It took a long time to believe our daughter has cancer....then it took even longer to believe she has a rare syndrome called ROHHAD - that ONLY 30 or so kids worldwide are diagnosed with. You can't imagine how lost you feel when your child is diagnosed with a disease that has no information and no treatment and most definitely no cure. And somehow through almost a year of reasearching and scouring the internet I have found other children who have ROHHAD and have become friends with their mothers. Such a rare disease and to actually find one is amazing. I know quite a few!
For months I fought with myself and argued with doctors about Haven's diagnosis....that there was no way she had this. I dug and dug through every forum on the internet, every article, compared notes with parents, and even broke down Haven's symptoms one by one in comparison only to find the awful truth that my daughter does in fact have ROHHAD Syndrome.
Over the past 1 1/2 years Haven has been on different chemotherapies testing how the ROHHAD reacts to the different types and different levels of immune suppression. Luckily, Haven has taken on all of these challenges with a smile (mostly) and handled all of the doctor appointments amazingly. She has probably been to over 150 doctor appointments in the past 1 1/2 years - and that might even be a low number. Not counting multiple hospital stays, MRIs, and surgeries.
What does all of this lead up to?
We have been testing to get a closer idea as to what ROHHAD could possibly be. Is it autoimmune or is it something else? We don't have a definite answer but with all the testing we're pretty pretty sure it's autoimmune. I believe Haven has an extremely high immune system that is very strong. My child has never been sick in her life - never had a cold - not even once. She is 5 1/2 years old. If it weren't for cancer and ROHHAD she'd be abnormally too healthy. Not even a runny nose!
Tomorrow Haven will receive a full dose of IVIG - which usually takes an entire day to administer.
Monday Haven will have surgery to remove her toe nails - they have been infected for over a year. I wouldn't have pushed this surgery and we would've continued soaking them and lotioning them if it weren't for the ultimate decision we had to make.
After the surgery on her toes Haven will have a couple of weeks to recover. Once recovered she will undergo a few more tests with Oncology and Pulmonary to prepare her for the next surgery. Removing her Portacath and inserting the Hickman Line.
Right now Haven has a Portacath (above) inserted in her chest that has a tube up her jugular vein and into the vein in her chest. She has a pretty large one due to the fatty tissue around her chest. She loves this thing. She thinks everyone should have one. She never has to get pokes or anything. I can't even tell her she is having this removed...I just can't.
Above picture shows how her Port is sewn in to her chest at the muscle. This is hidden under the skin - it can be felt but not seen (other than the 2 inch scar across her chest)
It involves two incisions, one at the jugular vein and one on the chest wall. The central line (the long white one) is inserted (tunnelled) under the skin of your chest into a vein. The tip of the tube sits in a large vein just above your heart.
The reason we need to change out her Port is because she will need multiple lines for multiple medications to be administered at the same time.
Once Haven recovers from this surgery she will undergo more testing from her doctors to ensure she is ready for the next step. Once again cleared from this surgery and testing Haven will begin her new chemo treatment.This is the big stuff....this has been the part we've been scared of but knew may be coming. We didn't know it would come so soon but the doctors have enough information and are confident with their findings that we will now begin this year long treatment.
Haven will receive 4 hard doses of chemotherapy. She will be admitted in to the inpatient Oncology wing of the hospital. We have read and been told by our doctors all of the risks and we understand the main ones they want us to know about. The doctors are always truthful and tell us everything.
The main risks of this treatment are:
- 2nd rate Leukemia - which is fatal
- high risk infertility
- extreme risk of infection (toe nail removal necessary)
There are a few things we are guaranteed to happen and the doctors wanted us to know so we wouldn't freak out when it does happen:
- hair will fall out
- ulcers ranging from her lips down through her stomach
- very sick and weak
Once Haven begins this new chemo she will remain in isolation for a few months as her body tries to rebuild. They will be bringing all of her counts down to 0. Once she is stable and can move from the hospital we will be put in temporary housing for a few months to ensure she is in a clean and sanitized environment. She will have nursing care and availability to doctors to make sure nothing goes wrong and that she doesn't get an infection. Any infection could kill her in these first few months. During this time we will not be able to have visitors in to see Haven. I do not want anything complicating her recovery. I have been reassured by our doctors that they have not lost a patient due to the treatment and don't plan to start now. This does make me feel good and I know they're not just saying it to make me happy. I feel the sincerity from our doctors and that's nice.
This treatment takes about a full year before Haven's immune system is back up in working order. I will ask that at the end of June we will not be able to give Haven any stuffed animals. She receives and loves the gifts from around the world and stuffed animals are her main pleasure in life. Taking these from her is going to be devastating. But it is a necessity. This weekend we will pack up her "pink" room and put all stuffed animals in large bags and put them in the basement. She will not be able to sleep with them or play with them.
The ultimate goal: Her new immune system will forget that it was attacking her body and instead start helping her body. They do not expect to reverse anything that has already happened but we've seen it reverse before. They only hope to stop it from progressing.
We ask that you please think of Haven during this rough time. She has this weekend to enjoy before everything starts. I plan to get her photos done one more time before we start. We are nervous, scared, and emotionally drained. We have no idea how we're going to handle this or how we're going to handle seeing her like this. But the good chance that this could help her and give her a normal life will bring us strength.
thank you to everyone who has followed along with Haven over the past 1 1/2 years. Your dedication to learning about our daughter and these rare diseases is definitely keeping me going.
All our love to our family and friends for understanding the position we are in and supporting us in our decision to move forward with this treatment. We appreciate all you have done for us and Haven and what you continue to do. When she is ready we'll be planning a large party and hope that all family members can come out.
Take care - and wish miss Haven many blessings through these next few surgeries and few months of chemotherapy.