Improved Outcomes in Congenital Insensitivity to Pain with Anhidrosis (CIPA) via a Multidisciplinary Clinic Model
Abstract
1. Introduction
2. Methods
2.1. Study Design
2.2. Study Population
2.3. Pediatric Multidisciplinary Clinic for Congenital Insensitivity to Pain
2.4. Pre- and Post-MDC Visits and Hospitalizations
2.5. Surgical Interventions
2.6. Infections
2.7. Data Collection
2.8. Statistical Analysis
3. Results
3.1. Hospitalization Episodes and Days
3.2. Day Hospitalization Clinic and MDC Visits
3.3. Surgical Interventions
3.4. Infections
4. Discussion
4.1. Clinical Interpretation
4.2. Comparison to Other MDC Models and Common Challenges
4.3. Implementation of the MDC for CIPA Patients
4.4. Study Limitations
5. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
Abbreviations
CIPA | Congenital insensitivity to pain with anhidrosis |
HSAN | Hereditary sensory and autonomic neuropathy |
NGF | Nerve growth factor |
OM | Osteomyelitis |
SUMC | Soroka University Medical Center |
MDC | Multidisciplinary clinic |
CRP | C-reactive protein |
RR | Rate ratio |
CI | Confidence interval |
SD | Standard deviation |
SPSS | Statistical Package for the Social Sciences |
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Type of Intervention | Details |
---|---|
Proactive approach | Every two weeks—MDC |
Scheduled appointments | |
Cohorting | Carriage status: sampling for antibiotic-resistant bacteria (AB-R 1; MRSA 2, ESBL 3, VRE 4) |
Separate areas for “clean” and “dirty/AB-R carriage” | |
Separate staff for “clean” and “dirty/AB-R carriage” | |
Avoidance of calling immunocompromised patients in regard to “CIPA day” | |
Infection control | Use of gown in “dirty” area |
Use of gloves in “dirty” area | |
Strict adherence to hand hygiene guidelines | |
Staff | Orthopedic surgeon—routine |
Infectious disease specialists—routine | |
Nurse (dedicated)—routine | |
Additional staff—1 or 2 pediatrics residents | |
Per need: ophthalmologist, oral and maxillofacial surgeon, plastic surgeon, psychiatrist; social work | |
Physical examination | Vital signs |
Mandatory full examination, including undressing, removal of all bandages, observation of wounds/lacerations/abscesses | |
Cultures | Review of previous results |
Active wounds/lacerations/abscesses, including superficial and deep tissue | |
Blood cultures if febrile/toxic/hypotension/hypothermia | |
Imaging | As indicated |
Laboratory | Review of previous results |
CRP for active cases/surveillance | |
Other lab exams as needed, such as blood count, electrolytes, ferritin, vitamin D levels, etc. | |
Decisions | Antibiotic treatment |
Hospitalization | |
Next MDC–CIPA appointment | |
Need for surgical interventions | |
Other therapeutics (zinc level, vitamin A, vitamin D, iron, etc.) |
Pre-MDC (n/Days); 2014–2017; n = 38,790 Days | Post-MDC (n/Days); 2018–2024; n = 95,340 Days | |
---|---|---|
Hospitalization range (days) | 0–275 | 0–527 |
Hospitalization mean ± SD (days) | 37.9 ± 63.9 | 63.3 ± 96.9 |
Clinic visits range (days) | 0–96 | 0–116 |
Clinic visits mean ± SD (days) | 16.8 ± 25.0 | 23.5 ± 24.6 |
Surgical interventions range (days) | 0–13 | 0–25 |
Surgical interventions mean ± SD (days) | 1.8 ± 3.5 | 4.4 ± 5.4 |
Infection episodes range (days) | 0–40 | 0–123 |
Infection episodes mean ± SD (days) | 4.4 ± 8.1 | 16.4 ± 29.3 |
Surgical interventions (overall) | 107 | 257 |
Orthopedic surgeries—incision and drainage | 75 | 160 |
Orthopedic surgeries—amputation | 5 | 20 |
Eyes—corneal ulcer repair | 16 | 15 |
Mouth and jaw—tooth extraction | 8 | 51 |
Mouth and jaw—internal fixation of pathologic fracture | 1 | 0 |
Plastic surgery—amputation of fingers, debridement | 2 | 9 |
Other surgery (general, gastro) | 0 | 2 |
Infections—overall | 257 | 963 |
Staphylococcus aureus | 90 | 244 |
Enterobacteriaceae * | 39 | 183 |
Enterococcus spp. | 17 | 49 |
Pseudomonas spp. | 43 | 129 |
Episodes per 1000 Days (Pre-MDC) | Episodes per 1000 Days (Post-MDC) | Rate Ratio (RR) (95% CI) | p Value | |
---|---|---|---|---|
Hospitalization—total | 57.7 | 40.0 | 0.693 (0.6586–0.7291) | <0.001 |
Clinic visits—total | 25.5 | 14.6 | 0.5708 (0.5266–0.6188) | <0.001 |
Surgery—total | 2.8 | 2.7 | 0.9772 (0.7802–1.2240) | 0.84 |
Surgery—orthopedics | 2.1 | 1.9 | 0.9154 (0.7037–1.1901) | 0.51 |
Surgery—eyes | 0.4 | 0.16 | 0.3814 (0.1886–0.7714) | 0.007 |
Surgery—mouth and Jaw | 0.23 | 0.53 | 2.3055 (1.1352–4.6826) | 0.02 |
Surgery—plastic surgery | 0.05 | 0.09 | 1.83 (0.3956–8.4735) | 0.77 |
Infections—overall | 6.6 | 10.1 | 1.52 (1.33–1.75) | <0.001 |
Staphylococcus aureus | 2.3 | 2.6 | 1.10 (0.87–1.40) | 0.80 |
Enterobacteriaceae * | 1.0 | 1.9 | 1.91 (1.35–2.70) | <0.001 |
Enterococcus spp. | 0.4 | 0.5 | 1.17 (0.68–2.04) | 0.57 |
Pseudomonas spp. | 1.1 | 1.4 | 1.22 (0.86–1.72) | 0.26 |
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Shmueli, M.; Ling, G.; Elamour, S.; Weisel, Y.; Ben-Shimol, S. Improved Outcomes in Congenital Insensitivity to Pain with Anhidrosis (CIPA) via a Multidisciplinary Clinic Model. J. Clin. Med. 2025, 14, 5258. https://doi.org/10.3390/jcm14155258
Shmueli M, Ling G, Elamour S, Weisel Y, Ben-Shimol S. Improved Outcomes in Congenital Insensitivity to Pain with Anhidrosis (CIPA) via a Multidisciplinary Clinic Model. Journal of Clinical Medicine. 2025; 14(15):5258. https://doi.org/10.3390/jcm14155258
Chicago/Turabian StyleShmueli, Moshe, Galina Ling, Siham Elamour, Yaron Weisel, and Shalom Ben-Shimol. 2025. "Improved Outcomes in Congenital Insensitivity to Pain with Anhidrosis (CIPA) via a Multidisciplinary Clinic Model" Journal of Clinical Medicine 14, no. 15: 5258. https://doi.org/10.3390/jcm14155258
APA StyleShmueli, M., Ling, G., Elamour, S., Weisel, Y., & Ben-Shimol, S. (2025). Improved Outcomes in Congenital Insensitivity to Pain with Anhidrosis (CIPA) via a Multidisciplinary Clinic Model. Journal of Clinical Medicine, 14(15), 5258. https://doi.org/10.3390/jcm14155258