Solid Organ Transplant

32 Solid Organ Transplant

Timothy Klouda, Debra Boyer, Lynne Helfand, Michael McCown, Nicholas Purol, and Dawn Freiberger

Every obstacle I have overcome

Has made me stronger in some way

And to get the best out of life

I must live it day by day.

So this bump I’ve now stumbled upon

Is no different from the rest

I must smile, hope, and fight

And try my very best.

As my hair may all fall out

My diabetes out of whack

I will continue to get better

And my hair will grow back

So accept me as I am

While I go around this bend

Cause I’m going to get through this

I’m fighting till the end.

cystic fibrosis patient

Providing palliative care to patients pursuing an intensive, life-extending treatment such as organ transplantation may seem paradoxical. However, the presence of severe end-stage disease, insufficient organ availability, and significant morbidity and mortality associated with transplantation requires the application of palliative care concepts through every step of the transplant process. The disparity between the number of patients listed for solid organ transplant and donors is significant. Finding a viable organ for a recipient leaves listed patients with unpredictable waiting times, potentially lasting years. This can leave both patients and care givers experiencing a wide array of emotions due to their uncertain future. During this period, the patient’s medical status may worsen and his or her care needs could change dramatically. Patients and caregivers may also no longer wish to pursue transplantation for medical or personal reasons. Therefore, transplant medicine is focused on providing life-extending treatments but must always recognize the medical realities, symptom management challenges, and emotional needs of the patients and families involved.

The frequency of organ transplantation has grown dramatically over the past 30 years and has increasingly become a part of patient care. In 2017, there were 34,770 solid organ transplants in the United States, with 1,899, roughly 5%, of these procedures occurring in patients less than 18 years of age (Table 32.1).¹ The number of total solid organ transplants has gradually increased for 6 consecutive years, and this trend is likely to continue due to greater experience with surgical techniques and improved treatment and therapies for end-stage disease. These advanced therapies, such as ambulatory extracorporeal membrane oxygenation (ECMO), have led to increased patient survival, thus increasing the number of recipients listed for organ transplant and prolonging the lives of those waiting for transplantation.

Table 32.1 Distribution of pediatric solid organ transplants in 2018

Organ	Number of pediatric transplants N (%)	5-year survival rates (%)
Kidney	756 (40.5)	98.4
Liver	563 (30.1)	88.4
Heart	473 (25.3)	81.5
Intestine	37 (2.0)	76.4
Lung	40 (2.1)	60.6

Data compiled from United Network for Organ Sharing 2018 and https://srtr.transplant.hrsa.gov/annual_reports/2018_ADR_Preview.aspx

The specific criteria to be listed for organ transplantation vary depending on which organ is involved, but generally patients are referred when the morbidity and mortality associated with their underlying disease is greater than that associated with transplant. Mortality rates reflect a variety of factors, including the impact of the patient’s underlying disease and the technical and medical difficulties associated with the transplant procedure and post-transplant complications. Morbidity rates are more difficult to quantify, and the impact of transplantation on a patient’s quality of life is similarly challenging. Reported morbidity associated with transplant includes complications from the surgery, chronic organ dysfunction due to ischemic injury or rejection, infection, and side effects associated with long-term immunosuppressive therapy (Table 32.1). Ultimately, all transplants require a patient and caregiver commitment to continued medical care and frequent follow-up. Transplantation should not be viewed as a complete cure of the underlying disease process, but rather a transition to a different chronic medical condition. Recipients of solid organ transplants may struggle with increased anxiety after transplantation resulting from trauma often associated with waiting for an organ offer and terminating treatments for their previous chronic illness as well as learning new therapies and complications associated with transplant.

Solid organ transplantation is offered in many but not all countries, generally in more developed and financially sound environments. However, patients often must travel great distances to obtain needed services, adding an emotional and potential financial burden to patients and their caregivers. In addition, patients do come to the United States from abroad seeking organ transplantation, adding further complexity to their clinical and psychosocial situations.

