Friday, September 02, 2011
Saturday, June 18, 2011
Yes, starting with next week’s shipment, she will only be getting fluids without nutritional additives and without Omegaven.
ONE MORE TIME!!!(click on me) listen while you read: NO MORE TPN!!!
Two and a half years ago she went off of TPN for the first time. She was growing enough in both in height and weight for her team to think she could stop her TPN(total parenteral nutrition, IV nutrition). She was off of TPN for 6 months that time.
Before her surgery to lose her ostomy, we put her back on to get her ready and fatten her up for surgery and keep her weight up during the recovery time while her reconnected bowel learned how to behave. That strategy worked and she did very well and for the next 2 plus years. But, she has done well....ON TPN.
This Wednesday we went in for clinic with Dr. Lo (the TPN guru at Children’s) and Ellie had gained enough weight and grown enough for the team to feel that it was time for her to come offof IV nutrition again. She will still be on IV hydration 7 nights a week, but no IV nutrition.
Those of you that have met Ellie have seen how she runs from place to place, swims all summer, dances from room to room and lives life on her toes. The heat of summer is not the time to decrease her IV hydration.
So we are now in a two month trial period away from the complex land of TPN. As with all things short gut we understand that TPN may come back to Ellie if she needs it, but for now this significant step is great and makes us think about the terrifying possibility of a night without the whirr of an IV pump coming through the monitor.
WHAT WERE WE THINKING?????
So no more soccer.
For now Ellie will continue to dance ballet, go to school (Kindergarten starts in September), take swimming lessons (at the Y!) and be a very typical 5 year old. And well, I am pretty happy about that. In the future, I think that she will rock T-ball as a left field daisy picker.
He has also decided that crawling is for sissies. I honestly think he started pulling himself to standing and cruising because we told him that Ellie started cruising at 7 months. A Month behind his sister Will started pulling himself up to standing and walking. Bring on the crash helmet.
Monday, May 23, 2011
by Abby Brogan
My daughter Ellie was born in April of 2006. She came a month early, but over all my pregnancy was normal and she was initially doing very well. Things began to change after her first hour. The next 24 hours were something I wouldn’t wish on anyone. It ended with both of us in a new hospital, her recovering from exploratory abdominal surgery, and the very nice surgeon explaining to us that Ellie was born missing 90% of her small bowel and 30% of her colon. After she fully explained what Ellie’s GI system looked like, she started talking about what Ellie’s life was going to be like and what the future could be like. It was a very scary and grim conversation.
Ellie as a baby before Omegaven
Ellie’s GI tract was not able to absorb enough nutrients to sustain life, so she was placed on IV nutrition support, also called Total Parenteral Nutrition. Total Parenteral Nutrition (TPN) is made up of two main parts. The first part contains all the fluids, vitamins, minerals, amino acids, and medications. The second half of the mixture is the lipid, consisting of a fatty acid that has been broken down enough to be absorbed through the blood stream, and blended into an emulsion that keeps the fats from separating from the fluids. TPN enables an individual to live and grow even though he or she cannot eat.
However, the down side of TPN is that for most individuals on it for an extended period of time, particularly children under two years of age, it will also cause a build-up of fat in the liver causing damage. For these individuals, there is a race to rehabilitate the GI tract and get off TPN before the liver becomes too damaged. If they cannot get off TPN fast enough, they often will be listed for a liver transplant and, if needed, other organs as well. For those ineligible for transplant, liver damage can lead to death.
What is Omegaven?
In 2001, Children’s Hospital Boston started doing research on TPN to find out what part of TPN was causing liver damage. Mark Puder, MD, a surgeon, and Kathleen Gura, PharmD, a pharmacist, collaborated on the research with other doctors at Children's Hospital Boston. Through studies done on mice, they discovered that it was the lipid portion of the TPN that was causing the damage. But the fats contained in lipids are essential for the body, so they needed to find an alternative.
Dr. Gura had previously used Omegaven, a lipid emulsion derived from fish oil that contains omega-3 fatty acids, to treat a patient with a soy allergy and severe fatty acid deficiency. From this experience she suggested they try Omegaven in their trials.
