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Survivorship in Hematological Malignancies��The Long Road

Sandra Garofalo MS, APRN, AOCNP

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Survivorship in Hematological Malignancies

Hopeful Living

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Sandra G. Garofalo MS, APRN, AOCNP

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Disclosures:

Janssen Oncology

Jazz Pharmaceuticals

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Sandra G. Garofalo MS, APRN,  AOCNP

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Learning Objectives

  • Define survivorship and it's phases
  • Outline National Comprehensive Cancer Network (NCCN) guidelines for survivorship care
  • Discuss survivorship in incurable malignancies
  • Identify impact of adverse affects on survivors 
  • Describe social, emotional and physical challenges of cancer survivors and etiology 
  • List interventions for challenges that survivors face

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Survivorship begins at diagnosis

As of January 2022, it is estimated that there are 18.1 million cancer survivors in the United States. This represents approximately 5.4% of the population. 

Over the next decade, the number of people who have lived 5 or more years after their cancer diagnosis is projected to increase approximately 30%, to 16.3 million.

The number of cancer survivors is projected to increase by 24.4%, to 22.5 million, by 2032.

Division of Cancer Control and Population Sciences (DCCPS). (n.d.). https://cancercontrol.cancer.gov/

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Phases of Survivorship

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Time of Diagnosis

Acute Survivorship

End of Initial Treatment

Extended Survivorship

End of Initial Assessment

Permanent Survivorship

Focus

Cancer Treatment

Immediate effects of cancer and treatment

Long term effects of cancer and treatment

Duration

Several weeks

Several months

Several years

Physical, social and emotional challenges exist through all phases of treatment

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Living with Blood Cancer

Smith A, Howell D, Patmore R, Jack A, Roman E. Incidence of haematological malignancy by sub-type: Nov 2011

Living with cancer as opposed to living after cancer

Blood cancers may be curable (acute leukemia, lymphoma) however, 60% are not (multiple myeloma, chronic leukemia, etc.)

Curable disease often comes with intense and long therapy such as cycles of chemotherapy, stem cell transplant and other cellular or immunotherapies.

Helping patients find hope in treatable disease that is incurable.

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NCCN Guidelines�

Screening for recurrence, progression or additional cancers

Monitoring long-term effects of cancer and treatment

Health Maintenance and management

Coordination of Care, referral to specialists 

Support and Education in survivorship

Denlinger CS, Sanft T, Moslehi JJ, et al. NCCN Guidelines Insights: Survivorship, Version 2.2020. J Natl Compr Canc Netw. 2020;18(8):1016-1023. doi:10.6004/jnccn.2020.0037

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Nursing Care Plans 

  • Survivorship care plans are recommended by The Institute of Medicine
  • Oncology Nursing Society conducted extensive literature review of the use and outcomes of care plans
    • Evidence and evaluation of the effectiveness of survivorship care plans in hematology care plans is lacking especially in comparison to those of solid tumors
    • Nurses have expertise in health promotion, information, support, resource provision and can develop and disseminate survivorship care plans to facilitate communication and action between patient and medical team. 
    • Current evidence shows benefit to well structured care plan in survivorship 

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Taylor K, Monterosso L. Survivorship Care Plans and Treatment Summaries in Adult Patients With Hematologic Cancer: An Integrative Literature Review. Oncol Nurs Forum. 2015 May;42(3):283-91. 

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Lasting Effects of Cancer Treatment

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Impact of Lasting Side Effects

  • Greatest Impact on Quality of life per patient report 9
    • Fatigue 42.9%
    • Chemotherapy induced peripheral neuropathy 27.4%
    • Pain 12.4%
    • Sexual dysfunction and/or infertility 9.6%
  • Disparities in adverse effects
    • Children and young adults (<1 yr – 39 yrs) have higher risk of severe and long term side effects 8
    • Women have a 34% high rate of severe adverse effects of cancer treatment  14
      • Higher compliance to treatment?
      • More likely to report symptoms?
      • Difference in metabolism?