Family Reflection

Thankfully many parents don’t have to ‘‘live” in a hospital with their child. They visit, have short or long stays, but they go home. Being in the hospital is like living in a fishbowl; there’s no privacy, your autonomy as a parent is gone but you have a wonderful support system if you’re lucky.

My son lived for a month after I was told he wouldn’t be coming, home and there was nothing more the hospital could do except keep him comfortable? I grieved even before he died. I grieved for his future, for the things he would never do, people he would never meet, fall in love with, fall out of love with. Even after virtually living in the hospital for 16 years with cystic fibrosis, I always expected to leave with him. It was a daze. The first day I walked around in pain, anger, resignation. I pleaded to all of the deities, wished I could go back in time and find out where I lost the ability to impact his life. How do you parent a child who is dying? He was justifiably ANGRY; at the doctors, nurses and at me, mostly at me. It’s my job as a mom to care for him, and I failed at it. He acted out and if he was disciplined, his response was “so what? I’m gonna die anyway.” At that point, all you can do is love them, soak in every moment, experience, word. You stay up late and talk, play games, cry together. You bust your butt trying to make wishes come true quickly in such a short period of time because you don’t know when his last breath will come. You lean on the nurses like they’re your brothers and sisters, you hide your soul-ripping grief behind smiles and laughs to keep from screaming at the top of your lungs. You cry in the shower, so your child doesn’t see you break down because you’ve always been the strong one. You wake up and wonder if that day is going to be the day your baby leaves you forever, and you hope for just one more day to love them. And one more day is never enough. Life shifts when you’re in the hospital with a dying child. Problems don’t seem as insurmountable or difficult anymore and it all becomes background noise.

I was angry, unjustifiably, at the doctors because they’re the ones who you trust to heal your child. They had done everything they could, I knew that, but as my son focused his anger at me, I focused mine at them. We had fought his disease for 17 years and now were stuck “waiting to die,” a situation that no parent should have to endure. It’s unnatural. We hold the hands of our elders as they pass, celebrate the life they lived, but what’s to celebrate about a teenager dying?

Transplant Evaluation

Transplantation is an extraordinarily stressful time from every perspective. Careful patient selection is required due to the physical demands involved with the actual procedure, the psychosocial upheaval involved throughout the process, and the extensive financial and social support required after a transplant. Transplant evaluation includes a comprehensive review of the patient’s medical history, current physical condition, psychological and/or emotional status, and available financial resources. Evaluation is also a time when the family and patient are introduced to the entire transplant team and the process of transplantation. The development of relationships with the care team during this process can have a lasting impact on patient care throughout the transplant process.

It is essential that the family learn about all aspects of transplantation, including anticipated time on wait lists, potential risks associated with the procedure, and what level of care and lifestyle they can expect post-transplant. In addition to their medical/psycho/social assessments, often the teams that are consulted as part of the evaluation are educating the patient and family about post-transplant care. The transplant consent process generally occurs during the transplant evaluation. At the end of the evaluation, the patient may be actively listed for transplant, placed on deferred status until they meet criteria, or be declined for a specific reason. Occasionally, the patient and/or family may elect to not pursue transplantation, may choose to visit other centers, or decide to postpone listing.

The medical evaluation includes an assessment of the underlying disease process for which the patient was referred, as well as a comprehensive assessment to identify comorbid conditions that may complicate or preclude transplantation. The substantial risks involved with transplantation and the relative scarcity of available organs mandates an intensive evaluation of the patient’s current and past medical history to ensure the best possible outcome. Each transplant team will have specific requirements for further testing, but, in general, patients undergo a variety of physical exams, laboratory tests, radiographic procedures, and consultations. Patients are frequently referred to a transplant program from remote locations with little or no preceding contact with the transplant center. Referral may follow a dramatic decline in the patients’ medical condition and can be at a particular time of crisis for the family. Because of this, it is not uncommon for transplant centers to repeat testing that has already been completed at the referring institution to confirm results and evaluate for disease progression. The tests and studies that are repeated vary between individual transplant centers, along with the criteria they use to determine transplant candidacy.

