Dr Mandy Man and Nurse Agnes Wong from Hong Kong’s Children Hospital presents a case of supporting young MM and his journey to go home with the help of transmucosal fentanyl.
MM is a 3-year-old boy diagnosed with retroperitoneal embryonal rhabdomyosarcoma since the age of 1. Parents understood the prognosis and wished to facilitate MM’s ability to enjoy every moment of life. When he shared a desire to be discharged and to go home, the team looked into ways to manage his cancer pain at home.
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My name is Agnes Wong, a paediatric palliative care nurse from the Hong Kong Children’s Hospital. I would like to share a case named MM, who suffers from a terminal disease. I am his case manager and facilitated him to go home with the help of transmucosal fentanyl.
MM, a 3 year-old-boy, was diagnosed with retroperitoneal embryonal rhabdomyosarcoma at around 1 year old. He received a full course of treatment, including chemotherapy, gross total tumor excision, and radiotherapy. He was disease-free for around one year. Unfortunately, he was noted to have disease relapse at 2.5 years old and the palliative care team was subsequently involved.
He presented with abdominal distension and poor appetite. CT was done and suggested diffuse tumor metastasis, especially over the peritoneum. MM received a further course of chemotherapy with curative intent. However, evaluation imaging showed further disease progression. Oncology team then switched to another chemo-regimen for MM. During that period, parents were in distress. They understood the grave prognosis of their son. They changed family plan to facilitate MM to enjoy every moment of life. But they still strived for the chance of a cure, otherwise they feel that they would regret not having tried any possible treatment.
They travelled to USA for HIPEC, a surgery meant to treat peritoneal cancer. However, this was not done when multiple bone metastasis was found before the procedure. Parents told us that the medical team in USA discussed the best course for MM with them, and did not suggest any further surgery. MM received further chemotherapy in USA and was brought back to Hong Kong. His disease continued to progress and MM started to present with various symptoms. The oncologist further changed the chemotherapy regimen with minimal effect.
MM had complained of dripping of urine and urinary frequency; PET-CT showed disease progression and a mass just posterior to the bladder. Suprapubic catheterization was inserted.
He also developed greenish vomitus with increased abdominal distension. CT abdomen showed likely mechanical small bowel obstruction and multiple foci of peritoneal metastases.
In view of disease progression, We discussed with family about the goals of care for MM. His parents agreed for conservative management of IO and not for surgery. They understand the disease progression and would like palliative chemotherapy as symptom control if the child can tolerate it.
We started PCA morphine to control his suprapubic pain. Ryle’s tube was also inserted for decompression. We also discussed comfort feeding with parents; they aspirated the stomach content after MM ate his favorite food. We also supported the family for limited daily home leave with perienteral nutrition infusion during night-time. With such support, MM was able to enjoy more outdoor activities, or play at home freely during the day.
MM told us that he wanted to be discharged, go home and play.
Palliative chemotherapy was given for symptom control and his IO symptoms improved. We tried to weaned off morphine PCA to fentanyl patch, oral Panadol/ morphine hoping to facilitate home care. However, the pain control was sub-optimal. It is noted that MM had on and off suprapubic pain, with sharp pain in nature which greatly disturbed his daily activities.
We noted that oral Panadol is too weak, the onset time oral morphine is too slow, while there is no diamorphine A/V in Hong Kong.
It is also noted MM has sub-optimal tolerance to oral medication due to on and off subacute intestinal obstruction, with occasional vomiting after oral medication. This affected his med absorption via the oral route.
With the above considerations, we tried sublingual Temgesic. However, the drug is bitter in taste; MM was unwilling to suck the drug and spat them out.
We were thus faced with a dilemma: Should we resume IV analgesic for better pain control, but keep MM in the hospital?
We knew that transmucosal fentanyl can be used for breakthrough episode cancer pain in patients on regular strong opioids. However, it is expensive and there is no transmucosal fentanyl products available in Hong Kong. With experience shared from other countries, we decided to use parenteral formulation of fentanyl as sublingual administration. We discussed the use of SL fentanyl with parents and educated them on how to prepare and administrate the drug.
First, we calculated the dosage and frequency to be used in breakthrough pain. We then educated parents how to aspirate the required drug from the vial and squirted the drug into buccal mucosal or sublingual areas. It was important to educate MM not to swallow the medication and to hold it for at least 1 minute. We also educated parents to throw sharps into the sharp box and to monitor the effect on MM.
