NOVEMBER/DECEMBER2006

What’s Inside:Tube Talk

Page 3

Tube FeedingWorkshops

Page 3

Lending a HelpingHand

Page 4

Conference DVDSummaries

Page 5

Equipment ExchangePage 5

Join Oley in PhoenixPage 5

HPN Centers ofExperience

Page 6

Welcome AboardPage 6

Be a Star!Page 6

Spotlight on NealSamara

Page 7

Contributor NewsPage 11

Toll-free NetworkingPage 12

Liver Disease cont., pg. 2 ☛

Parenting cont., pg. 8 ☛

Parenthood and TPNLiz Tucker, HPN Consumer and Parent

Over the last six months or so, we have had severalrequests from young women on TPN with questionsabout pregnancy,parenting and TPN.This article is a com-pilation of the ques-tions, concerns and ex-periences of the fol-lowing TPN consum-ers: Bettemarie Bond,Dannene Bone,Malisa Matheny, SallyMoore, CandaceVerners, David Youngand myself. Obviouslywe won’t have all thepotential questions and answers, but we hope we willgive you something to think about.Start Thinking Ahead

Everyone agreed that parenting is not a decision tobe made lightly or quickly. Give yourself plenty of

David, Cheryl & Ryan Young

The Cleveland Clinic’s Experience with HomeParenteral Nutrition Associated Liver DiseaseTherese Austin, MS, RD, LD, CNSD; Douglas L. Seidner, MD, FACG, CNSP

Parenteral nutrition (PN) is a lifesaving treatmentfor patients with intestinal failure who are unable toingest or absorb an adequate amount of nutrition,fluid and electrolytes from their diet. While thebenefits of this therapy are obvious, the complexnature of providing intravenous nutrition is accom-panied by a variety of problems, the most commonbeing infections and mechanical complications asso-ciated with the vascular access device used to receivethis therapy. Metabolic complications include fluidand electrolyte abnormalities which are usually asso-ciated with the underlying disease. One of the moreserious metabolic complications is the developmentof liver disease. In this article, we will discuss theexperience of the Home Parenteral Nutrition Serviceat the Cleveland Clinic. These results were recentlypublished in the Journal of Parenteral and EnteralNutrition, a professional publication of the Ameri-can Society of Parenteral and Enteral Nutrition.

How Common Is Liver Disease?Numerous studies have been conducted to investi-

gate how many patients on long-term PN (usuallydefined as 6 months or more) develop liver test abnor-malities and significant liver disease to help determinethe cause of this problem. Some of these studies haveshown an incidence of liver test abnormalities rangingfrom 25 to 100 percent and that advanced liver diseasemay occur in 15 to 40 percent of these patients.

The Home Parenteral Nutrition (HPN) programat the Cleveland Clinic is one of the largest HPNprograms in the country. A computerized medicalrecord has been used to help us manage consumerswho require long-term PN. We conducted a studyto investigate the prevalence of abnormal liver enzymesand advanced liver disease in an attempt to identifyrisk factors that might be associated with theseabnormalities. The study was done by querying our

Annual Conference ‘07Plan to join us June 27 to July ,1 2007 at the Cape

Codder Resort and Spa (www.capecodder .com)resort inHyannis, MA, on Cape Cod. Offering crisp oceanair and rolling sand dunes, scenic back roads andquintessential charm,endless attractions andgreat shopping — CapeCod is the perfect set-ting. Your days arecrammed with opportu-nities to learn moreabout the therapy thatsustains you, and yourevenings filled with greatfellowship and plenty ofopportunities to relaxand enjoy!

The conference rate at the Cape Codder is $129 forsingle or double occupancy and is being offered toattendees three days pre and post conference. Hurry!A limited number of rooms have been reserved at thisreduced rate. Book now by calling (508) 771-3000.

Conference cont., pg. 6 ☛

Cape Cod

LifelineLetterLiving with home parenteral and/or enteral nutrition (HPEN)

Medical Update

2 — LifelineLetter • (800) 776-OLEY November/December 2006

Liver Disease, from pg. 1

LifelineLetterNov/Dec 2006 • Volume XXVII, No. 6

Publisher:The Oley Foundation

For Home Parenteral and Enteral Nutrition214 Hun Memorial, MC-28

Albany Medical CenterAlbany, NY 12208

1-800-776-OLEY, or (518) 262-5079Fax: (518) 262-5528

www.oley.org

Executive Director:Joan Bishop

BishopJ@mail.amc.edu

Director of Communications & Development:Roslyn Dahl

DahlR@mail.amc.edu

Volunteer Editor:Elizabeth Tucker

evtucker@charter.net

Outreach CoordinatorKate Swensen

SwenseK@mail.amc.edu

Administrative Assistant:Cathy Harrington

HarrinC@mail.amc.edu

Lifeline Advisory Group:Felice Austin; PatriciaBrown, RN, CNSN; Ben Hawkins;Alicia Hoelle; Robin Lang; Jerry Mayer; Rex Speerhas,RPh; Stephen Swensen; Ann Weaver

Oley Board of Trustees:Rick Davis, President; Darlene Kelly, MD, Vice President;Laura Ellis, PhD, RD, Treasurer; Michael Medwar, Secretary;Jane Balint, MD; Alan Buchman, MD; Jane Golden; KishoreIyer, MD; Jim Lacy, RN; Sheila Messina, RN, MA; DougSeidner, MD

Subscriptions:The LifelineLetter is sent free of charge to those on homeparenteral or enteral nutrition. For all others, the annualrate due each January is $40.* * * * *

The LifelineLetter is the bi-monthly newsletter of theOley Foundation. Items published are provided as anopen forum for the homePEN community and shouldnot imply endorsement by the Oley Foundation. Allitems/ads/suggestions should be discussed with yourhealth care provider prior to actual use. Correspondencecan be sent to the Director of Communications &Development at the address above.

