A labor of love: Life with total parenteral nutrition

By Kathryn Michalski

Peter

When my son Peter and I go out, it’s not unusual for people to fawn over him a bit.

“What a happy kid.” “He’s so smart.“ “It makes me smile when I see him,” we often hear. As a mom raising a child with as many health issues as Peter has faced, these comments brighten my day.

Peter was born with an arteriovenous malformation (AVM) in his liver, meaning his veins and arteries weren’t connected properly. When he was just seven months old, the AVM completely disrupted the blood flow to his liver and small intestine, causing multiple holes in his small intestine. He became gravely ill after that. At our local hospital, Peter had one surgery to remove the AVM and three more to salvage what was left of his intestines.

When it was all over, Peter had four ostomies (a surgical opening made in the skin as a way for waste products to leave the body) and a gastrostomy (a surgical opening into the stomach, where a feeding device can be inserted). He couldn’t digest food properly, so he had to receive all his nutrients intravenously (IV), through a medication called total parenteral nutrition (TPN).

Having a child on TPN is a lifestyle, for both patient and parents. It requires refrigerating IV bags and medications, and storing huge amounts of tubing, caps, alcohol preps, pumps, dressing change kits, and other supplies. There is a daily ritual of preparing the medicine and hooking it up, and because TPN requires a permanent central line for IV access, there is always a risk of infection that needs to be closely monitored.

Peter and his evening medical equipment
A typical day with TPN

At 7:30 a.m., Peter wakes up and I take down his TPN bag, flushing the line with saline and a small amount of either heparin or ethanol to keep it clean. We protect the peripherally inserted central catheter (PICC) line in his arm with a footless sock, and we head downstairs to have breakfast. (Some mornings the sheets are wet since he gets so much fluid overnight.)

Around noon, I take a new TPN bag from the refrigerator, so it will be room temperature by evening.

After dinner, Peter’s dad or I wipe down the dining room table and take out a plastic cafeteria tray we use for TPN preparation. The tray is easy to wipe down with alcohol and gives us a very clean surface to work on. We inject the TPN bag with a syringe of multivitamins, assemble the tubing, attaching it to the TPN bag and the IV pump. Peter also takes a lipid (fat) called Omegaven, which requires a second pump, and also gets a tubing set-up.

preparing TPN treatment at home
At bedtime, Peter’s dad or I don a pair of gloves, clean the cap of the PICC line for a good 30 seconds with an alcohol prep, flush it with saline, and attach the TPN and Omegaven tubing. We double-check the rates and amounts programmed on the pump and then tuck him in for the night.

At midnight, we hang up a second bottle Omegaven. In the morning, one of us attaches a small saline bag to the tubing to flush the last little bit of Omegaven out of the tubing.

After that the routine starts all over again.

Caring for a central line at home

Once a week, my husband and I change Peter’s PICC line dressing. This is a sterile procedure, done with mask and gloves. Even though Peter is six-and-a-half years old, and we have been doing this with him for years, two adults are still needed because Peter sometimes forgets to stay still. If the PICC line comes out during the procedure, it means a trip to the emergency room. My husband holds his arm still, and I do the cleaning and placement of the new dressing. While this is happening Peter watches a DVD and is quite fine with the 20-minute procedure.

Once a week, I also change his PICC line cap. This is also done sterilely. A cap change only takes about three minutes, and Peter is able to stay still with only one parent doing this.

Securing supplies

Omegaven and tubing
Once a week, our local homecare infusion company sends out Peter’s TPN for the week, along with tubing, alcohol and other necessary supplies. I put the items away in a cabinet we use just for TPN supplies, and the TPN bags go into a small refrigerator that we purchased specifically for Peter’s TPN and IV medications. (A separate refrigerator helps to reduce the chance of the medications getting contaminated.)

We get Peter’s Omegaven at Boston Children’s during visits with the Center for Advanced Intestinal Rehabilitation (CAIR) team every month to every other month.

A team effort

The HPN Program Team (l-r): Clifford Lo; Brittany Tellier; Jacqueline Riso; Carlotta Hayes; Bram Raphael; Lisa Summers; Kathleen Gura; Meghan Dalton; Daniel Kamin

Because we live in Pennsylvania, and the CAIR team is in Boston, a lot of communication happens over the phone. At least once a week, I talk with Peter’s Home Parenteral Nutrition (HPN) nurse practioner, Meghan Dalton, NP, MSN, CPNP. Meghan has been a welcome addition to Peter’s medical team since we met last fall. I give her updates on how Peter is doing with his food intake, weight, urine and ostomy outputs, and overall health. Meghan talks with Daniel Kamin, MD, Peter’s dietitian Brittany Tellier, and Bram Raphael, MD, director of the HPN program, as well as the rest of the CAIR team. Anywhere from once a week to once a month, Peter has blood work drawn off his PICC line and we drop it off at a local lab. The results are then faxed to Boston Children’s for review by his medical team.

Once the team has discussed Peter’s case and recent blood work, Meghan gets back to me with a plan. Sometimes we talk with greater frequency if there is something new going on with Peter. I have absolute confidence that they have Peter’s best interest always at heart, even when medical issues happen at inconvenient times.

