Version May 2024
INTRODUCTION — This topic was written by an individual patient diagnosed with lymphangioleiomyomatosis (LAM). It is intended to offer clinicians insight into the experience of a single individual from that individual's point of view. This description of a particular patient's experience is not intended to be comprehensive or to provide recommendations regarding diagnosis, treatment, and/or medication information. It is not intended to be medical advice or to be a substitute for the medical advice, diagnosis, or treatment of a health care provider based on the health care provider's examination and assessment of a patient's specific and unique circumstances.
For related clinical topics, please see:
●(See "Sporadic lymphangioleiomyomatosis: Epidemiology and pathogenesis".)
●(See "Sporadic lymphangioleiomyomatosis: Clinical presentation and diagnostic evaluation".)
●(See "Sporadic lymphangioleiomyomatosis: Treatment and prognosis".)
●(See "Tuberous sclerosis complex associated lymphangioleiomyomatosis in adults".)
●(See "Diagnostic approach to the adult with cystic lung disease".)
●(See "Clinical presentation and diagnosis of pneumothorax".)
●(See "Pneumothorax: Definitive management and prevention of recurrence".)
●(See "Pneumothorax in adults: Epidemiology and etiology".)
●(See "Pneumothorax and air travel".)
●(See "Thoracostomy tubes and catheters: Indications and tube selection in adults and children".)
●(See "Thoracostomy tubes and catheters: Placement techniques and complications".)
●(See "Thoracostomy tubes and catheters: Management and removal".)
●(See "Lung transplantation: An overview".)
●(See "Causes and epidemiology of subacute and chronic cough in adults".)
●(See "Evaluation and treatment of subacute and chronic cough in adults".)
●(See "Approach to the patient with dyspnea".)
UpToDate also offers patient education materials. "The Basics" topics are short overviews written in plain language, at the 5th to 6th grade reading level, that answer the main questions a patient is likely to have about their condition. You can share this content directly with your patients:
●(See "Patient education: Lymphangioleiomyomatosis (The Basics)".)
BACKGROUND
Signs and symptoms — I had minimal symptoms until a few months before my first pneumothorax (ie, lung collapse) from LAM on April 12, 2020.
During those months, I had very slight shortness of breath, more difficulty doing the exercises I was used to doing (ie, running), and more difficulty working as a nurse. Eventually, I was breathless to the point where I struggled to speak with patients if I had been walking around the unit. I then began having a persistent cough. My symptoms worsened in the spring of 2020, so I thought my cough was due to my seasonal allergies. The cough did not go away, even after taking medication (cough drops, cough syrup, allergy medications, etc). Finally, my symptoms progressed to the point where I began having blood-tinged sputum, consistent coughing at night, and difficulty sleeping, all of which prompted me to seek medical care.
Seeking medical care — I originally sought medical care with a local physician when I began coughing up blood-tinged sputum. I considered my symptoms as just a part of being somewhat out of shape or my seasonal allergies but became concerned when I saw the blood-tinged sputum. Unfortunately, I did not follow through with this appointment because of coronavirus disease 2019 (COVID-19).
I was lucky enough to be working at the hospital the day of my first pneumothorax. I felt short of breath and tried to focus on my breathing on my way to work that morning, thinking I was just anxious about working on COVID units. Once I got to work and started my shift, I had a bout of nonstop, nonproductive coughing, and immediately after, my shortness of breath became so severe that I could not walk a few feet without stopping to try and catch my breath. At this point, my plan was just to go home and rest. However, a coworker checked my oxygen saturation, which was low and in the 70 to 80 percent range (normal is over 90 percent). I felt like I was gasping for air and started to worry that I had potentially contracted COVID-19, but with the sudden onset of my symptoms, I felt this was unlikely. I was wheeled down to the emergency department (ED) because my shortness of breath became so severe that I found it too difficult to walk or talk. I could not catch my breath enough at this point to even undress and get into the hospital gown or onto the stretcher without assistance.
Initial health care provider — The first provider I saw for what eventually turned out to be LAM was an ED physician.
Because my first pneumothorax was at the very beginning of the COVID-19 pandemic and I had been working closely with COVID-19 patients, the original assumption was that I had contracted the virus. The ED physician took a chest x-ray (ie, chest radiograph) along with a rapid COVID-19 swab and told me I had a very large pneumothorax with around 80 percent of my right lung collapsed. He also stated that my radiograph looked "different" (not like COVID-19), but again, the assumption was that I had the virus since I was 27 years old and otherwise completely healthy.
The diagnosis at this point was a spontaneous pneumothorax, so a chest tube was inserted into my right anterior chest cavity. My lung inflated almost completely, my oxygen levels immediately improved, and I was moved to a floor for monitoring. The plan was to give my lung more time to completely reinflate and then send me home. It was deemed that I just had a spontaneous pneumothorax. My COVID-19 test came back negative.
The process of having a chest tube inserted was excruciating. It was a very unnatural feeling, and I felt as though I had no control. I was given numbing medication and a sedative for the procedure but was still very much awake and aware of what was happening. Not only was I afraid, but I could still feel when the incision was made despite the numbing medicine and sedative. The worst part was the actual tube itself being inserted into my chest wall. There was a lot of pressure and intense burning and sharp, stabbing pain. This was relieved once the tube was inserted, and I was given narcotic pain medication. The relief I felt once I could breathe again after the chest tube was inserted made me not as worried about the pain I felt during the procedure because I knew it had to be done.
I had 12 chest tubes over the course of 2 years. Through the process of getting multiple chest tubes, I became frustrated with the fact that I had to repeatedly ask for sedation during the insertion process. Although sedation is unusual for the insertion of chest tubes, I found it to be so painful and such an unpleasant, traumatic procedure that my cardiothoracic surgeon and I advocated for the use of conscious sedation with ketamine and propofol (provided by anesthesiologists) for the procedure, provided the pneumothorax was not life-threatening. The possibility of not being given sedation for the insertion of a new chest tube was (and still is) a source of significant anxiety for me.
Due to the safety protocols that were in place during COVID-19, patients were not allowed to have any visitors, so my first time spent in the hospital was spent alone. Fortunately, my husband was able to send clothes, a phone charger, and other necessary items with my coworkers. Otherwise, I would have had no form of communication with my family. It was so important to be connected to my family and friends at this time. The nurses were extremely busy caring for COVID-19 patients, so I was left mostly alone in the hospital room. I was always terrified if my call light was not within reach because if something happened, my phone was the only way I could reach anyone. This also made me nervous to take any form of narcotic pain medication because I felt I could not advocate for myself while on medications. However, I was lucky to have coworkers working at this hospital because of COVID-19, so I was able to have them visit me and make sure I was okay.
