Original Investigation

The Health Economic Impact of Nephrotic Syndrome

in the United States
Christine A. Simon ,1 Eloise Salmon ,1 Hailey E. Desmond,1 Susan F. Massengill ,2 Wilson P. Gipson,1 and
1
Debbie S. Gipson

Key Points
 Median annual direct costs (including medication, diet, emergency room visits, hospitalizations, and clinic vis-
its) were $3464 (interquartile range [IQR] $844–$5865) for adult patients and $1687 (IQR $1035–$4763) for
caregivers.
 The time spent planning/prepping meals was 183 h/yr (IQR 114–331) for adults and 173 h/yr (IQR 84–205) for
caregivers.
 Providers can better understand the burden of living with nephrotic syndrome, consider barriers when treating
patients, and develop supportive strategies.

Abstract
Background Nephrotic syndrome (NS) is a rare kidney syndrome with high morbidity. Although a common
contributor to the burden of chronic kidney disease, the direct and indirect costs of NS to patients and family
caregivers are unrecognized. The objective was to characterize the direct and indirect costs of NS to patients.

Methods Adults with NS and family caregivers of children with NS were eligible to participate if they had a
diagnosis of primary NS, had disease for at least 1 year, and had no other severe health conditions. Data-
collection surveys were generated with input from the Kidney Research Network Patient Advisory Board, and
surveys were mailed to the eligible participants. Participants were provided $50 for the return of completed
surveys. Costs were defined as either direct out-of-pocket costs or indirect costs (e.g., time). Descriptive statistics,
including percentage and median (interquartile range [IQR]) are reported.

Results Respondents included 28 adult patients and 17 caregivers of patients who were minors. Reported health
insurance coverage included 35 (78%) with private insurance, 12 (27%) with public insurance, six (13%) with
Children’s Special Health Care Services, and one (2%) uninsured. Median annual direct costs were $3464
($844–$5865) for adult patients and $1687 (IQR $1035–$4763) for caregivers. Of these costs, diet-associated costs
contributed $1140 (IQR $600–$2400). The most substantial indirect cost was from the time spent planning/
prepping meals (adults: 183 h/yr [IQR 114–331]; caregivers: 173 h/yr [IQR 84–205]).

Conclusions Adults and caregivers of children with NS face substantial disease-related direct and indirect costs
beyond those covered by insurance. Following replication, the study will help health care providers, systems,
and payers gain a better understanding of the financial and time burden incurred by those living with NS,
consider barriers when treating patients, and develop supportive strategies.
KIDNEY360 3: 1073–1079, 2022. doi: https://doi.org/10.34067/KID.0005072021

Introduction subtype of primary NS, with an incidence of approxi-

The clinical diagnosis of nephrotic syndrome (NS), mately 2 per 100,000 in the United States (2). In adults,
defined by hypoalbuminemia, hyperlipidemia, edema, the incidence of primary NS has been estimated at 3
and proteinuria, impacts individuals of all ages. Pri- per 100,000, with membranous nephropathy and
mary causes of NS are minimal change disease, focal FSGS each accounting for 30%–35% of NS cases, and
glomerulosclerosis (FSGS), membranous nephropathy, minimal change disease and immunoglobulin A
and hereditary nephropathies. When systemic dis- nephropathy each accounting for approximately 15%
eases such as diabetes mellitus or systemic lupus ery- of cases (3).
thematosus cause NS, it is labeled secondary NS (1). For all subtypes, primary NS is a chronic health
Primary NS was the focus of this study. In children, condition; it may be relapsing and remitting or
minimal change nephropathy is the most common progressive to kidney failure. Management strategies

1
Department of Pediatrics-Nephrology, University of Michigan, Ann Arbor, Michigan
2
Department of Pediatrics-Nephrology, Levine Children’s Hospital at Atrium Health, Charlotte, North Carolina