Liver Disease cont., pg. 10 ☛

computerized medical record for consumerswho had received HPN for at least 6 monthsfrom July 1991 through June 2002.

For the purpose of our study we classifiedliver tests into two main categories; eitherliver associated enzymes (LAEs) or liver func-tion tests (LFTs). LAEs include aspartateaminotransferase (AST), which reflects in-

flammation or injury to hepatocytes (one ofthe major cells within the liver) and alkalinephosphatase (AP), which reflects inflamma-tion or obstruction of the biliary system of theliver. We categorized the degree of abnormal-ity for these labs as mild, moderate or severedepending on the degree of elevation (seetable 1). LFTs were defined as blood tests thatreflect the metabolic capacity of the liver andinclude bilirubin, albumin and prothrombintime. These labs were combined to determineoverall liver function. This measure was onlyfelt to be important when the bilirubin wasover 3 mg/dL, the albumin was less than 3.2mg/dL and the prothrombin time was morethan 3 seconds prolonged. Patients with theseresults were defined as having severe liverdysfunction. (Note: these values vary slightlyfrom table 1 because they are based on a Childs-Turcotte-Pugh score.) Patients with a knownliver disease, drug induced liver injury, alcohol-

ism and cancer, all of which can cause anelevation in LAEs, were excluded from thestudy. Patients with a transient elevation in LAEswere excluded by only studying patients whohad lab abnormalities on more than one occasionover a period of at least 6 months. In addition tothese laboratory results we gathered informationon the patient’s age, gender, underlying disease,indication for PN and PN formula.

There were a total of 162 patients thatconstituted the study group. The clinical char-acteristics of these patients are shown in table2. The average duration of PN in these pa-tients was 2.14 years. While this may seemrelatively brief, this reflects the fact that manypatients are able to discontinue PN withinone year. Many patients were on PN for morethan two years and one patient was on therapyfor more than 10 years. We found that 92(57%) patients had chronically abnormal LAEs

Table 2: Clinical Characteristics of 162 Cleveland Clinic PatientsReceiving Long-term PN

DemographicsAge 53 years +/- 14 yearsMale 60Female 102

Underlying DiagnosisInflammatory bowel disease 56Mesenteric ischemia 15Radiation enteritis 9Other gastrointestinal disorders 32Other medical disorders 43

Indication for parenteral nutritionMalabsorption 79Fistula 35Bowel obstruction 20Other 18

*Diagnosis was only available for 155 patients and indication for parenteral nutrition wasonly available for 152 patients.

Table 1: Categorization of Liver Test Levels

Mild Moderate SevereLiver-Associated Enzyme (LAEs)

aspartate aminotransferase upper normal to 2-5 times >5 timesalkaline phosphatase <2 times normal normal normal

Liver Function Tests (LFTs)total bilirubin 1.5-1.9 mg/dL 2-3 mg/dL >3 mg/dLalbumin 3.6-3.9 mg/dL 2.8-3.5 mg/dL <2.8 mg/dLprothrombin time 13-16.9 sec. 17-19 sec. >19 sec.

Note: these levels were used for the purpose of this study and are not used for general medical care.

Volume XXVII, No. 6 (800) 776-OLEY • LifelineLetter — 3

HEN Tips

Tube TalkThank you to everyone who sentmaterial for the “Tube Talk” column. Any-one who is interested in participating can send theirtips, questions and thoughts about tube feeding to: TubeTalk, c/o The Oley Foundation, 214 Hun Memorial MC-28, Albany Medical Center, Albany, NY 12208; or E-mailDahlR@mail.amc.edu. Information shared in this columnrepresents the experience of that individual and shouldnot imply endorsement by the Oley Foundation. The Foun-dation strongly encourages readers to discuss any sugges-tions with their physician and/or wound care nurse beforemaking any changes in their care.

Clothes Hold Tubes, IV Lines, BagsThe BundieBaby is a onesie-like outfit designed for children with G/

J-tubes, ostomy bags, bile bags, central lines, and/or other medicaldevices attached to the torso. Jody Williams designed the outfit toovercome some of the challenges she faced as a foster parent ofmedically fragile infants; namely, keeping active infants/toddlers safefrom tugging on necessary tubes, lines and bags, and comfortableduring frequent changes due toleaks or doctor exams.

The front panel is equippedwith two special pockets. Asmaller front pocket on the leftstores gastrostomy tubing, andcan hold a standard nursing padas well to absorb leaks. (see photo)A larger pocket behind the G-tube pocket accommodates os-tomy bags, bile bags or othermedical devices attached to thetorso. The inside side seams haveties to secure a central line orapnea monitor leads.

The Bundie’s front panel canbe removed and replaced with a clean panel without lifting or disrobingthe wearer. The armholes open completely for ease in dressing,especially those with IV lines attached to the arms. The snaps are plasticso the outfit can be worn during MRI testing. For more informationor to order, visit www.BundieBaby.com or call (410) 884-0982.

Tube Feeding WorkshopsA series of workshops designed to teach professionals and parents about

the medical and social aspects of tube feeding will be held in several citiesin 2007: January 20-21 in San Diego, CA; March 9-10 in Birmingham,AL; and October 25-26 in Cincinnati, OH. Entitled “Tube Feedingwith Love,” the workshops feature Marsha Dunn Klein, MEd, OTR/L, a pediatric occupational therapist who specializes in feeding infantsand young children. Attendance costs $350 for professionals and $200family members. CEUs are available. For more information call (520)323-3348 or visit www.mealtimenotions.com.

The BundieBaby accommodatestubes, IV lines and ostomy bags.

