CPC SIG 10:17 pm on January 12, 2021
Tags: baby ( 3 ), change, decision-making ( 2 ), esophageal atresia, trisomy 18 ( 2 )

Supporting parents’ decision making for baby with Trisomy 18

Wishing everyone a pleasant and safe new year! For our very first post of 2021, we are pleased to share a case presentation by Dr Min Sun Kim from Seoul National University Hospital.

The case centers on the family’s decision-making and preference of care for their baby with Trisomy 18 and esophageal atresia. Please tune in to the case presentation through the link below:

If you have trouble accessing Youtube videos, you can download the Powerpoint Presentation Show to listen to the presentation here (you will need the Powerpoint app on your device).

Click on the button to download this case presentation!Supporting parents’ decision-making

We encourage everyone to tune in and share your thoughts in the post! Please click here to comment at the bottom of the page.

Marianne Joy Naria-Maritana 4:42 pm on January 20, 2021

Thank you very much for this presentation. I remembered my patient when i was still a medical intern diagnosed to have trisomy 18 which we were able to send home alive but also expired days after. Since medical school, trisomy 18 is being taught to us as a lethal condition. but i encountered one physician who shared that there are some that has survived because of the advancements in science available and afforded in their countries. Knowing that, like in this case discussion, i am asking myself too on how best to approach such case.

learning from colleagues, trisomy 18 is considered as one of the emergencies where genetic counseling is needed immediately. it is an emergency in this aspect because different organs are affected differently by number and severity and in our country, immediate genetic confirmation test need to be done to help in communicating and discussing diagnosis, prognosis and goals of care.

As parents it would be hard to see and accept their child dying that they would choose to do everything. As i was listening to the discussion, parents initially decided to let go of the child but seeing the child and i guess hearing that operations can be done and give chance to life, or to comfort, changed their decision. Their goal was clear that they wanted to be with the child as long as possible.
I am thinking availability and affordability of getting the needed operations and services done seem not to be a problem even caring for the child. Hence they have came up with such decisions.

in some cases, i tend to explore the psychosocial status of the family. The information gathered somehow helps in planning the goals of care. i.e. if the proposed operations would push the family to sell their only home in order for them to pay for it, then there is a need to weigh the options and check for possible alternatives in management or in getting the funds needed.

In the end, i am thinking it would be the family who would take care of the child most of the time, and would experience loss if the child dies, if all determinants of health were considered, I would respect their decision and be with them as long as they need a Palliative doctor.

I am happy with the things you and your team did for this family. May you be blessed with good health and happiness. I am amazed as how the child was able to reach 8 mos. truly, only God knows what’s in store for us. 🙂

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LEE CHEE CHAN 4:56 pm on January 20, 2021

Thank you for your sincere sharing of your patient management.
I think your team has done the best to suit to the parents’ wish.
Every country has their own culture of working , resource and team spirit.
I will foreseen very much different process and outcome if this happen in my country.
What is past is passed. Most important is the whole team ( including parents) had sat down discussion and made the best interest decision for the patient and the family, then now, is time to move on, and supporting the patient and parents in the best palliative care. Anticipating grief support and education is one of the major component at this moment as well.

Again, appreciate your effort to share this unique experience with us.
Thank you

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XioX 6:52 pm on January 20, 2021

Thank you for an interesting and well-presented case Dr. Min Sun.

It is interesting to me because perinatal palliative care is not well explored in the Philippines, moreso detecting Trisomy 18 antenatally. For some reason, genetic abnormalities like these are not always well detected so families and healthcare professionals have no opportunity to prepare and plan ahead for a concrete management. Also, we don’t always have the privilege to do all surgical operations just for one patient and it is amazing how all measures were exhausted for this case as what this baby experienced. The procedures done to her were also being done here, but for just one patient, it is already a luxury because of costs most of the time so I think you all have been a blessing to this family.

I think because all the interventions are readily available in your country, survivability for this case is not anymore in question. What we can focus on now is the quality of life of the patient and the family. On your question about if you should’ve been aggressive in comfort care in the beginning… When the mother saw her child she struggled to change her decision to do everything for her. I think that was what was called for at that moment — to respect the mother’s wish and give her request to spend more time with her after you have explained the nature of her child’s condition. And you did a great job on that Dr Min Sun!

Apparently, “more time” then can now mean “as long as she lives” so we will go back again to the question of quality of life. How are they now coping financially, socially, psychologiclally, spiritually? Are they happy? Are they not burned out? and things like that. That is exactly the reason why Palliative Care exists–to help, guide, and support them in this journey.

What I wrote here are my realizations from your beautiful case. If it would’ve been in our setting, I think we have resorted to comfort care already because of financial limitations here but just the same, Palliative Care will still be there to provide a another kind of service like grief and bereavement instead. So I think that is the beauty of Palliative Care, we can do or offer something at any stage of the process to alleviate suffering in all forms.