Initial management of my condition — I had multiple providers manage my condition while I was in the hospital, but ultimately when they were attempting to diagnose me, a local pulmonologist saw me and directed my care.
Initial care in the local hospital — I was placed on a unit for patients with COVID until a coworker of mine spoke with the house supervisor to have me placed on a COVID-free floor for my safety. This happened after it was determined that I did not have COVID but an underlying condition causing my lungs to collapse.
My chest tube was connected to suction and would not stay inflated without it. One night, the suction had been turned off and my oxygen levels started to drop again. They called a "rapid response" because of my shortness of breath and low blood pressure. The ED doctor came to my room ready to insert a new chest tube at the bedside. I just kept pointing at the suction canister on the wall of my hospital room, and the ED doctor realized it had been turned off. Upon turning the suction back on, my lung inflated, and I could breathe again. It was at this point that I became very concerned about my care and nervous that things were being missed. I will admit, the suction being turned on caused a significant amount of pain as well, but it was pain I was able to get used to after a few minutes and knew was necessary.
Three to five days into my admission, my health care providers, which included a pulmonologist, decided to get a computed tomography (CT) of my chest. After this CT, I was asked if I smoked, if I had been around secondhand smoke often, or if I had been told I had a lung disease in the past. I also did not have any family history of lung disease. I answered no to all, and this is when my pulmonologist said, "It is extremely rare, but I have a hunch this could be LAM." He also told me at this point that he felt more comfortable assigning my care to a younger pulmonologist who would be around longer than he would since my diagnosis was likely to be something I would have for my entire life. Many doctors since have either not felt comfortable managing my care or have managed my care in a way that is not best for me, resulting in me needing to shop around for a pulmonologist who is willing to work closely with my LAM specialist. I have had some pulmonologists still try to diagnose me with asthma, chronic obstructive pulmonary disease (COPD), and emphysema, despite my LAM diagnosis. I have recently switched back to seeing this younger pulmonologist to manage my care after mine moved to a different facility, and I appreciate that she trusts me to coordinate with my LAM specialist to ensure my care remains consistent and well managed.
I felt slightly dismissed and unheard, but this was mainly because of the COVID-19 pandemic and the staff being extremely busy. I felt as though many of the nurses simply wanted to give me anxiety medications, but in actuality, I was anxious because I could not breathe; I had never had problems with anxiety in the past. It took a while before the staff realized I knew my body well and recognized what was happening. It took time for them to listen to me when I would tell them I thought I was having another lung collapse. I have since been able to completely wean off of the anxiety medications that were given to me in the hospital and have found other coping mechanisms to deal with the anxiety that can come with being diagnosed with LAM, although anxiety surrounding my health continues to be a factor.
At this point, my chest tube on the right side was not functioning properly, so I had a bedside procedure to insert a new pigtail catheter into my right side. This procedure was done on the regular nursing floor, so again, I had this tube inserted with no sedation.
A few nights later, I started to feel short of breath again and felt a strange "bubbling" or "folding" sensation on the left side of my chest. I requested multiple times to have a chest radiograph that night. The nurses told me I was just anxious and gave me anxiety medications as well as medications to help me sleep through the night. Eventually, after asking multiple times, I had my chest radiograph. At first, they told me everything looked normal. A few hours later, the provider came back into my room and said, "You were right, and you know your body. You have a very small pneumothorax on the left side that is nearly undetectable on the radiograph." I was told they would increase my oxygen flow and monitor me overnight, but at this point, I did not need another chest tube. By the next morning, I was so short of breath that when my pulmonologist came to see me, she ordered a STAT chest radiograph. The pneumothorax on my left side had worsened substantially overnight. I was rushed to the intensive care unit (ICU), which was COVID ICU at the time, where they inserted a chest tube (under conscious sedation) for my left lung, resulting in bilateral chest tubes.
I was fortunate that because of COVID-19, the certified registered nurse anesthetists (CRNAs) I normally work with were in this ICU, and they were able to sedate me for the insertion of the new chest tube. The general surgeon who inserted my chest tube was also someone I had worked with in the past, so I felt comfortable and safe with this procedure being done with conscious sedation. Being sedated for the procedure this time made all the difference for both me and the medical team because I was not experiencing the excruciating pain that this procedure can cause. The team was able to insert the chest tube quickly and efficiently because they did not have to worry about my level of pain during the process.
I felt as though the night before, the staff had been treating only the numbers and not the patient. I had complained multiple times that I felt extremely short of breath. Because my oxygen levels were in the 90s on oxygen, the concern for a collapse was missed by my team. Ultimately, my shortness of breath was because my lung was actively collapsing, not because I was anxious. Although seeing my oxygen levels at higher numbers often gives me peace of mind, I now know that just because my pulse oximeter says a higher number does not necessarily mean that my lung is not collapsed.
Transfer to another hospital — I was then transferred by ambulance to a larger, better-equipped hospital in New Orleans, where I met my cardiothoracic surgeon for the first time. At this point, I was extremely weak and had two chest tubes, one on each side. I will never forget the relief I felt when he walked into my room and said he could help me. It was the first time I felt reassured since the ED on April 12.
We were able to do a clamp trial on my right chest tube and remove it. My left lung, however, had more trouble healing during this hospital stay. My surgeon ended up placing a peripherally inserted central catheter (PICC) line in my right arm, removing around 120 mL of blood, and instilling it into my left-sided chest tube (also known as a blood patch pleurodesis). The process of having the PICC tube inserted was uncomfortable, but the actual blood patch pleurodesis was not overly painful. Once the blood was instilled, I was asked to roll around to allow it to coat my lungs. After this procedure, I did cough up some old blood from the procedure for a few days, which was alarming for me, but not concerning to my care team. I was hospitalized for around two weeks for my lung collapses. I then went home with the left chest tube and had it pulled about a week later in the clinic. I remember being afraid of the chest tube being pulled as it made me feel like my lung was protected, despite how uncomfortable it was. The process of the chest tube being pulled out was quick and was more of a strange sensation than a painful one. I was often afraid I would inhale before the occlusive dressing was secured. I was managed by home health nurses and physical therapy to change my dressings until my family learned how to manage them as I began to build up my stamina again. When I was discharged from the hospital for the first time, I had to use a walker due to how weak I had become from not being able to walk around during my stay, but I regained my strength quickly.
Over the summer in 2020, I was hospitalized a few times for several collapses of my right lung. We learned over the next few months that I would have to do longer clamp trials than expected (ie, for two to three days) instead of just a few hours to be sure my lung would not collapse again. For example, after a fairly large chest tube was pulled and I was discharged the same day, I remember leaving the hospital and feeling that something wasn't quite right and was still fairly short of breath, but at the time my oxygen levels were okay, and I was going home on supplemental oxygen. I ended up back in the ED two to three days later with another large collapse and was transferred back to the hospital in New Orleans for additional chest tube treatment. This incident prompted my care team to do longer-than-normal clamp trials for future pneumothoraces to ensure it was safe to pull the chest tubes.
