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Coding for Mucositis
 ICD-9-CM Coordination and
Maintenance Committee Meeting

           Patrick J. Stiff, MD
    Loyola University Medical Center
          September 30, 2005
What is Mucositis?
    Mucositis is inflammation of the
        mucosal surfaces throughout
        the body.
    It typically involves redness and
        ulcerative sores in the soft
        tissues of the mucosa.
    Oral mucositis manifests as erythema,
        inflammation, ulceration, and hemorrhage in
        the mouth and throat.

Image from: Spielberger, Ricardo; KepivanceTM: A Breakthrough for Oral Mucositis Associated with Myeloablative
Hematopoietic Stem Cell Transplantation; City of Hope National Medical Center, Department of Hematology and Bone
Marrow Transplantation
Causality
 Mucositis is a frequent complication of
    anticancer treatment, including chemotherapy
    and radiation therapy.
 Due to high-dose chemotherapy and/or
    radiation preconditioning, it is particularly
    common in patients preparing for bone
    marrow transplantation (BMT).


Source: Bellm LA, Epstein JB, Rose-Ped A, Martin P, Fuchs HJ. Patient reports of complications of bone
marrow transplantation; Support Care Cancer. 2000 Jan;8(1):33-9
Mechanisms
 Characterized by damage to the epithelium of the
    oropharyngeal cavity and GI tract
 Rapidly dividing basal cells of the oral mucosa are among the
    body cells vulnerable to damage by chemotherapy and radiation
    therapies.*
                                     Phases of Mucositis**




*National Institutes of Health Symptom Research: http://symptomresearch.nih.gov/Chapter_17/sec7/cghs7pg1.htm
**Adapted from Sonis. Nat Rev Cancer. 2004;4:277-284.
Sites of Mucositis
 While the oral mucosa is the most frequent site of
    mucosal toxicity, mucositis also is common along the
    entire alimentary tract:
      – Esophagus                – duodenum                    – colon
      – Stomach                  – ileum/jejunum               – rectum
 GI mucositis occurs via a mechanism similar to that
    in the oral mucosa, only the damage to the mucosal
    layer is more aggressive than in oral mucositis.
 Although less common, treatment of ovarian and
    nasopharyngeal cancer also can result in vaginal and
    nasal mucositis.
Source: http://symptomresearch.nih.gov/Chapter_17/sec2/cghs2pg1.htm
Severity Scales
 Many validated instruments have been
  developed to stage and measure mucositis
  severity.
  – National Cancer Institute’s Common Toxicity
    Criteria (NCI CTC)
  – World Health Organization’s (WHO’s) Oral Toxicity
    Scale (OTS)
WHO’s Oral Toxicity Scale
           World Health Organization’s Oral Toxicity Scale
                                               Severe Mucositis


    Grade 1            Grade 2           Grade 3             Grade 4
                                      Ulcers with        Mucositis
                     Erythema,
                                      extensive          to the extent
                     ulcers;
   Soreness                           erythema;          that
                     patient can
   ± erythema                         patient            alimentation
                     swallow
                                      cannot             is not
                     solid food
                                      swallow food       possible
Epidemiology
     40% of patients receiving standard-dose chemotherapy
     80% of patients receiving radiation therapy for head and neck
        cancer
     75% of all patients undergoing BMT
                                                                 Mucositis Frequency: BMT with
Mucositis Frequency:            Mucositis Frequency: Radiation   High-Dose Chemotherapy and/or
  Standard-Dose Chemotherapy      for Head and Neck Cancer         Radiation Pre-Conditioning
                40%                             80%                           75%




                       60%                                            25%
                                      20%
                                Mucositis Reported
                                Mucositis Not Reported

    Source: Bellm et al. 2000
Clinical Consequences
    Painful condition that interferes significantly with
        patient functioning and tolerance for cancer therapy
    Functional implications may include:
         – Inability to tolerate food or fluid intake
         – Difficulty or inability to talk*



    In a recent study, BMT patients ranked mucositis as the
     most debilitating side effect of their treatment.**


*Borbasi S, Cameron K, Quested B, Olver I, To B, Evans E. More than a sore mouth: patients' experience of
oral mucositis. Oncol Nurs Forum. 2002;29:1051-1057, and Bellm et al. 2000.

