Hematologic – leukemia, lymphoma, myeloma

Leukemia is different from many cancers, as there is no tumor. Rather, the cancerous cells are found throughout the body, in the blood or in bone marrow. Leukemia interferes with the normal production of red cells, white cells, and platelets in the bone marrow. The medications used to treat this disease may also injure the healthy stem cells that produce white blood cells, red blood cells and platelets. It’s common for patients with leukemia to develop anemia (low levels of red cells) and thrombocytopenia (low levels of platelets), and in some cases, leukopenia (low levels of white cells). Red cells and platelets may be replaced by healthy donated cells in the form of blood transfusions. Most patients with leukemia require some transfusions during their care.

Platelet transfusions are common in leukemia, but not everyone needs them. Usually blood can still clot even if the platelet count is low, as long as the platelets are functional. Platelet transfusions are sometimes given if the number gets low or if the person is having distressing symptoms, such as nosebleeds or bleeding gums. If there is no distressing bleeding and the disease is in the final stages, then platelet transfusions may never be necessary.

Red cell transfusions are used to treat low hemoglobin (red cell) counts. When considering the need for a red cell transfusion blood count results are important, but often the focus is on how the person feels. For example, a person can be conscious and able to communicate with a hemoglobin count in the 40s, which is quite low. This is more likely if the decrease in hemoglobin has been gradual and the person has become accustomed to low levels. When hemoglobin is this low, people commonly feel profound fatigue, confusion and short of breath. With good palliative care these symptoms are manageable.

As leukemia progresses, the frequency of transfusions may increase to two or three times a week. Requiring transfusions more frequently is usually considered a milestone. More frequent transfusions usually are not helpful and the patient is likely to live only a few more days, or a very few weeks at most.

When transfusions are needed several times a week, the disease has progressed to the point where other symptoms may diminish quality of life. At this point the immune system is extremely weak and infections are a real threat. Infections can be treated with antibiotics, but without a functioning immune system, they can’t be overcome completely. Also at this point people are very weak overall, have little energy, and so spend most of their time in bed. This does not mean, however, that they can’t still find some quality in their life.

Someone who has leukemia may die from different things. There may be a sudden loss of blood or a stroke, because of the inability of the blood to clot. There may be complications from low hemoglobin levels. Infection is possible. Pneumonia is a common complication because people are at risk of aspiration. Sometimes death comes from a combination of things. Sometimes someone just gradually gets weaker and body systems shut down.

People with leukemia often come to think of transfusions as a lifeline, because it’s the only thing that stands in the way of death. It’s often difficult for people and their families to let go of that lifeline as death approaches. Yet there comes a time when the lifeline no longer works. With good palliative care, the symptoms that may develop at the end of life can be well controlled to keep the person comfortable.

Non-Hodgkin's lymphoma (NHL) is not a single disease. It’s a complex group of cancers that start in the body’s immune system. The diseases have similarities at the cell level, but they have different effects on the body and show different responses to treatment. A person’s experience with NHL will depend on the type of disease, its location and the treatments given.

NHL may progress slowly (low-grade lymphoma) or quickly (high-grade lymphoma). Low-grade lymphomas typically develop over several years. High-grade lymphomas develop more quickly, but they also tend to respond better to treatment. When a high-grade lymphoma doesn’t respond to treatment, disease progression tends to be quite rapid.

NHL is usually found in parts of the body's lymphatic system, such as the lymph nodes. It also can be found in the blood and bone marrow, and at times in the brain, chest, abdomen or other areas. The primary tumor may spread to other places in the body. The location of the primary tumor and of any metastases determines many of the possible symptoms. In general, the body organs or systems that have a tumor eventually fail to function properly. For example, someone with a tumor in a lymph node in the neck may eventually have a very visible lump on the neck that can interfere with breathing or swallowing. Someone with NHL in the brain eventually shows changes in mental processes. Someone with NHL in the chest may have trouble with breathing or circulation.

The type of treatment given to someone with NHL may also affect what someone experiences. Chemotherapy, radiation therapy, bone marrow or stem cell transplants are among the most common treatment options, depending on the disease. Each of these treatments may produce ongoing symptoms.

While these and other variables play a role in what happens with NHL, people generally experience these symptoms as the disease advances:

• Pain
  The tumor or the metastases can create pressure or an obstruction in an area, which produces pain. Pain medications are used to control the pain and keep the person comfortable.
   
• Infections
  Infections may be common if the immune system is not working well.
   
• Bleeding or anemia and related fatigue
  These symptoms may occur if the disease is in the bone marrow. They are treated with blood and platelet transfusions. In late stages of the illness, a patient may become exhausted by the effort to have the necessary blood tests and transfusions. As well, eventually the transfusions will not have the desired effect. At that point the patient and the health care team need to decide whether they will decrease or stop the transfusions.

Someone with NHL may experience symptoms that are common to most cancers generally, which affect energy, strength, appetite, breathing and responsiveness.