Because I had not been in contact with a LAM specialist at this point, all of my trust was in my cardiothoracic surgeon. I knew he could fix my pneumothorax, but I was not confident in anyone knowing about LAM because of how rare it is. I was introduced to a transplant specialist who had treated a few LAM patients before. It was not until I met him that I felt confident that I was not going to die in the hospital. However, because he was a transplant specialist, his views on pleurodesis were different from other doctors who treat LAM patients. Pleurodesis on my left lung was put off for a while and I think that was because of their opinion regarding LAM patients requiring transplants at some point in their lives. They wanted to create as little scarring as possible, which could potentially be problematic if I needed a transplant in the future. Later, when I transitioned to a full-time LAM specialist, I became much more confident in my health care providers since their care was more focused on treating my current problems than worrying about potential issues with a transplant that I may or may not need in the future. Looking back, I think being treated initially with pleurodesis on both lungs would have ultimately prevented my recurring hospital stays.
Transitioning home — I was transitioned home with a provisional diagnosis of possible LAM. However, my transition to home was punctuated with several visits back to the hospital in New Orleans.
I was home for about a month, working on physical therapy and trying to regain some strength. I was walking around the neighborhood and felt slightly more short of breath than I had in the days before. Once again, I tried to convince myself that I was worrying for nothing, but by that evening, my husband was rushing me to the ED, where I required yet another chest tube for a large lung collapse on my right side (the lung that did not have a blood patch). When I was rushed into the ED, they had to look up LAM since they had never encountered it before. I vividly remember the ED doctor saying "Lyme disease? That wouldn't cause your lung to collapse." My LAM specialist has since been wonderful at reassuring me that I can continue to do the things I could do before. This gave me the confidence to start exercising again, which is an improvement considering there was a time I was afraid to even cough or sneeze.
I was sent back to the same hospital in New Orleans where I stayed for quite some time. I underwent many procedures, including video-assisted thoracoscopic surgery (VATS) with mechanical pleurodesis, endobronchial valve placement, and three to four rounds of chemical pleurodesis on my right lung, which wouldn't fully inflate. I was in and out of the hospital at this time. My surgeon recognized that the longer I spent in the hospital, the more my morale decreased, and this negatively impacted my healing, so we tried treatment as an outpatient. I went home with a persistent air leak and a chest tube. My surgeon and team felt confident that I could care for myself and manage my chest tube on my own. I considered the chest tube my security blanket – I knew that if the tube was in place, my lung could stay inflated. I remember having an office visit where a clamp trial was done to see if the tube could be pulled, and within minutes, I had to unclamp it myself in the lobby because I couldn't breathe – my lung wasn't ready at this point.
I had a few air leaks in the hospital as well, but this particular clamp trial caused me to have crepitus, also known as subcutaneous emphysema (air under my skin from an air leak), on the right side of my chest that traveled up my neck and down my right arm. The crepitus was slightly painful due to the location, but eventually reabsorbed over time. I came back to the hospital for an outpatient procedure and ended up staying in the hospital again for about a month. Once again, my surgeon allowed me to go home with a chest tube after a very long hospital stay. As soon as I got home this time, I had a bout of coughing and could breathe better. My surgeon's physician's assistant allowed me to check for bubbling with a saline syringe like she had showed me in the past. I instilled a tiny amount of saline into the valve opening of my chest tube and there was no bubbling, indicating that my air leak had possibly sealed. I let my surgeon know, and we waited a little while before checking on things again. A week or so later, I had an outpatient chest radiograph done and my lung was inflated. I did a three-day clamp trial on my own with the guidance of my surgeon, and he was able to remove my chest tube on August 6, 2020. After removing the tube, I was able to start pulmonary rehabilitation. The reason for so many tubes was the initial collapses I had, coupled with needing to replace or reposition tubes to try and get my lung to fully reinflate. I had at least five collapses before having pleurodesis.
In July 2021, I continued to work on my endurance. My oxygen levels were in the upper 90s at rest. During exertion, my oxygen levels declined slightly, but they bounced back quickly. I think having to retrain my lungs, especially my right lung, after multiple pleurodesis procedures has caused my breathing to be less than ideal during activity. For a few months, it was difficult for me to lie flat or on my side when sleeping; I had to prop myself up with multiple pillows. I have heard from many other LAM patients that this is a normal feeling after pleurodesis. This feeling has resolved over time, but I do often get twinges on my sides or pain in my shoulder blade and chest that go away after a few seconds to minutes.
On July 25, 2021, my left lung collapsed while riding my bike on vacation. This was the lung that had only had the blood patch procedure done. My husband took me to a local ED in Florida, where it was found that my left lung had collapsed around 40 percent. Interventional radiology was not available at this facility, but with the extent that my lung had collapsed, the ED physician felt confident that she could place my chest tube without disrupting any previous scar tissue. I was given conscious sedation, a chest tube was placed, my lung reinflated, and I spent three days in this hospital before being sent home with a chest tube. The local pulmonologist at this facility was familiar with LAM. When I told him this was my 11th chest tube, he felt confident I could manage it at home. Because this happened at the height of the Delta variant for COVID-19, we scheduled my surgery at the same hospital the following Friday, as an outpatient, but we were worried that it would not be approved due to the pandemic at the time. I had both mechanical and chemical pleurodesis, the chest tube was replaced with a larger one (my 12th tube), and after just a few days of being hospitalized, I went home chest tube free with a completely inflated lung.
In June of 2022, I was diagnosed with COVID-19 after avoiding it for two years. I had body aches, low-grade fever, chills, and some congestion, but my oxygen levels remained in the upper 90s without the use of oxygen. I also had some slight loss of taste and smell. I currently have three doses of the Pfizer vaccine, all administered before I contracted COVID-19. I took nirmatrelvir-ritonavir and had to stop my sirolimus while taking it. I had trouble getting nirmatrelvir-ritonavir prescribed to me because it is contraindicated with sirolimus. This required my local pulmonologist to speak with my LAM specialist to get approval for me to take the nirmatrelvir-ritonavir. Nirmatrelvir-ritonavir led to other symptoms including dysgeusia, dizziness, nausea, and fatigue. My symptoms resolved after about 5 to 7 days. I have had no additional problems related to COVID-19 and have since resumed taking my sirolimus.
As of July 2022, I felt nearly back to normal, but I am still building up my endurance as I do get breathless with exertion at times. I have worked on exercising more and more, and I am working in person again at a capacity that I can keep up with while studying to become a nurse practitioner.