Oley News

4 — LifelineLetter • (800) 776-OLEY November/December 2006

Lending a Helping HandOley has many volunteers who generously give of their time and

talents to support Foundation programs. Some take the toll-free lineeach month, Regional Coordinators offer education and support,

while others write articles for the news-letter or help organize conference events.Here we’d like to offer a special thanksto our “behind the scenes” volunteerswho help at the Oley office.

Nancy Linsley has come to the Oleyoffice every Wednesday, since Ellie Wil-son (former Outreach Coordinator) firstintroduced her to us four years ago. Shehelps the Oley staff by addressing thehundreds of information requests wereceive each month. Nancy is also in-strumental in getting out large mailings,and prepar-

ing staff materials for special events. Sheis loads of fun and raises our spiritstremendously.

Blanche Hoffman is another terrifichelper who volunteers at the officeregularly, doing mailings and the like.In addition to her sense of humor,Blanche offers the understanding thatcomes with living with a husband whohas been on tube or IV therapy for morethan ten years. We are so happy to have her

on board!A third volunteer, who helps out in

the office whenever we have specialprojects, is Joyce Hydorn. Joyce and herhusband Bob have also been a big help atlocally-held events and conferences. Joyceis a long-term TPN patient, and beganvolunteering after her health preventedher from working full time.

A good friend of Joan Bishop, (Oley’sExecutive Director,) Marjorie Quinnhas a warm personality that’s endearedher to therest of the

staff and many Oley members. Marjoriehas worked on a number of projects in theoffice, and helps out at the annual confer-ence and local events too. She attendedher first conference in 2003and has helpedat every conference since – donating hertime as well as paying her own travelexpenses! She is very dedicated to Oley.

We are grateful to all of our volunteersfor their many contributions. Truly wecould not run Oley without you. Marjorie Quinn

Joyce Hydorn

Blanche Hoffman

Nancy Linsley

Oley News

Volume XXVII, No. 6 (800) 776-OLEY • LifelineLetter — 5

Don’t Miss Us in Phoenix!Start the new year out right by joining Oley on January 27,

2007 in Phoenix, Arizona! This one-day regional conference willshow you what’s new in homePEN therapy for 2007. Topicsinclude the emergent therapies, Pre/Probiotics, as discussed by KellyTappenden, RD, PhD and Growth Fac-tors: GLP2 and Teduglutide, coveredby Palle Jeppesen, MD, as well as Com-plications Associated with Vascular Ac-cess, by Sheila Messina, RN. Roundtablesessions will be hosted by Lyn Howard, MDand Jane Balint, MD, on Pediatric Issues; Kishore Iyer, MD,on Intestinal Failure; Doug Seidner, MD, on AvoidingComplications; and Sheila Messina, RN, on Sharing from theConsumer Perspective. Registration begins at 9:00 a.m. andthe program will run from 10:00-4:30 p.m. Walk-ins arewelcome. Stay tuned to www.oley.org for the exact location ofthe conference and for program updates.

Oley’s conference has been developed in conjunction with theannual meeting of the American Society for Enteral and ParenteralNutrition (A.S.P.E.N.). A.S.P.E.N.’s “Clinical Nutrition Week”runs from January 28-31 at the Phoenix Civic Plaza. We inviteyou to join us there too by volunteering at Oley’s booth inA.S.P.E.N.’s exhibit hall. Don’t miss this rare opportunity to beamong hundreds of exhibitors showcasing their newest prod-ucts. Volunteers are needed January 28, 29 and 30 to work oneor more shifts at the Oley booth from 9:30 a.m. to 11:15 a.m.,12:15 p.m. to 1:45 p.m. and/or 3:00 p.m. to 4:15 p.m. each day.For more information or to volunteer, contact Kate Swensen at(800) 776-OLEY or email Swensek@mail.amc.edu.

Equipment ExchangeNewly available items offered free of charge include:

Formula:• 3 cases Complete, exp.4/07• 6-7 cases Elecare Pediatric Formula (powder), exp. 8/07• 3 cases Jevity 1.2, exp. 7/07• 5+ cases Jevity 1.5, exp. 8/07• 3 cases Fibersource HN, exp. 11/07• 4 cases Glucerna, exp. 1/07 & 4/07*• 2 cases Osmolite 1 Cal, exp. 7/07• 9 cases Osmolite 1.2 Cal, exp. 4/07*• 5+ cases Pediasure, vanilla, exp. 7/07 & 8/07*• 14 cases Peptamen, exp. 7/07 & 8/07• 4 cans powder, Portagen, exp. 8/07 &1/08• 6.5 cases Promote with fiber, exp. 3/07• 2 cases Promote, exp. 1/07• 3 cases Pulmocare, exp. 3/07• 4.5 cases Traumacal, exp. 2/07 & 9/07

Bags/Tubes:• 17 Kangaroo 1000 ml bags• 30 Kangaroo gravity feed sets, 1000 ml, #8884702500• 30 Ross Easy Feed Enteral Bag, 1000 ml, #56• 1 Viasys enteral feeding tube w/o stylet, 8 Fr., 36”, #20-1368• 56 Zevex Infinity 1200 ml bags, #INF1200*

*multiple donors

MORE SUPPLIES are available! Visit www.oley.org, or contactour volunteer, Ben Hawkins (benhawkins@fuse.net; toll free 866-454-7351). This number reaches Ben’s home, so PLEASE, CALLBEFORE 9 P.M. EST! (Not CST as incorrectly printed last issue.)Oley cannot guarantee the quality of the supplies donated or beresponsible for their condition. We ask that those receiving goods offerto pay the shipping costs.