Each patient being referred for solid organ transplantation will be evaluated by an interdisciplinary team including various medical and surgical providers, social workers, financial planners, mental health professionals, and pharmacists. Social workers will assess a family’s ability to handle post-transplant care and identify their practical and emotional means of support. There are numerous psychosocial stressors present throughout the transplant process, including living with chronic disease, frequent medical evaluation and treatment, the uncertainty of when the transplant will occur, and the substantial requirements for ongoing care throughout the transplant process. In addition to the impact of these stressors on the patient’s and family’s psyche, there are significant associated financial expenses involved in the process. These include the need for frequent admissions, clinic visits, and procedures that are commonly at centers remote from their home. Families may miss work and have travel and food expenses that they would not normally have. Depending on the patient’s insurance, uncovered medical expenses can be overwhelming. A social worker will evaluate the family’s strengths and ability to cope with these stressors as well as identify potential areas of concern that may impact the care of the transplanted patient. The social worker will also assist the family in locating and developing the resources and supports they will need to successfully navigate this process. A mental health professional will assess the readiness of the patient and family for transplant and evaluate the level of understanding regarding the child’s health and prognosis. There are times when a family and medical team are ready to list for transplant, but the child is not ready. A child may be fearful of the surgery itself or its associated pain and potential complications. Some children may doubt the necessity of the transplant. In these instances, the psychosocial support team can be very helpful in preparing the child and navigating issues of assent in these young patients.

Prior adherence to medical regimens must also be considered, as well as a patient and family’s understanding of pre- and post-transplant care needs. It is not uncommon for patients and families to be presented with patient/caregiver agreements, with the goal of setting clear expectations around what is expected while on the wait list and after transplant. Such agreements may also help identify barriers to a successful care plan and allow the interdisciplinary care team to help support the family in reaching expectations. Examples of areas for agreement could include maintaining fluid restrictions or meeting enteral nutrition goals, which may involve assessment and concrete support planning from a transplant nutritionist, psychologist, and/or social worker. As the inability to meet these expectations puts the patient at risk of being inactivated on the transplant list, clearly identifying these expectations and addressing specific challenges to them becomes crucially important in meeting the shared goal of a successful transplant.

At the conclusion of the evaluation process, the patient’s disposition regarding transplant will be determined. Patients are formally presented to the transplant team for discussion and all factors—medical, psychosocial, financial, developmental, and family resources—are taken into consideration. Therefore, both medical and psychosocial factors play a large role in the ultimate decision about whether to list a patient for transplantation.

A decision to list a patient will occur when the benefits of a transplant are greater than the predicted morbidity and mortality of the patient’s current disease. There are no clear criteria as to when this threshold is met, and therefore listing can be a complicated decision. Importantly, the patient’s quality of life is a vital factor in deciding when to proceed with transplantation. Application of this standard must be tempered by a thorough understanding of the overall health of the patient, and care must be taken to not wait too long when comorbid conditions and overall poor health may adversely affect the post-transplant prognosis. Consideration of a subspecialty palliative care team consult is often made during the evaluation phase of transplant. The decision if or when to list is not always straightforward. Palliative care teams can help families sort out goals of care, quality of life, and treatment burden as it relates to the medical teams listing recommendations.

Ethical issues arise when transplant teams are faced with a family with psychosocial limitations. The team must ensure that resources are available to allow the family to overcome obstacles so that they do not compromise the child’s care. It is well-documented that proper post-transplant care is essential to ensure a good outcome. Therefore, in some instances, patients are declined for transplant due to insurmountable psychosocial limitations. On rare occasions, support from child protective services is sought out to provide a safe environment for the child post-transplant (Table 32.2).

