Goodbye 2018



Happy Holidays and a Happy New Year to you all.  It has been exactly 9 months (to the day) that we received Nolan’s diagnosis.   I couldn’t forget that day and all the emotions it brought if I tried.  It seemed like as soon as we received his diagnosis, Menkes Disease started taking over his little body.  This year has understandably been a HARD year for me and for our family.  But, the love and support that we have received from family and friends has really made an indescribable impact on our lives.  I am so shocked and thankful for those near who bring us meals and for those far who send cards and words of encouragement.  Thank you for following along with Nolan’s Story and for supporting our family.

Nolan has had a pretty good (and busy) December.  At the beginning of November, Nolan’s urologist suggested inserting almost an ounce of diluted antibiotic directly into Nolan’s bladder every single day for 3 hours.  This practice would be an attempt to help decrease his monthly urinary tract infections.  If there’s a chance he would stop getting monthly UTIs, this would delay any more bladder surgery.   Of course we were willing to try, and our amazing nanny, Amy, was on board as well (just like she has been with literally every thing we/she has had to do for him).  So we started the Gentamicin bladder flush every day.  To my surprise and relief, Nolan didn’t get a urinary tract infections the entire month of November.

Nolan had an appointment with his urologist in Chicago on December 13th.  This appointment was just to switch out his bladder button.  I would be the one switching it out while our urologist stood by in case I needed help.  If it went well, I could change it at home every 8 weeks.  Despite being a bit nervous, the bladder button change went very well, and Nolan only fussed a little.  He was happy the second I picked him up.  What a relief!  And I was happy to report to our urologist that it had been 6 weeks without a UTI!

Well of course a few days later (the week we were supposed to fly to Florida to visit my parents), Nolan developed a fever, started throwing up, and was quite cranky: all symptoms of a UTI for him.  I was skeptical, but when we took him in sure enough he had developed a UTI.  He was placed on an antibiotic (Cipro) for 10 days and was noticeably feeling better after two days on the medication.  Nolan still has a chronic cough which hasn’t seemed to go away (since September), and in the past week he seems to have more spit and secretions.  We plan to mention this to his doctors soon and maybe try some allergy medicine.

At the beginning of December, Nolan was able to meet and be photographed by a wonderful lady who started the Precious Baby Project.  Her name is Angela, and she photographs medically fragile babies for parents at no charge.  Angela has gained international recognition for her work and lives right here in Indiana.  She raises awareness for these families and tries to showcase the tubes, medical issues, and sweetness of these babies.  Angela’s main goal is to help families with medically fragile children feel as if they are not alone.  Nolan’s shoot involved zebras because they symbolize being rare.  In the medical field, doctors are told to look for horses and not zebras.  Nolan is our sweet little zebra.



Angela Forker Article #preciousbabyproject

We were still able to travel to Florida this year.  We had originally bought Nolan a seat on the plane in hopes that his FAA-approved car seat could fit in between the seats.  Unfortunately, the only way it would fit was if it was upright and not reclined.  Nolan has very little to no head control and is not able to sit in his car seat unless it is reclined. Because of this, his car seat takes up to much space and would not fit. Luckily we were able to get the seat refunded and I made a mental note to make an appointment with my chiropractor as soon as we got back in town since I would be holding Nolan on the entire flight there and back.  Just for the record, Steve was already so scrunched in his own seat that he was unable to provide Nolan with the necessary head support when he did try to hold him (tall people problems, I guess)!


Nolan did great in the plane, and his big sis was an excellent helper.  That being said, if we plan to keep visiting my parents in Florida for the Christmas holidays, we will need to try and figure out a way to fly with Nolan.  He will be >2 years old and will require his own seat and also be required to be buckled up for taxiing, take-off, and landing.  Having him in my lap obviously won’t be allowed, not to mention I don’t think I would be able to support his weight in another year even if it was allowed.  The disability consultant from the airlines was kind enough to send me the “Approved Restraint Systems for Individuals with Disabilities” resource guide, but unfortunately those seats don’t recline either.  So far, the only way we may be able to go would be to get a restraint seat belt system that would allow Nolan to lay on the seat itself and be strapped in.  On the positive side, we could use this in any vehicle that has a bench seat, on the negative side, we would have to purchase two seats for one 2-year-old child, which is obviously not very economical (aka it’s insane)!   So if anyone has any other ideas, please let me know!!!  Thanks in advance.

EZ ON restraint device
EZ ON restraint device

Nolan has been doing well with the increased Gabapentin although it didn’t stop his flailing arms.  Our pediatric neurologist decided to increase the dose of Onfi medication, and the past couple of weeks we have seen a reduction in spastic arm movements at night.  It does appear that Nolan is sleeping better.  He also seems to be happier in general.

Nolan’s physical therapy has been going very well.  He has not yet started to use the stander because it doesn’t support his arms enough.  We will continue our search for a different one.

Working on our Physical Therapy in Florida

Last, but not least, Nolan transitioned to a new bedroom on our first floor.  We were able to convert our office into his bedroom, and he really didn’t mind not having a closet, so it worked out.  As Nolan grows and continues to gain weight, having his room on the first floor will make it much easier for us to take care of him and minimize carrying him up and down the stairs.  I’m sure more modifications will be needed in the future, but we will tackle those hurdles as they come.

