Primary care pediatricians who care for children with medical complexity may be unsure of their role in helping these families make difficult decisions. Pediatricians may find this particularly challenging if the decision is related to an issue managed by a subspecialty provider.
A 15-year-old girl with medical complexity including spastic quadriparesis related to extremely premature birth, seizure disorder, chronic lung disease requiring supplemental oxygen at night, and neurologic dysphagia with gastrostomy tube dependence presents for clinical care. She has neuromuscular scoliosis for which she has regularly seen an orthopedic surgeon for follow-up care. She has been using a back brace for the past several years. However, her curve has continued to progress and was at a 73-degree curve at her most recent visit with the orthopedic surgeon. The surgeon is recommending a posterior spinal fusion. Her parents are unsure of what to do. What advice would you give them?
Families of children with medical complexity (CMC) face many types of health-related decisions, each of which can be difficult for different reasons. In this article, we explore the types of decisions faced by families, challenges to decision-making, influences on decision-making, models of decision-making, and the role of the primary care pediatrician in helping families make complex decisions.
Here are some examples of complex care decisions:
- Whether to enroll in a 3-week-long intensive physical therapy course, which would require one parent taking time off work and a significant out-of-pocket expense, but may result in meaningful functional gains and a plan for continuing progress at home
- Whether to start a new medication(such as an antiepileptic) due to new symptoms, side effects of existing medications, or loss of symptom control by established medicines
- Whether to insert a gastrostomy tube due to declining oral intake or weight loss or concerns about oral feeding aspiration
- Whether to undergo spinal fusion for progressive scoliosis, for concerns about pulmonary health or seating comfort
- Whether to undergo tracheostomy for mounting respiratory failure despite noninvasive ventilation
- Whether to have a do not resuscitate order
These decisions vary (Table 1) in magnitude of consequences, degree of risk, time scale, degree of tradeoffs, degree of uncertainty, degree of linkage with other decisions, and reversibility.
Decisions Faced by Families of Children with Medical Complexity
Challenges to Decision-Making for CMC
In addition to the decision attributes detailed above, there are other aspects of decision-making for CMC that can make the process challenging.
CMC not only have multiple medical conditions, but their conditions are often rare or not well understood. This can lead to uncertainty regarding the child's response to interventions, their long-term outcomes, and potential complications.
Parents of CMC experience stressors and pressures beyond what a typical family experiences and have an increased complexity of information to process. These factors can make it difficult for parents to focus on and to analyze relevant information for a decision.
Significant health care decisions may need to be made on an unanticipated time frame, or during a state of illness or deterioration. There may be little applicable evidence to guide a decision. Consequences and trade-offs may be subjective and value-laden.
The physician who is most knowledgeable about a proposed therapy or intervention for CMC may have limited understanding of the other medical issues affecting the child. They may not have a long-term relationship with the child and family and may not understand the family's values.
There are also additional uncertainties in the decision-making process, including questions of how we evaluate the quality of life of others with various health conditions, how CMC affects the family as a whole and whether those effects influence decision-making, and what approach to decision-making is preferred by parents.1 Sometimes it is not even clear which problem should be the focus or how to approach the subsequent decisions that are inevitably linked to each other.2
During the decision-making process, parents may experience decisional conflict. In one study looking at decision-making about tracheostomy placement, parents' decisional conflict was related to feeling as if they had no choice, needing more information, struggling to maintain hope, feeling pressured by the medical team, balancing their needs with the needs of their child, and weighing options without judgement.3
Influences on Decision-Making
Health-related decisions for CMC are influenced by a variety of factors. Ideally evidence will be the first factor considered; however, some decisions faced by families of CMC are particularly challenging due to the paucity of applicable evidence. Many decisions fall into the realm of having not one particular “right” or recommended course of action. These types of decisions are then influenced by other factors.
