We're making lemonade folks!! Lots and lots of lemonade! What else can you do when life hands you a boat load of lemons? It's either lemonade or get out the tequila!
It's been a really long winter in our house as usual. Several rounds of croup, one round of pneumonia, feeding struggles, sugar issues (one landed us inpatient again) and 5 months of failed surgery clearance.....so goes life in our house.
There's so much going on I'm not sure where to start. Ear infections, fluid build up and hearing loss is first. We started with chronic ear infections last fall which led to numerous surgery clearance failures so we saw ENT in March. Madilyn showed significant hearing loss and massive fluid build up. Tubes are set to go into her ears on May 18th along with a sedated hearing test (ABR) to determine if the hearing loss is permanent or will be remedied when the fluid is drained. We have our fingers and toes and everything else possible crossed that the tubes will "fix it". They are going to do her eye exam under anesthesia at the same time with possible scleral buckle. We haven't noticed anything off with her vision so we are again hopeful that the fluid pocket has remained stable and her vision will be safe.
On our recent trip to Milwuakee Children's Hospital we had a cluster of appointments (as usual) and we'll start with the good news. GI and Endochrine are super thrilled with Madilyn's growth!! She has grown 4 inches and gained 4 pounds in about 10 months! Endochrine said if she keeps growing at the rate she is she will "catch up" in the next 18 months. That's HUGE! They didn't know if she'd ever grow so this news made me very happy. Endochrine is not so happy with her sugar issues. They've been very significant since November and we do glucose monitoring at home. If the sugar crashes continue then we will have to be inpatient for a controlled critical sugar crash so they can do testing to try and figure out why it does it. Multiple times now Madilyn's sugar has crashed to organ failure and comatose ranges with her showing no outward signs of an issue. I won't lie...it's terrifying to hear that if I put her to bed she never would've woken up. I'll never get used to those words no matter how often we hear them. I'm super in sync with my mommy gut and always trust it.
Now for the lemons....we saw Ortho for our yearly check up. They follow Madilyn due to her Hypotonia. Hypotonia is a state of low muscle tone (the amount of tension or resistance to stretch in a muscle) often involving reduced muscle strength. Madilyn was born with this and it causes her to be "abnormally flexible". Well while the massive growth spurt has it's upside it has a huge downside too..... due to the growth spurt and the hypotonia Madilyn has developed Kyphoscoliosis. She has the s shaped curve of scoliosis in her lower spine and the hunchback curve of the kyphosis in upper spine. This can cause heart and lung issues as well as pulmonary hypertension (high blood pressure) as the curves combat each other and compress (crush) her heart and lungs. They are significant curves and her Ortho specialist wants her in a corrective wheelchair for multiple hours per day. No more high chairs, normal seats, strollers, shopping carts etc. They do not provide the appropriate support for her back. The problem is insurance and the amount of time it takes to get a custom built wheelchair. Realistically we are looking at 6 months to a year. We have to see Ortho again in September and if he does not see improvement she will be fitted for a custom back brace that will go from her hips up over her shoulders (it causes her hips to be out of place also). We will quickly work our way from 10 minutes multiple times per day up to 23 hours per day in the brace and she would wear it for 2 years or more if necessary. If the brace and wheelchair do not help they would insert a metal rod into her spine and she would have surgery every few months to adjust the rod and this is how she would "grow". Some children with kyphoscoliosis end up on oxygen also due to the compression of the heart/lungs. We are hopeful that it won't go this far since it has been caught now. I did extensive research and ordered a "chair" yesterday that will hopefully help until we can get the wheelchair.
The hardest part of the diagnosis is hearing that if we end up going the brace route in September Madilyn will lose all of the mobility we have fought so hard for. The brace is very restrictive with movement and she will have to relearn absolutely everything. She/we can do it, it's just extremely devastating to hear that we very well may end up there.
More lemons....Madilyn snores incredibly loud about 80% of the time, the other 20% it's very difficult to tell if she's even breathing. I brought this to the attention of our ENT doctor at our appointment in March and really felt like she blew it off. When we returned home from that trip I made a video of her snoring and sent it to our care coordination team to get their input. They were very concerned and forwarded it to the doctor. She called me last Friday morning herself apologizing up and down for not listening to my concerns and said Madilyn's video was alarming to her as she clearly stopped breathing in the video. While in Milwaukee they scheduled an emergency sleep study because "Madilyn's way too medically complex to assume anything" now we wait for an eternity. Actually only 7 to 10 days but it feels like an eternity to find out the results. If it's tonsils and adenoids they will remove them when she has surgery on May 18th, if it's something else we'll deal with it once we know. I'm really not able to let my mind go into the unknown because it just causes too much stress and isn't realistic at all.
Everything with Madilyn is an unknown. With her Complex X-chromosome abnormality she is one of a kind in the world and according to hundreds of years of genetic research she should not even be alive. They have no idea what to expect and are shocked and thrilled with all of the things she does, as are we. We live in the moment and deal with things one at a time...we learn to breathe all over again daily with the obstacles that her life throws at us.
Our future is unknown and sometimes quite scary but if Madilyn can keep smiling and thriving and being as happy as she is we'll continue to be thankful for every second that we get...good and bad...please keep us in your prayers for "fixable" sleep study results and that her upcoming surgery goes well as she will be under anesthesia for quite a while to have so many procedures done at once
And when you come to our lemonade stand be sure to wear a smile and little pink shoes..:)
Madilyn's Medical, Travel, and Equipment Fund
