Lymphangioleiomyomatosis (LAM) in Grand Rapids, Michigan

Lymphangioleiomyomatosis (LAM) is a rare, progressive lung disease that almost always affects women, most often during their reproductive years. The average age at diagnosis is around 34. In LAM, abnormal smooth muscle–like cells grow in the lungs and form cysts, which gradually damage lung tissue and lead to breathing problems.

LAM can also affect other organs, including the kidneys, uterus, and lymphatic system.

In Grand Rapids and West Michigan, women with unexplained shortness of breath—especially if asthma treatments are not helping—should consider evaluation with a lung specialist (pulmonologist) at major centers such as Spectrum Health, Trinity Health Grand Rapids, Metro Health, or Mercy Health.

What Causes LAM?

The exact cause of LAM is unknown. There are two main forms:

  • Sporadic LAM (sLAM)

    • Occurs in women who do not have any other related genetic condition
    • Estimated to affect about 1 woman in a million

  • LAM associated with tuberous sclerosis complex (TSC)

    • Occurs in some women (and rarely men) who have tuberous sclerosis complex, a rare genetic disorder that causes benign (non‑cancerous) tumors in the brain and other organs

There is currently no known cure for LAM.

How LAM Affects the Lungs

LAM mainly targets the lungs. The smooth muscle–like cells in the lungs begin to overgrow and form:

  • Clumps of abnormal cells
  • Cysts (fluid- or air-filled sacs) around:
    • Airways
    • Blood vessels
    • Lymph vessels

As LAM progresses, these growths damage healthy lung tissue and interfere with normal breathing. In early stages, symptoms can look very similar to more common conditions such as asthma, chronic bronchitis, or emphysema—conditions frequently seen in West Michigan, especially among smokers and older adults.

Symptoms of LAM

Common Lung Symptoms

LAM symptoms often worsen gradually over time. They may include:

  • Shortness of breath (especially with activity, climbing stairs, or walking hills—important in a city with varied terrain like Grand Rapids)
  • Breathlessness during physical exertion or exercise
  • Chest pain or chest tightness
  • Chronic cough
  • Coughing up thick, white sputum
  • Coughing up blood (hemoptysis)

Because LAM is rare and Michigan winters can worsen breathing issues in general, symptoms are sometimes mistaken for:

  • Asthma (which occurs in about 30% of women with LAM)
  • Chronic bronchitis
  • Chronic obstructive pulmonary disease (COPD)
  • Recurrent respiratory infections

If symptoms persist despite standard asthma or bronchitis treatments, a specialist evaluation is important.

Other Parts of the Body That May Be Affected

LAM can also involve other organs:

  • Kidneys

    • Benign tumors called angiomyolipomas (AMLs)
    • About 1 in 3 women with LAM develop these kidney tumors
    • They may bleed and cause:
      • Flank or back pain
      • Blood in the urine

  • Abdomen and pelvis

    • Cysts or tissue masses called lymphangioleiomyomas
    • Often develop behind the lining of the abdomen (peritoneum)
    • May cause abdominal discomfort, fullness, or swelling

  • Lymph nodes

    • Enlargement of lymph nodes, usually harmless but may be found on imaging tests

Complications of LAM

LAM can lead to serious complications that may require urgent care at Grand Rapids emergency departments (for example, at Spectrum Health Butterworth Hospital or Trinity Health Grand Rapids).

Collapsed Lung (Pneumothorax)

A pneumothorax occurs when a lung collapses, often because a cyst in the lung bursts and leaks air into the chest cavity.

  • Symptoms:
    • Sudden sharp chest pain
    • Sudden worsening shortness of breath
  • Mild, partial collapses may reinflate on their own
  • More severe cases may require:
    • Hospitalization
    • Chest tube placement
    • Surgery to repair the leak and prevent recurrence

Pneumothorax is relatively common in people with LAM and may happen repeatedly.

Pleural Effusion and Chylothorax

LAM can cause pleural effusion—a buildup of fluid between the lungs and the chest wall (pleura).

  • In some women, the fluid is a milky lymphatic fluid called chyle, leading to chylothorax
  • About 1 in 3 women with LAM have abnormal leakage of lymphatic fluid into the chest cavity
  • This can cause:
    • Shortness of breath
    • Chest heaviness
    • Cough

Bleeding Kidney Tumors

Kidney angiomyolipomas (AMLs) may bleed, causing:

  • Sudden or persistent back or flank pain
  • Blood in the urine
  • Drop in blood pressure in severe cases

These situations may require urgent evaluation in a hospital with imaging and urology support, such as Spectrum Health or Metro Health in Grand Rapids.

LAM develops in about 40% of women with tuberous sclerosis complex (TSC) and occasionally in men with TSC.

Tuberous sclerosis complex is a rare genetic disorder that causes benign, root-like growths (hamartomas) in:

  • Brain
  • Kidneys
  • Heart
  • Liver
  • Lungs
  • Skin

These growths begin forming before birth and can interfere with normal organ function, especially in the brain (leading to seizures, developmental delays, or behavior changes).