**Bellm et al. 2000
Clinical Consequences (continued)
 Hospital admission or extended hospitalization for
    total parenteral nutrition, intravenous analgesia, and
    intravenous antibiotics
      – 62% of patients require hospitalization.*
      – 70% of patients with Grade 3-4 oral mucositis require
        feeding tubes.*
 Reduction or cessation (dose-limiting toxicity) of
    cancer treatment in 35% of patients*


*Sonis ST, Elting LS, Keefe D, Peterson DE, Schubert M. Perspectives on cancer therapy-induced
mucosal injury. Cancer. 2004;100(9 Suppl):1995-2025
Differential Diagnosis and Treatment
 Mucositis is distinguishable from other forms
   of inflammatory disorders in terms of:
   – causality
   – clinical presentation/lab findings
   – severity
   – treatment

 The presence and severity of radiation- or chemotherapy-
  induced mucositis is routinely documented in clinical
  progress notes.
Inconsistency in Coding
             ICD-9-CM Codes That Are Sometimes Used to Describe Mucositis
             Code(s)                                                          Limitation(s)
 288.0, Agranulocytosis;                Agranulocytosis and mucositis are distinct conditions without causal relationship.
 V58.0, Encounter or
   admission for radiotherapy;
   and
 V58.1, Encounter or
   admission for chemotherapy

 528.0, Stomatitis                       Stomatitis coding is non-specific and does not permit differentiation based on
    – NOS                                   etiology (viral, fungal, etc.).
    – ulcerative                          May be used to represent mucosal damage that occurs due to local trauma
    – Excludes stomatitis:                  (for example, biting, denture irritation, or localized infection)
        • acute necrotizing ulcerative
                                          Generally is used to describe conditions less severe and more localized than
        • aphthous
        • gangrenous                        oral mucositis, as well as conditions that may be histologically distinct from oral
        • herpetic
                                            mucositis
        • Vincent’s

 996.85, Complications of                Some physicians are using 996.85 to describe mucositis.
   transplanted organ, bone               ICD-9-CM code 996.85 is used for all BMT complications, for example, graft-
   marrow                                   versus-host disease.
 530.10, Esophagitis                     Mucositis of the stomach, duodenum, and other parts of the alimentary tract is
   unspecified, and 535.XX,                 clinically distinct from other types of inflammatory diseases of the GI system
   Gastritis and duodenitis                 (for example, esophagitis resulting from gastroesophageal reflux disease).
Stomatitis: Non-Descriptive and
Outdated
 Non-descriptive term
 Not representative of current clinical
  nomenclature
 Used to describe conditions that are:
   – less severe than,
   – more localized than, and
   – histologically distinct from oral mucositis
Differential Diagnosis: Oral
                                           Clinical Presentation/Lab
Disease/Injury           Causality                  Findings                  Severity            Treatment Options
Oral mucositis      Chemotherapy           Diffuse redness,              Varies; in BMT       Palliative rinses,
                    and radiation          ulcerations, and pain,        setting up to 98%    narcotics, palifermin in
                    therapy                particularly in areas         have Grade 3/4       the BMT setting
                                           where teeth abut tissue

Aphthous            Etiology not           Single painful ulcer          Localized, but       Topical
stomatitis          identified                                           painful; maximum
                                                                         grade 2
Herpetic            HSV1                   Usually several spots;        Usually grade 1-2    Acyclovir, valacyclovir,
mucositis                                  ulcerative                                         foscarnet
Oral thrush         Candida                Varies from painless to       Usually grade 0-1    Nystatin rinses; fluconazole
                                           mild soreness; whitish                             and other azoles
                                           plaques
Denture/oral        Dentures               Common in elderly patients    Can limit calories   Repair, removal of
trauma                                     with loose-fitting dentures                        dentures
Gangrenous          Bacterial infections   Necrotic                      Rare, can be         Antibacterials that treat oral
stomatitis                                 pseudomembranes               severe               aerobes and anaerobes
Acute necrotizing   Bacterial infections   Pain, fever, necrotic,        Grade 3/4            Control of infection
stomatitis          in immune deficient    bloody ulcers
                    patients
Differential Diagnosis: Oral
 Local, Denture-Related Lesion                     Oral Thrush                           Aphthous Ulcer




           Oral Mucositis*

                                          Because these conditions can coexist
                                              in immunocompromised patients,
                                              differential diagnosis is critical.