Summaries of ConferenceSessions Available

Summaries of the 2006 Oley Conference sessions that were filmedfor DVD are available. Sessions include: What Is Rational Manage-ment?, Being All That YouCan Be, Reducing HPNDependency, What AreProbiotics, MicroNutrients,and the History and Futureof HPN. The summaries canhelp you decide which DVDyou’d like to borrow fromthe Oley Library.

Summaries of other OleyDVDs/videos are cominglater in 2007. To read the summaries or order the DVDs, visitwww.oley.org or contact Cathy Harrington at (800) 776-OLEY.

Regional News

6 — LifelineLetter • (800) 776-OLEY November/December 2006

Be a Star!Your face could be the new face of Oley! Send us pictures

of YOU doing what you like best: hanging with friends andfamily; biking, hiking or another activity; with or without your tubes/IVlines showing. You can also shape Oley materials by telling us how Oleyhas helped you and more importantly, how oley can help you better.Let us know what is working and what needs fixing or creating. Wewant to hear from you! Send your photos and ideas toDahlR@mail.amc.edu, or The Oley Foundation, 214 Hun Memorial,MC-28, Albany Medical Center, Albany, NY 12208. Be sure to includeyour name, daytime phone or email address, permission to print / postthem on the web, and your home address if you want prints returned.

HPN Centers of ExperienceBecause of the complicated nature of home parenteral nutrition (HPN),the potential for serious complications is always a concern. This columnis meant to highlight the institutions around the country that specializein caring for HPN consumers. At least one study has shown thatconsumers who are treated by programs specializing in HPN have betteroutcomes. Oley does not endorse any center but brings this to ourconsumers strictly as an informational tool. For a listing of otherexperienced centers visit www.oley.org.

University of California, Los Angeles Medical CenterThe Home Parenteral Nutrition (HPN) program at the UCLA

Medical Center was founded in 1974 by Dr. Marvin Ament. Theprogram was the first of its kind in California and serves both pediatricand adult patients. Consumers are seen in a designated PN clinic,where besides receiving care from an expert multidisciplinary team ofphysicians, RN specialists, a social worker, dietitian and pharmacist,there are opportunities for meeting other HPN consumers.

The team at UCLA cares on average for 70 to 75 HPNconsumers, as well as individuals who are supported on enteralnutrition or who have successfully been weaned from PN. Theteam has a strong commitment to consumer and caregivereducation, advocacy and support. Quality of life issues forconsumers and caregivers are a strong team focus.

The UCLA Medical Center provides a full range of experiencedmedical and surgical specialists to support the special needs of theHPN population. The UCLA Homecare Pharmacy was one of thefirst homecare pharmacies in Southern California and continues as anexperienced provider of HPN and other home infusion therapies.

In 1991, the UCLA Intestinal Transplant program was foundedunder the direction of Dr. Douglas Farmer, completing the full scopeof services available to individuals with intestinal failure. For moreinformation regarding the HPN program at UCLA contact LaurieReyen, RN, MN (310) 825-4823 or nsler@mednet.ucla.edu.

Welcome Aboard!Please join us in welcoming our two newest Regional Coordinators:

Shannon Curran (HEN) — Ontario, CanadaShannon began using her first j-tube at the age of 16. Now 27, she

still uses a j-tube but is currently waiting for a MIC-KEY. Shannonfeels she is a very good troubleshooter. She also has been surprised

that so few Canadians really know any-thing about HEN and Oley. Shannon ishoping to educate Canadians and en-hance OLEY’s presence in Canadathrough a variety of seminars and meet-ings. She is available to talk and offer herinsights to new users as well. Shannoncan be reached at (705) 728-6123 orshannon122122@hotmail.com.Joseph Rodgers (HEN) —New Bedford, MA

As a teenager, Joseph was diagnosedwith Achalasia, a rare and degenerative

disorder of the esophagus. Joseph experienced progressive difficultyswallowing, until the year 2000 when his esophagus finally had to beremoved. He temporarily relied on a j-tube immediately following thesurgery, but was unable to thrive once it was removed. His j-tube wasreinserted permanently in 2005. Joseph is a consumer who does “notmiss eating at all.” Joseph goes to work as an RN while hooked up toa portable pump. He and his wife, Robyn, are raising 3-year old autistictwins and he tries to spend as much time as possible with his family.He is a motivational resource for consumers. Joseph can reached at(508) 999-7363, or JMR12667@aol.com.

Shannon Curran

To secure this reduced rate be sure to let the reservationist knowthat you are attending the Oley conference and be sure to indicatewhether you will need a refrigerator and/or handicap accessibility, orif stairs present a problem. Questions? Need additional information?Call us at (800) 776-6539.

As always we invite you to send along suggestions regarding topicsyou’d like to see covered and/or recommendations for speakers.

Conference, from pg. 1

Member Profile

Volume XXVII, No. 6 (800) 776-OLEY • LifelineLetter — 7

Spotlight on Neal Samara, a Jordanian TPN ConsumerLiz Tucker

This past July during an appointment at the Mayo Clinic, myphysician, Darlene Kelly, MD, asked if I would talk to a new TPNpatient at St. Mary’s Hospital. I agreed, and so she introduced me toNeal Samara, the TPN patient, and her father, Dr. MohammadSamara from Amman, Jordan.

We spent almost two hours together. Neal and her father askingquestions about different aspects of living with TPN; while I askedquestions about Neal’s historyand how she came to be onTPN. She is married and herhusband is an engineer for amajor company in Amman.They have five children, twogirls and three boys, ranging inage from nine to sixteen. It wasobvious she was very proud ofher family.Differences in Care

Neal had a thrombosis in the mesenteric vein that resulted ingangrene in her small bowel. Surgery removed all but one foot of hersmall intestine. Luckily her large intestine was not affected. Theoriginal surgery occurred ten months previous to her trip to the US.When she went home she wasn’t able to maintain her weight and hadto be hospitalized again. They inserted a catheter and put her on TPN.She told me a number of things about TPN in Jordan are very differentfrom here in the U.S. They don’t mix all the components together inone bag but instead give a number of small bags with differentcomponents over the course of the night. For ex-ample, dextrose would be in one bag, lipids in an-other, potassium in a third, etc. Because of that, TPNis not done at home, only in the hospital.

