"Due to hypothalamic dysfunction, individuals with PWS have intrinsic abnormalities of sleep/wake cycles. They have more sleep cycles per night and more REM periods, but the overall amount of REM sleep is decreased. Further, their sleep efficiency may be compromised by the occurrence of apneas, increasing their “sleep debt” on a daily basis. Because excessive daytime sleepiness has been correlated with the intrusion of REM sleep into wakefulness, individuals with PWS may appear to have narcolepsy-like symptoms" (Forster, J. Sleep Problems in PWS, http://www.pwsausa.org/medical/SleepProblems.htm).
Before Ellie could start taking HGH (Human Growth Hormone, don't worry, there is a blog all about this coming soon), she was referred to a pediatric sleep medicine doctor. He ordered a sleep study right away. We were sent to the hospital to sleep for the night, and they tested the quality of Ellie's sleep. The technicians applied small electrodes all over Ellie's head and chin to measure brain waves, eye movements, and muscle tone. They hooked her up to a heart monitor, attached leads to her arms and legs, put a device similar to a nasal cannula in her nose, along with a device to measure snoring. Finally, they attached two stretchy belts around her waist to measure her breathing movements.
That first sleep study was terrifying. I had no idea what to expect. Only one parent could be there, so it was just me and my 4 month old. David checked on us frequently. We were having to keep Ellie on a very strict feeding schedule that was so stressful for me at the time, so I was a ball of nerves about that. Once they hooked her up to everything and the technician left, I just remember crying and crying. Seeing your child hooked up to all these wires and leads just did me in, plus the diagnosis was still so raw and recent. I felt alone, and remember really being angry that this had to happen to Ellie that night...the sleep study, the doctor's appointment, the PWS. Ellie did fabulous, though...of course. And, I got through the night as well once I saw that she was sleeping peacefully. The picture below is our 5th sleep study, after we got the hang of these things.
So, clearly because we have had more than 5 sleep studies at this point, it means Ellie has sleep apnea. The very first sleep study showed that she had moderate to severe central sleep apnea. Central apnea is a mis-fire in the brain that makes your lungs forget to breathe. We all have central apnea events at night, but in Ellie's case, she had too many. Her lower muscle tone had a big role to play in this and Dr. Gault hoped with time, age and HGH, that it would improve. Dr. Gault recommended that Ellie be placed on oxygen any time she slept. Miss Anne helped us get oxygen delivered to our house and we were vigilant about making sure she had oxygen anytime she slept. We talked at length to Dr. Gault about everything. All he had to tell us was that getting good sleep was imperative for Ellie's development, and that treating apnea can increase a child's IQ, and we were all in. It makes complete sense, imagine how you feel when you do not get a good night's sleep...cranky, tired, irritable, drained? Add low muscle tone to the mix and it becomes so difficult. Ellie did really well tolerating the oxygen, and we navigated transporting her and the oxygen fairly well. We definitely got lots of stares in public and many, many people would stop us and ask what was wrong. I was a little bit better at responding to people than David wanted to be, as you can imagine. We joked that we were going to start telling people that asked, that Ellie was trying to quit smoking, but it was just really hard for her! Sometimes you just get tired of talking about it. Sense of humor is key.
Ellie stayed on oxygen until right after her first birthday, and then she got a little break from the oxygen for a few months before our next sleep study. At that next sleep study, we hoped and expected to see improvement. There was improvement, but there was still apnea. She still had that darned central sleep apnea, but also had obstructive sleep apnea as well. Obstructive apnea is repeated episodes of complete or partial blockage of the upper airway. It is the apnea associated with snoring. And in Ellie's case, since she is on HGH, it can cause organs like adenoids and tonsils to grow too large. Dr. Gault wasn't concerned about her tonsils needing to come out, but he decided the best treatment plan would be a CPAP, or continuous positive airway pressure. It is a machine that uses mild air pressure to keep airways open. So, Ellie wears this every night to keep her safe, and also help her get the "best possible" sleep that she can get.
Getting to this point, has not been easy. David and I have wanted to throw the CPAP machine out the window many times. There have been many sleepless nights for us, and continue to be. Once Ellie got used to the mask, we gradually increased the time she would wear the CPAP. Eventually, she could wear it through the night! Yay! But then she started waking up and taking off the mask. We have chosen that instead of put Ellie on medication to help her "stay" asleep (so she will not keep taking the CPAP mask off at night), rather will just get up, and keep putting it back on her. I am not going to lie, it is exhausting, but Ellie has yet again shown me how strong she is through all of this. She doesn't put up a fuss when we repeatedly put the mask back on. She has also been a total champ and sweetheart at every sleep study. As she has gotten older, she has gotten upset at some of the sleep studies, which is understandable, but we have worked through it, and overall she has been such a trooper. Every technician she has had falls in love with her and can't get over what a great patient she is when they put all the wires and probes on. That's our girl...strong, brave, and absolutely happy.
We have our next sleep study in June, so please keep us in your thoughts and prayers. Recently, Ellie had surgery for tubes placed in her ears and a partial adenoid removal. It is our hope and prayer that she has made great strides with her quality of sleep, and will no longer have to wear the CPAP at night. Let's hope the results show this, and Ellie knocks her sleep study out of the park!
We have been blessed to be apart of a great organization called Sweet Dreamers. It is a parent led group of individuals that support one another through CPAP, BiPAP and oxygen treatment. They support parents in the early days of getting their child desensitized to the mask, building up to wearing the mask or cannula, and supporting one another through the process. They educate the community on this treatment as well, and we couldn't be more thankful for this group. Below is a picture of Sweet Dreamer bears...one is using his CPAP!
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