Hope is one factor. Some think hope is too irrational to play a role in decision-making, but others argue that discussing and identifying hopes can help parents clarify what they care about and what their guiding principles are for making decisions.4 Hopeful thinking serves as a resource to help parents cope with their child's illness.5 Clinicians do not have to choose between being honest with parents about the seriousness of their child's condition and allowing parents to maintain their hope. Parents of children who are seriously ill report wanting information about their child's prognosis even if that information is upsetting,6 and accurate negative information about a child's prognosis does not necessarily lower hope among parents.7
Cultural values are another factor. One study examining parents' decision-making for adolescents using hypothetical scenarios found that parents made more risk-averse decisions for their children than they did for themselves; this is an example of decision-making influenced by a cultural norm to make risk-averse decisions for one's child.8
Parental personal sense of duty is also a factor. Work with parents of children who are seriously ill has shown that decision-making for their child is influenced by the parents' belief of what a “good parent” would do in a situation, and what they perceive to be their duty as a “good parent.”2 Not all parents share the same idea about what makes a “good parent.” It may mean making sure the child feels loved, focusing on the child's health, focusing on the child's quality of life, putting their child's needs ahead of their own, advocating for their child, or making informed medical decisions.9,10 “Good parent” beliefs may evolve, changing over time.11
Models of Shared Decision-Making
Many different models of shared decision-making have been developed and written about. The ANSWER framework has been proposed for answering the question, “Doctor, what would you do?” in the setting of a preference-sensitive or value-laden decision where there is limited evidence.12 This framework involves Active listening, assessing the Needs of the patient or parent asking for advice, having Self-awareness of our own biases, determining Whose values the patient or parent is interested in applying to the decision, Eliciting the patient's and parent's values, and Responding to the patient's or parent's question.
More broadly, collaborative communication, encompassing not only the exchange of information but also a particular focus on the collaborative relationship between participants, has been advocated as a foundation for effective decision-making.13 Participants in collaborative communication must share a desire to establish common goals, exhibit mutual respect, understand differing perspectives, communicate with clarity, and manage intrapersonal and interpersonal influences on sending, receiving, and processing information.
Definitions for shared decision-making vary, but the process can be broadly understood as involving patients, families, and clinicians in the decision-making process through multidirectional information exchange and incorporation of all participants' knowledge and values. Specific strategies including an outline of practical steps, sticking points, and application to different types of decisions have been published.14–16 A systematic review and meta-analysis of pediatric shared decision-making interventions suggests that the process may improve knowledge and decrease decisional conflict.17
Role of the Primary Care Pediatrician
The primary care pediatrician is in a unique position to assist families of CMC with decision-making. Several of the decision-making models above include aspects of building a trusting relationship, understanding and incorporating a family's values, and understanding and managing influences (such as “good parent” beliefs) on decision-making. Primary care pediatricians can establish a trusting relationship over time, and develop understanding of the family's values, beliefs, and preferences during periods of relative health and stability.
A specific “values history” has been suggested as a mechanism for encouraging discussion of important topics and allowing the family to share their values and beliefs with the physician.18,19 One article looked at the feasibility of using a values history to develop advance directives for children with HIV.18 The authors developed a set of open-ended questions focused on burdens and positive aspects of the health condition, identifying important things in life, identifying conditions that would make life no longer worth living, tolerance of pain and risk in hopes of recovery, and reflection on prior experiences with illness.18 Another article proposed that a values history could be used as a routine part of health management beginning soon after meeting the patient and family or after a new diagnosis, with regular updates thereafter.19
The primary care pediatrician will also likely have opportunities to develop skills in collaborative decision-making with lower-risk decisions that have shorter time frames and are reversible, such as switching a medication or referral to a subspecialist. Parents and clinicians have both endorsed the desire for the primary care provider to be involved in all aspects of referral, consultation, and decision-making with another subspecialist.20
Returning to the Illustrative Case: Surgery for Neuromuscular Scoliosis
The parents of the 15-year-old patient with medical complexity and severe spinal scoliosis were uncertain regarding whether to pursue surgery. Based on what we have covered in the article, how can the parents best be helped?
First, there are things that a primary care pediatrician may or may not know about the patient's particular condition and the pros and cons of surgery. Neuromuscular scoliosis tends to be more severe and progressive than idiopathic scoliosis, progressing even after skeletal maturity. Progressive scoliosis can cause pain along with impairments in cardio-pulmonary function, sitting posture (including pelvic obliquity), trunk stability, use of hands and arms, and activities of daily living.21–23 Nonoperative management includes observation, bracing, and seating modifications.21,23 Surgery has significant risks and high complication rates, including prolonged pain, infection, skin breakdown, aspiration and other pulmonary complications, and the potential for significant blood loss.21–23 Limited data show quality of life to be improved after surgery.24
Reviewing these complicated issues, primary care pediatricians might wonder what guidance they can offer. Although the patient's primary care pediatrician has likely cared for other patients with neuromuscular scoliosis and is probably familiar with some of the data surrounding complications and outcomes, we are not suggesting that the primary care pediatrician should be the primary source of this data. Specific information about the procedure itself, including risks, benefits, and alternatives should be provided by the surgeon who is recommending the procedure.
Instead, the girl's primary care pediatrician can be more helpful by assisting the family in applying the data they have been given to their specific situation, helping them clearly define their values and goals for their daughter and their family, and partnering with them to use those values and goals to develop guiding principles for this decision and others.
