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Hematology & Thromboembolism – Hemoglobinopathies & Red Cell Disorders Fellowship

Fellowship Program at McMaster

Welcome to the Hemoglobinopathies & Red Cell Disorders Fellowship program at McMaster University.

Welcome

Welcome to the Hemoglobinopathies & Red Cell Disorders Fellowship at McMaster University. McMaster is a leader in the laboratory diagnosis and clinical care of patients with sickle cell disease, thalassemia and other inherited red blood cell disorders.

Fellows joining this program will have an opportunity to learn from internationally renowned clinicians who are deeply engaged in medical education, research, health policy and advocacy. The goal of the fellowship training program in Hemoglobinopathies & Red Cell Disorders is to develop the next generation of experts and leaders in this rapidly expanding area of Hematology.

By the end of training, the fellow will be able to:

  1. Effectively diagnose and treat patients with Hemoglobinopathies and Red Cell Disorders, including complex, multisystem acute and chronic complications.
  2. Provide patient-centred counseling and expert management of targeted disease modifying therapy, including hydroxyurea, transfusions, chelation therapy, and to provide patient education about novel and emerging therapies.
  3. Take proactive measures in a structured, systematic way to prevent the development of chronic complications of red cell disorders.
  4. Act as an expert resource and leader in diagnosis and clinical care of Hemoglobinopathies and Red Cell Disorders in their chosen practice setting.

Certification Outcome

The successful completion of the fellowship will grant a McMaster Fellowship Certification.

Entry Requirements

Trainees must be certified in Internal Medicine and Hematology, or be eligible to receive such certification during Fellowship training, to be accepted into the program.

Recent graduates are required to submit an application form and comply with McMaster University Postgraduate Medical

Application Deadline

The application deadline is November 1st for the training period beginning July 1st of the subsequent year.

Length of Training

This is a 1-year fellowship program.

The fellowship can be extended up to 2 years if combined with significant research and/or graduate degree.

Goals of Training

The Hemoglobinopathies & Red Cell Disorders (H&RCD) objectives include:

  1. Medical Expert

As Medical Experts, physicians integrate all of the CanMEDS Roles, applying medical knowledge, clinical skills, and professional attitudes in their provision of patient-centered care. Medical Expert is the central physician Role in the CanMEDS framework.

H&RCD Diagnosis

  • Understand the core pathophysiologic principles responsible for hemoglobinopathies
    • Understand normal and disordered hematopoiesis including changes related to age
    • Understand normal and disordered red cell structure and function
  • Understanding of the natural history of disease, across the spectrum of hemoglobinopathy diagnoses, both with and without medical intervention
  • Develop a robust understanding of the clinical evaluation to establish an H&RCD diagnosis
  • Expert understanding of the essential components of history and physical examinations requisite to establish a confident diagnosis
  • Understand and apply current Sickle Cell and Thalassemia diagnostic guidelines
  • Understand the role and utility of the investigations required to evaluate hemoglobinopathies
  • Appropriately order, perform, and interpret the results from:
    • Laboratory evaluation
      • Molecular analysis including PCR, restriction endonuclease mapping,
      • Hemoglobin separation methods including electrophoresis, HPLC, IEF, capillary electrophoresis
      • Sickle solubility test
      • Hemoglobin H prep
      • Morphology and interpretation of peripheral blood smears
  • Apply and integrate the findings of investigations and procedures in clinical decision making taking into account clinical presentation, natural history, comorbidities and prognostic assessment
  • Understand the skills and personnel required to coordinate care for H&RCD in multi-disciplinary discussions
  • Genetic counseling for patients and their family members

H&RCD Management

  • Develop and coordinate comprehensive treatment strategy focusing on both pharmacologic and non-pharmacologic intervention
  • Understand and apply current sickle cell and thalassemia treatment guidelines
  • Demonstrate proficient and appropriate use of up-to-date therapeutic options in patients with hemoglobinopathies and other red cell disorders
    • Erythroid stimulating factors and maturation agents (eg luspatercept), hydroxyurea, voxeletor, crizanlizumab, pyruvate kinase activators, etc.
    • Develop a firm understanding of the indication, mechanism of action, expected treatment response, and associated adverse events associated with the therapies utilized in the management of hemoglobinopathies, including prophylactic therapies
    • Develop firm understanding of the presentation and management of acute exacerbations of sickle cell disease managed in both an outpatient and inpatient setting
    • Vasoocclusive crises, Acute chest crisis, Splenic or hepatic sequestration, Aplastic crisis, Stroke, Priapism
  • Understand the physiologic changes and management of sickle cell disease and thalassemia in pregnancy and post-partum
  • Appropriately recognize the indications for referral for Stem Cell Transplant evaluation
  • Appropriately recognize the indications for referral for genetic therapy evaluation
  • Understand the pathophysiology of iron metabolism and overload, chelation including monitoring therapy, side effects, and relevant patient counseling
  • Develop firm understanding of the presentation and management of chronic complications of red cell disorders managed in both an outpatient and inpatient setting
    • Sickle Cell Disease: Retinopathy, pulmonary hypertension, functional asplenia, nephropathy, leg ulcers
    • Thalassemia: complications of chronic transfusion including chelators and their side effects, extamedullary hematopoiesis,
    • Membranopathies and hemolytic disorders: thromboembolic risk, pigmented gallstones
  • Recognize the key comorbidities associated with hemoglobinopathies and develop a firm understanding their appropriate management and when to refer for subspecialist expertise
  • Understand and apply the principles of transfusion medicine in thalassemia and sickle cell disease
    • Role of red blood cell phenotyping and phenotype-matched transfusion
    • Indications and monitoring for chronic simple transfusion in sickle cell disease
    • Role and management of red blood cell exchange transfusion in sickle cell disease
    • Mechanism and principles of management of hyperhemolysis
  • Develop management plan for treatment in collaboration with patient and their family