Correspondence: Dr. Eloise Salmon, University of Michigan, Division of Pediatric Nephrology, 1540 East Hospital Drive, Ann Arbor,
MI 48109. Email: [email protected]

www.kidney360.org Vol 3 June, 2022 Copyright # 2022 by the American Society of Nephrology 1073
1074 KIDNEY360

include disease-modifying therapies such as immunosup- Eligible participants received a paper survey and
pressive agents (e.g., cyclosporin, tacrolimus, mycopheno- postage-paid return envelope and were asked to return the
late, and rituximab), adjunctive therapies, and diet modifi- survey within 1 month. The survey took 60–90 minutes to
cations (4). complete. Upon return of a completed survey, participants
There is a paucity of literature addressing the time and received a $50 honorarium. The recruitment goal was 25
financial burdens of chronic conditions such as NS. adults and 25 family caregivers with completed surveys.
However, a health economics study focused on phenylke-
tonuria illustrated that there are significant direct (out- Data Organization, Classification, and Analysis
of-pocket) costs and indirect (time and opportunity loss) Data from completed surveys were entered into the
burdens associated with a condition whose management study REDCap database by a member of the study team.
also includes both health care visits and lifestyle changes, Costs were classified as either direct or indirect. Direct costs
including dietary modification (5). As a result, our study included out-of-pocket inpatient, outpatient, surgery, drug,
aimed to characterize the direct (out-of-pocket) and indi- and other health care service costs, whereas indirect costs
rect costs to patients and families affected by NS to under- were related to time lost due to NS, such as loss of produc-
stand better the financial implications and burden of tivity at work (7) (Figure 1). Indirect time costs were mone-
chronic illness and to guide strategies to address these tized using the average hourly earnings of January 2021
issues. from the US Bureau of Labor Statistics, with 1 day mea-
sured as 8 hours lost in average hourly earnings (8).
Descriptive statistics using percentage, median, and inter-
Materials and Methods quartile range (IQR) were calculated.
Survey Development
The Kidney Research Patient Advisory Board (PAB) (5)
had a key role in survey development. The PAB is com- Ethics
prised of volunteer patients and family caregivers and pro- The study was reviewed and approved as exempt by the
vides strategic leadership to the Kidney Research Network University of Michigan Institutional Review Board. Com-
regarding perspectives on selected research, educational, pletion of the survey served as consent to participate in this
and network initiatives. PAB members reflected on the study.
costs of NS and were asked: “How has kidney disease
impacted your/your family’s finances?” From this discus-
sion, NS cost-related concepts were defined and used to Results
modify “The financial and time burden associated with Overall, 89 adults with NS expressed interest in partici-
phenylketonuria (PKU) treatment in the United States pation, of whom 48 were eligible and 28 returned surveys.
survey,” with permission (6). Two 240-item surveys were With regard to caregivers, 81 expressed interest in partici-
designed: one for adults aged $18 years living with NS, pation, of whom 29 were eligible and 17 returned surveys.
and one for family caregivers of children aged ,18 years A summary of the patient demographics and clinical char-
living with NS. A summary of the survey content can be acteristics are presented in Tables 1 and 2. Of the 28 adults
found in Supplemental Tables 1 and 2. with NS and 17 family caregivers who responded to our
survey, 34 (76%) were women and 35 (78%) were non-
Recruitment Hispanic White. Thirty-five (78%) respondents had private
Survey respondents for this pilot study were recruited insurance only, 12 (27%) had public insurance, six (13%)
within nephrology practices, website advertisements had Children’s Special Health Care Services (CSH), and
on kidneyresearchnetwork.org and umhealthresearch.org, one (2%) paid for all costs out of their own pocket. In addi-
electronic study invitations through the Kidney Research tion, 17 (38%) participants had a household income of
Network registries, and Facebook NS support groups. #$50,000, and 40 (89%) patients had had NS for more than
3 years. The study included nine (20%) patients with
Eligibility ESKD, defined as dialysis or kidney transplant dependent.
To be eligible for this study, participants needed to be
$18 years old, have primary FSGS, minimal change dis- Annual Direct Costs
ease, IgM nephropathy, membranous nephropathy, or Median annual direct costs were $3464 ($844–$5865) for
childhood-onset idiopathic NS for at least 1 year, or be a adult patients and $1687 (IQR $1035–$4763) for caregivers.
caregiver of a child who has any of the above conditions Of these costs, diet-associated costs contributed $1140 (IQR
for at least 1 year, and reside in the United States. NS must $600–$2400) for adults and $750 (IQR $388–$1008) for
have been active on the basis of abnormal proteinuria or family caregivers. Furthermore, transplant-associated costs
NS-related therapy within the past 12-month period. Indi- (n53, 11% of adults, and n51, 6% of family caregivers)
viduals with CKD stages 1–5 were eligible, as were individ- contributed $3350 (IQR $1900–$5275) for adults and $1800
uals post transplant or receiving chronic dialysis. Exclusion for family caregivers (Table 3). All but one participant had
criteria included non-English-speaking individuals because insurance for health care costs. One family caregiver partic-
the surveys were only available in English, secondary NS, ipant paid $120 per year for all health care related to NS
and co-existing chronic illnesses such as diabetes, inflam- and attributed this low out-of-pocket cost residual cost bur-
matory bowel disease, cancer, etc. that may impart addi- den to supplementary insurance with CSH (Supplemental
tional economic impact nondistinguishable from NS. Table 3).
KIDNEY360 3: 1073–1079, June, 2022 Health Economics of Nephrotic Syndrome in the US, Simon et al. 1075

