Nicole started a new medicine, Remodulin, on July 25th which is delivered subcutaneously (SQ) . This means that the medicine is absorbed through the skin through a tiny catheter. She uses a pump that gives her a continuous supply of medicine... like a diabetic.
Nicole seemed to be doing well. On Aug. 27th she took her inhaled medicine for the last time. It is Remodulin in the inhaled form and the doctor wanted her to slowly wean off of it. It seems ever since this time she has had more shortness of breath and more fatigue/tiredness. If she is without oxygen for more than 10 minutes sometimes her lips will start to turn blue. Her ability to exercise at rehab has decreased.
Until we start with a stronger concentration vial of the medicine we have to change Nicole's syringe of medicine and the pump every 2 days a process that takes about 20 minutes if everything goes smooth. Every other pump change came with an increase in the medicine. Nicole seems to be tolerating the medicine well.
I contacted the PH nurse earlier in the week as I am concerned that Nicole isn't feeling well. She was complaining of site pain again also. The nurse recommended the site be changed. On Friday the Accredo nurse and a local nurse were here to help Nicole and I do the site change. This means that the administration site is changed. Nicole's initial site was on her left side stomach area. A decision had to be made where to put her next site. It was decided that we would try the middle abdomen area. Nicole put in the site which at the time of insertion should have no pain other than a prick when the needle goes it and then when it is pulled out. Nicole kept having burning pain so the nurse said that wasn't normal so the site had to be taken out and was moved over closer to her side. It is almost in the same spot as before except on her opposite side. This worked a lot better.
We had an appointment with the doctor that afternoon. She was suppose to do a six minute walk test to see if there has been improvement from a month ago but she wasn't feeling well and just her walking to the back had her SOB (short of breath). Liz could tell she didn't feel well and her oxygen saturation levels were low, even for her. Liz had said she was going to tell the doctor to see her first and then to tell her to do the six minute walk. He didn't make her do it. She would have done worse than 6 weeks ago and she did pretty bad then. I explained to him her symptoms and how ever since she stop the Tyvaso she has felt bad. She was squeezed in for an echo which he said showed no sign of decline from the last one she had which was earlier this year. He said that he is stumped as to why her symptoms are worsening. He told me to get her a chest x-ray next week just to rule out pneumonia since she has had some congestion. He also said that instead of every other syringe/pump change do an increase of medicine every syringe change.
I started the 2.5 mL vial yesterday so according to the pharmacist this will last 12 days. Since we will be doing an increase every two days this means it will only last for maybe 5 more pump changes. The pharmacy called during her doctor's appointment and I wasn't able to take the call. I was told earlier in the day that the pharmacy had gotten the authorization from Medicaid for the 5 mL strength vial :). I know they will be getting that to me next week once I call them on Monday (which will be another long, busy day more for me than Nicole). I also have to compare the dosing chart that I have that tells me how much medicine to put in the syringe, what to set the pump at and how many nanograms of medicine it is and coordinate it to the 5 mL chart.
I'm hoping that this increase in medicine will start to make her feel better. Nicole tends to downplay everything but I know that something is bothering her when she has been quite grumpy which isn't like Nicole. Today she will start the battle of site pain which will probably last until for about 7- 10 days during which time she will be taking pain medication every six hours so it doesn't get too unbearable. Last night she started walking hunched over because of the pain and that will probably continue through the weekend so sitting is the best option for her. My prayer is that she won't have the pain but if she must it will be tolerable.
Yesterday when we were at the doctors, a lady who was there with her husband came over and asked me about Nicole portable oxygen concentrator. I answered her questions and she told me about her husband. She told Nicole how beautiful she was (which she is) and that she hoped that she would get better. Another lady who worked at the office saw us when Nicole was getting her echo and she said hi to me and wished my luck and blessing and seemed very nice. When we were done with the doctor the office had already closed. The doctor pushed Nicole in a wheelchair out into the waiting room as my husband was coming from work to pick us up. The same lady that I saw earlier outside of the echo room came out and told the doctor that she would make sure we got out of the building when Rick got their. She stayed with us and talked to Nicole and I. She said that she knew the doctor would do everything he could for her and she was in good hands. She asked if we had been there before and I said "yes". She said she recognized Nicole. She also told Nicole how pretty she was.
While we were waiting for the doctor to read the echo a friend called me. This person we only know because of Nicole's illness. She had a dinner for us and wanted to drop it off as she knew we had had a long day. She has done so much for me and especially Nicole and for that I am forever grateful. I am forever grateful for her doctor who has also gone above and beyond to help Nicole.
It is very difficult to watch Nicole be so ill, but I am no different or more special than any other parent who has a seriously ill child. It saddens me to watch Nicole some days only focus on her illness and how bad she feels and how hard her life is instead of all the blessings she does have. Nicole doesn't complain but I can see it through her attitude and anxiety. She is beautiful, sweet, kind, intelligent, a very gifted artist and has people who really care about her. I know she is mad at God for letting her be born sick (her congenital heart defect) and doesn't understand. I try to tell her that in some ways she is more special because not every teenager could go through what she does every day. She is an inspiration and some day I hope that she will see that.