Dealing with flying and altitude — To get to the LAM conference, I had to fly for the first time since my diagnosis. I brought my portable oxygen concentrator (POC) with us in case I needed oxygen, and it alarmed during most of the flight (it said there was a ventilation issue, but my husband and I could not find the source of the problem). We had never flown with oxygen before, so this process was new to us. When we got to around 15,000 to 20,000 feet, my oxygen levels started to drop to the mid to high 80s, so I learned that I do require oxygen for flying. The pressure flying put on my lungs made me a little uncomfortable, but I was glad to have overcome my fear of flying after diagnosis.
In the summer of 2023, I traveled to Yellowstone and the Smoky Mountains at very high elevations. In Yellowstone, I was breathless but proud of myself for accomplishing something I didn't think I would be able to do. I had to use oxygen for hiking and for sleeping on this trip. At one point, I checked my levels, and my oxygen saturation was in the low to mid 80s. My husband also had much lower levels than normal because we live at sea level and were not adjusted to the elevations of the national park. I slept with my oxygen for the first few nights until my body could adjust a little more.
I also flew on the plane with my POC again. The POC I have been using alarms when the plane reaches a certain elevation. We did have to register the POC before our flights, but the rules of the airline we flew with were not well explained beforehand. I boarded later on a fuller flight, and my husband and I had to sit in different areas. I sat in a middle seat, which was all that was available. The flight attendants came over the intercom to ask if I was on the plane. They came to my seat and told me I had to sit in a window seat, so before we could take off, I had to find someone willing to switch with me. Although they checked on me a few times during the flight, this experience was a difficult one emotionally. I was embarrassed when I was called out about my oxygen use. The alarm of my POC likely disturbed other people on the flight. It made it hard to relax on the plane, and the fact that I needed oxygen at all to fly upset me quite a bit, but I refuse to let this stop me from traveling.
On the next flight, we checked in with my POC, and the woman at the desk informed us that she was upgrading our boarding passes and that we could board first on every flight from now on because of my oxygen. This has made flying much easier for us. They also ensured that my batteries were fully charged before taking flight. On my last flight, my batteries did get low, but it was as we were descending, so I was able to manage it. In the future, before we fly again, I plan to look at other options for POCs to hopefully alleviate some of the stress of flying with oxygen. They are expensive, and my new oxygen company does not provide POCs, so this is another challenge that we will need to deal with when the time comes.
DIAGNOSIS AND TREATMENT
Diagnostic testing and what to expect — Working as a nurse allowed me to have a better understanding of what was happening and what tests needed to be done and why. I can see where this could become problematic for LAM patients who do not have any kind of medical background. The pulmonologist had many laboratory tests performed on me to rule out different diseases (pulmonary Langerhans cell histiocytosis, Birt-Hogg-Dubé syndrome, lymphoid interstitial pneumonitis).
I had a vascular endothelial growth factor (VEGF) level drawn locally, which was "normal," but it was the wrong type of VEGF that was sent. My doctors and I did not know at this point that the laboratory test needed was a VEGF-D level that can only be done at Cincinnati Children's. Once sent, my VEGF-D level was markedly elevated at 4339 pg/mL. This is such an important biomarker for LAM that tends to get missed because of a lack of information and could be problematic if not sent to the correct place. I remember initially thinking that my level was normal, which caused me to believe at the time that I might not have LAM. I also requested to have a CT done of my abdomen and pelvis to check for any other indicators of LAM.
With a high VEGF-D level and the cysts on my lungs, along with a lymphangioleiomyoma found in my pelvic cavity on CT, it was confirmed that I have sporadic LAM. This clinical diagnosis of LAM meant that I was able to avoid having a lung biopsy. It was important for me to avoid having a lung biopsy done because this is a more invasive test and could lead to potential complications. In addition, it would have required another chest tube and more time in the hospital, as well as increased the chances of another chronic pneumothorax.
It was not easy to find this information to help me decide the best approach to my diagnosis. I was, however, able to utilize an article about diagnosing LAM to guide my testing and care. The diagram walks the reader through how to diagnose LAM and described which tests needed to be completed. While it was difficult to convince my doctors in the hospital to have the CT of my pelvis completed to check for lymphangioleiomyomas, it ultimately paid off since the presence of a lymphangioleiomyoma on the CT contributed to my doctors' ability to make a clinical diagnosis of LAM.
I was lucky enough to connect with my LAM specialist through the LAM Foundation. I now trust him with my care and get my LAM-related advice through him by email and virtually. I find it such a relief to be connected with a doctor familiar with LAM who can guide me and my local physicians with my care.
I spend a good amount of time reading articles and participating in webinars to get as much information as I can about LAM so that I can stay informed and be able to make informed decisions and advocate for myself when necessary.
Understanding test results — I understood most of the results of tests I had done. I did have to do a little bit of research to understand VEGF-D and utilized an article about diagnosing LAM to allow my physicians to collect the correct laboratory tests and imaging to confirm my diagnosis of LAM disease. If I had not had this testing, a lung biopsy may have been needed to diagnose LAM.
I now have a "LAM binder" with every article I've read regarding LAM, my own personal LAM timeline, and a table that shows my doctors and me what laboratory tests and other testing I need and how often. I have this binder because I feel that with this rare disease, each patient has to advocate for themselves. This is due to the lack of knowledge in the medical community regarding LAM. I often have to act as the mediator between doctors and specialists and really educate the providers caring for me based on what I had learned on my own and through the LAM Foundation. This can be both empowering and a little unsettling. I try to make sure I bring my LAM binder to the hospital and to appointments. I have had a few nurses really value the binder and read a little from it. Every patient has a different plan of care, but a general idea of how to handle both the emergencies and treatments associated with LAM is beneficial for patients who may not be connected to the LAM Foundation or a LAM specialist.
I have had trouble with having consistent pulmonary function tests (PFTs). Because I have had new doctors, my PFTs have been in quite a few places. My most recent PFTs were done at the same hospital I stayed at for my lung collapses, and I felt this place was very thorough and coached me very well through the tests. I had never been inside of a pressurized box for a PFT before, and I did a few tests I have never done before. I will continue to use this facility for my PFTs to remain consistent.
