Living with LAM

While there is no cure for LAM, living with the disease is much different today than in years past. LAM research has come a long way and has led to some amazing breakthroughs, including an effective treatment for the disease. Living with LAM is different for every patient depending on the progression of their disease. Below is useful information about living with and treating this complex disease.

Coping with Your Diagnosisplus

Receiving a diagnosis of LAM may cause you to experience feelings of anger, denial, shock, grief, helplessness, confusion, despair, sadness or fear. These feelings are common and many women experience one or all of them during various stages of the disease. These emotions are normal responses and are all a part of the grieving process. Grief isn’t related just to death; it can be the result of any loss—including the loss of your health and/or the lifestyle you once enjoyed. Understand that you’ll probably grieve because of some of the changes you may face with LAM. Many changes could be far in the future or may not occur at all, so it’s best to stay optimistic, focus on the positives, and try not to dwell on your disease.

You may experience another common reaction to your diagnosis: relief. If you, like many patients, have experienced symptoms such as shortness of breath or lung collapses without your doctor finding a reason for your problems, you’ll likely be relieved to finally have an explanation for your health issues. You’ll feel validated knowing that you didn’t imagine all of these problems and that ever-powerful fear of the unknown you’ve been living with now has a name.

As with any chronic illness, your family and friends will want to provide emotional support for you. And, just like you, they will experience a flood of emotions and will have many questions and concerns regarding your diagnosis. But you need to understand that your closest supporters may not feel any more comfortable in their new roles than you do. Good communication is essential for all of you. Let your loved ones know what you need from them. Doing so enables them to feel helpful and supportive—rather than helpless and powerless—in their relationships with you.

Good communication also helps to ensure that your relationships remain healthy, even in the tough times. In fact, you may find that some of your relationships become even stronger! Being treated differently by friends and family is one of the greatest concerns for many LAM patients. It might help your support people to accept and better understand your desire for things to remain the same if you assure them that when you do need assistance with something, you’ll let them know. A diagnosis of LAM can be difficult, but poor communication will only lead to more difficulties.

In addition to concerns regarding finances, insurance coverage and employment, you may have concerns regarding parenting and marital relations. All of these issues can trigger additional worries and anxieties. To help manage these stresses consider complementary therapies to nurture your body, mind, and spirit. Exercise, acupuncture, massage therapy, yoga and reflexology are just some ways to relax and enhance your coping skills. If the stresses accompanying your diagnosis of LAM become overwhelming, seek emotional support from a friend or relative or from a professional such as a psychologist, a counselor, or a member of the clergy. Learning how to manage stress, anxiety, and depression is necessary in all phases of life, but it’s even more essential in maintaining an optimum level of health now that you’re living with a chronic disease. Be patient with yourself.

When you’re feeling ready to confront LAM on a larger scale, you may want to attend the annual LAM conference—LAMposium. During this conference, you can attend informational sessions to learn more about LAM and specially designed sessions are also available just for your group of support people. Medical professionals, who work with LAM and who are themselves attending concurrent clinical and scientific sessions, give presentations to women with LAM and their families and they are also available to answer your questions. LAMposium is an opportunity to spend time with other women and family members who can relate to what you’re experiencing.

Vaccinationsplus

Patients with LAM should remain up to date with appropriate vaccinations. Live vaccines should be avoided in patients taking immunosuppressive agents, so vaccination recommendations differ between LAM patients who are on mTOR inhibitors and those who are not, so we will consider these separately below.

For LAM patients who are NOT taking mTOR inhibitors, we recommend:

  1. 1. Annual influenza vaccination with the inactivated vaccine. Flumist (live attenuated influenza vaccine) is not recommended in LAM patients, because diffuse lung disease is a relative contraindication.
  2. Vaccination against pneumococcus:
    • All patients should receive Prevnar, one dose in lifetime; ideally given before Pneumovax, but at least 1 year after Pneumovax.
    • All patients should receive Pneumovax, one dose every 5 years; given at least 2 months after Prevnar.
  3. Shingles (H. Zoster) vaccination:
    • For those over age 60.
    • For those over age 50 who are about to begin mTOR inhibitor therapy, optimally at least 4 weeks prior to starting.
    • Since immunity only lasts 5 years, revaccination may be necessary (although not if on mTOR inhibitor therapy by that time).
  4. Hepatitis (A and B) vaccines: recommended for all patients
  5. Tetanus vaccine: recommended for all patients

In specials circumstances such as travel to areas of endemicity; other vaccines may be considered where applicable.

For LAM patients who ARE taking mTOR inhibitors, we recommend:

  1. Annual influenza vaccination with the inactivated vaccine. Flumist (live attenuated influenza vaccine) is not recommended in patients with LAM.
  2. Vaccination against pneumococcus:
    • All patients should receive Prevnar, one dose in lifetime; ideally given before Pneumovax, but at least 1 year after Pneumovax.
    • All patients should receive Pneumovax, one dose every 5 years; given at least 2 months after Prevnar.
  3. Shingles (H.Zoster) vaccination should NOT be given while on mTOR inhibitors:
    • Patients over age 50 who are about to begin mTOR inhibitor therapy should be vaccinated, optimally at least 4 weeks prior to starting.
    • Patients could be vaccinated during a hiatus in therapy with mTOR inhibitors, such as when they are held for upcoming surgery.
    • A new zoster subunit vaccine that would be appropriate for use in patients on mTOR inhibitors is under development (Cunningham, A.L. NEJM 2016) and may be available soon.
  4. Hepatitis (A and B) vaccines: recommended for all patients.
  5. Tetanus vaccine: recommended for all patients.
  6. Avoid other live virus vaccines:
    • Measles, mumps, rubella
    • Oral polio
    • Smallpox
    • Rotavirus
    • Yellow fever
    • Rabies

Table 2 summarizing recommendations for patient on mTOR inhibitors

In specials circumstances such as travel to areas of endemicity; other vaccines may be considered where applicable.

General Comments:

Inactivated or recombinant flu vaccines (i.e. the injectable types of flu vaccine that your physician will offer to you) should not be used in anyone with prior severe allergy without consulting an allergist, and should be used with caution in: patients with moderate or severe acute illness with or without fever, history of Guillain-Barré syndrome within 6 weeks of previous influenza vaccination, people with egg allergy (hives only allergy can be mitigated with additional safety measures).

Minor illnesses (such as diarrhea, mild upper respiratory infection with or without low-grade fever, other low-grade febrile illness) are not contraindications to vaccination. Adults with egg allergy of any severity can receive inactivated vaccines with the same indications as those without egg allergy, since the new preparations contain much smaller quantities of egg products.

Contraindications to Pneumovax and Prevnar include severe prior allergic reaction, and moderate or severe acute illness. Patients with a documented true allergic reaction (rather than a history of egg allergy) to Prevnar or Pneumovax should seek the advice of an allergist.

Although inactivated and recombinant flu and pneumococcal vaccinations can result in soreness and low grade fever and muscle aches, they cannot produce flu or pneumonia.

There is a growing literature suggesting that patients on low dose immunosuppression (such as low dose sirolimus) can take shingles vaccine safely, but further studies are needed before this recommendation can be made.