New Bedroom


Thank you all again for keeping tabs on our family.  We are constantly blown away by the support.  Have a happy and safe New Year.  We will cling to the happy parts of 2018, but I’d be lying if I said we were sad to see this year come to a close.  We are hopeful for 2019 and look forward to many more happy memories and hopefully a lot less heartache.


Nolan’s legacy

Nolan passed away today at approximately 3:30 pm.  He passed away peacefully in our arms at home.  Right before he took his last breath, he closed his mouth and gave us a little smile, in true Nolan fashion.

Nolan made an impact on so many people’s lives.  He was truly here for a reason, and he has made us better people and better parents.   He is no longer in discomfort, but oh how we will miss him and his sweet antics.

We have tentative plans to have a funeral at St. Pius X Church in Granger, IN on Monday, February 10th at noon with a viewing at 10 am at the church before the service.  We plan to post more details as they become available.


Nolan’s Final Chapter

We have been told that Nolan only has a few days to live.

December was a rough month with his feeding tube being displaced 5 times within a 45 day span.  We did make it to Florida for a few days.  We were a couple days late and we left early due to Nolan’s health.  While we were there, Steve and I were on Nolan duty 24/7.  He had constant fevers, slept most of the day, was dehydrated, and was up most of the nights.  On a positive note, there were many family members around to help distract Naya, and she had a great time playing with cousins, both new and familiar.

We thought Nolan would bounce back to “baseline” and be his smiley self in January, but we noticed that he had fevers on and off, and his breathing had become more labored.  We noticed it was taking him much longer to recover this time around, and we were seeing less smiles.   Nolan was diagnosed with an ear infection in early January and a UTI a week after that.  He started an antibiotic for the ear infection followed by another antibiotic to treat the UTI and yet another one in attempts to help his breathing in case he had the beginnings of pneumonia.  After all the antibiotics had run their course, we were optimistic.  Unfortunately, his breathing continued to get worse.

At this point, Steve and I were setting our alarms at 3 am to get up and give Nolan some pain medication and anti-anxiety medication that would help him have more restful sleep and ease the discomfort from the labored breathing.   Once 5 am hit, we were both taking turns running downstairs every 10 minutes to suction his mouth, because he was unable to control his secretions.

Daily, Steve and I discussed what no parent should ever have to.  What our goal was for Nolan’s life, what end of life measures we were comfortable with, whether or not we wanted to put him through extensive surgeries to try and prolong his life, etc. We decided that we didn’t want to put Nolan through any major surgeries, and we revisited our personal goal for him daily: quality of life over quantity.    We reached out to our hospice nurse and requested to have a meeting with her and see what our options were to keep Nolan comfortable.  On January 17th, the nurse practitioner (NP) at hospice along with our hospice nurse met with us at home.  Of course, Nolan put on a show with squeals and smiles.  I hadn’t seen him like that in weeks.  Nevertheless, the NP gave Nolan “months”to live.  Although common sense told me that I shouldn’t be surprised, I still was. 

After that, we increased the frequency of pain medication that we were giving Nolan throughout the day.  We had to give him “breaks” during the day and put him in bed with his CPAP breathing machine on to give him a rest from working so hard.  His schedule became more erratic, and we decided to “spoil him” as much as we could with his favorite activities.  Every single thing was dependent upon Nolan and we let him guide us.

This week, our hospice nurse decided she wanted to come more frequently than twice a week:  another clear sign that things were headed South.  Steve, Naya, and I cancelled our trip to Florida that was planned for this weekend.  Nolan was initially going to stay at A Rosie Place while we were gone this weekend, but then our nanny suggested that she stay home with Nolan instead, and we felt much more comfortable with that given his recent decline in health.  Yesterday, we received a call from our nanny and hospice nurse requesting that we “come home soon” because Nolan could pass anytime between now and a few days from now.  They are fairly confident that he won’t make it through the weekend.  Our hospice nurse calls this stage “transitioning”.  They’ve suggested that we stop all feeds and medications aside from around-the-clock vicodin and morphine.  And now, it’s a waiting game.  And as always, but even so much more now, we are fighting over who gets to snuggle him the most.

We ask for your prayers that Nolan is comfortable for however few hours/days that he has here on Earth with us, as well as your understanding that we would like privacy during this extremely challenging and heartbreaking time.

For any patient’s that may be reading this, we sincerely apologize for the inconvenience that some of you will inevitably experience with having your appointments rescheduled or being given the option of seeing a substitute dentist.  We trust in our team to help you all out in our absence.

Wrapping up 2019


Although it’s been ~4 months since posting last, it seems like it’s been only one month. Time is flying!!!

Nolan turned 2 on September 1st!!! We celebrated at our home with both Steve’s immediate family as well as mine.  Also in September, Nolan had his first routine G-J feeding button switched out locally at St Joseph Regional Medical Center (SJRMC). Steve, Naya, and I all accompanied him and were cheering him on. It was an outpatient procedure without any sedation or anesthetic. It took about 30 minutes and went very smoothly! It was truly a relief to know that we had a place in town that could help him for this.  Without his G-J button in place, we cannot provide Nolan with any type of food/nutrition since he can’t eat by mouth.  It was a good thing, too, because Nolan somehow pulled out his G-J tube a couple days before Halloween and back to SJRMC we went.