The second big difference has to do with the cath-eter. Neal told me that the care given her Hickmancatheter was much different than what she was taughtto do at the Mayo Clinic. The emphasis is not onsterility. Consequently Neal got a blood infectionand had a fever over 104 degrees for two weeks whilethey gave her intravenous antibiotics. Her fever didnot go down until they pulled the catheter. Neal hadspent four of the last ten months in the hospital andshe was still very underweight. It was at that pointthat her father decided she should come to the MayoClinic in Rochester, Minnesota.Forging a Friendship

Dr. Samara is a highly regarded pediatrician inAmman, but getting a visa from the United States tobring Neal to Rochester was difficult. Once at Mayo,Neal was hospitalized because she was so emaciated. Tests were doneand once she was stabilized a new catheter was placed. I met she andher father a week or so after she started her training to take care of hercatheter and do TPN at home.

Neal and Dr. Samara’s questions for me ranged from how long I had

been on TPN, to whether had I traveled, to what were the problemswith doing TPN at home. They were in awe at how healthy I looked.

As luck would have it, three days later I ended up at St. Mary’s witha blood infection of my own. Our rooms were across the hall fromeach other and we spent a great deal of time together walking thehalls, sitting outside and just talking. I was more and more impressedwith her positive attitude and hard work to make sure she could do

TPN once she got home, as well as her father’s unwaveringsupport and help whenever she needed it.Long-term Solutions

Dr. Kelly did a tremendous amount of work finding asource for a premixed TPN solution that would work forNeal, and then a doctor in her region who would be ableto carefully monitor her condition. Because the throm-bosis was caused by a hereditary blood problem, Nealhas the added complication of having to take significantdoses of cumadin.

Neal and her father stayed an additional month inRochester so she could take human growth hormone (HGH)shots. The hope is that the HGH will help her one foot of smallbowel absorb better so she won’t need quite as much TPN.Building Bridges

After my hospitalization I drove down at least once or twice a weekto take Neal and her father shopping, to take pictures of the MayoClinic and just talk about our lives and the world in general. Theytold me several times that their impression of the United States waschanged dramatically by coming to this country and their experi-

ences with the peoplehere. They both saidthat without exceptioneveryone they met hereor who had taken careof Neal in the hospitalwas kind, generous andhelpful. They werealso amazed by the factthat so many peoplehere smile. People inJordan don’t smilenearly as often; par-ticularly not at otherpeople when they areout in public. Beforethe end of their stay,Dr. Kelly and hermother, Dorothy,brought Neal and Dr.

Samara to my house for a visit. Neal and Dr. Samara brought all theingredients needed to make a real Arab meal. It was wonderful.

Neal and her father have returned to Amman, but we plan to keepin touch. They are wonderful human beings and it is a privilege tocall them friends.

I was more and more impressed

with her positive attitude and hard

work to make sure she could do

TPN once she got home.

Neal Samara visiting Liz Tucker in Minnesota this summer.

Parenting, from pg. 1

8 — LifelineLetter • (800) 776-OLEY November/December 2006

Coping Skills

Nutrishare's Ad

time to make your decisions. There is a great deal to think about. Oneof the first questions is whether you can carry the baby yourself. TPNshould not, in and of itself, be a barrier. The medications you take foryour underlying condition, however, could bea problem. This is one of those very importantquestions for your doctor. It may be possibleto change to another medication, lower thedose or even go off the medication during thepregnancy/nursing period.

Similarly your underlying disease or energylevel may present a significant obstacle. Oneparticipant spent the entire nine months shewas pregnant with nausea and abdominal painand it didn’t go away until right after her babywas born. One of the others, not only didn’thave any nausea; she actually felt her underly-ing disease was much better while she was pregnant. Another of ourparticipants talked about how helpful her homecare company wasduring her pregnancy. They worked closely with the doctor to makesure both she and the baby were getting enough nutrition. The resultwas a healthy baby. As with a person who is not on TPN, everyone’spregnancy experience can be different.

Another consideration if you want to carry the baby yourself, iswhether your underlying diagnosis is hereditary. This has been avery difficult issue for some patients and their families. Geneticcounseling should be done before you make any decisions. One

of our participant’s and her husband believe that is a good ideaand are already working with a geneticist to determine anypossible impact should they decide to try pregnancy.

What are the chances that your disease or being on TPN will affectthe pregnancy or your long-term life expectancy? If the answer is

significant, you need to face the possibility oflosing the baby or, after the baby’s birth, thebaby losing you. Because she had lost a childdue to her underlying condition, one partici-pant gave up on ever having any children. Atage 44 she became pregnant and one of herconcerns was how would it affect her child ifshe were to die before she became an adult.After very serious thought she decided shewould just be the best mother she could beand if something unforeseen happened shewould have faith that her child would be okaywith one parent. After all, there have been

many children in this world who have lost a parent and grown up tobe healthy, happy and productive human beings.Other Options

If you are not able to carry your own pregnancy to term, or have ahereditary disease, surrogate birth mothers, adoption and foster careare options, though they each come with a different set of issuesSurrogate Mother

Do you have a person you know and trust to be a surrogate birthmother for you? One of our participants has a sister who has offered tocarry the baby. Other people have needed to go outside of their

Dannene Bone with her son at the Oleyconference in Salt Lake City.