The results were very promising.
In an article written by Children’s Hospital Boston, Dr. Puder explained the results that showed that parenteral nutrition without the fat makes the liver fatty, and by adding the Intralipid, it becomes even more so.1 "That fat becomes more or less a fuel that burns the liver, causing it to become inflamed....But the Omegaven is an anti-inflammatory lipid that prevents fat build-up in the liver. So if there's no fuel—fat—there's no fire—injury."1
The results were so promising that on September 26, 2004, the team at Boston Children’s Hospital started the first child on Omegaven. They saw improvements in one week, and a month later the child’s liver function tests were good enough that he was taken off of the transplant list. In most children, during the first month or so the direct bili (liver function test) goes up and then comes down. It is known as the Omegaven roller coaster. But, during this time you get to watch your child turn pink again.
Ellie's bili chart
Some hospitals are using Omegaven in combination with regular vegetable oil-based lipids such as Intralipid. Dr. Gura commented on this, stating, “Based on published papers, it appears that children with PN liver injury do better with Omegaven alone (not mixed with Intralipid). The Diamond paper from Canada actually shows this, although they still like to do the combination until the child fails and then they switch them to plain Omegaven.”2
To date Children’s has treated 166 children with Omegaven and administered over 100,000 doses. Omegaven has helped almost all 166 children, but the team has learned that the faster these children get on Omegaven, the better they do. Using it as a last resort or rescue is not always successful. The damage to the liver is already done.
How to Get Omegaven
Omegaven has not been approved by the FDA to be used broadly. Because of this, it has a Compassionate Use Only status, meaning that it can only be used if it will save the child’s life. To show that the child needs Omegaven, he must have failing liver function tests, including a direct bilirubin number greater than 2 mg/dL, indicating liver damage called cholestasis.
In the beginning, patients had to transfer to Boston to get Omegaven therapy. Now, thanks to the passionate outreach by Puder and Gura to the medical community, it has been used by almost every free-standing children’s hospital in the United States. Physicians can also apply to be able to administer Omegaven themselves, and the Boston Omegaven team has gone so far as to publish their therapy instructions with the Oley Foundation, a support group for those who rely on TPN.
Each year, more and more insurance companies are paying for Omegaven enabling patients to be treated locally and not requiring them to travel to Boston. The FDA now allows billing of insurance companies upon request. Approval, however, isn’t mandatory, and insurance companies are not required to pay for it. For a list of the hospitals that have used Omegaven and the insurance companies that have covered it, visit the Short Gut Wiki.
Making Omegaven available to everyone and covered by insurance will continue to be an issue until it is approved by the FDA for use in all people receiving TPN. Currently, Fresenius Kabi AG, the manufacturer of Omegaven, is working with Children’s Hospital Boston to get the necessary data for a new drug application submission to the FDA. The process to get a drug approved in the US is lengthy. There was hope in the beginning that additional animal testing would not have to be done or could be avoided; however, these tests were ultimately required and are currently underway.
Children’s Hospital Boston has not seen any adverse effects of the use of Omegaven, but they are monitoring all their children with long-term blood work and other follow up examinations.
The child that received the first dose as an infant in 2004 is still on TPN and Omegaven at age seven, goes to school and has shown no adverse effects from the TPN that likely would have destroyed his liver just a few years ago.
A recent picture of Ellie
My daughter Ellie was the 23rd child to receive Omegaven, and has now been on it for almost five years. She is doing well, growing and thriving. Both my husband and are so thankful for Omegaven, and the doctors that brought it to our attention. I will not let myself imagine where she would be without it.
Thanks to Mark Puder, MD, and Kathleen Gura, PharmD, for their personal assistance in providing information for this article.
Abby lives in the Boston area with her husband and daughter Ellie. Ellie was born in 2006, missing 90% of her small bowel and 30% of her colon due to Jejunal Atrisia. Ellie is diagnosed as having Short Bowel Syndrome. Ellie has had multiple surgeries since birth and is on tube feeds and Home Parenteral Nutrition (HPEN). Abby and her family have faced the many challenges that come along with having a child with tubes, yet lead a remarkably active lifestyle. Abby is also a consumer advocate for ThriveRx. Please feel free to contact Abby at
1-774-270-3425 or via email at abrogan@ThriveRx.net.
