953 support structure and inheritance patterns of their underlying disease In addition, opportunities for living donation may increase in places where there are kidney exchange programs or access to A[.]
49 Evaluating and Preparing the Pediatric Dialysis Patient for Kidney Transplantation support structure and inheritance patterns of their underlying disease In addition, opportunities for living donation may increase in places where there are kidney exchange programs or access to ABO-incompatible transplantation The transplantation of a kidney donated by a living donor offers several advantages compared to a deceased donor: (i) the kidney donors are generally young and healthy and have undergone thorough screening; (ii) the procedure can be planned appropriately over time; (iii) immunological tolerance is usually better due to the haploid entity between parent and child compared to a deceased donor kidney – hence less intense immunosuppressant therapy is often required with consequently fewer side effects, and (iv) prolonged organ preservation is not needed so the structure and function of the transplant are improved These factors therefore explain, at least in part, why the 5-year graft survival rate is approximately 10% better with a living kidney donor compared to a deceased kidney donor Furthermore, a pre-emptive approach (i.e., prior to dialysis therapy) is also more feasible with a living kidney donor, thereby preventing any potential dialysis-related complications [3] The advantages of living kidney donation must be weighed against a very small surgical risk to the donor The peri-operative mortality risk associated with living kidney donation is very low (0.03%) The risks immediately associated with surgery, i.e., active hemorrhage, corrective surgery, and thrombosis, range from less than 1% to less than 5% The overall risk of developing any complication is less than 9% Long-term, life expectancy is similar to the general population However, the risk to the donor increases slightly compared to healthy non-donors in terms of developing end-stage renal failure (absolute risk of approximately 0.8%) Nevertheless, the living donor actually has a slightly lower probability of developing end-stage kidney failure compared to the normal population as living kidney donors have been thoroughly pre-screened for renal disease risk and will usually undergo regular nephrological follow-up There is also a slightly elevated risk that the donor will develop arterial hypertension and/or proteinuria [41] 953 The donor will have approximately 80% of normal kidney function after donating a kidney An annual check-up is therefore required in order to promptly identify any further impairment of kidney function or the development of arterial hypertension In the Eurotransplant countries, the annual check-up comprises a 24-h blood pressure recording on an outpatient basis, determination of kidney function and urine protein elimination, an ultrasound scan of the remaining kidney, completion of a questionnaire or advice in terms of quality of life, and the offer of psychological support, if required In the United States, living donor follow-up is mandated to be reported by the transplant center at 6 months, 1 year, and 2 years after donation [42] Compared to the normal population, the majority of kidney donors comment in questionnaires that they feel better and have a positive feeling about kidney donation Occasionally, there will be negative feedback from the kidney donor, essentially due to surgical complications and dissatisfaction expressed by the recipient following transplant, and a reduction in the quality of life In this context, there have been individual reports of the onset of an adaptation disorder (diminished performance, withdrawal from social circles, and depressive episodes) [43] A 4-week recovery phase should be planned following living donor surgery Physical ability returns to preexisting levels after approximately 3 months The donor can then resume work, but decisions must be made on an individual basis This information is often helpful to present to families during the child’s evaluation since the potential donor may also be the pediatric recipient’s caregiver Assistance in developing a plan for additional family support in the household after transplant should be discussed with the transplant social worker For example, living donors are not able to drive for several weeks after transplant and, thus, a driver to bring the child to the hospital for the child’s post-transplant clinic