Indirect Costs

Missed
Speaking with
work/school days
insurance
Direct Costs

Nephrotic Diet &

Inpatient Syndrome lifestyle
and needs
Time spent shopping Outpatient
and prepping special visits Blood Draws
meals

Diagnostics Medication

Dialysis/ transplant

Quit work or
dropped out of Childcare
school

Figure 1. | Conceptual model of patient/family indirect and direct costs associated with nephrotic syndrome. These costs exclude costs
to insurance plans.

Table 1. Participant demographics

Respondent Type
Demographics All (N545) Adult (N528), n (%) Family Caregiver (N517), n (%)

Women 34 (76) 20 (71) 14 (82)

Race and ethnicity of respondent
White, not of Hispanic origin 35 (78) 20 (71) 15 (88)
Asian/Pacific Islander 3 (7) 3 (11) 0 (0)
Black, not of Hispanic origin 2 (4) 1 (4) 1 (6)
Hispanic 4 (9) 4 (14) 0 (0)
Mixed 1 (2) 0 (0) 1 (6)
Age at survey completion, yr
18–29 13 (29) 12 (36) 1 (6)
30–39 11 (24) 6 (21) 5 (29)
40–59 16 (36) 6 (21) 10 (59)
60–79 5 (11) 4 (14) 1 (6)
Education of respondent
High school/GED 10 (22) 6 (21) 4 (24)
2-year college/trade school/college certificate 10 (22) 7 (25) 3 (18)
4-year college 16 (36) 13 (47) 3 (18)
Master’s degree 5 (11) 1 (6) 4 (24)
Doctoral or professional degree (e.g., MD, PhD, JD) 4 (9) 1 (6) 3 (18)
Employment status of respondent
Employed full time ($40 hours per week) 21 (47) 13 (47) 8 (50)
Employed part time (,40 hours per week) 10 (22) 7 (25) 3 (19)
Not employed outside of the home 14 (31) 8 (29) 6 (31)
Insurance
Private insurance 35 (78) 23 (82) 12 (75)
Public insurance 12 (27) 7 (25) 5 (42)
Children’s Special Health Care Services 6 (13) 1 (4) 5 (42)
Self-pay or out-of-pocket 1 (2) 1 (4) 0 (0)
Household income, US$
#15,000 3 (7) 3 (11) 0 (0)
.15,000 to 25,000 4 (9) 2 (7) 2 (12)
.25,000 to 35,000 3 (7) 3 (11) 0 (0)
.35,000 to 50,000 7 (16) 4 (14) 3 (18)
.50,000 to 75,000 7 (16) 5 (18) 2 (12)
.75,000 21 (47) 11 (39) 10 (59)
1076 KIDNEY360

Table 2. Patient disease characteristics

Patient Type
Disease Characteristics All (N545) Adult (N528), n (%) Childa (N517), n (%)

