The stages of an infusion

 

1. Anxiously waiting

 2. The IV, includes screaming and lots of stress for all involved

3. Exhaustion

The infusion is no fun, Emily hates being back in the hospital even for a few hours, she hates the IV and let's face it those hurt, she is always asking about when the next one is and how long till it happens.  "How many days till my fusion?" is the big question on this sweet girls mind.  It seems to be working so far, other then being tired a lot there is no rash or fever present so we are happy about that.  We also talk about how she has no more shots.  Since the Kineret burned so much we try to tell her how this is so much better the infusion is, but I don't know that helps much.  Nothing about this nasty disease is pleasant.

Rheumatologist

We had our bi-weekly visit with Emily's Rheumatologist and my suspicions were right.  She's been more tired then usual and I had a feeling as we were weaning down on the steroids the disease was becoming more active and labs confirmed this.

There are two different inflammation markers the doctor looks at; one tells if MAS is coming back and one tells us about SOJIA and how active the disease itself is. 

It's now I have to remind myself there is no cure only control through medication.  The medication she is on right now is Prednisone, Cyclosporine, and Kineret.  Kineret is the long term medicine that is supposed to control everything and eventual be the only medicine she takes.  Prednisone is like a band aid medicine, it's what she got in the hospital because she was so very sick.  Prednisone reduced the inflammation quickly and controls it very well, but the side effects are pretty nasty (think best case two hip replacements eventually) and can be life threatening if taken too long so we have to get her off it as quickly as possible. 

We found out yesterday her D-dimer, white blood cell count and platelet counts were all elevated which means SOJIA is still with her and becoming more active as we wean down on the steroids.  Good news was her feritin levels were low, not normal, but not 24,000.  Normal is around 60, Emily's now is a little above 100.  In the hospital with MAS they were 24,000.  So anyways MAS is under control; SOJIA not so much.

I'm worried it's not working as well as we wanted it to, but happy we can try a different medicine.  This one is given via IV in the hospital twice a month.  They can also do labs at the same time so if the IV goes in well that means just two pokes a month...hallelujah!  There is a huge emotional toll that is being paid and if we can reduce that cost just a bit I will take it.  Let's just hope this new medicine works.

Our daily dose of pain

Sometimes the daily injection goes well and sometimes it does not.  Last night it did not.  Emily tried to grab the needle twice.  I have to assume it is the anxiety about the pain that makes her do this because no rational child would try to grab something they know is very sharp and would hurt their hand as much as any other part of their body.

In this video the patient talks about having adult on-set Stills, same thing as SOJIA except in an adult, and he cringes before each Kineret injection.  He says it hurts if he touches the injection site and even three hours after it will still hurt him without toughing it. 

Emily screamed in anger after the shot last night.  It was painfully loud (I'm holding her during this).  Then I think about the fact I have to travel for work for two months very soon and my husband will be the one.  His wonderful sister is giving a week of her vacation time to come out and help when I first go, and it's only for two months, but I already know what she will be screaming; "I want my mommy!" and that will be so loud it already hurts my heart.

We've talked to the doctor about this and we could eventually try a weekly infusion instead, but that would be time in the hospital once a week.  It sure wouldn't hurt as bad so we may go that road, but we have to wait until I get back from my travel and Emily has come down more on the steroid dose.  Then there is still a chance she may relapse because the IV medicine could take up to a week to start working.  Then there is also the fact that Kineret is supposedly the best there is out there for SOJIA especially.  Would we be risking her health only to improve her quality of life slightly?

I'll leave you with something I read last night because it seems to fit (although I am sure Emily would not think this trial very "light" and to me is feels heavy too).

2 Corinthians 4: 17 
For our light affliction, which is but for a moment, worketh for us a far more exceeding and eternal weight of glory;

Side Effects

Every time I give Emily her medicine I think about the side effects.  Short and long term; I wonder if when she wants to have children someday if she will be able to.  A six year-old boy died last year from complications of all these medications so I worry about that too.  The thought that I could be hurting her as much as this nasty disease is always in my mind, but what choice do we have?

Prednisone

Get emergency medical help if you have any of these signs of an allergic reaction to prednisone: hives; difficulty breathing; swelling of your face, lips, tongue, or throat. Call your doctor at once if you have any of these serious side effects:

blurred vision, eye pain, or seeing halos around lights;

swelling, rapid weight gain, feeling short of breath;

severe depression, unusual thoughts or behavior, seizure (convulsions);

bloody or tarry stools, coughing up blood;

pancreatitis (severe pain in your upper stomach spreading to your back, nausea and vomiting, fast heart rate);

low potassium (confusion, uneven heart rate, extreme thirst, increased urination, leg discomfort, muscle weakness or limp feeling); or

dangerously high blood pressure (severe headache, blurred vision, buzzing in your ears, anxiety, confusion, chest pain, shortness of breath, uneven heartbeats, seizure).

Less serious prednisone side effects may include:

sleep problems (insomnia), mood changes;

acne, dry skin, thinning skin, bruising or discoloration;

slow wound healing;

increased sweating;

headache, dizziness, spinning sensation;

nausea, stomach pain, bloating; or

changes in the shape or location of body fat (especially in your arms, legs, face, neck, breasts, and waist).

Cyclosporine

headache

diarrhea

heartburn

gas

increased hair growth on the face, arms, or back

growth of extra tissue on the gums

acne

flushing

uncontrollable shaking of a part of your body

burning or tingling in the hands, arms, feet, or legs

muscle or joint pain

cramps

pain or pressure in the face

ear problems

breast enlargement in men

depression

difficulty falling asleep or staying asleep

Some side effects can be serious. If you experience any of the following symptoms, or those listed in the IMPORTANT WARNING section, call your doctor immediately:

unusual bleeding or bruising

pale skin

yellowing of the skin or eyes

seizures

loss of consciousness

changes in behavior or mood

difficulty controlling body movements

changes in vision

confusion

rash

purple blotches on the skin

swelling of the hands, arms, feet, ankles, or lower legs

Taking cyclosporine or cyclosporine (modified) may increase the risk that you will develop an infection or cancer, especially lymphoma (cancer of a part of the immune system) or skin cancer.

Kineret

Injection Site Reaction

Upper Respiratory Tract Infections

Headache

Nausea

Diarrhea

Sinusitis  

Arthralgia

Flu Like Symptoms

Abdominal Pain

Kineret has been associated with an increased incidence of serious infections.