Table 32.2 Transplant team and their roles

Transplant team member	Role
Surgeon	Performs transplant surgery and post-transplant care
Physician	Provides medical care both pre- and post-transplant
Nurse coordinator	Responsible for pre- and post-transplant care from a nursing perspective, coordination of care, evaluations and patient and/or family education
Social worker	Provides assessment and care around psychosocial needs of family during all phases of transplant process
Psychologist	Performs pre-transplant evaluation to identify any contraindications to transplant. Identifies need for continued therapy pre- and post-transplant
Registered dietitian	Performs nutritional assessment and evaluation during pre- and post-transplant process
Physical therapist	Provides pre- and post-transplant care to encourage recovery and ambulation
Administrative team	Centralized team consisting of clinical and administrative staff focused on outcomes and patient care experiences
Infectious disease	Responsible for infection-related pre- and post- transplant care for immunocompromised organ recipients
Child life specialist	Provides support before and after medical procedures, aids with patients and families adjustment and understanding of the transplant process
Transplant pharmacist	Provides education and medication management of immunosuppression medications pre- and post-transplant
Financial coordinator	Aids families with the financial aspect of transplant care
Chaplaincy	Works with families and patients alongside the medical team to prove psych-social-spiritual services
Palliative care team	Assists the medical team, family, and patient by providing an additional layer of support and focusing on advanced care planning, quality of life, and symptom burden

While many transplant clinicians practice and effectively utilize strong communication and symptom management skills as part of their regular practice, there may also be times to consult a separate subspecialty palliative care service. This referral can assist the primary team with managing complex symptom burden, ethical considerations with exceptionally high-risk transplant cases, and clarity when there may be competing or deeply unclear goals in the family. At these times, introducing the idea of a separate yet collaborative palliative care team may ultimately provide an added layer of focused symptom management and goals of care decision-making conversations that enhances the already complex evaluation process. In addition, the palliative care team can aid the patient and family with many of these competing and conflicting aspects of their care.

Transplant Preparations

Once the determination has been made to list a patient for transplantation, the child is placed on the national wait list, thus beginning a new period of uncertainty. The waitlist process varies by organ type, with some organs using allocation scores based on disease severity to determine the place on the list. Some organs use pure time on the list, with some preference given to children and adolescents. For some organs, allocation may depend on where the donor hospital is located. When this system is used, it is a combination of distance and medical urgency that determines to which patients the organs are offered. The goal of this allocation process is to ideally transplant the sickest patients with the highest likelihood of a wait list death.

Waiting for a transplant can be very stressful for the patient and family. Families are aware that in hoping for a transplant for their child, they are often waiting for a tragic event to happen to another family. Understandably, this can be very difficult for older children and families to process. It is explained that, for a donor family, organ donation enables them to see some benefit come from their tragic situation. While no religion outright forbids the donation or receipt of solid organs, some may discourage donation in certain scenarios. For example, in Japan, the national Shinto religion states that the integrity of a dead body should be preserved, and the injuring of one is a serious crime.² Other religions share similar beliefs, making solid organ transplantation less common. A chaplain can help provide spiritual guidance and counseling to patients and families, emphasizing their religious beliefs and helping facilitate communication between the patient and transplant team to provide the best possible care.

Waiting times on the list vary from a day to years. Families are required to be easily reached and remain available to come quickly to the transplant center when an organ becomes available. Arrangements need to be made in advance for other family responsibilities such as sibling childcare. Not uncommonly, patients are called in for a transplant only to be sent home without the surgery when complications with the donor arise. Patients can also be listed in more than one region, which may mean twice the clinic visits and added travel expenses. Certainly, this compounds the time and financial investment for the family.

It can feel as if life is put on hold while a patient is listed for transplant. With the need to be near the transplant center, vacations generally need to be close to home or require a period of inactivation on the list. Activities that have a high exposure to potentially sick contacts should be curtailed. The uncertainty that the patient may not survive to transplant leads to an extraordinarily stressful time in many patients’ and families’ lives.

