A 7-month-old male with severe hemophilia A (less than 1% factor VIII [FVIII] activity) presented to his pediatrician with fussiness and inability to sleep for 3 days. He had received his influenza vaccine 3 days earlier. His mother attributed the fussiness to introducing lentils into his diet.

The infant was born to a 32-year-old primigravida mother via cesarean delivery after a normal pregnancy. Severe hemophilia A was diagnosed due to bleeding after a circumcision done at birth. There were no subsequent bleeding episodes and he was not on FVIII replacement therapy.

The infant’s review of systems was positive for atopic dermatitis, intermittent constipation, mild gastroesophageal reflux, and co-sleeping. His growth and development were normal. He was not on medications and his immunizations were up-to-date, including his influenza vaccine 3 days prior to this presentation. There was no history of fever, cough, cold, vomiting, diarrhea, or trauma. The child was treated initially with reflux precautions, dietary advice, and observation but the fussiness worsened. He continued to feed well and smiled when held but started crying when laid down. He slept only in his mother’s arms and awakened screaming at night.

The mother called the hematologist for worsening fussiness and was advised to take the patient to the emergency department (ED). The Hematology service was consulted in the ED. The patient’s vital signs and physical exam were normal. Abdominal imaging (X-ray and abdominal ultrasound [US]) showed no evidence of constipation or intussusception.

The patient saw the pediatrician for a follow-up visit. He was afebrile, fussy, and tired appearing with normal vital signs and a normal physical exam other than crying when laid on the examination table that stopped when he was picked up. Rapid tests for respiratory syncytial virus (RSV) and influenza were negative. He was evaluated at the Hemophilia Clinic the next day when he had a complete blood count (CBC) and a head computed tomography (CT), which were normal.

He followed up again with the pediatrician. His mother reported continuing fussiness, increased flatulence, and pasty stools with straining and crying. On examination, the patient appeared uncomfortable and curled up when laid down. A repeat abdominal X-ray was normal. He was put on an elemental formula and advised polyethylene glycol 1 teaspoon per day in water. The family was advised to return to the ED if the fussiness continued.

The patient returned to the pediatrician the next day for worsening fussiness with a preference to curl up and turn to his left. Hospital admission with hematologic consultation was requested due to the concern for a hematologic, possibly intra-abdominal cause of his fussiness. During the hospital stay, a second abdominal US, a CT scan of his abdomen and pelvis, urinalysis, and blood tests including CBC, lipase, amylase, comprehensive metabolic panel, and C-reactive protein (CRP) were normal.

The patient was noted to be intermittently fussy but had normal vital signs and continued to feed well. He was noted to arch his back during episodes of fussiness. He showed a preference to turn his head to the left. Neck exam showed no asymmetry or evidence of muscle bleed. He did not awaken to an abdominal exam when asleep. The occupational therapist recommended gentle neck exercises for his neck stiffness that was thought to be related to his preferred sleep position in the parents’ arms for several days. His arching was attributed to gastroesophageal reflux. After 2 days, he was discharged.

Fussiness in infants is a common pediatric outpatient complaint and has many causes that range from trivial to life threatening.

 Both hematologic and nonhematologic causes of fussiness were considered in the differential diagnosis for this infant with hemophilia (Table 1). At the onset of fussiness, the patient appeared well, and nonorganic causes were considered such as his recent influenza vaccination, recent introduction of a new food, co-sleeping with parents, frequent awakening at night, and the onset of teething. His exam was negative for thrush, corneal abrasion, or musculoskeletal trauma. Infections appeared unlikely due to the lack of fever, cold symptoms, ear pain, or rash. Because of his history of mild gastrointestinal reflux, intermittent constipation, and fussiness, he was treated with reflux precautions, polyethylene glycol, and a trial of elemental formula.

The patient was simultaneously evaluated for signs and symptoms of hemophilia (Table 2). He demonstrated no obvious bruising, tenderness, swelling, or deformity. Additionally, he had no hemarthroses. He did exhibit worrisome signs such as refusal to lay on his back, turning to 1 side, and curving forward. Hence the child was evaluated for intracranial and intra-abdominal hemorrhage, which were ruled out based on clinical exam, laboratory evaluation, and imaging studies.