visits should be identified a priori Even if living donation is deemed the best option for the recipient from a medical perspective, deceased organ donation is almost always offered as an alternative In view of the decrease S Amaral and L Pape 954 in organ donations and long waiting times, when considering children with end-stage renal disease, the risk to the living donor must be weighed against the risk of deterioration in the child’s health if he/she is placed on dialysis pending transplant surgery The child’s family must often discuss this issue with the medical and psychosocial teams, with consideration of the individual family situation when making a decision The extent to which the child will tolerate dialysis therapy must also be factored into the decision- making process Since the average waiting list for children is considerably shorter than the adult waiting list given the priority provided to children, many parents want to wait until their child is over 16 and then consider any substantial increases in terms of waiting time at this point if transplant surgery is still required However, it should be noted that because of age-related morbidity, the parents may no longer be eligible to act as donors Furthermore, a deceased donor’s kidney may have been damaged in the past, e.g., due to the deceased donor’s previous condition such as hypertension and atherosclerosis, which may also account in part for the previously mentioned longer survival of living donor vs deceased donor kidney transplants A study in 2015 examined which donor order was preferred for a child if only one living donor was available, i.e., living then deceased or deceased then living The authors concluded that the only condition under which it was favorable for a child to receive a deceased donor kidney first was when the child was highly sensitized [44] Overall, a healthy kidney from a living donor is a remarkable treatment option during a crucial phase of development In conclusion, the transplant evaluation process is comprehensive and multi-disciplinary with the intent of ensuring the safety, efficacy, and long-term success of the graft and benefits in duration and quality of life to the child Referral for transplantation should occur early, as soon as a child reaches advanced stages of chronic kidney disease, and ideally before the initiation of dialysis Appropriately executed, the transplant evaluation affords the opportunity for the trans- plant team to optimally prepare a child and their caregivers medically, surgically, and psychosocially for favorable short- and long-term outcomes (Table 49.2) Table 49.2 Examinations transplantation needed for listing for Evaluations for anatomic conditions Ultrasound of jugular, subclavian veins, and arteries Ultrasound of abdominal vessels (aorta, V cava, iliac veins) History of abdominal surgery Urologic situation and need of pre-transplant interventions (UTIs, bladder emptying problems, megacystis, uretheral valves, neurogenic bladder) MCUG Cystoscopy (in individual patients) Cystomamonetry (in individual patients) Indications for nephrectomy (i.e., Denys-Drash syndrome, non-treatable arterial hypertension, nephrotic syndrome with persisting gross albuminuria, UTIs, dilatation of kidneys, ureter) Laboratory, imaging, and other studies WBC, RBC, GOT, GPT, yGT, Potassium, Sodium, Chloride, Calcium, Glucose, Creatinine, Urea Virology: Hepatitis A, B, C, CMV IgG/IgM; EBV IgG/IgM, HIV, Measles IgG, Mumps IgG, Rubella IgG, Varicella IgG, Herpes Simplex Virus IgG, RPR Quantiferon test Urine: Dipstick Analysis – U-Protein, U-Glucose Ophthalmologic exam Echocardiogram, ECG X-ray of chest and left hand Vaccination history Dental evaluation Blood group, transfusion history HLA-identification HLA-antibodies/Panel-reactive antigens Other considerations Cystatin C, CK, LDH Endocrinology: fT4, TSH, PTH, 25-OH Vitamin D3, Testosterone, Oestradiole, FSH, LH, HbA1c Coagulation: INR, PTT, Thrombin Time, Fibrinogen, AT II, Protein C, S, APC, Factor II and V Mutation, MTHFR Mutation, Antiphopholipid- Antibodies, LP(a) 24-h Urine for Creatinine-Clearance and Calciuria, U-alpha-1 Microglobulin, U-Creatinine, U-Albumin Ultrasound abdomen Pulmonary function test 24-h Blood Pressure Audiometry, ENT-Evaluation Gynecologic evaluation (girls after menarche) 49 Evaluating and Preparing the Pediatric Dialysis Patient for Kidney Transplantation References https://transplantliving.org/living-donation/history/ Van Arendonk KJ, Boyarsky BJ, Orandi BJ, et al National trends over 25 years in