Coping Skills

Volume XXVII, No. 6 (800) 776-OLEY • LifelineLetter — 9

families. There are hefty financial costs to consider as well: fertilitytreatments, harvesting of your eggs, invitro-fertilization, implanta-tion of the eggs and medical care for the surrogate mother, to namea few. Don’t forget the physical considerations of these actions aswell. They can be very taxing on the body. Finally, there aresignificant legal and psychological issues regarding having someoneelse carry your child that should belooked into and dealt with before youstart the process. In addition to yourphysician and family support, you willlikely need to retain a lawyer and possi-bly a counselor as well.Adoption

Adoption gets around many of thephysical obstacles; however, with theexception of minority and disabled chil-dren, adoption in the US can be a long and expensive process.There just are not enough healthy babies to go around. Because ofthat, many couples have looked to countries in Eastern Europe, theFar East or Africa for babies to adopt. There can be many regula-tions and a significant amount of money involved to adopt childrenfrom these areas. In addition, in some cases parents have adoptedsupposedly healthy children only to bring them home and find theyhave very serious health issues. Can your support system handle asecond chronically ill person?Foster Parent

Becoming a foster parent also gets around the physical issues associ-ated with pregnancy, but comes with other risks. Because many

children in the fostercare system comefrom problem or dys-functional homes,being mentally andphysically equippedto care for one of thesechildren is essential.You also have to beprepared to give thechild back to theirbiological family ifthere is a positivechange in their homesituation. One of our

participants is looking at the possibility of taking on a school-age fosterchild to overcome the energy issues of pregnancy and caring for asmall child, as well as the expense of all-day childcare (she works). Shewill still need a solid support system for those times when the childmay be ill, she may be ill or hospitalized or, as a single parent, thosetimes when she feels the total weight and responsibility for bringingthis child up and needs a break.Quality of Life

How might the pregnancy and then the baby affect your quality oflife? Every parent involved with this article said that having childrenhas enhanced their quality of life. They look forward to getting upevery morning and watching their children grow.

Are they a great deal of work and consume a tremendous amount ofenergy? Of course. Are there future complications (fatigue, relapse ofyour disease, surgery, infections, obstructions, etc.) that might impactyour ability to parent your child? Again, this is almost a certainty. It isapparent from all the stories gathered for this article that having astrong support team assembled is a must, but can help you realize your

dream of having children. Being able tocount on your husband, parents and/or sib-lings, in-laws or some of your friends is veryimportant. The in-laws of one young womanhave offered to add an addition to theirhome. It would be a place to stay when theyneed help with the baby. How much yoursupport team can handle is a considerationtoo. The one father who helped with thisarticle had undergone transplant surgery and

was so looking forward to the time when he would be able to take someof the responsibility for their child off of his wife’s shoulders.

In summary, everyone who contributed towards this article expresseda similar sentiment though in a number of different ways. They all feltthat having a child may not have cured their illness or allowed them toget off TPN, (and they still had problems occur because of those things),but participating in their children’s lives and watching them grow hasonly been a positive experience. As one parent said “It helps us see theworld as something bigger than ourselves. We are not just our disease andnot just a person on TPN. We are a parent, and being involved with ourchildren means being involved in a larger world.”

As you can see, many of the questions and concerns are not thatmuch different than the average person, there are just more of themfor those of us on TPN. Addressing them ahead of time can keepthe unpleasant surprises that come with pregnancy and parenthoodto a minimum. We hope our experiences will help you if you arethinking about becoming a parent.

Candace Verners celebrating Christmas withher family.

Having a child may not have curedtheir illness or allowed them to get off

TPN, but participating in theirchildren’s lives and watching them grow

has only been a positive experience.

Medical Update

10 — LifelineLetter • (800) 776-OLEY November/December 2006

(AST and/or AP), 161 (99%) had abnormalLFTs (bilirubin, albumin and/or PT) and onlyone patient had normal liver tests. Closer inspec-tion on an individual basis revealed that mostabnormal liver function tests were in the mild ormoderate range. The high rate of LFT abnor-malities reflects the fact that one of the mea-sures used to define LFTs is albumin, which isalso a marker of nutritional status. When albu-min was removed from our definition of LFTsthe rate of abnormality dropped to 57%.

Despite this seemingly high rate of liver testabnormality, only seven of the 162 (4.3%)patients had severe liver dysfunction. Afterreviewing the records of these patients wefound only one instance where severe liverdysfunction could be attributed solely to theuse of PN. We also found that female genderwas associated with a greater likelihood ofdeveloping liver failure. Because only sevenpatients developed severe liver dysfunction andsix happened to be women, it is possible thatthe association between gender and liver failurewas by chance alone and not a true result.What to Look For

The study also assessed individual patient’sPN formula. They received an average of 24.7total calories per kilogram per day and 18.5calories per kilogram per day of dextrose.Approximately 10% of total energy was sup-plied as intravenous (IV) lipid emulsion. It isour practice to provide patients with IV lipidonly one or two times per week to meet theiressential fatty acid requirement. Daily IVlipid emulsion is reserved for patients withdiabetes or elevated blood sugar levels that arenot easily controlled by adding regular insulinto the PN formula. In our study there was atrend for a higher dose of PN calories, dex-trose calories and a greater duration of PN tobe associated with worsening liver enzymeabnormalities. This observation was not sta-tistically significant; however, other studieshave found an association between these fac-tors and liver failure. It is possible that thesefactors did not reach statistical significancebecause our patients were not exposed to PNfor as long as patients in these other studies.

There are several theories as to why patients onPN have abnormal liver associated enzymes orliver function tests. It may be due to a nutrientdeficiency or toxicity in the PN formula.

