Phoebe was very excited when we got to this new doctor's office because they were playing Moana. She couldn't see the tv as it was in the corner, but Moana was singing "How Far I'll Go," and Phoebe immediately recognized the song from the singing necklace Cordelia has at home.
We got signed in and also got set up with Stanford Children's online account. It was much easier to set up than John Muir's.
A nurse took Phoebe's weight and height. Phoebe was nervous, but I tried my best to "sportscast" everything that was going to happen to her before I did it. "This is the scale to see how big you are. You get to sit on it. I'm going to put you down on it so you can sit." And then I put her down. She still cried about the scale, but then when I described how we would measure her her, she was actually very calm and listening to me.
Then we went to the exam room and Dr. B was already there and ready to meet with us. It ended up being a super long appointment.
She already had the results sent over from the MRI, which showed...... Phoebe has arthritis! I didn't actually look at the MRI yet, but I guess it showed evidence in her synovial tissue (Definition: connective tissue that lines the inner surface of capsules of synovial joints and tendon sheath. It makes direct contact with the synovial fluid lubricant, which it is primarily responsible for maintaining) and also in the lymph nodes of her knee pit.
She said, she wished we had seen her first, she might not have needed to go forward with the MRI, but whatever, at least they didn't tell me there was a ^%&$-ing tumor or something, which is what I was scared they were implying-but-not-saying-it to me before. We have at least gotten to this place. I think the orthopedics were still more open to other possibilities because she didn't change much under Motrin that first weekend, but Dr. B pointed out that anti-inflammatories take time (several months) to build up in your system. She said that she felt like this was a very classic presentation of JIA, just needed to determine which type.
She said that when her nurse said they wanted to prioritize our appointment and squeeze us in, she at first thought she'd said "There's an 18 year old who may have JIA..." and didn't see the urgency, but then she realized it was an 18 MONTH old, and of course wanted to see us right away! :)
At first, she was hopeful it was oligoarticular JIA, (oligo, meaning "few"). This would mean only a few joints would be affected, four or less, and typically large ones like knees and elbows.
We discussed how Phoebe's left knee is the most obvious problem, but I thought it could also be in her right knee. She agreed, and as she examined Phoebe, she said she also believes it is in her left elbow (she could not rotate/twist it fully) and also in her fingers, between her finger knuckles and the knuckles at the base of her hand. Honestly, I never would have noticed. But they are very, very so slightly more swollen than on her left hand and she seemed to not want to bend them.
This was very interesting to me because I still see Phoebe using both hands without an obvious dominant choice. Like, sometimes I think she is right handed, but then she goes to using her left hand so often. Now I wonder if she IS going to be right hand dominant, but her right fingers hurt, so she switches to the left. Phoebe kept wanting to draw with the pen I was using to take notes with and we experimented by trying to make her hold it in her right or hold two pens at once. She passed the pen to her left hand and wouldn't use the right. It was super interesting to observe with the doctor right there. Dr. B was very gentle and thorough in her examination, and Phoebe stayed very relaxed. Some crackers to munch on also helped.
Because of these other locations, Dr. B classified her as having polyarticular JIA. (poly, meaning "many"). This means that 5 or more joints were affected.
She examined Phoebe's left leg more and measured it, compared to the right. She said there is about a 10 degree significant contracture, as she couldn't fully straighten it. We need to treat the arthritis or she will develop leg discrepancies, like they can grow to be different lengths and stuff. She also said that there seemed to be a loss of muscle mass in her leg, as she has been favoring her right. (Noticeable in the asymmetrical leg fold in her left thigh but not in her right that Dr. Jen had pointed out to me) We will be needing to do some PT in the future.
She said that JIA does have genetic polymorphisms, so basically there is a genetic factor, but just because one person has it, doesn't mean another will get it. For instance, in identical twins, even with the same genetic make up, one could have it, while the other does not. More likely for Phoebe, it was triggered into activity by a viral infection, such as what she had in November.
So now we are going to start talking treatments. I am again glad for the facebook page, because even though I don't know anything about any of these medicines, all the names were familiar to me already, as I've seen people posting about them.
First, we are going to be starting naproxen, which is a nonsteroidal anti-inflammatory, similar to ibuprofen. It is a liquid and she will take it twice a day.
