All about blood and a little besides

Friday, David mentionned that Sébastien had bled through his ostomy and his hemoglobin count was low. I get confused between hemoglobins, white blood cells, red blood cells and platelets, so I googled “What is blood made of” and thought of sharing the answers with you…

In one sentence, blood is a mixture of three cells and plasma – a watery liquid. It also contains hormones, nutrients, clotting agents and waste products on their way out. The three kinds of cells are red blood cells, white blood cells and platelets. Each one has a different mission.

Red blood cells are the most numerous. Shaped like donuts with an indentation instead of a hole, they carry oxygen from the lungs and disperse it throughout the body, carrying it in molecules called hemoglobin. Imagine the hemoglobin as a bucket with four red compartments. When it arrives at the lungs, a molecule of oxygen fits in each compartment, and is carried away to the capillaries. The capillaries are the ports of arrival in our body, the tiniest blood vessels, where the exchange of water, oxygen, carbon dioxide, nutrients and waste chemicals takes place. Capillaries don’t have alot of oxygen, so, our bucket of hemoglobin, on the ship of a red blood cell, gives up its oxygen and picks up some carbon dioxide in exchange. The four red compartments of the bucket are really four atoms of iron, which give the red blood cell its color.

White blood cells are our body’s military, fighting off infection and invasion. As in the armed forces, there are three main branches: the army, the navy and the air force. White blood cells have granulocytes, lymphocytes, and monocytes. In detail, the granulocytes, come in three different types: neutrophils, eosinophils, and basophils. (The more in detail you get, the longer the names…) The first take care of bacteria, ingesting and digesting them. The second take care of parasites and the third is involved in allergies. Back up a level, to the second kind of white blood cells, the lymphocytes are subdivided in two: T cells and B lymphocytes. The T cells are the immune system directors. The B lymphocytes produce antibodies. Back up to the three kinds of white blood cells, the third, the monocytes are the largest white blood cells, and enter the body’s tissue to turn into even larger cells called macrophages which eat foreign bacteria and destroy damaged, old or dead cells of the body.

Platelets are the clotting cells of the blood family, taking care of wounds and cuts in a variety of ways: clumping up to form a clot, or breaking down when exposed to air and releasing a substance that starts a chain of chemical events, ending in a protein transforming into fibrin which forms into long threads that tangle up red blood cells and form a clot. (Yes, that was a run-on, but this is biology and not grammar!)

Plasma, the fluid that the blood cells travel in, is 90% water. The rest is protein molecules, sugar, electrolytes, hormones, vitamines and cholesterol.

My dad would find that this blog post is in keeping with my researcher’s attitude… during summer, he would laugh as I brought home a pile of books from the library on some research project or other that I’d invented for myself. The main source of information for this post comes from a PBS website article and Wikipedia.

Sunday, David left for Toronto early, as he had only been planning to leave Wednesday morning. We’d only just left David after a leisurely breakfast together at Stella’s when he called to ask for a ride. Sébastien bled again in his ostomy, and developped a fever on Sunday. David mentionned that he was septic and rather than ask him the definition, we looked it up later, and found that the web-as-doctor was still just as frightening. My husband and I hate to prod figuring that eventually the questions we don’t ask will get answered when the sting of less-than-cheerful news fades. Some view it as a fault, as though avoiding the subject shows a lack of interest, but we see it more as a desire to tread lightly. It is quite well known that men and women have very different emotional responses. A quote from “Married with Special-Needs Children” explains it well:

One wife who understood this provided excellent advice: “I think it is very important to remember that moms and dads react very differently to the diagnosis. The journey to acceptance is a grieving process. Moms often blame themselves, while the dads are more removed.”

/…/ In general, men are more prone to withdraw or attack problems pramatically rather than with emotional expression, whereas women place more value on talking about their feelings.