In October, our dear friends had an adult tricycle race competition which ended up being a surprise fundraiser for Nolan!  “Rollin’ for Nolan” was an absolute blast, and we had friends come in from Chicago and Indianapolis to participate.  They raised quite a bit of money which will be spent on a new special needs swing for Nolan, since he has outgrown the infant swing on our swing set.  Nolan is also outgrowing his bath chair, so we will use some of the funds for that.  The rest of the funds will be donated to A Rosie Place and the Menkes Foundation.  A big thank you to all who attended, participated, and donated!  And a huge thanks to the McCreas for coming up with the creative idea and for hosting 🙂  We love you!

November brought a couple of new appointments with new doctors.  We saw both a urologist at Riley Hospital in Indianapolis as well as a neurologist at Peyton Manning’s Children’s Hospital at St. Vincent in Indianapolis.

Our urologist reviewed different options for Nolan.  We could keep going the way we were with a bladder button, or we could get rid of this “foreign body” in his bladder, which would be ideal.  The foreign body is a vehicle for bacteria to get into an otherwise sterile bladder.   One option involved Nolan urinating on his own, if possible, and us using a catheter through a little opening in his stomach to relieve him a couple of times a day if he couldn’t empty his bladder on his own.  Although a much more complicated surgery, we liked the idea of Nolan being able to do something “normal”.  After some testing of his bladder (called urodynamics), we found that anytime Nolan had to urinate on his own, he would cry out in pain.  This, of course, broke our hearts for a couple of reasons; a) that he was in pain  b) that he couldn’t perform a normal bodily function anymore.  The surgery for Nolan urinating on his own was clearly out of the question.  For now, we have decided to keep going the way we are going.  Nolan seems to be doing fine with his bladder button, and we feel comfortable with it’s maintenance.  Obviously, we know that could change at any moment, and we have a back up plan when it does change.   As far as UTIs, Nolan has seemed to be either dodging them or having “asymtpomatic” ones.  After discussing with our urologist in both Indianapolis and Chicago, we have re-defined what a UTI means for Nolan as it means something completely different for him than a healthy child.

We also saw a new neurologist.  She let us know that our dosages for Nolan’s two seizure meds were quite low.  She ended up increasing his dose of Keppra from 2.5ml to 3.5 ml, which was a large step up.  She also asked us to discontinue the other seizure med (Onfi) because she suspected that it wasn’t doing much for him at this point.  Once discontinuing the Onfi, we saw no change and felt comfortable with that.  Nolan had an overnight EEG, and although he showed no “true seizures”, he is “high risk” for them.  Due to this and the increased twitching during the day, the doctor added a different seizure medicine.  We have been on that for about a month now and have seen slightly less twitching during the day, but nothing significant.

Nolan’s sleep has been quite troubling lately.  In November, he had started crying out in the middle of the night.  Sometimes he would be crying in his sleep and other times while awake.  Some nights he would wake up at 11 pm or 2 am and lay away the entire rest of the night.  After doing some research by asking other Menkes parents, I asked Nolan’s sleep doctor if we could try a different sleep medication, because the one Nolan was on clearly wasn’t helping.   We have recently switched to a different drug, and although I think it may be helping, the dose is extremely low compared to what the other Menkes boys are on.  I’m sure this is because these drugs aren’t recommended in children under the age of 6, and it’s always better to start more cautiously, but I think we are ready to increase the dosage.

Most recently, Nolan gave us a little scare with fever, vomiting, and strange fluid coming out of his stomach at night.  Our GI doctor was worried about a blockage in his stomach.  Of course, when we heard this we were extremely nervous.  There was another Menkes boy who had a blockage which turned into sepsis, and he ended up passing away from the resulting organ failure.  After waiting the weekend (like our doctor recommended) with no improvement, we called Monday and finally received an order for a GI study with contrast to see if there was a blockage.  Then I had to call around locally to see which radiology department could get him in the fastest.  He ended up having an appointment two days later at 7 am at SJRMC.  It turns out that the internal tubing from his G-J button, which is supposed to go from his stomach down to his intestine, had actually looped up into his esophagus and back down into his intestine.  It’s probably that was causing the excess acid build up in the stomach and Nolan’s vomiting.

After we were done in radiology, I called the interventional radiologists and let them know that Nolan would need to have his G-J fixed or replaced once again.  The scheduler, who is absolutely wonderful as I’ve spoken to her many times on the phone, told me to stay put, and she would ask the doctor if they could work Nolan in this morning.  Sure enough, she called me back less than 10 minutes later and asked me to head on up.  Nolan had his G-J button and tubing fixed and was home by 1 pm.  His fever was gone, he had no more strange fluid coming out of his stomach, and the vomiting had ceased as well.  Best of all, there was no blockage.