Diagnosis
Focal segmental 18 (40) 12 (43) 6 (38)
glomerulosclerosis
Minimal change disease, 22 (49) 11 (39) 11 (65)
IgM nephropathy,
childhood-onset
nephrotic syndrome,
not otherwise specified
Membranous nephropathy 4 (9) 4 (14) 0 (0)
Unknown 1 (2) 1 (4) 0 (0)
Duration of disease, yr
1–2 5 (11) 4 (14) 1 (6)
3–5 13 (29) 6 (21) 7 (41)
6–10 11 (24) 7 (25) 4 (25)
111 16 (36) 11 (39) 5 (29)
ESKD 9 (20) 7 (25) 2 (12)
a
Parents filled out disease characteristics for their children because only those aged $18 years could fill out the survey.

Annual Indirect Costs education (n53, 11%), and decline of promotion at work
The most substantial indirect cost was time spent plan- (n52, 7%) due to kidney disease. Family caregivers
ning/prepping meals (adults: 183 hours/year [IQR reported premature discontinuation of education (n51, 6%)
114–331]; caregivers: 173 h/yr [IQR 84–205]). Adults and decline of promotion at work (n51, 6%) due to their
also spent more time visiting special services (median of 19 child’s kidney disease. In addition, children of the family
h/yr [IQR 17–30 h/yr]), whereas family caregivers spent a caregivers missed a median of four school days (IQR 0–13
median of 3 h/yr (IQR 2.4–6.5 h/yr). These special services school days) due to NS.
included nutrition counseling, behavioral therapy, social
work, the dentist, and more. The number of annual blood
draws was 12 (IQR 8–13) for adults and five (IQR 3–7 Diet
draws) for children (Table 4). Indirect costs from lost Diet-related responses are summarized in Table 5. Fif-
opportunities were reported by both adults and family teen (71%) adults and 14 (88%) family caregivers reported
caregivers. Adults reported premature discontinuation of that they or their child partially or fully adhere to their spe-
employment (n54, 14%), premature discontinuation of cial diets. A total of 13 (68%) adults and 13 (81%) family

Table 3. Annual direct out-of-pocket costs (US$)a for medical, diet, and other special products

Adult (N528) Family Caregiver (N517)

# Reporting, Costs (US$), # Reporting, Costs (US$),
Cost Category n (%) Median (Interquartile Range) n (%) Median (Interquartile Range)

Diagnosis 16 (57) 725 (89–3445) 9 (53) 140 (40–1000)

Total medication 25 (89) 210 (75–484) 16 (94) 54 (22–210)
Dialysis 5 (18) 0 (0–0) 2 (12) 850 (475–1225)
Kidney transplant 3 (11) 3350 (1900–5275) 1 (6) 1800 (1800–1800)
Diet 16 (57) 1140 (600–2400) 12 (71) 750 (388–1008)
Special producta 16 (57) 38 (0–73) 13 (77) 26 (0–50)
Emergency room 4 (14) 330 (160–521) 1 (6) 10 (10–10)
Hospitalization 6 (21) 918 (178–1485) 4 (24) 150 (83–1325)
Nephrology visit 26 (93) 80 (41–140) 16 (94) 76 (54–110)
Primary care provider visit 19 (68) 42 (23–92) 15 (88) 40 (21–83)
Specialist visitb 9 (32) 165 (72–360) 6 (35) 95 (39–485)
Psychiatry visit 4 (14) 15 (8–25) 0 (0) —
Other servicesc 8 (29) 90 (62–186) 3 (18) 70 (50–1135)
Annual total costs per patient 28 (100) 3464 (844–5865) 16 (94) 1687 (1035–4763)
a
Special products include blood pressure monitors, urine dipsticks, and other cleaning products.
b
Specialists include geneticists, cardiologists, dermatologists, pulmonologists, and optometrists.
c
Other services include nutrition counseling, genetic counseling, behavioral therapy, social work, and the dentist.
KIDNEY360 3: 1073–1079, June, 2022 Health Economics of Nephrotic Syndrome in the US, Simon et al. 1077