We trialed sublingual fentanyl for MM. The video attached shows how a 3 years old child can take this drug after education.
MM shared with us that he thought that the drug tasted good; like a lollipop! This may be due to the citrate content in the drug. He learned not to swallow the drug and keep the month shut till one minutes passed by with counting.
Overall the drug effect is well tolerated with satisfactory pain control which suits MM’s condition a lot. It has fast onset time within 5 minutes after administration, which is perfect for the nature of MM’s sharp onset breakthrough pain. The taste is also well tolerated by children.
MM was eventually discharged with (i) fentanyl patch, and (ii) prn Sublingual fentanyl after drug titration. No IV analgesic was required.
While his symptoms were being controlled at home, the family focused on using every living moment with MM to enhance the quality of life for him. They went to Disneyland, Ocean park, the beach. They let him eat ice-creams, play with friends and his elder sister, let him go back to school, and enjoy quality time with family members.
MM wished to be a fireman when he grows up. With the help from “Make-a-Wish”, MM’s had a chance to visit the fire station on his birthday. He enjoyed the day with the firemen, joining their daily activities, riding on the fire trunk and even saved his beloved teddy bear from a small fire.
During this period of time, we further discussed with MM’s parents the goals of care and future care plans for MM. Funeral arrangements for MM and any preference on legacy making were brought up. Ways to maintain continuing bonds with MM were also explored.
We also empowered parents to discuss death and dying issues with MM and his elder sister. Story books were suggested, plus skills and techniques were shared by their medical social worker.
If I become an angel, I will not have tubes anymore?– MM
During the discussion, the parents discovered MM’s care preferences and agreed on the goals of care.
When the mother talking about the afterlife with her children, MM asked his mother “If I become an angel, I will not have tubes anymore?” His parents then understood MM really hated the tubes on his body. Following this, the parents told us that they did not want MM to undergo any surgery or procedure with tubes, like tapping for ascites or pleural effusion or epidural anesthesia. Mother said “Although he is just 3 years old, he is an individual who needs our respect”. Our focus of care were based on MM and his family’s values and beliefs.
Although he is just 3 years old, he is an individual who needs our respect– MM’s Mother
When MM continued to deteriorate due to the natural progression of disease (an irreversible trajectory), the family prefers NOT to transfer MM to ICU. They refused respiratory and cardiac supports as it would induce suffering for MM. For other decisions like the ways to maintain nutrition and fluid and the use of medication, they preferred the medical team to keep it if clinically required.
Although MM was responsive to the palliative chemotherapy, the honeymoon period gradually passed. He required increasing dosage of analgesic for pain control and underwent superior hypogastric plexus block twice. The effect of palliative chemotherapy was less effective over time, as his disease further progressed. In the end, MM needed to be readmitted to hospital for PCA fentanyl.
At this stage, we found different parties held different points of view on the best care plan. The team recommended to withhold all chemotherapy and life-sustaining treatments in MM’s best interests. While his mother expressed her grievances regarding treatment management; she wished for further palliative chemotherapy for symptom control. She stated “Letting go is decided by the parents, not by the medical team.” What the medical team was doing went against MM’s mother’s core value of trying to do the best. Father perceived MM had already suffered a lot, he hoped to maximize comfort care and worried that further chemotherapy would only prolong MM’s suffering. We tried to facilitate the three parties to understand each other. Finally, we came up with a care plan with mutual consensus.
MM was changed to another palliative chemotherapy regime with less toxicity and complications. MM has partially responsive to this chemotherapy regime, and was able to be tailed off IV analgesic with continued symptoms control using: (i) Fentanyl patch, (ii) Methadone, (iii) Pregabalin, and (iv) prn Sublingual fentanyl & (v) oral Oxycodone.
He was discharged home and celebrated his 4-year-old birthday with family members and friends. This time, MM enjoyed a few days at home, was admitted to hospital after his birthday as the symptoms worsened with disease progression. Though the time period of discharge was short, the family was happy that they managed to enjoy this family gathering at home, especially on his birthday.
He received care in hospital and his care plans were guided by quality of life as defined by the child and the family. MM finally passed away peacefully in his sleep last week, accompanied by his parents and sister.
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