I appreciate your interesting case. Thank you. I’m learning a lot from this.
All the best!

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Fahisham 7:21 pm on January 20, 2021

Many thanks for the case.

We are often facing dilemma entertaining parental wishes versus knowing futility of the condition versus burden of care plus support in our set up and estimating suffering in this case. Juggling from what parent want, it is wise to say that their preferences and wishes are fulfilled. But, what is our paradigm and opinion? I dont think we want to be too paternalistic dictating what the family wishes. If you ask me, to study and manage the condition in my setting, i would say it is unlikely this child would made with a lot of interventions. This has got to do with clinical set up, home support, technical abilities on top of family willingness.

At the end, even though there is no ‘correct’ way to deal with such situations- sometimes the use of zone of parental discretion is allowed provided no harm is done to the child. But the perspective between healthcare professionals and caregivers, but when coming to a dichotomous opinions, i believe sometimes gut instinct may prevail to ensure optimisation of our palliative approach.

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Chong Lee Ai 10:00 pm on January 20, 2021

Hi Min Sun,
Many thanks for sharing this story and you are certainly not alone when you wonder about clinical decisions made. We often do question ourselves if we have served our patients and families ‘well’. Often many of these life-changing decisions are unrecognised ethical dilemmas.

The surgeries and interventions at PICU are medical decisions done in the best interest of the child and the family. In this case there is also the consideration of the best interest as well as harm towards the 2 other young children to consider. Harm to this little baby needs to be considered too. What do parents consider as suffering could be explored.

Trisomy 18 is a clinical spectrum and when there is cardiac and CNS abnormalities, the prognosis is often worse. Parents are guided by our medical advice and we are guided by their values and beliefs. I notice that the medical and surgical teams were not giving consistent advice and it can be confusing to parents.

I was curious that this parents were ready for an abortion at 23 weeks but changed their minds and hoped for comfort care only at birth and then went on to have more and more interventions to fix each organs systems, this was from advice from the different medical teams. Although at 35 weeks and a clinically well neonate, repairing the TEF may be of low risk with benefits of being able to feed and be discharged home.

Understanding how and why parent’s wishes and values changed with time will help you answer your question at the end. What they are willing to and have to give up for the life they have now or that would be considered not beneficial to their little one would be something I would want to explore.

Available health resources play a role as well. They are fortunate that the health care system is able to support intensive care at home for this infant for up to 8 month and for as long as need be.

I hope this family continues to cope well with your support and that everyone has the infant’s best interest, balancing benefit and harm. Thinking about what is done to the infant versus what is done for the infant.

Thanks again for sharing, it must have been a challenging 8 months for you too!

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pohhengc 9:34 am on January 21, 2021

The first thing that struck me as I read your case study – how similar it was to our experience here in Singapore. Except, yours has a happy ending, or not? Nonetheless, it brought back memories of our own experience, the difficult struggles and lingering questions.

Different perspectives shared here by colleagues, even with varying healthcare resourcing, broadened my perspectives richly. I share my thoughts here, with the hope that you will find them helpful, as you continue to reflect on the wonderful experience you and your team had supporting this family. After all, the job is not finished, yet.

1. Hopes and wishes evolve along disease trajectories. From “send the child well” to “after meeting the child, my mind changed”. Throughout the journey, parents often experience ambivalence and confusion. About the latter, see next point.
2. Alignment among healthcare providers is critical. Noted the differences between the neonatologist and the surgeon. This can make the experience even harder for parents who often look to us for guidance. Otherwise, they will turn to Mr Google instead!
3. Baby is already showing apnoea, requiring ventilator support via tracheostomy. This is often a bad sign. More importantly, even though parents seemed to have achieved their goals of having baby for “as long as possible”, what tradeoffs (discussed by colleagues above) are there and what outcomes (quality of life or meaningful time together) make sense right now? Going back to my first point, at some stage (may be soon) you might need to be ACTIVE in engaging with parents once more (underpinned by a trusting relationship you built before) to evolve their goals once more – to one of comfort. This may be through a new advance care plan, like setting limits on future life sustaining treatments, or even IV antibiotics for the next pneumonia.

I am so grateful to you Min Sun for graciously agreeing to contribute a case study in this blog when asked. I have learnt much. Wishing you and the team the best in continuing care for this family. Well done!

Poh Heng

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Min Sun Kim 7:35 am on January 23, 2021

I am very happy to have the opportunity to hear the thoughts of many colleagues. In Korea, national insurance covers most of the costs of hospitalization and surgery especially for children, so it is seldom to decide not to do treatment because of the medical expenses. However, since many medical supplies necessary for home care are not entirely covered by health insurance, baby H’s family received support through donations.

On the one hand, one of the concerns of Korean physicians is whether there is a tendency to decide toward intensive care because the burden of medical expenses is not large. The question is whether the medical staff and parents can easily do medical decisions that may cause pain for the child(patient) because the cost of surgery or treatment is not high.

