A 26-year-old male came to the inpatient unit for increasing respiratory distress. He had cystic fibrosis and over the past year had had frequent hospitalizations for respiratory distress, pneumonia and “tune-ups” and his pulmonary function was diminishing rapidly over the time. Over the past week he had been more tired and somnolent. He had an increased oxygen requirement with increased coughing and secretions. The coughing and oxygen make it difficult for him to communicate and he had told his mother that he wanted to come to the hospital now. He was well known to the hospital staff and his parents were always very attentive to him during his hospitalizations. The social history was that he was an only child. His mother was a homemaker and his father was a businessman.
The pertinent physical exam showed a very thin male in moderate respiratory distress. He was breathing deeply 24 times/minute with frequent coughing. His saturation was only 88% on 2 liters of oxygen/minute by nasal cannula and only increased to 90% with 5 liters/minute. A 100% non-rebreather was used increasing his oxygen saturation to 92-94%. He was somnolent but would waken and be appropriate with the staff. He was barrel-chested. His lungs had crackles with diminished breath sounds throughout. His nail-beds were blue with marked clubbing.
The diagnosis of marked respiratory distress in a patient with cystic fibrosis whose pulmonary status had been diminishing was made. His parents asked to talk with the staff outside his room. “He’s dying and we know it. He does too. He didn’t want to come to the hospital until now otherwise we would have been here earlier. The coughing is really bothering him because he wants to talk when he is awake. We’ve never specifically talked about what to do when the time comes, but it’s here. What more can we do?” they asked. The staff and parents discussed options and then went back into the room to discuss them with the patient. He indicated that he wanted help with the secretions and didn’t like the non-rebreather. He also didn’t want any antibiotics started and just wanted to be with his family and the hospital staff. When asked about resuscitation he said, “Ask my mom.” She shook her head to indicate no as did his father. So the team used medication to help with the secretions along with some pulmonary toilet techniques. A nasal cannula was again tried but he then indicated that he wanted the re-breather back. He denied much pain but his mother thought that the coughing was hurting him, so small doses of oral morphine were given. After that he seemed more comfortable and slept well. Over the next 18 hours, he would drift in and out of sleep and would talk briefly. At one point he asked for the nasal cannula back because it was more comfortable. He went to sleep and over time his respiratory rate began to slow and he died peacefully with his family and staff members around him.
Pediatric pallative care (PPC) is care for patients who have life-threatening conditions or need end of life care. The main stakeholders receiving PPC are the patients and all members of the family which may include siblings, grandparents and other members. Other stakeholders are the medical care team including ancillary staff at the hospital and other venues where the patient receives care. PPC is provided by an interdisciplinary team including physicians, nurses, social workers, pharmacists, psychologists, chaplains/faith workers and others who assist families in four main areas: “…relieve suffering, improve quality of life, facilitate informed decision-making and assist in care coordination between clinicians and across sites of care.”
PPC providers can help provide treatment to relieve suffering including management of pain, nausea and emesis, pruritis, agitation, anxiety, depression, delirium, seizures, constipation, sleep disturbances, and dyspnea. Providers can also improve quality of life through frank and open discussions among stakeholders to provide support, relieve family conflict and distress and streamline care that supports the decisions made in a culturally sensitive manner. PPC providers facilitate informed decision-making through frank and compassionate communication regarding treatment options, goals of care, advanced planning, and education of end of life trajectory. Assisting with communication among stakeholders is often key. The providers also assist with care coordination between clinicians and across sites of care, obtaining and coordinating resources so patients have care provided in the most appropriate settings for them. “Often the most therapeutic intervention by the PPC teams is impartial listening”
In a recent paper on barriers to PPC for children the most common barriers included uncertain prognosis (55%), family is not ready to acknowledge the incurable condition (51%), language barriers (47%) and time restraints (47%). Other barriers in the literature include lack of resources for PPC, communication problems, inappropriate eligibility criteria, fragmented care, lack of training and expertise, communication problems, false-hope for cure and inappropriate continuation of life-saving technology. Many families who are offered PPC say that they wish that they had had end of life discussions earlier in the course of the illness. The patient above died in the early 1990’s before many newer treatments were available for cystic fibrosis and many years before PPC became established as a specialty in 2006.
