Maya's reward for going through bloodwork in Boston In November we took Maya back up to the Children's Hospital of Boston, where she visited with two specialists to evaluate Maya's continuing sinus infections/ear infections/bronchialitis. The first specialist was with hematology, and he ordered a test for Chronic Granulomatus Disease (CGD), which fit with her medical history. We also met with an Immunology/Allergy specialist who ordered an array of tests which included her protective titer responses to her childhood immunizations.
Maya screamed bloody murder when the lab technicians (one of whom looked like Moby [the musician, not the whale]) held her arm still and inserted the needle for the blood draw. Forewarned that Maya was very hard to draw blood from, and prone to "blown" veins, "Moby" did an awesome job of getting her on the first stick and she actually calmed down and was able to say thank you to the lab techs when I prompted her to before leaving the lab.
One of the issues we spoke with the Immunological specialist about was her strange response to Levaquin and Bactrim, very strong antibiotics that work well for her. She get strange red bumps dispersed in clusters on her body, like a mild case of chicken pox. She had them on her while we were there, but they were disappearing. The Dr. said we should try Bactrim again, which we did after her bumps disappeared the next week and while she had a runny nose again and sickly symptoms. Sure enough the bumps returned in 3 days. The Dr. (via a phone call) changed her to another antibiotic in the ceph family at a "prophylactic dose," at the same time he asked for her to get re-immunized for her infant Prevnar and HiB vaccines, as enough of her protective titers from those vaccines were low enough to not provide her good protection against those illnesses. He wanted her to return for testing one month after receiving the vaccines for retesting. We were also informed that her test for Chronic Granulomatous Disease (CGD) was negative, and her red/white blood counts were normal.
So she got the immunizations, yet continued to be ill with sinus drainage and occasional low-grade fevers, even a case of pink-eye. Of course she became mostly clear just before visiting Boston last week for a consultation and re-testing. I should mention that during the days prior to the re-check I also treated her with some of the "cold-killer tea," albeit at a weaker concentration than usual, liberally sweetened with agave nectar, and mixed 40/60 with milk. I also used a NeilMed Nasal Irrigation device on her during the time between the visits, with a lot of nasty mucous coming out with each use. She actually thanked me after the irrigations, saying she felt better each time. So the net result was that she was still a bit "gunky" sounding at the re-check visit, but her nose was clear.
The visit last week to Boston was not very enlightening. The specialist repeated the previous tests, and added AH-50 and C3 and C4 levels, while telling me that he suspected her immune system was normal and that we should return to working with her Ear, Nose and Throat (ENT) doctor to check her out for anatomical issues. I explained to him that we had now come full circle, as it was the ENT specialist who recommended the Infectious Disease specialist in Hartford, who then recommended the Immunologist in Boston. He explained that from an immunological perspective she seemed within range of normal, and that he expected her follow-up blood work would confirm than her immunity was ok. He strongly recommended that we have our ENT perform a Ciliary Biopsy to check if her cilia (the little hairs in various parts of the body, especially sinuses and lungs that pulse in waves to move foreign matter and mucous out of the body) were properly working. He also upped her Ceph dose to the strongest possible.
We had the labwork performed by the same technician, whom I now call Moby, and he again did a spectacular first try draw from Maya. Smooth as Porcelain.
Upon my return from Boston, I emailed the ENT and brought him up to speed on Maya's continuing infections and what had occurred in Boston, along with the request for the Ciliary Biopsy. The Immunology specialist explained that a ciliary biopsy was a simple test with a brush a bit up the nose that scrapes off some cilia that can be examined under a microscope. Maya's ENT emailed back that the nasal scrape biopsy has been proven to be clinically useless, and let me know that to even have a 50% shot of a proper biopsy result Maya would need to go under general anesthesia and a sample would need to be taken via a bronchoscopy, not the nose.
Just today I was contacted back by the Immunologist, who had not yet received my email exchanges with the ENT, nor had he been able to win the first round of phone-tag with the ENT. The Immunologist explained that all of Maya's tests, with the exception of the AH-50 (which would take 2-3 weeks longer) came back normal, with excellent protective titers for all of the Prevnar-related pnuemococcal strains and the HiB. He said that from his perspective, her immune system responses were normal, with perhaps a bit of allergic response that doesn't show up in allergy testing. I agreed with that, because Brianna and I get the same allergic symptoms ("hay fever") to mold, ragweed and a few other things several times a year, with the difference being that when it progresses to an infection, Brianna and I respond positively to our first antibiotic, while Maya responds only to incredibly strong antibiotics, which happen to also give her bad diaper Candida rash and, in the case of Bactrim and Levaquin, body bumps.
The Immunologist said he had no explanation for her lack of response, other than suggesting some sort of ENT issue, such as the cilia. He said I should now work with the ENT with regular checkups every six months. When I asked if she should be rechecked annually for the same protective titers she needed a booster for, he said that we should, and that annual boosters might be helpful if the tests indicated lowered titers again.
So now we are left wondering why she is basically clear right now and for the past week (probably the Ceph, as she has finally developed her nasty diaper rash), and what to do to keep her clear in the future. It is very frustrating to have to "wait and see" with no real answers to very serious recurrent infections that often keep her home and unable to participate in daycare or even visiting people or playing outside. While I don't want Maya to have to undergo a surgical procedure, nor do we have the easy funds to pay for it (at least the portion that the insurance doesn't cover in our "donut-hole" period), I really feel that the seriousness of what might be Primary Ciliary Diskinesia (PCD) needs to be discovered or ruled out, so that we can have a plan for her treatment and know what to expect for the future. On the flip side, she may just have back luck and be a sickly kid who will grow out of it sometime in the next few years. Nobody can answer that with any certainty. There are many symptoms of PCD that Maya exhibits, and there are even possible genetic links with diseases associated with PCD on the maternal side. So for now the mystery continues and life goes on.
