Big Head Baby: "Snow Falling Down"

 Back in August, before I arrived here, a Burmese mother arrived at the hospital in labor. She had not wanted another pregnancy, so she took some medicines to try to abort the baby. The medicines didn't take, though, and she remained pregnant. She did not have any prenatal care, so did not know to take vitamins or alter her nutrition. It is also possible she just wasn't able to obtain any of those things. 

When she was in the prenatal area, a cursory ultrasound showed that the fetus had an abnormally large head, so the mother was transferred to the bigger hospital for a c-section.

The baby girl was born with severe congenital defects, including cleft lip and palate, microphthalmia of her right eye, one shortened arm that never developed a forearm and had a vestigial non-bony finger at the end, abnormal fingers of her other hand and toes of both feet, and of course hydrocephaly.

I have not been able to procure the early imaging of the baby's head, but another volunteer here says around the time of the baby's birth, he was queried about Dandy Walker syndrome. The medics wanted to know what it was. We are presuming that this little girl is the one with Dandy Walker Syndrome. That is when there is an absence of malformation of the cerebellum at the back of the brain and the fourth ventricle. It is often complicated by hydrocephalus. 

The baby was sent to our little hospital for further care. Given its poor prognosis, the bigger hospital did not perform any procedures. They essentially expected her to die within a year or two and have very poor quality of life. 

Unfortunately, the family abandoned the baby at our hospital for the first two months, so our staff looked after the baby girl, whom they nicknamed "Apple" because of her large head. Then the family came back to take care of the baby. Who knows what happened. Maybe they thought she would die quickly, maybe there are cultural interpretations of this magnitude of birth defects, maybe the mother felt guilty for taking the abortive medications or maybe the medicines were harmless and she felt guilty just because she is the mother and her baby had so many problems. Maybe the mom was recovering at home from her c-section. No one knows and it doesn't matter now. 

I saw this baby a few months ago when the parents brought it for outpatient evaluation. The baby was crying and it was the largest head I'd ever seen on a baby. At the time, I couldn't believe its head had been allowed to expand so much. The head was about 2-1/2 times the size of my head but on a (at that time) 4 month old baby. 

Flash forward to this month. Little Apple has been with us almost 2 weeks because she developed a pressure ulcer on the back of her extremely heavy head. The bulbous head is so cumbersome, she cannot move her own neck and lays on her back all day long. Her head probably weighs 12-14 pounds all on its own; baby's total weight is only 22 lbs. The mother has had to go back to work, so the 10-year old sister is the baby's caretaker at home. The sister clearly loves little Apple, and plants soft kisses on the baby's head from time to time and plays with her little feet.

The wound was about 2-3cm diameter when she arrived and not too deep but had begun to eat deeper into the skin layers. On the 2nd or 3rd day here, she developed a high fever. She was started on IV antibiotics and continued to get dressing changes for her head ulcer. I kept trying to educate the mom to take pressure off the ulcer by positioning the pillow adjacent to it, but every time I checked on them, the pillow was back flat under the head. Their baby pillow was also stained and dirty, so I bought a brand new pink and white baby pillow in the market so Apple's head would have a clean surface.

After 3 days of antibiotics, the IV failed and they gave no antibiotics that day. Apple had continued to be febrile every day in spite of the antibiotics anyways. On the 5th day, they were able to give antibiotics again, but I decided to change the medications since it didn't seem like they were working. I started her on broad spectrum IV antibiotics for presumed meningitis, since she had no signs of other sources of infection besides her head wound. Her urine test was clean, lungs sounded clear, she was eating and not having diarrhea. On the 6th day, her fever broke and we were all ecstatic. By we I mainly mean me. For the rest of the staff, they get less excited because they are used to poor prognoses and patients dying rather often. I also know Apple's prognosis sucks, but I still don't want her sick or suffering. Every day I tell the adult staff, "I'll be back. I'm going to see the big head baby."