pediatric kidney transplant outcomes Pediatrics 2014;133(4):594– 601 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Trends in living donation by race and ethnicity among children with end- stage renal disease in the United States, 1995–2015 Transplant Direct 2020;6(7):e570 19 Flechner SM, Thomas AG, Ronin M, et al The first years of kidney paired donation through the National Kidney Registry: characteristics of donors and recipients compared with National Live Donor Transplant Registries Am J Transplant 2018;18(11): 2730–8 20 Sypek MP, Alexander SI, Cantwell L, et al Optimizing outcomes in pediatric renal transplantation through the Australian Paired Kidney Exchange Program Am J Transplant 2017;17(2):534–41 https://doi org/10.1111/ajt.14041 21 Hotter A. The physiology and clinical implications of wound healing Part I. 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Short stature in advanced pediatric CKD is associated with faster time to reduced kidney function after transplant Pediatr Nephrol 2019;34(5):897– 905 https://doi.org/10.1007/s00467-018-4165-2 956 26 Francis A, Johnson DW, Melk A, et al Survival after kidney transplantation during childhood and adolescence Clin J Am Soc Nephrol 2020;15(3):392–400 https://doi.org/10.2215/CJN.07070619 27 Nelson DR, Neu AM, Abraham A, Amaral S, Batisky D, Fadrowski JJ. Immunogenicity of human papillomavirus recombinant vaccine in children with CKD. Clin J Am Soc Nephrol 2016;11(5):776–84 https://doi.org/10.2215/CJN.09690915 28 Nelson DR, Fadrowski J, Neu A. Immunogenicity of the meningococcal polysaccharide conjugate vaccine in pediatric kidney transplant patients Pediatr Nephrol 2018;33(6):1037–43 https://doi org/10.1007/s00467-017-3878-y 29 Kaur K, Jun D, Grodstein E, et al Outcomes of underweight, overweight, and obese pediatric kidney transplant recipients Pediatr Nephrol 2018;33(12):2353–62 https://doi.org/10.1007/s00467-018-4038-8 30 Winnicki E, Dharmar M, Tancredi DJ, Nguyen S, Butani L. Effect of BMI on allograft function and survival in pediatric renal transplant recipients Pediatr Nephrol 2018;33(8):1429–35 https://doi org/10.1007/s00467-018-3942-2 31 He S, Le NA, Frediani JK, et al Cardiometabolic risks vary by weight status in pediatric kidney and liver transplant recipients: a cross-sectional, single-center study in the USA. 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Repeat kidney transplantation after failed first transplant in childhood: past performance informs future performance Transplantation 2015;99(8):1700–8 https://doi.org/10.1097/ TP.0000000000000686 40 Butani L, Troppmann C, Perez RV. Outcomes of children receiving en bloc renal transplants from small pediatric donors Pediatr Transplant 2013;17(1):55– https://doi.org/10.1111/petr.12021 41 Matas AJ, Hays RE, Ibrahim HN. 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Warady Introduction Children and adolescents with end-stage kidney disease (ESKD) have severely impaired quality of life and poor psychosocial outcomes compared with the general population [1–6] Quality of life is worse in children on dialysis compared with kidney transplant recipients or those with chronic kidney disease (CKD) not yet requiring kidney replacement therapy [2, 4, 5, 7, 8] Children on dialysis report substantial decrements in the domains of pain, emotion, physical function, and peer and family interaction [2, 7, 8] Dialysis is highly burdensome, painful, and invasive Children on dialysis report a higher burden attributed to kidney disease and treatment compared with other stages of CKD [7, 9] Children on dialysis and their caregivers are required to manage multiple medications and adhere to dietary and lifestyle restrictions They have high rates of hospitalization, including for A Tong (*) The Children’s Hospital at Westmead, Centre for Kidney Research, Sydney, NSW, Australia e-mail: Allison.tong@sydney.edu.au A H Piebenga Parent of Child with Chronic Kidney Disease, Mt Pleasant, SC, USA B A Warady Department of Pediatrics, Division of Pediatric Nephrology, Children’s Mercy Kansas City, Kansas City, MO, USA e-mail: bwarady@cmh.edu surgical procedures and complications such as infection and hypertension [10–12], which can disrupt their daily activities at home, and social and school participation All these challenges are particularly difficult as children and adolescents are at the same time negotiating development tasks, milestones, and transition to adulthood In a recent study, children with CKD identified and prioritized the outcomes of survival, ability to participate