Kids and Kidney Disease Catherine Picarelli, RN, BSN, CNN Hackensack University Medical Center Hackensack, NJ

Objectives List common causes of kidney disease in children. Name two differences in caring for children as compared to adults with kidney disease. Describe treatment options for children with stage 5 Chronic Kidney Disease.

Common Kidney Diseases in Children Nephrotic Syndrome Cystic/Hereditary/Congenital Glomerulonephritis Vasculitis Acquired Diseases

Nephritis /Nephrosis ‘itis ‘osis Glomerular inflammation Hematuria Proteinuria RBC casts Hypertension Renal Insufficiency Edema ‘osis ↑ Glomerular capillary wall permeability Proteinuria Hypoalbuminemia Edema Hyperlipidemia Lipiduria

Nephrotic Syndrome Applicable to any condition with heavy proteinuria, hypoalbuminemia, and edema Disorder of the glomerular filtration system May be primary or secondary to systemic disease

Minimal Change Disease 90% respond to steroids Remission achieved in 1-4 weeks Relapse when proteinuria and hypoalbuminemia recur Frequent relapses 2 or more episodes in 6 months 4 or more episodes in 12 months

Minimal Change Disease Treatment Steroids: Prednisone 2 mg/Kg/Day Cyclophosphimide Prograf Cellcept Rituximab

Steroid-Resistant Nephrotic Syndrome Due to FSGS MPGN Steroid-resistant MCD Alport Disease Diagnosis by kidney biopsy Known progression to ESRD 50% or more after 10 year follow-up

Steroid-Resistant Nephrotic Syndrome Treatment: Combined immunosuppression Prednisone Cytoxan/Cellcept Prograf/Cyclosporin ACE/ARB: decreases proteinuria to slow progression of disease

Steroid-Resistant Nephrotic Syndrome End- Stage Renal Disease (ESRD) Hemodialysis Peritoneal Dialysis Kidney Transplant Recurrence of FSGS with graft loss about 30-40% Recurrence of MPGN, type I, with graft loss about 30%

Causes of Renal Failure in Children Age 0-4:Genetic Causes, Congenital Defects Age 5-9: Dysplastic Kidneys, Hypoplastic Kidneys, Triad Kidneys Age 10-19:Glomerulonephritis Very rare for cause to be diabetes or hypertension

Effects of Renal Failure on Children Growth Problems School/Cognitive Problems Cardiovascular Infectious Complications Social Isolation Family/Financial Stress

Goals in Caring for Children Maximize growth and development potential Diminish behavioral, social, and family dysfunction Insure child has age appropriate equipment

Growth Issues Anorexia Behavioral aversion Acidosis Anemia Renal failure at times of growth spurts Supplement nutrition Monitor closely Correct acidosis Calcium/Phos control Growth hormone therapy

School/Cognitive Development Behind peers cognitively Missed school days Failing grades Coping skills limited Decreased social skills Treatment (anemia, uremia, sleep) Encourage school attendance (IEP, 504) Peer activities Summer camp

Cardiovascular Hypertension (LVH, Microvascular damage) Dyslipidemias Long-term mortality risk Keep BP <90th %ile Echocardiograms/ ABPM Low sodium/Low fat diet Regular exercise

Infection Decreased immunity in ESRD Access infections Second most common morbidity in children after CV Good nutrition Teach good hand washing Prompt recognition and treatment of infections

Gastrointestinal Constipation/ diarrhea Anorexia/nausea Feeding problems High fiber diet Regular bowel program PPI’s High incidence of G-tubes

Social Issues Child and family can become isolated Financial stress Insurance coverage Job instability Social work and Child life therapy involvement School support Prescribe covered medications FMLA

Treatment Options Hemodialysis Peritoneal Dialysis Transplantation No Treatment

Hemodialysis Small volume dialyzers/extracorporeal circuit Calculate prime / rinse volume Program machine in pediatric mode Adjust blood pump segment according to prescribed extracorporeal circuit Small amounts of fluid make a big difference Need precise weights Bicarbonate dialysate

Prime Normal Saline 5% Albumin Blood

Extracorporeal Blood Volume Blood volume of child is 80cc/kg body weight No more than 10% of blood volume should be in circuit during treatment Example-10kg child: 80cc x 10kg= 800cc blood volume No more than 80cc out during treatment

Blood Flow Rate 3-5 cc/kg per minute Access is the driver in rate Central venous catheters Grafts Fistulas

Monitoring Signs often subtle Watch closely Irritability Yawning Fidgeting Heart rate may change before BP drops

Peritoneal Dialysis Treatment of choice for infants and small children Peritoneal membrane in children is very large in relation to their BSA Usually high transporters Initial fluid volumes are 10-20mL/kg, then up to 40mL/kg

Peritoneal Dialysis CCPD CAPD Manual Machine programmed with prescription Maintain a running tabulation of UF CAPD Backup for power outages, vacations Manual Used in infants with fill volumes < 50mL

Renal Transplant Definitive form of RRT for children Preemptive transplant when possible

Transplantation Recipient 6 months/10kg Pt/Family able to comply with meds and follow-up Stable social/home situation

Donors Living Donor Deceased Donor Shorter waiting time/ischemic time Closer matches Better graft survival/overall outcomes Deceased Donor Advantage given to pediatric patients on waiting list

Bladder/Urology Many pediatric diseases are associated with bladder dysfunction Posterior urethral valves Severe vesico-ureteral reflux Other obstructions Dysfunctional voiding Inadequate bladder emptying

Bladder/Urology Collaborative evaluation and care between the Pediatric Urologist and Nephrologist Minimally invasive approach Bladder augmentation CIC, mitrofanoff Native nephrectomy

Immunizations/Viral Surveillance Assure pre-transplant immunizations No live-virus vaccines post transplant MMR Varicella Oral polio Determine pre-transplant viral exposure and antibody response EBV, CMV, HIV, Hepatitis, VZV

Additional Transplants Goal is to keep transplant as long as possible. Most children will require additional transplants.

Tips in Caring for Children Need a basic knowledge of developmental milestones and capabilities of the age child you are caring for. Explanations and teaching should be age appropriate.

Tips in Caring for Children Set limits and stick to them. Be firm- don’t let a child manipulate you. Consider having parents assist in getting a child to cooperate. Avoid patient/parent/staff power struggles.

Tips in Caring for Children Don’t feel sorry for them because they have renal failure. Don’t treat them as if they are sick. Treat them as much like their peers as possible. Expect them to behave as their peers would.

Tips in Caring for Children Expect cooperation- they will rise to the occasion and meet your expectations. Never lie! Telling a lie destroys trust with you. Determine activities and interactions based on developmental age- not chronological age.

Tips in Caring for Children Children like consistent routines. Explain what you are doing- before you do it. Don’t offer a choice if none are available. Treat each child as you would want your own child treated!