Peter at Boston Children's
People often ask why we drive seven hours to go to Boston Children’s instead of traveling somewhere closer by. It all started when Peter’s grandfather came across an article in The Wall Street Journal that detailed how the work of Boston Children’s researcher Mark Puder, MD, PhDwas saving the lives of children on TPN with a lipid called Omegaven. We originally came to Boston Children’s so Peter could start on Omegaven, but all these years later we continue to come for the remarkable care.

Peter has an excellent surgeon, Rusty Jennings, MD, who has painstakingly spent hours in surgery fixing the problems with Peter’s bowel. Peter continues to see the same doctors that he first met in December 2006 and that continuity of care is very important to us. When they met us, Dr. Puder thoroughly explained how Omegaven works to protect the liver in kids who are on TPN, and Dr. Kamin and Dr. Kim spent countless hours sorting out how to work with Peter‘s damaged bowel. They never handed him off to another team of doctors because they were done with their rotations.

Dr. Kamin especially has been instrumental in Peter’s care. Over the five and a half years we have been coming to Boston, he has maintained excellent recall of Peter’s lengthy health history. He keeps us closely involved in the medical decisions, and constantly keeps in mind the overall goals we have for Peter. He focuses on Peter as a person, not just an illness. And even though he’s only six, Dr. Kamin’s dedication to our family isn’t lost on Peter; last Halloween he went trick-or-treating as his favorite super-hero, Dr. Kamin.

Treating a child with chronic illness is a learning process that can seem never-ending. Based on my six years of managing Peter’s TPN, here are few tips I’ve learned:

1.   Learn proper sterilization techniques. One you’ve mastered them, be ready to teach to anyone (family, medical professions, etc.) who will be looking after your child. Don’t be afraid to speak up if you feel someone is not being properly sterile with the central line.

2.   Find a primary doctor or medical team immediately. When facing long-term illness, your child may be cared for by many people, a good primary doctor greatly facilitates communication and direction of care.

3.   Find a support group.Yahoo Short Bowel Syndrome group and Oley have been very helpful for my family. It is remarkable to see what very full lives people can live while on TPN. Their stories provides me with inspiration when I am feeling frustrated.

4.   Keep a reserve stock of supplies. Glitches in insurance payment, supply availability, holidays or weekends and other unforeseen circumstances will happen. It’s best to extra supplies (dressings, medications, formula, etc.) on hand.

5.   Keep TPN supplies in a clear, organized place. All those small boxes add up and take up a lot of space!

  • Monica Forte

    That is also our life for the past 6 years foing on 7 next month. Our son Tony was born with Total Colonic Hirschsprung’s Disease that delibitates the intestinal tract. From the time he was properly diagnosed at the tender age of just 6 days old, he had to have his entire colon and 1/3 of small intestine removed leaving him an ileostomy,a Central Line Veinous Cath for his IV Nutrition and a G Tube Placement for sromach feeds with elemental formula in hopes that his remaining small intestuine would regain strength for him to be weaned off TPN. However, after having a 2 GI Motility Study at the Nationwide Children’s Hospital in Columbus Ohio, we had a gut feeling what the results would be that he has severe dysmotility in the reamining what was thought to be healthy bowel. Tony has been waiting on a stomach,full intestine and will receive liver transplant list for 2 years There was many lengthy admissions at the our closest Children’s Hospital of Penn State in Hershey Pa. He currently receives 2250mls of TPN daily for 18hrs a day along with his 180ml of Lipids every Mon-Wed-Fridays. Tony carries an Backpack everywhere he goes with his IV Nutrition and Pumps. His story can be found at http://www.aftft.com and http://www.giveforward.com/tonyschallenge. We will keep your son and family in our prayers. GOD Bless from one mom to another of our amazing boys and the miracle they are!

  • Izzy2c

    What a great and brave kid Peter must be.  I just wanted to add a tip that might make Peter’s PICC a bit more fun.  When I had a PICC line I got baby leg warmers for about $4.00/pair at Target in the baby sock section in a whole slew of fun designs. It kept my PICC protecting and the tubing from catching on something even when it wasn’t in use and I could have fun matching the baby legs to my outfits.  There were animal prints, stripes, polka dots, (and some other girly designs Peter wouldn’t be to interested in.  Best of luck!

  • Erinfoster614

    Stories like these from parents always amaze me.  One year ago, my 1 day old infant was diagnosed with Hypoplastic Left Heart Syndrome and catapulted me into this little known world of medically fragile children.  You are right in saying that it is a lifestyle.  I applaud you and your husband for advocating and taking such great care of Peter!  The care at Children’s Boston is invaluable.  Best of luck!

  • http://www.ihcco.net/detailed_engineering_design.html Leslee

    such an inspiring story..i salute the parents like you with great devotion to their kids with ailments.. God bless!

  • Kit Freeman

    My daughter, Debra O’Connor, posted this link on Face Book. Her son is Sammy O;Connor who has Microvillous Inclusion Disease. Deb’s Dad has AVMs in the brain and in the brain stem.  So I can understand a little of what you are going through. I have met Dr. Puder a few times and he is one of the best doctors I have met.