I had my laboratory work done recently. This consisted of a complete blood count (CBC), comprehensive metabolic panel (CMP), sirolimus trough, and a lipid panel. My sirolimus level has remained stable. My total cholesterol has increased to 211, likely from the effects of sirolimus. My hemoglobin and hematocrit have always remained slightly elevated but were more elevated with this laboratory draw. I considered that this may be due to being at higher elevations right before my laboratory work. My pulmonologist had me start aspirin 81 mg and asked that I start wearing oxygen more, and my transplant doctor put in a referral for hematology to rule out that this was being caused by anything other than LAM. After starting the aspirin, my laboratory tests were redrawn along with an iron panel per the hematologist's request. My iron studies were unremarkable, and with this second laboratory draw, my hemoglobin and hematocrit started to decrease back to my normal levels. After speaking with many other LAM patients, I realized that many of us have slightly elevated levels of red blood cells, hemoglobin, and hematocrit due to LAM. I was having adverse effects from the aspirin, including tinnitus (ringing in the ears), cramping in my legs, and an upset stomach. My laboratory tests after a few days of taking aspirin showed a spike in my anion gap and a sharp decrease in my carbon dioxide levels. Because of this, my hematologist and pulmonologist agreed I should stop the aspirin and just sleep with oxygen. I have since asked for a sleep study to know how low my oxygen gets at night, since I have not had this done before to know if I am needing nocturnal oxygen. Hematology consultation deemed that my elevated hemoglobin and hematocrit were related to LAM and LAM-related hypoxia and not a different underlying cause. She was not worried about this, and she and my LAM specialist agreed that I should just redraw the laboratory tests in three months. This experience made me worried that I was declining when in all actuality, I am still stable and feeling great.
Treatment options
●Pneumothorax treatment options – I had multiple pneumothoraces, so my options for treatment of this complication of LAM varied. I had 12 chest tubes, some put in for new collapses, and others put in because the chest tubes I already had were not functioning properly or needed to be repositioned to allow for my lung to reinflate.
I have had a blood patch pleurodesis on my left lung. This procedure was not very painful; it just felt a little strange. The worst part was the peripherally inserted central catheter (PICC) line insertion and removal, but that is likely a result of my nursing background and understanding what exactly was happening and what could go wrong. I was asked to roll around on the bed in different positions to coat my lung in the hope that my blood would patch any air leaks that I had. This was difficult to do because I was so weak at this time and struggled to get into certain positions with chest tubes in place. This procedure kept my lung from collapsing for over a year, but ultimately failed, and I needed mechanical and chemical pleurodesis on my left lung for a recurrent pneumothorax.
On my right lung, I ended up requiring chemical and mechanical pleurodesis by video-assisted thoracoscopic surgery (VATS) multiple times. I had chemical pleurodesis using doxycycline while awake, and eventually the pain was bad enough that they had to sedate me. I vividly remember feeling like the pain took my breath away and looking at the physician's assistant who immediately requested I be sedated. They had brought me down to the post anesthesia care unit (PACU) for this procedure, so the anesthesia team was able to provide sedation for the remainder of the procedure. I required a pain pump after pleurodesis. The apical portion of my right lung was very stubborn. I ended up going home with chest tubes multiple times. Over time and many collapses, they also started sedating me to insert chest tubes. I now must have my chest tubes placed by interventional radiology (if possible) due to scar tissue I may have.
Having both mechanical and chemical pleurodesis on both lungs gives me more peace of mind about my life moving forward. I no longer live in fear of if my lung will collapse. While the possibility is still there, I feel much more secure with my lungs at this point and knowing that a collapse is likely to be small and I have a well-executed plan in the event that it does happen again.
They also placed endobronchial valves to help with my persistent air leaks from my pneumothorax. This procedure is done via bronchoscopy (a camera in the lungs). On a few occasions, they could not find a good place to put the valves. After having two of the three valves removed, the last valve caused problems including a productive cough, mucus buildup, and a very odd rattling noise that made me feel that my airflow was somewhat limited and impacted. I worried about the risk of infection with it.
In April of 2022, I finally had the last endobronchial valve removed. Because my cardiothoracic surgeon had already tried a few times to remove the valve with no success due to the angle the scope would need to be in to retrieve it (described to me as a V-shaped angle), I reached out to a LAM specialist in Houston who referred me to an interventional pulmonologist. I went to have the bronchoscopy done, but the doctor was unsure he would be able to retrieve the valve. I was given propofol for sedation because the procedure would be somewhat difficult due to the angle, and after a few attempts, the valve was removed. The LAM specialist was present during the procedure just in case anything went wrong, which put my mind at ease. I was thankful for the sedation I was given, because before the procedure in the preoperative area, I was told I would not be completely asleep since it is common for flexible bronchoscopies to be done with minimal sedation. Upon waking up, I found out that there was quite a bit of mucus collection from the valve, increasing my risk for infection. This was sent for testing to make sure I did not have any infection, and the results were negative. Since having the valve removed, I have not had any rattling noises or mucus, the chronic coughing has resolved, and I feel less short of breath.
Having this procedure done was another financial strain for my husband and me. We had quite a bit of difficulty having this procedure covered by insurance. Thankfully, my surgeon was able to intervene on our behalf, and the hospital waived our balance. This was a huge financial weight lifted from us.
●LAM treatment options – Sirolimus can be used to treat LAM. Some patients need a lung transplant.
•Sirolimus – Many females with LAM do not start sirolimus until their lung function gets to a certain point. I was started on sirolimus a few months after my last chest tube was removed in 2020. This allowed time for my lungs to recover, since sirolimus can inhibit healing. Because my local pulmonologist was unfamiliar with sirolimus dosing for the treatment of LAM, my LAM specialist has been involved with my dosing and making sure my levels are in a good range. As of August 2023, I am still only on 1 mg of sirolimus a day.
I have been on sirolimus since around September of 2020 with minimal side effects or complications. I did notice an increase in mucus production and battled some acne for a few months. The acne has since cleared up and the mucus is responding to saline nebulizers as needed. I have to stop sirolimus when I have a lung collapse because of its ability to inhibit healing.
With sirolimus, I have been tracking when I need laboratory work done and notify my local pulmonologist when it is time to have laboratory studies completed again. I send these results to my LAM specialist via email to make sure I do not need to change my dose.
At this time, my lung function has improved slightly on sirolimus. My laboratory levels of sirolimus have been maintained at a good level.
•Lung transplant – Since many people think LAM means that every patient will eventually require a lung transplant, the doctors were cautious with my interventions for pneumothorax. I was told that if I had pleurodesis of any kind, it would make transplant near impossible in the future. I later found out this is not the case, although it does make lung transplant more difficult for the surgical team. I think I absolutely would have required fewer interventions if my doctors had been well-versed in the care of LAM patients. As painful as pleurodesis was, I wish that it had been the initial treatment for my lung collapses. I think I would have spent much less time in the hospital if this was the case.
I met with a transplant specialist as well who assured me that what I was reading on the internet about LAM disease was outdated. I was given information about the LAM Foundation at this time, which connected me to a network of women with LAM and LAM specialists who continue to help me manage my care.