Genetics and LAM

  • TSC is caused by mutations in one of two tumor suppression genes:
    • TSC1
    • TSC2
  • In most cases, the mutation happens spontaneously during development
  • In about 20% of cases, the mutated gene is inherited

LAM is a separate condition from TSC and is not thought to be inherited in the same way, but:

  • The strong association with TSC suggests that genetics plays an important role
  • Doctors believe both TSC and LAM are caused by mutations in the TSC1 or TSC2 genes

The exact reason these mutations occur is still unknown.

Why LAM Almost Always Affects Women

LAM almost exclusively affects women, typically:

  • Before menopause
  • With disease progression often slowing after menopause

Pregnancy is known to speed the progression of LAM, and:

  • The female hormone estrogen is believed to be an important risk factor
  • LAM cells appear to be sensitive to estrogen

Estrogen and Treatment Decisions

Based on current understanding, women with LAM are often advised to avoid medications containing estrogen, including:

  • Combined oral contraceptive pills
  • Estrogen-containing hormone replacement therapy (HRT)
  • Certain fertility treatments

Any decisions about birth control, hormone therapy, or pregnancy should be made in close consultation with:

  • A pulmonologist
  • An obstetrician-gynecologist (OB-GYN)
  • Often an endocrinologist

In Grand Rapids, these specialists are available through major health systems like Spectrum Health, Trinity Health, Metro Health, and Mercy Health.

How LAM Is Diagnosed in Grand Rapids

Because LAM is rare and mimics more common lung diseases, diagnosis can be challenging. Many women are initially told they have asthma, COPD, or recurrent bronchitis.

Medical Evaluation

Diagnosis typically involves:

  • Detailed medical history

    • Symptoms and how long they have been present
    • Smoking history
    • Past lung problems or pneumothorax
    • Family history of TSC or similar conditions

  • Physical examination

    • Listening to the lungs
    • Checking oxygen levels and overall health

Lung Function and Oxygen Tests

  • Lung function tests (pulmonary function tests)

    • Measure how much air the lungs can inhale and exhale
    • Assess how well oxygen moves into the blood

  • Pulse oximetry

    • A simple, non-invasive test using a finger clip
    • Checks oxygen levels in the blood

Imaging Studies

  • Chest X-ray

    • May show changes in lung structure or a collapsed lung

  • High-resolution CT scan of the chest

    • Key test for LAM
    • Shows multiple thin-walled lung cysts typical of the disease

  • CT scan of the abdomen

    • Looks for kidney angiomyolipomas and lymphangioleiomyomas

  • Ultrasound of the kidneys

    • Helps detect and monitor kidney tumors

These imaging tests are widely available at Grand Rapids hospitals and advanced imaging centers.

Lung Biopsy

In some cases, a lung biopsy is needed to confirm the diagnosis:

  • Performed under local or general anesthesia
  • A small sample of lung tissue is removed and examined under a microscope
  • Can be done via:
    • Bronchoscopy (using a camera passed into the airways)
    • Surgical biopsy (video-assisted thoracoscopic surgery, or VATS)

Not all patients require a biopsy if imaging and clinical features are very typical of LAM.

How LAM Progresses

LAM is a progressive disease, meaning it tends to get worse over time. However:

  • The rate of progression varies greatly from person to person
  • Some women have slow disease progression and remain active for many years
  • Others may experience more rapid decline in lung function

There is currently no treatment that has been definitively proven to cure LAM or completely stop cyst formation, but several therapies can:

  • Ease symptoms
  • Slow disease progression in some cases
  • Reduce the risk of complications
  • Improve quality of life

Treatment of LAM in Grand Rapids

Goals of Treatment

Treatment aims to:

  • Control symptoms (especially shortness of breath and cough)
  • Prevent or manage complications (such as pneumothorax and chylothorax)
  • Preserve lung function as long as possible
  • Support overall health and daily functioning

Medications and Supportive Care

Common treatment options include:

  • Bronchodilators

    • Medications that open the airways
    • Can improve airflow and ease breathing
    • Often used in inhaler form, similar to asthma medications

  • Diuretics (water tablets)

    • Help remove excess fluid from the body
    • May be used if there is fluid buildup in the legs or abdomen

  • Influenza (flu) vaccination

    • Strongly recommended every year
    • Reduces the risk of serious respiratory infections, which can be especially dangerous during cold Grand Rapids winters

  • Pneumonia vaccines

    • Often recommended to reduce the risk of bacterial pneumonia

  • Supplemental oxygen

    • Oxygen delivered through a nasal cannula or mask
    • Used if blood oxygen levels are low at rest, during activity, or while sleeping
    • Can improve energy levels, exercise tolerance, and organ function

Procedures and Surgery

  • Treatment of pneumothorax (collapsed lung)

    • May include:
      • Observation for small, stable pneumothoraces
      • Chest tube placement to remove air and allow lung re-expansion
      • Surgery (such as pleurodesis) to prevent recurrence

