One patient’s journey to managing a chronic lung disease

Posted: February 01, 2022 | Word Count: 1,980

For the longest time, Judy thought her persistent cough and chronic pneumonia were something she just had to live with. It wasn’t until she cracked a rib and ended up in the emergency room that she realized how serious her condition was. It was at that point Judy was diagnosed with MAC lung disease, which is a serious infection caused by a type of bacteria called Mycobacterium avium complex (MAC).

MAC are the most common type of bacteria in the U.S. that cause nontuberculous mycobacterial (NTM) lung disease, so doctors often call an NTM infection "MAC" or "MAC lung disease." MAC bacteria are usually found in soil and water, and can be breathed in during daily activities, such as showering or gardening.

While most people who come in contact with MAC do not develop an infection because their lungs are healthy enough to clear the bacteria, people with lung conditions, including bronchiectasis, chronic obstructive pulmonary disease (COPD), pneumonia or asthma, are more likely to develop MAC lung disease.

Some people don’t realize their symptoms may be a sign of MAC lung disease. Dr. Pamela McShane, a specialist in pulmonary and critical care medicine at the University of Texas Health Science Center at Tyler, says the most common symptoms of MAC include cough, fatigue, shortness of breath, loss of appetite, and weight loss. “It’s true that other diseases such as bronchiectasis, pneumonia and COPD have similar symptoms, but with the addition of pulmonary MAC infection, patients may experience a worsening of their usual symptoms. Because of this, patients may suspect their underlying lung condition is getting worse, but in reality, it may actually be a symptom of MAC.”

MAC Lung Disease Diagnosis

For Judy, it was a relief to finally have a diagnosis and explanation for her persistent cough. “Getting to a correct diagnosis is probably the most challenging part of MAC lung disease. I was relieved when I finally had a diagnosis, but I wasn’t prepared to ask questions about the condition and my initial treatment plan. Now, I’ve learned that the best way to manage my MAC lung disease is to work with my doctor on my treatment plan and make sure I’m taking notes and asking questions.”

Looking back on how important it was to ask questions and be an advocate for her own health, Judy says that there’s one question that she makes sure to ask at the close of every appointment, “Is there anything more I should be asking that I haven’t yet?”

Talking to Your Doctor About Treatment

Treatment of MAC lung disease is important because if left untreated, lung damage can get worse. Treatment typically involves multiple antibiotic therapies as well as lifestyle changes, such as airway clearance techniques, diet changes and ways to reduce your exposure to MAC bacteria. Dr. McShane stressed the importance for patients to talk to their doctor about establishing a treatment plan, what side effects may occur and strategies to manage them. “It’s important for patients to keep track of how they are feeling and communicate with their care team. In-person visits are crucial for testing and the physical interaction really helps the physician to evaluate if treatment is working. As a supplement, telehealth is a great tool to communicate with my patients in between visits and helps them manage any side effects from treatment.”

In Judy’s case, one thing that helped her prepare for appointments was to compile a list of questions and concerns before each appointment. In her experience, “It’s creating that partnership with your team of doctors and asking them to explain and show you all of your scans and tests, that helps make you and your doctor more successful with identifying solutions that may work for you.”

It’s also important to stay in contact with your physician to understand when you will start to see treatment results. Judy tried different antibiotics for 12 years, but the infection persisted. Tired of continuously testing MAC-positive, Judy began searching patient websites for more information about the disease and potential treatment options. It was then that she learned about ARIKAYCE® (amikacin liposome inhalation suspension). After discussing it with her doctor, they decided that adding the medication to her treatment plan was her best option.

Dr. McShane says that ARIKAYCE is for patients whose sputum continues to test positive for MAC bacteria after 6 months of guideline based antibiotic therapy, and is recommended by the International NTM Treatment Guidelines, which were recently updated and published in 2020. “ARIKAYCE works differently than other inhaled antibiotics because the amikacin is contained inside tiny particles called liposomes. These liposomes are designed to help the medicine reach the lungs. In the clinical trial, results demonstrated that adding ARIKAYCE was more effective at converting patients’ sputum to MAC-negative than oral antibiotics alone. In addition, patients who received ARIKAYCE were more likely to stay MAC-negative, even after completing their treatment compared to patients who only received oral antibiotics.”

Since starting ARIKAYCE, Judy was excited to recently receive her third negative sputum culture test. “I was so excited when I found out my sputum samples were MAC-negative. I’m thankful that I didn’t give up after failing other treatments and continued to advocate for myself by working with my doctor to discuss and initiate treatment with ARIKAYCE.”

ARIKAYCE is the first and only FDA-approved treatment designed specifically to treat refractory MAC lung disease as part of a combination treatment plan in adult patients who did not respond to combination treatment alone.

This product was approved by the FDA using the Limited Population pathway. This means the FDA has approved this drug for a limited and specific patient population, and studies on the drug may have only answered focused questions about its safety and effectiveness.

When Judy was prescribed ARIKAYCE, she was also enrolled in the Arikares Patient Support Program, a program provided by Insmed, which helps patients get started on treatment and become familiar with taking it. Additionally, the Arikares Patient Support Program provides information about insurance coverage and financial support options, as well as voluntary in-person or virtual training about how to use the Lamira® Nebulizer System for ARIKAYCE. Judy says she’s thankful for the support that Arikares provides, “The onboarding of ARIKAYCE with the Arikares Support Program was easier than I thought, especially when it came to receiving information about possible financial support options. It was all pretty smooth – actually it was very smooth.”