I carefully reviewed her chart and saw that her initial antibiotic was only dosed at half strength to cover meningitis. So I switched her back to that medicine and increased her dose as well as kept her on 2 of the other antibiotics. Given her age, she didn't necessarily need them, but I cannot perform a lumbar puncture in a hydrocephalus baby due to the increased cranial pressure, so I decided to cover all our bases. 

All was going well and her fever was controlled until the evening of 7th day when her IV failed again. I went to the neonatal staff and asked them to come help. The head medic was able to get a foot IV.  I told the baby's staff that if there was any IV problem overnight, please call neonatal staff again. 

However, the next morning, I was quite upset because when I made it over to the children's department near noon, the staff told me the IV wasn't working all morning. So she hadn't gotten her morning antibiotics. And she was febrile again. They were kind of just waiting around, unsure what to do next. I asked them if they had asked our neonatal department to come try. They said no. In the meantime, I got the ultrasound out and started looking for a vein.  I also suggested that I do a scalp IV because her scalp veins were VERY prominent due to the hydrocephalus. The staff was very scared at that prospect, so we opted to try everything else first.

Sadly, the ultrasound battery died and since it was a Thursday. Why does it matter that it was Thursday? Well! Our ultrasounds are old. The one in our department cannot plug in and charge up its own battery, so we have to run to the outpatient department where they have an identical machine that can plug in and recharge batteries. That outpatient department is only open on Wednesdays and Fridays due to COVID. So we could not exchange the battery because there is no key to the department! I have suggested we keep a spare key in the admin office...

At this time, another staff came to try but when I came back around an hour later, there was still no IV, and no one had told me.  By then, the day staff had gone home, and I was pretty riled up that the baby had missed an entire day of antibiotics, was febrile, and the IV issue hadn't been solved. I got my Butterfly portable ultrasound probe out and looked again. I saw a good vein in her ankle but Unfortunately I also could not get an IV. I'm very good at U/S guided IV's but for some reason I could not visualize their needle tips at all. I was dismayed. 

I called the department head and she suggested a venous cutdown in the morning. I was madly Googling what to do in low resource countries if you only have oral antibiotics for meningitis. Obviously IV is better, but a few studies said oral antibiotics is better than none. So that's what I gave her overnight. 

In the morning, I communicated with one senior staff member and he went to look. Amusingly, he scoured her limbs for peripheral venipuncture sites, and when he could not find one, guess what he did? A scalp IV. Hahahahahaha. I was so happy there was an IV. 

ELATED.

Anyhow, I spoke with the administration about this IV situation and about educating the staff to Ask For Help Immediately if they cannot establish an IV. A meningitis patient cannot go with antibiotics. Hopefully this will change.

I love this little girl so much. She is beautiful to me, even though she may be freakishly horrifying to the public. She coos when you talk to her, she loves to drink her milk, she looks at your with her one wild left eye when you talk or sing to her, she has soft fuzz for hair on her stretched out scalp. 

I asked the mom for the baby's Burmese name, half expecting it to be "Baby Girl Number 2" like in China when the family was hoping for a boy and doesn’t bother to name the baby daughter. But the mom had given her a beautiful name. It means "Snow Falling Down." It is an odd name, since I'm sure they have never even seen snow. Maybe it is a sad name. I don't know. But when I check on this baby, I like to spend extra time talking to her and playing with her and calling her beautiful. So sweet and sad. She doesn't understand what is happening. She's just a 6 month old infant that wants to be fed and comforted and safe. 

I cried with my husband on the phone about this baby. I told him I want to spend a lot of time with her, but I cannot bear to watch her die. I was crying on video call in our admin office. Eventually, her skull will stop being so expandable and this brain fluid will have nowhere to go. Her brain will herniate out of the skull base and she will stop breathing. Sometimes these kids make it to a year, apparently the clinic had one that lived to 2 years old. I just want her to not suffer. To go peacefully and be loved until she goes.