Hemoglobinopathies and Red Cell Disorders Research objectives

  • Refine critical and scholarly skills in the methodological aspects of applied clinical research
  • Understand the core concepts of evidence-based medicine to critically appraise the hemoglobinopathy and red cell disorder literature
  • Develop skills in the domains of:
    • Research synthesis and design
    • Hypothesis generation
    • Data gathering
    • Knowledge translation
    • Data-analysis and interpretation

Curriculum Highlights

Clinical and Laboratory (1-year: approx. 70-80% of time; 2-year: approx. 45% of time):

  • Develop a thorough understanding of the clinical and laboratory aspects of hemoglobinopathies and red cell disorders including (but not limited to): laboratory and genetic test interpretation, laboratory management, hemoglobinopathy and red cell disorder diagnosis and management, iron metabolism and chelation, and chronic pain management.
  • On average, 3-5 half-day outpatient clinics per week that will include both hemoglobinopathy/Red cell disorder clinics at MUMC and benign hematology clinics at HGH
  • Additional focused subspecialty clinic blocks and electives (e.g. pain clinics, high risk MFM clinic) for approximately 14 weeks per year
  • Regular attendance at hemoglobinopathy team meetings, hematology grand rounds
  • Laboratory assay and management learning sessions (self-led and traditional)

Research (1-year: approx. 10% of time, 2-year: approx. 45% of time):

  • Publish 1 peer reviewed journal article per year and/or present at a hematology conference
  • Present once at Hematology Research in Progress rounds or Grand rounds
  • Submit results of novel research for presentation at Internal Medicine Resident Research Day or an alternative research conference
  • 2-year:
    • Masters degree in health policy or health services research or equivalent
    • Publish 2-3 peer reviewed journal articles and/or present at 2-3 hematology conferences over the 2-year fellowship.

Education (approximately 5-10% of time)

  • Participate in undergraduate and postgraduate education through in-patient and outpatient bedside teaching and didactic teaching sessions.
  • Minimum of 6 presentations per year (e.g. academic half-day for hematology, internal medicine, pediatric hematology-oncology;  internal medicine CTU rounds; SJH hematology rotation learners; Hemoglobinopathy clinic residents)

Supervision & Feedback

Setting expectations: An orientation session will take place when commencing work in each new setting to discuss the goals, description, expectations and evaluation metrics with the trainee. The specific objectives of the trainee will be discussed and an attempt made to integrate these objectives into the overall objectives of the Fellowship.

Supervision and Informal Feedback: Trainees will receive direct supervision in clinical settings, through which they will be provided with feedback concerning their proficiency in managing clinical problems and their performance in informal and formal teaching sessions.

Written assessment forms: In-training Evaluation Reports (ITERs) will be collated by the fellowship supervisor quarterly and discussed with the trainee. Input for these assessments will be sought from all attending Faculty members, as well as members of the team who have worked with the trainee.

Feedback specifically from non-physician healthcare team members, including Hemoglobinopathy clinic interdisciplinary team members and ward staff, will be collected using a Multisource Feedback Form. The H&RBC Fellow must collect at least 2 Multisource Feedback Forms from 2 different people during the fellowship.

The Fellow must collect 3 mini-CEXs during the fellowship.

  1. Counseling a patient on initiation/recent start of hydroxyurea in the ambulatory setting
  2. Physical examination of a hemoglobinopathy patient with iron overload, splenomegaly, and/or SOBOE NYD
  3. Data-gathering (review of records and interview) of a patient regarding new diagnosis of thalassemia

The Fellow must collect a minimum of 4 teaching evaluations.

Meetings with Fellowship Supervisor:  A scheduled assessment session with the trainee will take place every 3 months, as well as at the end of the fellowship. At each meeting there will be two-way feedback on the rotations and discussion of the goals for the remainder of the fellowship. Feedback from faculty members, review of the Multisource Feedback Forms, mini-CEXs, teaching evaluations, and the mid unit and final evaluation sessions will all be used to summarize the trainee’s progress in meeting the goals of this fellowship.

Assessment of Fellowship

At all quarterly meetings with the trainee, feedback regarding the fellowship, including its strengths and shortcomings, is provided by the trainee using the Fellowship evaluation form. If issues arise during the fellowship, the trainee is encouraged to bring these to the attention of the supervisor.

Assessment of Faculty

All faculty require evaluation of their teaching as part of their ongoing University appointment. Not only is it required for the purpose of Promotion and Tenure but also as part of career development.  Trainees will be encouraged to complete evaluations of all faculty with whom they have had significant contact during this rotation.