If a patient develops chronic GVHD, it will usually happen three-to-eighteen months after transplant.
Patients who have had acute GVHD have the greatest risk of developing chronic GVHD. There is also an increased risk for older patients, patients who were transplanted with unrelated or mismatched donor cells, patients transplanted with stem cells collected from the bloodstream rather than the bone marrow, and those who received additional white blood cells from the donor after transplant (a donor leukocyte infusion).
Chronic GVHD may last several months or even years. Most patients who develop chronic GVHD experience a mild or moderate form of the disease, but approximately 15 percent develop a more severe, life-threatening form.
Chronic GVHD can affect many organs in the body but most often occurs in the mouth, on the skin, in the eyes and/or in the lungs.
There are several different treatments for chronic GVHD and new treatments are constantly being tested. The drugs used most often to control chronic GVHD are cyclosporine, prednisone, mycophenolate mofetil (Cellcept®) and rapamycin (sirolimus). Each of these drugs has side effects which are described in our Drug Database.
Chronic GVHD is most often seen in the mouth. It may appear as red ulcers which, in some cases, extend down the throat. It may also cause a very dry mouth, although the chemotherapy drugs you received before transplant can cause this symptom as well.
Since a lack of saliva can lead to cavities, it is important to see a dentist once or twice a year for a thorough cleaning and check-up. Make sure you inform your dentist that you've had a transplant, particularly if you are on medications that suppress your immune system.
Mouth GVHD is typically treated with an oral topical steroid alone, or a combination of drugs to suppress your immune system and control chronic GVHD throughout your body. Medication such as lidocaine may be prescribed to control the pain.
If you experience dry mouth, avoid caffeinated and alcoholic beverages and drink plenty of water each day. There are also several over-the-counter products that can help with dry mouth.
A procedure called PUVA therapy has helped some patients who developed GVHD in their mouth. Patients are given a drug called psoralen that sensitizes the skin, and an ultraviolet light is shone into the mouth. The therapy requires special equipment and is not available at all transplant centers.
Chronic GVHD can also appear on the skin. It may appear as a dry, itchy rash, a change in skin color or thickening of the skin that can restrict joint movement. It can also cause brittle nails, and thickening of the tissue and muscles under the skin.
Several therapies are available to treat skin GVHD. Topical steroids or topical prograf ointment may relieve the problem. If tight skin is restricting joint movement, physical therapy is often recommended.
Staying out of the sun and using a strong sun screen or clothing designed to protect you from the ultraviolet rays of the sun is essential. This is a precaution you'll need to take for the rest of your life. Companies like Sun Precautions offer sun protective clothing and the makers of RIT Dye make SunGuard™, a laundry detergent additive that will add sun block to your everyday clothing and maintain its effectiveness for up to 20 washings.
Extracorporeal photopheresis (ECP) is another potential treatment for skin GVHD. This procedure, which is not available at all transplant centers, removes white blood cells from the patient, mixes them with psoralen, and exposes them to ultraviolet light. The cells are then reinfused into the patient. Studies have shown this to help many, but not all, patients with skin GVHD.
GVHD in the Eyes
When chronic GVHD affects the eyes, the result is dry eyes. The eyes may be itchy and uncomfortable. In severe cases, GVHD in the eyes can affect vision.
Dry eyes are usually managed with artificial tears, ointments or tear duct plugs, which plug the tear drainage system resulting in more moisture in the eyes. Oral medications like Salagen® and Evoxac® can stimulate tears that moisturize the eyes.
New treatments for severely dry eyes include the PROSE lens available at several locations throughout the United States. You can learn more about this option through the Boston Foundation for Sight.
A special type of glasses called chamber glasses provide relief for some patients by retaining more moisture in the eyes. The glasses can be cumbersome to wear and interfere with peripheral vision, however, so some patients prefer other options.
Some patients have found that a serum made from their blood (autologous serum) and used as eye drops helps.
Consult an ophthalmologist to determine which of these treatments is right for you.
If you have GVHD in your eyes, wear sunglasses to protect your eyes from sun damage. Be sure to choose glasses that have ultraviolet protection (UV). To add additional protection from sunlight, wrap around sunglasses are preferable.
When chronic GVHD affects the lungs, it can cause a condition called bronchiolitis obliterans, which makes breathing difficult. This is a very difficult condition to treat. Extracorporeal photopheresis has helped some with this condition.
National Cancer Institute Chronic GVHD Program
An important resource for people living with chronic GVHD is the National Cancer Institutes's chronic GVHD program. Patients between the ages of 1 and 75 can schedule an appointment for a comprehensive evaluation of their GVHD. Specialists familiar with how chronic GVHD affects every part a patient's body conduct the four-day physical examination, and make recommendations on how best to manage the disease.
The medical evaluation is free of charge and takes place in Bethesda, Maryland. It is part of a clinical trial designed to gather a national database of information about chronic GVHD so that better treatments can be developed.
Monitor Yourself for Symptoms of GVHD
Like many conditions, chronic GVHD is easier to treat if detected early. Contact your doctor if you develop any of the following symptoms:
- Skin: rash, discoloration, tightness or changes in texture
- Hair: thinning
- Nails: changes in texture, brittleness or ridges
- Eyes: irritation, dryness, blurred vision, a gritty feeling
- Mouth: dryness, sensitivity to foods or toothpaste
- Vagina: dryness, irritation, tightening
- Penis: irritation
- Digestive System: nausea, vomiting, diarrhea, loss of appetite, unexplained weight loss
- Lungs: chronic cough, wheezing, shortness of breath
- Joints: difficulty fully extending fingers, wrists, elbows, knees or ankles
- Low grade fever
These symptoms may also be caused by something other than chronic GVHD, but you should report them to your doctor immediately so that you can be evaluated.
Treatment Side Effects
The steroids often used to treat chronic GVHD can cause unpleasant temporary side effects such as weight gain, bloating, mood swings and depression. It may also elevate blood glucose levels, which may require the use of diabetic medications. You can check our Drug Database to learn more about the side effects of the medications you may be prescribed.
After a time, your doctor will try to wean you off these drugs. However, if the chronic GVHD flares up again, you may need to be put back on them for a time.
While you are taking steroids, it is very important that you follow your doctor's guidelines for preventing infection such as those listed in the preventing infections section of this website. Infection is the leading cause of death among patients with GVHD, so taking steps to reduce your exposure to infection is critical. Most patients are either put on antibiotics to prevent bacterial infections, or are given antibiotics to have on hand in case they develop a fever.
Most patients recover from GVHD, though some symptoms, such as skin sensitivity to sunlight, may linger.
New drugs and strategies are constantly being tested to reduce the incidence and severity of chronic GVHD. You may be invited to join a clinical trial to test the effectiveness of these new therapies. Clinical trial participation is a long-standing process to test new medical procedures or medication.
Consult our online Resource Directory for additional resources to help cope with chronic GVHD.