TABLE 2

Medical, Social, and Education Milestones

Medical milestones: should be assessed annually and should be done before transfer to an adult health care provider; requires some coordination with specialty and primary health care teams.
 1. Ophthalmology evaluation to assess retinopathy and other ophthalmic complications
 2. Dental evaluation to evaluate dental hygiene
 3. Pulmonary function tests to include spirometry and lung volumes
 4. Cardiac evaluation for assessment of elevated tricuspid regurgitant velocity and measurement of brain natriuretic protein to asses pulmonary hypertensiona
 5. Urine albumin/creatinine ratio to assess microalbuminuria and sickle cell disease nephropathy
 6. Magnetic resonance of the head to assess for silent cerebral infarct and, if indicated, cognitive testingb
 7. Level of education achieved (GED, high school, college, etc)
 8. Immunization status
 9. Assessment of partner’s trait status (if applicable)
 10. Assessment of knowledge on safe-sex practices, STDs, and pregnancy
 11. Assessment of ability to make future independent decisions as a young adult; if deemed not likely, then conversations should begin regarding conservatorship or power of attorney by 18 y of age
Social milestones: should be addressed sequentially and require action on the part of the adolescent
 1. Ability to conduct a health care visit with the guardian in the room but with the majority of the interview, assessment, and plan directed toward the patient
 2. Documentation that instructions for medical care, such as taking medication or making a medical appointment, are accomplished initially with parental support and subsequently without parent support
 3. Ability to conduct part or the entire health care visit without the guardian in the room, with assessment and plan directed toward the patient and subsequently validation of the assessment and plan with the guardian who accompanied the minor to the visit
 4. Ability to conduct a health care visit without the guardian at the health care facility, but adolescent must be of legal age
 5. Ability to make appointments and follow through on self-management medical care plan (appointments, prescription refills, etc) without dependence on guardian
 6. Visit to adult health care team office before transition
 7. Ability to seek out and obtain acute medical care independently or with minimum supervision by guardian
 8. Ability to determine health insurance status, before transition, with knowledge about benefits and copays (may require visit to financial administrator)
Education milestones
 1. Document knowledge about the following:
  SCD phenotype
  SCD pain plan including how to take long- and short-acting opioids
  Ability to manage pain according to pain plan
  Preventive measures for SCD complications
   Pain (extreme climate conditions, environmental tobacco smoke, other self-triggers)
   Fever, importance of seeking out prompt medical attention
   Symptoms of stroke
 2. Knowledge of current primary care health team
 3. Documentation that instructions for medical care, such as taking medication or making a medical appointment, are accomplished initially with parental support and subsequently without parent support
 4. Ability to describe current medications, allergies to medications, or medications that have not proven effective for patient’s intended consequence
 5. Ability to articulate most important components of medical history including, but not limited to, surgeries, life-threatening medical complications, and knowledge about challenges with blood transfusion therapy
 6. Ability to seek out medical care with minimum parental supervision
 7. Knowledge of future adult primary health care team
 8. If considering offspring, awareness of partner’s hemoglobinopathy trait status
 9. If partner has been tested for hemoglobinopathy trait testing, counseling to assess risk of offspring having sickle cell disease
  • The milestones in this table should be considered as part of the transition planning that starts at age 12 years for all children with SCD and continued annually until completion of transition to an adult health care provider. The progress with each milestone is independent of age and primarily dependent on ability of the adolescent. Assessments of the milestones are best handled in annual transition visits. GED, general educational development test; STD, sexually transmitted disease.

  • a Clinical utility of tricuspid regurgitant velocity has not been established in pediatrics and if performed should be done in conjunction with adult hematologists or adult primary care provider with a clear plan as to how the results may alter therapy.

  • b Recommendation is specifically for individuals with hemoglobin sickle cell anemia (most severe). Less evidence supports MRI of the head for young adults with hemoglobin sickle cell disease (less severe) or hemoglobin sickle cell beta plus thalassemia, and a case-by-case decision should be made as to whether a MRI of the head should be obtained before transfer to the adult provider.