The second main reason we need to expect – rather than resist – differences in emotional response and coping styles is that opposites attract. We tend to become partners with people who have qualities we desire but lack. We are initially attracted to these differences and intuitively seek them as a kind of balance to ourselves. Unfortunately, we often subsequently have a growing discomfort with these same differences. For example, the man who represses his own feelings yet chooses an emotional wife may discover that her expressiveness churns his own emotions up un a way that he can’t stand.

Before setting a record for the longest post on Place de choix, I’ll finish with a quote from Emily Dickinson:

It’s all I have to bring today
This, and my heart beside

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Medication schedule

Out of curiosity, I asked Jasmina what Sébastien’s medications were, aware that they change according to his health. Jasmina is always happy to oblige and provided me with his schedule, already typed out and organized…

• 3:00 a.m. – Domperidone (1.6 ml)
• 6:00 a.m. – Omeprazole  (1 ½ tablets)
• 9:00 a.m. – FK (0.5 mg-1 tablet), Penecillin (2.1 ml), Calcium (½ tablet) without feeds running, taken orally, D-Vi-Sol Cholecalciferol (1 ml) (Vitamin D)
• 10:00 a.m. – Domperidone (1.6 ml)
• 3:00 p.m. – Omeprazole (1 ½ tablets)
• 4:00 p.m. – Calcium (½ tablet) taken orally
• 9:00 p.m. – FK 0.5 mg (1 tablet), Tacrolimus, Replavite (½ tablet), Penecillin (2.1 ml), Calcium (½ tablet) taken orally
• 11:00 p.m. – Domperidone (1.6 ml), Omeprazole (1 ½ tablets)

Sébastien’s feeds are regualted at about 645 ml of formula per day, fed through his central line, starting in the late afternoon and ending in the morning.

Dialysis days (Monday, Wednesday and Friday) bring a slight change to the schedule, as Sébastien wakes up for dialysis at 7:30 a.m. Saturday morning is bloodwork, and on dialysis days, Sébastien is weighed… his least favorite morning ritual! During dialysis, the nurse administers two other medications: Calcitriol and Darbepoetin.

Eight different medications a day, ten on dialysis days, four of which are vitamins (Calcitriol, Replavite, Calcium and Cholecalciferol), and only one major anti-rejection drug (Tacrolimus). The four remaining treat different things; Darbepoetin for low red blood cell counts – due to kidney failure, Domperidone to increase movement through the digestive system and inhibit nausea and vomiting, Omeprazole to block acid production in the stomac – as Sébastien has acid reflux, and finally Penecillin, which generally prevents infections especially in the blood.

Considering all that, Jasmina and David have a fantastic memory! And on a lighter side, things could be worse.

Check out the blog on Moday for a list of this blogs goals.

Taking it for granted

Sometimes I’m asked a question about Sébastien that takes me by surprise… “Does he still have an ostomy? Is he re-connected? Does he eat? Does he walk?” They’re questions I’ve already asked too, and I forget that what I take for granted is sometimes unknown to others. More often than not people simply ask how Sébastien is doing, without the finer details of his medical condition. But, that is what the blog is for… the medical details. After all, we want to raise awareness here!

Does he still have an ostomy?
Yes, Sébastien has an ostomy which helps the medical staff keep tabs on his digestion. It was to be removed a while back, but rather than having Sébastien go through another operation, the team in Toronto decided to wait, and combine operations instead. Sébastien understands that when it is time to empty the ostomy (every so often in a day), it is done in the bathroom, and he is pretty good about it, though at times the smell can make him gag, as he is quite sensitive to smell, Jasmina tells us.

Does he eat?
Sébastien’s diet is very closely watched due to his kidney failure, since last year. He is placed on feeds in the afternoon and evening, which decrease his hunger. He plays with food when he is at the table in his high chair, and pretends to chew like the adults. His intake of fluids is also limited, this because fluid overload is a problem that could lead to others when you have kidney failure.