During the follow-up with his pediatrician on the day after discharge from the hospital, the patient’s mother reported worsening fussiness with “his hands numb from pain.” On examination, he had torticollis with a head tilt to the left and a partial ptosis of his left eyelid. Tone and spontaneous movements of his trunk and upper extremities were decreased and he reached for a toy with his feet.

Unable to sit, he curved his spine forward when placed in a seated position. He was unable to lift himself up from the prone position. Grasp reflex was absent, and he appeared to have no sensation in his palms. The pediatrician advised the parent to return to the ED and called the hematologist about the new neurologic findings. The child was intubated in the ED and an imaging study confirmed the diagnosis.

The patient was diagnosed with spinal epidural hematoma (SEH) with cord compression presenting with Horner syndrome.

Magnetic resonance imaging (MRI) of the patient’s spine showed multiple epidural hematomas extending from C2 down to S1 with significant cord compression. This was especially pronounced from C6 to T4 where the spinal cord was severely compressed and displaced anteriorly and to the left. Multiple septations suggested that the hematomas could be subacute or chronic. (Figures 1 and 2 present preoperative T1- and T2-weighted sagittal and axial images of the spine.)

Factor VIII replacement was given immediately after the MRI and perioperatively per standard guidelines for hemophilia.

 He underwent multiple partial hemilaminectomies for decompression of epidural hematomas. His neurologic exam improved the next day with increased spontaneous movements of his upper extremities.

One week postoperatively, another spinal MRI showed significant improvement (Figure 3). The patient was found to have a low titer inhibitor (0.8 BU) during the admission that self-resolved on follow-up testing. Postoperatively, further bleeding complications were successfully prevented with recombinant FVIII product, 200 units/kg/dose given every 6 hours that was gradually spaced out. He was discharged after 3 weeks with residual right-sided hemiparesis and left-sided ptosis.

Three months later, a central line was placed to facilitate the administration of the recombinant FVIII product every other day. Six months later, he transitioned to an extended half-life (EHL) FVIII product (50 units/kg/day every 4 days). He received physical and occupational therapy with steady improvement and was enrolled in Early Intervention services for children.

Hemophilia, an X-linked bleeding disorder, is the most common hereditary bleeding disorder in the United States. Its incidence is estimated to be approximately 1 in 5000 live male births.

 Hemophilia A, the condition affecting this patient, results from deficiency in FVIII. Central nervous system bleeding in patients with hemophilia is a rare complication with an incidence between 2% and 8%.

 Fewer than 10% of these hemorrhages occur within the spinal canal.

This case highlights the importance of recognizing infancy as a high-risk age group for the occurrence of SEH. Infants may be predisposed to epidural bleeding as their cervical musculature begins to develop between ages 4 and 6 months.

 Increased cervical motion in the developing muscle body (due to acceleration and deceleration of the head mass in babies with poor head control) leads to cervical spine injury

Spontaneous SEH can be either venous or arterial in origin. In venous SEH, absence of valves in the venous plexus allows for venous backflow and sudden increases in pressure (from an infant’s crying, straining, coughing, or sneezing), inducing spontaneous rupture of these fragile vessels.

 In arterial SEH, it is hypothesized that the higher pressure of arterial bleeding could result in cord compression with a rapid onset in patients who previously performed strenuous activities without incident.

 Young infants with hemophilia also are at higher risk for hemorrhage because they typically do not begin prophylactic factor replacement until after age 1 year or following their first joint or muscle bleed once ambulatory.

Babies with SEH have nonspecific symptoms such as unexplained irritability, inconsolable crying, poor feeding, or refusal to bear weight

 The classic clinical triad without history of trauma, making it difficult to recognize.