* Spielberger, Ricardo; KepivanceTM: A Breakthrough for Oral Mucositis Associated with Myeloablative Hematopoietic Stem
Cell Transplantation; City of Hope National Medical Center, Department of Hematology and Bone Marrow Transplantation
Differential Diagnosis: GI

                                                Clinical
                                             Presentation/
Disease/Injury            Causality          Lab Findings         Severity      Treatment Options
Mucositis of           Chemotherapy       Typhilitis,           Mild to life-   Supportive care,
the GI tract           and radiation      diarrhea, ileus,      threatening     treatment of
                                          bowel obstruction                     infectious
                                                                                complications
Crohn’s disease        Autoimmune         Diarrhea, pain,       Mild to life-   Steroids, antibody
                                          bowel obstruction     threatening     therapy with agents
                                                                                such as infliximab
Ulcerative colitis     Autoimmune         Diarrhea,             Mild to life-   Steroids, anti-
                                          hematochezia, ileus   threatening     inflammatory agents
C difficle colitis     C difficle toxin   Diarrhea              Mild to life-   Antibacterials:
                                                                threatening     metronidazole,
                                                                                vancomycin
Viral colitis, e.g.,   Specific viral     Diarrhea              Mild to life-   Antivirals: ganciclovir,
CMV                    infection                                threatening     foscarnet
Data Issues with Current Coding
 As indexed, ICD-9-CM currently classifies
  mucositis with localized inflammation.
      Mucositis – see also Inflammation by site
  necroticans agranulocytica 288.0
 Classifying mucositis with all other types of
  inflammation is non-specific.
 Codes for generalized inflammation are too
  broad and non-descriptive to allow distinct
  identification of mucositis for retrospective
  disease identification and data analysis.
Precedent for Specific Code
   The ICD-9-CM code for stomatitis (528.0) currently
   excludes more specific diseases of the oral mucosa.



                                        Stomatitis (528.0)


               Acute
             Necrotizing   Aphthous         Gangrenous        Herpetic        Oral
             Ulcerative    Stomatitis        Stomatitis      Stomatitis     Mucositis
             Stomatitis


ICD-9-CM                                                                  Separate Code
                 101         528.2             528.1           054.2        Essential
      Code

