~I~ . \1 , :i

The Quality of Life After Liver Tra nspla n ta tion~:~ T. E. Slarzl, l. J. Koep, G. P. J. Schroler. J. Hood, C. G. Halgrimson, .. :~;

K. A. Porter, and R. Weil III -i.-:ii

To ASSESS the quality of life achieved responsible (T~ble I). Chronic rejection had~. with liv.er transplantation. we have destroyed the livers of five of the patients who:~-!

looked at 44 Jiver recipients who lived for at died of Jiver failure. .' .. ~ least I year after operation. Their subsequent The same kind of rejection also was tbe·~l1 survivability, what they have done with their chief diagnosis in 3 of the 4 livers that were' ~ lives. and what have been their handicaps and removed after 1-2Yz years and repJaced by .;~ triumphs will be the subject of this article. second transplants. These 4 patients with late . i~

CASE MATERIAL

Thc 44 I-year survivors repre.\enled a residual from 139 consecutive liver recipients treated 1-14'f- years ago ...... ith orthotopic liver transplantation. The story or those ... ho did notli"e for a year has been told c:lseh .... crc.1J and \Iie will not deal .... ith that herc. Ob\·iously. the gruup of 44 finalists "'erc highly preselected by their ability to survive the events surrounding and following operation.

PATIENTS DYING AFTER 1 YEAR

Causes of Death

Eighteen of the 44 patients died after passing the I-yea r mark (Table). Their total survival averaged about 2 Ye'drs with a range of) 21/" months to 6 year!'. There were always multiple causes of death. For example. infec­tion was almost always a factor in the finaJ events.

However. 10 Table ) we have listed the single most important factor in each case. Liver failure was the most common. but in three patients this clearly was due to biliary obstruction. and in two more. hepatitis was

From Iht' Dl'partnll'nl of S/lrgt'ry. [Nn"('r 1'l'ltrans Admini.<lrOliun Hospilal and Cni'''''S;I.\" of Colorado Mt'dica/ Cl'ntt'r. Dc-mN. Colo. and Ihr D.'panmrnt of Palhoiogy. 51. Marr's HUV';la/ Ml'dical School. London. Ellgland.

SUPf'Ortf'd in parI by r('s('arrh gran IS frum IIIr Vtlt'r­ans Admini.flralion; by Grants AM·J7:60 and AM-07772 from tht' ,,'aliono} }fL<litutI'S of Hralln: and by Grants RR-OOOjJ and RR..()()(J69 from IIII' Gmua/ Clinical Rl's('arcll c('nlUS Program of Ihl' Di";sion of Rt'Sl'arch Rt'SourCt'.<, Nalional In.slilu/~l of Hrolth.

Rl'print "qut.<u should br addrl'ssrd to T. E. Slor:l. DtpoTlmt'nt of Sur guy. Dl'm·u VrlrranJ AdminislrOlion Hospilal. Drnl't'r. Colo. 80120.

I: 1979 by Grunt' .r. Slrallon. I~. 0041-134-V7911101..()()j.~SOI.OO/O

252

rctransplantation for liver failure (orthotopic 3i't transplant nos. lOT] 13, J4, 54, 74) died ;':;; within 2 months after the retransplantations;;~ their deaths were due to technical complica-:.:~: tions. such as enteric fistulas. and to infec- ~ tions. Two patients each died from recurrent .~ cancer and overwhelming infections. . J,

Although the 4 patients with late ret Tans- .;; plantation derived little or no benefit from the ':!~ second liver. there were 2 others whose .. primary graft failed early after 5 and 9 weeks. Second transplants were successful for 11 and .~

15 months (OT 16 and 98. see Table 1) before ~,

the supervention of chronic rejection. . ;'\.