 The classic clinical triad of SEH—radicular pain, severe localized spinal pain, and sensorimotor deficits—may initially be difficult to perceive in a nonverbal infant. However, a diligent physical exam demonstrating specific neurologic signs such as decreased neck range of motion, diminished arm reflexes, or decreased strength, tone, and sensation in the upper extremities

Review of literature revealed 2 previous cases describing torticollis as a presentation of SEH in hemophilia A.

 Similar to this case, a 10-month-old infant with a cervicothoracic epidural hematoma presented initially with irritability and a normal neurologic exam but developed torticollis approximately 48 hours later that progressed to diffuse hypotonia, flaccid upper extremity paralysis, and respiratory distress requiring emergent decompressive laminectomy.

 Another 10-month-old boy developed torticollis 48 hours after an incident of repeated neck overextension and went on to develop flaccid upper extremity paralysis.

 There are no previous cases of SEH in hemophilia presenting with Horner syndrome. However, a similar presentation was found in a 72-year-old woman receiving anticoagulant therapy for atrial fibrillation.

Early diagnosis and treatment of SEH in hemophilia patients is necessary to prevent progression of neurologic deficits leading to long-term sequelae. Magnetic resonance imaging is the diagnostic modality of choice for SEH. However, spinal ultrasonography is a reasonable screening tool should MRI not be readily available.

 Empirical factor replacement, even prior to imaging, is the initial therapy in cases of suspected central nervous system bleeding and is dosed to achieve factor levels of 100%. The role of surgical intervention is less clear. Prompt surgical decompression with or without laminectomy should be considered.

 However, surgery may be deferred in the presence of a stable neurologic exam with successful correction of the underlying coagulopathy.

Kiehna and colleagues reviewed 24 cases of spontaneous epidural hemorrhage in children with hemophilia and found that prompt conservative treatment with FVIII and correction of coagulopathies was critical in patient outcomes. Aggressive medical management without surgery provided for full neurologic recovery in hemodynamically and neurologically stable patients.

 Importantly, these authors and other proponents of conservative management (under the appropriate circumstances) recommend a multidisciplinary approach to the treatment of these patients with a team consisting of a pediatric hematologist, neurosurgeon, and pediatric intensivist.

A high index of suspicion for spinal hemorrhage and recognition of subtle neurologic signs of cord compression, which can be difficult in a nonverbal and nonambulatory child, are essential in order to obtain the correct imaging and make the diagnosis in a timely way.

Horner syndrome or oculosympathetic paresis is a classic neurologic syndrome whose signs include ptosis, miosis, and anhydrosis due to a lesion along the sympathetic pathway that supplies the head, eye, and neck. Children with new onset Horner syndrome must be evaluated for an underlying disorder and paraspinal masses must be considered as an etiology.

 The history of severe hemophilia with new onset torticollis, Horner syndrome, and upper extremity weakness suggested cord compression from cervical spinal hemorrhage in this patient.

The patient at age 6 years has no neurologic deficits and his general health is very good. His cognition and speech are excellent. He has had no further bleeding episodes. He recently transitioned from EHL FVIII product to a new prophylactic agent called emicizumab given subcutaneously once a week.

1. Arumugam J, Sivandam A, Vijayalakshmi A. The evaluation and management of an incessantly crying infant. 

. 2012;41(4):192-198. doi: 10.4038/sljch.v41i4.4985

2. Srivastava A, Brewer AK, Mauser-Bunschoten EP, et al; Treatment Guidelines Working Group on Behalf of the World Federation of Hemophilia. Guidelines for the management of hemophilia. 

3. Soucie JM, Evatt B, Jackson D. Occurrence of hemophilia in the United States. The Hemophilia Surveillance System Project Investigators. 

4. Eyster ME, Gill FM, Blatt PM, Hilgartner MW, Ballard JO, Kinney TR.Central nervous system bleeding in hemophiliacs. 

5. de Tezanos Pinto M, Fernandez J, Perez Bianco PR. Update of 156 episodes of central nervous system bleeding in hemophiliacs. 

6. Kiehna EN, Waldron PE, Jane JA. Conservative management of an acute spontaneous holocord epidural hemorrhage in a hemophiliac infant. 

