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PubMed This is a summary of 19 peer-reviewed journal articles Updated
Pulmonology

Living Well with LAM Long-Term

At a Glance

Living with LAM requires consistent long-term monitoring through pulmonary function tests (PFTs), VEGF-D blood work, and imaging. Managing the disease effectively also involves navigating pregnancy risks with specialists, participating in pulmonary rehabilitation, and addressing scanxiety.

Living with LAM long-term means becoming an expert in your own health and working closely with a specialized care team. While the diagnosis is lifelong, many women find that with consistent monitoring and proactive wellness strategies, they can lead full and active lives [1][2].

Long-Term Monitoring

Routine check-ups are the backbone of LAM management. These tests allow your doctor to see how your lungs are functioning and if your treatment needs to be adjusted.

  • Pulmonary Function Tests (PFTs): You will likely have PFTs every 3 to 6 months initially, then perhaps annually if you are stable [3]. These tests measure FEV1 (how much air you can exhale) and DLCO (how well your lungs move oxygen into your blood) [4].
  • VEGF-D Monitoring: Periodic blood tests for VEGF-D help track the biological activity of your LAM cells and how well medications like sirolimus are working [5][6].
  • Imaging: You may have occasional High-Resolution CT (HRCT) scans to monitor the size and number of lung cysts, though these are done less frequently than PFTs once a baseline is established [7].

Navigating Pregnancy

Pregnancy with LAM is a complex topic and requires early and open discussion with both a pulmonologist and a maternal-fetal medicine specialist [8].

  • The Risks: Because estrogen acts as the primary “fuel” for LAM cell growth, the massive, natural surge of estrogen that occurs during pregnancy can significantly accelerate the disease. This is why pregnancy can cause a temporary or permanent decline in lung function for some women, and greatly increases the risk of a collapsed lung (pneumothorax) [9][10].
  • Kidney Health: Hormonal changes in pregnancy can also cause kidney tumors (angiomyolipomas) to grow or even bleed. Doctors may recommend a procedure called embolization to shrink these tumors before you conceive [11].
  • Medication: Sirolimus is generally not recommended during pregnancy due to potential risks to the developing baby [12][10]. Your team will help you weigh the risks of stopping medication against the risks of continuing it.

Quality of Life and Physical Activity

One of the most powerful tools for managing LAM is physical activity.

  • Pulmonary Rehabilitation: These structured exercise and education programs are highly effective for LAM patients [13]. They help improve your muscle strength, increase your exercise capacity, and—most importantly—reduce the sensation of breathlessness [13][14].
  • Aerobic Exercise: Regular, moderate exercise like walking, swimming, or cycling is generally encouraged, as it helps your body use oxygen more efficiently [13].

Managing “Scanxiety”

It is completely normal to feel anxious before a scheduled scan or while waiting for results—a feeling many in the chronic illness community call scanxiety [15].

  • Information is Power: Asking for a clear explanation of why a scan is being done and exactly when you will receive the results can help lower stress [16][17].
  • Support Systems: Sharing your feelings with a partner, friend, or a LAM support group can provide emotional relief during the waiting period [16].
  • Holistic Health: Remember that your quality of life is influenced by more than just your “lung numbers” [2]. Addressing symptoms of anxiety or depression with a mental health professional is just as important as treating the physical symptoms of LAM [18].
Aspect of Care Frequency/Goal Why it Matters
PFTs Every 3–12 months [3] Detects small changes in lung function early [4]
VEGF-D Periodic blood tests [5] Monitors disease activity and drug response [6]
Pulmonary Rehab As needed/Regular exercise Reduces breathlessness and boosts energy [13]
Hormone Review Annual or pre-pregnancy Minimizes risks from estrogen and manages AMLs [19]

Common questions in this guide

How often do I need PFTs and VEGF-D blood tests for LAM?
You will likely need pulmonary function tests (PFTs) every 3 to 6 months initially, and then annually once your condition is stable. VEGF-D blood tests are also done periodically to monitor disease activity and how well medications are working.
Is it safe to get pregnant if I have LAM?
Pregnancy with LAM carries significant risks because estrogen acts as a fuel that accelerates the growth of LAM cells. The hormonal surge can lead to a decline in lung function and increase the risk of a collapsed lung, so careful planning with specialists is essential.
Can pulmonary rehabilitation help with my LAM symptoms?
Yes, pulmonary rehabilitation is highly effective for patients with LAM. These structured exercise and education programs can improve your muscle strength, increase your exercise capacity, and significantly reduce the feeling of breathlessness.
What is scanxiety and how can I manage it?
Scanxiety is the normal feeling of stress before a medical scan or while waiting for results. You can manage it by asking your doctor exactly when to expect results, sharing your feelings with a support system, and prioritizing your overall mental health.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.How frequently do I need PFTs and VEGF-D blood tests to monitor my disease?
  2. 2.What are the specific risks for me if I decide to become pregnant?
  3. 3.Is there a pulmonary rehabilitation program you recommend for LAM patients?
  4. 4.How soon can I expect my scan results to be available to help manage 'scanxiety'?
  5. 5.What level of exercise is safe for me, and should I avoid certain high-impact activities?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

References (19)
  1. 1

    Determinants of Progression and Mortality in Lymphangioleiomyomatosis.

    Xu W, Yang C, Cheng C, et al.

