Table 2.
PHIS hospital data for Krabbe use, 10/2013 −12/2020. Data is from 37 children’s hospitals. Abbreviations: DRG, diagnosis related group; GERD, gastroesophageal reflux disease; ICU, intensive care unit; KD, Krabbe disease. Full results in Supplemental Data 1.
|
Number of KD visits total (mean, median, range) |
736 (20, 9, 1–83) | |
|
Number of KD patients total (mean, median, range) |
98 (3, 2, 1–26) | |
| Visit type/characteristics | N (%) | |
| Inpatient | 260 (35%) | |
| Emergency department | 154 (21%) | |
| Ambulatory surgery | 46 (6%) | |
| Observation unit | 46 (6%) | |
| Clinic visit | 189 (26%) | |
| Other | 41 (6%) | |
| In Hospital Death | 7 (7%) | |
| ICU stay | 32 (33%) | |
| of those in ICU, Days | 21.2 days (1–134) | |
| Diagnosis * | Visits N (%) | Patients N (%) |
| Epilepsy; convulsions | 220 (30%) | 33 (34%) |
| Esophageal disorders | 104 (14%) | 39 (40%) |
| Fluid and electrolyte disorders | 91 (12%) | 45 (46%) |
| Dysphagia | 88 (12%) | 32 (33%) |
| Other specified and unspecified gastrointestinal disorders | 80 (11%) | 34 (35%) |
| Procedures ** | Visits N (%) | Patients N (%) |
| Administration of nutritional and electrolytic substances | 75 (10%) | 39 (40%) |
| Gastrostomy | 47 (6%) | 42 (43%) |
| Mechanical ventilation | 40 (5%) | 15 (15%) |
| Venous and arterial catheter placement | 37 (5%) | 30 (31%) |
| Lumbar puncture | 36 (5%) | 33 (34%) |
| Most common medications ^ | Visits N (%) | Patients N (%) |
| Levetiracetam | 120 (16%) | 36 (37%) |
| Albuterol | 115 (16%) | 23 (23%) |
| Propofol | 114 (15%) | 61 (62%) |
| Baclofen | 107 (15%) | 39 (40%) |
| Gabapentin | 101 (14%) | 39 (40%) |
| Other Conditions | Visits N (%) | Patients N (%) |
| Gastrostomy | 285 (39%) | 58 (59%) |
| Feeding difficulties | 130 (18%) | 46 (47%) |
| Epilepsy | 220 (30%) | 33 (34%) |
| GERD | 103 (14%) | 39 (40%) |
| Failure to thrive | 72 (10%) | 44 (45%) |
| Developmental Delays | 103 (18%) | 39 (40%) |
| Tracheostomy | 44 (6%) | 2 (2%) |
| Visits N (%) | Patients N (%) | |
| MRI, brain | 31 (4%) | 21 (21%) |
| CT, brain | 80 (11%) | 58 (59%) |
| Ultrasound, brain | 10 (1%) | 10 (10%) |
| EEG | 99 (13%) | 49 (50%) |
| EKG | 70 (10%) | 41 (42%) |
| Nebulizer treatment | 107 (15%) | 25 (26%) |
| Top DRGs for Inpatient, Observation or Emergency Department Visits (98 patients, 460 visits) | ||
| Visits N (%) | Patients N Visits (%) | |
| DRG - Description | ||
| Seizure | 50 (11%) | 20 (20%) |
| Degen nerv syst dis X MS | 48 (10%) | 35 (36%) |
| Upper resp tract infect | 30 (7%) | 16 (16%) |
| Malnut/fail thrive/oth | 22 (5%) | 17 (17%) |
| Digestive system dx NEC | 22 (5%) | 14 (14%) |
*
Top five most common diagnoses. Excluding KD, Leukodystrophy, Lack of expected normal physiological development, Other disorders of psychological development, Other sphingolipidoses.
**
Top five most common procedures using CCSR ICD procedure coding groupings from HCUP AHRQ.
^
Top five most common medications, excluding acetaminophen and sodium chloride.