7. Lee JS, Yu CY, Huang KC, Lin HW, Huang CC, Chen HH. Spontaneous spinal epidural hematoma in a 4-month-old infant. 

8. Beatty RM, Winston KR. Spontaneous cervical epidural hematoma. A consideration of etiology. 

9. Jackson FE. Spontaneous spinal epidural hematoma coincident with whooping cough. Case Report

10. Caldarelli M, Di Rocco C, La Marca F. Spontaneous spinal epidural hematoma in toddlers: description of two cases and review of the literature. 

11. Cuvelier GD, Davis JH, Purves EC, Wu JK. Torticollis as a sign of cervico-thoracic epidural haematoma in an infant with severe haemophilia A. 

12. Oymak Y, Muminoglu N, Ay Y, et al. Torticollis in a haemophilic infant with inhibitor: a case of spinal epidural haematoma. 

13. Morelli N, Mancuso M, Guidetti D. Spinal epidural hematoma onset with Horner syndrome. 

14. Brown T, Purves E, Sargent M, Wu J. Clinical application of spinal ultrasound in screening for spinal haematoma in haemophilia A infants. 

15. Rois PV, López MR, de Vergara BC, de la Lama Zaragoza A, García JG, Uxo JM. Spinal epidural hematoma in hemophilic children: controversies in management

 2009;25(8):987-991; discussion 993, 995.

16. Borkar SA, Prasad GL, Satyarthee GD, Mahapatra AK. Spontaneous spinal extradural hematoma in a child with hemophilia B, surgery or medical management—a dilemma? 

17. Barrea C, Vigouroux T, Karam J, Milet A, Vaessen S, Misson JP. Horner syndrome in children: a clinical condition with serious underlying disease. 

Articles

A 7-month-old male with severe hemophilia A (less than 1% factor VIII [FVIII] activity) presented to his pediatrician with fussiness and inability to sleep for 3 days. He had received his influenza vaccine 3 days earlier. What's the diagnosis?

Epidural bleeding in an infant with hemophilia

Warm greetings! It is hard to believe that summer is almost upon us.

Over the last 4 months, the spreading COVID-19 pandemic has made us rethink how we practice medicine in order to continue to provide outstanding comprehensive care to all our patients. This is especially important for our patients aged younger than 2 years for whom it is critically important that they receive their recommended vaccines on time and for medically complex patients who require close follow-up for the management of their conditions.

The pandemic has challenged us to be creative in the ways we evaluate patients and provide reassurance to their parents. In this issue of 

COVID-19: A battle plan for pediatricians

—This article highlights some strategies that pediatric practitioners can incorporate into their practices to safely and effectively provide care to their patients.

CDC issues preliminary information on MIS-C

—Multisystem Inflammatory Syndrome in Children (MIS-C) associated with COVID-19 was first described in the United Kingdom in late April 2020 and has since been reported in Spain, Italy, France, and the United States. This is a serious condition with some symptoms that overlap those seen in Kawasaki disease and/or toxic shock syndrome. Preliminary data indicate that many individuals who develop this syndrome have either COVID-19 infection or a history of exposure and that MIS-C may be a postinfectious inflammatory process. This article presents the CDC case definition of MIS-C and the need to report MIS-C to local, state, or territorial health departments.

Updated consensus guidelines issued for unexplained pediatric death

—There is limited medical awareness of SUDC, which is the sudden and unexpected death of a child aged 12 months or older that remains unexplained after a thorough investigation including autopsy. This document provides comprehensive guidance for the pediatric practitioner on the investigation of the event and the important role that they play in serving as liaisons to help families maneuver through the processes that occur after the death.

Chronic care management: Increase the value of your practice

—Fifteen to 25% of children in the United States have one or more chronic health conditions that require some level of chronic care management. This is an important Practice Improvement article that provides the information to properly code for the management of children with multiple chronic conditions and ensure proper reimbursement.