Now to recap some of the fun Nolan has had in the past few months.   Nolan had fun floating in the pool this summer with friends.  He visited a pumpkin farm and was able to pet a mini-pony named Grandma.  He attended A Rosie Place’s Superhero Halloween party as Spiderman, and he dressed up as a cute, little tiger on Halloween day.  Nolan participates in “Sucker Saturday”, which is a weekly holiday made up by his big sister, Naya.  He loves his baths and even gets to use the kitchen sink as his own personal hot tub on special occasions (while he still fits in it!).   He even managed to make the best of his overnight EEG at the hospital.


As 2019 is wrapping up, we are excited for the holiday season.  We will be able to spend time with both sides of the family.  Although we did not think we would be able to travel with Nolan this year, we will be able to head down to Florida (with Nolan) to see my parents, sister, and our extended family, many of whom have not yet met Nolan.  A HUGE thanks to my parents who are making it possible.   We wish everyone a happy, safe, and healthy holiday season.  Happy New Year!







Summer update on Nolan


Nolan has had an eventful two months!  And I mean that in a good way.   We had two trips to Indianapolis in July.  The first one was on July 5th for an overnight sleep study.  We found out that the CPAP breathing machine was the appropriate treatment for Nolan, rather than something called a BiPAP.  We also found out that he did not need any supplemental oxygen through the breathing mask.  Before, he had been receiving 2 liters of oxygen via a large machine called an oxygen concentrator.  Now, we know he wis okay with regular air.  In between Nolan’s afternoon appointment and his overnight sleep study appointment, Nolan, Steve, and I enjoyed walking around Mass Avenue, grabbing dinner, and visiting some shops.  Nolan loved seeing a new place!

The second time we went to Indianapolis was for the Mulligans for Menkes Charity Golf Fundraiser that was orchestrated by one of Steve’s best friends from childhood.  A huge thank you to Nick Murray and everyone that helped sponsor the event!!!  This time we took both kids and visited the Children’s Museum, went out to dinner, and attended the golf outing.  Naya was even able to visit the Indianapolis Zoo, and she had a blast riding around in a golf cart with her daddy.

*A quick update:  we raised over $31,000 from Nolan’s Menkes March in South Bend!!!

Nolan did get another urinary tract infection in July.  He was having daily fevers for over a week with no other symptoms.  He was treated with two rounds of strong antibiotics which seemed to do the trick.  We also met with his urologist at Chicago Lurie Children’s hospital.  We reviewed other surgical options for Nolan, although none of them would guarantee any drastic reduction in UTIs.  For now, we decided to keep things as is and see how Nolan does.  Of course, the day after we visited the urologist, we noticed blood gushing out of Nolan’s bladder.  We notified his doctor who advised us to keep a close eye on him.  It lasted on and off for a couple of hours, but Nolan had no fever and didn’t seem upset by it in the least.  On the contrary, it gave Steve and me quite the scare!


Nolan had a follow up with his GI doctor and his neurologist in town.  Both appointments went well.  We modified the dosage of one of Nolan’s seizure medications, because we have noticed that he has been becoming much more twitchy and not sleeping as soundly.   We have decided to continue using CBD oil because Nolan is tolerating it well and has been extremely happy since starting the oil.  He has only cried once in 3 months, and that includes having an IV placed, fevers, and a UTI!!!   Last, but not least, we received confirmation that an interventional radiologist at St. Joe Regional Medical Center in Mishawaka is willing to replace Nolan’s G-J tube every 3 months without sedation!  This is great news for many reasons, especially if Nolan has another mishap and his G-J tube comes out.  If something like that happens again, we won’t have to run to Chicago overnight for the procedure and can just stay in town!

Also in July, Nolan was able to meet some of my extended family for the first time.  He enjoyed all of the attention, gifts, and clothes 🙂  Nolan has enjoyed many outings to the library, Target, restaurants, the mall, and even surprised Naya at one of her school field trips!

In August, so far we have celebrated Naya’s birthday, had some pool days, and met with a GI doctor from Riley.  We have decided to establish Nolan with some doctors at Riley Children’s Hospital in Indianapolis for Nolan’s care.  We are fairly certain he will need more surgeries, and it would be nice to stay in-state.  The Riley GI doctor comes to South Bend every two months, and we were able to meet with him on Monday.  He approved of our routine, how much we were feeding Nolan, and his meds.   Nolan is over 30 lbs already!!!  He reassured us that we were doing a great job and said he would take care of us in the event that Nolan needed surgery at Riley.   We are hoping to meet with a neurologist and urologist at Riley as well in the coming months.


EQUIPMENT UPDATE:  Nolan is outgrowing his wheelchair, and we are hoping to get a new custom molded “activity chair” that will be much more supportive for use around the house.  He also received some custom fitted hand braces, ankle braces, and a back brace to provide more support and slow the process of tightness in his muscles, ligaments as well as slow his scoliosis.  He was able to try a new stander, but finding any piece of equipment that has adequate support for Nolan is an extreme challenge.  It’s hard for Steve and I to see Nolan “standing” since we know it’s a feat he will never accomplish on his own, but we are glad he is able to have access to such equipment and hope that it helps improve his circulation.