Assessment al I Year

Generally speaking. the patit!nts who died late: \\'ere already in trouble at the 14\'C3r . . ~ mark. Only 7 were thought to be satisfac·tory '~~ at that time (Table 2); The other) 1 were~t receiving too much predni!'one to have a good ''f: long-term outlook. Doses in individual cases : ~ are given in T:lble I. In the entire group of ':: 18, the prednisone doses at ) year averaged '.' 0.76 mg/kg/day l Table 2), Ten of the 18 pa- .. tients were jaundiced at I vear, with biliru- \ .. bins ranging from 2 to 40 (Table I). The -~' average bilirubin in all 18 patients was 9.2 • mg/ I 00 m!. ' •

Hospilali:ation ~;-

The generally poor course of the 18 pa- ~.: tients ..... ho died subsequent to ) year waS ,~~ reflected in their hospitalization times·i· (TabJe 3). During the first year. the)' "ere ~~ institutionalized an average of 54% of their ':~ time. Subsequent to 1 year. the)' still spent. \! major part of their time on hospital wards .~ ... (56%) until the time of their death. ..~-.

Transplanralian Proc~dmgs. Vol. XI. No.1 (Mltchl. 197' .. j

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254

Table 2. Status a' 1 Ya., of Survivors Who UYed Bayond This Time

18 Who DIed lJIte< 26SuilAIM

BiliNbin 9.2±13.1(SO) 1.« ± 2.5IS01·

(mgl100mO Prednisone 0_76 ± 061 ISO) 0.59 * 0.40 ISOI

Img/kglday) In trouble at 11118 3126

1 year

• At 1 year. only 3 of the 25 patients had bilirubins of 3 mgl100 ml ()( higha' (3. 3.5. and 12.6 mgll00 mil.

Rehabilitation

The combination of high-dose steroid ther­apy, suboptimal liver function, and a need for close medical scrutiny militated against good rehabilitation. The eight infants were hard to classify in this respect. Of the 10 preadoles­cents. teenagers. and adults, 60% returned to school or work for significant periods of time (Table 4).

The best reh:lbilitation was in 4 patients (Table 1) who. at I year, had excellent clinical results \10 i (h reasona bl e steroid doses and good E,er function (OT 19,27,36,78). One of the four developed recurrence of the duct-cell c:lrcin(lma. which had been the original indiC:lIion for operation (OT 78). Another patient was in perfect condition until 3 years postoperative, but sustained severe liver and renal damage after a nearly fatal Hemophilus infection. He died several weeks later. The liver showed chronic rejection (OT 19). One of these patients de\'cloped biliary tract obstruction that led to death despite futile efforts at secondary reconstruc­tion (OT 27). The fourth recipient had recur­rence of the .:hronic aggressive hepatitis. HBsAg-positivc. which had destroyed the native liver (OT 28).

r

ST ARZl. ET AI..

PATIENTS STILL ALIVE . :~l

Hospitalization :;5t The overall conclusion from the foregoing,;~

experience was that a poor long-term'prognO-.~·i sis could often be established by evaluation at ~:~ I year. The converse, namely a good progn()o"~; sis, was usually equally evident at 1 year, as .• could be identified in the 26 patients who are still alive. During the first year, these patients .­also spent a large amount of time in the ::: hospital, averaging 39% (Table 3). However, ~.~ they eventually became independent of insti· ;... tutions, and subsequently spent an average of-:. only 5% on hospital wards. Thus, they became . free to pursue normal interests. Eleven of the 26 p:ltients required secondary procedures in the biliary tract (Table 3, footnote), but this was usually completed before the end of the' fi rst yl!ar.

Assessment at J Year

The generally superior state of these 26 patients was easily quantified. At I year, only 3 were jaundiced (Table 2), and the average bilirubin in the entire group of 26 was 1.44 mg/ 100 ml. Finally, the prednisone doses were lower than in the patients who died after 1 year, averaging 0.59 mg/kg/day (Table 2).

It was not surprising to find retransplanta­tion less commonly represented in such patients still alive than in those who eventu­ally died. Only one patient still alive has had retransplantation. After her first graft failed in 23 months. a second liver has supported life for another 13 months. The second graft eventually became obstructed. causing intra­hepatic and subhepatic abscesses (Fig. I). Treatment was bile duct reconstruction.

Table 3. Hospitalization (% Time) of ,. Year Survivors Who Uved Beyond This Tim.

1 e Who died later' 26 Still alive·t

Flrs1 Ve.,

54% ± 29% ISOI· 39% ± 21% (501

Aft., 12 Month.

