X-ray, CT scan, MRI or a biopsy. These diagnostic tools render useless when dealing with a CF patient as we already have too much lung damage to know what is what. So, your best bet is trying a hormone therapy to see if it stops the bleeds. If it does, hey presto, you’ve found your answer!

First Line Of Treatment

After meeting with a gynecologist, he suspected I had both conditions and started me on the combined contraceptive pill to manage the symptoms. The theory is that by using the hormones in the pill to trick your body into not having periods, the endometrial tissue doesn’t have a chance to grow or thicken as quickly as it normally would.

For this treatment to work, it is important you use it back to back for three full months and then break for 1 week and repeat. I started that in August 2018 and I have not had a lung period since which indicates confirmation of thoracic endometriosis.

What To Do If You Suspect You Have Endometriosis

It may sound obvious but keep a diary and then speak to your team! I think the Big Dogs like some evidence of what’s going on before referring you onto gynecology. I noted down my current health (if I was on antibiotics or had low iron) as these things can all help to work out what’s really causing the problem.

When treating a CF patient, it is impossible to say thoracic endometriosis or lung damage are causing the bleeds but, if you feel like there is a relation, it is worth speaking to you team about it or asking for a referral to a gynecologist. It may mean you need to treat both.