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.

Happy Mother's Day

Wow, I cannot believe it has been several weeks since I posted. We have another transfusion under our belts. Kaitlyn's hemoglobin got as low as 65 this time in a little less than five weeks :( I think it's time for a referral to hemotology on this issue!

We are also meeting with our Nephrologist this Wednesday to see what we can do about Kaitlyn's hypercalcemia. There are ways to treat hypercalcemia if the cause is identified. Our nephrologist believes that Kaitlyn is probably leching calcium from her bones. This concerns my husband and I a great deal!! Kaitlyn is already osteopaenic. Her bones are brittle and she has now been leeching calcium from them for the past almost six months!!!! We have had bone density scans done for the past three years. Kaitlyn was due for more scans this past January! Our doctors felt that the scans wouldn't be much help? I'm not sure I agree :( I feel that we would have a fairly good comparison from the past three scans. Perhaps it would give us some insight as to the extent of bone degredation (if any) to the calcium being slowly removed from her bones! If she is leeching calcium from her bones then there are ways to prevent this from continuing!

One way is to treat Kaitlyn with bi-phosphonates. One such drug is called Pamidronate which is used by several SMA children for brittle bones. It helps keep calcium in the bones. Currently we are adding ZERO calcium to Kaitlyn's diet!! This concerns me greatly as calcium is good for more than just bones! Our doctors have increased Kaitlyn's sodium and fluid intake trying to get her to "pee" off the excess calcium in her blood. So far this has been working, but it is only a band-aid! I do not believe that she has an indefinite supply of calcium and I also do not want to wait until we break one of her bones before we treat the problem!

We had a rough week two weeks ago. Kaitlyn's J-tube blocked when I was giving her afternoon meds! This happened on Monday afternoon. We couldn't take Kaitlyn in until Monday evening. By then, being Easter Monday, there was nobody around who knew what to do with Kaitlyn. They admitted us so that we could see our surgeon early Tuesday morning. By admitting us they could also administer some IV fluids/meds to replace the fluids Kaitlyn would be missing from her J-tube. Kaitlyn gets about 300cc's per day through her J-tube. Not to mention medications.

Anyway, my story....Tuesday morning arrives and our surgeon comes to tell us the bad news. We haven't a replacement tube in the hospital. Kaitlyn has a GJ Mickey Button. To my knowledge she is the ONLY child using this tube in our children's hospital. Our surgeon had to order TWO new tubes from the States. He had surgery time tentatively booked on Thursday. Rather than discharge us from the hospital, they sent us home on a pass so that we had the IV fluids we needed and IV medications. We went back to the hospital on Thursday in the morning. We were scheduled for afternoon surgery. Our surgeon came up in the afternoon to tell us that the J-tube hadn't arrived, it was stuck in customs at the airport :( We decided to stay overnight and Kaitlyn would have her surgery Friday morning. Kaitlyn had her surgery and everything went well. She lost another tooth when the anesthetist intubated her :( She is now missing both her two front teeth.

This weekend has been quiet. We went "shopping" Saturday. Shopping is in quotations because first you have to have money to shop! LOL Owen needed a new pair of rubber boots and the family wanted to get me a movie I've been wanting for Mother's Day. The kids each picked a toy as well...nothing big. Well, Kaitlyn did spy an Ernie that she NEEDED!!! She said "need Ernie...need Ernie"!! How can we refuse when she asks so sweet!! I hope everyone had a pleasant Mother's Day!!    

Kaitlyn and Daddy (with Ernie) having a cuddle in the rocking chair.

Kaitlyn's new toothless smile :)

Kaitlyn having a cuddle with Mama! She doesn't exactly fit in my lap anymore!!

Owen holding his Freckle Face plant! He picked it out and has been doing EVERYTHING with it since we brought it home! He even took it to watch him have a bath and ate with it at the dinner table :P

My WILDMAN!!! Kaitlyn's twin Alexander :)