S6% ;to 40% ISOI 5% * 10%(501

• Nine of the 1 e patients had 1 or more reoperations exclusive of retransplantation. In four instances. the operation ... duct reconstruction.

t Thineen of the 25 patients had 1 or more 'eoperltions exclusive of retransplantation. Eleven of the reinterventions ...... for biliary tract problems.

AFTER LIVER TRANSPlANTATION 255

T.ble... Rehabilitation in 4-4 1-Year SUMvon·

18 Who Oit'd Aft", 1 V .. " 26Stln~

Number R.t .. , .... to Sehool

R.turned 10 Woril Numb«

Returned R."" .... to School toWoriI

.. ~ · .

Infants

5-18 Years

Adults

8 6 4

• Being housewife classified as wort!.. 1 Twel\,e no real rehabihtatlon.

o 3

o

fTwo no real rehabilitation: one retired. the other derelict.

.. ~- .

. :- Rthabililation · c· ;.; The dc!gree of rehabilitation was very high :. in those 26 patients still alive. The adults al-

I . most unifurmly rc!turncd to work. The adoles­. 'cents, tccn;Jgers. :1 nd children are or have I '_~.' been in public or special schools. The fact that

.'~!·so many .:hildren who were infants ultimately ~l"" ::) became studenls reflects the fact that there >~Yare more than a dozen 4-year survivors and 7 :l:.W"hO have been living for more than 5 years.

-,.,....:,; -.~.

!::.\ • -to;:': '" .

. :~:~-.~ : ~ .:; Fl9.'" Partl.' bill.ry ob.lrvcUon at Ihe cy.tlc duct :~ _ larrow, arter cholecy.tojejunoltomy. An InlrahepatJc L~ a.bsc:e •• developed (marked 1) as ... 11 as • subhep.tic: i~·:;.a.bsc:e .. (doubl. arrow). The homotl,aft .... a •• cond . ~~~ transplant. placed 23 monUls atte, the IIrst IInr grail. Th. :i.~ ,bIaart complication was lr8~I.d by op8caU". eonv.relon

'~.,Io chol.docltoleJunostomy.

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2

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12

6 0 4 2 2 8

One of our liver reCIpients had a normal baby in February 1977. She is now almost 4 years posttransplantation. Another patient is in midterm pregnancy.

MISCELLANEOUS OBSERVA nONS

Infants and children requiring long-term steroid therapy are apt to be small as exem­plified by our longest survivor whose liver replacement for biliary atresia was at the age of 3 years. She is now 8 years and 9 months postoperative. She attends public school and lives a normal life. She is only 3 feet, 2 inches tall. but she is growing steadily and has gained more than a foot in height during the

• last several years. As the song goes. there is a place for short people.

The same kinds of bone complications as seen in kidney recipients have been noted in the liver patients. Among the 18 patients who died after 1 year. there were 5 examples of osteoporosis and spontaneous fracture usually involving vertebrae. Such fractures have also been seen in 5 of the 26 patients still living. One of our patients, who is almost 6'/2 years posttransplant, is scheduled next month for bilateral hip replacement.

Psychiatric complications have been rela­tively uncommon among the recipients. One patient is a narcotic addict, a problem from which he suffered preoperatively. However, he is gainfully employed. Another patient, who is now 71/ 2 years posttransplant, has been a social delinquent and marijuana dealer. This latter young man stopped all medication for 8 months postoperatively and developed a rejection that was easily controlled by resumption of treatment.

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256

SUMMARY

The quality of life after liver transplanta­tion ranges from poor to superior. The social and vocational outcom'e is dependeilt on the quality, of homograft function and on the steroid doses necessary to maintain function. A good long-term prognosis is usually evident by 1 year postoperatively. The complete reha­bilitation of so many patients has encouraged

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, REFERENCES

~·t ..... ~.

: ......... .:.

~ ..... ~rt~ ... ';..:..'"

\. Slarzl TE. POrler KA. Putnam CWo et a1: Surl ~;~ GynccolObslcl 142:487. 1976 . -

2. Slarzl TE: Johns Hopkins Med J (in press) ;~~

3. Slarzl TE. Kocp U. Halgrimson CG. el al: Trans- < \:

plan I Proc (this issue) . , __ .. ~:.

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