In June of 2023, I had another PFT and six-minute walk test. I have been established with the transplant team just in case I ever need a transplant, which so far is not the case and may never be the case. I see transplant yearly out of an abundance of caution. The six-minute walk test is always a source of frustration because the sensors never pick up on my fingers, so I am never able to get an accurate reading while being active. My six-minute walk test showed that I do require oxygen with exertion. My other frustration is that the six-minute walk test was done before my PFT this time, so I was tired before my breathing tests. My PFTs were stable and my forced expiratory volume (FEV1) and forced vital capacity (FVC) improved slightly, according to my transplant doctor, but my diffusing capacity of the lungs for carbon monoxide (DLCO) dropped a small amount, which prompted my pulmonologist to order an echocardiogram to make sure I do not have pulmonary hypertension. My echo came back with a pulmonary artery (PA) pressure of 27. This was lower than my previous echo with a PA pressure of 31. The cardiologist who read my reports and my LAM specialist said I do not have pulmonary hypertension. I will have repeat PFTs in a few months. My LAM specialist reassured me that my DLCO can fluctuate and that what matters most is how I am doing clinically.
•Oxygen, diet, and menstruation – In order to get oxygen equipment for exertion and for sleep, I had to have a company bring me oxygen tanks and a concentrator. This company does not provide portable oxygen concentrators (POCs) but does provide smaller tanks for exertion. I was able to get M9 tanks that fit in a backpack and are easier to use than rolling tanks. This gives me the freedom to exercise outside of my home and continue to do the things I enjoy.
I am also now trying to make dietary changes and really watch what supplements I take. I had been taking a prenatal vitamin that includes vitamin K and other vitamins that I do not currently need. Vitamin K helps your blood to clot, and interestingly, my hemoglobin and hematocrit started to increase more when I started the prenatal vitamin. At this time, the prenatal is something I will be avoiding, and the hematologist agreed that if I do become pregnant, she can monitor my iron levels, and I can supplement with just folic acid instead of an entire prenatal vitamin. At LAMposium, many doctors discussed the importance of our diets to help our lungs. They continuously mentioned the Mediterranean diet, which I am trying to implement into my own life. I have also been considering meeting with a dietician to find more natural ways to help my lungs and my laboratory levels remain stable.
I am planning to participate in my LAM specialist's study regarding LAM and our menstrual cycles. It is believed that LAM may be fueled by estrogen. Because I notice a significant change in my breathing the week before and the week of my cycle, I hope this study provides more information and, ideally, better potential treatments for LAM.
COMPLICATIONS AND FOLLOW-UP
Potential complications — Pneumothorax has been my most significant complication. It took me a little time to learn my body, but I now can tell when something is wrong or if I am having a lung collapse.
I had respiratory failure and needed oxygen at first, but I am currently only using oxygen for sleep and exertion. I am still not sure if I desaturate during sleep, so I am hoping to have a sleep study completed to determine if I need oxygen at night. Sleeping with oxygen has been quite an adjustment over the last few weeks, and because I have not yet had a sleep study, I am unsure of how much oxygen to use at night. I did also require oxygen for a while after my last chest tube was removed. I used it all through pulmonary rehabilitation. I originally stayed on about 2 to 3 L of oxygen constantly. I would increase to 6 L for exercise, and then stay on 2 to 3 L for sleep. Now, my oxygen levels stay in the mid to upper 90s at rest and decrease during exertion. If I am active, I simply take my time and take breaks when I need to, but now I have smaller oxygen tanks that allow me to be more active outside of my home. I find I am able to do more and more each day, but I also find that on my menstrual cycle I am much more short of breath.
The lymphangioleiomyoma in my pelvis did not cause any problems. It was seen on my CT, but I otherwise would have never known it was there. My gynecologist could not feel it and was unconcerned. I was told that sirolimus would likely shrink this over time and when I followed up, it was completely gone. This was a good indication for me that sirolimus is doing what it should be doing.
Pregnancy and LAM is a factor that many younger LAM patients are forced to consider, and it can be a source of stress and worry for many of us who want to have families of our own. Many doctors say that pregnancy and LAM is contraindicated due to the increase in hormones like estrogen that can potentially progress our disease, making it very important for women on hormonal birth control to know their options. After my diagnosis, I switched to a progesterone-only birth control but have since stopped taking this as well. Since discontinuing my progesterone-only birth control, I have also noticed improvement in my breathlessness. I track my menstrual cycle using non-hormonal methods and have had success with this method.
I have not yet tried to have children (another big concern for LAM patients my age), so I do not know if this will affect my ability to become pregnant. After speaking with my LAM specialist and a local maternal fetal specialist along with my gynecologist, the conclusion was made that pregnancy with LAM is not out of the question for me and that taking my 1 mg of sirolimus is acceptable for my situation because it is such a small dose and the benefits of continuing outweigh any possible risks. The maternal fetal specialist even went as far as to say that, contrary to popular belief, I would not require an automatic cesarean delivery. Pregnancy and LAM is individual and deeply personal, requiring evaluation on a case-by-case basis. There is always a risk with pregnancy, even without LAM, so if I become pregnant, I will be closely monitored. I plan to document this experience when the time comes to hopefully help other younger LAM patients debating this. If I do become pregnant, I know I have the support of many doctors to help me through anything.
I was originally put on anxiety medications in the hospital. I still get anxious at times about the future and about my lungs potentially collapsing again, but overall, I am in a much better headspace than I was in the spring and summer of 2020. I have a plan, I have specialists and doctors that I trust, and I have a strong community of other women with LAM to talk to, and all of this has been extremely helpful.
Strategies for communication
●Self-advocacy – I have now become my greatest advocate. If my lung collapses, I have a note stored on my phone and on all of my family members' phones that has my history, my medications, and the names and phone numbers of my doctors to contact. I also utilize my phone's medical ID app to store my information as well.
There were many times in the hospital that I felt I was having problems (air leak, lung collapse, etc), and it took a while for some of the providers to intervene. Because I was alone for much of my hospital stays due to COVID-19 restrictions, I really had to advocate for myself and for my care and push for things that they may have thought were silly or unnecessary at the time. It helped once my husband was allowed to be there with me because when I was too short of breath to talk, he could tell the team what they needed to know about my lungs for me.
Having my emergency note on my phone is very helpful if I am in a situation where I can't speak for myself due to being short of breath. I also carry the LAM Foundation's emergency card in my wallet. I've learned that with LAM, there are no "silly" questions, and oftentimes, asking questions leads to new ideas (for example, my using a saline nebulizer to decrease the mucus production). I make sure I use my timeline for laboratory work and testing to remind my providers of what I need done and when I need it done so we are all always on the same page.
In addition, in the hospital, I would always make a list of questions that I had at night so when the doctors would round the following morning, I would not forget to ask anything.
●Facilitating provider communication – I need several doctors to take care of me.