    Chest 2023; (164(1)):137-148 doi:10.1016/j.chest.2023.02.026.

    PMID: 36801466
  2. 2

    Clinical and Functional Outcomes Associated with Quality of Life in Patients with Lymphangioleiomyomatosis: A Cross-Sectional Study.

    Queiroz DS, da Silva CCBM, Oliveira MR, et al.

    Lung 2024; (202(6)):757-765 doi:10.1007/s00408-024-00751-w.

    PMID: 39402376
  3. 3

    Long-term clinical course and progression of lymphangioleiomyomatosis in a single lung transplant referral centre in Korea.

    Chang S, Choi JS, Leem AY, et al.

    Scientific reports 2022; (12(1)):8260 doi:10.1038/s41598-022-12314-1.

    PMID: 35585116
  4. 4

    Predicting Individualized Lung Disease Progression in Treatment-Naive Patients With Lymphangioleiomyomatosis.

    Palipana AK, Gecili E, Song S, et al.

    Chest 2023; (163(6)):1458-1470 doi:10.1016/j.chest.2022.12.027.

    PMID: 36610667
  5. 5

    Methacholine reactivity in lymphangioleiomyomatosis is inversely related to FEV1 and VEGF-D.

    Cassandro R, Elia D, Caminati A, et al.

    The European respiratory journal 2021; (57(6)) doi:10.1183/13993003.04270-2020.

    PMID: 33653809
  6. 6

    Analysis of the MILES cohort reveals determinants of disease progression and treatment response in lymphangioleiomyomatosis.

    Gupta N, Lee HS, Young LR, et al.

    The European respiratory journal 2019; (53(4)) doi:10.1183/13993003.02066-2018.

    PMID: 30846465
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    Effects of sirolimus in lymphangioleiomyomatosis patients on lung cysts and pulmonary function: long-term follow-up observational study.

    Kim C, Do KH, Cha J, et al.

    European radiology 2020; (30(2)):735-743 doi:10.1007/s00330-019-06412-4.

    PMID: 31471750
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    Pregnancy in lymphangioleiomyomatosis: clinical and lung function outcomes in two national cohorts.

    Taveira-DaSilva AM, Johnson SR, Julien-Williams P, et al.

    Thorax 2020; (75(10)):904-907 doi:10.1136/thoraxjnl-2020-214987.

    PMID: 32788260
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    Complications of lymphangioleiomyomatosis in pregnancy: a case report and review of the literature.

    Wang-Koehler E, Kern-Goldberger AR, Srinivas SK

    AJOG global reports 2024; (4(1)):100309 doi:10.1016/j.xagr.2024.100309.

    PMID: 38327672
  10. 10

    Pregnancy after the diagnosis of lymphangioleiomyomatosis (LAM).

    Shen L, Xu W, Gao J, et al.

    Orphanet journal of rare diseases 2021; (16(1)):133 doi:10.1186/s13023-021-01776-7.

    PMID: 33731151
  11. 11

    Prophylactic renal artery embolization before pregnancy in patients with lymphangioleiomyomatosis and renal angiomyolipoma.

    Toei H, Zhang X, Seyama K, et al.

    The Journal of international medical research 2022; (50(9)):3000605221123897 doi:10.1177/03000605221123897.

    PMID: 36124884
  12. 12

    Lymphangioleiomyomatosis and Pregnancy-Do We Have All the Answers for a Woman Who Desires to Conceive?-Literature Review.

    Constantin AA, Gaburici AD, Malaescu AN, et al.

    Cancers 2025; (17(2)) doi:10.3390/cancers17020323.

    PMID: 39858105
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    Pulmonary rehabilitation in lymphangioleiomyomatosis: a controlled clinical trial.

    Araujo MS, Baldi BG, Freitas CS, et al.

    The European respiratory journal 2016; (47(5)):1452-60 doi:10.1183/13993003.01683-2015.

    PMID: 26917604
  14. 14

    Benefits of different characteristics of pulmonary rehabilitation programs in patients with interstitial lung disease: a systematic review and meta-analysis.

    Chen Q, Qiu Y, Xu M, Dong L

    Annals of medicine 2025; (57(1)):2566868 doi:10.1080/07853890.2025.2566868.

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    Scanxiety among Adults with Cancer: A Scoping Review to Guide Research and Interventions.

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    Cancers 2023; (15(5)) doi:10.3390/cancers15051381.

    PMID: 36900174
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    Prevalence, severity, and modifiable predictors of scanxiety in patients undergoing routine oncologic imaging: a prospective longitudinal study.

    Shah MS, Memon JA, Malik U, et al.

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    PMID: 41077027
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    Survey of maternal anxiety and perceptions towards foetal MRI and pre-scan education.

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    Journal of medical radiation sciences 2024; (71(1)):10-20 doi:10.1002/jmrs.725.

    PMID: 37724764
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    [Anxiety about Health of Patients Suffering from Epilepsy].

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    Psychotherapie, Psychosomatik, medizinische Psychologie 2022; (72(6)):243-249 doi:10.1055/a-1686-9789.

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    Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guidelines: Lymphangioleiomyomatosis Diagnosis and Management.

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    PMID: 27628078

This page provides educational information about long-term management of LAM. Always consult your pulmonologist or maternal-fetal medicine specialist before changing treatments or planning a pregnancy.

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