Table 4. Annual indirect costs of nephrotic syndrome

Adult (N528) Family Caregiver (N517)

Time Reported Time Costs Time Reported Time Costs
Median (US$) Median Median (US$) Median
# Reporting, (Interquartile (Interquartile # Reporting, (Interquartile (Interquartile
Activity n (%) Range) Range)a n (%) Range) Range)a

Emergency room, h 4 (14) 7.4 (5–10) 222 (150–300) 1 (6) 5 (5–5) 150 (150–150)
Hospitalization, d 7 (25) 4 (2–13) 960 (480–3121) 4 (24) 2.5 (1–4) 600 (240–960)
Nephrology visit, h 26 (93) 2.5 (2–4) 75 (60–120) 16 (94) 3.5 (2–4.1) 105 (60–123)
Primary care provider 22 (79) 1.3 (1–2) 39 (30–60) 16 (94) 1.5 (1–2.1) 45 (30–63)
visit, h
Specialists visitb, h 11 (39) 4.5 (3–7) 135 (90–210) 7 (41) 4 (1.8–5.5) 120 (54–165)
Other services visitc, h 7 (25) 19 (17–30) 570 (510–900) 3 (18) 3 (2.4–6.5) 90 (72–195)
Traveling and shopping 14 (50) 19 (3–48) 570 (90–1440) 12 (71) 12 (4.6–45) 360 (138–1350)
for special foods, h
Planning and preparing 13 (46) 183 (114–331) 5492 (3421–9933) 10 (59) 173 (84–205) 5,204 (2509)
special meals, h
Speaking with insurance, h 26 (93) 0 (0–1) 0 (0–30) 17 (100) 0.5 (0–1.5) 15 (0–45)
Work reduction, h 6 (21) 9 (4–18) 270 (120–540) 2 (12) 10 (7.5–12.5) 300 (225–375)
Work absentee, d 28 (100) 1 (0–12) 240 (0–2881) 15 (88) 5 (0–9) 1200 (0–2161)
School absentee, d 8 (29) 4 (2–9) — 0 (0) — —
Blood draws 26 (93) 12 (8–13) 360 (240–390) 16 (94) 5 (3–7) 135 (90–195)
a
$30.01 mean hourly earnings 2021 in US$.
b
Specialists include geneticists, cardiologists, dermatologists, pulmonologists, and optometrists.
c
Other services include nutrition counseling, genetic counseling, behavioral therapy, social work, and the dentist.

caregivers acknowledged that this special diet is very or of $3960 (IQR $3464–$7109) compared with $4985
extremely important. Sixty-two percent (n58) of adults and (IQR $4343–$5628). In addition, non-ESKD adults spent
75% (n56) of family caregivers responded that special diet 182.5 h/yr (IQR 121.7–285.1 h/yr) planning and prepping
products were difficult to acquire due to availability, special meals, and caregivers of non-ESKD children also
whereas other factors such financial reasons, distance, and spent 182.5 h/yr (IQR 91.3–250.9 h/yr) planning and prep-
time played a role in complying with the diet. Although ping special meals. Whereas ESKD adults spent 228 h/yr
few adults (n55, 25%) asserted that following a special diet (IQR 160–297 h/yr), a single caregiver, whose child had
was difficult or very difficult, more family caregivers (n57, ESKD, spent 150 h/yr.
44%) claimed that it was difficult for their child to follow
this diet. All reported that following the special diet was Influence of Insurance Status
difficult due to the burdens of the diet itself (100%). How- Direct out-of-pocket costs also varied by insurance type
ever, 16 (94%) family caregivers reported that it is not diffi- (Supplemental Tables 8 and 9). Among non-ESKD adults,
cult or somewhat difficult to meet their child’s needs, and individuals with private insurance alone had higher medi-
11 (65%) family caregivers claimed that their child needed cation, hospitalization, and visit costs that non-ESKD adults
no more or a little more care than other children. Through with at least some public insurance. Similarly, caregivers of
the free-text survey section, those with difficulty purchas- non-ESKD children with private insurance alone had
ing items and following the special diet reported specific higher medication costs than those with public insurance.
reasons for this difficultly. One participant explained, “I’m
unemployed due to the disease and the sole income earner.
Food banks do not cater to special diets.” Another partici-
Discussion
pant discussed loss of social opportunity for the child at Adults and caregivers of children with NS face substan-
school because the child cannot eat the same food as other tial disease-related direct and indirect costs far exceeding
children in the cafeteria (see Supplemental Table 3). costs covered by insurance. Adult patients experienced
more costs when compared with pediatric patients, with
Comparison of non-ESKD to ESKD Costs median annual direct cost of $3364 (IQR $844–$5865).
Within this study sample, both ESKD adults and care- Annual diet costs were a significant proportion of this cost
givers of ESKD children had more direct costs than for both adult patients $1140 (IQR $600–$2400) and
non-ESKD adults and caregivers of non-ESKD children caregivers $750 (IQR $388–$1008) and required a time com-
(Supplemental Tables 4–7). Non-ESKD adults faced more mitment of 183 h/yr (IQR 114–331 h/yr) for adults and
direct costs than caregivers of non-ESKD children, with a 173 h/yr (IQR 84–205 h/yr) for caregivers. The time
median of $2594 (IQR $728–$4881) compared with $1217 commitment was primarily due to limited availability of
(IQR $608–$4214). However, ESKD adults had fewer direct recommended food products, thereby necessitating home-
costs than caregivers of ESKD children, with a median prepared meals; likewise, respondents found that
1078 KIDNEY360