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Mucositis oral

  • 1. Coding for Mucositis ICD-9-CM Coordination and Maintenance Committee Meeting Patrick J. Stiff, MD Loyola University Medical Center September 30, 2005
  • 2. What is Mucositis?  Mucositis is inflammation of the mucosal surfaces throughout the body.  It typically involves redness and ulcerative sores in the soft tissues of the mucosa.  Oral mucositis manifests as erythema, inflammation, ulceration, and hemorrhage in the mouth and throat. Image from: Spielberger, Ricardo; KepivanceTM: A Breakthrough for Oral Mucositis Associated with Myeloablative Hematopoietic Stem Cell Transplantation; City of Hope National Medical Center, Department of Hematology and Bone Marrow Transplantation
  • 3. Causality  Mucositis is a frequent complication of anticancer treatment, including chemotherapy and radiation therapy.  Due to high-dose chemotherapy and/or radiation preconditioning, it is particularly common in patients preparing for bone marrow transplantation (BMT). Source: Bellm LA, Epstein JB, Rose-Ped A, Martin P, Fuchs HJ. Patient reports of complications of bone marrow transplantation; Support Care Cancer. 2000 Jan;8(1):33-9
  • 4. Mechanisms  Characterized by damage to the epithelium of the oropharyngeal cavity and GI tract  Rapidly dividing basal cells of the oral mucosa are among the body cells vulnerable to damage by chemotherapy and radiation therapies.* Phases of Mucositis** *National Institutes of Health Symptom Research: http://symptomresearch.nih.gov/Chapter_17/sec7/cghs7pg1.htm **Adapted from Sonis. Nat Rev Cancer. 2004;4:277-284.
  • 5. Sites of Mucositis  While the oral mucosa is the most frequent site of mucosal toxicity, mucositis also is common along the entire alimentary tract: – Esophagus – duodenum – colon – Stomach – ileum/jejunum – rectum  GI mucositis occurs via a mechanism similar to that in the oral mucosa, only the damage to the mucosal layer is more aggressive than in oral mucositis.  Although less common, treatment of ovarian and nasopharyngeal cancer also can result in vaginal and nasal mucositis. Source: http://symptomresearch.nih.gov/Chapter_17/sec2/cghs2pg1.htm
  • 6. Severity Scales  Many validated instruments have been developed to stage and measure mucositis severity. – National Cancer Institute’s Common Toxicity Criteria (NCI CTC) – World Health Organization’s (WHO’s) Oral Toxicity Scale (OTS)
  • 7. WHO’s Oral Toxicity Scale World Health Organization’s Oral Toxicity Scale Severe Mucositis Grade 1 Grade 2 Grade 3 Grade 4 Ulcers with Mucositis Erythema, extensive to the extent ulcers; Soreness erythema; that patient can ± erythema patient alimentation swallow cannot is not solid food swallow food possible
  • 8. Epidemiology  40% of patients receiving standard-dose chemotherapy  80% of patients receiving radiation therapy for head and neck cancer  75% of all patients undergoing BMT Mucositis Frequency: BMT with Mucositis Frequency: Mucositis Frequency: Radiation High-Dose Chemotherapy and/or Standard-Dose Chemotherapy for Head and Neck Cancer Radiation Pre-Conditioning 40% 80% 75% 60% 25% 20% Mucositis Reported Mucositis Not Reported Source: Bellm et al. 2000
  • 9. Clinical Consequences  Painful condition that interferes significantly with patient functioning and tolerance for cancer therapy  Functional implications may include: – Inability to tolerate food or fluid intake – Difficulty or inability to talk*  In a recent study, BMT patients ranked mucositis as the most debilitating side effect of their treatment.** *Borbasi S, Cameron K, Quested B, Olver I, To B, Evans E. More than a sore mouth: patients' experience of oral mucositis. Oncol Nurs Forum. 2002;29:1051-1057, and Bellm et al. 2000. **Bellm et al. 2000
  • 10. Clinical Consequences (continued)  Hospital admission or extended hospitalization for total parenteral nutrition, intravenous analgesia, and intravenous antibiotics – 62% of patients require hospitalization.* – 70% of patients with Grade 3-4 oral mucositis require feeding tubes.*  Reduction or cessation (dose-limiting toxicity) of cancer treatment in 35% of patients* *Sonis ST, Elting LS, Keefe D, Peterson DE, Schubert M. Perspectives on cancer therapy-induced mucosal injury. Cancer. 2004;100(9 Suppl):1995-2025
  • 11. Differential Diagnosis and Treatment  Mucositis is distinguishable from other forms of inflammatory disorders in terms of: – causality – clinical presentation/lab findings – severity – treatment  The presence and severity of radiation- or chemotherapy- induced mucositis is routinely documented in clinical progress notes.