Understanding the Possible Side Effects

Everyone responds to treatment differently. Part of any treatment plan is understanding and managing side effects. Dr. McShane says that it’s important to communicate expectations around treatment, “I ensure that my patients understand their treatment plan; including how long they may be on therapy, the goals of treatment, how we will be tracking progress and the possible side effects including what to do if they experience them.”

If you do experience any side effects while taking ARIKAYCE, be sure to talk to your doctor. When you have a plan for dealing with side effects, you have a better chance of taking treatment as prescribed and testing MAC-negative.

ARIKAYCE can cause serious side effects, including:

  • allergic inflammation of the lungs. These respiratory problems may be symptoms of allergic inflammation of the lungs and often come with fever, wheezing, coughing, shortness of breath, and fast breathing
  • coughing up of blood (hemoptysis). Coughing up blood is a serious and common side effect of ARIKAYCE
  • severe breathing problems. Severe breathing problems can be symptoms of bronchospasm. Bronchospasm is a serious and common side effect of ARIKAYCE. Bronchospasm symptoms include shortness of breath, difficult or labored breathing, wheezing, and coughing or chest tightness
  • worsening of chronic obstructive pulmonary disease (COPD). This is a serious and common side effect of ARIKAYCE
  • serious allergic reactions. Serious allergic reactions that may lead to death have happened to people who take ARIKAYCE. Stop taking ARIKAYCE right away and get emergency medical help if you have any of the following symptoms of a serious allergic reaction: hives, itching, redness or blushing of the skin (flushing), swollen lips, tongue or throat, trouble breathing or wheezing, shortness of breath, noisy high-pitched breathing (stridor), cough, nausea, vomiting, diarrhea, feel cramps in your stomach area, fast heart rate, feeling light headed, feeling faint, loss of control of the bowels or bladder (incontinence), and dizziness

While using ARIKAYCE, these side effects may become serious enough that treatment in a hospital is needed. Call your healthcare provider or get medical help right away if you have any of these serious side effects while taking ARIKAYCE. Your healthcare provider may ask you to stop using ARIKAYCE for a short period of time or completely stop using ARIKAYCE.

Do not use ARIKAYCE if you are allergic to any aminoglycoside, or any of the ingredients in ARIKAYCE.

Before using ARIKAYCE, tell your healthcare provider about all medical conditions, including if you:

  • have asthma, COPD, shortness of breath, or wheezing (bronchospasm)
  • have been told you have poor lung function
  • have hearing problems, such as ringing in your ears or hearing loss
  • have dizziness or a sense of the room spinning
  • have kidney problems
  • have neuromuscular disease, such as myasthenia gravis
  • are pregnant or plan to become pregnant. It is not known if ARIKAYCE can harm your unborn baby. ARIKAYCE is in a class of medicines that may be connected with complete deafness in babies at birth. The deafness affects both ears and cannot be changed
  • are breastfeeding or plan to breastfeed. It is not known if the medicine in ARIKAYCE passes into your breast milk and if it can harm your baby. Talk to your healthcare provider about the best way to feed your baby during treatment with ARIKAYCE

Tell your healthcare provider about all the medicines you take, including prescription medicines and over-the-counter medicines, vitamins, and herbal supplements.

ARIKAYCE may cause serious side effects, including:

  • hearing loss or ringing in the ears (ototoxicity). Ototoxicity is a serious and common side effect of ARIKAYCE. Tell your healthcare provider right away if you have hearing loss or you hear noises in your ears, such as ringing or hissing. Tell your healthcare provider if you start having problems with balance or dizziness (vertigo)
  • worsening kidney problems (nephrotoxicity). ARIKAYCE is in a class of medicines which may cause worsening kidney problems. Your healthcare provider may do a blood test to check how well your kidneys are working during your treatment with ARIKAYCE
  • worsening muscle weakness (neuromuscular blockade). ARIKAYCE is in a class of medicines which can cause muscle weakness to get worse in people who already have problems with muscle weakness (myasthenia gravis)

The most common side effects of ARIKAYCE include: changes in voice and hoarseness (dysphonia), sore throat, diarrhea, muscle pain, nausea, tiredness (fatigue), fever, vomiting, headache, decreased weight, increased sputum, rash, chest discomfort, or cough during or after a dose of ARIKAYCE, especially in the first month after starting treatment.

These are not all of the possible side effects of ARIKAYCE. Call your doctor or pharmacist for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088 or

Please see full Prescribing Information at

Judy’s advice for people with MAC lung disease is to not give up, even if multidrug treatment hasn’t worked. “With ARIKAYCE, in combination with the other things that a person can do in working with their physicians, there’s an opportunity to get results, positive results. For me, personally, I feel like I have more control, and I’m not looking back.”

For important questions to ask your doctor about ARIKAYCE, and tips on how to make the most of your next appointment, see here for a discussion guide. You can also find the study results and more information at

Sponsored by Insmed Incorporated. Judy is a real patient who was compensated for her time for sharing her story.

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