As we continue to work through the COVID-19 pandemic, remember that:

“No matter how hard things may seem, don’t get down and don’t give up. The clouds will clear and there are brighter days ahead.” —Anonymous

Please stay safe and well. I welcome your suggestions, comments, and questions.

The great unknown caused by COVID-19 has led to many questions and few answers. The one certainty is that pediatrics will have to change in order to ensure proper care.


Pediatrics’ new paradigm for practice in the era of COVID-19

Previously, the perceived quality of health care had declined and had opened the door for a value-based care model. The “value” principle is derived from measuring health quality and outcomes against the cost of delivering the care. Quality of care is a composite of traditional treatment outcomes that includes patient quality of life, safety, prevention of emergency department (ED) visits, and hospital admissions.

In acknowledgment of the value of chronic disease management and the impact that it has on health care expenses and outcomes, the Centers for Medicare and Medicaid Services (CMS) had compromised to reimburse for chronic care management (CCM) services on a regular basis.

The Final Rule of the 2015 CMS Physician Fee Schedule included the new Current Procedural Terminology (CPT 99490) and is defined as (Table 1).

This unique physician fee schedule service was created to pay separately for non-face-to-face care coordination services provided to patients with multiple chronic conditions.

In early 2017, CMS revised the care planning element for Complex CCM adding CPT codes 99487 and 99489. Both are available in the cases that require the establishment and implementation of a 

 comprehensive care plan. In the same month, providers may bill for either CCM (99490) or Complex CCM (99487/99489) according to individual patient needs. Payment fee schedule of the above codes is primarily intended to reimburse for clinical staff time spent on CCM services, rather than physician time.

Effective January 1, 2020, CMS created an add-on code as well for Non-Complex CCM, Healthcare Common Procedure Coding System G2058, with the intent to incentivize private practitioners to embrace this type of management program.

Care coordination has been defined as “the deliberate integration of patient care activities between 2 or more participants involved in a patient’s care to facilitate the appropriate delivery of health care services.”

Tertiary medical centers have developed Pediatric Care Coordination or Complex Care Programs that provide individualized CCM where all medical decisions are family centered. These programs are practically unachievable without a strong primary care system.

Pediatric CCM programs are relatively inexpensive to operate compared with the overall cost of patient care. The most recent Florida Department of Health (DOH) data, where I do practice, revealed that from the 4.1 million children that live in our state, only 2% (~80,000) meet complexity criteria yet they utilize one-third of our pediatric annual budget (Figure).

 A similar expenditure percentage was published by Cohen and colleagues on a large retrospective study in Ontario, Canada.

Traditional vs nontraditional health care outcomes

A CMS evaluation contractor, Mathematica, reported substantial expenditure reduction in acute care utilization of $74 per-beneficiary-per-month (PBPM) during an 18-month period surveillance on adults.

 In contradiction, recent evidence in 2 randomized trials on adult complex “super-utilizer” populations that were enrolled on an “intensive” outpatient monitoring registered no decline on readmission rates. However, an overwhelming “positive” patient experience—96% of the survey participants—was reported in one of the studies.

 No well-controlled studies have been published in pediatric literature addressing treatment CCM outcomes despite that this subset of children accounts for approximately 25% of the total hospital days.

Pediatric CCM programs focus as well on nontraditional quality-of-care markers such as family “fulfilling” experience and patient functionally. In very complex cases, implementing the “integrative” palliative care (IPC) mindset model of “being with” instead of “doing to” seems congruent.

 It is expected that our complex care pediatric practice will benefit as payment models move away from fee-for-service (FFS) to a risk-based model adjusted to each patient “hierarchical” condition category (HCC).

The current Medicare Physician Fee Schedule (MPFS) conversion factor is $36.0391 per relative value unit. Applying the national average MPFS values (actual payment varies by geographic location), payment for CCM services ranges from $42.17 for code 99490 to $92.98 for code 99487 (Table 2).

 A noteworthy example is that if 100 patients are enrolled to CCM services, an additional amount of $42,000 a year will be generated. Per CMS rules, billing practitioners cannot submit code 99491 in the same calendar month as CPT codes 99487, 99489, or 99490.