The process of being with baby H’s family has been a series of worries, but at the same time has given our team great joy. In the future journey, our team will try to contemplate for the child’s best interests. If there is a chance, I will share the progress of baby H’s family.

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Frank Lu 9:29 am on February 3, 2021

Dear Dr. Kim,

Thank you for the very interesting Trisomy 18 case presented here. Sorry that I noticed the message very late, as we don’t frequently use WhatsApp here in Taiwan. It really brought back many memories of some Trisomy 18 babies that we went through together with the families. We also have a National Health Insurance that pays mostly of the medical cost with little self paid, also with the home care ventilators and visits, but that family has to buy some of their own medical supplies, like suction tubes. So the economic challenges are less a consideration for the families here in Taiwan.

Firstly, I would like to say that having a palliative physician involved in the prenatal stage is very good, as we still cannot get involved here in Taiwan, Here we majorly train our neonatologists for palliative care, and hope they can be involved with the most reasonable and best interest view for the baby. We still need to do more about prenatal palliative care here in Taiwan, hope that can be done in the future.

Secondly, during prenatal counselling and discussion with the obstetrician, genetic specialist, and neonatologists, there are really many challenging issues and uncertainties to discuss. If Trisomy 18 is prenatally diagnosed early enough, mostly the families here in Taiwan choose to terminate pregnancy when law is allowed. In the early times, and recently if not diagnosed prenatally, or if diagnosed but the family decided to continue pregnancy, then as neonatologist, we will usually discuss the various conditions that may happen after born, and what kind of treatment we will pursue under different conditions, whether the care plan would want CPAP, intubation, or CPR would be given under what kind of condition, and to the best have a consensus with the family about the care plan. And it is frequently as the situation that you mentioned, that parents don’t want the baby to suffer as the condition is still mostly lethal with very rare cases that can survive to teenage even under surgery.

Literature reviews and cross talk discussions across different medical and surgical specialties when possible is a very helpful tool to facilitate consensus of suggest care plan between different medical teams. Sometimes this is very difficult, but at least we understand each others views. “Starve” is also a frequent myth here in PPC, that we all need more discussion and reflection about it. Better to have a consensus about care plan suggestions and options before discussion with the family if possible. Then during the discussion, the families attitudes and values would come into consideration, and hopeful get a consensus. Especially of possible different outcomes, situations and long term care issues, all need to be elaborated as clear as possible for better understanding of different conditions. If the families accepts the conditions and strongly express their consent about performing surgeries, surgeries would also be carried out here in Taiwan.

In the early days, we had a baby with ASD, VSD and PDA that received cardiac surgery, and then started to use Nasal CPAP at the beginning after referred to our Pediatrics ICU for later post-op care. We then later diagnosed Trisomy 18, and after the results came out and family meeting discussion, we stop using NCPAP and the baby passed away comfortably as like sleeping. The surgeon was involved with our discussion process. So then, we started to have a consensus of how the workup will be performed first at the Pediatric Cardiology department and Pediatric units, then refer to surgery if indicated. So PPC can then be involved earlier if needed.

After full discussion, we respect the families views and choice of whether they want to pursue surgery or not. But we will try to discuss DNR issues at the same time, so usually the families here in Taiwan if they choose to perform surgery, they also decide to DNR if unfortunately the condition became very critical despite treatment.

It is hard to judge the families choice, especially when the progress or outcome is shown. But we also reflect on how our suggestions were and the family consents come out. It is sometimes very challenging. But the practical issue is we only can try to make our best choices at that specific time, and learn from the experience that the baby teaches us. All have its own ways, and this is life. I have families that think tracheostomies, home ventilators and lots of home care managements, and even bed-ridden, are all sweet burdens, that they would like to take and share the precious time with their babies. Of course some families think the burden is too heavy. We can only provide medical information, facilitate best interest decision-makings, and accompany the journey with the baby and the family altogether.

I think what you have done in Korea is very good and important for the family. Best wishes for the child and the family, and for your team, which has done a great job to support the family.

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- Poh Heng 10:39 am on February 3, 2021
  
  Thanks Frank for sharing your experience in Taiwan. What is striking in your narrative is again how similar our experiences are, despite differences in healthcare systems, funding framework and clinical practice. This statement particularly resonated with me “But the practical issue is we only can try to make our best choices at that specific time, and learn from the experience that the baby teaches us. All have its own ways, and this is life.”
  The example of the baby with withdrawal of nasal CPAP illustrates how PPC can change things for the better slowly over time. This is within our sphere of influence. As for other issues outside of our control, we can only do as you wisely shared “accompany the journey with the baby and the family together”.
  Appreciate all the exchange of ideas thus far and thanks Min Sun for starting this rich discussion thread!
  
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