  • 12. Inconsistency in Coding ICD-9-CM Codes That Are Sometimes Used to Describe Mucositis Code(s) Limitation(s)  288.0, Agranulocytosis; Agranulocytosis and mucositis are distinct conditions without causal relationship.  V58.0, Encounter or admission for radiotherapy; and  V58.1, Encounter or admission for chemotherapy  528.0, Stomatitis  Stomatitis coding is non-specific and does not permit differentiation based on – NOS etiology (viral, fungal, etc.). – ulcerative  May be used to represent mucosal damage that occurs due to local trauma – Excludes stomatitis: (for example, biting, denture irritation, or localized infection) • acute necrotizing ulcerative  Generally is used to describe conditions less severe and more localized than • aphthous • gangrenous oral mucositis, as well as conditions that may be histologically distinct from oral • herpetic mucositis • Vincent’s  996.85, Complications of  Some physicians are using 996.85 to describe mucositis. transplanted organ, bone  ICD-9-CM code 996.85 is used for all BMT complications, for example, graft- marrow versus-host disease.  530.10, Esophagitis  Mucositis of the stomach, duodenum, and other parts of the alimentary tract is unspecified, and 535.XX, clinically distinct from other types of inflammatory diseases of the GI system Gastritis and duodenitis (for example, esophagitis resulting from gastroesophageal reflux disease).
  • 13. Stomatitis: Non-Descriptive and Outdated  Non-descriptive term  Not representative of current clinical nomenclature  Used to describe conditions that are: – less severe than, – more localized than, and – histologically distinct from oral mucositis
  • 14. Differential Diagnosis: Oral Clinical Presentation/Lab Disease/Injury Causality Findings Severity Treatment Options Oral mucositis Chemotherapy Diffuse redness, Varies; in BMT Palliative rinses, and radiation ulcerations, and pain, setting up to 98% narcotics, palifermin in therapy particularly in areas have Grade 3/4 the BMT setting where teeth abut tissue Aphthous Etiology not Single painful ulcer Localized, but Topical stomatitis identified painful; maximum grade 2 Herpetic HSV1 Usually several spots; Usually grade 1-2 Acyclovir, valacyclovir, mucositis ulcerative foscarnet Oral thrush Candida Varies from painless to Usually grade 0-1 Nystatin rinses; fluconazole mild soreness; whitish and other azoles plaques Denture/oral Dentures Common in elderly patients Can limit calories Repair, removal of trauma with loose-fitting dentures dentures Gangrenous Bacterial infections Necrotic Rare, can be Antibacterials that treat oral stomatitis pseudomembranes severe aerobes and anaerobes Acute necrotizing Bacterial infections Pain, fever, necrotic, Grade 3/4 Control of infection stomatitis in immune deficient bloody ulcers patients
  • 15. Differential Diagnosis: Oral Local, Denture-Related Lesion Oral Thrush Aphthous Ulcer Oral Mucositis*  Because these conditions can coexist in immunocompromised patients, differential diagnosis is critical. * Spielberger, Ricardo; KepivanceTM: A Breakthrough for Oral Mucositis Associated with Myeloablative Hematopoietic Stem Cell Transplantation; City of Hope National Medical Center, Department of Hematology and Bone Marrow Transplantation
  • 16. Differential Diagnosis: GI Clinical Presentation/ Disease/Injury Causality Lab Findings Severity Treatment Options Mucositis of Chemotherapy Typhilitis, Mild to life- Supportive care, the GI tract and radiation diarrhea, ileus, threatening treatment of bowel obstruction infectious complications Crohn’s disease Autoimmune Diarrhea, pain, Mild to life- Steroids, antibody bowel obstruction threatening therapy with agents such as infliximab Ulcerative colitis Autoimmune Diarrhea, Mild to life- Steroids, anti- hematochezia, ileus threatening inflammatory agents C difficle colitis C difficle toxin Diarrhea Mild to life- Antibacterials: threatening metronidazole, vancomycin Viral colitis, e.g., Specific viral Diarrhea Mild to life- Antivirals: ganciclovir, CMV infection threatening foscarnet
  • 17. Data Issues with Current Coding  As indexed, ICD-9-CM currently classifies mucositis with localized inflammation. Mucositis – see also Inflammation by site necroticans agranulocytica 288.0  Classifying mucositis with all other types of inflammation is non-specific.  Codes for generalized inflammation are too broad and non-descriptive to allow distinct identification of mucositis for retrospective disease identification and data analysis.
  • 18. Precedent for Specific Code The ICD-9-CM code for stomatitis (528.0) currently excludes more specific diseases of the oral mucosa. Stomatitis (528.0) Acute Necrotizing Aphthous Gangrenous Herpetic Oral Ulcerative Stomatitis Stomatitis Stomatitis Mucositis Stomatitis ICD-9-CM Separate Code 101 528.2 528.1 054.2 Essential Code