We are excited and hopeful for September.  Nolan will turn 2 years old on September 1st!!!  He may not have many birthdays in his lifetime, compared to the rest of us, so we plan to make sure he is showered with so much love and attention.

As always, thank you to our family, friends, and communicate who continue to lift us up with good vibes, love, care, support, letters, messages, etc.  Your kindness means more than you know!  🙂

Menkes March…and then the ER…



Happy Father’s Day to all the Dads out there 🙂  Especially our own Steve Zent.

The 1st annual Menkes March is now in the books!!!  Thank you to everyone who came.  We had such a great turnout.  The March was live on WNDU Saturday, June 8th, and we had 300-400 people that came.  Donations are still rolling in, and we have raised over $28,000 for A Rosie Place.  The results are so gratifying because not only did so many people learn about Menkes Disease, but they also learned about A Rosie Place.  It’s unbelievable how many people in our community had never heard of A Rosie Place.  And now, they do!  Most people were in disbelief at how large and successful the event was.  Others who attended were in awe at the sense of community that was apparent.  Steve and I shared both of these thoughts as well.  In part, I’m sure I downplayed the mission, because I was nervous that I may not be able to execute a big, successful fundraiser, since I’ve never done anything like it before.  Personally, I am touched by how many people were willing to listen, spread the word, share their time, and contribute funds and resources to a cause that is so near and dear to my heart.

We would love to thank again our dear family, friends, work team, and nanny as well as the following: Hygo Branding, Martin’s Supermarkets, Meijer, Zoup!, South Bend Venues, Parks, and Arts, ABC57, WNDU, Froggy 102.7, Alpha Dog Agency, Kathy Borlik, Once Upon A Child, Clothes Mentor, Moulton Mold & Machine, Colorful Solutions & Design, Cor-A-Vent, Mosaic Salon, Rocco’s Pizza, Ottawa Dental Lab, Potawatomi Zoo, Rise N’ Roll, Robert Merrill, the Knights of Columbus, Junior League of South Bend, and  A Rosie Place.  We are so privileged and thankful to be a part of such a wonderful community.

A couple of weeks before the Menkes March, Nolan had an elevated temperature and was fussy.  We obtained a urinalysis and culture and sure enough, he had a urinary tract infection.  He went through one round of antibiotics and was still fussy.  We started another round of a different type of antibiotic, and that seemed to clear it up.  We will meet with our urologist at the end of July to discuss Nolan’s infections.  It seems like they are still occurring every couple months, and the bacteria is growing more hardy and becoming harder to treat.  Other options would include surgery to remove the outpouches of his bladder which accumulate urine and cause infections or have surgery to bypass the bladder altogether.  We aren’t thrilled about putting him through another surgery, but it doesn’t hurt to explore the options if it means cutting down on infections.

Then, earlier this week, Nolan was doing physical therapy at home when his G-J feeding tube popped out.  We realized after a couple of hours that his food was collecting in his stomach instead of draining into his intestine.  Nolan’s stomach no longer is able to tolerate his continuous 17 hour feeds, so it was imperative that we replace his G-J tube.  Nolan went to Beacon to have a CT image with contrast to confirm that his J portion (the tube that deposits food into his intestine) had been dislodged.  Sure enough, the tub was coiled up in Nolan’s stomach.  Not good.  We were told that we must take Nolan to Chicago Lurie Children’s Hospital right away because no one in South Bend felt comfortable replacing the G-J tube with or without sedation.   Because we had no way to give Nolan nutrition, I headed to Chicago after work with Nolan, and my mother accompanied us as well.

We started in the ER and Nolan was able to get an IV placed on the first shot.  He was laughing the entire time as I played peek-a-boo with him.  Around 2 am, they finally had a room for us, and I was able to obtain a couch to lay on instead of a chair 🙂  At 9:15 am, Nolan was taken to radiology to have the procedure done.  They did not have to sedate him and the procedure took 15 minutes.  Nolan did great.  He absolutely loved being the hospital and getting showered with attention.  I honestly believe he was bummed to leave.  I, on the other hand, was grateful to head back home, and thankful that Steve was able to cover my patients at work without having to cancel more than 1-2 patients.

Our nanny informed me of a friend whose daughter has the same procedure done here (replacing the G-J tube) in South Bend without sedation.  Her daughter also is medically complex.  I am hoping to contact that doctor and have him meet Nolan and see if he is willing to do this procedure for Nolan since it’s recommended to have the G-J tube replaced every 3 months.

Nolan is doing great and had no soreness or issues with his G-J tube placement from Wednesday 6-12-19.  Later that night, Steve and I switched out his bladder button at home and Nolan had a few tears, but bounced back within 5 minutes.  On Friday 6-14-19, Nolan gave us a little scare when his temperature dropped to 94 degrees all day.  Luckily, after bundling him up in fleece blankets & a hat, sitting in the sun, and giving him lots of cuddles, his temperature slowly started climbing up and was back to normal by Saturday.   Thankfully, Nolan is UTI free and continues to give us smiles and laughs.


Ask and you shall receive



We have been home from the hospital for a couple months now.  Steve, Amy, Nolan, and I have gotten used to the new equipment and routine, for the most part.   Nolan did have another bout of pneumonia at the end of March, but he bounced back after a round of strong antibiotics.