I struggled to find a local pulmonologist willing to work with my LAM specialist regarding my care. I have had difficulty in the past finding a pulmonologist who could admit to not having taken care of LAM patients before. Finding someone who is willing to seek my LAM specialist's guidance was difficult. I have found a pulmonologist who allows me to voice my concerns and takes what my LAM specialist recommends into consideration. I am thankful she trusts me to communicate with my specialist and relay information to her. She listens to any concerns I may have and makes an effort to ensure I have what I need to thrive. I had to make sure my surgeon was also on the same page as my LAM specialist while in the hospital for every procedure.
I had some trouble at first getting a communication workflow that works for everyone, but ultimately if there is a question about my care, I ask my local doctors to communicate with my LAM specialist. I find it very helpful to try to have my local pulmonologist on the same page as my LAM specialist and cardiothoracic surgeon. My LAM specialist was the first to reach out to me and is quick to respond to my questions and concerns. My cardiothoracic surgeon is in New Orleans, which is about one hour away from my hometown. I am sent by ambulance there any time I go to the emergency department (ED) for a collapse because this hospital in New Orleans can provide more specialized care for me. My pulmonologist is about 40 minutes away from my home, but it is worth driving to this clinic to ensure my care remains consistent.
Explaining possible outcomes — I felt like I am always given good explanations for possible outcomes, and that my surgeons and specialists take time to really answer my questions and follow up with me if they are unsure about anything.
I have had some doctors who were unfamiliar with LAM and presented it in a very negative way, especially in the ED. I was told I would not be able to do a lot of things, or it was suggested that I not do certain things instead of discussing my options, especially for topics like pregnancy and exercise. This was the case until I met my LAM specialist. I find that being able to advocate for myself and do my own research causes my providers to take my concerns seriously and take the time to discuss my options with me. I make it a point to try to educate my providers if they are unfamiliar with LAM because so much of the information out there is outdated.
COMMUNICATION AND RESOURCES
What I wish I had been told — I wish several things:
●When I was first diagnosed, I wish the first thing I had heard was that LAM is not a death sentence.
●I wish I had been given access to resources much sooner, such as the LAM Foundation and being connected with other women with LAM disease.
●I also wish I had been told not to search LAM on the internet before talking with a LAM specialist. If a doctor with knowledge of LAM had told me at first that LAM is not a death sentence, and that the information about LAM online was incredibly outdated, I would have been less likely to search the internet for information. I was given an outdated handout saying that LAM patients have a life expectancy of 5 to 10 years. This terrified me and sent me searching for more information.
●I wish I had access to a LAM clinic in my state of Louisiana.
●I wish I had been told early on that it was okay for me to have pleurodesis. I believe this would have kept me out of the hospital.
●I wish I had been told that having LAM does not mean that I will not have a full and happy life or that I will automatically need a lung transplant, which is a common assumption.
●I wish I had more information about supplements, vitamins, or anything else I could potentially take that could ease some of my symptoms. I also wish I had more information on what not to take to ensure my laboratory tests remain normal and to make sure I do not take anything that may cause harm. I would ultimately like to meet with someone (possibly a dietician) about this.
●I wish I had more affordable options as far as portable oxygen for flying and better oxygen sensors that work during exercise so that I could titrate my oxygen appropriately during exertion.
●I wish there was more information for younger women with LAM who want to consider pregnancy. I realize this is difficult to study, but I am grateful for a team that is willing to work with me if this is something I decide to do.
Questions I wish I had been asked — I wish I had been asked more about my pain levels, especially when it comes to chest tubes and pleurodesis. I wish we had come up with a pain management plan ahead of time (we eventually did). I wish they had asked me if I had information provided to me about LAM.
Making decisions about my care — At first, I was completely lost regarding my diagnosis. My local providers and nurses knew very little about LAM, so I found I had to do a good bit of research on my own to make informed decisions about my care. That was alleviated when I was put in touch with the LAM Foundation and my LAM specialist. I now have a large binder of medical articles regarding LAM disease, its treatments, diagnosis, and potential complications. This allows me to educate myself and be my own advocate.
I required a lot of help from my family at first when I got home from the hospital. During my first two-week stay in the hospital, I was not allowed to get up from the bed or walk, in part due to having bilateral chest tubes and also because of COVID-19 restrictions at the time, so I basically had to learn to do this again with their help and with outpatient physical therapy. I was 27 years old and needed a walker for the first few days out of the hospital due to weakness. I required help with bathing because I was so weak and initially went home with a chest tube. Over time, as I got stronger, I needed less help with activities of daily living. Once my caregivers were familiar with my husband, it was very helpful to have him relay information about my care and about LAM if I was too breathless to speak. Again, I think if clinicians had been more familiar with LAM, and if there had not been COVID-19 restrictions that prevented me from walking the hallways, I would have required less help at home.
Sharing information — I use the electronic MyChart account frequently, but I also had many in-person appointments. With COVID-19, I feel as though the medical field has really embraced virtual visits and that has allowed me to easily speak face-to-face with my LAM specialist and primary care team.
I was given just one printout of information about LAM and really had to try to do my own research at first until I found the LAM Foundation. I have found the virtual education presented in "LAM in your living room" available from the LAM Foundation to be extremely beneficial, especially because it allows LAM patients to voice concerns, ask questions, and get to know each other.
In September of 2022, I had the privilege of attending LAMposium in Chicago. This is an event where LAM patients, doctors, and researchers can meet each other and learn about the latest research. Patients can discuss with doctors and researchers what is needed to improve their quality of life. This gave me an opportunity to meet many women I have only talked to over the phone or social media. It was refreshing to be surrounded by people who understand the everyday struggles of LAM on a personal level. These women have become some of my dearest friends and sources of support. It was also a great opportunity for my husband to meet other spouses of women with LAM. I was fortunate to be asked to speak about my LAM experience, and we left the conference feeling hopeful and excited for the future of LAM.
Doing things differently — Requesting time off of work and traveling are concerns for my husband and me. We have to consider insurance coverage, flight and hotel costs, and requests off of work. I often wish we had a LAM clinic here in Louisiana, or at least more doctors familiar with this disease. Because of this and because of my utmost trust in my LAM specialist, I seek all of my LAM-related advice from him and ask my local physicians to do the same. I have finally had the opportunity to fly after my LAM diagnosis and learned that I do need some oxygen for altitude.
Impact of LAM disease and treatment — LAM disease has impacted my life in so many ways. It would be easy to say that it has only impacted me negatively, but in more ways than one, having LAM disease has been a positive experience.
●My exercise capacity – Obviously, my endurance is a struggle. I went from being a regular runner to getting winded just walking around. This is frustrating when trying to do normal activities that I love, and even perform my basic job duties as a nurse. I still struggle to walk long distances without becoming breathless. I am able to bike ride at a leisurely pace, I have started doing more yoga and some Pilates and barre classes from home, and I try to walk for at least 30 minutes with my husband and my dog. I have noticed that weight loss plays a big role in my ability to breathe easily and plan to maintain a healthy weight. I now have oxygen to use during exercise. I do hope that the more my endurance improves, the less I will need to use the oxygen, but time will tell.