Does he walk?
Sébastien is cared for by an excellent physiotherapy team in Toronto. David and Jasmina continue his exercises at home, usually when there isn’t any company, as the extra effort on Sébastien’s part makes him whine, a high-pitched kind of cry that can break your heart if you’re too soft! Sébastien has a tendency of doing the splitz when he sits down, and to counteract that, Jasmina will ask him: “Sébastien! Make your feet kiss!” making it like a game. He puts some effort into bringing his legs and toes together, and sometimes he’ll do it spontaneously by himself, and look to see if we’ve noticed. The more difficult exercises involve getting him to stand to reach for toys on the couch. Jasmina or David sit on their feet behind Sébastien, who sits up on their knees, feet together. Jasmina or David will place a toy away from him on the couch, and with alot of enthusiasm, encourage him to “go get it” by standing, as they kneel. Crawling is not his favorite idea either, as he prefers to scoot about on his bum in circles. He is also learning to bend his leg to reach over for a toy, rather than doing gymnastic-worthy stretches to get what he wants.

It may seem a bit overwhelming as Sébastien has to develop his gross motor skills AND deal with his kidneys… But, he couldn’t be better placed to have a family that is up to the challenge!

Do you have questions you would like to ask? We would love to answer them! Write them in the comments or send me an email at: jpalud at gmail dot com. Tune in Friday for a look at what a day with Sébastien is like.

Omegaven Part II

It was about a month ago, when I first learned of Omegaven from a blog. I’d researched Short Bowel Syndrom on Google Blog Search and landed on the Short Gut News. A lot can be said of patients who keep an online journal of their recovery. As one blog post describes, patients are becoming the new authority.

Once, during a phone call with Jasmina she mentioned Omegaven, having heard about it in Toronto. “Yes!” my voice nearly squeaked in excitement “I just read about it.” And went on to explain my recent ‘discovery’. “Why didn’t Sébastien get Omegaven?” I asked. Jasmina is always calm when she explains things… The short of her answer was that it wasn’t available in Winnipeg. And it’s very expensive. The cost is from $50 to $100 a day per child. In fact, Omegaven isn’t covered by Health Canada. It isn’t even approved by the FDA in the U.S. Why? Omegaven was created in Germany by the company Fresenius Kabi AG. An article in the Wall Street Journal from November of last year explains the German company’s reluctance to bring the drug to the U.S. market:

Omegaven isn’t what “we see as the best product for this kind of application,” says John R. Ducker, president of the research, development and strategic marketing division of Fresenius Kabi. He says Omegaven, developed 15 years ago, was intended as a supplement, and not to be given alone. The company says it doesn’t contain all the essential fatty acids babies need.

Mr. Ducker says the company’s new product, called SMOFlipid, “presents a better option for pediatric feeding.” The company believes the new product does contain all the essential fatty acids babies need and can be used on its own.

Fresenius Kabi says it doesn’t want to invest the resources required to test both products for approval by the U.S. Food and Drug Administration. It hopes to eventually sell the new product in the U.S., Mr. Ducker says, although no timetable has been set and no trials are under way.

SMOFlipid has been approved for adults in a few European countries. Dr. Puder responds on the continued use of Omegaven versus waiting for the new product:

While Dr. Puder says he isn’t opposed to testing the new drug, “I have a sense of urgency here,” he says. “We have something that works, let’s do Omegaven first. Then once we have something approved so these babies don’t die, we can test something else.” He adds: “We are miles and miles ahead on Omegaven. We have safety data on the patients. We have excellent outcomes on the patients.”

The news, that affects so many children born with Short Bowel Syndrom has sparked some debate, and we are anxious to see how the matter develops. Though Sébastien is no longer affected by Short Bowel Syndrome since receiving a transplant, the research is something that touches Jasmina. She doesn’t hesitate to encourage people who come to the hospital who have similar conditions to what Sébastien had. “I’m so proud of Sébastien,” she once told me. “He is an example for other kids…”

What is Omegaven?