A list of nutrient deficiencies and toxicities that

may lead to liver disease is shown in Table 3.Other possibilities include small bowel bacterialovergrowth that is associated with extensivebowel resection and mucosal disease; patientswith short bowel syndrome; the presence of anunderlying inflammatory disorder such asCrohn’s disease, cancer or pancreatitis as well aspatients who are unable to take any nutrition bymouth. Another theory is that frequent sepsisfrom the patient’s catheter or another sourcemay lead to abnormal liver tests.

The conclusions reached after analyzing thedata in our study are encouraging for long-term PN patients. We found that, althoughabnormal liver tests are quite common inthese patients, severe liver dysfunction is un-common when consumers are given moder-ate amounts of total calories and IV lipidemulsions that are prescribed in low amountsto meet essential fatty acid requirements.

When a patient does have a liver functiontest abnormality it is important for the physi-cian managing the PN formula to diagnoseand treat any other causes of hepatobiliarydisease such as gallstones, viral or autoimmunehepatitis, Wilson’s disease, and hemochroma-

tosis. A review of a patient’s current list ofmedications should also be conducted to elimi-nate any that may be toxic to the liver. Thisincludes H

2 antagonists such as famotidine

(Pepcid) or ranitidine (Zantac), which are com-monly used to reduce fluid loss in high outputshort bowel patients, and should be discontin-ued if there is significant liver dysfunction.(Note: The authors have never found thatwithdrawal of H

2 antagonists has helped and

always resume the therapy because of patientneed.) Another medication to consider elimi-nating is octreotide (Sandostatin) which is usedto inhibit gastric, pancreatic or biliary secre-tions and intestinal motility. PN consumersshould also not drink alcohol in excess.

If no reversible cause for the abnormal livertests are found, the managing physician shouldfollow the labs closely and consider referral toa liver specialist when they remain elevated formore than six months, or if they are elevatedto a moderate or greater degree (see table 1).A rapid increase in liver tests may necessitatethe withdrawal of PN and the provision ofregular intravenous fluids to maintain hy-dration until the cause of the abnormal livertest can be determined. Patients with pro-gressive liver dysfunction or liver failureshould discuss with their physician the needfor referral to a transplant center whereboth small bowel and liver transplantationcan be performed. Hopefully future studieswill help better define the cause of PNassociated liver disease so that physicianscan better manage and treat this condition.

Table 3: Possible nutrient deficiencies and toxicities that maycontribute to PN associated liver disease

Nutrient DeficienciesCarnitine Carnitine may be supplied by lysine and methionine in PN amino

acids. Serum levels can be measured; if low, may supplement in PN.

Choline Not currently approved by the US FDA. Clinical trials are underwayto determine if it is useful in the prevention or treatment of fatty liver.

Essential fatty May occur in patients on lipid-free PN and with inadequate oralacid deficiency fat intake. Lipid emulsion should provide > 4-8% of total calories.

Nutrient ToxicitiesExcess calories/ Total calories should be appropriate for patient’s goal weight. Excesscarbohydrate carbohydrate may lead to fatty liver or high blood glucose levels.

Excess IV Avoid lipid administration of greater than 1 g/kg/d.lipid emulsion

Manganese and Manganese and copper are excreted by the biliary tract. Ifcopper cholestasis or restricted bile flow is present these trace elements

should be temporarily removed from the PN.

Only 7 of the 162 patients hadsevere liver dysfunction...and in

only one instance could severe liverdysfunction be attributedsolely to the use of PN.

Liver Disease, from pg. 2

Contributor News

Volume XXVII, No. 6 (800) 776-OLEY • LifelineLetter — 11

Oley CorporatePartners

The following companies provide overone-half of the funds needed to supportOley programs. Corporate relationships

also strengthen our educational andoutreach efforts. For their

continued interest and strongcommitment we remain grateful.

PLATINUM LEVEL PARTNERS($70,000+)

Nutrishare, Inc.

GOLDEN MEDALLIONPARTNERS ($50,000-$69,999)

Coram Healthcare

SILVER CIRCLE PARTNERS($30,000-$49,999)Novartis Nutrition

BRONZE STAR PARTNERS($20,000-$29,999)

Apria HealthcareSerono, Inc.

BENEFACTOR LEVELPARTNERS ($10,000-$19,999)

Ross Products Division/Abbott Laboratories

PATRON LEVEL PARTNERS($5,000-$9,999)

Critical Care SystemsHospira WorldwideOption Care, Inc.

BLUE RIBBON PARTNERS($2,500-$4,999)Baxter Healthcare

Daniel F. & Ada L. Rice FoundationNestle

CONTRIBUTORS ($1,000-$2,499)Zevex, Inc.

FRIENDS ($500-$999)Accreditation Commission for

Health Care, Inc.

Oley Foundation Horizon SocietyMany thanks to those who have arranged a planned gift to ensure continuingsupport for HPEN consumers and their families. To learn how you can make adifference call Joan Bishop or Roslyn Dahl at 800-776-OLEY.

John Balint, MDJoan Bishop

Ginger BolingerPat Brown, RN, CNSN

Katherine CotterJim Cowan

Ann & Paul DeBarbieriTom Diamantidis, PharmD

Selma EhrenpreisHerb & Joy Emich

Don FreemanLinda Gold

Linda GravensteinThe Groeber Family

Valerie Gyurko, RNAlfred HaasAlicia Hoelle

Jeff & Rose HoelleLyn Howard, MD

William HoytDarlene Kelly, MD

Family of Shirley KleinRobin Lang

Hubert MaidenJudi Martuscelli

Kathleen McInnesMeredith NelsonNancy Nicholson

Rodney & Paula Okamoto, RPhKay Oldenburg

Judy Peterson, MS, RNClemens PietznerBeverly Promisel

NEW! Abraham RichRoslyn & Eric Scheib Dahl

Steve SwensenCathy Tokarz

Eleanor & Walter WilsonJames Wittmann

Patty & Darrell WoodsRosaline Ann & William Wu

Individual Donors Make A Difference!The following list represents everyone who generously contributed towards Oley’s effortsbetween October 5 and November 15, 2006. We also want to thank all of those who are notlisted below, yet have supported the Foundation by donating gifts earlier this fiscal year or havevolunteered their time and talents.