Nolan stayed at A Rosie Place for a few days at the beginning of April so that Steve and I could get away for the first time in a couple of years!  He did spike a high fever during his stay and luckily our nanny was “on call” for us, and she picked him up early and brought him to our house until we came home.  THANK YOU AMY!!!

In April, Nolan developed a UTI.  He hadn’t had one since January.  It had been the longest break from monthly UTIs in 12 months!!!  After another round of antibiotics, Nolan once again bounced back.

Nolan is still battling sleep issues.  They’ve surprisingly gotten worse since we left the hospital in March.  He has only slept through the night less than a dozen times in the past two months.  Although he takes medications to sleep, Nolan still wakes us up most nights crying in his sleep.  Sometimes he wakes himself up doing that as well.  We have spoken with doctors and adjusted his CPAP levels.  Nolan is having a titration sleep study on July 5th in Indianapolis.  The titration study is an overnight sleep study that is supposed to narrow down exactly what settings are best for Nolan.  Hopefully that will help his sleep (and ours!).

Nolan has been a part of the concurrent care program with Center for Hospice.  He has a nurse who visits him twice a week.  Nurse Abby is very sweet and knowledgeable and always willing to help.   We are so thankful that we joined the program.  Recently, I asked Nurse Abby if she knew of a special mattress that circulated air.  I was hoping it would help prevent bed sores for Nolan as well as keep him cooler since he can’t move around during the night.  Less than twelve hours later, Nolan had a brand new twin air-circulating mattress on a hospital bed!  The bed moves up, down, and reclines all with the push of a few buttons.  Now, I have the ability to lay in bed with my sweet boy to read him stories or comfort him when he is fussy.  I’m so glad I asked and so ecstatic that Nurse Abby was so willing to help.

Most of you know that we are having a fundraiser in honor of Nolan and to benefit A Rosie Place.  It is a family walk around the 0.4 mile path of Potawatomi Park.  Please consider joining Nolan on his walk by purchasing tickets online and/or donating if you aren’t able to make it.  Click HERE for the Nolan’s Menkes March website  We asked for support from everyone, and we received it.  I can not believe that we have raised over $16,000!!!  This is all due to the support and generosity of our family, friends, and colleagues.  Thank you so, so much.   We are so blessed to have your thoughts and support 🙂


Thank you to Marisa Oberle for having me on ABC57 South Bend local news at the end of February.  Thank you to Shannan Miller for writing a beautiful article on our family that printed in the March edition of Granger Living Magazine.  Thank you to Carl Stutsman for having me Live on Froggy 102.7 and for Liz Goshert for organizing that!  Thank you to Kathy Borlik for writing a heartfelt and informative article that ran in The South Bend Tribune on Mother’s Day.  Thank you to Kyle O’Connor with Alpha Dog Agency for having us on his podcast, The South Bend Beat.

Also, if you are a golfer and will be in the Indianapolis area on July 12th, one of Steve’s best friend’s (Nick) is hosting a fundraiser in Nolan’s honor.  The money raised will go to the Menkes Foundation for further research on a cure for this awful disease.  Nick’s company, Hygo branding, is also sponsoring our tee shirts for the Menkes March!  Click HERE to sign up for Mulligans for Menkes Golf Outing

We are continuing to keep Nolan’s happiness and quality of life as our #1 goal.   Thank you for helping us do this!


Home sweet home



We have been home for about a week and a half now.  I waited to post because things were a bit messy in the beginning.  We were expecting to go back to our previous routine with less issues and a happier Nolan.  Unfortunately, the first week back was not as expected.  Steve, Amy, and I were flustered trying to figure out all of the new equipment and how to incorporate the new breathing treatments, etc. into the new routine.  Nolan was not himself and very unhappy despite the fact that he was breathing better and was not vomiting at all.  He was also not sleeping much at night and would wake up crying multiple times per night.

The last few days have been much, much better.  Nolan is smiling often and laughing as well.  He seems to have a lot more energy.  He flails his arms, straightens his legs, and points his toes when he gets excited and has gained the nickname “twinkle toes” (whether he likes it or not).  Sometimes, he gets so excited that he almost wiggles his whole body out of his wheelchair!  We are so glad to see his sweet self back again.  The nights are still hit or miss, but we have been trending a little better in the past few days and have only had to get up once or twice (compared to 4-10 times!).  Nolan is tolerating his CPAP breathing mask much better and is also doing better with his shaker vest and nebulizer treatments.  We hope this phase lasts a while with nothing but improvement.  Fingers crossed.

We are continuing to keep Nolan’s happiness and quality of life as our #1 goal.  Recently, we started receiving some help from the Palliative Care division of the Center for Hospice.  Nolan has a very nice nurse who comes to visit twice a week.  She assesses him, takes inventory of his medications, and orders them to be delivered to our house the very next day.   One less thing for us to keep track of!  A nurse is also available to us 24/7 for phone calls and/or home visits.  The goal is to try and stay out of the ER unless absolutely necessary.  We are grateful to them for informing us about this service and being able to take advantage of their help.


Heading Home Soon!