In a previous post, we talked about the incredible team that takes care of patients like Sébastien who have short bowel syndrome. Today we’ll take a look at one new treatment that the GIFT team is using in Toronto Sick Kids… it’s called Omegaven.

But first, a briefing… As Sébastien was born with gastroschisis and endured an operation to remove all but 20 cm of his bowel after birth, he became dependant on TPN for nourishment. TPN is composed of two solutions: one consisting of carbohydrates, proteins, vitamins and minerals, the second consisting of plant-derived fat (lipid) – mostly made of soybean oil. With time, however, the TPN takes a toll on the liver, to such an extent that patients like Sébastien, who are over a year on the TPN, end up needing not only a bowel transplant, but a liver as well. As an article of Dream Online from the Children’s Hospital in Boston explains, the reason TPN causes liver damage has eluded the medicine for years.

In 2001, Jenna Garza, MD, was interested in researching the reason for liver damage from TPN and began experimenting with mice under Dr Mark Puder’s supervision. Kathleen Gura also participated in the research. Many experiments later, the team discovered the dramatic difference of results when using Omega-3 (Omegaven) instead of the usual soybean oil, as a fatty acid supplement. There was no liver damage in any of the mice being fed Omegaven. As Dr. Puder explains in the same article, with regular Intralipid: “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.”

In September of 2004, a young baby named Charles was the first to ever receive Omegaven. It came as a saving grace for the tiny boy, who after a few weeks showed improvement and now no longer needs a liver transplant. As of January of this year, 30 patients at the Children’s Hospital in Boston have been receiving Omegaven acording to an article in Newsday. In Toronto at Sick Kids, 11 babies are being fed Omegaven. This new method of feeding could affect over 30 000 patients in the U.S. alone according to the Boston Globe, however there is a problem. Omegaven is not yet approved by the FDA or by Health Canada and it may take months or years for it to be approved. Why?

To avoid too long a post, check back here for the second part next week.

Crocodile

“Sébastien is into crocodiles!” Jasmina exclaimed over the phone. “He likes playing with Weebles. I try to encourage him to learn to kneel up in bed, by holding onto the railing and then getting him to stand…” The exercises are all part of getting Sébastien to develop his gross motor skills, whereas his fine motor skills are excellent.

During the weekend, David told us “Sébastien says ‘crocodile’ now! He actually said it on the phone: croc-uh-dile!”

For those who were wondering about Sébastien’s procedure on Friday: it’s called a Barium Enema and the tests revealed no anomalies.

A balancing act

Between hope and worry of Sébastien’s situation, there are the calm spots, when one simply listens and learns… They are the moments I most enjoy. Like when I hear Jasmina explain something I didn’t know before. It happened in our last call when we came upon the subject of Sébastien’s kidneys. It is unfortunate that he had a kidney failure, otherwise he might have had a faster recuperation. But, far from bitter, with an attitude that takes things as they come, Jasmina explains that there are two kinds of kidney failure… chronic and acute, or in her terms: “some children pee like crazy but don’t clear toxins, and others like Sébastien, don’t pee at all”. So besides needing dialysis to rid the body of toxins, Sébastien also needs dialysis to regulate his fluids and electrolytes. And in order to control the amount of toxins in his body, Sébastien has certain diet restrictions – like a controlled amount of protein - which helps a child grow and develop. Limited protein thus causes a slower growth rate. But far from worrying, Jasmina proudly tells me that Sébastien is 69.5 cm long. “Last month he was 67cm!” 

“Sébastien is an acrobat!” I tease thinking of how finely balanced the human body is. Sébastien is the acrobat walking a tightrope, as Jasmina and David are the safety net below him. We watch as he moves along, slowly, one measured step at a time.