Understanding the regulatory elements is key to implementing an effective plan. All CCMs require compliance with the following (Table 3)

To be enrolled in a CCM program. the child must meet the following required elements:

1. Multiple (2 or more) chronic conditions expected to last at least 12 months, or until the death of the patient.

2. Chronic conditions place the patient at significant risk of death, acute exacerbation/decompensation, or functional decline.

3. Needs a comprehensive care plan that requires moderate or high-complexity medical decision making (MDM).

The CMS maintains a Chronic Condition Warehouse with 27 chronic condition-defined categories, the great majority of them are compiled from adult morbidity data. No such statistics exist for the child-aged population. However, it is not an exclusive list to qualify for the CCM pediatric program.

In the event of an audit, documentation of a beneficiary consent is crucial. Oral consent to enroll is acceptable until a personal explanative encounter is set, when the parent and/or patient should sign the appropriate written consent. Once the initial visit is completed, a comprehensive person-centered plan for CCM will be developed and shared with family and patient.

ELIGIBLE PRACTITIONERS AND CLINICAL STAFF

The CPT manual defines a “clinical staff member” as “a person who works under the supervision of a physician or other qualified health care professional and who is allowed by law, regulation, and facility policy to perform or assist in the performance of a specified professional service, but who does not individually report that professional service.” There are no specific credentialing requirements for staff personnel. They could be directly employed by the clinician or a contracted third party. The American Medical Association (AMA) has developed an online training module “Implementing Health Coaching” for physicians who want to incorporate health coaches into their practices.

The “incidence to” billings rules applies, and the clinician is not required to be on the premises providing direct supervision. The provider must have accessibility 24 hours a day.

Any non–face-to-face care management and coordination service provided on behalf of an enrolled beneficiary by a provider or clinical staff member counts.

The following are acceptable documentation categories:

Telephone calls/messages and emails with the family or patient.

Referring to and consulting with subspecialists.

Post-hospitalization or emergency department (ED) follow-up contact.

Home care plan elaboration, revision, and review with family and/or patient.

Our institution’s electronic medical record (EMR) team helped us to develop a Patient Outreach Navigator tool that enormously facilitates our staff to calculate the time spent and create an electronic descriptive report. In addition, we had used this instrument for Transition of Care Management (TCM) billing CPT codes 99495 and 99496 after hospital admissions or ED visits.

The following 3 requirements are necessary to submit a claim for CCM:

3. Ensuring beneficiary access to the care plan.

Our EMR offers worldwide interoperability allowing patients to view and share their electronic chart with any clinician worldwide using their mobile Internet devices (smartphone or tablet).

Our EMR Comprehensive Plan of Care includes:

1. A problem list with descriptive overview.

2. Planned interventions to address patient chronic health conditions.

3. Measurable goals and a time frame to achieve the goals.

4. Beneficiary’s agreement with the goals and plan.

State Medicaid agencies and Medicaid Managed Care Organizations (MCOs) have the option of providing CCM as part of an enhanced benefit package. In our experience, CCM reimbursement on patients with commercial insurance is a challenge and it would be advisable to involve your institution’s billing department or, in rare cases, seek for advocacy from the American Academy of Pediatrics (AAP) coding resources.

The CMS had recognized that CCM is a critical component of primary care that promotes outstanding quality service, improves patient satisfaction, and reduces overall health care costs. For all these reasons, a distinctive non-face-to-face physician fee service was created to incentivize practitioners to adopt this type of management programs.

An effective CCM scope entails a timely and comprehensive exchange of the patient plan of care among the team. It is key to understanding the regulatory elements to implement an effective billing plan. Confidently, with the emergence of new clinician EMR navigator tools designed to capture and share information, pediatric outcome data will be available in the near future. At the end, fee-for-value will be accepted as the best model for lowering health care costs while increasing quality care resulting in healthier patients.

CPT 2019 (Current Procedural Terminology), Professional Edition

. Chicago, IL: American Medical Association: 2019.