Second, we are going to have joint injections of corticosteroids in her knees and elbows. She will be put to sleep for this as well, and we will have to go to Paolo Alto for it. As she will be asleep, they will also do our next blood draw for more labs. They are going to want to see if she tests positive for a rheumatoid factor, ie the more classic rheumatoid arthritis that adults get and a small subgroup of children can also have.
Third, we are going to see a pediatric ophthalmologist to have her eyes checked for inflammation. They will also want to know if there is a genetic component, if her blood work tests positive for antinuclear antibodies (ANA). If so, she will also need to have her eyes checked every three months. If not, they will want to check her eyes every four-six months, as they determine how she progresses.
Those are the first steps. The next things we will do after, will be adding more medications.
Methotrexate will be given by injection, which we will get to administer ourselves at home several times a week. This is a disease-modifying anti-inflammatory drug. This medicine is not supposed to relieve the pain or lessen inflammation right away, but they prevent joint damage and bone destruction. This drug is also used in chemotherapy and has a bunch of side effects. But my handout says it has been used to treat JIA for more than 25 years.
Next, we would add a biologic drug. This is a new class of drugs used to treat diseases with biologic response modifiers. These medicines stop the immune system from causing arthritis inflammation. These have more familiar names from those annoying pharmaceutical commercials, like Enebrel and Humira.
All of these medicines come with fun lists of side-effects, like nausea and loss of appetite and headaches, but the methotrexate and biologics are immuno-suppressants and come with the terrifying warning of increased cancer risk. 😱 😱 😱
This is some serious shit, guys. My little baby is going to need all these medicines. It's really scary.
But for today, I'm overall not really upset. Instead, I'm kind of amped that we have a diagnosis and are going to do what we need to make Phoebe better.
This is not something she is going to outgrow, but if we can treat it aggressively, hopefully we can attain remission. (And even then, flare ups are still possible). Remission is only declared after at least 6 months of treatment, and then 6 months without treatment and no symptoms returning.
My handout says that children with JIA today are already less affected by joint damage and disability than children 10 years ago.
Anyway, Dr. B and I talked so long that she actually said she needed to move on to other patients and she is going to call me tonight. She gave me paperwork to finish up (Phoebe was ready to leave at this point) and also consent form for her joint injections to be faxed back.
My appointment ran late, so I wasn't sure how to best use my time next. Was it still worth it to drop off Phoebe and go in to work for 45 minutes? And yet it was also still too early to pick up Cordelia from school, she'd be in the middle of lunch. My school's director was able to say they'd manage without me (even though today was crazy with it being Valentine's Day), so I took Phoebe to her Meme's house to have her lunch. That way I was able to sit and go through all this information with Meme, without interruption. Then I took Phoebe with me and picked up Cordelia.
I also spoke to Chuck from the orthopedic's today. While I was on the phone with him, the optometry office called about our "urgent referral". I called them back; Phoebe's eye exam scheduled for the 23rd at 8:15.
CVS pharmacy called and said they do not have naproxen and will have to order it, but hopefully in by tomorrow.
UPDATE: Dr. B did call me and went over a few more things. She said that another patient of hers was trying to get their naproxen at a CVS and they were unsuccessful. She was trying to find out if it's just the same store or that there could be a shortage, which is apparently something that can happen sometimes?!
I also asked her about CCS. She said she can make a referral, but it was her experience that there were a lot of paper work hoops to jump through and also took time to be seen. She wants us seen soon, so she is going to send us to someone back with John Muir for PT and OT for her hand.
P.S. Phoebe's cheeks looked fine this morning, she had one tiny red dot. My other theory was that she had a bug bite outside or something that was making her puffy, so maybe that was the case. That's pretty much gone now too.
In other news, there was another horrible school shooting today. As a teacher, it just appalls me, and it never stops. Do people not realize what is important in this world? When your baby is in pain and facing a Diagnosis..... this is happening all over. These are the things that matter, and yet we can't even keep our healthy babies safe at school because of gun violence? It's just sickening to think about.
In the meantime, I didn't take any videos today, so I will just end with some pictures (not from today) of my brave little girl. Isn't she just the sweetest?
Child Life Specialist from Mills (I bet). That's what Magali and my float, Christine, were (both went to Mills). --Judy
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