Comprehensive review and screening for complications in adults with SCD
| I. Identify a primary care physician to coordinate total patient care |
| II. Comprehensive multisystem review to evaluate complications |
| Pain: days off work because of pain |
| i. Acute pain: frequency of hospital admissions or ER or Infusion Center visits, frequency of pain at home |
| ii. Chronic pain: including use of opiate analgesia |
| iii. Individualized pain plan for acute and chronic pain |
| Medical history/details of comorbidities |
| i. Sickle related: |
| 1. Renal dysfunction (proteinuria, hematuria) |
| 2. Cardiorespiratory symptoms |
| 3. Neurological: any memory concerns |
| 4. Leg ulcers |
| 5. Visual: ophthalmologic symptoms and previous review |
| 6. Priapism |
| 7. History of thrombosis and anticoagulant therapy |
| ii. Nonsickle related: diabetes, hypertension, gout |
| Medication: consider HU if applicable, vaccinations |
| Transfusion history (to include frequency, transfusion reaction) |
| Vital signs (blood pressure, pulse oximetry, weight) |
| Baseline laboratory testing (complete blood count, biochemistry, hemolysis panel, liver panel, Hb electrophoresis and HbF percentage, iron studies to include ferritin and iron saturation, vitamin D, urinalysis) |
| Investigation: ECHO, pulmonary function, sleep study |
| III. Evaluate for evidence of organ dysfunction* |
| Proteinuria with or without hematuria → renal consult |
| Cardiorespiratory symptoms → ECHO, TRV ≥ 2.5 ms → 6MWD, and NT-proBNP → cardiopulmonary consult |
| Liver function, evidence of intrahepatic cholestasis → hepatology consult |
| Avascular necrosis → orthopedic consult |
| Headaches, cognitive decline → neurology consult/neuropsychology assessment |
| Visual symptoms → yearly ophthalmology review |
| Daytime or nocturnal hypoxia → sleep/respiratory consult |
| Stuttering priapism or acute priapic episodes → urology consult |
| IV. Emotional/psychological review and whether support needed for education/work |
| V. Reproductive review |
| VI. Discussion of treatment options and potential new therapies |
| VII. Management of other comorbidities, if applicable |
| I. Identify a primary care physician to coordinate total patient care |
| II. Comprehensive multisystem review to evaluate complications |
| Pain: days off work because of pain |
| i. Acute pain: frequency of hospital admissions or ER or Infusion Center visits, frequency of pain at home |
| ii. Chronic pain: including use of opiate analgesia |
| iii. Individualized pain plan for acute and chronic pain |
| Medical history/details of comorbidities |
| i. Sickle related: |
| 1. Renal dysfunction (proteinuria, hematuria) |
| 2. Cardiorespiratory symptoms |
| 3. Neurological: any memory concerns |
| 4. Leg ulcers |
| 5. Visual: ophthalmologic symptoms and previous review |
| 6. Priapism |
| 7. History of thrombosis and anticoagulant therapy |
| ii. Nonsickle related: diabetes, hypertension, gout |
| Medication: consider HU if applicable, vaccinations |
| Transfusion history (to include frequency, transfusion reaction) |
| Vital signs (blood pressure, pulse oximetry, weight) |
| Baseline laboratory testing (complete blood count, biochemistry, hemolysis panel, liver panel, Hb electrophoresis and HbF percentage, iron studies to include ferritin and iron saturation, vitamin D, urinalysis) |
| Investigation: ECHO, pulmonary function, sleep study |
| III. Evaluate for evidence of organ dysfunction* |
| Proteinuria with or without hematuria → renal consult |
| Cardiorespiratory symptoms → ECHO, TRV ≥ 2.5 ms → 6MWD, and NT-proBNP → cardiopulmonary consult |
| Liver function, evidence of intrahepatic cholestasis → hepatology consult |
| Avascular necrosis → orthopedic consult |
| Headaches, cognitive decline → neurology consult/neuropsychology assessment |
| Visual symptoms → yearly ophthalmology review |
| Daytime or nocturnal hypoxia → sleep/respiratory consult |
| Stuttering priapism or acute priapic episodes → urology consult |
| IV. Emotional/psychological review and whether support needed for education/work |
| V. Reproductive review |
| VI. Discussion of treatment options and potential new therapies |
| VII. Management of other comorbidities, if applicable |
ECHO, echocardiogram; Hb, hemoglobin; 6MWD, 6-minute walk distance; NT-ProBNP, N-terminal pro b-type natriuretic peptide; TRV, tricuspid regurgitant jet velocity.
If specialist review is recommended, referral should be made to a specialist with an interest/expertise in SCD or to a joint hematology/specialist clinic where these are available.