2. Centers for Medicare and Medicaid Services (CMS). Chronic Care Management Services. Published July 2019. Accessed April 9, 2020.

 https://www.cms.gov/Outreach-and-Education/Medicare-Learning-Network-MLN/MLNProducts/Downloads/ChronicCareManagement.pdf

3. Bodenheimer T. Coordinating care—a perilous journey through the health care system. 

. 2008;358(10):1064-1071. doi:10.1056/nejmhpr0706165

4. Florida Health. The future of CMS. Statewide leadership meeting. December 9, 2017. Accessed April 9, 2020. 

http://www.floridahealth.gov/programs-and-services/childrens-health/cms-plan/cms-plan-invitation-to-negotiate/_documents/the-future-of-cms.pdf#search=%22keisha%20reid%22

5. Cohen E, Berry JG, Camacho X, Anderson G, Wodchis W, Guttmann A. Patterns and costs of health care use of children with medical complexity. 

. 2012;130(6):e1463-e1470. doi:10.1542/peds.2012-0175

6. Schurrer J, O’Malley A, Wilson C, McCall N, Jain N. 

Evaluation of the Diffusion and Impact of the Chronic Care Management (CCM) Services: Final Report

. Mathematica Policy Research; 2017. Accessed April 9, 2020.

 https://innovation.cms.gov/files/reports/chronic-care-mngmt-finalevalrpt.pdf

7. Finkelstein A, Zhou A, Taubman S, Doyle J. Health care hotspotting—a randomized, controlled trial. 

. 2020;382(2):152-162. doi:10.1056/NEJMsa1906848

8. Zulman DM, Pal Chee C, Ezeji-Okoye SC, et al. Effect of an intensive outpatient program to augment primary care for high-need Veterans Affairs patients: a randomized clinical trial. 

 2017;177(2):166-175. doi:10.1001/jamainternmed.2016.8021

9. Bergman DA, Keller D, Kuo DZ, et al. Costs and use for children with medical complexity in a care management program. Pediatrics. 2020;145(4):e20192401. doi: 10.1542/peds.2019-2401.

10. Milstein J. A paradigm of integrative care: healing with curing throughout life, “being with” and “doing to.” 

. 2005;25(9):563-568. doi:10.1038/sj.jp.7211358

11. Centers for Medicare and Medicaid Services (CMS). Revisions to payment policies under the Medicare Physician Fee Schedule, Quality Payment Program, and other revisions to Part B for CY 2020. CMS.gov website. Published November 15, 2019. Accessed April 9, 2020.

 https://www.cms.gov/Medicare/Medicare-Fee-for-Service-Payment/PhysicianFeeSched/PFS-Federal-Regulation-Notices-Items/CMS-1715-F

12. Simon TD, Cawthon ML, Popalisky J, Mangione-Smith R; Center of Excellence on Quality of Care Measures for Children with Complex Needs (COE4CCN). Development and validation of the pediatric medical complexity algorithm (PMCA) Version 2.0. 

. 2017;7(7):373-377. doi:10.1542/hpeds.2016-0173

13. Centers for Medicare and Medicaid Services. Chronic Conditions Data Warehouse (CCW). Condition categories. Accessed April 9, 2020. 

https://www2.ccwdata.org/web/guest/condition-categories

As primary care providers, pediatricians often manage children with multiple chronic conditions. Knowing how to code properly for chronic care management (CCM) of these patients will ensure proper reimbursement for CCM services.

Chronic care management: Increase the value of your practice!

EDITOR’S NOTE: Dr. Erin Bowen herself experienced the sudden unexpected death of a child—her 17-month-old son Conor—in 2016. In her family’s search for answers and support for their grief, she turned to the SUDC Foundation and found a mission. Click 

The death of a child is devastating for everyone involved, including families and medical professionals alike. It defies the natural order of life that we expect. When the death is sudden and unexpected, families are overwhelmed with trauma, shock, and grief as the medicolegal death investigation begins.

