From Hadley's site:
Wednesday, October 29, 2008 4:30pm
Hello,
We're still here plugging along. It's been another hard week. My Papa Bishop passed away Friday night. He was ready to go and has been ill for quite some time...but, my heart is still quite broken. Do you ever just feel like 'it' never ends? I'm feeling a little emotionally trampled these days. I'm sure things will get 'better'. I hope.
Hadley has had good days and bad days...and days that seem stable when compared to the past several weeks. Her alertness is sort of hit or miss, her nail beds are blue...sometimes up to her first knuckle. Her days and nights are all confused. She doesn't like being moved around a lot...but tolerates it like a champ. She is such a sweet girl.
I have a special prayer request today. I haven't shared this with very many people because I was afraid that I was just being 'paranoid' mom who's in a vulnerable emotional place...but, Keegan needs our prayers. Well, our whole family does. Keegan is losing hearing and vision in his right eye and ear. He has failed both vision and hearing exams. We are quite concerned with this and have already seen our family doctor. There is a genetic disorder that we had discussed with Hadley's doctors early on in her treatment but never really explored. It is called Neurofibromatosis.
What Is NF?
Neurofibromatosis encompasses a set of distinct genetic disorders that cause tumors to grow along various types of nerves and, in addition, can affect the development of non-nervous tissues such as bones and skin. Neurofibromatosis causes tumors to grow anywhere on or in the body.
Types Of Neurofibromatosis
Types Of Neurofibromatosis
Neurofibromatosis (NF) has been classified into three distinct types: NF1, NF2 and Schwannomatosis.
Neurofibromatosis 1 (NF1): also known as von Recklinghausen NF or Peripheral NF. Occurring in 1:3,000 births, web characterized by multiple cafe-au-lait spots and neurofibromas on or under the skin. Enlargement and deformation of bones and curvature of the spine (scoliosis) may also occur. Occasionally, tumors may develop in the brain, on cranial nerves, or on the spinal cord. About 50% of people with NF also have learning disabilities.
Neurofibromatosis 2 (NF2): also known as Bilateral Acoustic NF (BAN), is much rarer occurring in 1:25,000 births. NF2 is characterized by multiple tumors on the cranial and spinal nerves, and by other lesions of the brain and spinal cord. Tumors affecting both of the auditory nerves are the hallmark. Hearing loss beginning in the teens or early twenties is generally the first symptom.
Schwannomatosis: a rare form of NF that has only recently been recognized and appears to affect around 1:40,000 individuals. It is less well understood than NF1 and NF2, and features may vary greatly between patients.
At this point we are still hoping to rule it out...but it will be a process. We are working on the best way to get a referral in to see the Genetics Specialists at Doernbechers/OHSU and in the meantime we have an appointment scheduled to see a pediatric ophthalmologist in Lake Oswego on the 17th. Our doctor's office is also working on getting us in to a pediatric ENT. None of this is being taken lightly and while that 'worries' me I am also very grateful it's being handled so quickly. My poor boy is complaining of headaches almost daily now (probably because of his vision issues) and seems frustrated with school work. That is hard because he loves school and learning. For his to struggle is so out of the norm. Anyway, please pray for our family. It's hard not to be terrified...and my heart is just broken in pieces. We haven't talked to Keegan about any of this yet. He is such a worrier and with all that is going on with Hadley well, I just don't want to traumatize the poor little guy. He will very likely be having an MRI of the brain sometime soon...but, we're hoping to meet with the genetics specialists first. Anyway, please keep this hush hush around our boys for the time being. They really don't need one more thing to worry about.
Baby Hope is thriving in our home. We all love her so much. She is a really fun little kitten with lots of personality. I'm so glad she found her way to us. =) She is funny and sweet and adorable and gives us an extra little someone to love on and giggle over. We're thankful for her.
Thank you for stopping by to check in on us and for praying for our girl and our family.
With Faith, Hope and Love,
Angela