in sports, fatigue, lifestyle restrictions, growth, kidney function, hospitalization, social functioning, medication burden, and infection, to be of highest importance, with lifestyle restriction indicated to be of greater importance in children on dialysis compared to other stages of CKD [13] Caregivers of children with CKD gave high priority to the outcomes of kidney function, survival, infection, anemia, growth, financial impact, cardiovascular disease, graft survival, impact on family, and blood pressure; reflecting concerns about their child’s prognosis and development [13] This reiterates the severe and broad-ranging impacts that dialysis can have on children and their caregivers This chapter will describe the spectrum of the lived experience of children on dialysis and their caregivers based on evidence from qualitative studies Qualitative studies can provide detailed and in-depth insights on the experiences, beliefs, and values of children on dialysis expressed in their own terms, which may often remain unspoken and underrecognized in clinical settings [14] The domains of the patient experience covered in © Springer Nature Switzerland AG 2021 B A Warady et al (eds.), Pediatric Dialysis, https://doi.org/10.1007/978-3-030-66861-7_50 957 A Tong et al 958 Body image and physical appearance Sickly and weak • Injustice • Being a burden • • Feeling different Prognostic uncertainty Relying on parental caregivers • Dependence on the dialysis machine • Unbearable and debilitating symptoms • Limiting future possibilities • • • • Social isolation Being absent from school Determination and selfawareness • Participating in activities • Hope for kidney transplant • Social support • Loss of control Lifestyle restrictions Coping strategies Managing treatment Taking ownership Communication and involvement in decision-making • Adhering to treatment • • Fig 50.1 Children’s experiences of living with dialysis this chapter will include: feeling different, loss of control, lifestyle restrictions, managing treatment, and coping strategies (Fig. 50.1) Parental experiences will also be summarized based on the existing studies [15, 16] and conveyed through real-life accounts from parents sharing their stories of caring for their child on dialysis (Boxes 50.1, 50.2, 50.3, 50.4, 50.5, and 50.6) Insights into the patient and caregiver experience can inform strategies to improve service delivery and policy for better outcomes in children and adolescents requiring dialysis, and their caregivers Box 50.1 Emotional Turmoil and Uncertainty • Initial Diagnosis: Lily’s Mother: My daughter, Lily, was 5 months old when she started hemodialysis Hemodialysis is a very risky procedure for an infant but was the only way to treat her condition I remember how shocked I was when we learned of Lily’s diagnosis and her path for treatment She would need dialysis and a g-tube to grow large enough to eventually receive a transplant Not only did I worry about the very long and difficult days that lie ahead, but I also worried about the kind of life she would one day lead I needed to know that there was a chance that she would be happy and “normal” one day Jacob’s Mother: My son, Jacob, was a healthy kid, with no remarkable health problems to speak of However, when he was 11, he seemed to be fighting a bug that he couldn’t quite shake, so his doctor ordered labs The labs revealed he was in renal failure, and he was rushed to the ER, taken straight to the ICU, and dialysis was started that very day When the kidney biopsy result came in, we had to adjust quickly to our new normal We would be going home with a kid that needed dialysis until he could eventually get a kidney transplant I remember fighting tears the entire time we were there for that first HD outpatient session I had to step out several times so that I didn’t upset my son Even though I had already witnessed him doing HD in the hospital, something about seeing the pro- ... their child’s prognosis and development [13] This reiterates the severe and broad-ranging impacts that dialysis can have on children and their caregivers This chapter will describe the spectrum of... their child is over 16 and then consider any substantial increases in terms of waiting time at this point if transplant surgery is still required However, it should be noted that because of age-related... if he/she is placed on dialysis pending transplant surgery The child’s family must often discuss this issue with the medical and psychosocial teams, with consideration of the individual family