Spinal fusion surgery for neuromuscular scoliosis is not a straightforward decision. Accordingly, attempts have been made to help families with the complex nature of this decision, including a decision aid with unbiased information, and parent-to-parent advice on what to include in the decision-making conversations.25,26
In this case, the primary care pediatrician knows that for the past year or so the patient had been expressing discomfort (using vocalizations and facial expressions) after sitting in her wheelchair for less than 1 hour. Her posture worsened (even while wearing her brace) to the point where she does not fit well in her wheelchair despite adjustments. The patient's speech therapist at school feels that her poor sitting posture is a main barrier to her being able to effectively learn to use a communication device. The natural course of neuromuscular scoliosis is such that it will not improve on its own and will probably continue to progress. These factors point to reduced pain and improved seating posture as relevant potential benefits from the surgery.
The primary care pediatrician also knows and will be able to make sure the family realizes that their daughter's multiple medical conditions, including chronic lung disease and seizure disorder, put her at increased risk for complications.
Once the potential benefits and risks pertinent to the patient's situation are discussed, the primary care pediatrician can further help the family clarify their values and goals as they relate to this decision. The family has always placed a priority on their daughter's comfort and happiness, and in response to the primary care pediatrician's inquiry they affirm those priorities. The patient is social, enjoys going to school and spending time with peers and siblings, as well as attending sporting and music performances. Because of her discomforts and inability to stay in a wheelchair for extended periods, she has not been able to participate in activities with family and friends.
In this case, the patient's short-term comfort and ability to go to school and to go on family outings will be negatively affected by surgery. She will likely have a hospital stay of approximately 1 week if things go well, longer if there are complications. She may continue to have postoperative discomfort for weeks, and her mobility will be diminished during the recovery period. However, if surgery does result in anticipated benefits of decreased chronic pain, improved sitting posture, and ability to comfortably sit in her wheelchair longer, then the patient's long-term comfort and enjoyment will be improved.
By framing the risks and benefits in terms of the family's values and goals, and helping them balance tradeoffs based on their priorities, the primary care pediatrician is able to help the family arrive at a decision with which they feel comfortable.
Families of CMC face many difficult health care decisions. Primary care pediatricians are able to play a strong role in supporting families in decision-making through building long-term relationships, helping families clarify their values and goals, and helping families apply those values to the decision at hand.
- Feudtner C. What we don't know about how we decide. Virtual Mentor. 2010;12(7):586–589. PMID:23182209
- Feudtner C, Schall T, Hill D. Parental personal sense of duty as a foundation of pediatric medical decision-making. Pediatrics. 2018;142(suppl 3):S133–S141. doi:10.1542/peds.2018-0516C [CrossRef] PMID:30385619
- October TW, Jones AH, Greenlick Michals H, Hebert LM, Jiang J, Wang J. Parental conflict, regret, and short-term impact on quality of life in tracheostomy decision-making. Pediatr Crit Care Med. 2020;21(2):136–142. doi:10.1097/PCC.0000000000002109 [CrossRef]. PMID:31568244
- Feudtner C. The breadth of hopes. N Engl J Med. 2009;361(24):2306–2307. doi:10.1056/NEJMp0906516 [CrossRef] PMID:20007559
- Reder EA, Serwint JR. Until the last breath: exploring the concept of hope for parents and health care professionals during a child's serious illness. Arch Pediatr Adolesc Med. 2009;163(7):653–657. doi:10.1001/archpediatrics.2009.87 [CrossRef] PMID:19581549
- Mack JW, Wolfe J, Grier HE, Cleary PD, Weeks JC. Communication about prognosis between parents and physicians of children with cancer: parent preferences and the impact of prognostic information. J Clin Oncol. 2006;24(33):5265–5270. doi:10.1200/JCO.2006.06.5326 [CrossRef] PMID:17114660
