Where Do We Turn When Prescribed Therapies Aren’t Effective?

Charlene Marshall avatar

by Charlene Marshall |

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Managing symptoms is an ongoing challenge for chronically ill patients. Whether they’re caused by an exacerbation, disease progression, or environmental factors, it’s often difficult for patients to get quick relief, including from respiratory symptoms.

Despite their name, rescue inhalers aren’t always effective in managing or improving pulmonary fibrosis symptoms. Neither are oral steroids. Unfortunately, since my diagnosis of idiopathic pulmonary fibrosis (IPF), I’ve dealt with this dilemma more than once. When my breathlessness or cough flares up, I struggle to find relief, even with prescribed therapies.

The past few weeks, I’ve been dealing with an increase in unpleasant respiratory symptoms, including shortness of breath, fatigue, and discomfort across the front of my chest and shoulders. I don’t have a cough, but shortness of breath is noticeable in everything I do.

I’ve been through a plethora of tests with my transplant team to see what might be causing the increase in symptoms, but everyone seems stumped. In an effort to provide me relief, my doctor prescribed inhaled steroids.

One steroid is delivered via a Turbuhaler, but I’m no longer able to use it effectively. Accessing the medication from a Turbuhaler requires a deep breath, but I don’t have the lung capacity anymore. So, the doctor prescribed an inhaler that mists at the click of a button, and I inhale the medication normally. He was convinced this would help, but I’m not.

I pride myself on being a compliant patient — you have to be in the transplant and chronic illness world. This means I take my medications on time, keep notes about how I feel, and try not to push my body too hard. As a result, I’ve tried my doctor’s prescriptions, but I’m struggling, and I suspect they may be worsening my symptoms.

Connecting with other patients

Despite being compliant, I also am active in my care. This means I’ll sometimes seek answers for things my doctor and I disagree on. Or, at the very least, I’ll ask clarifying questions to help me understand diagnostic testing, a treatment regimen, or bloodwork results.

Lately, I’ve been connecting with IPF and cystic fibrosis patients about the effectiveness of inhaled steroids. Following are recommendations when therapies aren’t helping.

Consider alternative delivery methods

It makes sense that the fastest way for medications to reach the lungs is via inhalers. However, taking medications this way sometimes isn’t possible for someone with a lung disease. Inhaled therapies can trigger a cough in respiratory patients, and for those with IPF, once a cough starts, it can be hard to stop.

When struggling with therapies, you may want to consider alternative methods of delivery. In my case, a fellow respiratory patient recommended asking my doctor if the inhaled medications are available in oral form. I plan to ask my pulmonologist about it this week.

Adjust dosages

Sometimes the amount of medications we put into our bodies can cause unpleasant side effects. When I was struggling to receive a diagnosis, many doctors thought I had asthma, so they prescribed Ventolin (albuterol). The amount of Ventolin they wanted me to take caused me to shake excessively, which was hard to tolerate.

I also had a hard time initially managing Ofev (nintedanib), one of two anti-fibrotic therapies approved by the U.S. Food and Drug Administration for IPF. I had to stop taking my originally prescribed dose in the beginning because of gastrointestinal (GI) symptoms.

After my doctor weighed the benefits of taking this drug, we tried again by adjusting the dosage. I was finally able to tolerate it, along with a gradual increase in dosage later.

Ask other patients for suggestions

I believe other patients are the best source of information about managing a rare disease. I have learned so much from fellow members of the PF News Forums. One tip I like to share with those who are struggling to manage Ofev-related GI symptoms is to purchase sublingual doses of Imodium instead of oral tablets.

The suggestions other patients provided were invaluable as I sought answers about how to manage new symptoms when my prescribed therapies weren’t effective.

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Note: Pulmonary Fibrosis News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Pulmonary Fibrosis News or its parent company, BioNews, and are intended to spark discussion about issues pertaining to pulmonary fibrosis.

Comments

Landy G, Prieto avatar

Landy G, Prieto

Thank you for posting . I totally agree with your comments. You are very young, I am 83 years old and was officially
diagnosed with IPF on September 2019. I say officially because in 2014 a chest X-Ray showed mild PF , my primary care physician comment was that I had no symptoms, and this was probably hereditary. I believed him. On August
2015 I had a scan of my heart due to fatigue and extra heart beat. Cardiologist stated my arteries had zero blockage,
so I was happy. I did not completely read the report stating that severe fibrosis of the lungs. In 2019 , severe dry cough
I , on my own, made an appointment with a Pulmonologist and CT Scan, blood work , pft tests, etc. made diagnosis
official. Since then I have educated myself as much as I can , I know what happened to me is not uncommon.
My Pulmonologist , at my request, gave me a direct answer to the severity of my condition.
I believe a young person like you should stay positive , IPF is getting some attention.
Support groups and a forum like this keeps me aware of clinical trials, etc.
I live one day at a time and enjoy the good days. Again, thank you for sharing.

Reply
Charlene Marshall avatar

Charlene Marshall

Hi Landy,

Thank you so much for reading my column and reaching out via the comments, I always appreciate hearing from readers! Unfortunately it sounds more and more common that people have been living with PF for many years before being diagnosed, which is so upsetting as early intervention with anti-fibrotics is so important. You're doing everything right in terms of educating yourself, and being an advocate for your own healthcare, keep up the great work. Take good care and thanks for writing, and sharing your kind words!
Charlene.

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Steve Dragoo avatar

Steve Dragoo

Hi Charlene,
I deeply empathize with your dilemma. You are in my prayers.
your friend,
Steve

Reply
Charlene Marshall avatar

Charlene Marshall

Thank you so much Steve - I deeply appreciate you and wishing you well, always.
Take care,
Char.

Reply
Rhonda Wilkens avatar

Rhonda Wilkens

I was diagnosed with PF in 2020. I had been caring for my husband with advanced Parkinson’s Disease and dementia, who recently died. As a former hospice nurse for 20 years, before I quit to care for my husband, I ignored a dry cough for 2 years, until I was coughing up blood. I am taking Humira, Imuran, 40 mg Prednisone, and a steroid inhaler.
In 2016 I was diagnosed with Crohn’s disease. Before starting me on Humira, a chest X-ray and blood work was done. All I was told was that everything was okay to start
the Humira. I recently started looking up my electronic medical records. I discovered that I had pulmonary fibrosis on the January 2016 chest X-ray, although I was asymptomatic. I would have educated myself about PF, and been aware that a dry cough / inflammation be treated right away to minimize lung scarring.
I don’t know why the doctor did not share that important information with me.

Reply
Charlene Marshall avatar

Charlene Marshall

Hi Rhonda,

Thanks so much for reading my column and reaching out via the comments. I'm so sorry to hear of your PF diagnosis after caring for your husband; what a difficult situation for you and to come from such a caring and giving profession too. I'm really sorry to hear your experience with the doctor too, that much feel very frustrating to not have had the opportunity to educate yourself more on the disease. We are here for you, so please reach out as you need and take good care.
Char.

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