Nolan did well in the ICU and was moved back to the General Medical floor on Saturday evening.  He uses a CPAP breathing mask at night and wears supplemental oxygen all day long.  He has three respiratory treatments per day involving a nebulizer and shaker vest.  The nebulizer helps to thin is mucus secretions in his airway and the shaker vest literally shakes his chest to break up the secretions so that he can cough them up into his mouth.  This seems to be enough to help him breathe more easily and clear his own secretions by coughing and having us suction his mouth.  The last couple of days, Nolan has been acting much more like himself with slight periods of fussiness amidst plenty of smiles.  He has had no fevers and has tolerated his feeds very well.  His feeding schedule will be quite similar to what we were doing before.  The doctors are pleased with Nolan’s progress and we are so happy to avoid/postpone a big life-changing surgery (tracheotomy).

Nolan’s doctors cleared Nolan to go home as long as we have all of the necessary medical equipment delivered on the same day.  We were assigned a case manager at the hospital who communicated the equipment requests with our home health company in South Bend (Alick’s Home Medical).  Alick’s was very efficient at receiving our order and contacting our insurance company.  In less than 24 hours, we had received a verbal OK from the insurance company (something that we were told could take a couple of weeks!).

Steve and Naya are heading back to South Bend at this very moment.  Steve will be picking up some of Nolan’s travel equipment from Alick’s tomorrow morning (portable oxygen and oxygen monitor) and heading back to Chicago with it.  Nolan is required to have this portable oxygen in the car on our trip back to South Bend.   The hospital is unable to provide us with portable oxygen and Alick’s cannot deliver it so far away.   The plan is to be home before 2 pm tomorrow so that the rest of Nolan’s equipment can be delivered to the house by a respiratory therapist who can give us a tutorial.  After that, we will be catching up on sleep, spending time with Naya, taking Nolan to various follow up appointments with doctors at home, adapting to a new routine with Nolan’s additional needs, etc. etc.   I absolutely can not wait to be home 🙂

Thanks again to everyone who has supported in all ways possible!

Things are happening :)



Nolan is 1.5 years old today!  Happy 1/2 birthday 🙂 

Two nights ago Nolan had a sleep study.  It took about two hours to hook him up completely.  He was so darn tired, he didn’t even wake up.  The sleep study came back showing significant obstruction (apnea).  This is something we anticipated, but were surprised at how significant the obstruction really was.


Yesterday Nolan gave us a little scare.  His oxygen levels kept dropping even as he was getting more supplemental oxygen.  After nebulizer treatments (inhaling moist saline air), chest percussion, and deep suction, the respiratory therapists were able to remove a lot of mucus from his lungs and Nolan’s oxygen levels increased.   They decided to continue the nebulizer and percussion treatments every 4 hours.  They also looked at his carbon dioxide levels (CO2) and found that those were higher than normal.  The ICU team came down to evaluate him twice and decided not to admit him to the ICU.

The good news is that his fevers were down and the IV antibiotic (Clindamycin) that he had been getting to treat aspiration pneumonia were most likely a good treatment.

Steve and I met with a palliative care team.  They informed us of all possible options for Nolan.  We were forced to think about decisions that no parent should ever have to discuss for their child.  At that point, we really weren’t convinced that Nolan would get better.

Our doctors decided to offer us the option to move Nolan to ICU to try a high flow respiratory cannula rather than the oxygen cannula that he was using.  They believed the high flow cannula would be more effective in helping Nolan rebound from the aspiration pneumonia.  If Nolan’s breathing did not improve on this high flow oxygen, the next step would be to try a CPAP mask.  If that didn’t work, the last resort would be a breathing tube.  We agreed to move Nolan to the ICU and try the high flow air.

Nolan was moved to the ICU Thursday night around 8 pm.  They decided to skip right to the CPAP mask.  As soon as the mask was on, Nolan’s breathing improved drastically.  He seemed much more comfortable.  Today (Friday), we met with our ICU doctors.  It was decided that we would have a trial run respiratory plan: Nolan would use the CPAP mask all night and during naps only.  He would have the regular oxygen cannula on while awake and we would decrease the oxygen slowly to see if he could go without it.  If that went well for a day and another night, we would be able to move back to the 21st floor.  Then we would need figure out which CPAP mask would fit Nolan best and order all the necessary equipment (mask, monitoring machines, oxygen tanks, shaker vest & machine, etc.) from our home medical company for our home.  Only then could we start discussing discharge.  If Nolan continued to do well, there would be NO tracheotomy!!!


Another piece of the puzzle was the G-J conversion.  It turns out, the interventional radiologists were able to place that TODAY without sedation, just a topical anesthetic around the site!  The procedure took about 20 minutes and Nolan is now being fed Pedialyte through the new G-J tube.  Tomorrow we will be able to try actual feeds with some type of formula.