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Social and Emotional Challenges and Causes

    • fear of progression and recurrence

Anxiety

    • fatigue, isolation, fear of infection

Depression

    • Common side effect of medications

Fatigue

    • steroid use, anxiety, depression 

Insomnia

    • cost of medical care, medications, chemotherapy

Financial toxicity

    • due to physical disabilities, side effects, frequency of treatment and/or distance from treatment

Loss of employment

    • role changes due to illness, co-morbities and physical changes in appearance 

Loss of "pre-cancer" identity

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Interventions

Anxiety

Depression

Fatigue

Insomnia

Financial toxicity

Loss of employment

Loss of "pre-cancer" identity

Counseling, support groups, frequent screening and clinic contact

Yoga, art therapy, physical therapy

Psychiatry referral for medications

Check labs for anemia, hypothyroidism, vitamin deficiency

Sleep hygiene, medications

Social work consult

Patient advocacy for financial resources

Side effect management 

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Physical challenges of Survivorship

Cardiotoxicity

    • Athracyclines (doxorubicin), BTK inhibitors (zanubrutinib, ibrutinib) 

Endocrine dysfunction 

    • Thyroid dysfunction from Tyrosine kinase inhibitors  (dastatinib) , hyperglycemia from steroid use 

Decreased bone density

    • Chronic steroids use, anti-resorptive therapy (denosumab for multiple myeloma)

Pulmonary dysfunction

    • Cyclophosphamide, busulfan, fludarabine, methotrexate

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Physical challenges of Survivorship

Kidney dysfunction

    • Disease state (multiple myeloma), chemotherapy toxicity, comorbid conditions

Immunocompromised state

    • Post cellular therapy
    • Chronic treatment

Fatigue

    • Common side effect of neoplastic agents, nutritional deficiency, depression, insommnia

Infertility and sexual dysfunction

    • Chemo toxicity, barrier methods and birth control for long term therapies

Malnutrition

    • Anorexia, dysgeusia, comorbid conditions

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Interventions

  • Cardiotoxicity
  • Endocrine dysfunction
  • Bone density
  • Pulmonary function
  • Fatigue
  • Kidney dysfunction
  • Infertility/Sexual dysfunction
  • Malnutrition
  • Neuropathy

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Cardiology referral, cardiac rehab

Monitor TSH, glucose

Chest CT, pulmonology, medications

Nephrology, monitor creatinine, control hypertension 

Check vitamin D, B12, iron

Fertility clinic (prior to treatment), sex therapist

Dietician consult, medications

Neurology , pain management, alternative therapy, medication

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Risk for Infection

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Incidence and Risk of Infection

Infectious disease is 2nd leading cause of death in cancer patients

60% of cancer deaths are related to infection especially in hematological malignancies 3

Pneumonia and influenza are most common infections, followed by parasitic disease, other infections and sepsis.

Risks include neutropenia, steroid use, physical deconditioning, need for re-immunization, drug and cellular therapies and central venous catheters which account for 25% of blood stream infections. 11

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Bispecific Antibody Drug therapy

First bispecific approved in 2014, blinatumomab for acute lymphoblastic leukemia

More than 10 new bispecific antibodies have been approved and are in use since 2021 for lymphoma, multiple myeloma, leukemia and solid tumors. 5

Analysis of clinical trials show that half of patients treated will develop infection and a quarter of patients will develop a grade III or grade IV infection. 4

75% of patients on bispecific antibodies will develop hypogammaglobulinemia (HGG) 6

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Reducing the Risk

Guido Lancman, et al; Infections and Severe Hypogammaglobulinemia in Multiple Myeloma Patients Treated with Anti-BCMA Bispecific Antibodies. Blood 2022

Studies presented at American Society for Hematology conference 2024 showed promise of intravenous immunoglobulin (IVIG) for the treatment of HGG.

Of the 70% of patients who had disease response to treatment, 100% also developed HGG in severe range IgG <200 mg/dL.

Patients were treated with IVIG and were varicella prophylaxis.

Infections grade III and higher were reduced by 80%.

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Management of IVIG  

Guo, Y., Tian, X., Wang, X., & Xiao, Z. (2018). Adverse Effects of Immunoglobulin Therapy. Frontiers in immunology, 9, 1299. https://doi.org/10.3389/fimmu.2018.01299

Side effects of IVIG include headache, flushing, malaise, fever, chills, chest tightness, rash. Long term use increases risk of thrombotic events and renal failure.

These can often be confused with symptoms of cytokine release syndrome.

Close monitoring is encouraged. Start IVIG therapy on different treatment day from bispecific antibody therapy until patient is stable.

Most patients will need continuous therapy and monitoring of IVIG levels.