Sudden unexplained death in childhood (SUDC) is the sudden and unexpected death of a child aged 12 months or older that remains unexplained after a thorough investigation including autopsy. According to the Centers for Disease Control and Prevention (CDC), SUDC affects approximately 400 children aged 1 to 18 years annually.

The death investigation system in our country is variable, comprising coroner and medical examiner systems in most areas. It is faced with challenges that include a lack of resources, a shortage of forensic pathologists, and, until recently, lack of procedural guidance, particularly for pediatric deaths. The lack of standardization for death investigation has far-reaching implications, including the resultant effects on the family as well as public health ramifications. Without standardization, accurate surveillance of these deaths is elusive.

Education regarding the death investigation system is limited in our pediatric training curriculum and, consequently, pediatricians may have difficulty providing appropriate guidance to families in the aftermath of these deaths. In sudden unexpected deaths of children aged 12 months and older, there is the additional challenge of minimal awareness of this category of death.

Although SUDC is the fifth-leading category of death in children aged 1 to 4 years, there is no targeted federal funding to support research. The SUDC Foundation (

) is the only organization whose mission is to promote awareness, advocate for research, and support those affected by the sudden loss of a child.

In recognition of the need for consistency in approaching sudden pediatric deaths, the SUDC Foundation funded a grant for a collaboration between the National Association of Medical Examiners (NAME) and the American Academy of Pediatrics (AAP). In January 2020, this expert panel of more than 30 multidisciplinary contributors from across the country published the first national consensus guidelines for sudden unexplained pediatric deaths: 

Unexplained Pediatric Deaths: Investigation, Certification and Family Needs

, the publication discusses the evolution of our understanding and practice in the area of pediatric death investigation; outlines procedural guidance for death investigation, autopsy, and ancillary testing as well as certification and reporting of these deaths; and promotes consistent classification of deaths in order to understand how often they occur.

There are also recommendations for prevention strategies and future research. Guidelines are provided for working with family members and other professional team members. The cornerstone of these guidelines is the provision of comprehensive and compassionate care, recognizing that the two are not mutually exclusive, but rather intimately connected for thorough investigations dedicated to high-quality care of survivors.

Currently, SUDC is unpredictable and unpreventable. For some families, the lack of explanation for their child’s death can be crippling. Although finding a cause does not mitigate their grief, in some cases it can allow them to better understand the death of their child and the implications for other family members. Pursuing appropriate medical screening may elucidate a cause for some of these deaths. However, we know that studies into the causes of sudden unexplained deaths in children aged older than 12 months are limited.

 showed an increased rate of febrile seizures in these children. This is one area where future research could be helpful to better identify at-risk children. Similarly, the grief responses and needs of families after the death of older children have not been studied as extensively as infant death. With more research into these areas, we can build upon these guidelines to provide improved, data-driven approaches to the care for families affected by SUDC.

Although we may not be able to provide answers for all these deaths, we can ensure that we adequately support families after these tragedies and connect them with the appropriate resources. It is important to remember that your role as the child’s pediatrician does not end with their death.

Pediatric deaths are, thankfully, relatively rare. Consequently, pediatricians may feel ill equipped to manage the needs of bereaved families, particularly when these deaths are sudden and unexpected. Creating protocols that incorporate these new recommendations in conjunction with all the agencies and professionals involved can minimize confusion, prevent further trauma, and ensure optimal care for families during an already overwhelming and traumatic time.

1. National Association of Medical Examiner’s Panel on Sudden Unexpected Death in Pediatrics; Bundock E, Corey T, eds. 

. Academic Forensic Pathology International; 2019. 

2. Baker AM, Crandall L. To hold or not to hold. 

. 2009;5(4):321-323. doi: 10.1007/s12024-009-9123-7

New consensus guidelines clarify the procedural guidance for investigation, certification, and reporting of sudden unexplained pediatric deaths to help medical professionals and families through these crises.

Vol 37 No 6

Epidural bleeding in an infant with hemophilia

Pediatrics’ new paradigm for practice in the era of COVID-19

Chronic care management: Increase the value of your practice!

Groundbreaking guidelines issued for unexplained pediatric death