The last couple of weeks have really been a whirlwind.  I am so glad we ended up here at Lurie Hospital.  Once again, everyone here (doctors, nurses, social workers, respiratory therapists, CNAs, etc.) did not disappoint.  Things seemed to progress slowly at first, but we were pleased to see that they all communicated efficiently and tried every option.  As far as the options, Steve and I feel like our doctors at Lurie left no stone unturned.  We were worried that rushing into different surgeries would leave us with regrets or so many “what if” scenarios.  We feel confident that we have done what we believe is best for our little guy.  He most likely will need a trach at some point, but right now is not the time for him.  I’m thankful that he continues to give us his smiles.  I’m relieved that our daughter has been so adaptive with all of the recent changes to her schedule and her life.  And I’m so glad that my husband is such an amazing partner and Dad.

A huge thank you to my father-in-law for being at our beck and call.  Also to my sister-in-law and her family for housing us and helping us take care of Naya.  Both my Dad and sister put their travel plans on hold to fly to Chicago.  My mom is also trying to fly back from India to come support us.  Our nanny, Amy came and spent the day with Noly on Wednesday.  We have had so many friends at home and in Chicago reach out and offer to bring food, to take care of our dog, to check our mail, a place to stay, and even to take Naya on play dates.  So many more have reached out with words of support and encouragement.   Additionally, Steve and I have had such peace of mind with our amazing team at Zent Family Dentistry and the generous dentists who have offered to come and fill in for us or be on standby.  We even have a dear friend who lives in Indianapolis that is coming up next week to sub for us.  On top of that, she put together a calendar of our classmates who were willing to take time out of their busy schedules to drive to South Bend and help us out too!  I LITERALLY could go on and on and on.  I can’t even believe how lucky we are to have all of you as our support system.  I can’t even begin to figure out how to give you all the thanks that you deserve.  All I can say is THANK YOU and WE LOVE YOU!


Progress so far



Steve is now here in Chicago and we have met with many teams of doctors.  On Monday we met with the sleep medicine doctor.  She stated that we should be seeing other specialties first.  Tuesday (yesterday) was a big day.  We met with the GI team, the pulmonology team, the Ear,Nose, & Throat (ENT) team, and the palliative team.   Nolan had an upper airway scope completed while he was awake and although he vomited during the procedure, they were able to get some good video footage of his airway from his nose to his vocal chords.  The video did show that some parts of his airway were considered “floppy” explaining some of his breathing issues.

The pulmonologist didn’t have much to say and agreed that we need to meet with ENT to determine the options.  The GI team offered two suggestions:  doing a G-J surgery which would be minimally invasive or doing a fundoplication surgery.  They would like to have an upper GI study done.  This would mean injecting some dye into Nolan’s G-tube and watching how it travels through his body.  That would give some information as to whether or not food is emptying the stomach in an appropriate time and manner as well as confirming that his intestines are functioning correctly.  If Nolan’s intestines are working well, that would make for a higher success of fixing most of his vomiting symptoms by the G-J surgery.

The ENT team came by and stated that they would like two more pieces of information.  They would like a sleep study.  The sleep study is more to see whether or not Nolan’s central nervous system (brain) is “remembering” to breathe versus just a physical anatomical obstruction that’s causing his breathing issues.  They also would like to obtain another scope (bronchoscope) that would show his airway from his vocal chords down past his trachea.  The scope would have to be done under light sedation and since Nolan is still having high fevers, they would have to wait until the fevers were gone.

Nolan had his upper GI study completed today.  They injected the dye and took some x-rays.  They then waiting an hour and were going to take more x-rays.  Right before they took more x-rays, Nolan vomited some of the dye.  They obtained x-rays shortly after and saw that there was dye in his trachea and he was aspirating some of it.  The question is: did Nolan aspirate the dye because he vomited?  Or, would he have aspirated the dye even without vomiting?

We were hoping to have Nolan’s sleep study done tonight and the bronchoscope done tomorrow, but because Nolan has high fevers, we know he is aspirating, and his chest x-ray looked a slightly inconclusive for pneumonia, our general medical team has decided to start IV antibiotics and treat for aspiration pneumonia.  The hope is that we can stop his fevers and try to get him a little healthier before doing any kind of sedation and scope.  Unfortunately, this sets our timeline back even further.  Now, the plan is to wait til early next week to have a sleep study and bronchoscope.

These two pieces of information will help the ENT team decide on what treatment may be appropriate.  One option is a tracheotomy (which we already knew).  The other option may be a surgery to improve Nolan’s airway and also remove some glands that cause some of his secretions (remove the submandibular glands and tie off the parotid gland).  The second option is a relatively newer surgery and the doctors can’t tell us whether or not it will work.  The additional tests/information will give them a better idea of whether or not Nolan is even a candidate for that surgery.  At best, it would postpone the need for a tracheotomy, but we don’t know for how long.  Steve and I will have to wait for all the information to be gathered so that the medical teams can conference with each other and communicate to us what the options are.  We are hoping for this to happen mid to late next week.  Until then, Nolan is sleeping quite a bit and still giving us some smiles.

FYI, Tomorrow is Rare Disease Day, so please say an extra prayer for our sweet Nolan.  I wanted to share some amazing photographs that Shanna Hendricks took of our family for the March issue of Granger Living magazine.  You may also see me on abc57 news tomorrow as I had a FaceTime interview with one of the anchors today.   Please excuse my ragged appearance.  The hospital couch hasn’t exactly treated me well 🙂