programs are also present in other states. The benefits

Table 5. Difficulty with nephrotic syndrome diet
include coverage of specialty medical bills and co-pays and
Adult, Family Caregiver, deductibles from private or public insurance for children
Question about Diet n (%) n (%) aged ,21 years with at least one of more than 2700 chronic
health conditions (9). A qualifying diagnosis is dependent
Have you/your child followed on the type, severity, chronicity of medical condition, and
a special diet? the need for pediatric specialty care (10). In this pilot study,
# of respondents 21 16 we could not quantify the impact of CSH, given the fre-
No 6 (29) 2 (13)
quency of overlap of public insurance and CSH participa-
Yes, partially 11 (52) 8 (50)
tion among respondents. Future investigations should
Yes, fully or daily 4 (19) 6 (38)
How difficult is it to obtain explore associations between insurance type, including
any part of the special diet? entities such as CSH, and out-of-pocket costs in a larger
# of respondents 21 16 cohort of individuals with NS.
Not difficult 8 (38) 8 (50) To our knowledge, there are no published studies that
Somewhat difficult 6 (29) 7 (44) have described the direct out-of-pocket and indirect costs
Difficult 5 (24) 0 (0) of NS in either the United States or other countries. How-
Extremely difficult 2 (10) 1 (6) ever, out-of-pocket costs have been described for other
Why is it difficult to get chronic diseases. For instance, the annual direct out-of-
special diet products?
pocket costs of asthma, which included medication, office
# of respondents 13 8
visits, hospitalizations, and emergency room visits were
Financial reasons 6 (46) 3 (38)
Availability of product 8 (62) 6 (75) $3761 for adults and $1737 for children (11). A phenylke-
Distance 2 (15) 1 (13) tonuria study illustrated that respondents spent $1961 for
Time 4 (31) 3 (38) low-protein foods and spent more than 300 hours shopping
How difficult is it for you/ and preparing special diets in a year (5). These costs are
your child to follow the comparable to the annual direct costs of adults and care-
recommended diet? givers with children living with NS reported in this study.
# of respondents 20 16 Participants in this study also reported a significant time
Not difficult 3 (15) 4 (25) burden attributed to NS. These indirect costs were related
Somewhat difficult 12 (60) 5 (31)
to dietary needs, medical appointments, and travel time.
Difficult 4 (20) 4 (25)
Furthermore, respondents reported opportunity costs from
Very difficult 1 (5) 3 (19)
Why are you/your child NS on their personal education with premature withdrawal
not able to closely follow from school and employment, missed promotions, and
the recommended diet? early retirement. Adults with ulcerative colitis reported a
# of respondents 4 4 median of eight medical-related absenteeism days and
Cost of diet 1 (25) 2 (50) incurred $5307 in indirect costs per year (12). However,
Diet is burdensome 3 (75) 4 (100) adults with NS reported one missed workday per year,
Emotional or social factors 2 (50) 1 (25) which resulted in a median of $240 lost per year. Further-
How important do you feel more, caregivers with children who have hemophilia
this diet is for your/your
missed a median of 3.2 days of work (13). In comparison,
child’s health?
caregivers with children with NS missed a median of
# of respondents 19 16
Somewhat important 1 (5) 1 (6) 5 days. Lastly, children of the family caregivers missed a
Important 5 (26) 2 (13) median of 4 days of school (IQR 0–13 days of school) due