Patients and families have better outcomes when PPC is integrated into the care early as a part of ongoing care particularly as children often receive concurrent disease-directed and palliative care therapy. Two main questions arise frequently: who to refer to PPC and when to refer to PPC. Patients can/should be referred who have life-threatening illnesses but the trajectories of the disease/illness may be different:
- 1. “Life-threatening conditions for which treatment is available but may fail” – irreversible kidney failure or cancer
2. “Conditions where premature death is inevitable but treatment may prolong life” – Duchenne muscular dystrophy or cystic fibrosis
3. “Progressive conditions without curative treatment options” – mucopolysaccaridoses
4. “Irreversible but non-progressive conditions causing severe disability, health complications and risk of premature death” – cerebral palsy or some brain injuries
When to refer patients includes evidence of disease progression, an increase in frequency or duration of hospitalizations or extensive intensive care unit hospitalizations, symptoms that are progressing and not readily managed, and at decision making times about adopting or discontinuing technology.
Questions for Further Discussion
1. What palliative care services are available in your local area?
2. What are the similarities and differences of palliative care consultations and ethics consultations?
- Disease: Cystic Fibrosis | Palliative Care | Hospice Care | End of Life Issues
- Symptom/Presentation: Respiratory Distress
- Specialty: Allergy / Pulmonary Diseases | Palliative Care
- Age: Young Adult
To Learn More
To view pediatric review articles on this topic from the past year check PubMed.
Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.
Information prescriptions for patients can be found at MedlinePlus for these topics: Cystic Fibrosis, Palliative Care, Hospice Care and End of Life Issues.
To view current news articles on this topic check Google News.
To view images related to this topic check Google Images.
To view videos related to this topic check YouTube Videos.
Feudtner C, Womer J, Augustin R, Remke S, Wolfe J, Friebert S, Weissman D. Pediatric palliative care programs in children’s hospitals: a cross-sectional national survey. Pediatrics. 2013 Dec;132(6):1063-70.
Section on Hospice and Palliative Medicine and Committee on Hospital Care. Pediatric Palliative Care and Hospice Care Commitments, Guidelines, and Recommendations. American Academy of Pediatrics. Pediatrics. 2013:132(5);966-972.
Levine D, Lam CG, Cunningham MJ, Remke S, Chrastek J, Klick J, Macauley R, Baker JN. Best practices for pediatric palliative cancer care: a primer for clinical providers. J Support Oncol. 2013 Sep;11(3):114-25.
Moore D, Sheetz J. Pediatric palliative care consultation. Pediatr Clin North Am. 2014 Aug;61(4):735-47.
Schwantes S, O’Brien HW. Pediatric palliative care for children with complex chronic medical conditions. Pediatr Clin North Am. 2014 Aug;61(4):797-821.
Johnson LM, Snaman JM, Cupit MC, Baker JN. End-of-life care for hospitalized children. Pediatr Clin North Am. 2014 Aug;61(4):797-821.
ACGME Competencies Highlighted by Case
1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
2. Essential and accurate information about the patients’ is gathered.
3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
4. Patient management plans are developed and carried out.
5. Patients and their families are counseled and educated.
17. A therapeutic and ethically sound relationship with patients is created and sustained.
18. Using effective nonverbal, explanatory, questioning, and writing skills, the healthcare professional uses effective listening skills and elicits and provides information.
20. Respect, compassion, and integrity; a responsiveness to the needs of patients and society that supercedes self-interest; accountability to patients, society, and the profession; and a commitment to excellence and on-going professional development are demonstrated.
21. A commitment to ethical principles pertaining to provision or withholding of clinical care, confidentiality of patient information, informed consent, and business practices are demonstrated.
22. Sensitivity and responsiveness to patients’ culture, age, gender, and disabilities are demonstrated.
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa Children’s Hospital