- Mack JW, Wolfe J, Cook EF, Grier HE, Cleary PD, Weeks JC. Hope and prognostic disclosure. J Clin Oncol. 2007;25(35):5636–5642. doi:10.1200/JCO.2007.12.6110 [CrossRef] PMID:18065734
- Dore RA, Stone ER, Buchanan CM. A social values analysis of parental decision making. J Psychol. 2014;148(4):477–504. doi:10.1080/00223980.2013.808603 [CrossRef] PMID:24946390
- October TW, Fisher KR, Feudtner C, Hinds PS. The parent perspective: “being a good parent” when making critical decisions in the PICU. Pediatr Crit Care Med. 2014;15(4):291–298. doi:10.1097/PCC.0000000000000076 [CrossRef] PMID:24583502
- Feudtner C, Walter JK, Faerber JA, et al. Good-parent beliefs of parents of seriously ill children. JAMA Pediatr. 2015;169(1):39–47. doi:10.1001/jamapediatrics.2014.2341 [CrossRef] PMID:25419676
- Hill DL, Faerber JA, Li Y, et al. Changes over time in good-parent beliefs among parents of children with serious illness: a two-year cohort study. J Pain Symptom Manage. 2019;58(2):190–197. doi:10.1016/j.jpainsymman.2019.04.018 [CrossRef] PMID:31026508
- Tucker Edmonds B, Torke AM, Helft P, Wocial LD. Doctor, what would you do? An ANSWER for patients requesting advice about value-laden decisions. Pediatrics. 2015;136(4):740–745. doi:10.1542/peds.2015-1808 [CrossRef] PMID:26416929
- Feudtner C. Collaborative communication in pediatric palliative care: a foundation for problem-solving and decision-making. Pediatr Clin North Am. 2007;54(5):583–607. doi:10.1016/j.pcl.2007.07.008 [CrossRef] PMID:17933613
- Adams RC, Levy SECouncil on Children with Disabilities. AAP Council on Children with Disabilities. Shared decision-making and children with disabilities: pathways to consensus. Pediatrics. 2017;139(6):e20170956. doi:10.1542/peds.2017-0956 [CrossRef] PMID:28562298
- Walter JK, Hwang J, Fiks AG. Pragmatic strategies for shared decision-making. Pediatrics. 2018;142(suppl 3):S157–S162. doi:10.1542/peds.2018-0516F [CrossRef] PMID:30385622
- Opel DJ. A 4-step framework for shared decision-making in pediatrics. Pediatrics. 2018;142(suppl 3):S149–S156. doi:10.1542/peds.2018-0516E [CrossRef] PMID:30385621
- Wyatt KD, List B, Brinkman WB, et al. Shared decision making in pediatrics: a systematic review and meta-analysis. Acad Pediatr. 2015;15(6):573–583. doi:10.1016/j.acap.2015.03.011 [CrossRef] PMID:25983006
- Wissow LS, Hutton N, Kass N. Preliminary study of a values-history advance directive interview in a pediatric HIV clinic. J Clin Ethics. 2001;12(2):161–172. PMID:11642069
- Sahler OJ. Comment: getting the most value out of a values history. J Clin Ethics. 2001;12(2):173–175. PMID:11642070
- Stille CJ, Fischer SH, La Pelle N, Dworetzky B, Mazor KM, Cooley WC. Parent partnerships in communication and decision making about subspecialty referrals for children with special needs. Acad Pediatr. 2013;13(2):122–132. doi:10.1016/j.acap.2012.12.003 [CrossRef] PMID:23356961
- Allam AM, Schwabe AL. Neuromuscular scoliosis. PM R. 2013;5(11):957–963. doi:10.1016/j.pmrj.2013.05.015 [CrossRef] PMID:24247014
- Halawi MJ, Lark RK, Fitch RD. Neuro-muscular scoliosis: current concepts. Orthopedics. 2015;38(6):e452–e456. doi:10.3928/01477447-20150603-50 [CrossRef] PMID:26091215
- Roberts SB, Tsirikos AI. Factors influencing the evaluation and management of neuromuscular scoliosis: a review of the literature. J Back Musculoskeletal Rehabil. 2016;29(4):613–623. doi:10.3233/BMR-160675 [CrossRef] PMID:26966821
- Obid P, Bevot A, Goll A, Leichtle C, Wülker N, Niemeyer T. Quality of life after surgery for neuromuscular scoliosis. Orthop Rev (Pavia). 2013;5(1):e1. doi:10.4081/or.2013.e1 [CrossRef] PMID:23705059
- Shirley E, Bejarano C, Clay C, Fuzzell L, Leonard S, Wysocki T. Helping families make difficult choices: creation and implementation of a decision aid for neuromuscular scoliosis surgery. J Pediatr Orthop. 2015;35(8):831–837. PMID:25551784
- Garrity B, Berry J, Crofton C, et al. Parent-to-parent advice on considering spinal fusion in children with neuromuscular scoliosis. J Pediatr. 2019;213:149–154. doi:10.1016/j.jpeds.2019.05.055 [CrossRef] PMID:31253410
Decisions Faced by Families of Children with Medical Complexity
||Intensive PT course
|Magnitude of consequences
|Risk of harm
|Time scale of decision impact
||Days to weeks
||Days to weeks
||Months to years
|Magnitude of trade-offs
|Degree of uncertainty of outcome
|Degree of linkage to other decisions
||Can stop early