Very important 7 (37) 8 (50) to NS, whereas about 13% of students in the United States
Extremely important 6 (32) 5 (31) missed 3–4 days of school in 2015 (14).
This study has some limitations relating to sample size
and recall bias. We conducted this as a pilot study to assess
adequately tasty options that met their restrictions (e.g., both the feasibility of data collection and to generate cost
low sodium) often cost more (see Supplemental Table 3) estimates. We enrolled fewer than the goal of 25 adults and
Although few adults (25%) asserted that following a special 25 family caregivers completing the surveys because the
diet was very difficult, more family caregivers (44%) study was prematurely terminated due to the onset of the
claimed that it was very difficult for their child to follow coronavirus disease 2019 pandemic and potential influence
this diet. Lastly, adults and children who had reached on health care utilization. Although the survey was thor-
ESKD had more direct out-of-pocket costs than those who ough and covered potential burdens, it was quite long. As
had not reached ESKD. This variation in cost burden by a result, participants with the most severe disease may
severity of disease highlights the heterogeneity of the pri- have been more likely to decline participation.
mary NS population, and future work should continue to This study is the first to characterize the out-of-pocket
characterize the experiences of both ESKD and non-ESKD direct and indirect costs, which can assist with decision
individuals. making in regard to NS management strategies and sup-
The children’s cost may be in part less than adults due to port services needed for implementation of recommended
the availability of CSH. This program is provided by the therapies (15). Replication of this work utilizing a larger
Michigan Department of Health and Human Services as and more diverse patient sample will be beneficial. Further-
part of Title V of the Federal Social Security Act. Similar more, future studies enumerating the costs to the health
KIDNEY360 3: 1073–1079, June, 2022 Health Economics of Nephrotic Syndrome in the US, Simon et al. 1079

system and payers from health care utilization on a patient Supplemental Table 5. Adult: Non-ESKD versus ESKD indirect
and national level are needed to complement this study costs (US$).
and generate a comprehensive understanding of the Supplemental Table 6. Caregiver: Non-ESKD versus ESKD
economic impact of NS as a chronic disease. direct costs (US$).
Supplemental Table 7. Caregiver: Non-ESKD versus ESKD indi-
Disclosures rect costs (US$).
D.S. Gipson reports consultancy between the University of Michi- Supplemental Table 8. Non-ESKD adult: direct out-of-pocket
gan and AstraZeneca, Boehringer-Ingelheim, Genentech, Goldfinch costs (US$) by insurance type.
Bio, Roche, Travere, and Vertex (no individual consultancy agree- Supplemental Table 9. Non-ESKD caregiver: direct out-of-
ments); research funding to the University of Michigan from Boeh- pocket costs (US$)a by insurance type.
ringerIngelheim, Goldfinch Bio, Novartis, Reata, and Travere; being
a scientific advisor for or a member of AstraZeneca, Goldfinch Bio, References
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Supplemental Table 3. Selected free text responses by cost
domain. Received: September 28, 2021 Accepted: April 25, 2022
Supplemental Table 4. Adult: Non-ESKD versus ESKD direct See related editorial, “The Forgotten Cost of Nephrotic Syndrome
out-of-pocket costs (US$). to Patients and Caregivers in the United States”, on pages 991–992.