  • Management of pleural effusion and chylothorax

    • Draining fluid from around the lungs
    • Dietary changes or medications to reduce lymphatic leakage
    • Surgery in severe or recurrent cases

  • Treatment of kidney angiomyolipomas

    • Monitoring with imaging
    • Interventional radiology procedures (such as embolization) to stop bleeding
    • Surgery in selected cases

Lung Transplantation

For advanced, life-threatening LAM where other treatments are no longer effective, lung transplantation may be considered:

  • Diseased lungs are surgically removed and replaced with healthy lungs from a deceased donor
  • This is a major operation with significant risks and requires lifelong follow-up and immunosuppressive medications
  • Considered a last resort for women with severe LAM

Patients in Grand Rapids may be evaluated locally and then referred to regional transplant centers in Michigan or neighboring states as appropriate.

Emerging and Research Treatments

Because LAM is rare, it is challenging to conduct large clinical trials. However, several medications are being studied or used under specialist guidance:

  • mTOR inhibitors (e.g., sirolimus/rapamycin)

    • Immunosuppressant drugs that target the mTOR pathway affected by TSC1/TSC2 mutations
    • May slow LAM cell growth and stabilize or improve lung function in some patients
    • Now commonly considered in LAM management under specialist supervision

  • Doxycycline

    • An antibiotic that has been studied for its potential to decrease LAM cell spread in lung tissue
    • Evidence is mixed; use is typically within research or specialist settings

  • Progesterone

    • A female hormone that has been tried as a treatment in the past
    • Its role remains uncertain; current practice varies and is guided by specialist recommendations

  • Other agents under study

    • Statins
    • Metformin (an oral diabetes medication)
    • Lymphangiogenesis inhibitors
    • Autophagy inhibitors

Access to clinical trials may require referral to specialized LAM or rare lung disease centers, often coordinated by local pulmonologists in Grand Rapids.

Living With LAM in Grand Rapids

Women living with LAM in West Michigan face unique considerations:

  • Cold, dry winters can worsen shortness of breath and cough

    • Using a scarf or mask outdoors in very cold air
    • Humidifying indoor air
    • Avoiding outdoor exertion on extremely cold or high‑pollution days

  • Air quality and environmental triggers

    • Avoiding tobacco smoke, vaping, and other lung irritants
    • Monitoring local air quality reports (especially during wildfire smoke events or high-ozone days)

  • Vaccinations and infection prevention

    • Staying current with flu and pneumonia vaccines
    • Practicing good hand hygiene and avoiding close contact with people who are ill

  • Activity and pulmonary rehabilitation

    • Supervised exercise programs and breathing training (pulmonary rehab) available through local hospitals can:
      • Improve stamina
      • Reduce breathlessness
      • Enhance quality of life

  • Emotional and social support

    • Connecting with national LAM organizations and rare disease networks
    • Seeking counseling or support groups for chronic illness, often available through Grand Rapids health systems or community organizations

The Kent County Health Department and Grand Rapids Public Health can also provide information on local respiratory health resources, smoking cessation programs, and chronic disease management support.

When to See a Doctor in Grand Rapids

You should seek medical evaluation if you:

  • Have ongoing or worsening shortness of breath, especially if you are a woman of childbearing age
  • Experience repeated “asthma” or “bronchitis” episodes that are not improving with usual treatment
  • Have a history of spontaneous collapsed lung (pneumothorax)
  • Notice blood in your sputum or urine
  • Have known tuberous sclerosis complex and develop new breathing problems

Start with your primary care provider or family doctor, who can refer you to:

  • Pulmonologist (lung specialist)
  • Endocrinologist
  • Obstetrician-gynecologist (OB-GYN)
  • Urologist (for kidney involvement)

These specialists are available through major Grand Rapids health systems, including Spectrum Health, Trinity Health Grand Rapids, Metro Health, and Mercy Health.

Key Points About LAM

  • Lymphangioleiomyomatosis (LAM) is a rare lung disease that almost always affects women, usually during the reproductive years.
  • Abnormal tissue growth in the lungs causes cysts that damage lung tissue and lead to progressive breathing problems.
  • The cause of LAM is unknown, but it is linked to genetic mutations in TSC1 or TSC2, the same genes involved in tuberous sclerosis complex (TSC).
  • LAM can affect other parts of the body, especially the kidneys, causing benign tumors (angiomyolipomas) that may bleed.
  • Pneumothorax (collapsed lung) and pleural effusions (including chylothorax) are relatively common and may require emergency care.
  • Estrogen appears to play a role in disease progression, and estrogen-containing medications are usually avoided.
  • There is no cure, but treatments can:
    • Ease symptoms
    • Reduce complications
    • Slow progression in some cases
  • Women in Grand Rapids with unexplained breathing problems, especially if standard asthma treatment is not helping, should seek evaluation with a pulmonologist and may benefit from care at local health systems and public health resources.