I use an albuterol inhaler before being active since pulmonary function tests (PFTs) have indicated I respond well to albuterol. I also use a combined fluticasone, umeclidinium, and vilanterol inhaler (brand name: Trelegy Ellipta) daily and a saline nebulizer on occasion to decrease any excess mucus.
I hope that my endurance continues to improve, and I am able to do the exercises I enjoy again.
●Family prospects – The idea that I may not be able to have children, or have them and risk progression of my disease, is a scary reality for me and other young LAM patients. Some clinicians I spoke with were empathetic about this, while others told me pregnancy was a bad idea and that I should not attempt it. My LAM specialist, gynecologist, and maternal fetal specialist have all said I do not have to rule it out at this point, and that when I am ready, they will support me and help me through the process. I plan to document as much as I can if I become pregnant to hopefully help other LAM patients my age who want to consider starting a family.
●Future therapies – The idea that there is currently no cure for LAM is daunting, along with the idea of potentially needing a lung transplant in the future. Some of the side effects of sirolimus can be frustrating, although I did adjust to them and fortunately have been able to remain on the same daily dose of 1 mg. I believe there has been much progress in the search for a cure for LAM, and I am still hopeful that we may have this cure in my lifetime.
The LAM Foundation provided a good bit of information regarding side effects of sirolimus, and I was able to speak with many other women regarding these side effects. At first, I did have a problem with some mild acne that has since resolved.
●Cosmetic impact – Body image can be an important factor as well. I have many chest tube scars and scars from surgeries, and although I am proud of the story they tell, they are also a reminder of what is slowly happening inside of my body. I am becoming more and more comfortable with my chest tube scars because they remind me of my strength and resilience during a time when I often did not know if I could push through to the next day.
●Psychological – Anxiety is another tricky emotion I have dealt with, along with the feeling of being treated differently by friends and family after all I went through. Even though I am the patient, my family and friends were also emotionally impacted by my diagnosis and first few years battling lung collapses. Although I am beyond appreciative of their care and consideration for me, I prefer to be treated as myself instead of someone who may be considered fragile because of this disease. It is hard not to consider life compartmentalized into two sections – life before LAM and life after LAM. It is difficult not to mourn the life I had before diagnosis, but after everything I have been through and witnessing the strength of so many other women and their daily battles with LAM, I think of us as extremely strong and resilient, not fragile. Although there is still a battle with health anxiety and with trying to figure out my new normal, I continue to look forward to the future and do everything in my power to slow the progression of this disease. The opportunity to connect with and help other women with LAM who are newly diagnosed has given me a different perspective on my situation. The ability to help and teach others about such a rare disease is a purpose I did not know I would appreciate as much as I do. At first, I thought having LAM was going to prevent me from doing everything I want to do in life. I now understand that the only thing that can prevent me from reaching my goals in life will be my own attitude about it. I have almost completed nurse practitioner school and hopefully, if it is safe enough, I will be able to have children of my own. I plan to continue to exercise and work on my endurance. I plan to not allow LAM to control my life or define me.
●Work-related – LAM has impacted what I am able to do as a nurse. It has slowed me down physically and trying to find accommodations as a nurse can be difficult because nursing can be physically demanding. I have gone down to per diem for work, and I now am only doing work that is not causing me to overexert myself. Going down to per diem has been a financial strain, but it allows me the time and flexibility to have appointments and reach my goals of a less physically demanding career. My first lung collapse happened one week before I was eligible for short-term disability through my job. Because of this, I went from April until October of 2020 with no paycheck. If I had not been married and my husband had not recently gotten a raise, we would have really struggled financially that year.
With all of that being said, LAM has had such a positive impact on my life as well. I have grown so much in the last few years. As a nurse, I am more compassionate and understanding and try even harder to advocate for my patients because I now know firsthand how important it can be. Knowing that your medical team really hears your concerns is a huge factor in patient outcomes that often gets forgotten. LAM has allowed me to meet so many incredible women who share this disease; I have found so much comfort and support through them. It has allowed me to raise awareness for a disease that is not well known and given me ample opportunities to do so. It has pushed me to make better lifestyle choices and to exercise more. It has made my relationships stronger with my family and friends. It has forced me to slow down and appreciate life.
Advice — My best advice for other LAM patients is to stay in touch with your specialist and make sure you have a solid idea of your care plan and find a local doctor who is willing to work with your LAM specialist. This enables you to truly advocate for yourself and understand what is going on. Being an active participant in your care is vital to ensure things go smoothly and everyone is on the same page. I would also advise against looking at outdated information.
Stay as active as you can while you can.
Make sure you have your medical information written down or have it somewhere easily accessible. One of the scariest moments for me was when I had a pneumothorax, and my emergency department (ED) doctor was not sure what LAM was. The LAM Foundation does have an emergency medical card that can be scanned to quickly access information about LAM. I have used this in the ED before and keep it in my wallet.
Don't be afraid to ask questions, even if you think they seem silly. Remember even though we have a rare disease, we are certainly not alone on this journey. Because LAM is rare, it is even more important to ask the questions that feel silly, because it may just trigger a new idea that can help other LAM patients in the future. You are not just a patient or a number.
Sign up for LAM-related clinical trials that apply to you. The LAM Foundation often has a list of current studies and trials available to LAM patients. Knowledge is power, and the more information we can provide to researchers, the more likely we are to find a cure for this disease.
Get involved with the LAM Foundation or other LAM organizations in your own country. It is because of this connection between patients, doctors, and scientists that research for LAM has made the leaps and bounds it has over the decades. Just because we are rare does not mean we cannot make a difference.
Resources — I wish I had known about the LAM Foundation much sooner. I also was fortunate enough to find the "Lammies" Facebook page where we are able to connect, talk about our experiences, and support each other. This page is a safe place for only LAM patients. It was terrifying at first knowing that I had a rare disease and not knowing anyone who could relate. I am surprised at how many women with LAM are added to this page weekly, especially since my diagnosis. I think this is because of the advances in making a diagnosis of LAM and recognizing it in practice due to increased awareness. I once read on the LAM Foundation's website that scientists believe there are possibly 250,000 women with LAM who are misdiagnosed or undiagnosed, so awareness is extremely important.
The articles that I found most helpful were the LAM Foundation's "Diagnosing LAM" page [1], their pages for patients who are newly diagnosed, and the clinical practice guidelines from the American Thoracic Society/Japanese Respiratory Society [2,3]. These guidelines would be helpful for patients who are going through the process of being diagnosed.
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