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Summary

  • People are surviving longer with hematological malignancies
  • Nurses and survivorship plans are essential in management
  • Long term effects include physical, social and emotional issues
  • More patients are being treated for incurable disease making long term management of survivorship essential particularly in hematological malignancy.
  • Newer therapies such as bispecific antibodies hold promise but increase risk of infection.
  • Use of IVIG can greatly reduce this infection risk

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References 

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  1. Denlinger CS, Sanft T, Moslehi JJ, et al. NCCN Guidelines Insights: Survivorship, Version 2.2020. J Natl Compr Canc Netw. 2020;18(8):1016-1023. doi:10.6004/jnccn.2020.0037
  2. Division of Cancer Control and Population Sciences (DCCPS). (n.d.). https://cancercontrol.cancer.gov/
  3. Elfaituri, M.K., Morsy, S., Tawfik, G.M., Abdelaal, A., El-Qushayri, A.E., Faraj, H.A., Hieu, T.H., & Huy, N.T. (2019). Incidence of Infection-related mortality in cancer patients: Trend and survival analysis. Journal of Clinical Oncology.
  4. Farah Mazahreh, Liyan Mazahreh, Carolina Schinke, Sharmilan Thanendrarajan, Maurizio Zangari, John D. Shaughnessy, Fenghuang Zhan, Frits van Rhee, Samer Al Hadidi; Risk of infections associated with the use of bispecific antibodies in multiple myeloma: a pooled analysis. Blood Adv 2023; 7 (13)
  5. https://www.fda.gov/drugs/spotlight-cder-science/bispecific-antibodies-area-research-and-clinical-applications
  6. Garfall, A. L., & Stadtmauer, E. A. (2023). Understanding Infection Risk with Anti-BCMA Bispecific Antibodies. Blood cancer discovery, 4(6), 427–429. https://doi.org/10.1158/2643-3230.BCD-23-0157
  7. Guido Lancman, Kian Parsa, Cesar Rodriguez, Joshua Richter, Hearn J. Cho, Samir Parekh, Shambavi Richard, Adriana Rossi, Larysa Sanchez, Santiago Thibaud, Sundar Jagannath, Ajai Chari; Infections and Severe Hypogammaglobulinemia in Multiple Myeloma Patients Treated with Anti-BCMA Bispecific Antibodies. Blood 2022; 140 (Supplement 1): 10073–10074. doi: https://doi.org/10.1182/blood-2022-163733
  8. Guo, Y., Tian, X., Wang, X., & Xiao, Z. (2018). Adverse Effects of Immunoglobulin Therapy. Frontiers in immunology, 9, 1299. https://doi.org/10.3389/fimmu.2018.01299
  9. Hudson MM, Bhatia S, Casillas J, Landier W; SECTION ON HEMATOLOGY/ONCOLOGY, CHILDREN’S ONCOLOGY GROUP, AMERICAN SOCIETY OF PEDIATRIC HEMATOLOGY/ONCOLOGY. Long-term Follow-up Care for Childhood, Adolescent, and Young Adult Cancer Survivors. Pediatrics. 2021;148(3):e2021053127. doi:10.1542/peds.2021-05312
  10. Taylor K, Monterosso L. Survivorship Care Plans and Treatment Summaries in Adult Patients With Hematologic Cancer: An Integrative Literature Review. Oncol Nurs Forum. 2015 May;42(3):283-91. doi: 10.1188/15.ONF.283-291. PMID: 25901380. 32199685.
  11. Seo, S. K., Liu, C., & Dadwal, S. S. (2021). Infectious Disease Complications in Patients with Cancer. Critical care clinics, 37(1), 69–84. https://doi.org/10.1016/j.ccc.2020.09.001
  12. Smith A, Howell D, Patmore R, Jack A, Roman E. Incidence of haematological malignancy by sub-type: a report from the Haematological Malignancy Research Network. Br J Cancer. 2011. Nov 22;105(11):1684–92. doi: 10.1038/bjc.2011.450
  13. Unger JM, Vaidya R, Albain KS, et al. Sex Differences in Risk of Severe Adverse Events in Patients Receiving Immunotherapy, Targeted Therapy, or Chemotherapy in Cancer Clinical Trials. J Clin Oncol. 2022;40(13):1474-1486. doi:10.1200/JCO.21.02377

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