The changes can be gradual, but crises can develop. People with NHL most often die from infections, bleeding or organ failure resulting from metastases. A serious infection or sudden bleeding can quickly lead to death, even if someone doesn’t appear very ill. For this reason, the end stages of life for people with NHL are harder to predict than for people with other cancers.

Caring for teenagers who are dying can be quite challenging. Your teenage patient is at the point where he is trying to exert his independence and gain some freedom. However, as his disease progresses, he will become more and more dependent on others. With his general decline, his relationships with friends and his family will be affected, and his illness will have an impact on every aspect of his life.

Your patient may describe the hopes he has, and may focus on the future and what he wants to do. Although he likely understands his illness trajectory, he may talk about what he will do in the distant future. This is because he does not want to live in the shadow of his illness all the time. He wants to have as much normalcy in life as possible and not have people focus on his illness all the time. This may seem like a form of denial, but it is a very normal way for teens to continue to live their lives in the face of their disease and its progression. Therefore, it is important that you acknowledge your patient’s desire to embrace life.

Changes in physical appearance and ability are major aspects of illness that deeply affect teenagers. Physical changes can include hair and weight loss and decreased energy.These changes can significantly affect a teen’s body image and how he views himself in the world. As a result, your patient may withdraw. During these times, you should assess for signs and symptoms of depression. Teens are usually straightforward, so ask your patient direct questions about his symptoms and their effects on his life. Teens are usually willing to talk about these issues with health care providers whom they trust and have a relationship with.

In terms of emotions, be prepared for your patient to display a variety of reactions. Medications, such as dexamethasone, may have a significant impact on mood. Expressing anger and frustration at his situation would be quite normal.

Peers and friendships are extremely important to teenagers. So it will be vital to recognize how your patient’s illness has affected these relationships and encourage him to spend time with his friends. He may need someone to help organize or coordinate time with friends when he is feeling well enough for some activity. However, he may only be able to tolerate short visits if he is tired from treatments or is experiencing a general decline in his overall health. Encouraging him to maintain his social media connections (which is likely happening anyway) may be a good way for him to stay connected with friends when he is quite ill or has limited energy.

You should consider the impact of your patient’s illness on his friends and on his significant other, if he has one. Identify resources that could help his friends and their families if they lack experience with death and dying. Knowing your patient’s limitations and abilities and how these may continue to change is also important for friends. So you should discuss with your patient and his family about how to communicate information about his illness and decline. The parents of his friends may want to know what is happening so they can prepare their children for changes and the loss they will experience.

Your patient’s relationships with family members will change as his illness progresses―in particular, his relationship with his parents. As he becomes more dependent on them, they will need to manage his symptoms and medications, and eventually his basic care needs, such as personal hygiene. Losing the ability to function independently can be particularly distressing for teens and also for parents. As these losses accumulate, the reality of your patient’s illness will become difficult to ignore. It will affect the whole family, particularly his siblings, if he has any. His family will realize that trying to maintain a routine and a degree of normalcy may become more difficult as his disease progresses. A key role for the health care team will be preparing the family for further changes and helping them cope.

Discussing specific treatments and end-of-life decisions, such as a “do not attempt resuscitation” order, can be quite difficult with teenagers. A challenge for the health care team is determining when to have such discussions and whom to involve. When the focus of care has changed to a palliative approach, research shows that teens actually want to be more involved in their health care decisions.Your patient may not have wanted to participate in treatment decisions in the past, but he may wish to now. This may be because of the limited treatment decisions usually offered with oncology treatments when first diagnosed, as well as the overwhelming nature of an initial diagnosis. So it is crucial to focus on how he wants to be involved in decisions about what care he receives, where care is provided and how often follow-up and clinic appointments are scheduled.

Be flexible in order to maximize your patient’s time. Avoid setting up long appointments or making him wait in a clinic to see you. If he is trying to attend school, work around his class schedule and help him get to school as much as possible. A crucial role for the health care team will be helping his school cope with the progression of his illness and his death. Preparing school staff to deal with students under these circumstances will be an important consideration for the health care providers and school administration.

Palliative care at any age requires managing symptoms to ensure good quality of life. Teenagers can have significant symptoms, so make assessing and managing your patient’s symptoms a priority.

References

Coccia PF, Altman J, Bhatia S, Borinstein SC,et al. Adolescent and young adult oncology: clinical practice guidelines in oncology. JNCCN. 2012;10(9):1112-1150.

Freyer, DR. Care of the dying adolescent: special considerations. Pediatrics. 2004;113:381-388.

Freyer DR, Kuperberg A, Sterken DJ, Pastyrnak SL, Hudson D, Richards T. Multidisciplinary care of the dying adolescent. Child AdolescPsychiatrClin N Am. 2006;15:693-715.

Pritchard S, Cuvelier G, Harlos M, Barr R. Palliative care in adolescents and young adults with cancer. Cancer. 2011;117(10 Suppl):2323-2328.