1http://www.childrenshospital.org/dream/dream_fall06/fishing_for_the_right_solution.html See also http://pediatrics.aappublications.org/cgi/content/full/118/1/e197
2Personal communication with Dr. Gura. The study she cites is Diamond IR, Sterescu A, Pencharz PB, Kim JH, Wales PW. Changing the paradigm: omegaven for the treatment of liver failure in pediatric short bowel syndrome. J Pediatr Gastroenterol Nutr. 2009;48(2):209-15.
Wednesday, March 09, 2011
Delegate Maggie McIntosh, Chair
Delegate James E. Malone Jr., Vice Chair
Room 250, House Office Building, Annapolis, MD 21401-1991
Wednesday, March 02, 2011
Tuesday, March 01, 2011
Thursday, January 27, 2011
Wednesday, January 12, 2011
2010 A YEAR IN REVIEW
We started the year with the huge bummer of Gus getting diagnosed with cancer. Then moved on to the excitement of Ellie’s first solid poops. In mid January we had a new refrigerator delivered and installed. The next morning we woke up to a flooded basement, and flood damaged kitchen. This led to a nice little kitchen and basement renovation that we have always wanted to do, Thanks Sears!!. During all this we also found out I was finally pregnant with baby number 2. I was rocked by ragging morning sickness, but I would have put up with anything, we were so excited to be pregnant again. I think this made loosing Gus in April a bit easier to deal with.
We struggled with hydration for Ellie through the end of the spring into the beginning of summer. We finally got things under control just in time for the Oley conference in NY. This kicked off a great summer with Ellie taking to swimming like a fish. We spent a week on the
Ellie was on the beach and in the water everyday. We managed to get our routine for covering her dressing down so well that she went the whole week on the beach, and in the water, without her dressing ever getting wet. She had such a blast she has been talking about going back ever since.
I loved the last part of my pregnancy and loved making it all the way to my due date. My pregnancy with William was much easier and I felt much better thought out the whole thing. This just made us realize how sick Ellie was in utero and how much of a toll that took on me. William was a breeze in comparison.
William was born via C-section because I went into labor but never progressed. We called the ball a bit earlier with William than we did with Ellie and that made my recovery much faster with William.
She doesn't like chocolate so that made the candy easier to deal with. And after the first few days she forgot that she had candy and that made it easier for Gib and I to finish it off.
She spent 5 days in the hospital getting hydrated, doing bowel prep and getting a scope of her bowels to see if we could see anything that was wrong. We were also able to sample some of the fluids that they found in her bowels and culture it see what bacteria we were dealing with. They typed it out and then tested different antibiotics on it and found that Bactrum was the new antibiotic that we should try.
We did a week of Bactrum right before Christmas and it finally stopped the cycle of SBBO that we had been seeing. We also finally started probiotics (VSL3) after talking to another family that has been using them for years. Our team at Children’s was against this decision but they understood why we wanted to try them. And so far so good, it has been just under a month since we saw any signs of SBBO. (knock on wood now)
We spent the week following Christmas and New Years in
All in all William has been so easy and laid back that if we could afford it I would be pregnant again. It has been great for Gib and I to have the chance to be typical parents. There is nothing like a very high maintenance baby to make a typical baby fell like a cake walk. Although we did have to remind our pediatrician to treat us like first time parents because we have no clue what to do with a baby that is under 8 months old and at home. All the typical kid stuff throws us for a loop. Things like… eating and pooping, those are things we didn’t have to deal with in Ellie’s first few months.
Today we are spending the day inside watching a true Nor’easter dump close to 2 feet of snow on our house. We have been out playing in it once already today and will head out again as soon as our mittens dry out. We promise to make more of an effort to keep this blog up to date this year. I know you have heard that before, but this time we really, really promise!