The short bowel syndrome heroes

Not long ago, the Toronto Star published a special section in their newspaper, concerning SickKids. One article in particular pertained to Sébastien, explaining Dr. Paul Wales’ initiative to save more children who are born with Sébastien’s condition. Dr. Wales took an interest in short bowel syndrome after seeing the case of a baby girl seven years ago. “Doctors are faced with a no-win dilemma: let the baby die that night or perform the transplant, which would give her six to 12 months of life before a ‘miserable death’”. After earning his master’s degree in Scotland, he returned to SickKids in 2002 and organized GIFT – the Group for the Improvement of Intestinal Function and Treatment. As Hospital News describes, it began as multidisciplinary “research group looking to develop best practice standards for the treatment of intestinal failure”. It is interesting to note that in 2001, there were about 20 new cases of Short Bowel Syndrome in a year at SickKids NICU. Jasmina has already mentionned Dr. Wales with admiration, understandable when you read what motivates him: “I always think to myself, ‘What would I do if this was my kid?’”.

So what does GIFT do?

• GIFT has a standardized approach
• GIFT puts babies on transplant lists sooner, “raising their chances of making it to surgery”
• GIFT assures continuity of care by meeting weekly to discuss patient cases and treatment plans
• GIFT thinks of ways to improve bowel function so that prolonged TPN isn’t necessary, in order to avoid a transplant

GIFT has become the largest program of its kind in Canada and caters to over 80 patients. The program obviously helps: “when 54 newborns with short bowel syndrome treated under GIFT over a three-year period were compared with 40 pre-GIFT newborns, the success of the program was clear: while the overall mortality rate stayed the same, the mortality rate for the babies with liver failure dropped to 46 per cent from the pre-GIFT era’s 90 per cent.”

What kind of new therapies are the team trying? For a detailed look, check back to find out!

Into the O.R.

Today, Sébastien is scheduled to have his NG tube removed and have instead a feeding tube placed directly into the stomac – a fairly short operation supposedly lasting under an hour, with very few risks. A short article from Medecin Net explains the procedure here. Wikipedia also has a short article on the subject and compares two techniques for placing a PEG here. A longer article from Health Extra, the website done by Cleveland Clinic also explains the procedure here and shows a diagram of the PEG tube.

Good luck little one!

UPDATE: Sébastien’s operation has been re-scheduled for the 15th of this month due to a misunderstanding. It has turned out to be for the better though, as Dadda will be back in Toronto for a visit during that time.

An arrival of good news

“Gone to the gym, back at 9:30″ I thought of writing… But Christian already knew I was going before he left for the airport. I grabbed my coat, keys and gym pass, feeling the excitement of getting back into my routine workouts, which had been abandonned for some time after the miscarriage, in favor of House and Grey’s Anatomy. I came back home to Christian who had decided to shovel the walk. These past few days, snow fell like icing sugar, enough to dust everything white, but not enough to worry about. It has noticeably accumulated on the non-major roads where our little Honda Civic spins and slides before finally getting ahead.

“So, everything went well?”
“Yes! The flight was only 10 minutes later than they said”
“And David is fine?”
“Yes… he’s really excited about Sébastien. He’s eating everything!”
“Is he digesting what he eats?”
“Yes. Except for that piece of carrot that came out of his ostomy, but yes, he digests everything. They give him yogurt in the morning… And they’re teaching him how to crawl!”
“Wow! That’s amazing!” Amazing how not long ago we were so worried, and now everything is looking so promising.

Later as we are about to go to bed Christian remembers: “Oh and it’s cute… Sébastien sits in his chair and plays with toys while listening to a movie and David and Jasmina can leave him alone. And when the credits role, he waves ‘Bye’ to the tv screen.”

I smile. I don’t recognize the Sébastien that I remember from some months ago. He is changing and growing and charming us all with his vivacity. His platelets are up and he is scheduled for a small operation on Tuesday to remove his NG tube and have instead a tube that goes throught the skin to the stomac, thus continuing his enteral nutrition. This will facilitate eating for Sébastien, as he will no longer have to contend with the irritation of a tube in his throat.