It is during this pre-transplant phase that applying palliative care strategies can be extremely helpful. The patient will frequently be receiving intensive medical care to optimize his or her overall health for when the transplant occurs. Regardless if a patient is hospitalized or waiting at home for a transplant, keeping up with the medical regime is important. This includes optimizing nutrition, physical therapy, and mental health. Patients waiting for transplant often struggle with nutrition, commonly due to the severity of their underlying illness and associated comorbidities. Patients may undergo gastrostomy (G) tube placement to provide adequate nutrition during this time period and after the transplant. This decision can lead to complications itself and may provide additional stress, especially in adolescents and young adults. Optimizing medical care when a patient is ill is not always consistent with quality-of-life goals. Balancing the benefits of intensive continued disease-directed therapy with an acceptable quality of life needs to be continually considered and assessed. Open and frank discussions with the patient and family regarding their goals and wishes should occur regularly between the patient and transplant team. There could come a time when a patient decides that continuing therapies is too difficult and severely interfering with their quality of life. In these instances, patients and their families should talk with their transplant teams to see if it is possible to alter treatments and still stay active on the transplant list. If it is not, some patients may decide they no longer wish to be listed for transplant. Clearer understanding of a patient and family’s goals and priorities for treatment may help avoid or ameliorate potential conflicts or tensions when addressing pain or symptom management as it relates to recovery from transplant. Similarly, palliative care teams can be very helpful in working with the patient’s primary physicians and the transplant teams to ensure that the goals and wishes of the patient and family are continually respected.

While waiting for transplantation, patients may experience chronic pain, discomfort, and dyspnea. Patients may have suffered from chronic pain for years and, as a result, been prescribed analgesics such as opioids and benzodiazepines. While these medications can improve quality of life, evidence concerning the effects of opioid use on transplant outcomes remains limited. Some studies have shown that pre-transplant opioid use is associated with an increased risk of graft loss, readmissions, and higher mortality rates, while others have demonstrated no association between pre-transplant opioid use and retransplant-free survival.³^,⁴ Other possible side effects include decreased gastrointestinal motility; suppression of respiratory drive, making it more difficult to wean from ventilator support; and increased tolerance, making pain control in the postoperative period more complex. While there are no national guidelines across specific organ systems, some centers consider the chronic use of these medications in the pre-transplant period a contraindication to listing. If the use of opioids is accepted by the transplant team, being on the smallest effective dose possible is preferable. The palliative care team can provide specialty guidance and recommendations around the safe use of opioids and other medications while supporting an underlying goal for transplant eligibility and success.⁵ To this end, the palliative care teams can also assist in identifying and supporting integrative, nonpharmacological strategies for symptoms while also providing education to families around risks and benefits of opioids when indicated.

It is important to acknowledge that some patients become too ill and no longer remain transplant candidates, meaning they no longer are predicted to survive a transplant. When facing the decision to inactivate a patient on the transplant list, providers are wrestling with the concern that the patient would not survive the transplant as well as consideration of the allocation of a scarce resource. Many families are conflicted over this, because some believe that if their child is going to die regardless, they ought to be given a chance with transplant. It is advised that the possibility of this happening be addressed with families at the time of transplant listing, especially for organs that have a higher waitlist mortality. This allows the family to mentally prepare for the possibility of their child no longer being a transplant candidate. Often, a palliative care specialist can help to facilitate these conversations and better prepare patients and families for these potential events.

There are also a number of waitlist deaths each year, where the appropriate organ is not found in time to save the child. Transplant caregivers can find themselves in the situation of concurrently managing and maintaining end-stage diseases while simultaneously navigating the dying process. This can feel contradictory to the requirements of staying active on a transplant list. It is not uncommon for members of the medical teams to have differing opinions on how to handle these situations. Families are also conflicted with their desire to not give up and yet spare their child any unnecessary pain or discomfort. Discussions about whether a patient can have a do not attempt resuscitation (DNAR) order in place and still be active on the transplant list are not infrequent. An example might be a patient with cystic fibrosis who faces potential intubation for progressive end-stage lung disease yet still hopes to reach transplantation. In this situation, some patients and/or families elect to proceed with intubation in the hope that an organ will be located in time. Others will choose to pursue medical management while maintaining firm limitations around specific interventions such as ventilatory support. This decision-making can become even more salient and challenging in situations where a ventricular assist device (VAD) or ECMO may need to be considered. While this level of mechanical support is not uncommon in patients waiting for heart or lung transplantation, for instance, it may represent varying degrees of quality or suffering to different patients and their families. The palliative care team can be instrumental in guiding the patient, family, and caregivers through these difficult decisions (Figure 32.1).