Tuesday, August 21, 2012

Life with TPN

I cannot believe we have been home for almost two years with TPN therapy! For those of you who think that your children/family members cannot survive on long term TPN...it's simply not true! My daughter, with all of her diagnoses is still thriving. She is living life and enjoying almost every day. The ONLY reason she is enjoying life is because we are able to be at home on Home TPN! We are very busy now, but I don't think Kaitlyn has ever been so stable :)

Sure we still have bad days. Kaitlyn is still chronically anaemic. Her doctors have diagnosed her with multifactorial anaemia. This basically means that there are multiple factors which cause Kaitlyn to be chronically anaemic. Some have also called Kaitlyn's anaemia: Anaemia of Chronic Disease. Not sure what to think. We have also, interestingly, found that Kaitlyn has reduced production of the hormone Erythropoietin. EPO (or Erythropoietin) is a hormone responsible for telling bone marrow to produce red blood cells. No wonder she is chronically anaemic! So what can we do about this? We have been replacing the Erythopoietin with a weekly shot. It is a sub-Q shot (subcutaneous injection) that totals 1/2 ml of EPO hormone :( Kaitlyn does not like this needle. Even freezing the site with Emla does not take away all of the burn with the larger volume! We freeze the site with Emla, cool the area with an ice pack for a few minutes, and then use BUZZY the Bee to help with the pain. All of this seems to make it easier for Kaitlyn :) She is such a trooper!

Our other biggest battle has been with Kaitlyn's Hypercalcaemia! She has been quite ill with this condition from time to time. Anaemia makes her feel bad, but hypercalcaemia makes her feel nauseous and adds to her dismotility issue. She will also start napping through her bad days, which is how we know she is feeling really not herself!

Kaitlyn's hypercalcaemia is caused by her bones leeching calcium into her blood stream! This is a continuous event! High levels of calcium in the blood stream is NOT normal and like any other electrolyte/vitamin imbalance can be very harmful! Our doctors feel that Kaitlyn's hypercalcaemia is linked to her SMA or lack of mobility. Curious to me that in the HUNDREDS (yes hundreds!!) of SMA families that we know that not another single family is dealing with this issue. I am inclined to believe that Kaitlyn's hypercalcaemia is NOT related to her "original" diagnosis of SMA (Spinal Muscular Atrophy).

We have been attempting to control Kaitlyn's hypercalcaemia with a biphosphonate. The biphosphonate we have chosen is Pamidronate. Pamidronate is well known in the SMA community as it is typically used to increase bone density. Weak bones are VERY common in SMA and many children with Spinal Muscular Atrophy suffer from dislocated, broken or fractured bones. As weak as Kaitlyn's bones were I am glad to say that her bones to our knowledge were never broken or fractured!

We have been doing pamidronate infusions for the past year. We did do a bone density scan (dexa scan) at the beginning of the pamidronate infusions. We were scheduled for a scan four weeks ago but the bone density machine was broken. I am not too sure what to expect from pamidronate in terms of bone density. I mean if you were constantly leeching calcium into the blood stream and pamidronate locks the calcium back into the bone....and we are not adding even a normal RDI for calcium....then I do not see how we could possibly be strengthening Kaitlyn's bones! I guess the dexa scan will tell.

Kaitlyn has also been diagnosed with bronzing. It is a condition where her skin appears to be bronzed, or tanned. Everyone comments on how Kaitlyn has a beautiful tan....like we would take our paralysed daughter out into the sun and let her burn!! URGH!!! Of course not!

Our next HUGE adventure! A larger van! We have been looking for vans in our price range in Canada for the past few years! Transporting Kaitlyn in our current van with our current ramp system (suitcase ramps 75lbs plus!!) is NOT possible for much longer! We have finally found a van that we are hoping will be perfect! We cannot afford this van,  but where there's a will there's a way!!! It will need a couple of modifications to make it safe for Kaitlyn to travel, but when done it will be better than what we have now.

Thanks for reading folks and I hope to make many more posts in the near furture!

Some pictures of our new way of life ;)

Kaitlyn's port....her life-line if you will:

How the lines are connected...making moving her around even more of a challenge ;)

Kaitlyn's IV pole is getting a wee bit crowded ;)

IV meds as her gut simply cannot absorb meds very well anymore.

It is critically important that we keep everything super clean now!

Our new way of administering medications.

A recent pic of our happy girl :) This one was before the bronzing diagnosis.

This picture was snapped only about a week ago. You can really see the bronzing in this pic.