And of course, please donate to and pray for the Haitians and those helping them!
-Chris
Maya screamed bloody murder when the lab technicians (one of whom looked like Moby [the musician, not the whale]) held her arm still and inserted the needle for the blood draw. Forewarned that Maya was very hard to draw blood from, and prone to "blown" veins, "Moby" did an awesome job of getting her on the first stick and she actually calmed down and was able to say thank you to the lab techs when I prompted her to before leaving the lab.
One of the issues we spoke with the Immunological specialist about was her strange response to Levaquin and Bactrim, very strong antibiotics that work well for her. She get strange red bumps dispersed in clusters on her body, like a mild case of chicken pox. She had them on her while we were there, but they were disappearing. The Dr. said we should try Bactrim again, which we did after her bumps disappeared the next week and while she had a runny nose again and sickly symptoms. Sure enough the bumps returned in 3 days. The Dr. (via a phone call) changed her to another antibiotic in the ceph family at a "prophylactic dose," at the same time he asked for her to get re-immunized for her infant Prevnar and HiB vaccines, as enough of her protective titers from those vaccines were low enough to not provide her good protection against those illnesses. He wanted her to return for testing one month after receiving the vaccines for retesting. We were also informed that her test for Chronic Granulomatous Disease (CGD) was negative, and her red/white blood counts were normal.
So she got the immunizations, yet continued to be ill with sinus drainage and occasional low-grade fevers, even a case of pink-eye. Of course she became mostly clear just before visiting Boston last week for a consultation and re-testing. I should mention that during the days prior to the re-check I also treated her with some of the "cold-killer tea," albeit at a weaker concentration than usual, liberally sweetened with agave nectar, and mixed 40/60 with milk. I also used a NeilMed Nasal Irrigation device on her during the time between the visits, with a lot of nasty mucous coming out with each use. She actually thanked me after the irrigations, saying she felt better each time. So the net result was that she was still a bit "gunky" sounding at the re-check visit, but her nose was clear.
The visit last week to Boston was not very enlightening. The specialist repeated the previous tests, and added AH-50 and C3 and C4 levels, while telling me that he suspected her immune system was normal and that we should return to working with her Ear, Nose and Throat (ENT) doctor to check her out for anatomical issues. I explained to him that we had now come full circle, as it was the ENT specialist who recommended the Infectious Disease specialist in Hartford, who then recommended the Immunologist in Boston. He explained that from an immunological perspective she seemed within range of normal, and that he expected her follow-up blood work would confirm than her immunity was ok. He strongly recommended that we have our ENT perform a Ciliary Biopsy to check if her cilia (the little hairs in various parts of the body, especially sinuses and lungs that pulse in waves to move foreign matter and mucous out of the body) were properly working. He also upped her Ceph dose to the strongest possible.
We had the labwork performed by the same technician, whom I now call Moby, and he again did a spectacular first try draw from Maya. Smooth as Porcelain.
Upon my return from Boston, I emailed the ENT and brought him up to speed on Maya's continuing infections and what had occurred in Boston, along with the request for the Ciliary Biopsy. The Immunology specialist explained that a ciliary biopsy was a simple test with a brush a bit up the nose that scrapes off some cilia that can be examined under a microscope. Maya's ENT emailed back that the nasal scrape biopsy has been proven to be clinically useless, and let me know that to even have a 50% shot of a proper biopsy result Maya would need to go under general anesthesia and a sample would need to be taken via a bronchoscopy, not the nose.
Just today I was contacted back by the Immunologist, who had not yet received my email exchanges with the ENT, nor had he been able to win the first round of phone-tag with the ENT. The Immunologist explained that all of Maya's tests, with the exception of the AH-50 (which would take 2-3 weeks longer) came back normal, with excellent protective titers for all of the Prevnar-related pnuemococcal strains and the HiB. He said that from his perspective, her immune system responses were normal, with perhaps a bit of allergic response that doesn't show up in allergy testing. I agreed with that, because Brianna and I get the same allergic symptoms ("hay fever") to mold, ragweed and a few other things several times a year, with the difference being that when it progresses to an infection, Brianna and I respond positively to our first antibiotic, while Maya responds only to incredibly strong antibiotics, which happen to also give her bad diaper Candida rash and, in the case of Bactrim and Levaquin, body bumps.
The Immunologist said he had no explanation for her lack of response, other than suggesting some sort of ENT issue, such as the cilia. He said I should now work with the ENT with regular checkups every six months. When I asked if she should be rechecked annually for the same protective titers she needed a booster for, he said that we should, and that annual boosters might be helpful if the tests indicated lowered titers again.
So now we are left wondering why she is basically clear right now and for the past week (probably the Ceph, as she has finally developed her nasty diaper rash), and what to do to keep her clear in the future. It is very frustrating to have to "wait and see" with no real answers to very serious recurrent infections that often keep her home and unable to participate in daycare or even visiting people or playing outside. While I don't want Maya to have to undergo a surgical procedure, nor do we have the easy funds to pay for it (at least the portion that the insurance doesn't cover in our "donut-hole" period), I really feel that the seriousness of what might be Primary Ciliary Diskinesia (PCD) needs to be discovered or ruled out, so that we can have a plan for her treatment and know what to expect for the future. On the flip side, she may just have back luck and be a sickly kid who will grow out of it sometime in the next few years. Nobody can answer that with any certainty. There are many symptoms of PCD that Maya exhibits, and there are even possible genetic links with diseases associated with PCD on the maternal side. So for now the mystery continues and life goes on.
And of course, please donate to and pray for the Haitians and those helping them!
-Chris