Ambassadors ($2,000+)

Presidents Circle($1,000-$1,999)

Benefactors ($500-$999)

Sponsors ($250-$499)

Patrons ($100-$249)Jason Bodzin, MDAnn & Paul DeBarbieriJudith PetersonBeverly Promisel

Supporters ($50-$99)Irene & Jennifer ColemanDeborah Groeber, in

memory of her father,Bruce F. Groeber

Kimberly Morgan

Contributors ($30-$49)Richard & Faith Dillon

Friends (Up to $30)Ameriprise Matching GiftJoan E. Bowling

Florence D. Bye, in honorof Samantha Bye

Lars DahlJack & Ruth Morgan TC,

in honor of Matthew W.Morgan

Florence ParmeleeBarry & Ronna Staley

In Memory of MichaelSpeshock, Sr.Bolton Bay Traders,/Wm.

& Clover-Leaf NurseriesBonnie ConnellyPilar G. & Richard A. DexterChris & Mary Anne

JohnsonFred & Pat KrenzerJohn Stone & Barbara

Krenzer, MDGraymont Materials (NY)

In Honor of BarbaraKlinger’s 20 Years on TPNBarbara & Lee KlingerMary Miller

In Honor of DominicCucinotta’s 80th Birthday(Alicia Hoelle’s Grandfather)Albert AzzriHarvey & Eleanor BartonMarianne & Bernie KellumShawn & Stacy Kelly, DMDVincent KellyE. MurphyAnthony & Andrienne

MusumeciKristy VelahosPaul & Mary WalterGeorge & Wilma Walter

In Memory ofCharles ZollwegMr. & Mrs. HillmanNola & Wayne Zeches

Toll Free Schedule ☎Toll Free Numbers Available to US and Canadian Consumers!

The Oley Foundation is able to offer its

toll-free lines to consumers in the US and

Canada. Two toll-free numbers are cir-

culated to experienced homePEN con-

sumers on a monthly basis. The goal is to

make speaking with fellowlifeliners more

affordable, and to provide Regional

Coordinators with a better grasp of

their region’s needs.

Advice given by volunteer coordinators

represents the experience of that indi-

vidual and should not imply endorsement

by the Oley Foundation.Due to the expense, a per-minute fee

charged to Oley, we ask that you limit

your conversations to 30 minutes.

The schedule of toll-free numbers and

volunteer coordinators is updated in

each LifelineLetter, and posted on our

web page @ www.oley.org. Comments?

Call (800) 776-OLEY.

JAN

. ’07

MA

R.

’07

Laura KeserMesa, AZ(888) 610-3008 MST

Matthew Van BruntApple Valley, CA(888) 650-3290 PST

Sheila MessinaSan Jose, CA(888) 610-3008 PST

Sue KopruckiWilliamsville, NY(888) 650-3290 EST

Michael MedwarFranklin, MA(888) 610-3008 EST

Sheila DeKoldFloyds Knobs, IN(888) 650-3290 EST

FEB

. ’07

NON-PROFIT ORG.U.S. POSTAGE

PAIDPERMIT NO. 687

ALBANY, N.Y.

LifelineLetterThe Oley Foundation214 Hun MemorialAlbany Medical Center, MC-28Albany, NY 12208

Laura has been on HPN since 1991 due to short bowel syndrome. She hasexperience with multiple catheters, dealing with infections and traveling. Shehas also returned to college at Oklahoma State and graduated in the spring 2006.She looks forward to sharing her experiences with you.

Matt has been on TPN for 20+ years due to Crohn’s disease and short bowelsyndrome – so he is experienced with the up’s and down’s of TPN. He likesriding his bike and spending time with friends. He is happy to talk aboutanything associated with TPN or otherwise.

An Oley trustee, Sheila has been on TPN for over 20 years due to short bowelsyndrome. She is an education and training specialist for case managers and hasa lot of experience with managed care. She enjoys helping other lifeliners functionas independently as possible and participate in their health care decisions.

Sue had a frightening, isolating and frustrating time when her daughter Emily wasdiagnosed with a form of pseudo-obstruction at birth and started on HPN andtube feeds. Connecting with other families in similar situations helped make lifework for her whole family. Emily now has short bowel and an ostomy. Sue haslots of experience with lines, g-tubes, and ostomies as well.

Michael was diagnosed with Crohn’s Disease at the age of 9 in 1979. He wasmostly HPN dependent from his teen years until late 2001 when he was fortunateenough to get off therapy thanks to good luck, diet and exercise. He has been aneditor at daily newspaper for many years. He is glad to chat with anyone.

Sheila has an 8-year-old son David and 6-year-old daughter Olivia, who wasdiagnosed with pseudo-obstruction at the age of 2 years. Olivia is TPN dependentand has tolerated enteral feeds in the past. Olivia has a G- and a separate J-tubeas well as an ileostomy. She requires catheterization overnight.

Meet Us in Cape CodPlan to join us June 28 to 30 at the

Cape Codder Resort & Spa in Hyannis,MA, for the 2007 Oley Conference.

Cape Cod offers close proximity to thenutrition support talent in Boston,

as well as a chance to get away with thefamily for a relaxing beach vacation.