A Matter of Life and Breath: Living with Interstitial Lung Disease

“Dr. C, please can you refer me to a pulmonologist? I am still feeling short of breath when I do anything physical, I have this dry cough, and I’m just exhausted all the time.”

Dr. C is my PCP. She takes her stethoscope and places it on my back and then my chest, having me take deep breaths while she listens. I can’t breathe nearly as deeply as I used to, but she shakes her head and says, “Sorry, but I don’t hear anything, so I can’t refer you to a pulmonologist. There has to be a reason.”

This is the third time we have had this discussion in the past year. I know I must find a way around this. So….I wait until I know she will be gone for a couple of weeks and I make an appointment to see Dr. A, who covers for her.

When I ask Dr. A the same question, she uses the stethoscope but then uses a spirometer and also tests my O2 with oximeter. She says the reason nothing is heard with the stethoscope is because I have restrictive lung disease, not congestive. And that the dry cough is a sign of this, as well as “it is not good that you cough after exhaling. And your lung capacity on the spirometer is only 75-77% of normal. I am making a stat referral to Dr. J in pulmonology.”

Dr. J saw me the next week. He ordered high-resolution lung CT scans and did further in-depth spirometry testing and took a very extensive medical history. When I came back for the results, he showed me the CT scans on the screen and explained the white parts of my lower lungs were “ground glass opacities” where the lung cells are dead.

You may have heard of ground glass opacities in the lungs as resulting from serious COVID infections but this may also occur as a result of …. you guessed it: an AiArthritis Disease co-condition. And the combination – getting COVID when you have an AiArthritis Disease -- will up your lung risks severely.

There are actually hundreds of types of ILD, but the autoimmune type is our focus here. Autoimmune Interstitial Lung Disease or Autoimmune ILD is usually associated with RA, Sjogren’s, Lupus, or Scleroderma but it may also be seen with some other AiArthritis Diseases.

Dr. J began to work with me on treatment. He explained he was not going to do a lung biopsy because it wouldn’t change the treatment and the risk of a lung infection from the procedure would be too high in someone on immune-suppression.

Why do I share this story with so much detail? Because you may run into what I did: a PCP not willing to refer you because they have no knowledge of restrictive lung disorders or ILD.

So…..if you have the symptoms of difficulty breathing, a chronic dry cough, and chronic

fatigue, start by telling your rheumatologist. Don’t wait too long! We can never bring those dead cells back to life, but we can slow down (not stop, but definitely slow) the death of more cells in our lungs.

Your rheumatologist will know that these symptoms could be ILD. They may refer you directly to a pulmonologist or they may recommend that your PCP do this, depending upon how your health plan works.

Autoimmune ILD severity depends on how advanced it is when you are diagnosed, the quality of healthcare you receive, how old you are, and how many other health conditions you have. Also, sadly, in the U.S. and some other healthcare systems, a huge factor in your prognosis is how much access you have to quality healthcare. Sometimes a medical social worker can assist you.

Treatment:

After diagnosis, it takes a while to discover which medications are best for you. First your pulmonologist may have you try some oral medications, then progress to inhalers and/or a nebulizer. The diagnosis of ILD may even influence which immune- suppression treatments your rheumatologist prefers for you.

If we have multiple conditions ALL our providers will need to know of our new diagnosis and our pulmonologist may need to consult with our other specialists.

Medications are not the only treatment:

• When the lungs weaken, the heart has to work harder, so we need to take care of our heart as well as our lungs.
  
• ILD eventually may lead to heart failure, so controlling blood pressure and following a heart-healthy eating style are also very important. You may request to receive a referral to a Registered Dietitian Nutritionist for help designing a personal plan.
• Breathing exercises and cardio exercises such as walking, cycling or swimming help keep our lungs working well and our heart strong. You can ask to be referred to a
• Physical Therapist or even a brief cardiac rehab program to build up your exercise tolerance.
• Many pulmonologists are also “sleep doctors” and can test you for sleep apnea (cessation of breathing while sleeping). Untreated sleep apnea will greatly stress your heart and lungs, so if your doctors suggest this testing/treatment, it is essential. Breathing is fundamental!

Diagnosis and Treatment is the beginning of our path….yet at the same time, we are faced with the emotional impact of yet another diagnosis, one which is serious. How to cope?

Let’s talk about that in Part Two of this series...stay tuned for A Matter of Life and Breath: Coping with the Diagnosis of Autoimmune Interstitial Lung Disease

(author: Denae Cedar, RDN)