Oh my dear...what has it been?? 6 months..has it really been 6 months since I posted last? A friend of mine says that she keeps checking the blog to read Mother's Day, and is sick of seeing the same post! I guess I better get back to my regular updates! So much has happened in the past six months!
Where did we leave off? Oh yeah..hematology. We had an appointment with hemotology back in the spring and they have diagnosed Kaitlyn with a Multifactorial Anemia. Which is a polite way of saying "we do not know what is causing her anemia" (their own words!). They have also used the term Anemia of Chronic Disease, just another way of saying the same thing really. There seem to be multiple factors involved in causing Kaitlyn's anemia. I had them test Kaitlyn's erythropoietin levels to see if she was making enough of this hormone. Erythropoietin is a hormone produced in the kidneys that tells bone marrow to make more red blood cells. Her levels came back very low normal....but just within normal range.

I remember questioning the hemotologist at the time and saying since she was low normal would we not start her on a trial of Erythropoietin replacement, and he said no her levels are normal. Two weeks ago when we were in seeing the nephrologist Kaitlyn was once again very anemic and he asked if we had ever had her Erythropoietin levels checked. I told him we had the test done back in the spring and that her levels came back low normal. He checked the records and noticed that she had been anemic at the time that the test was taken. The nephrologist stated that the Erythropoietin levels should have come back high normal or even higher as her body should have been trying to stimulate red cell production. I am a little perturbed that we didn't start the Erythropoietin therapy back in the spring when I questioned the levels. The nephrologist started Kaitlyn on Erythropoietin that very day and we have since had another transfusion. If the hormone replacement therapy works then hopefully Kaitlyn will not need such frequent transfusions. Erythropoietin is the second hormone related to the kidneys that Kaitlyn does not produce in sufficient amounts. More research needs to be done on SMA children and hormone levels!

Kaitlyn has also started Pamidronate Infusions. Pamidronate is a bi-phosphonate drug that will help Kaitlyn bind the calcium into her bones. It is that drug that will have a twofold effect for Kaitlyn. For one, it will help control her hypercalcemia and it will help increase her bone density. Kaitlyn's serum calcium levels are much more stable now. Her ionized calcium still comes back high, but not near as high as pre Pamidronate. We feel like the drug is working for her. We are currently using a quarter dose of Pamidronate (based on weight) as Kaitlyn had a bad reaction to Pamidronate after the first round.

In the beginning we gave Kaitlyn a half dose of Pamidronate the first day and a half dose the second day. We wanted to be cautious with Kaitlyn and divide the first dose over two days to avoid Kaitlyn dropping her calcium levels too quickly! But Kaitlyn had her own twist! Kaitlyn developed a secondary Fanconi Syndrome. This is where the kidneys shut down and start to spill glucose and amino acids in the urine! Since we believed the Fanconi Syndrome to be secondary to the Pamidronate all we had to do was wait and see if the condition corrected itself in the absence of the medication. Kaitlyn's body slowly recovered from the Fanconi Syndrome. We waited about eight weeks and then decided to try Pamidronate again at a much lower dose. When Fanconi Syndrome is medication/drug induced then there is a level of the drug that should both produce the desired effect medically without causing the Fanconi Syndrome. We have been giving Kaitlyn a 1/4 dose based on weight since the summer. We do the four hour infusions once a month and so far Kaitlyn has had no issues. We recently went up a smidge on her dose as her calcium levels came back high again. Pamidronate one of the few drugs we have to try and control Kaitlyn's hypercalcemia.

We have (a month ago) also started Kaitlyn on a VERY weak vivonex solution running into her J-tube. The gastroenterologist has stated that if we can get even 10% of her diet delivered enterally then it would go a LONG way towards protecting her internal organs (including her liver, kidneys) and the lining of her intestine. For those of you familiar with the AA Diet and vivonex specifically; weare mixing 1 tbs (about 1/8 of a package of vivonex) with 240mls of water to make a .2 cal/ml mix. Kaitlyn gets this very dilute formula at a rate of 10cc per hour for almost 24 hours per day. So far Kaitlyn seems to be tolerating this mixture fairly well. She does have more green bile backing up into her stomach,but she is still straight drianage (and likely always will be!). This means that we leave Kaitlyn's G-button open at all times and allow any stomach fluids and bile to drain from her stomach. Since Kaitlyn's stomach is paralysed completely she does not have the ability to even tolerate her own gastric secretions. Her stomach does not contract to pus these fluids down into the intestine to be digested/used in the appropriate way. If we cap off Kaitlyn's G-tube she is gagging within minutes!!! She will vomit if her tube remains blocked for more than a couple of minutes.

Our hope is that the green bile we see backing up from her small intestine will lighten in time. Before starting the vivonex in her J-tube we were running water at the same rate 10ccs for 24 hours. The drainage at that point was pretty clear, almost all the time. Occasionally there would be a bit of yellowy bile but next to no green ever came out. I'm not sure if we will ever see a lack of green bile while on any sort of stomach feeds. However, she seems to be tolerating the feeds in every other way :) She is not bloated, she does not complain of tummy pain, she is not gagging on a daily basis and her bowels are still working!

Our latest news is of a more "normal" fashion! Kaitlyn is now requiring the use of glasses to correct her vision. She has astigmatism and is near sighted. Right now her prescription is pretty weak but her opthalmologist wanted her to have glasses in the hopes that it may help her Strabismus. She seems to really like her glasses :) Good thing! She looks pretty cute :) We have altered some ear pillows to accomodate her side lying position. So far we have had zero issues with the glasses. They do not mark her face or head at all!